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Shimomiya Y, Nagao M, Kogure T, Asagai S, Inoue A, Yamamoto A, Sakai S, Inai K, Shirasaka T, Kojima T, Yabuuchi H. Right Ventricular Strain With 4-Dimensional Computed Tomography Identifies Pulmonary Hypertension in Adults With Repaired Tetralogy of Fallot. Circ J 2024; 89:62-68. [PMID: 39168609 DOI: 10.1253/circj.cj-24-0386] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 08/23/2024]
Abstract
BACKGROUND This study evaluated right ventricular (RV) volume, strain, and morphology using cardiac 4-dimensional computed tomography (4D-CT) to detect pulmonary hypertension (PH) in adults with repaired tetralogy of Fallot (TOF) scheduled for transcatheter pulmonary valve implantation (TPVI). METHODS AND RESULTS Using cardiac 4D-CT data, we calculated RV strain in 3 different geometries and RV outflow tract (RVOT) mass in 42 patients with repaired TOF. We compared RV strain and RVOT mass between patients with and without PH. Receiver operating characteristic (ROC) analysis was conducted to evaluate the diagnostic performance of these measurements for identifying PH. Four-chamber (4ch) strain was significantly smaller for patients with (n=10) than without (n=32) PH (8.8±1.7% vs. 11.1±2.4%, respectively; P<0.01), whereas RVOT mass was significantly larger in the PH group (12.5±3.5 vs. 9.2±3.2 cm2; P<0.01). ROC analysis of the diagnostic performance revealed that the respective sensitivity and specificity was 70% and 84% (area under the curve [AUC]=0.784) for 4ch strain of 8.8%; 80% and 69% (AUC=0.766) for RVOT mass of 10.7 cm2; and 80% and 81% (AUC=0.844) for a 4ch strain/RVOT mass ratio of 0.97. CONCLUSIONS RVOT mass and 4ch strain obtained from cardiac 4D-CT may be helpful for identifying PH in patients with repaired TOF.
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Affiliation(s)
- Yamato Shimomiya
- Department of Health Sciences, Graduate School of Medical Sciences, Kyushu University
| | - Michinobu Nagao
- Department of Diagnostic Imaging and Nuclear Medicine, Tokyo Women's Medical University
| | - Tomohito Kogure
- Department of Adult Congenital Heart Disease and Pediatric Cardiology, Tokyo Women's Medical University
| | - Seiji Asagai
- Department of Adult Congenital Heart Disease and Pediatric Cardiology, Tokyo Women's Medical University
| | - Akihiro Inoue
- Department of Diagnostic Imaging and Nuclear Medicine, Tokyo Women's Medical University
| | - Atsushi Yamamoto
- Department of Diagnostic Imaging and Nuclear Medicine, Tokyo Women's Medical University
| | - Shuji Sakai
- Department of Diagnostic Imaging and Nuclear Medicine, Tokyo Women's Medical University
| | - Kei Inai
- Department of Adult Congenital Heart Disease and Pediatric Cardiology, Tokyo Women's Medical University
| | - Takashi Shirasaka
- Division of Radiology, Department of Medical Technology, Kyushu University Hospital
| | - Tsukasa Kojima
- Division of Radiology, Department of Medical Technology, Kyushu University Hospital
| | - Hidetake Yabuuchi
- Department of Health Sciences, Faculty of Medical Sciences, Kyushu University
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Singh M, Roubertie F, Ozturk C, Borchiellini P, Rames A, Bonnemain J, Gollob SD, Wang SX, Naulin J, El Hamrani D, Dugot-Senant N, Gosselin I, Grenet C, L'Heureux N, Roche ET, Kawecki F. Hemodynamic evaluation of biomaterial-based surgery for Tetralogy of Fallot using a biorobotic heart, in silico, and ovine models. Sci Transl Med 2024; 16:eadk2936. [PMID: 38985852 DOI: 10.1126/scitranslmed.adk2936] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/23/2023] [Revised: 03/12/2024] [Accepted: 06/20/2024] [Indexed: 07/12/2024]
Abstract
Tetralogy of Fallot is a congenital heart disease affecting newborns and involves stenosis of the right ventricular outflow tract (RVOT). Surgical correction often widens the RVOT with a transannular enlargement patch, but this causes issues including pulmonary valve insufficiency and progressive right ventricle failure. A monocusp valve can prevent pulmonary regurgitation; however, valve failure resulting from factors including leaflet design, morphology, and immune response can occur, ultimately resulting in pulmonary insufficiency. A multimodal platform to quantitatively evaluate the effect of shape, size, and material on clinical outcomes could optimize monocusp design. This study introduces a benchtop soft biorobotic heart model, a computational fluid model of the RVOT, and a monocusp valve made from an entirely biological cell-assembled extracellular matrix (CAM) to tackle the multifaceted issue of monocusp failure. The hydrodynamic and mechanical performance of RVOT repair strategies was assessed in biorobotic and computational platforms. The monocusp valve design was validated in vivo in ovine models through echocardiography, cardiac magnetic resonance, and catheterization. These models supported assessment of surgical feasibility, handling, suturability, and hemodynamic and mechanical monocusp capabilities. The CAM-based monocusp offered a competent pulmonary valve with regurgitation of 4.6 ± 0.9% and a transvalvular pressure gradient of 4.3 ± 1.4 millimeters of mercury after 7 days of implantation in sheep. The biorobotic heart model, in silico analysis, and in vivo RVOT modeling allowed iteration in monocusp design not now feasible in a clinical environment and will support future surgical testing of biomaterials for complex congenital heart malformations.
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Affiliation(s)
- Manisha Singh
- Institute for Medical Engineering and Science, Massachusetts Institute of Technology, Cambridge, MA 02142, USA
| | - François Roubertie
- IHU Liryc, Electrophysiology and Heart Modeling Institute, F-33604 Pessac, France
- Congenital Heart Diseases Department, CHU de Bordeaux, F-33604 Pessac, France
| | - Caglar Ozturk
- Institute for Medical Engineering and Science, Massachusetts Institute of Technology, Cambridge, MA 02142, USA
| | - Paul Borchiellini
- University of Bordeaux, INSERM, BioTis, U1026, F-33000 Bordeaux, France
| | - Adeline Rames
- University of Bordeaux, INSERM, BioTis, U1026, F-33000 Bordeaux, France
| | - Jean Bonnemain
- Institute for Medical Engineering and Science, Massachusetts Institute of Technology, Cambridge, MA 02142, USA
- Department of Adult Intensive Care Medicine, Lausanne University Hospital and University of Lausanne, CH-1011 Lausanne, Switzerland
| | - Samuel Dutra Gollob
- Department of Mechanical Engineering, Massachusetts Institute of Technology, Cambridge, MA 02139, USA
| | - Sophie X Wang
- Institute for Medical Engineering and Science, Massachusetts Institute of Technology, Cambridge, MA 02142, USA
- Department of Surgery, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA
| | - Jérôme Naulin
- IHU Liryc, Electrophysiology and Heart Modeling Institute, F-33604 Pessac, France
| | - Dounia El Hamrani
- IHU Liryc, Electrophysiology and Heart Modeling Institute, F-33604 Pessac, France
| | - Nathalie Dugot-Senant
- Plateforme d'histopathologie, TBMcore INSERM US005-CNRS 3427, F-33000 Bordeaux, France
| | - Isalyne Gosselin
- Plateforme d'histopathologie, TBMcore INSERM US005-CNRS 3427, F-33000 Bordeaux, France
| | - Célia Grenet
- University of Bordeaux, INSERM, BioTis, U1026, F-33000 Bordeaux, France
| | - Nicolas L'Heureux
- University of Bordeaux, INSERM, BioTis, U1026, F-33000 Bordeaux, France
| | - Ellen T Roche
- Institute for Medical Engineering and Science, Massachusetts Institute of Technology, Cambridge, MA 02142, USA
- Department of Mechanical Engineering, Massachusetts Institute of Technology, Cambridge, MA 02139, USA
| | - Fabien Kawecki
- University of Bordeaux, INSERM, BioTis, U1026, F-33000 Bordeaux, France
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Ho YL, Khamis AY, Abdul Kareem BA. Left anterior mini-thoracotomy: an alternative approach for pulmonary valve replacement after surgically corrected tetralogy of fallot. J Cardiothorac Surg 2024; 19:427. [PMID: 38982433 PMCID: PMC11234653 DOI: 10.1186/s13019-024-02817-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/08/2024] [Accepted: 05/25/2024] [Indexed: 07/11/2024] Open
Abstract
INTRODUCTION Pulmonary regurgitation (PR) remains a common sequela in patients following surgically corrected TOF, and may lead to progressive right ventricle dilatation and dysfunction. The conventional approach of redo-sternotomy for pulmonary valve replacement (PVR) is associated with increased operative time as well as risks of bleeding and injury to the heart and great vessels. Thus, left anterior mini-thoracotomy has become an alternative approach in eliminating the risks of redo-sternotomy in these patients. This series aimed to determine the outcomes of minimally invasive pulmonary valve replacement after surgical TOF correction. METHODS A retrospective analysis was conducted on 24 patients with severe PR post-surgical TOF correction who underwent left anterior mini-thoracotomy PVR in Penang General Hospital from January 2021 to January 2023. RESULTS The median age was 23.5 years (I.Q.range 17.6-36.3), with a male:female ratio of 1:4. Majority of patients had mild to moderate symptoms prior to surgery and 19 patients (79.1%) were on regular diuretics medication. All patients had severe free-flow PR with evidence of right ventricular dilatation and dysfunction. Magnetic Resonance Imaging and computed tomography of pulmonary artery were performed prior to surgery. Minimally invasive PVR was performed on all patients via left upper anterior mini-thoracotomy and femoral-femoral bypass without cardioplegic arrest. The operative time and cardiopulmonary bypass time were 208 (I.Q.range 172-324) and 98.6 minutes(I.Q.range 87.4-152.4) respectively. The time to wean off inotropes postoperatively was 6.2 hours (I.Q.range1.4-14.8), and no postoperative arrhythmia and chest re-exploration were reported. Most patients stayed in Intensive Care Unit (ICU) for 10.8 hours (I.Q.range 8.4-36.5), and the total hospital stay was 4.2 days (I.Q.range 3.4-7.6). 2 patients (11.1%) required blood transfusion postoperative. There was no paravalvular leak and no mortality during the follow-up period of up to 28 months. CONCLUSION Minimally invasive PVR after surgical correction of TOF is a safe alternative to the conventional redo-sternotomy approach in patients with favorable anatomy. This approach is able to reduce the risks associated with redo-sternotomy, particularly bleeding and injury to mediastinal structures, with the additional benefit of expedited recovery and hospital discharge. Our series has shown a safe and efficient approach in these patients with favorable outcomes.
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Affiliation(s)
- Yan Le Ho
- Department of Cardiothoracic Surgery, Penang General Hospital, George Town, Penang, Malaysia.
| | - Abu Yamin Khamis
- Department of Cardiothoracic Surgery, Penang General Hospital, George Town, Penang, Malaysia
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Liu Y, Xu R, Zhou D, Yang Y, Xu G, Zeng S. Pulmonary artery stiffness in fetuses with tetralogy of Fallot. Ultrasonography 2024; 43:220-227. [PMID: 38715210 PMCID: PMC11079504 DOI: 10.14366/usg.23196] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/21/2023] [Revised: 04/10/2024] [Accepted: 04/11/2024] [Indexed: 05/12/2024] Open
Abstract
PURPOSE This study evaluated the elastic characteristics of the pulmonary trunk and distal branches in fetuses diagnosed with tetralogy of Fallot (TOF) using Doppler echocardiography. METHODS Data on 42 fetuses diagnosed with TOF and 84 gestational age-matched normal fetuses were prospectively collected from the Second Xiangya Hospital of Central South University between August 2022 and January 2023. The severity of TOF was classified into three categories based on the z-score of the pulmonary annulus diameter: mild (z-score ≥-2), moderate (-4 RESULTS Compared with the normal group, the MPA-PAS in fetuses with TOF was significantly higher, while the DLPA-PAS and DRPA-PAS were notably lower (all P<0.05). The MPA-PAS of fetuses with severe TOF was higher than that of those with mild and moderate TOF (all P<0.05). However, there were no significant differences in the DLPA-PAS or DRPA-PAS among fetuses with mild, moderate, and severe TOF (all P>0.05). CONCLUSION Fetuses diagnosed with TOF exhibited increased vascular stiffness in the MPA and reduced stiffness in the distal pulmonary artery (PA). Larger-scale follow-up studies are required to elucidate the relationships between these changes in vascular stiffness and PA development in patients with TOF.
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Affiliation(s)
- Yushan Liu
- Department of Ultrasound Diagnosis, Second Xiangya Hospital, Central South University, Changsha, China
| | - Ran Xu
- Department of Urology, Second Xiangya Hospital, Central South University, Changsha, China
| | - Dan Zhou
- Department of Ultrasound Diagnosis, Second Xiangya Hospital, Central South University, Changsha, China
| | - Yang Yang
- Department of Ultrasound Diagnosis, Second Xiangya Hospital, Central South University, Changsha, China
| | - Ganqiong Xu
- Department of Ultrasound Diagnosis, Second Xiangya Hospital, Central South University, Changsha, China
| | - Shi Zeng
- Department of Ultrasound Diagnosis, Second Xiangya Hospital, Central South University, Changsha, China
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Arvanitaki A, Diller G, Giannakoulas G. The Right Heart in Congenital Heart Disease. Curr Heart Fail Rep 2023; 20:471-483. [PMID: 37773427 DOI: 10.1007/s11897-023-00629-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 09/17/2023] [Indexed: 10/01/2023]
Abstract
PURPOSE OF REVIEW To analyze the pathophysiologic importance of the right heart in different types of congenital heart disease (CHD), summarize current diagnostic modalities, and discuss treatment options. RECENT FINDINGS The right ventricle (RV) plays a key role in disease progression and prognosis, either as the subpulmonary or as the systemic ventricle. Volume and/or pressure overload as well as intrinsic myocardial disease are the main factors for RV remodeling. Echocardiography and cardiac magnetic resonance imaging are important noninvasive modalities for assessing anatomy, size, and function of the right heart. Timely repair of related lesions is essential for preventing RV dysfunction. Few inconclusive data exist on conventional pharmacotherapy in CHD-related RV dysfunction. Cardiac resynchronization therapy and ventricular assist devices are an option in patients with advanced systemic RV failure. Right heart disease is highly related with adverse clinical outcomes in CHD. Research should focus on early identification of patients at risk and development of medical and interventional treatments that improve RV function.
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Affiliation(s)
- Alexandra Arvanitaki
- 1st Department of Cardiology, AHEPA University Hospital, St. Kiriakidi 1, 54621, Thessaloniki, Greece
- Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield NHS Foundation Trust, London, UK
| | - Gerhard Diller
- Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield NHS Foundation Trust, London, UK
- Department of Cardiology III-Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Albert-Schweitzer-Campus 1, Muenster, Germany
- National Register for Congenital Heart Defects, Berlin, Germany
| | - George Giannakoulas
- Department of Cardiology, AHEPA University Hospital, School of Medicine, Aristotle University of Thessaloniki, St. Kiriakidi 1, 54636, Thessaloniki, Greece.
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Yin X, Wang Y. Effect of pulmonary regurgitation on cardiac functions based on a human bi-ventricle model. COMPUTER METHODS AND PROGRAMS IN BIOMEDICINE 2023; 238:107600. [PMID: 37285726 DOI: 10.1016/j.cmpb.2023.107600] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 12/23/2022] [Revised: 04/27/2023] [Accepted: 05/13/2023] [Indexed: 06/09/2023]
Abstract
BACKGROUND AND OBJECTIVE Assessing the severity of pulmonary regurgitation (PR) and identifying optimal clinically relevant indicators for its treatment is crucial, yet standards for quantifying PR remain unclear in clinical practice. Computational modelling of the heart is in the process of providing valuable insights and information for cardiovascular physiology research. However, the advancements of finite element computational models have not been widely applied to simulate cardiac outputs in patients with PR. Furthermore, a computational model that incorporates both the left ventricle (LV) and right ventricle (RV) can be valuable in assessing the relationship between left and right ventricular morphometry and septal motion in PR patients. To enhance our understanding of the effect of PR on cardiac functions and mechanical behaviour, we developed a human bi-ventricle model to simulate five cases with varying degrees of PR severity. METHODS This bi-ventricle model was built using a patient-specific geometry and a widely used myofibre architecture. The myocardial material properties were described by a hyperelastic passive constitutive law and a modified time-varying elastance active tension model. To simulate realistic cardiac functions and the dysfunction of the pulmonary valve in PR disease cases, open-loop lumped parameter models representing systemic and pulmonary circulatory systems were designed. RESULTS In the baseline case, pressures in the aorta and main pulmonary artery and ejection fractions of both the LV and RV were within normal physiological ranges reported in the literature. The end-diastolic volume (EDV) of the RV under varying degrees of PR was comparable to the reported cardiac magnetic resonance imaging data. Moreover, RV dilation and interventricular septum motion from the baseline to the PR cases were clearly observed through the long-axis and short-axis views of the bi-ventricle geometry. The RV EDV in the severe PR case increased by 50.3% compared to the baseline case, while the LV EDV decreased by 18.1%. The motion of the interventricular septum was consistent with the literature. Furthermore, ejection fractions of both the LV and RV decreased as PR became severe, with LV ejection fraction decreasing from 60.5% at baseline to 56.3% in the severe case and RV ejection fraction decreasing from 51.8% to 46.8%. Additionally, the average myofibre stress of the RV wall at end-diastole significantly increased due to PR, from 2.7±12.1 kPa at baseline to 10.9±26.5 kPa in the severe case. The average myofibre stress of the LV wall at end-diastole increased from 3.7±18.1 kPa to 4.3±20.3 kPa. CONCLUSIONS This study established a foundation for the computational modelling of PR. The simulated results showed that severe PR leads to reduced cardiac outputs in both the LV and RV, clearly observable septum motion, and a significant increase in the average myofibre stress in the RV wall. These findings demonstrate the potential of the model for further exploration of PR.
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Affiliation(s)
- Xueqing Yin
- School of Mathematics and Statistics, University of Glasgow, Glasgow, United Kingdom
| | - Yingjie Wang
- School of Mathematics and Statistics, University of Glasgow, Glasgow, United Kingdom.
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7
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Arya N, Schievano S, Caputo M, Taylor AM, Biglino G. Relationship between Pulmonary Regurgitation and Ventriculo-Arterial Interactions in Patients with Post-Early Repair of Tetralogy of Fallot: Insights from Wave-Intensity Analysis. J Clin Med 2022; 11:6186. [PMID: 36294505 PMCID: PMC9604580 DOI: 10.3390/jcm11206186] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2022] [Revised: 10/07/2022] [Accepted: 10/18/2022] [Indexed: 11/16/2022] Open
Abstract
This study aimed to investigate the effect of pulmonary regurgitation (PR) on left ventricular ventriculo-arterial (VA) coupling in patients with repaired tetralogy of Fallot (ToF). It was hypothesised that increasing PR severity results in a smaller forward compression wave (FCW) peak in the aortic wave intensity, because of right-to-left ventricular interactions. The use of cardiovascular magnetic resonance (CMR)-derived wave-intensity analysis provided a non-invasive comparison between patients with varying PR degrees. A total of n = 201 patients were studied and both hemodynamic and wave-intensity data were compared. Wave-intensity peaks and areas of the forward compression and forward expansion waves were calculated as surrogates of ventricular function. Any extent of PR resulted in a significant reduction in the FCW peak. A correlation was found between aortic distensibility and the FCW peak, suggesting unfavourable (VA) coupling in patients that also present stiffer ascending aortas. Data suggest that VA coupling is affected by increased impedance.
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Affiliation(s)
- Nikesh Arya
- Faculty of Mathematical and Physical Sciences, University College London, London WC1E 6BT, UK
| | - Silvia Schievano
- Institute of Cardiovascular Science, University College London, London WC1E 6BT, UK
- Centre for Cardiovascular Imaging, Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3HJ, UK
| | - Massimo Caputo
- Bristol Medical School, Faculty of Health Sciences, University of Bristol, Bristol BS8 1TH, UK
- Bristol Heart Institute, University Hospitals Bristol & Weston NHS Foundation Trust, Bristol, BS2 8HW, UK
| | - Andrew M. Taylor
- Institute of Cardiovascular Science, University College London, London WC1E 6BT, UK
- Centre for Cardiovascular Imaging, Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3HJ, UK
| | - Giovanni Biglino
- Bristol Medical School, Faculty of Health Sciences, University of Bristol, Bristol BS8 1TH, UK
- National Heart and Lung Institute, Imperial College London, London SW7 2BX, UK
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Samal S, Sharma R, Minhas HS, G G, Agarwal S, Naqvi SEH, Geelani MA. Role of great artery annulus ratio to predict transannular patch enlargement in repair of tetralogy of Fallot. THE CARDIOTHORACIC SURGEON 2022. [DOI: 10.1186/s43057-022-00086-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/10/2022] Open
Abstract
Abstract
Background
Traditionally, the pulmonary valve annulus (PVA) z-score is used to predict the requirement of transannular patch enlargement (TAPE) of right ventricular outflow tract (RVOT) and main pulmonary artery (MPA) in repair of tetralogy of Fallot (TOF). PVA z-score is highly variable and many other parameters are being reported to be accurate in predicting need of TAPE. In this study, we analyze the role of great artery annulus ratio (pulmonary valve annulus to aortic valve annulus ratio, PVA/AVA) to be used as a predictor for TAPE.
Methods
We analyzed 90 patients of TOF retrospectively who underwent repair between January 2021 and December 2021. The patients were divided as TAPE group who required TAPE of RVOT and MPA and non-TAPE group who did not have TAPE. Their baseline parameters, PVA Z-score, and cut-off great artery annulus ratio were compared.
Results
Total 44 (48.9%) patients had transannular patch (TAPE) repair and 46 (51.1%) patients had non-transannular patch repair. The great artery annulus ratio and PVA z-score was lower in case of TAPE group with statistical significance. Receiver operating characteristic curve analysis showed great artery annulus ratio as more accurate predictor of TAPE.
Conclusion
Great artery annulus ratio can be used as a simple and accurate predictor for transannular patch enlargement along with PVA z-score during repair of TOF.
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Guariento A, Schiena CA, Cattapan C, Avesani M, Doulamis IP, Padalino MA, Castaldi B, di Salvo G, Vida V. Pulmonary valve preservation during tetralogy of Fallot repair: midterm functional outcomes and risk factors for pulmonary regurgitation. EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY : OFFICIAL JOURNAL OF THE EUROPEAN ASSOCIATION FOR CARDIO-THORACIC SURGERY 2022; 62:6644955. [PMID: 35848949 DOI: 10.1093/ejcts/ezac365] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 02/07/2022] [Revised: 06/06/2022] [Accepted: 06/27/2022] [Indexed: 01/01/2023]
Abstract
OBJECTIVES Many centres have recently adopted pulmonary valve (PV) preservation (PVP) during tetralogy of Fallot (ToF) repair. We sought to identify the midterm functional outcomes and risk factors for pulmonary regurgitation after this procedure. METHODS All patients undergoing PVP during transatrial-transpulmonary repair for ToF with PV stenosis at our institution between January 2007 and December 2020 were reviewed. RESULTS Overall, 73 patients were included. At the index surgery, the body surface area was 0.31 ± 0.04 m2, the age was 4.9 ± 2.9 months and the preoperative PV z-score was -3.02 ± 1.11. At a mean follow-up of 5.3 ± 2.7 years, the fractional area change of the right ventricle (RV) was 47.1 ± 5.2%, and the tricuspid annular plane systolic excursion z-score was -3.31 ± 1.89%. The 5-year freedom from moderate/severe PV regurgitation was 61.3% [95% confidence interval (CI): 48, 73%]. There was a significant correlation between RV function and moderate/severe PR at follow-up (R2: 0.08; P = 0.03). A comparison with a group of patients undergoing a transannular patch procedure (N = 33) showed superior outcomes for patients with PVP. The preoperative PV z-score and the degree of PR at discharge were risk factors for the early development of moderate/severe PR at follow-up [hazard ratio (HR): 0.64; 95% CI: 0.48, 0.86, P = 0.01 and HR: 2.31; 95% CI: 1.00, 5.36, P = 0.04, respectively]. A preoperative PV annulus z-score ≤ -2.85 was found to be predictive for moderate/severe PR at 5 years after PVP (HR: 2.56; 95% CI: 1.31, 5.01, P = 0.002). CONCLUSIONS A pulmonary valve preservation strategy during tetralogy of Fallot repair should always be attempted. However, a preoperative PV annulus z-score < -2.85 and moderate/severe regurgitation upon discharge are risk factors for midterm pulmonary regurgitation.
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Affiliation(s)
- Alvise Guariento
- Pediatric and Congenital Cardiac Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padua, Padua, Italy
| | - Chiara A Schiena
- Pediatric and Congenital Cardiac Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padua, Padua, Italy
| | - Claudia Cattapan
- Pediatric and Congenital Cardiac Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padua, Padua, Italy
| | - Martina Avesani
- Pediatric Cardiology Unit, Departments of Women's and Children's Health, University of Padua, Padua, Italy
| | - Ilias P Doulamis
- Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
| | - Massimo A Padalino
- Pediatric and Congenital Cardiac Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padua, Padua, Italy
| | - Biagio Castaldi
- Pediatric Cardiology Unit, Departments of Women's and Children's Health, University of Padua, Padua, Italy
| | - Giovanni di Salvo
- Pediatric Cardiology Unit, Departments of Women's and Children's Health, University of Padua, Padua, Italy
| | - Vladimiro Vida
- Pediatric and Congenital Cardiac Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padua, Padua, Italy
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Bhende VV, Sharma TS, Mehta DV, Subramaniam KG, Kumar A, Thacker JP, Patel V, Panesar G, Soni K, Dhami KB, Majmudar H, Patel N, Pathan SR. Higher pulmonary vein index on computed angiography and optimum surgical resection ensures smooth postoperative recovery in Fallot's tetralogy: Special emphasis on indices of evaluation and Monocusp preparation. Clin Case Rep 2022; 10:e6100. [PMID: 35912370 PMCID: PMC9327839 DOI: 10.1002/ccr3.6100] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/19/2022] [Revised: 06/29/2022] [Accepted: 07/02/2022] [Indexed: 11/17/2022] Open
Abstract
Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease. Its surgical correction requires ventricular septal defect (VSD) closure and right ventricular outflow tract obstruction (RVOTO) relief, with transannular patch enlargement (TAPE) of the pulmonary valve. The first successful repair of TOF was reported in 1954 and consisted of closure of the VSD through a large right ventriculotomy, and RVOTO relief with TAPE of the pulmonary valve. To predict the intraoperative requirements and postoperative course of patients with this condition, various evaluation indices are available that can provide a good indication of patient prognosis. We performed this study in a male child (age, 1 year, 9 months; weight 8.5 kgs.) who underwent intracardiac repair for TOF as a primary procedure. We calculated the pulmonary vein index (PVI), McGoon ratio, and Nakata index. The McGoon ratio was 1.97, Nakata index was 539.22 mm2/m2, and PVI was 368.12 mm2/m2. The child had an uneventful post-operative course with no symptoms of low cardiac output syndrome. He was ventilated for 122 h. The length of intensive care unit and hospital stays were 11 and 14 days, respectively. The PVI is a novel indicator offering prognostic indications for pediatric cardiac patients who have undergone surgical correction of TOF.
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Affiliation(s)
- Vishal V. Bhende
- Pediatric Cardiac Surgery, Bhanubhai and Madhuben Patel Cardiac CentreShree Krishna Hospital, Bhaikaka UniversityKaramsadIndia
| | - Tanishq S. Sharma
- Pediatric Cardiac Surgery, Bhanubhai and Madhuben Patel Cardiac CentreShree Krishna Hospital, Bhaikaka UniversityKaramsadIndia
| | - Deepakkumar V. Mehta
- Department of Radiodiagnosis & ImagingPramukhswami Medical College & Shree Krishna Hospital, Bhaikaka UniversityKaramsadIndia
| | | | - Amit Kumar
- Pediatric Cardiac Intensive CareBhanubhai and Madhuben Patel Cardiac CentreShree Krishna Hospital, Bhaikaka UniversityKaramsadIndia
| | - Jigar P. Thacker
- Department of PediatricsPramukhswami Medical College, Bhaikaka UniversityKaramsadIndia
| | - Viral B. Patel
- Department of Radiodiagnosis & ImagingPramukhswami Medical College & Shree Krishna Hospital, Bhaikaka UniversityKaramsadIndia
| | - Gurpreet Panesar
- Cardiac Anaesthesiology, Bhanubhai and Madhuben Patel Cardiac CentreShree Krishna Hospital, Bhaikaka UniversityKaramsadIndia
| | - Kunal Soni
- Cardiac Anaesthesiology, Bhanubhai and Madhuben Patel Cardiac CentreShree Krishna Hospital, Bhaikaka UniversityKaramsadIndia
| | - Kartik B. Dhami
- Cardiac Anaesthesiology, Bhanubhai and Madhuben Patel Cardiac CentreShree Krishna Hospital, Bhaikaka UniversityKaramsadIndia
| | - Hardil P. Majmudar
- Pediatric Cardiac Surgery, Bhanubhai and Madhuben Patel Cardiac CentreShree Krishna Hospital, Bhaikaka UniversityKaramsadIndia
| | - Nirja Patel
- Cardiac Anaesthesiology, Bhanubhai and Madhuben Patel Cardiac CentreShree Krishna Hospital, Bhaikaka UniversityKaramsadIndia
| | - Sohilkhan R. Pathan
- Clinical Research ServicesBhanubhai and Madhuben Patel Cardiac CentreShree Krishna Hospital, Bhaikaka UniversityKaramsadIndia
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11
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Shang X, Dong N, Zhang C, Wang Y. The Clinical Trial Outcomes of Med-Zenith PT-Valve in the Treatment of Patients With Severe Pulmonary Regurgitation. Front Cardiovasc Med 2022; 9:887886. [PMID: 35783837 PMCID: PMC9243481 DOI: 10.3389/fcvm.2022.887886] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/02/2022] [Accepted: 05/12/2022] [Indexed: 11/28/2022] Open
Abstract
Objective Nearly 2/3 of patients with dilated right ventricular outflow tract (RVOT) were excluded from pulmonary valves transplantation due to the lack of size-matched valves. Here, we explored the safety and efficacy of the Med-Zenith PT-Valve for the treatment of patients with severe pulmonary regurgitation. Methods 22 Patients with severe PR (grade 3+,4+) were enrolled based on the anatomical features of native RVOT and the valve design. The immediate, 3-months and 1-year post-procedural follow-up data were analyzed. Results The baseline mean systolic diameters in the distal main pulmonary artery (MPA), MPA sinus junction, MPA sinus, pulmonary annulus, RVOT aneurysm and muscular outlet measured with computed tomography were 33.6 ± 6.1, 34.0 ± 5.8, 37.9 ± 6.0, 32.4 ± 7.3, 41.9 ± 9.3, and 34.4 ± 8.0 mm, respectively. The PT-Valve landing zone was set within these levels. Successful valve implantations were achieved in all patients without noticeable device malposition, coronary artery compression, pulmonary branch obstruction or paravalvular leak during follow-ups. Post-procedural pulmonary artery diastolic pressure increased from 5.8 ± 3.1 to 11.3 ± 2.5 mmHg. In the 3-month and 1-year follow-up, the right ventricular end diastolic volume index reduced from the baseline 181.6 ± 29.0 to 143.7 ± 29.7 ml/m2 and 123.4 ± 31.2 ml/m2, and the trans-pulmonary valve gradient decreased from 25.6 ± 22.2 to 10.64 ± 3.54 mmHg and 11.16 ± 3.0 mmHg, respectively. The 6-min walk distance increased from 416.6 ± 97.9 to 455.9 ± 64.6 m and 467.8 ± 61.2 m, respectively. Conclusion This clinical trial revealed favorable outcomes for the safety, efficacy and feasibility of the Med-Zenith PT-Valve in the treatment of severe PR with significantly enlarged RVOT.
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Affiliation(s)
- Xiaoke Shang
- Department of General Medicine and Geriatrics, Zhongnan Hospital of Wuhan University, Wuhan University, Wuhan, China
| | - Nianguo Dong
- Department of Cardiovascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Changdong Zhang
- Department of Cardiovascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Yanggan Wang
- Department of General Medicine and Geriatrics, Zhongnan Hospital of Wuhan University, Wuhan University, Wuhan, China
- Medical Research Institute of Wuhan University, Wuhan University, Wuhan, China
- *Correspondence: Yanggan Wang
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12
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Fujiwara J, Tatebe S, Nochioka K, Ota H, Funamizu Y, Miki T, Saiki Y, Yasuda S, Saijo Y. Usefulness of Right Ventricular Free Wall Strain Obtained with Two-Dimensional Speckle-Tracking Echocardiography in Patients with Repaired Tetralogy of Fallot and Pulmonary Regurgitation. TOHOKU J EXP MED 2022; 257:7-15. [PMID: 35321981 DOI: 10.1620/tjem.2022.j011] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
Affiliation(s)
- Junko Fujiwara
- Biomedical Imaging Laboratory, Tohoku University Graduate School of Medicine.,Physiological Laboratory Center, Tohoku University Hospital
| | - Shunsuke Tatebe
- Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
| | - Kotaro Nochioka
- Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
| | - Hideki Ota
- Department of Diagnostic Radiology, Tohoku University Graduate School of Medicine
| | | | - Takashi Miki
- Physiological Laboratory Center, Tohoku University Hospital
| | - Yoshikatsu Saiki
- Department of Cardiovascular Surgery, Tohoku University Graduate School of Medicine
| | - Satoshi Yasuda
- Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
| | - Yoshifumi Saijo
- Biomedical Imaging Laboratory, Tohoku University Graduate School of Medicine
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13
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Cheng S, Li VWY, So EKF, Cheung YF. Right Ventricular-Pulmonary Arterial Coupling in Repaired Tetralogy of Fallot. Pediatr Cardiol 2022; 43:207-217. [PMID: 34463803 DOI: 10.1007/s00246-021-02719-6] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/22/2021] [Accepted: 08/25/2021] [Indexed: 10/20/2022]
Abstract
We assessed right ventricular (RV)-pulmonary arterial (PA) coupling in patients with repaired tetralogy of Fallot (TOF). Sixty patients (34 males) aged 18.6 ± 8.3 years at 14.8 ± 7.4 years after repair and 60 controls were studied. Two-dimensional, tissue Doppler and speckle tracking echocardiography and colour flow mapping were performed to assess RV end-systolic (ESA) and -diastolic areas, tricuspid valve Doppler and myocardial velocities, left ventricular (LV) and RV deformation and pulmonary (PR), tricuspid regurgitation (TR), respectively. The ratios of RV area change to ESA and peak tricuspid annular systolic (s) velocity to RV ESA indexed to body surface area reflected RV-PA coupling. Patients had greater RV areas and reduced tricuspid annular and myocardial velocities, LV and RV myocardial mechanics compared to controls (all p < 0.05). Both RV area change/ESA ratio and peak tricuspid annular s velocity/indexed RV ESA ratio were reduced in patients (all p < 0.001). Sixty-one and 100% of patients had, respectively, RV area change/ESA ratio and peak tricuspid annular s velocity/indexed RV ESA ratio < -2SD of controls. Indices of RV-PA coupling correlated positively with tricuspid myocardial velocities, LV and RV deformation and inversely with PR and TR (all p < 0.05). Multivariate analysis showed RV systolic strain rate, PR and TR as independent predictors of both RV-PA coupling indices, whilst age, gender and LV systolic strain were also predictors of peak tricuspid annular s velocity/indexed RV ESA ratio (all p < 0.05). In conclusion, RV-PA coupling is impaired and is associated with RV and LV mechanics and severity of PR and TR in patients with repaired TOF.
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Affiliation(s)
- Sabine Cheng
- Division of Paediatric Cardiology, Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, 102, Pokfulam Road, Hong Kong, People's Republic of China
| | - Vivian Wing-Yi Li
- Division of Paediatric Cardiology, Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, 102, Pokfulam Road, Hong Kong, People's Republic of China
| | - Edwina Kam-Fung So
- Division of Paediatric Cardiology, Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, 102, Pokfulam Road, Hong Kong, People's Republic of China
| | - Yiu-Fai Cheung
- Division of Paediatric Cardiology, Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, 102, Pokfulam Road, Hong Kong, People's Republic of China.
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14
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Wu M, Fan C, Liu J, Iroegbu CD, Chen W, Huang P, Tang M, Wu X, Wang C, Xiang K, Zhou W, Yang J. Clinical Application of Individualized Pulmonary Bi-Orifice for the Reconstruction of Right Ventricular Outflow Tract in Tetralogy of Fallot. Front Cardiovasc Med 2021; 8:772198. [PMID: 34901232 PMCID: PMC8661005 DOI: 10.3389/fcvm.2021.772198] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/07/2021] [Accepted: 10/31/2021] [Indexed: 11/16/2022] Open
Abstract
Objective: The study aims to establish a new method in the Tetralogy of Fallot (ToF) called the pulmonary valve bi-orifice method (pulmonary annular sparing with an individualized autologous pericardial patch; thus, two orifices are formed at the level of the pulmonary valve annulus) to reconstruct the right ventricular outflow tract (RVOT). Methods: A retrospective analysis of 128 TOF patients from October 2009 to June 2018 with severe pulmonary valve dysplasia who underwent transvalvular annular patch (TAP) procedure (control group) or an individualized pulmonary valve bi-orifice procedure (observation group) were studied. The RVOT for each patient in the observation group was individually reconstructed per the patient's weight and the size of the autologous pulmonary valve using the bi-orifice method; however, increasing the cross-sectional area of the pulmonary valve annulus without destroying its integrity. The result was then compared to the control group, where TAP procedures were applied to evaluate the short to mid-term outcome(s). An in vitro simulation test was used to verify the anti-regurgitation mechanism of the new method. Results: The in vitro simulation test indicated that the anti-regurgitation mechanism was completed by the pericardial patch and the autologous pulmonary valve movement toward each other. Thus, for clinical applications, patients in both groups were compared. The results showed no significant differences in cardiopulmonary bypass and aortic cross-clamp time, mechanical ventilation, and ICU and post-operative residence between the two groups. During the follow-up period (3- to 12-years), 14 patients in the observation group had mild regurgitation after surgery (22.2%), while 10 patients had moderate pulmonary regurgitation (15.8%) with no right ventricular (RV) dilation. On the other hand, 22 patients (39.6%) had moderate to severe regurgitation in the control group, while left pulmonary artery stenosis occurred in one patient. In the control group, six patients (9.2%) with severe RV dilation were reoperated. Conclusion: Individualized pulmonary valve bi-orifice procedure is a safe and excellent method for reconstructing RVOT in ToF.
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Affiliation(s)
- Ming Wu
- Department of Cardiovascular Surgery, The Second Xiangya Hospital, Central South University, Changsha, China.,Department of the Cardiovascular Surgery, The Hunan Provincial People's Hospital, Changsha, China
| | - Chengming Fan
- Department of Cardiovascular Surgery, The Second Xiangya Hospital, Central South University, Changsha, China
| | - Jian Liu
- Department of Cardiothoracic Surgery, Hunan Children's Hospital, Changsha, China
| | - Chukwuemeka Daniel Iroegbu
- Department of Cardiovascular Surgery, The Second Xiangya Hospital, Central South University, Changsha, China
| | - Wangping Chen
- Department of Cardiovascular Surgery, The Second Xiangya Hospital, Central South University, Changsha, China
| | - Peng Huang
- Department of Cardiothoracic Surgery, Hunan Children's Hospital, Changsha, China
| | - Mi Tang
- Department of Cardiovascular Surgery, The Second Xiangya Hospital, Central South University, Changsha, China
| | - Xun Wu
- Department of Cardiovascular Surgery, The Second Xiangya Hospital, Central South University, Changsha, China
| | - Chunle Wang
- Department of Cardiovascular Surgery, The Second Xiangya Hospital, Central South University, Changsha, China
| | - Kun Xiang
- Department of Cardiovascular Surgery, The Second Xiangya Hospital, Central South University, Changsha, China
| | - Wenwu Zhou
- Department of the Cardiovascular Surgery, The Hunan Provincial People's Hospital, Changsha, China
| | - Jinfu Yang
- Department of Cardiovascular Surgery, The Second Xiangya Hospital, Central South University, Changsha, China
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15
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Mauger CA, Govil S, Chabiniok R, Gilbert K, Hegde S, Hussain T, McCulloch AD, Occleshaw CJ, Omens J, Perry JC, Pushparajah K, Suinesiaputra A, Zhong L, Young AA. Right-left ventricular shape variations in tetralogy of Fallot: associations with pulmonary regurgitation. J Cardiovasc Magn Reson 2021; 23:105. [PMID: 34615541 PMCID: PMC8496085 DOI: 10.1186/s12968-021-00780-x] [Citation(s) in RCA: 17] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/23/2020] [Accepted: 05/26/2021] [Indexed: 11/29/2022] Open
Abstract
BACKGROUND Relationships between right ventricular (RV) and left ventricular (LV) shape and function may be useful in determining optimal timing for pulmonary valve replacement in patients with repaired tetralogy of Fallot (rTOF). However, these are multivariate and difficult to quantify. We aimed to quantify variations in biventricular shape associated with pulmonary regurgitant volume (PRV) in rTOF using a biventricular atlas. METHODS In this cross-sectional retrospective study, a biventricular shape model was customized to cardiovascular magnetic resonance (CMR) images from 88 rTOF patients (median age 16, inter-quartile range 11.8-24.3 years). Morphometric scores quantifying biventricular shape at end-diastole and end-systole were computed using principal component analysis. Multivariate linear regression was used to quantify biventricular shape associations with PRV, corrected for age, sex, height, and weight. Regional associations were confirmed by univariate correlations with distances and angles computed from the models, as well as global systolic strains computed from changes in arc length from end-diastole to end-systole. RESULTS PRV was significantly associated with 5 biventricular morphometric scores, independent of covariates, and accounted for 12.3% of total shape variation (p < 0.05). Increasing PRV was associated with RV dilation and basal bulging, in conjunction with decreased LV septal-lateral dimension (LV flattening) and systolic septal motion towards the RV (all p < 0.05). Increased global RV radial, longitudinal, circumferential and LV radial systolic strains were significantly associated with increased PRV (all p < 0.05). CONCLUSION A biventricular atlas of rTOF patients quantified multivariate relationships between left-right ventricular morphometry and wall motion with pulmonary regurgitation. Regional RV dilation, LV reduction, LV septal-lateral flattening and increased RV strain were all associated with increased pulmonary regurgitant volume. Morphometric scores provide simple metrics linking mechanisms for structural and functional alteration with important clinical indices.
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Affiliation(s)
- Charlène A. Mauger
- Department of Anatomy and Medical Imaging, University of Auckland, Auckland, New Zealand
- Auckland Bioengineering Institute, University of Auckland, Auckland, New Zealand
| | - Sachin Govil
- University of California San Diego, La Jolla, CA USA
| | - Radomir Chabiniok
- University of Texas Southwestern Medical Centre, Dallas, TX USA
- Inria, Palaiseau, France
- LMS, École Polytechnique, CNRS, Institut Polytechnique de Paris, Palaiseau, France
- Department of Mathematics, Faculty of Nuclear Sciences and Physical Engineering, Czech Technical University in Prague, Prague, Czech Republic
| | - Kathleen Gilbert
- Auckland Bioengineering Institute, University of Auckland, Auckland, New Zealand
| | - Sanjeet Hegde
- University of California San Diego, La Jolla, CA USA
- Division of Cardiology, Rady Children’s Hospital, San Diego, CA USA
| | - Tarique Hussain
- University of Texas Southwestern Medical Centre, Dallas, TX USA
| | | | | | - Jeffrey Omens
- University of California San Diego, La Jolla, CA USA
| | - James C. Perry
- University of California San Diego, La Jolla, CA USA
- Division of Cardiology, Rady Children’s Hospital, San Diego, CA USA
| | | | | | - Liang Zhong
- National Heart Centre, Singapore, Singapore
- Duke-NUS Medical School, Singapore, Singapore
| | - Alistair A. Young
- Department of Anatomy and Medical Imaging, University of Auckland, Auckland, New Zealand
- Department of Biomedical Engineering, King’s College London, London, UK
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16
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Tompkins R, Garg R. Reduced exercise capacity in the adult with repaired tetralogy of Fallot: More evidence. Now what to do? INTERNATIONAL JOURNAL OF CARDIOLOGY CONGENITAL HEART DISEASE 2021. [DOI: 10.1016/j.ijcchd.2021.100227] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022] Open
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17
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Kobayashi Y, Kotani Y, Kuroko Y, Kawabata T, Sano S, Kasahara S. Staged Repair of Tetralogy of Fallot: A Strategy for Optimizing Clinical and Functional Outcomes. Ann Thorac Surg 2021; 113:1575-1581. [PMID: 33771498 DOI: 10.1016/j.athoracsur.2021.01.087] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/03/2020] [Revised: 11/28/2020] [Accepted: 01/12/2021] [Indexed: 11/26/2022]
Abstract
BACKGROUND This study evaluated the impact of a staged surgical strategy incorporating a modified Blalock-Taussig shunt (BTS) for tetralogy of Fallot (TOF) on pulmonary valve annulus (PVA) growth, the rate of valve-sparing repair (VSR) at the time of intracardiac repair (ICR), and long-term functional outcomes. METHODS This retrospective study included 330 patients with TOF who underwent ICR between 1991 and 2019, including 57 patients (17%) who underwent BTS. The mean follow-up period was 15.0±7.3 years. We compared the data of patients who underwent BTS and those who did not undergo BTS before ICR. RESULTS The median age and body weight before BTS were 71 (28-199) days and 4.3 (3.3-6.8) kg respectively. There were no in-hospital or interstage deaths after BTS. The PVA Z-scores of patients with BTS revealed significant growth after BTS (from -4.2±1.8 to -3.0±1.7, P<0.001). VSR was eventually performed in 207 (63%) patients, including 26 (46%) patients who underwent staged repair. The overall freedom from pulmonary regurgitation-related reintervention were 99.7%, 99.1%, and 95.8% at 1, 5, and 20 years, respectively. CONCLUSIONS A staged surgical strategy incorporating BTS as the first palliation for symptomatic patients resulted in no mortality. BTS may have contributed to the avoidance of primary transannular patch repair (TAP) and facilitated PVA growth; therefore, approximately half of the symptomatic neonates and infants were recruited for VSR. Staged repair may have led to functionally-reliable delayed TAP repair, thereby resulting in less surgical reinterventions.
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Affiliation(s)
| | | | - Yosuke Kuroko
- Cardiovascular Surgery, Okayama University Hospital, Japan
| | | | - Shunji Sano
- Pediatric Cardiothoracic Surgery, University of California, San Francisco
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18
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Lyu Z, Jin M, Yang Y. Value of pulmonary annulus index in predicting transannular patch in tetralogy of Fallot repair. J Card Surg 2021; 36:2197-2203. [PMID: 33749928 DOI: 10.1111/jocs.15500] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/29/2021] [Accepted: 03/07/2021] [Indexed: 11/28/2022]
Abstract
OBJECTIVE It is very important to accurately assess the transannular patch (TAP) in the surgical treatment of tetralogy of Fallot (TOF). The pulmonary annulus index (PAI; the actual pulmonary annulus diameter divided by the expected pulmonary annulus diameter), GA ratio (the ratio of pulmonary annulus and aortic annulus), PAAI (the ratio of pulmonary annulus cross-section and aortic annulus cross-section), and pulmonary annulus Z score (PA Z score) were compared. This study aimed to analyze and explore the application value of PAI in predicting the need for TAP in children undergoing TOF repair. METHODS In total, 130 patients who were diagnosed with TOF and underwent TOF repair in Beijing Anzhen Hospital affiliated to Capital Medical University from December 2018 to December 2019 were retrospectively studied. In total, 112 cases were included in this study and 18 cases were excluded, of which 16 cases were aged above 18 years and 2 cases suffered from Down syndrome. They were divided into TAP group and no TAP group; the values of the pulmonary annulus and aortic annulus were measured. GA ratio, PAI, PAAI, the pulmonary annulus Z score, and main pulmonary artery (MPA) Z score were calculated to perform statistical analysis. RESULTS A total of 112 patients were included in the study, aged 4-177 months, with an average of 22.87 ± 30.21 months; 66 males and 46 females; weighing 5.3-29 kg, with an average of 9.94 ± 4.08 kg; three cases died, one case died of sepsis caused by pulmonary infection, one case died of low cardiac output syndrome, and one case died of multiple organ failure. In total, 62 cases (55.8%) did not undergo TAP and 50 cases (44.2%) underwent TAP. The pulmonary annulus Z score, main pulmonary artery Z score, and PAI in the TAP group were smaller than those in the no TAP group (p < .05). Receiver operating curve (ROC) analysis showed that when the cut-off value of pulmonary annulus was -1.98, the area under the curve (AUC) was 0.88, the sensitivity was 80%, and the specificity was 71%; when the cut-off value of PAI was 0.53, AUC was 0.85, the sensitivity was 75%, and the specificity was 80%; when the cut-off value of GA ratio was 0.55, AUC was 0.85, the sensitivity was 76%, and the specificity was 80%. The area under the PAAI curve was 0.85, the sensitivity was 76%, and the specificity was 79%. When the pulmonary valve Z score, PAI, GA ratio, PAAI, and MPA Z score were all greater than the dividing value, TAP was avoided in more than 90% of children with TOF. When the pulmonary valve Z score, PAI, GA ratio, PAAI, and the main pulmonary artery Z score were all below the dividing value, more than 90% of children with TOF needed TAP. CONCLUSION The predictive effect of PAI as a simple and effective predictor of TAP in TOF radical operation is the same as that of pulmonary annulus Z score, and combining it with the main pulmonary artery Z score was the most accurate method of prediction.
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Affiliation(s)
- Zhenyu Lyu
- Department of Pediatric Cardiac Center, Beijing Anzhen Hospital, Capital Medical University, Beijing, China
| | - Mei Jin
- Department of Pediatric Cardiac Center, Beijing Anzhen Hospital, Capital Medical University, Beijing, China
| | - Yifei Yang
- Department of Pediatric Cardiac Center, Beijing Anzhen Hospital, Capital Medical University, Beijing, China
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Thapmongkol S, Sayasathid J, Methrujpanont J, Thatsakorn K, Jittham W, Puitm S, Thapmongkol M, Namchaisiri J. Growth of the Pulmonary Valve Annulus after the Modified Blalock-Taussig Shunt in Patients with Tetralogy of Fallot. CONGENIT HEART DIS 2021. [DOI: 10.32604/chd.2021.015770] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 03/05/2023]
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Physiologic effects and functional outcome after treatment of dysfunctional right ventricular outflow tract in congenital heart disease using a two-stage intervention. Int J Cardiol 2020; 321:69-74. [PMID: 32593726 DOI: 10.1016/j.ijcard.2020.06.026] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/11/2019] [Revised: 04/15/2020] [Accepted: 06/14/2020] [Indexed: 10/24/2022]
Abstract
BACKGROUND Pathophysiological differences in relief of pulmonary stenosis (PS) as opposed to pulmonary regurgitation (PR) by percutaneous pulmonary valve implantation (PPVI) remain elusive, but might impact current assessment of procedural success and ultimately indications. METHODS Invasive pressure measurements, cardiac magnetic resonance imaging and cardiopulmonary exercise testing were performed before pre-stenting (BMS), after BMS and after PPVI in patients with either PS or PR. RESULTS In PS (n = 14), BMS reduced the right ventricular (RV) to systemic pressure ratio (0.8 ± 0.2 vs. 0.4 ± 0.1%; p < .01), improved RF EF (53 ± 14 vs. 59 ± 12%; p = .01) but introduced free PR (PR fraction post 39 ± 12%; p < .01) with no changes in effective RV stroke volume (SV). PPVI eliminated PR (PR fraction 5 ± 3%; p < .01) and improved effective RV SV (p < .01) with no changes in RV EF (p = .47). Peak VO2 improved significantly after BMS, with no changes following PPVI (26 ± 9 vs. 30 ± 11 vs. 31 ± 10 ml/kg*min). In PR (n = 14), BMS exaggerated PR (PR fraction post 47 ± 10) with reduction in effective RV SV (pre 43 ± 9 vs. post 38 ± 8 ml/m2; p = .01), which improved after PPVI (post PPVI 49 ± 9 ml/m2; p < .01), secondary to elimination of PR (PR fraction 5 ± 4%; p < .01). RV EF (pre 53 ± 11 vs. post 53 ± 9 vs. post PPVI 50 ± 9%) and Peak VO2 (pre 22 ± 7 vs. post 21 ± 7 vs. post PPVI 23 ± 7 ml/kg*min) remained unchanged. CONCLUSIONS Exercise capacity in patients with right ventricular outflow tract dysfunction is primarily afterload-dependent.
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21
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Liu H, Liu S, Zaki A, Wang X, Zhu K, Lu Y, Yang Y, Hamidi R, Wei L, Wang C. Pulmonary valve replacement in primary repair of tetralogy of Fallot in adult patients. J Thorac Dis 2020; 12:4833-4841. [PMID: 33145056 PMCID: PMC7578467 DOI: 10.21037/jtd-20-1475] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022]
Abstract
Background Adults with unrepaired tetralogy of Fallot (ToF) are common in developing countries. Long-term overload of the right ventricle places adult patients at risk for postoperative right heart failure after primary repair, which contributes to morbidity and mortality. The effect of pulmonary valve replacement (PVR) in reducing postoperative morbidity and mortality in adults has never been validated. Methods We conducted a retrospective cohort study in adults (age ≥18 years) with ToF undergoing primary repair from January 2014 to December 2019 at our institution. Patients were divided into three groups according to techniques used to enlarge the right ventricle outflow tract (RVOT). Baseline variables and perioperative outcomes were collected. The primary endpoint was operative mortality. Secondary endpoints were incidences of right heart failure and stage 3 acute kidney injury (AKI). Results A total of 56 patients were enrolled (mean age 41.5±11.7 years, 30 females, 53.6%). They were divided into three groups designated as the following: TA-PVR group for trans-annular patch enlargement with PVR; TA group for trans-annulus patch enlargement without PVR; and group AP for annulus preservation. Four patients (7.1%) died postoperatively, all due to right heart failure. All twelve patients in the TA-PVR group survived. There was no significant difference in mortalities among groups. Ten patients (17.9%) developed right heart failure after surgery with no significant difference among groups. Three patients (5.4%) developed stage 3 AKI after surgery, none belonging to the TA-PVR group, however, not statistically significant. Conclusions Right heart failure is a common complication after primary repair of adult ToF. Trans-annulus patch enlargement should be cautiously selected in this population. PVR with trans-annulus patch enlargement may be a promising technique to protect against postoperative right heart failure and mortality when annulus preservation is not feasible.
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Affiliation(s)
- Huan Liu
- Department of Cardiovascular Surgery, Zhongshan Hospital, Shanghai Cardiovascular Institution, Fudan University, Shanghai, China
| | - Shun Liu
- Department of Cardiovascular Surgery, Zhongshan Hospital, Shanghai Cardiovascular Institution, Fudan University, Shanghai, China
| | - Anthony Zaki
- Department of Thoracic and Cardiovascular Surgery, Cleveland Clinic, Cleveland, OH, USA
| | - Xiuwen Wang
- Department of Cardiovascular Surgery, Zhongshan Hospital, Shanghai Cardiovascular Institution, Fudan University, Shanghai, China
| | - Kai Zhu
- School of Clinical Medicine, Jiujiang University, Jiujiang, China
| | - Yuntao Lu
- Department of Cardiovascular Surgery, Zhongshan Hospital, Shanghai Cardiovascular Institution, Fudan University, Shanghai, China
| | - Ye Yang
- Department of Cardiovascular Surgery, Zhongshan Hospital, Shanghai Cardiovascular Institution, Fudan University, Shanghai, China
| | - Rafi Hamidi
- Department of Cardiovascular Surgery, Zhongshan Hospital, Shanghai Cardiovascular Institution, Fudan University, Shanghai, China
| | - Lai Wei
- Department of Cardiovascular Surgery, Zhongshan Hospital, Shanghai Cardiovascular Institution, Fudan University, Shanghai, China
| | - Chunsheng Wang
- Department of Cardiovascular Surgery, Zhongshan Hospital, Shanghai Cardiovascular Institution, Fudan University, Shanghai, China
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22
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Blais S, Marelli A, Vanasse A, Dahdah N, Dancea A, Drolet C, Colavincenzo J, Vaugon E, Dallaire F. The 30-Year Outcomes of Tetralogy of Fallot According to Native Anatomy and Genetic Conditions. Can J Cardiol 2020; 37:877-886. [PMID: 33059007 DOI: 10.1016/j.cjca.2020.10.002] [Citation(s) in RCA: 27] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/03/2020] [Revised: 09/14/2020] [Accepted: 10/04/2020] [Indexed: 01/14/2023] Open
Abstract
BACKGROUND The reported survival of tetralogy of Fallot (TOF) is > 97%. Patients with pulmonary atresia and/or genetic conditions have worse outcomes, but long-term estimates of survival and morbidity for these TOF subgroups are scarce. The objective of this study was to describe the 30-year outcomes of TOF according to native anatomy and the coexistence of genetic conditions. METHODS The TRIVIA (Tetralogy of Fallot Research for Improvement of Valve Replacement Intervention: A Bridge Across the Knowledge Gap) study is a retrospective population-based cohort including all TOF subjects born from 1980 to 2015 in Québec. We evaluated all-cause mortality by means of Cox proportional hazards regression, and cumulative mean number of cardiovascular interventions and unplanned hospitalisations with the use of marginal means/rates models. We computed 30-year estimates of outcomes according to TOF types, ie, classic TOF (cTOF) and TOF with pulmonary atresia (TOF-PA), and the presence of genetic conditions. RESULTS We included 960 subjects. The median follow-up was 17 years (interquartile range, 8-27). Nonsyndromic cTOF subjects had a 30-year survival of 95% and had undergone a mean of 2.8 interventions and 0.5 hospitalisations per subject. In comparison, TOF-PA subjects had a lower 30-year survival of 78% and underwent a mean of 8.1 interventions, with 4 times as many hospitalisations. The presence of a genetic condition was associated with lower survival (< 85% for cTOF and < 60% for TOF-PA) but similar numbers of interventions and hospitalisations. CONCLUSIONS The anatomic types and the presence of genetic conditions strongly influence the long-term outcomes of TOF. We provided robust 30-year estimates for key markers of prognosis that may be used to improve risk stratification and provide more informed counselling to families.
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Affiliation(s)
- Samuel Blais
- Department of Pediatrics, Faculty of Medicine and Health Sciences, University of Sherbrooke, Sherbrooke, Québec, Canada; Centre de Recherche du Centre Hospitalier Universitaire de Sherbrooke, Sherbrooke, Québec, Canada
| | - Ariane Marelli
- McGill Adult Unit for Congenital Heart Disease Excellence, McGill University Health Centre, Montréal, Québec, Canada
| | - Alain Vanasse
- Centre de Recherche du Centre Hospitalier Universitaire de Sherbrooke, Sherbrooke, Québec, Canada; Department of Family Medicine and Emergency Medicine, Faculty of Medicine and Health Sciences, University of Sherbrooke, Sherbrooke, Québec, Canada
| | - Nagib Dahdah
- Division of Pediatric Cardiology, Centre Hospitalier Universitaire Sainte-Justine, Montréal, Québec, Canada
| | - Adrian Dancea
- Division of Pediatric Cardiology, McGill University Health Centre, Montréal, Québec, Canada
| | - Christian Drolet
- Division of Pediatric Cardiology, Centre Hospitalier Universitaire de Québec, Québec, Québec, Canada
| | | | - Esther Vaugon
- Division of Pediatric Cardiology, McGill University Health Centre, Montréal, Québec, Canada
| | - Frederic Dallaire
- Department of Pediatrics, Faculty of Medicine and Health Sciences, University of Sherbrooke, Sherbrooke, Québec, Canada; Centre de Recherche du Centre Hospitalier Universitaire de Sherbrooke, Sherbrooke, Québec, Canada.
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23
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Relationship between right and left ventricular diastolic dysfunction assessed by 2-dimensional speckle-tracking echocardiography in adults with repaired tetralogy of Fallot. Int J Cardiovasc Imaging 2020; 37:569-576. [PMID: 33006716 PMCID: PMC8702514 DOI: 10.1007/s10554-020-02045-7] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/22/2020] [Accepted: 09/26/2020] [Indexed: 11/30/2022]
Abstract
Several studies have reported a correlation between right ventricular (RV) and left ventricular (LV) systolic dysfunction in adults with repaired tetralogy of Fallot (TOF). However, data are lacking regarding the relationship between RV and LV diastolic dysfunction assessed by 2-dimensional speckle-tracking echocardiography. We studied 69 adults with repaired TOF (mean age 34 years, 61% male) who had been regularly followed up and had routinely undergone echocardiography. In addition to conventional echocardiography, global longitudinal strain (GLS) and early diastolic strain rate (SRe) of both ventricles were assessed using 2-dimensional speckle-tracking echocardiography. Results were compared with 30 age- and sex-matched controls. RV and LV GLS were decreased in TOF patients compared with controls (− 18.4 ± 3.3% vs. −23.5 ± 4.2%, p < 0.001 and − 16.0 ± 3.8% vs. −20.0 ± 3.0%, p < 0.001, respectively). RV and LV SRe were also decreased in TOF patients compared with controls (1.22 ± 0.34 sec− 1 vs. 1.47 ± 0.41 sec− 1, p = 0.003 and 1.29 ± 0.42 sec− 1 vs. 1.63 ± 0.42 sec− 1, p < 0.001, respectively). A correlation between RV and LV SRe was found in TOF patients (r = 0.43, p < 0.001) as well as between RV and LV GLS (r = 0.45, p < 0.001). Two-dimensional speckle-tracking echocardiography reveals subclinical RV and LV diastolic dysfunction in adults with repaired TOF. A correlation is observed between RV and LV diastolic dysfunction as well as between RV and LV systolic dysfunction.
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24
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Outflow tract geometries are associated with adverse outcome indicators in repaired tetralogy of Fallot. J Thorac Cardiovasc Surg 2020; 162:196-205. [PMID: 33097218 DOI: 10.1016/j.jtcvs.2020.09.072] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/08/2020] [Revised: 08/25/2020] [Accepted: 09/10/2020] [Indexed: 11/23/2022]
Abstract
OBJECTIVES A wide variety of right ventricular outflow tract (RVOT) and pulmonary artery (PA) geometries has been reported in patients with repaired tetralogy of Fallot (rTOF). We aimed to investigate the associations between RVOT/PA geometries and outcome indicators in a large rTOF cohort receiving non-conduit repair. METHODS Three-dimensional magnetic resonance angiographic images of 206 patients with rTOF who had a pulmonary regurgitation (PR) fraction ≥20% were reviewed. Patients' RVOT geometry was quantitatively classified into 4 distinct shapes (tubular, hourglass, pyramid, and inverted trapezoid). Bilateral PA size discrepancy was defined as the diameter of the smaller side being less than 70% of that of the bigger side. RESULTS Based on lateral projection of the 3-dimensional images, patients with an inverted trapezoid-shaped RVOT had the smallest RV end-diastolic volume index (EDVi) (108.7 ± 24.3 mL/m2) and pulmonary valve annulus diameter, and shortest QRS duration, whereas those with a pyramid-shaped RVOT had the largest RV EDVi (161.0 ± 44.6 mL/m2) and pulmonary valve annulus diameter. Similar trends of differences were also observed if such classifications were based on the frontal projections. Multivariable analysis revealed that RVOT shapes, subvalvular diameter, PR fraction, QRS duration, and the presence of bilateral PA size discrepancy were independent determinants of RV EDVi. Furthermore, having bilateral PA size discrepancy (25.2%) was independently associated with lower peak oxygen consumption (P = .041). CONCLUSIONS Distinct RVOT morphologies and branch PA size discrepancy are associated with variations in RV remodeling and exercise capacity in patients with rTOF. These findings may aid decision-making regarding reintervention for PR and branch PA size discrepancy.
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25
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Brayson D, Holohan S, Bardswell SC, Arno M, Lu H, Jensen HK, Tran PK, Barallobre‐Barreiro J, Mayr M, dos Remedios CG, Tsang VT, Frigiola A, Kentish JC. Right Ventricle Has Normal Myofilament Function But Shows Perturbations in the Expression of Extracellular Matrix Genes in Patients With Tetralogy of Fallot Undergoing Pulmonary Valve Replacement. J Am Heart Assoc 2020; 9:e015342. [PMID: 32805183 PMCID: PMC7660801 DOI: 10.1161/jaha.119.015342] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/05/2019] [Accepted: 05/28/2020] [Indexed: 02/06/2023]
Abstract
Background Patients with repair of tetralogy of Fallot (rToF) who are approaching adulthood often exhibit pulmonary valve regurgitation, leading to right ventricle (RV) dilatation and dysfunction. The regurgitation can be corrected by pulmonary valve replacement (PVR), but the optimal surgical timing remains under debate, mainly because of the poorly understood nature of RV remodeling in patients with rToF. The goal of this study was to probe for pathologic molecular, cellular, and tissue changes in the myocardium of patients with rToF at the time of PVR. Methods and Results We measured contractile function of permeabilized myocytes, collagen content of tissue samples, and the expression of mRNA and selected proteins in RV tissue samples from patients with rToF undergoing PVR for severe pulmonary valve regurgitation. The data were compared with nondiseased RV tissue from unused donor hearts. Contractile performance and passive stiffness of the myofilaments in permeabilized myocytes were similar in rToF-PVR and RV donor samples, as was collagen content and cross-linking. The patients with rToF undergoing PVR had enhanced mRNA expression of genes associated with connective tissue diseases and tissue remodeling, including the small leucine-rich proteoglycans ASPN (asporin), LUM (lumican), and OGN (osteoglycin), although their protein levels were not significantly increased. Conclusions RV myofilaments from patients with rToF undergoing PVR showed no functional impairment, but the changes in extracellular matrix gene expression may indicate the early stages of remodeling. Our study found no evidence of major damage at the cellular and tissue levels in the RV of patients with rToF who underwent PVR according to current clinical criteria.
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Affiliation(s)
- Daniel Brayson
- School of Cardiovascular Medicine and SciencesKing's College London BHF Centre for Research ExcellenceLondonUnited Kingdom
| | - So‐Jin Holohan
- School of Cardiovascular Medicine and SciencesKing's College London BHF Centre for Research ExcellenceLondonUnited Kingdom
| | - Sonya C. Bardswell
- School of Cardiovascular Medicine and SciencesKing's College London BHF Centre for Research ExcellenceLondonUnited Kingdom
| | - Matthew Arno
- Genomics CentreFaculty of Life Sciences and MedicineKing’s College LondonLondonUnited Kingdom
| | - Han Lu
- Genomics CentreFaculty of Life Sciences and MedicineKing’s College LondonLondonUnited Kingdom
| | | | | | - Javier Barallobre‐Barreiro
- School of Cardiovascular Medicine and SciencesKing's College London BHF Centre for Research ExcellenceLondonUnited Kingdom
| | - Manuel Mayr
- School of Cardiovascular Medicine and SciencesKing's College London BHF Centre for Research ExcellenceLondonUnited Kingdom
| | | | | | - Alessandra Frigiola
- Great Ormond Street HospitalLondonUnited Kingdom
- Guys and St Thomas’ NHS Foundation TrustSt Thomas’ HospitalLondonUnited Kingdom
- School of Biomedical Engineering and Imaging SciencesKings CollegeLondonUnited Kingdom
| | - Jonathan C. Kentish
- School of Cardiovascular Medicine and SciencesKing's College London BHF Centre for Research ExcellenceLondonUnited Kingdom
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26
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Lau LY, So EKF, Chow PC, Cheung YF. Frontal QRS-T angle and ventricular mechanics in congenital heart disease. Heart Vessels 2020; 35:1299-1306. [PMID: 32246195 DOI: 10.1007/s00380-020-01601-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/09/2020] [Accepted: 03/27/2020] [Indexed: 11/26/2022]
Abstract
BACKGROUND The QRS-T angle has been associated with adverse cardiovascular events and sudden cardiac deaths. We determined frontal QRS-T angle in patients with complete transposition of the great arteries (TGA) after atrial switch operation and repaired tetralogy of Fallot (TOF) and explored its relationships with ventricular mechanics. METHODS Thirty TGA patients aged 32.3 ± 4.4 years after atrial switch operation and 47 repaired TOF patients aged 28.7 ± 6.0 years were studied. The frontal planar QRS-T angle and QRS duration were measured from 12-lead electrocardiograms. Right (RV) and left ventricular (LV) strain parameters were determined using speckle tracking echocardiography. RESULTS Compared with TOF patients, TGA patients after atrial switch operation had significantly greater frontal QRS-T angle (136.3° ± 43.5° vs 74.5° ± 59.6°, p < 0.001), greater prevalence of QRS-T angle ≥ 100° (83.3% vs 29.8%, p < 0.001), and showed progressive increase in QRS-T angle over a duration of 3.3 ± 1.0 years (p = 0.035). The QRS-T angle correlated positively with QRS duration in both the TGA (r = 0.61, p < 0.001) and TOF (r = 0.30, p < 0.043) groups. Among TGA patients, QRS-T angle was found to correlate negatively with systemic RV global longitudinal strain (r = - 0.49, p = 0.007), early diastolic strain rate (r = - 0.41, p = 0.026), and fractional area change (r = - 0.38, p = 0.045), but not subpulmonary LV strain indices. By contrast, among repaired TOF patients, there were no significant correlations between QRS-T angle and systemic and subpulmonary ventricular strain indices (all p > 0.05). CONCLUSION Increased frontal QRS-T angle is prevalent in TGA patients after atrial switch operation and is related to worse systemic RV mechanics.
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Affiliation(s)
- Lok-Yee Lau
- Division of Paediatric Cardiology, Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, 102, Pokfulam Road, Hong Kong, China
| | - Edwina Kam-Fung So
- Division of Paediatric Cardiology, Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, 102, Pokfulam Road, Hong Kong, China
| | - Pak-Cheong Chow
- Division of Paediatric Cardiology, Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, 102, Pokfulam Road, Hong Kong, China
| | - Yiu-Fai Cheung
- Division of Paediatric Cardiology, Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, 102, Pokfulam Road, Hong Kong, China.
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27
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Lumens J, Fan CPS, Walmsley J, Yim D, Manlhiot C, Dragulescu A, Grosse-Wortmann L, Mertens L, Prinzen FW, Delhaas T, Friedberg MK. Relative Impact of Right Ventricular Electromechanical Dyssynchrony Versus Pulmonary Regurgitation on Right Ventricular Dysfunction and Exercise Intolerance in Patients After Repair of Tetralogy of Fallot. J Am Heart Assoc 2020; 8:e010903. [PMID: 30651018 PMCID: PMC6497336 DOI: 10.1161/jaha.118.010903] [Citation(s) in RCA: 30] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Background The relative impact of right ventricular ( RV ) electromechanical dyssynchrony versus pulmonary regurgitation ( PR ) on exercise capacity and RV function after tetralogy of Fallot repair is unknown. We aimed to delineate the relative effects of these factors on RV function and exercise capacity. Methods and Results We retrospectively analyzed 81 children with tetralogy of Fallot repair using multivariable regression. Predictor parameters were electrocardiographic QRS duration reflecting electromechanical dyssynchrony and PR severity by cardiac magnetic resonance. The outcome parameters were exercise capacity (percentage predicted peak oxygen consumption) and cardiac magnetic resonance ejection fraction (RV ejection fraction). To understand the relative effects of RV dyssynchrony versus PR on exercise capacity and RV function, virtual patient simulations were performed using a closed-loop cardiovascular system model (CircAdapt), covering a wide spectrum of disease severity. Eighty-one patients with tetralogy of Fallot repair (median [interquartile range { IQR} ] age, 14.48 [11.55-15.91] years) were analyzed. All had prolonged QRS duration (median [IQR], 144 [123-152] ms), at least moderate PR (median [IQR], 40% [29%-48%]), reduced exercise capacity (median [IQR], 79% [68%-92%] predicted peak oxygen consumption), and reduced RV ejection fraction (median [IQR], 48% [44%-52%]). Longer QRS duration, more than PR , was associated with lower oxygen consumption and lower RV ejection fraction. In a multivariable regression analysis, oxygen consumption decreased with both increasing QRS duration and PR severity. CircAdapt modeling showed that RV dyssynchrony exerts a stronger limiting effect on exercise capacity and on RV ejection fraction than does PR , regardless of contractile function. Conclusions In both patient data and computer simulations, RV dyssynchrony, more than PR , appears to be associated with reduced exercise capacity and RV systolic dysfunction in patients after TOF repair.
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Affiliation(s)
- Joost Lumens
- 2 Department of Biomedical Engineering Cardiovascular Research Institute Maastricht Maastricht University Maastricht the Netherlands.,3 IHU LIRYC Electrophysiology and Heart Modeling Institute Fondation Bordeaux Université Pessac France
| | - Chun-Po Steve Fan
- 1 Division of Cardiology Labatt Family Heart Centre and Department of Paediatrics Hospital for Sick Children and University of Toronto Toronto Ontario Canada
| | - John Walmsley
- 2 Department of Biomedical Engineering Cardiovascular Research Institute Maastricht Maastricht University Maastricht the Netherlands
| | - Deane Yim
- 1 Division of Cardiology Labatt Family Heart Centre and Department of Paediatrics Hospital for Sick Children and University of Toronto Toronto Ontario Canada
| | - Cedric Manlhiot
- 1 Division of Cardiology Labatt Family Heart Centre and Department of Paediatrics Hospital for Sick Children and University of Toronto Toronto Ontario Canada
| | - Andreea Dragulescu
- 1 Division of Cardiology Labatt Family Heart Centre and Department of Paediatrics Hospital for Sick Children and University of Toronto Toronto Ontario Canada
| | - Lars Grosse-Wortmann
- 1 Division of Cardiology Labatt Family Heart Centre and Department of Paediatrics Hospital for Sick Children and University of Toronto Toronto Ontario Canada
| | - Luc Mertens
- 1 Division of Cardiology Labatt Family Heart Centre and Department of Paediatrics Hospital for Sick Children and University of Toronto Toronto Ontario Canada
| | - Frits W Prinzen
- 4 Department of Physiology Cardiovascular Research Institute Maastricht Maastricht University Maastricht the Netherlands
| | - Tammo Delhaas
- 2 Department of Biomedical Engineering Cardiovascular Research Institute Maastricht Maastricht University Maastricht the Netherlands
| | - Mark K Friedberg
- 1 Division of Cardiology Labatt Family Heart Centre and Department of Paediatrics Hospital for Sick Children and University of Toronto Toronto Ontario Canada
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28
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Egbe AC, Vallabhajosyula S, Connolly HM. Trends and outcomes of pulmonary valve replacement in tetralogy of Fallot. Int J Cardiol 2020; 299:136-139. [DOI: 10.1016/j.ijcard.2019.07.063] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/19/2019] [Revised: 05/10/2019] [Accepted: 07/17/2019] [Indexed: 01/10/2023]
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29
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Warmerdam E, Krings GJ, Leiner T, Grotenhuis HB. Three-dimensional and four-dimensional flow assessment in congenital heart disease. Heart 2019; 106:421-426. [PMID: 31857355 DOI: 10.1136/heartjnl-2019-315797] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/13/2019] [Revised: 11/08/2019] [Accepted: 11/20/2019] [Indexed: 01/27/2023] Open
Abstract
Congenital heart disease (CHD) is the most common form of congenital defects, with an incidence of 8 per 1000 births. Due to major advances in diagnostics, perioperative care and surgical techniques, the survival rate of patients with CHD has improved dramatically. Conversely, although 70%-95% of infants with CHD survive into adulthood, the rate of long-term morbidity, which often requires (repeat) intervention, has increased. Recently, the role of altered haemodynamics in cardiac development and CHD has become a subject of interest. Patients with CHD often have abnormal blood flow patterns, either due to the primary cardiac defect or as a consequence of the surgical intervention(s). Research suggests that these abnormal blood flow patterns may contribute to diminished cardiac and vascular function. Serial assessment of haemodynamic parameters in patients with CHD may allow for improved understanding of the often complex haemodynamics in these patients and thereby potentially guide the timing and nature of interventions with the aim of preventing progression of cardiovascular deterioration. In this article we will discuss two novel non-invasive four-dimensional (4D) techniques to evaluate cardiovascular haemodynamics: 4D-flow cardiac magnetic resonance and computational fluid dynamics. This review focuses on the additional value of these two modalities in the evaluation of patients with CHD with abnormal flow patterns, who could benefit from advanced haemodynamic evaluation: patients with coarctation of the aorta, bicuspid aortic valve, tetralogy of Fallot and patients after Fontan palliation.
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Affiliation(s)
- Evangeline Warmerdam
- Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.,Pediatric Cardiology, Wilhelmina Children's Hospital University Medical Center, Utrecht, The Netherlands
| | - Gregor J Krings
- Pediatric Cardiology, Wilhelmina Children's Hospital University Medical Center, Utrecht, The Netherlands
| | - Tim Leiner
- Radiology, University Medical Center Utrecht, Utrecht, The Netherlands
| | - Heynric B Grotenhuis
- Pediatric Cardiology, Wilhelmina Children's Hospital University Medical Center, Utrecht, The Netherlands .,Pediatric Cardiology, Universitair Medisch Centrum Utrecht - Locatie Wilhelmina Kinderziekenhuis, Utrecht, The Netherlands
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30
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Driesen BW, Warmerdam EG, Sieswerda GJ, Meijboom FJ, Molenschot MMC, Doevendans PA, Krings GJ, van Dijk APJ, Voskuil M. Percutaneous Pulmonary Valve Implantation: Current Status and Future Perspectives. Curr Cardiol Rev 2019; 15:262-273. [PMID: 30582483 PMCID: PMC8142351 DOI: 10.2174/1573403x15666181224113855] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/29/2018] [Revised: 12/12/2018] [Accepted: 12/17/2018] [Indexed: 02/07/2023] Open
Abstract
Patients with congenital heart disease (CHD) with right ventricle outflow tract (RVOT) dysfunction need sequential pulmonary valve replacements throughout their life in the majority of cases. Since their introduction in 2000, the number of percutaneous pulmonary valve implantations (PPVI) has grown and reached over 10,000 procedures worldwide. Overall, PPVI has been proven safe and effective, but some anatomical variations can limit procedural success. This review discusses the current status and future perspectives of the procedure.
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Affiliation(s)
- Bart W Driesen
- Department of Cardiology, University Medical Center Utrecht, Utrecht, Netherlands.,Department of Cardiology, Radboudumc, Nijmegen, Netherlands
| | | | - Gert-Jan Sieswerda
- Department of Cardiology, University Medical Center Utrecht, Utrecht, Netherlands
| | - Folkert J Meijboom
- Department of Cardiology, University Medical Center Utrecht, Utrecht, Netherlands
| | | | - Pieter A Doevendans
- Department of Cardiology, University Medical Center Utrecht, Utrecht, Netherlands.,Netherlands Heart Institute, Utrecht, Netherlands.,Central Military Hospital, Utre cht, Netherlands
| | - Gregor J Krings
- Department of Cardiology, University Medical Center Utrecht, Utrecht, Netherlands
| | | | - Michiel Voskuil
- Department of Cardiology, University Medical Center Utrecht, Utrecht, Netherlands
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31
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Zhao J, Cai X, Teng Y, Nie Z, Ou Y, Zhuang J, Wen S, Cen J, Xu G, Cui H, Chen J. Value of pulmonary annulus area index in predicting transannular patch placement in tetralogy of Fallot repair. J Card Surg 2019; 35:48-53. [PMID: 31899832 DOI: 10.1111/jocs.14321] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
BACKGROUND Precisely evaluating the need for transannular patch (TAP) placement is very important in the surgical treatment of tetralogy of Fallot. We hypothesized that the pulmonary annulus area index (PAAI, the pulmonary-to-aortic valve annulus cross-sectional area ratio) could be a useful and accessible predictor for TAP placement. METHODS The medical records of patients who underwent tetralogy of Fallot repair between 1 January 2016 and 31 December 2017 were reviewed retrospectively. A total of 255 patients were included and categorized into two groups: patients who needed TAP placement and patients who did not. Various candidate predictors for TAP placement (PAAI, pulmonary annulus z-score, and velocity across the pulmonary annulus) were compared using receiver operating characteristic curves. The optimal cutoff for each predictor was assessed. RESULTS Among the 255 patients included, 156 needed TAP placement (156/237, 65.8%). Both the PAAI (0.28 [0.20/0.34] vs 0.14 [0.09/0.19]; P < .0001) and z-score (-1.5 [-2.9, -0.4] vs -3.6 [-5.3/-2.6]; P < .0001) were smaller in the TAP group. The PAAI is a useful predictor of the pulmonary annulus z-score (AUC 0.830 vs 0.811, P = .19). Combination analysis of the PAAI and velocity across the pulmonary annulus (PV vmax ) showed better predictive value than the PAAI and z-score (AUC 0.860, sensitivity 89.7%, specificity 61.7%, P < .0001). CONCLUSIONS Our results suggest that the PAAI is a useful and accessible predictor for TAP placement and can be applied readily and simply in clinical practice. A combination with the velocity across the pulmonary annulus could promote the accuracy of prediction.
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Affiliation(s)
- Junfei Zhao
- Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, PR China
| | - Xiaowei Cai
- Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, PR China
| | - Yun Teng
- Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, PR China
| | - Zhiqiang Nie
- Department of Epidemiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, PR China
| | - Yanqiu Ou
- Department of Epidemiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, PR China
| | - Jian Zhuang
- Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, PR China
| | - Shusheng Wen
- Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, PR China
| | - Jianzheng Cen
- Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, PR China
| | - Gang Xu
- Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, PR China
| | - Hujun Cui
- Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, PR China
| | - Jimei Chen
- Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, PR China
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32
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Egbe AC, Kothapalli S, Borlaug BA, Ammash NM, Najam M, Bajwa N, Tarek K, Mathew J, Connolly HM. Mechanism and Risk Factors for Death in Adults With Tetralogy of Fallot. Am J Cardiol 2019; 124:803-807. [PMID: 31272701 DOI: 10.1016/j.amjcard.2019.05.048] [Citation(s) in RCA: 17] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/03/2019] [Revised: 05/10/2019] [Accepted: 05/29/2019] [Indexed: 11/26/2022]
Abstract
One of the goals of lifelong care in adults with tetralogy of Fallot (TOF) is early identification and treatment of patients at high risk for adverse events. Clinical risk stratification tools are critical for achieving this goal. We reviewed the Mayo Adult Congenital Heart Disease database and identified 465 TOF patients (age 37 ± 14 years, men 223 [48%]) seen at Mayo Clinic Rochester between 1990 and 2017. The aim was to determine the risk factors for death and/or heart transplant through a comprehensive analysis of 8 groups of variables (demographics, co-morbidities, medications, heart rhythm, echocardiography, cardiac magnetic resonance imaging, cardiac catheterization, and cardiopulmonary exercise test data) using univariable and multivariable Cox proportional hazard models. The end point of death and/or transplant occurred in 57 (12%) patients during a follow-up of 13.6 ± 8.2 years, yielding an event rate of 0.9% per year. Independent risk factors were age >42 years, atrial fibrillation, ≥moderate QRS fragmentation, left ventricular ejection fraction <50%, right ventricular end-diastolic pressure >16 mm Hg, and left ventricle end-diastolic pressure >16 mm Hg. There is nearly a twofold increase in the risk of death and/or transplant per unit increase in number of risk factors (hazard ratio 1.92, 95% confidence interval 1.62 to 2.27, p <0.001). In conclusion, the current study provides risk stratification indices based on a comprehensive risk model of all clinical variables in an unselected TOF population. Further studies are required to determine whether interventions targeted at modifying these risk factors will alter the annual event rate.
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Heidari Sarvestani A, Separham A, Khezerloo N. Assessment of Strain and Strain Rate in Patients with Coronary Artery Disease Before and After Percutaneous Intervention on Left Anterior Descending Coronary Artery. INTERNATIONAL JOURNAL OF CARDIOVASCULAR PRACTICE 2019. [DOI: 10.29252/ijcp-24016] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/31/2022] Open
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Chango Azanza DX, Munín MA, Sánchez GA, Spernanzoni F. Prolapse and regurgitation of the four heart valves in a patient with Ehlers-Danlos Syndrome: a case report. EUROPEAN HEART JOURNAL-CASE REPORTS 2019; 3:5481902. [PMID: 31449614 PMCID: PMC6601149 DOI: 10.1093/ehjcr/ytz052] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 10/03/2018] [Accepted: 04/02/2019] [Indexed: 11/27/2022]
Abstract
Background The Ehlers–Danlos Syndrome (EDS) is part of a group of connective tissue diseases that affect the synthesis and processing of collagen leading to alterations in the structure of many tissues and organs. Case summary Herein, we reported a case of a patient with prolapse of the four cardiac valves. Non-significant regurgitation of the mitral, aortic, and tricuspid valves was observed. The pulmonary regurgitation (PR) was considered significant. Dilatation of the right ventricle with preserved systolic function was also noted. A cardiac magnetic resonance confirmed the findings of the echocardiogram and determined a severe PR (regurgitant fraction of 41%). The physical examination revealed hyperlaxity of the joints, skin hyperelasticity, defects in wound healing, and abdominal hernias suggesting EDS. The stress test did not develop any symptoms or complex arrhythmias. In this patient, the heart team initially decided medical treatment and evolutionary control. At the moment, he remains asymptomatic. Discussion Valvular involvement in EDS is an infrequent event and the compromise of the four cardiac valves is exceptional. The existence of severe PR with a marked increase in ventricular volumes, even in the absence of symptoms, in most cases requires an intervention on the valve. However, in patients with EDS, there are a high rate of complications and interventions should be avoided as much as possible.
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Affiliation(s)
- Diego Xavier Chango Azanza
- Department of Cardiovascular Ultrasound, CEMIC (Center for Medical Education and Clinical Research "Norberto Quirno"), AV. E Galván 4102, C1431FWO, Buenos Aires, Argentina.,Olazabal 2170 6 to 13 CABA, CP 1427, Argentina
| | - Martin Alejandro Munín
- Department of Cardiovascular Ultrasound, CEMIC (Center for Medical Education and Clinical Research "Norberto Quirno"), AV. E Galván 4102, C1431FWO, Buenos Aires, Argentina.,Francia 2048 Florida Vicente Lopez, Provincia de Buenos Aires CP 1602, Argentina
| | - Gustavo Anibal Sánchez
- Department of Cardiovascular Ultrasound, CEMIC (Center for Medical Education and Clinical Research "Norberto Quirno"), AV. E Galván 4102, C1431FWO, Buenos Aires, Argentina.,Donato Alvarez 2770 CABA, CP 1427, Argentina
| | - Fernando Spernanzoni
- Department of Cardiovascular Ultrasound, CEMIC (Center for Medical Education and Clinical Research "Norberto Quirno"), AV. E Galván 4102, C1431FWO, Buenos Aires, Argentina.,Helguera 1751 CABA, CP 1416, Argentina
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Assessment of Right Ventricular-Pulmonary Arterial Coupling in Chronic Pulmonary Regurgitation. Can J Cardiol 2019; 35:914-922. [PMID: 31292091 DOI: 10.1016/j.cjca.2019.03.009] [Citation(s) in RCA: 17] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/08/2019] [Revised: 03/05/2019] [Accepted: 03/12/2019] [Indexed: 12/26/2022] Open
Abstract
BACKGROUND We hypothesized that noninvasively measured right ventricular (RV) to pulmonary arterial (RV-PA) coupling would be abnormal in chronic pulmonary regurgitation (PR) even in the setting of normal RV ejection fraction, and that RV-PA coupling indices would have a better correlation with peak oxygen consumption (VO2) compared with RV systolic indices alone. METHODS This was a retrospective study of 129 adults (repaired tetralogy of Fallot [TOF] n = 84 and valvular pulmonic stenosis [VPS] with previous intervention n = 45) with ≥ moderate native PR and RV ejection fraction > 50%. The 84 TOF patients were propensity matched with 84 patients with normal echocardiogram (control); age 28 ± 7 years and male sex n = 39 (46%). RV-PA coupling was measured according to fractional area change (FAC)/RV systolic pressure (RVSP) and tricuspid annular plane systolic excursion (TAPSE)/RVSP. RESULTS RV systolic function indices were similar between TOF and control groups (FAC 43 ± 6% vs 41 ± 5% [P = 0.164] and TAPSE 22 ± 5 mm vs 24 ± 6 mm [P = 0.263]). However, RV-PA coupling was lower in the TOF group (FAC/RVSP 1.10 ± 0.29 vs 1.48 ± 0.22 [P < 0.001]; TAPSE/RVSP 0.51 ± 0.15 vs 0.78 ± 0.11 [P < 0.001]) because of higher RV afterload (RVSP 42 ± 3 mm Hg vs 31 ± 3 mm Hg [P = 0.012]). FAC/RVSP (r = 0.61; P < 0.001) and TAPSE/RVSP (r = 0.69; P < 0.001) correlated with peak VO2 especially in the patients with impaired exercise capacity whereas FAC and TAPSE were independent of peak VO2. Similar comparisons between VPS and control groups showed no difference in TAPSE and FAC between groups, but lower FAC/RVSP and TAPSE/RVSP in the VPS group. CONCLUSIONS There is abnormal RV-PA coupling in chronic PR, and noninvasively measured RV-PA coupling might potentially be prognostic because of its correlation with exercise capacity.
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Bedair R, Iriart X. EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Tetralogy of Fallot: diagnosis to long-term follow-up. Echo Res Pract 2019; 6:R9-R23. [PMID: 30557849 PMCID: PMC6301192 DOI: 10.1530/erp-18-0049] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/23/2018] [Accepted: 11/07/2018] [Indexed: 11/17/2022] Open
Abstract
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect, affecting 3 in 10,000 live births. Surgical correction in early childhood is associated with good outcomes, but lifelong follow-up is necessary to identify the long-term sequelae that may occur. This article will cover the diagnosis of TOF in childhood, the objectives of surveillance through adulthood and the value of multi-modality imaging in identifying and guiding timely surgical and percutaneous interventions.
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Affiliation(s)
- R Bedair
- Department of Adult Congenital Cardiology, Bristol Heart Institute - University Hospitals Bristol NHS Foundation Trust, Bristol, UK
| | - X Iriart
- Department of Pediatric and Adult Congenital Cardiology, Hôpital Cardiologique du Haut-Lévêque, CHU de Bordeaux, Pessac, France
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Accuracy of a New Echocardiographic Index to Predict Need for Trans-annular Patch in Tetralogy of Fallot. Pediatr Cardiol 2019; 40:161-167. [PMID: 30178189 DOI: 10.1007/s00246-018-1973-x] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/25/2018] [Accepted: 08/28/2018] [Indexed: 10/28/2022]
Abstract
The long-term outcome after repair of tetralogy of Fallot (TOF) is critically dependent pulmonary valve competence that is compromised by trans-annular patch (TAP). We compared a new echocardiographic index [pulmonary annulus index (PAI)] to conventional methods of predicting need for TAP in infants undergoing TOF repair. Consecutive infants undergoing TOF repair were prospectively studied. Pre-operative aortic and pulmonary annuli and main pulmonary artery (MPA) diameters were measured and z scores determined. PAI was a ratio of observed to expected pulmonary annulus (PA) diameter. TAP was based on intra-operative sizing by surgeons blinded to PAI values. Receiver operator curves (ROC) were generated for all PAI, MPA z scores and pulmonary annulus z scores. Of 84 infants (8.6 ± 2.6 months; 7.5 ± 1.3 kg), 36 needed TAP (43%). All the three indices viz. PAI, Pulmonary annulus and MPA z scores performed similarly in predicting need for TAP (ROC curves ~ 80%). Combining cut-offs of MPA z scores (> - 3.83) with either PAI (> 0.73) or PA z score (> - 1.83) predicted avoidance of TAP with ~ 90% accuracy. When both PAI and MPA z scores were below the cut-offs there was an 80% likelihood of TAP. Failure to predict TAP was associated with unicommisural pulmonary valves. PAI was equal to PA z scores in predicting need for TAP during repair of TOF. Combining either with MPA z scores was the most accurate method of prediction. Failure of prediction was mainly due to presence of a unicommissural pulmonary valve.
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Kwak JG, Kim WH, Kim ER, Lim JH, Min J. One-Year Follow-up After Tetralogy of Fallot Total Repair Preserving Pulmonary Valve and Avoiding Right Ventriculotomy. Circ J 2018; 82:3064-3068. [PMID: 30298850 DOI: 10.1253/circj.cj-18-0690] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
BACKGROUND We reviewed our revised surgical strategy for tetralogy of Fallot (TOF) total correction to minimize early exposure to significant pulmonary regurgitation (PR) and to avoid right ventriculotomy (RV-tomy). METHODS AND RESULTS Since February 2016, we have tried to preserve, first, pulmonary valve (PV) function to minimize PR by extensive commissurotomy with annulus saving; and second, RV infundibular function by avoiding RV-tomy. With this strategy, we performed total correction for 50 consecutive patients with TOF until May 2018. We reviewed the early outcomes of 27 of 50 patients who received follow-up for ≥3 months. Mean patient age at operation was 10.2±5.0 months, and mean body weight was 8.8±1.2 kg. The preoperative pressure gradient at the RV outflow tract and the PV z-score were improved at most recent echocardiography from 82.0±7.1 to 26.8±6.4 mmHg, and from -2.35±0.49 to -0.55±0.54, respectively, during 11.1±1.6 months of follow-up after operation. One patient required re-intervention for residual pulmonary stenosis. Twenty-two patients had less than moderate PR (none, 1; trivial, 8; mild, 13), and 5 patients had moderate PR. There was no free or severe PR. CONCLUSIONS At 1-year follow-up, the patients who underwent total TOF correction with our revised surgical strategy had acceptable results in terms of PV function. The preserved PV had a tendency to grow on short-term follow-up.
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Affiliation(s)
- Jae Gun Kwak
- Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital
| | - Woong-Han Kim
- Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital
| | - Eung Re Kim
- Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital
| | - Jae Hong Lim
- Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital
| | - Jooncheol Min
- Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital
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Egbe AC, Miranda WR, Madhavan M, Ammash NM, Missula VR, Al-Otaibi M, Fatola A, Kothapalli S, Connolly HM. Cardiac implantable electronic devices in adults with tetralogy of Fallot. Heart 2018; 105:538-544. [PMID: 30377259 DOI: 10.1136/heartjnl-2018-314072] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/25/2018] [Revised: 10/03/2018] [Accepted: 10/10/2018] [Indexed: 12/16/2022] Open
Abstract
BACKGROUND Tachyarrhythmias and bradyarrhythmias affect 20%-50% of adult patients with tetralogy of Fallot (TOF) and some of these patients will require cardiac implantable electronic devices (CIED) such as pacemaker and/or internal cardioverter defibrillator. METHODS The Mayo Adult Congenital Heart Disease database was queried for patients with repaired TOF and history of CIED implantation, 1990-2017. The study objectives were: (1) determine the occurrence of device-related complications defined as lead failure, lead recall, device infection and lead thrombus; and (2) determine the occurrence and risk factors for defibrillator shock. RESULTS There were 99 patients (age 46±14 years and 66 (66%) men) with CIED, and the CIEDs were 41 (41%) pacemakers and 73 (73%) defibrillators. Indication for defibrillator implantation was for primary prevention in 28 (38%) and secondary prevention in 45 (62%). Device-related complications occurred in 20 (20%) patients (lead failure 17, lead recall 4, device infection 12 and thrombus 3). Twenty-five per cent of all device infections occurred within 30 days from the time of device generator change. Annualised rates of appropriate and inappropriate shocks were 5.7% and 6.2%, respectively. The use of class III antiarrhythmic drug was protective against defibrillator shock. CONCLUSION The current study provides useful outcome data to aid patient counselling and clinical decision-making. Further studies are required to explore ways to decrease the risk of postprocedural infection after device generator change, and to determine which patients will benefit from empirical antiarrhythmic therapy as a strategy to decrease incidence of inappropriate defibrillator shock.
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Affiliation(s)
- Alexander C Egbe
- Department of Cardiovascular Medicine, Mayo Clinic Rochester, Rochester, Minnesota, USA
| | - William R Miranda
- Department of Cardiovascular Medicine, Mayo Clinic Rochester, Rochester, Minnesota, USA
| | - Malini Madhavan
- Department of Cardiovascular Medicine, Mayo Clinic Rochester, Rochester, Minnesota, USA
| | - Naser M Ammash
- Department of Cardiovascular Medicine, Mayo Clinic Rochester, Rochester, Minnesota, USA
| | - Venkata R Missula
- Department of Cardiovascular Medicine, Mayo Clinic Rochester, Rochester, Minnesota, USA
| | - Mohammed Al-Otaibi
- Department of Cardiovascular Medicine, Mayo Clinic Rochester, Rochester, Minnesota, USA
| | - Ayotola Fatola
- Department of Cardiovascular Medicine, Mayo Clinic Rochester, Rochester, Minnesota, USA
| | - Srikanth Kothapalli
- Department of Cardiovascular Medicine, Mayo Clinic Rochester, Rochester, Minnesota, USA
| | - Heidi M Connolly
- Department of Cardiovascular Medicine, Mayo Clinic Rochester, Rochester, Minnesota, USA
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Hickey E, Pham-Hung E, Halvorsen F, Gritti M, Duong A, Wilder T, Caldarone CA, Redington A, Van Arsdell G. Annulus-Sparing Tetralogy of Fallot Repair: Low Risk and Benefits to Right Ventricular Geometry. Ann Thorac Surg 2018; 106:822-829. [DOI: 10.1016/j.athoracsur.2017.11.032] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/09/2017] [Revised: 11/03/2017] [Accepted: 11/08/2017] [Indexed: 10/18/2022]
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El-Harasis MA, Connolly HM, Miranda WR, Qureshi MY, Sharma N, Al-Otaibi M, DeSimone CV, Egbe A. Progressive right ventricular enlargement due to pulmonary regurgitation: Clinical characteristics of a "low-risk" group. Am Heart J 2018; 201:136-140. [PMID: 29793063 DOI: 10.1016/j.ahj.2018.04.013] [Citation(s) in RCA: 17] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/12/2018] [Accepted: 04/16/2018] [Indexed: 12/18/2022]
Abstract
BACKGROUND The optimal interval between serial cardiac magnetic resonance imaging (CMRI) scans for monitoring right ventricular (RV) enlargement in the setting of severe pulmonic valve regurgitation (PR) is unknown. The purposes of this study were to (1) determine the annual change in RV volume on serial CMRI scans and (2) identify the risk factors for rapid progression of RV enlargement. METHODS A retrospective study of adults with postintervention native valve PR and ≥2 CMRI scans at Mayo Clinic Rochester from 2000 to 2015 was conducted. Rapid progression of RV enlargement was defined as first upper quartile of annual increase in RV end-diastolic volume index (RVEDVi) for the cohort. RESULTS Of the 63 patients (age, 36 ± 9 years) in the study, 43 (68%) had tetralogy of Fallot, whereas 20 (32%) had valvular pulmonic stenosis. Right ventricular outflow tract interventions that resulted in PR were balloon pulmonary valvuloplasty (n = 4; 7%), transannular patch repair (n = 30; 58%), and nontransannular patch repair (n = 18; 35%). Interval between baseline and second CMRI was 2 (1-4) years. In comparison to baseline CMRI, RVEDVi increased from 130 (109-141) to 135 (126-155) mL/m2 and median annual change in RVEDVi was 3.1 (1.7-5.9) mL/m2. Univariate risk factors for rapid progression of RV enlargement (annual increase in RVEDVi >6 mL/m2) were ≥moderate tricuspid regurgitation and RVEDVi >130 mL/m2. Among the 24 patients without these risk factors (low-risk subgroup), RVEDVi increased by only 3 (0-7) mL/m2 over 7 (5-9) years. CONCLUSIONS Patients with PR without RVEDVi >130 mL/m2 and/or ≥moderate tricuspid regurgitation represent a low-risk subgroup that may be appropriate for clinical and echo follow-up but may potentially require infrequent CMRI follow-up.
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Affiliation(s)
| | - Heidi M Connolly
- Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN
| | | | | | - Nandini Sharma
- Department of Internal Medicine, Mayo Clinic, Rochester, MN
| | | | | | - Alexander Egbe
- Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN.
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Bergersen L, Benson LN, Gillespie MJ, Cheatham SL, Crean AM, Hor KN, Horlick EM, Lung TH, McHenry BT, Osten MD, Powell AJ, Cheatham JP. Harmony Feasibility Trial: Acute and Short-Term Outcomes With a Self-Expanding Transcatheter Pulmonary Valve. JACC Cardiovasc Interv 2018; 10:1763-1773. [PMID: 28882284 DOI: 10.1016/j.jcin.2017.05.034] [Citation(s) in RCA: 97] [Impact Index Per Article: 13.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/19/2016] [Revised: 04/17/2017] [Accepted: 05/21/2017] [Indexed: 12/13/2022]
Abstract
OBJECTIVES This study sought to obtain in vivo data to confirm assumptions on device loading conditions and assess procedural feasibility, safety, and valve performance. BACKGROUND The Harmony transcatheter pulmonary valve (Medtronic, Minneapolis, Minnesota) was designed for patients with severe pulmonary regurgitation who require pulmonary valve replacement. METHODS Three sites participated in this first Food and Drug Administration-approved early feasibility study using an innovative device design to accommodate the complex anatomy of the right ventricular outflow tract. Potentially eligible patients underwent review by a screening committee to determine implant eligibility. Six-month outcomes are reported. RESULTS Between May 2013 and May 2015, 66 subjects were enrolled, and 21 were approved for implant and underwent catheterization; 20 were implanted. Catheterized patients had a median age of 25 years, were predominantly diagnosed with tetralogy of Fallot (95%), had severe pulmonary regurgitation (95%), and had trivial or mild stenosis. The device was delivered in the desired location in 19 of 20 (95%) patients. Proximal migration occurred in 1 patient during delivery system removal. Two devices were surgically explanted. Premature ventricular contractions related to the procedure were reported in 3 patients; 2 were resolved without treatment. One patient had ventricular arrhythmias that required treatment and later were resolved. At 1 month, echocardiography revealed none or trivial pulmonary regurgitation in all and a mean right ventricular outflow tract gradient of 16 ± 8 mm Hg (range 6 to 31 mm Hg). CONCLUSIONS In this feasibility study of the Harmony transcatheter pulmonary valve device, there was high procedural success and safety, and favorable acute device performance.
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Affiliation(s)
- Lisa Bergersen
- Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts.
| | - Lee N Benson
- Division of Cardiology, Labatt Family Heart Center, Hospital for Sick Children, Toronto, Ontario, Canada
| | - Matthew J Gillespie
- Department of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
| | - Sharon L Cheatham
- Department of Cardiology, Nationwide Children's Hospital, Columbus, Ohio
| | - Andrew M Crean
- Peter Munk Cardiac Centre, University Health Network, Toronto General Hospital, Toronto, Ontario, Canada; Joint Department of Medical Imaging, Toronto General Hospital, Toronto, Ontario, Canada
| | - Kan N Hor
- Department of Cardiology, Nationwide Children's Hospital, Columbus, Ohio
| | - Eric M Horlick
- Peter Munk Cardiac Centre, University Health Network, Toronto General Hospital, Toronto, Ontario, Canada
| | - Te-Hsin Lung
- Coronary and Structural Heart Clinical Department, Medtronic, Santa Rosa, California
| | - Brian T McHenry
- Coronary and Structural Heart Research and Innovation Department, Medtronic, Mounds View, Minnesota
| | - Mark D Osten
- Peter Munk Cardiac Centre, University Health Network, Toronto General Hospital, Toronto, Ontario, Canada
| | - Andrew J Powell
- Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts
| | - John P Cheatham
- Department of Cardiology, Nationwide Children's Hospital, Columbus, Ohio
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Tatewaki H, Shiose A. Pulmonary valve replacement after repaired Tetralogy of Fallot. Gen Thorac Cardiovasc Surg 2018; 66:509-515. [PMID: 29779123 DOI: 10.1007/s11748-018-0931-0] [Citation(s) in RCA: 23] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/17/2018] [Accepted: 04/28/2018] [Indexed: 10/16/2022]
Abstract
In this review article, we describe pulmonary valve replacement (PVR) late after repaired Tetralogy of Fallot (TOF). Since the introduction of surgical intervention for patients with TOF in 1945, surgical management of TOF has dramatically improved early survival with mortality rates, less than 2-3%. However, the majority of these patients continue to experience residual right ventricular outflow tract pathology, most commonly pulmonary valve regurgitation (PR). The patients are generally asymptomatic during childhood and adolescence and, however, are at risk for severe PR later which can result in exercise intolerance, heart failure, arrhythmias, and sudden death. While it has been shown that PVR improves symptoms and functional status in these patients, the optimal timing and indications for PVR after repaired TOF are still debated. This article reviews the current state of management for the patient with PR after repaired TOF.
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Affiliation(s)
- Hideki Tatewaki
- Department of Cardiovascular Surgery, Kyushu University Hospital, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 8128582, Japan.
| | - Akira Shiose
- Department of Cardiovascular Surgery, Kyushu University Hospital, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 8128582, Japan
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Zampi JD, Whiteside W. Innovative interventional catheterization techniques for congenital heart disease. Transl Pediatr 2018; 7:104-119. [PMID: 29770292 PMCID: PMC5938250 DOI: 10.21037/tp.2017.12.02] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/14/2017] [Accepted: 12/01/2017] [Indexed: 11/06/2022] Open
Abstract
Since 1929, when the first cardiac catheterization was safely performed in a human by Dr. Werner Forssmann (on himself), there has been a rapid progression of cardiac catheterization techniques and technologies. Today, these advances allow us to treat a wide variety of patients with congenital heart disease using minimally invasive techniques; from fetus to infants to adults, and from simple to complex congenital cardiac lesions. In this article, we will explore some of the exciting advances in cardiac catheterization for the treatment of congenital heart disease, including transcatheter valve implantation, hybrid procedures, biodegradable technologies, and magnetic resonance imaging (MRI)-guided catheterization. Additionally, we will discuss innovations in imaging in the catheterization laboratory, including 3D rotational angiography (3DRA), fusion imaging, and 3D printing, which help to make innovative interventional approaches possible.
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Affiliation(s)
- Jeffrey D Zampi
- University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI, USA
| | - Wendy Whiteside
- University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI, USA
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Improving the role of echocardiography in studying the right ventricle of repaired tetralogy of Fallot patients: comparison with cardiac magnetic resonance. Int J Cardiovasc Imaging 2017; 34:399-406. [PMID: 28988308 DOI: 10.1007/s10554-017-1249-1] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/04/2017] [Accepted: 09/21/2017] [Indexed: 10/18/2022]
Abstract
Right ventricular (RV) evaluation represents one of the major clinical tasks in the follow-up of repaired tetralogy of Fallot patients (rToF) with pulmonary valve regurgitation, as both severe RV dilatation and dysfunction are key factors in defining the need of pulmonary valve replacement. The aim of our study was to report the diagnostic accuracy of echocardiography in the identification of rToF patients with severely dilated and/or depressed RV as compared to cardiac magnetic resonance (CMR). Among our patients with rToF, a subgroup of 95 (17.6 ± 6.8 years; 60% male), who underwent right ventricular qualitative and quantitative evaluation with CMR following echocardiographic suspicion of severe dilation/dysfunction, were included in the analysis. When comparing echocardiographic RV functional parameters to CMR findings, we found no association between CMR-ejection fraction (EF) and either tricuspid annulus plane systolic excursion (TAPSe) nor tissue Doppler systolic tricuspid excursion velocity (all p = ns). In contrast RVFAC was strongly associated with CMR-EF (r = 0.44; p < 0.01) as well as to longitudinal components of RV mechanics including tissue Doppler s' (r = 0.40; p < 0.01) and TAPSE (r = 0.36; p < 0.01). When comparing echocardiographic and CMR structural parameters of the RV, we found that CMR RV volume was strongly related to echocardiographic measurements of RV end diastolic area (from the 4 chamber apical view) and with proximal parasternal short axis right ventricle outflow-dimension. Accordingly a regression model was derived from multiple regression analysis, which allows a more accurate estimate of CMR RV volume from echocardiography (r2 = 0.59, p < 0.001). Our study demonstrates a significant, although imperfect, correlation between echocardiographic and CMR RV functional and geometrical parameters. Combining echocardiographic measures of RV inflow and RV outflow, we deliver a simple formula to estimate CMR-RV volume, improving the echocardiographic accuracy in RV volume quantification.
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Abstract
PURPOSE OF REVIEW Our review is intended to provide readers with an overview of disease processes involving the pulmonic valve, highlighting recent outcome studies and guideline-based recommendations; with focus on the two most common interventions for treating pulmonic valve disease, balloon pulmonary valvuloplasty and pulmonic valve replacement. RECENT FINDINGS The main long-term sequelae of balloon pulmonary valvuloplasty, the gold standard treatment for pulmonic stenosis, remain pulmonic regurgitation and valvular restenosis. The balloon:annulus ratio is a major contributor to both, with high ratios resulting in greater degrees of regurgitation, and small ratios increasing risk for restenosis. Recent studies suggest that a ratio of approximately 1.2 may provide the most optimal results. Pulmonic valve replacement is currently the procedure of choice for patients with severe pulmonic regurgitation and hemodynamic sequelae or symptoms, yet it remains uncertain how it impacts long-term survival. Transcatheter pulmonic valve replacement is a rapidly evolving field and recent outcome studies suggest short and mid-term results at least equivalent to surgery. The Melody valve® was FDA approved for failing pulmonary surgical conduits in 2010 and for failing bioprosthetic surgical pulmonic valves in 2017 and has been extensively studied, whereas the Sapien XT valve®, offering larger diameters, was approved for failing pulmonary conduits in 2016 and has been less extensively studied. Patients with pulmonic valve disease deserve lifelong surveillance for complications. Transcatheter pulmonic valve replacement is a novel and attractive therapeutic option, but is currently only FDA approved for patients with failing pulmonary conduits or dysfunctional surgical bioprosthetic valves. New advances will undoubtedly increase the utilization of this rapidly expanding technology.
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Diagnostic value of Doppler echocardiography for identifying hemodynamic significant pulmonary valve regurgitation in tetralogy of Fallot: comparison with cardiac MRI. Int J Cardiovasc Imaging 2017; 33:1723-1730. [PMID: 28567705 PMCID: PMC5682867 DOI: 10.1007/s10554-017-1165-4] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/21/2017] [Accepted: 05/08/2017] [Indexed: 12/01/2022]
Abstract
Quantification of pulmonary regurgitation (PR) is essential in the management of patients with repaired tetralogy of Fallot (TOF). We sought to evaluate the accuracy of first-line Doppler echocardiography in comparison with cardiac magnetic resonance imaging (MRI) to identify hemodynamic significant PR. Paired cardiac MRI and echocardiographic studies (n = 97) in patients with repaired TOF were retrospectively analyzed. Pressure half time (PHT) and pulmonary regurgitation index (PRi) were measured using continuous wave Doppler. The ratio of the color flow Doppler regurgitation jet width to pulmonary valve (PV) annulus (jet/annulus ratio) and diastolic to systolic time velocity integral (DSTVI; pulsed wave Doppler) were assessed. Accuracy of echocardiographic measurements was tested to identify significant PR as determined by phase-contrast MRI (PR fraction [PRF] ≥ 20%). Mean PRF was 29.4 ± 15.7%. PHT < 100 ms had a sensitivity of 93%, specificity 75%, positive predictive value (PPV) 92% and negative predictive value (NPV) 78% for identifying significant PR (C-statistic 0.82). PRi < 0.77 had sensitivity and specificity of 66% and 54%, respectively (C-statistic 0.63). Jet/annulus ratio ≥1/3 had sensitivity 96%, specificity 75%, PPV 92% and NPV 82% (C-statistic 0.87). DSTVI had sensitivity 84%, specificity 33%, PPV 84% and NPV 40%, (C-statistic 0.56). Combined jet/annulus ratio ≥1/3 and PHT < 100 ms was highly accurate in identifying PRF ≥ 20%, with sensitivity 97% and specificity 100%. PHT and jet/annulus ratio on Doppler echocardiography, especially when combined, are highly accurate in identifying significant PR and therefore seem useful in the follow-up of patients with repaired TOF.
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Reddy S, Hu DQ, Zhao M, Blay E, Sandeep N, Ong SG, Jung G, Kooiker KB, Coronado M, Fajardo G, Bernstein D. miR-21 is associated with fibrosis and right ventricular failure. JCI Insight 2017; 2:91625. [PMID: 28469078 DOI: 10.1172/jci.insight.91625] [Citation(s) in RCA: 31] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/07/2016] [Accepted: 03/23/2017] [Indexed: 12/14/2022] Open
Abstract
Combined pulmonary insufficiency (PI) and stenosis (PS) is a common long-term sequela after repair of many forms of congenital heart disease, causing progressive right ventricular (RV) dilation and failure. Little is known of the mechanisms underlying this combination of preload and afterload stressors. We developed a murine model of PI and PS (PI+PS) to identify clinically relevant pathways and biomarkers of disease progression. Diastolic dysfunction was induced (restrictive RV filling, elevated RV end-diastolic pressures) at 1 month after generation of PI+PS and progressed to systolic dysfunction (decreased RV shortening) by 3 months. RV fibrosis progressed from 1 month (4.4% ± 0.4%) to 3 months (9.2% ± 1%), along with TGF-β signaling and tissue expression of profibrotic miR-21. Although plasma miR-21 was upregulated with diastolic dysfunction, it was downregulated with the onset of systolic dysfunction), correlating with RV fibrosis. Plasma miR-21 in children with PI+PS followed a similar pattern. A model of combined RV volume and pressure overload recapitulates the evolution of RV failure unique to patients with prior RV outflow tract surgery. This progression was characterized by enhanced TGF-β and miR-21 signaling. miR-21 may serve as a plasma biomarker of RV failure, with decreased expression heralding the need for valve replacement.
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Affiliation(s)
- Sushma Reddy
- Department of Pediatrics, Division of Cardiology, Stanford University, Stanford, California, USA
| | - Dong-Qing Hu
- Department of Pediatrics, Division of Cardiology, Stanford University, Stanford, California, USA
| | - Mingming Zhao
- Department of Pediatrics, Division of Cardiology, Stanford University, Stanford, California, USA
| | - Eddie Blay
- Department of Surgery, Temple University, Philadelphia, Pennsylvania, USA
| | - Nefthi Sandeep
- Department of Pediatrics, Division of Cardiology, Stanford University, Stanford, California, USA
| | - Sang-Ging Ong
- Cardiovascular Institute, Stanford University, Stanford, California, USA
| | - Gwanghyun Jung
- Department of Pediatrics, Division of Cardiology, Stanford University, Stanford, California, USA
| | - Kristina B Kooiker
- Department of Pediatrics, Division of Cardiology, Stanford University, Stanford, California, USA
| | - Michael Coronado
- Department of Pediatrics, Division of Cardiology, Stanford University, Stanford, California, USA
| | - Giovanni Fajardo
- Department of Pediatrics, Division of Cardiology, Stanford University, Stanford, California, USA
| | - Daniel Bernstein
- Department of Pediatrics, Division of Cardiology, Stanford University, Stanford, California, USA
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The value of right ventricular longitudinal strain in the evaluation of adult patients with repaired tetralogy of Fallot: a new tool for a contemporary challenge. Cardiol Young 2017; 27:498-506. [PMID: 27226193 DOI: 10.1017/s1047951116000810] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/23/2023]
Abstract
OBJECTIVE The role of right ventricular longitudinal strain for assessing patients with repaired tetralogy of Fallot is not fully understood. In this study, we aimed to evaluate its relation with other structural and functional parameters in these patients. METHODS Patients followed-up in a grown-up CHD unit, assessed by transthoracic echocardiography, cardiac MRI, and treadmill exercise testing, were retrospectively evaluated. Right ventricular size and function and pulmonary regurgitation severity were assessed by echocardiography and MRI. Right ventricular longitudinal strain was evaluated in the four-chamber view using the standard semiautomatic method. RESULTS In total, 42 patients were included (61% male, 32±8 years). The mean right ventricular longitudinal strain was -16.2±3.7%, and the right ventricular ejection fraction, measured by MRI, was 42.9±7.2%. Longitudinal strain showed linear correlation with tricuspid annular systolic excursion (r=-0.40) and right ventricular ejection fraction (r=-0.45) (all p<0.05), which in turn showed linear correlation with right ventricular fractional area change (r=0.50), pulmonary regurgitation colour length (r=0.35), right ventricular end-systolic volume (r=-0.60), and left ventricular ejection fraction (r=0.36) (all p<0.05). Longitudinal strain (β=-0.72, 95% confidence interval -1.41, -0.15) and left ventricular ejection fraction (β=0.39, 95% confidence interval 0.11, 0.67) were independently associated with right ventricular ejection fraction. The best threshold of longitudinal strain for predicting a right ventricular ejection fraction of <40% was -17.0%. CONCLUSIONS Right ventricular longitudinal strain is a powerful method for evaluating patients with tetralogy of Fallot. It correlated with echocardiographic right ventricular function parameters and was independently associated with right ventricular ejection fraction derived by MRI.
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Agrawal H, Alkashkari W, Kenny D. Evolution of hybrid interventions for congenital heart disease. Expert Rev Cardiovasc Ther 2017; 15:257-266. [DOI: 10.1080/14779072.2017.1307733] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
Affiliation(s)
- Hitesh Agrawal
- The Lillie Frank Abercrombie Section of Cardiology, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, USA
| | - Wail Alkashkari
- King Faisal Cardiac Center, King Saud Bin Abdulaziz University, King Abdulaziz Medical City, Jeddah, Saudi Arabia
| | - Damien Kenny
- Department of Cardiology, Our Lady’s Children’s Hospital, Crumlin, Dublin, Ireland
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