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Tan RB, Pierce KA, Nielsen J, Sanatani S, Fridman MD, Stephenson EA, Rangu S, Escudero C, Mah D, Hill A, Kane AM, Chaouki AS, Ochoa Nunez L, Kwok SY, Tsao S, Kallas D, Asaki SY, Behere S, Dubin A, Ratnasamy C, Robinson JA, Janson CM, Cecchin F, Shah MJ. Dual- vs Single-Chamber Ventricular Pacing in Isolated Congenital Complete Atrioventricular Block in Infancy. JACC Clin Electrophysiol 2025; 11:987-998. [PMID: 40019417 DOI: 10.1016/j.jacep.2024.12.025] [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: 06/06/2024] [Revised: 12/20/2024] [Accepted: 12/23/2024] [Indexed: 03/01/2025]
Abstract
BACKGROUND The optimal pacemaker programming strategy for infants with isolated congenital complete atrioventricular block (CCAVB) remains unresolved. Dual-chamber pacing maintains atrioventricular synchrony and physiological heart rate variability but increases the burden of ventricular pacing on a myocardium that may be inherently prone to left ventricular (LV) dysfunction. OBJECTIVES This study sought to compare clinical outcomes of dual (DDD)- vs single (VVI)- chamber pacing in infants with CCAVB (DAVINCHI). METHODS A multicenter retrospective study (2006-2023) identified infants with CCAVB and pacemaker implant at <1 year, with single-site ventricular pacing and no significant congenital heart disease. Outcome measured were clinically significant LV dysfunction, mortality, and complications. RESULTS A total of 109 infants (64% autoimmune CCAVB) were identified, 60.6% had VVI pacing. Over a median follow-up of 5 years, 60 complications occurred in 47 subjects (43.1%). Smaller infants had more complications. Clinically significant LV dysfunction developed in 11 (10.1%) and was more frequent in DDD (21% vs 3%; P = 0.006). LV dysfunction resulted in mortality in 1 patient and 10 patients required a change in pacing mode. Independent risk factors for LV dysfunction were DDD pacing and neonatal implant. Right ventricular pacing lead placement had a higher HR (HR: 2.67) for LV dysfunction but was not statistically significant (P = 0.20). CONCLUSIONS DDD pacing increases LV dysfunction risk compared with VVI in infants with CCAVB. Single-chamber LV apical pacing should be considered in infants with isolated CCAVB who require pacing. There is a high risk of pacing-related complications, particularly with an increased risk of ventricular lead complications in low-weight neonates.
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Affiliation(s)
- Reina Bianca Tan
- Department of Pediatrics, NYU Grossman School of Medicine, Hassenfeld Children's Hospital, New York, New York, USA.
| | - Kristyn A Pierce
- Department of Pediatrics, NYU Grossman School of Medicine, Hassenfeld Children's Hospital, New York, New York, USA
| | - James Nielsen
- Department of Pediatrics, NYU Grossman School of Medicine, Hassenfeld Children's Hospital, New York, New York, USA
| | - Shubhayan Sanatani
- Department of Pediatrics, BC Children's Hospital, Vancouver, British Columbia, Canada
| | - Michael D Fridman
- Department of Pediatrics, Alberta Children's Hospital, University of Calgary, Calgary, Alberta Canada
| | - Elizabeth A Stephenson
- Department of Paediatrics, The Hospital for Sick Children, Labatt Family Heart Centre, University of Toronto Department of Paediatrics, Toronto, Ontario, Canada
| | - Sowmith Rangu
- Department of Pediatrics, The University of Texas at Austin and Dell Children's Medical Center, Austin, Texas, USA
| | - Carolina Escudero
- Department of Paediatrics, Stollery Children's Hospital, University of Alberta, Edmonton, Alberta, Canada
| | - Douglas Mah
- Department of Pediatrics, Boston Children's Hospital; Harvard Medical School, Boston Massachusetts, USA
| | - Allison Hill
- Department of Pediatrics, Children's Hospital Los Angeles, Los Angeles, California, USA
| | - Austin M Kane
- Department of Pediatrics, University of Alabama at Birmingham, Birmingham, Alabama, USA
| | - A Sami Chaouki
- Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - Luis Ochoa Nunez
- Department of Pediatrics, The University of Texas Health Science Center at Houston, Children's Memorial Hermann Hospital, Houston, Texas, USA
| | - Sit-Yee Kwok
- Department of Pediatrics, Hong Kong Children's Hospital, The University of Hong Kong, Hong Kong, China
| | - Sabrina Tsao
- Department of Pediatrics, Hong Kong Children's Hospital, The University of Hong Kong, Hong Kong, China
| | - Dania Kallas
- Department of Pediatrics, BC Children's Hospital, Vancouver, British Columbia, Canada
| | - S Yukiko Asaki
- Department of Pediatrics, University of Utah/Primary Children's Hospital, Salt Lake City, Utah, USA
| | - Shashank Behere
- Department of Pediatrics, University of Oklahoma, Oklahoma City, Oklahoma, USA
| | - Anne Dubin
- Department of Pediatrics, Stanford University, Pediatric Cardiology, Palo Alto, California, USA
| | - Christopher Ratnasamy
- Department of Pediatrics, Helen DeVos Children's Hospital, Grand Rapids, Michigan, USA
| | - Jeffrey A Robinson
- Department of Pediatrics, University of Nebraska Medical Center, Omaha, Nebraska, USA
| | - Christopher M Janson
- Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania, USA
| | - Frank Cecchin
- Department of Pediatrics, NYU Grossman School of Medicine, Hassenfeld Children's Hospital, New York, New York, USA
| | - Maully J Shah
- Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania, USA
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Weinreb SJ, Ampah SB, Okunowo O, Griffis H, Vetter VL. Longitudinal echocardiographic parameters before and after pacemaker placement in congenital complete heart block. Heart Rhythm 2024; 21:454-461. [PMID: 37981292 DOI: 10.1016/j.hrthm.2023.11.015] [Citation(s) in RCA: 2] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/09/2023] [Revised: 11/14/2023] [Accepted: 11/15/2023] [Indexed: 11/21/2023]
Abstract
BACKGROUND Congenital complete heart block (CCHB) is seen in 1:15,000-1:20,000 live births, with risk of left ventricular (LV) dysfunction or dilated cardiomyopathy in 7%-23% of subjects. OBJECTIVE The purpose of this study was to investigate serial changes in LV size and systolic function in paced CCHB subjects to examine the effect of time from pacemaker on echocardiographic parameters. METHODS Single-center retrospective cohort analysis of paced CCHB subjects was performed. Echocardiographic data were collected before and after pacemaker placement. Linear mixed effect regression of left ventricular end-diastolic dimension (LVEDD) z-score, left ventricular shortening fraction (LVSF), and left ventricular ejection fraction (LVEF) was performed, with slopes compared before and after pacemaker placement. RESULTS Of 114 CCHB subjects, 52 had echocardiographic data before and after pacemaker placement. Median age at CCHB diagnosis was 0.6 [interquartile range 0.0-3.5] years; age at pacemaker placement 3.4 [0.5-9.0] years; and pacing duration 10.8 [5.2-13.7] years. Estimated LVEDD z-score was 1.4 at pacemaker placement and decreased -0.08 per year (95% confidence interval [CI] -0.12 to -0.04; P = .002) to 0.2 (95% CI -0.3 to +0.3) 15 years postplacement. Estimated LVSF decreased -1.1% per year (95% CI -1.7% to -0.6%; P <.001) from 6 months prepacemaker placement to 34% (95% CI 32%-37%) 4 years postplacement. There was no significant change in LVSF between 4 and 15 years postplacement. Estimated LVEF did not change significantly after pacemaker placement, with estimated LVEF 59% (95% CI 55%-62%) 15 years postplacement. CONCLUSION In 52 paced CCHB subjects, estimated LVEDD z-score decreased significantly after pacemaker placement, and estimated LVSF and LVEF remained within normal limits at 15 years postpacemaker placement.
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Affiliation(s)
- Scott J Weinreb
- Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
| | - Steve B Ampah
- Data Science and Biostatistics Unit, Department of Biomedical and Health Informatics, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
| | - Oluwatimilehin Okunowo
- Department of Computational and Quantitative Medicine, Division of Biostatistics, Beckman Research Institute of City of Hope, Duarte, California
| | - Heather Griffis
- Data Science and Biostatistics Unit, Department of Biomedical and Health Informatics, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
| | - Victoria L Vetter
- Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania
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Kyriakou S, Lubig A, Sandhoff CA, Kuhn Y, Jockenhoevel S. Influence of Diameter and Cyclic Mechanical Stimulation on the Beating Frequency of Myocardial Cell-Laden Fibers. Gels 2023; 9:677. [PMID: 37754359 PMCID: PMC10528042 DOI: 10.3390/gels9090677] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/22/2023] [Revised: 08/12/2023] [Accepted: 08/21/2023] [Indexed: 09/28/2023] Open
Abstract
Atrioventricular block (AVB) is a severe disease for pediatric patients. The repetitive operations needed in the case of the pacemaker implantation to maintain the electrical signal at the atrioventricular node (AVN) affect the patient's life quality. In this study, we present a method of biofabrication of multi-cell-laden cylindrical fibrin-based fibers that can restore the electrical signal at the AVN. We used human umbilical vein smooth muscle cells (HUVSMCs), human umbilical vein endothelial cells (HUVECs) and induced pluripotent stem cell cardiomyocytes (iPSC-CMs) cultivated either statically or dynamically to mimic the native AVN. We investigated the influence of cell composition, construct diameter and cyclic stretch on the function of the fibrin hydrogels in vitro. Immunohistochemistry analyses showed the maturity of the iPSC-CMs in the constructs through the expression of sarcomeric alpha actinin (SAA) and electrical coupling through Connexin 43 (Cx43) signal. Simultaneously, the beating frequency of the fibrin hydrogels was higher and easy to maintain whereas the concentration of iPSC-CMs was higher compared with the other types of cylindrical constructs. In total, our study highlights that the combination of fibrin with the cell mixture and geometry is offering a feasible biofabrication method for tissue engineering approaches for the treatment of AVB.
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Affiliation(s)
- Stavroula Kyriakou
- Department of Biohybrid & Medical Textiles (BioTex), AME-Institute of Applied Medical Engineering, Helmholtz Institute, RWTH Aachen University, 52074 Aachen, Germany; (S.K.); (C.A.S.); (Y.K.)
| | - Andreas Lubig
- Department of Biohybrid & Medical Textiles (BioTex), AME-Institute of Applied Medical Engineering, Helmholtz Institute, RWTH Aachen University, 52074 Aachen, Germany; (S.K.); (C.A.S.); (Y.K.)
| | - Cilia A. Sandhoff
- Department of Biohybrid & Medical Textiles (BioTex), AME-Institute of Applied Medical Engineering, Helmholtz Institute, RWTH Aachen University, 52074 Aachen, Germany; (S.K.); (C.A.S.); (Y.K.)
| | - Yasmin Kuhn
- Department of Biohybrid & Medical Textiles (BioTex), AME-Institute of Applied Medical Engineering, Helmholtz Institute, RWTH Aachen University, 52074 Aachen, Germany; (S.K.); (C.A.S.); (Y.K.)
| | - Stefan Jockenhoevel
- Department of Biohybrid & Medical Textiles (BioTex), AME-Institute of Applied Medical Engineering, Helmholtz Institute, RWTH Aachen University, 52074 Aachen, Germany; (S.K.); (C.A.S.); (Y.K.)
- AMIBM-Aachen-Maastricht-Institute for Biobased Materials, Maastricht University, 186260 Geleen, The Netherlands
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Jičínský M, Kubuš P, Pavlíková M, Ložek M, Janoušek J. Natural History of Nonsurgical Complete Atrioventricular Block in Children and Predictors of Pacemaker Implantation. JACC Clin Electrophysiol 2023; 9:1379-1389. [PMID: 37086232 DOI: 10.1016/j.jacep.2023.02.016] [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: 07/21/2022] [Revised: 02/02/2023] [Accepted: 02/15/2023] [Indexed: 04/23/2023]
Abstract
BACKGROUND Data on the natural history of complete atrioventricular block (CAVB) in children are scarce, and criteria for pacemaker (PM) implantation are based on low levels of evidence. OBJECTIVES This study aimed to evaluate the natural course and predictors of PM implantation in a nationwide cohort of pediatric patients with nonsurgical CAVB. METHODS All children with CAVB in the absence of structural heart disease presenting from 1977 to 2016 were retrospectively identified, yielding 95 subjects with a mean age of 4.05 years at the first presentation with a follow-up median of 0.80 years (IQR: 0.02-6.82 years). PM implantation was performed according to the available guidelines. Serial 24-hour Holter recordings and echocardiograms were reviewed. Predictors of PM implantation performed >1 month after the first presentation were evaluated. RESULTS The minimum and mean 24-hour heart rates and maximum RR intervals had a nonlinear correlation with age (P < 0.0001 for all). The left ventricular (LV) size was moderately increased, and the shortening fraction was normal in the majority throughout follow-up. PM implantation was performed in 62 patients (65.3%) reaching guideline criteria. The mean 24-hour heart rate at presentation was a predictor of subsequent PM implantation (HR: 0.938; 95% CI: 0.894-0.983; P = 0.003 per unit increase) regardless of age at presentation. Patients presenting with a mean 24-hour heart rate >58 beats/min (>75th percentile) had a high probability of freedom from PM within the subsequent 5 years (91.7% vs 44.4%; P < 0.001). CONCLUSIONS Pediatric patients with CAVB showed an age-dependent decrease in heart rate, moderate LV dilation, and preserved LV function. The probability of subsequent PM implantation could be predicted by the heart rate profile at presentation, defining a low-risk group and allowing for individualized follow-up.
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Affiliation(s)
- Michal Jičínský
- Children's Heart Centre, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic.
| | - Peter Kubuš
- Children's Heart Centre, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic
| | - Markéta Pavlíková
- Faculty of Mathematics and Physics, Charles University in Prague, Prague, Czech Republic
| | - Miroslav Ložek
- Children's Heart Centre, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic; 1st Faculty of Medicine, Charles University in Prague, Prague, Czech Republic
| | - Jan Janoušek
- Children's Heart Centre, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic
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Abstract
Congenital complete heart block (CCHB) defines atrioventricular conduction abnormalities diagnosed in utero or within the first 27 days of life. Maternal autoimmune disease and congenital heart defects are most commonly responsible. Recent genetic discoveries have highlighted our understanding of the underlying mechanism. Hydroxychloroquine shows promise in preventing autoimmune CCHB. Patients may develop symptomatic bradycardia and cardiomyopathy. The presence of these and other specific findings warrants placement of a permanent pacemaker to relieve symptoms and prevent catastrophic events. The mechanisms, natural history, evaluation, and treatment of patients with or at risk for CCHB are reviewed.
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Affiliation(s)
- Leonard Steinberg
- Pediatric Cardiology, Children's Heart Center, Ascension St. Vincent, 8333 Naab Rd, Ste 320, Indianapolis, IN 46260, USA.
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Navarre BM, Clouthier KL, Ji X, Taylor A, Weldy CS, Dubin AM, Reddy S. miR Profile of Chronic Right Ventricular Pacing: a Pilot Study in Children with Congenital Complete Atrioventricular Block. J Cardiovasc Transl Res 2023; 16:287-299. [PMID: 36121621 PMCID: PMC10151311 DOI: 10.1007/s12265-022-10318-w] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/16/2022] [Accepted: 09/01/2022] [Indexed: 11/28/2022]
Abstract
Chronic ventricular pacing can lead to pacing-induced cardiomyopathy (PICM). Clinical data alone is insufficient to predict who will develop PICM. Our study aimed to evaluate the circulating miR profile associated with chronic right ventricular pacing in children with congenital complete AV block (CCAVB) and to identify candidate miRs for longitudinal monitoring. Clinical data and blood were collected from chronically paced children (N = 9) and compared with non-paced controls (N = 13). miR microarrays from the buffy coat revealed 488 differentially regulated miRs between groups. Pathway analysis predicted both adaptive and maladaptive miR signaling associated with chronic pacing despite preserved ventricular function. Greater profibrotic signaling (miRs-92a, 130, 27, 29) and sodium and calcium channel dysregulation (let-7) were seen in those paced > 10 years with the most dyregulation seen in a patient with sudden death vs. those paced < 10 years. These miRs may help to identify early adverse remodeling in this population.
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Affiliation(s)
- Brittany M Navarre
- Department of Pediatrics (Cardiology), Lucile Packard Children's Hospital, Stanford University, 750 Welch Road, Suite 325, Stanford, CA, 94304, USA
| | - Katie L Clouthier
- Department of Pediatrics (Cardiology), Lucile Packard Children's Hospital, Stanford University, 750 Welch Road, Suite 325, Stanford, CA, 94304, USA
| | - Xuhuai Ji
- Human Immune Monitoring Center and Functional Genomics Facility, Stanford University, Stanford, CA, 94305, USA
| | - Anne Taylor
- Department of Pediatrics (Cardiology), Lucile Packard Children's Hospital, Stanford University, 750 Welch Road, Suite 325, Stanford, CA, 94304, USA
| | - Chad S Weldy
- Department of Medicine (Cardiovascular), Stanford Medical Center, Stanford University, 300 Pasteur Drive, Stanford, CA, 94305, USA
| | - Anne M Dubin
- Department of Pediatrics (Cardiology), Lucile Packard Children's Hospital, Stanford University, 750 Welch Road, Suite 325, Stanford, CA, 94304, USA
| | - Sushma Reddy
- Department of Pediatrics (Cardiology), Lucile Packard Children's Hospital, Stanford University, 750 Welch Road, Suite 325, Stanford, CA, 94304, USA.
- Cardiovascular Institute, Stanford University, Stanford, USA.
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Adverse effects of right ventricular pacing on cardiac function: prevalence, prevention and treatment with physiologic pacing. Trends Cardiovasc Med 2023; 33:109-122. [PMID: 34742888 DOI: 10.1016/j.tcm.2021.10.013] [Citation(s) in RCA: 28] [Impact Index Per Article: 14.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/27/2021] [Revised: 10/24/2021] [Accepted: 10/30/2021] [Indexed: 02/07/2023]
Abstract
Right ventricular (RV) pacing is the main treatment modality for patients with advanced atrioventricular (AV) block. Chronic RV pacing can cause cardiac systolic dysfunction and heart failure (HF). In this review, we discuss studies that have shown deleterious effects of chronic RV pacing on systolic cardiac function causing pacing-induced cardiomyopathy (PiCM), heart failure (HF), HF hospitalization, atrial fibrillation (AF) and cardiac mortality. RV apical pacing is the most widely used and studied. Adverse effects of RV pacing appear to be directly related to pacing burden and are worse in patients with pre-existing left ventricular (LV) dysfunction. Chronic RV pacing is also associated with heart failure with preserved ejection fraction (HFpEF). Mechanisms, risk factors, clinical and echocardiographic features, and strategies to minimize RV pacing-induced cardiac dysfunction are discussed in light of the latest data. Studies on biventricular (Bi-V) pacing upgrade in patients who develop RV PiCM, use of alternate RV pacing sites, de novo Bi-V pacing, and physiologic pacing using HIS bundle pacing (HBP) and left bundle area (LBBA) pacing in patients with an anticipated high RV pacing burden are discussed.
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Histological finding of maternal antibody-associated congenital heart block accompanied by large atrial septal defect and severe cardiac dysfunction. Cardiol Young 2022; 32:2032-2035. [PMID: 35351221 DOI: 10.1017/s1047951122001007] [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] [Indexed: 12/16/2022]
Abstract
Cardiac dysfunction commonly occurs in congenital heart block associated with maternal anti-SSA antibodies, especially after pacemaker implantation. We report the case of a 4-year-old girl with antibody-associated congenital heart block and a large secundum atrial septal defect who presented with significant cardiac dysfunction 4 years after pacemaker implantation. Histological findings were useful for determining the course of treatment and perioperative risk of intracardiac repair.
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Abstract
BACKGROUND Permanent pacing in children with isolated congenital complete atrioventricular block may cause left ventricular dysfunction. To prevent it, alternative pacing sites have been proposed: left ventricular epicardial or selective right ventricular endocardial pacing. AIMS To compare the functional outcome (left ventricular systolic function and synchrony) in paediatric patients with congenital complete atrioventricular block and left ventricular apical epicardial or right ventricular transvenous mid-septal pacing. METHODS Retrospective study. Epicardial leads were implanted by standard surgical technique, transvenous leads by 3D electroanatomic mapping systems. 3D mapping acquired 3D right ventricular local pacing map and defined the narrowest paced QRS site. 3D mapping guided screw-in bipolar leads on that ventricular site. Electrocardiogram (ECG) (QRS duration) and echocardiographic data (synchrony: interventricular mechanical delay, septal to posterior wall motion delay, systolic dyssynchrony index; contractility: global longitudinal strain, ejection fraction) were recorded. Data are reported as median [interquartile ranges]. p < 0.05 was significant. RESULTS There were 19 transvenous systems (age 8.8 [6-14] years; right ventricular mid-septum) and 17 epicardial systems (0.04 [0.001-0.6] years; left ventricular apex). Post-implantation QRS significantly widened either in endocardial or in epicardial patients. Most patients reached 4-year follow-up. One-year and 4-year ejection fraction and global longitudinal strain were mostly within normal limits and did not show significant differences between the two groups and between the same endocardial/epicardial group. Synchrony parameters were within normal limits in the two groups. CONCLUSIONS Left ventricular apical epicardial pacing and 3D mapping-guided right ventricular mid-septal pacing preserved left ventricular contractility and synchrony in children and adolescents with congenital complete atrioventricular block at short-/mid-term follow-up, without relevant significant differences between the two groups.
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Weinreb SJ, Okunowo O, Griffis H, Vetter V. Incidence of Morbidity and Mortality in a Cohort of Congenital Complete Heart Block Patients Followed Over 40 Years. Heart Rhythm 2022; 19:1149-1155. [PMID: 35217197 DOI: 10.1016/j.hrthm.2022.02.019] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/03/2021] [Revised: 02/14/2022] [Accepted: 02/16/2022] [Indexed: 01/11/2023]
Abstract
BACKGROUND Congenital Complete Heart Block (CCHB), seen in 1:15,000-20,000 births, commonly requires pacemaker placement by young adulthood. There is limited understanding of cardiac morbidity and mortality. OBJECTIVE Determine long-term incidence of cardiac morbidity and mortality in subjects with CCHB and identify associated risk factors. METHODS Retrospective cohort analysis of subjects with CCHB at Children's Hospital of Philadelphia between 1976-2018. The primary outcome is a composite of death, left ventricular systolic dysfunction, heart failure, cardiomyopathy, or cardiac resynchronization therapy (CRT). Cox proportional hazard models assessed independent risk factors for the primary outcome and its components (death, heart failure and/or cardiomyopathy, CRT). RESULTS One-hundred-fourteen subjects (58% female, median age at last visit 15.2 years) were included. Eighty-eight (77%) underwent pacemaker implantation, with median age at placement 1.9 years (IQR, 0.1-8.0 years). Twenty-six subjects (23%) reached the primary outcome - 7 (6%) died and 14 (12%) were diagnosed with heart failure and/or cardiomyopathy. Median time from diagnosis to primary outcome was 3.1 years (IQR, 0.0-10.8 years). There were no significant associations between age at diagnosis less than 1-year (hazard ratio [HR]: 1.5, 95% CI 0.6-3.9), fetal diagnosis (HR: 2.3, 0.96-5.6), or maternal antibody positivity (HR: 2.4, 0.9-6.6) and the primary outcome. Fetal diagnosis had a higher associated hazard of heart failure and/or cardiomyopathy (HR: 4.5, 1.3-15.0). CONCLUSION In 114 subjects with CCHB, 23% reached the composite outcome of cardiac morbidity and mortality, with no significant association between age at diagnosis, fetal diagnosis, and maternal antibody status with composite cardiac morbidity and mortality.
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Affiliation(s)
- Scott J Weinreb
- Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, 19146.
| | - Oluwatimilehin Okunowo
- Data Science and Biostatistics Unit, Department of Biomedical and Health Informatics, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, 19146
| | - Heather Griffis
- Data Science and Biostatistics Unit, Department of Biomedical and Health Informatics, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, 19146
| | - Victoria Vetter
- Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, 19146; Perelman School of Medicine at the University of Pennsylvania, 3400 Civic Center Blvd, Philadelphia, PA, 19146
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Mawad W, Hornberger L, Cuneo B, Raboisson MJ, Moon-Grady AJ, Lougheed J, Diab K, Parkman J, Silverman E, Jaeggi E. Outcome of Antibody-Mediated Fetal Heart Disease With Standardized Anti-Inflammatory Transplacental Treatment. J Am Heart Assoc 2022; 11:e023000. [PMID: 35001672 PMCID: PMC9238501 DOI: 10.1161/jaha.121.023000] [Citation(s) in RCA: 20] [Impact Index Per Article: 6.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
Background Transplacental fetal treatment of immune-mediated fetal heart disease, including third-degree atrioventricular block (AVB III) and endocardial fibroelastosis, is controversial. Methods and Results To study the impact of routine transplacental fetal treatment, we reviewed 130 consecutive cases, including 108 with AVB III and 22 with other diagnoses (first-degree/second-degree atrioventricular block [n=10]; isolated endocardial fibroelastosis [n=9]; atrial bradycardia [n=3]). Dexamethasone was started at a median of 22.4 gestational weeks. Additional treatment for AVB III included the use of a β-agonist (n=47) and intravenous immune globulin (n=34). Fetal, neonatal, and 1-year survival rates with AVB III were 95%, 93%, and 89%, respectively. Variables present at diagnosis that were associated with perinatal death included an atrial rate <90 beats per minute (odds ratio [OR], 258.4; 95% CI, 11.5-5798.9; P<0.001), endocardial fibroelastosis (OR, 28.9; 95% CI, 1.6-521.7; P<0.001), fetal hydrops (OR, 25.5; 95% CI, 4.4-145.3; P<0.001), ventricular dysfunction (OR, 7.6; 95% CI, 1.5-39.4; P=0.03), and a ventricular rate <45 beats per minute (OR, 12.9; 95% CI, 1.75-95.8; P=0.034). At a median follow-up of 5.9 years, 85 of 100 neonatal survivors were paced, and 1 required a heart transplant for dilated cardiomyopathy. Cotreatment with intravenous immune globulin was used in 16 of 22 fetuses with diagnoses other than AVB III. Neonatal and 1-year survival rates of this cohort were 100% and 95%, respectively. At a median age of 3.1 years, 5 of 21 children were paced, and all had normal ventricular function. Conclusions Our findings reveal a low risk of perinatal mortality and postnatal cardiomyopathy in fetuses that received transplacental dexamethasone±other treatment from the time of a new diagnosis of immune-mediated heart disease.
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Affiliation(s)
- Wadi Mawad
- The Hospital for Sick Children Toronto Ontario Canada
| | - Lisa Hornberger
- Stollery Children's Hospital University of Alberta Edmonton Alberta Canada
| | | | | | | | - Jane Lougheed
- Children's Hospital of Eastern Ontario Ottawa Ontario Canada
| | - Karim Diab
- Rush University Medical Center Chicago IL
| | - Julia Parkman
- Stollery Children's Hospital University of Alberta Edmonton Alberta Canada
| | | | - Edgar Jaeggi
- The Hospital for Sick Children Toronto Ontario Canada
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Hernstadt H, Regan W, Bhatt H, Rosenthal E, Meau-Petit V. Cohort study of congenital complete heart block among preterm neonates: a single-center experience over a 15-year period. Eur J Pediatr 2022; 181:1047-1054. [PMID: 34704129 PMCID: PMC8548064 DOI: 10.1007/s00431-021-04293-8] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/21/2021] [Revised: 10/02/2021] [Accepted: 10/10/2021] [Indexed: 11/24/2022]
Abstract
Congenital complete heart block (CCHB) is a very rare condition, with high risk of mortality. Prematurity is associated with immaturity of the cardiovascular system. Morbidity related to CCHB and prematurity has never been described. We describe a tertiary perinatal center experience over a 15-year period on CCHB management and complications in preterm infants. This is a single-center observational cohort study. All neonates admitted to neonatal intensive care unit with a diagnosis of isolated CCHB between January 2006 and January 2021 were identified. All preterm neonates (< 37 weeks) were compared with a control cohort of term neonates (≥ 37 weeks). Antenatal data, complications of prematurity, medical, and surgical management of CCHB were recorded. Twenty-four neonates with isolated CCHB (16 preterm and 8 term) were born during the study period, including 5 very preterm (< 32 weeks) and 11 preterm (32 to 37 weeks). All very preterm were born via emergency caesarian section without antenatal steroid administration. They had multiple severe morbidities including chronic lung disease, necrotizing enterocolitis, grades 3-4 intraventricular hemorrhage, cystic periventricular leukomalacia, and longer periods of mechanical and non-invasive ventilatory support than preterm. Thirteen out of sixteen preterm infants had permanent pacemakers inserted, compared to 1/8 for term newborns. All babies born before 35-week gestation were either paced or died.Conclusion: Premature neonates with CCHB have high risk of mortality and morbidity especially if undiagnosed and born by unnecessary emergency caesarian section without antenatal steroids. Prematurity below 35 weeks may be associated with death or pacemaker insertion. This supports better antenatal screening to avoid induced prematurity. What is Known: • Congenital complete heart block is a very rare condition associated with high morbidity and mortality. • Antenatal risk factors for poor outcome include fetal hydrops, low ventricular rate (HR <55 beats per minute), and congenital heart defect. What is New: • Infants born <32 weeks with CCHB had no antenatal steroid administration, and sustained high burden of morbidity (chronic lung disease, intraventricular hemorrhage, and cystic periventricular leukomalacia). • Birth <35 weeks is strongly associated with requiring pacing prior to discharge or death.
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Affiliation(s)
- Hayley Hernstadt
- Department of Neonatology, Evelina London Children's Hospital, London, UK. .,Department of Paediatrics, Monash University, Melbourne, VIC, Australia.
| | - William Regan
- Department of Paediatric Cardiology, Evelina London Children’s Hospital, London, UK
| | - Hitarth Bhatt
- Department of Paediatric Cardiology, Evelina London Children’s Hospital, London, UK
| | - Eric Rosenthal
- Department of Paediatric Cardiology, Evelina London Children’s Hospital, London, UK
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13
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Sagiv E, Hill AC, Silka MJ, Wood JC, Bar-Cohen Y. Assessment of echocardiographic parameters in children with permanent ventricular pacing. PROGRESS IN PEDIATRIC CARDIOLOGY 2021. [DOI: 10.1016/j.ppedcard.2021.101457] [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: 11/27/2022]
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14
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Bin S, Heng R, Im S. Complete heart block in neonatal lupus: a forgotten cause of fetal bradycardia. BMJ Case Rep 2021; 14:e246747. [PMID: 34785520 PMCID: PMC8596036 DOI: 10.1136/bcr-2021-246747] [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] [Accepted: 11/04/2021] [Indexed: 11/04/2022] Open
Abstract
The most common cause of congenital heart block (CHB) is neonatal lupus, an acquired autoimmune disease caused by transplacental transfer of maternal antibodies to the fetus. A full-term female neonate was admitted to neonatal intensive care unit for severe bradycardia with stable haemodynamics. The mother, showing no clinical symptoms or any particular history, was transferred to our tertiary centre for profound fetal bradycardia. At birth, the infant's ECG showed a third-degree atrioventricular block and echocardiography was normal. Cardiac neonatal lupus was confirmed with positive maternal anti-Ro antibodies. Under close monitoring, the infant tolerated the bradycardia well (median 67 beats per minute (bpm)) and was discharged on day 6 of life. There was no indication for pacemaker, but she would be on regular follow-up with a paediatric cardiologist. This article holds an important insight as it is the first confirmed case of autoimmune CHB in Cambodia in which the mother's antibody was found only after diagnosis on the neonate.
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Affiliation(s)
- Sakviseth Bin
- Neonatal Intensive Care Unit, Calmette Hospital, Phnom Penh, Cambodia
| | - Rathmony Heng
- Cardiology Department, Calmette Hospital, Phnom Penh, Cambodia
| | - Sethikar Im
- Neonatal Intensive Care Unit, Calmette Hospital, Phnom Penh, Cambodia
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15
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Silka MJ, Shah MJ, Avari Silva JN, Balaji S, Beach CM, Benjamin MN, Berul CI, Cannon B, Cecchin F, Cohen MI, Dalal AS, Dechert BE, Foster A, Gebauer R, Gonzalez Corcia MC, Kannankeril PJ, Karpawich PP, Kim JJ, Krishna MR, Kubuš P, LaPage MJ, Mah DY, Malloy-Walton L, Miyazaki A, Motonaga KS, Niu MC, Olen M, Paul T, Rosenthal E, Saarel EV, Silvetti MS, Stephenson EA, Tan RB, Triedman J, Von Bergen NH, Wackel PL. 2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients: Executive summary. Indian Pacing Electrophysiol J 2021; 21:349-366. [PMID: 34333142 PMCID: PMC8577082 DOI: 10.1016/j.ipej.2021.07.006] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022] Open
Abstract
Guidelines for the implantation of cardiac implantable electronic devices (CIEDs) have evolved since publication of the initial ACC/AHA pacemaker guidelines in 1984 [1]. CIEDs have evolved to include novel forms of cardiac pacing, the development of implantable cardioverter defibrillators (ICDs) and the introduction of devices for long term monitoring of heart rhythm and other physiologic parameters. In view of the increasing complexity of both devices and patients, practice guidelines, by necessity, have become increasingly specific. In 2018, the ACC/AHA/HRS published Guidelines on the Evaluation and Management of Patients with Bradycardia and Cardiac Conduction Delay [2], which were specific recommendations for patients >18 years of age. This age-specific threshold was established in view of the differing indications for CIEDs in young patients as well as size-specific technology factors. Therefore, the following document was developed to update and further delineate indications for the use and management of CIEDs in pediatric patients, defined as ≤21 years of age, with recognition that there is often overlap in the care of patents between 18 and 21 years of age. This document is an abbreviated expert consensus statement (ECS) intended to focus primarily on the indications for CIEDs in the setting of specific disease/diagnostic categories. This document will also provide guidance regarding the management of lead systems and follow-up evaluation for pediatric patients with CIEDs. The recommendations are presented in an abbreviated modular format, with each section including the complete table of recommendations along with a brief synopsis of supportive text and select references to provide some context for the recommendations. This document is not intended to provide an exhaustive discussion of the basis for each of the recommendations, which are further addressed in the comprehensive PACES-CIED document [3], with further data easily accessible in electronic searches or textbooks.
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Affiliation(s)
- Michael J Silka
- University of Southern California Keck School of Medicine, Los Angeles, California, USA.
| | - Maully J Shah
- University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.
| | | | | | | | - Monica N Benjamin
- Hospital de Pediatría Juan P. Garrahan, Hospital El Cruce, Hospital Británico de Buenos Aires, Instituto Cardiovascular ICBA, Buenos Aires, Argentina
| | | | | | - Frank Cecchin
- New York Univeristy Grossman School of Medicine, New York, NY, USA
| | | | - Aarti S Dalal
- Washington University in St. Louis, St. Louis, Missouri, USA
| | | | - Anne Foster
- Advocate Children's Heart Institute, Chicago, IL, USA
| | - Roman Gebauer
- Heart Centre Leipzig, University of Leipzig, Leipzig, Germany
| | | | | | - Peter P Karpawich
- University Pediatricians, Children's Hospital of Michigan, Detroit, MI, USA
| | | | | | - Peter Kubuš
- Children's Heart Center, Charles University in Prague and Motol University Hospital, Prague, Czech Republic
| | | | | | | | - Aya Miyazaki
- Shizuoka General Hospital and Mt. Fuji Shizuoka Children's Hospital, Shizuoka, Japan
| | | | - Mary C Niu
- University of Utah Health Sciences Center, Salt Lake City, UT, USA
| | | | - Thomas Paul
- Georg-August-University Medical Center, Göttingen, Germany
| | - Eric Rosenthal
- Evelina London Children's Hospital and St Thomas' Hospital, Guy's & St Thomas' NHS Foundation Trust, London, UK
| | | | | | | | - Reina B Tan
- New York University Langone Health, New York, NY, USA
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16
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Silka MJ, Shah MJ, Silva JNA, Balaji S, Beach CM, Benjamin MN, Berul CI, Cannon B, Cecchin F, Cohen MI, Dalal AS, Dechert BE, Foster A, Gebauer R, Gonzalez Corcia MC, Kannankeril PJ, Karpawich PP, Kim JJ, Krishna MR, Kubuš P, LaPage MJ, Mah DY, Malloy-Walton L, Miyazaki A, Motonaga KS, Niu MC, Olen M, Paul T, Rosenthal E, Saarel EV, Silvetti MS, Stephenson EA, Tan RB, Triedman J, Von Bergen NH, Wackel PL. 2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Executive Summary. Heart Rhythm 2021; 18:1925-1950. [PMID: 34363987 DOI: 10.1016/j.hrthm.2021.07.051] [Citation(s) in RCA: 19] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/19/2021] [Accepted: 07/19/2021] [Indexed: 10/20/2022]
Affiliation(s)
- Michael J Silka
- University of Southern California Keck School of Medicine, Los Angeles, California.
| | - Maully J Shah
- University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania.
| | | | | | | | - Monica N Benjamin
- Hospital de Pediatría Juan P. Garrahan, Hospital El Cruce, Hospital Británico de Buenos Aires, Instituto Cardiovascular ICBA, Buenos Aires, Argentina
| | | | | | - Frank Cecchin
- New York University Grossman School of Medicine, New York, New York
| | | | - Aarti S Dalal
- Washington University in St. Louis, St. Louis, Missouri
| | | | - Anne Foster
- Advocate Children's Heart Institute, Chicago, Illinois
| | - Roman Gebauer
- Heart Centre Leipzig, University of Leipzig, Leipzig, Germany
| | | | | | - Peter P Karpawich
- University Pediatricians, Children's Hospital of Michigan, Detroit, Michigan
| | | | | | - Peter Kubuš
- Children's Heart Center, Charles University in Prague and Motol University Hospital, Prague, Czech Republic
| | | | | | | | - Aya Miyazaki
- Shizuoka General Hospital and Mt. Fuji Shizuoka Children's Hospital, Shizuoka, Japan
| | | | - Mary C Niu
- University of Utah Health Sciences Center, Salt Lake City, Utah
| | | | - Thomas Paul
- Georg-August-University Medical Center, Göttingen, Germany
| | - Eric Rosenthal
- Evelina London Children's Hospital and St Thomas' Hospital, Guy's & St Thomas' NHS Foundation Trust, London, United Kingdom
| | | | | | | | - Reina B Tan
- New York University Langone Health, New York, New York
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17
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2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients: executive summary. Cardiol Young 2021; 31:1717-1737. [PMID: 34796795 DOI: 10.1017/s1047951121003395] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
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18
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Abstract
Congenital complete heart block (CCHB) defines atrioventricular conduction abnormalities diagnosed in utero or within the first 27 days of life. Maternal autoimmune disease and congenital heart defects are most commonly responsible. Recent genetic discoveries have highlighted our understanding of the underlying mechanism. Hydroxychloroquine shows promise in preventing autoimmune CCHB. Patients may develop symptomatic bradycardia and cardiomyopathy. The presence of these and other specific findings warrants placement of a permanent pacemaker to relieve symptoms and prevent catastrophic events. The mechanisms, natural history, evaluation, and treatment of patients with or at risk for CCHB are reviewed.
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19
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Cioffi GM, Gasperetti A, Tersalvi G, Schiavone M, Compagnucci P, Sozzi FB, Casella M, Guerra F, Dello Russo A, Forleo GB. Etiology and device therapy in complete atrioventricular block in pediatric and young adult population: Contemporary review and new perspectives. J Cardiovasc Electrophysiol 2021; 32:3082-3094. [PMID: 34570400 DOI: 10.1111/jce.15255] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/13/2021] [Revised: 08/24/2021] [Accepted: 09/11/2021] [Indexed: 11/30/2022]
Abstract
Complete atrioventricular block (CAVB) is a total dissociation between the atrial and ventricular activity, in the absence of atrioventricular conduction. Several diseases may result in CAVB in the pediatric and young-adult population. Permanent right ventricular (RV) pacing is required in permanent CAVB, when the cause is neither transient nor reversible. Continuous RV apical pacing has been associated with unfavorable outcomes in several studies due to the associated ventricular dyssynchrony. This study aims to summarize the current literature regarding CAVB in the pediatric and young adult population and to explore future treatment perspectives.
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Affiliation(s)
- Giacomo M Cioffi
- Division of Cardiology, Luzerner Kantonsspital, Luzern, Switzerland
| | - Alessio Gasperetti
- Department of Cardiology, ASST-Fatebenefratelli Sacco, Luigi Sacco University Hospital, Milan, Italy.,Cardiology and Arrhythmology Clinic, Department of Biomedical Sciences and Public Health, University Hospital "Umberto I-Lancisi-Salesi", Marche Polytechnic University, Ancona, Italy.,Department of Cardiology, Johns Hopkins Medicine, Baltimore, Maryland, USA
| | - Gregorio Tersalvi
- Division of Cardiology, Fondazione Cardiocentro Ticino, Lugano, Switzerland.,Department of Internal Medicine, Hirslanden Klinik St. Anna, Lucerne, Switzerland
| | - Marco Schiavone
- Department of Cardiology, ASST-Fatebenefratelli Sacco, Luigi Sacco University Hospital, Milan, Italy
| | - Paolo Compagnucci
- Cardiology and Arrhythmology Clinic, Department of Biomedical Sciences and Public Health, University Hospital "Umberto I-Lancisi-Salesi", Marche Polytechnic University, Ancona, Italy
| | - Fabiola B Sozzi
- Department of Cardiology, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy
| | - Michela Casella
- Cardiology and Arrhythmology Clinic, Department of Clinical, Special and Dental Sciences, University Hospital "Umberto I-Lancisi-Salesi", Marche Polytechnic University, Ancona, Italy
| | - Federico Guerra
- Cardiology and Arrhythmology Clinic, Department of Biomedical Sciences and Public Health, University Hospital "Umberto I-Lancisi-Salesi", Marche Polytechnic University, Ancona, Italy
| | - Antonio Dello Russo
- Cardiology and Arrhythmology Clinic, Department of Biomedical Sciences and Public Health, University Hospital "Umberto I-Lancisi-Salesi", Marche Polytechnic University, Ancona, Italy
| | - Giovanni Battista Forleo
- Department of Cardiology, ASST-Fatebenefratelli Sacco, Luigi Sacco University Hospital, Milan, Italy
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20
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Oliveira FR, Valim V, Pasoto SG, Fernandes MLMS, Lopes MLL, de Magalhães Souza Fialho SC, Pinheiro AC, Dos Santos LC, Appenzeller S, Fidelix T, Ribeiro SLE, de Brito DCSE, Libório T, Santos MCLFS, Tanure L, Gennari JDA, Civile VT, Pinto ACPN, Rocha-Filho CR, Miyamoto ST, Guedes LKN, Pugliesi A, Trevisani VFM. 2021 recommendations of the Brazilian Society of Rheumatology for the gynecological and obstetric care of patients with Sjogren's syndrome. Adv Rheumatol 2021; 61:54. [PMID: 34479630 DOI: 10.1186/s42358-021-00208-1] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/01/2021] [Accepted: 08/03/2021] [Indexed: 12/29/2022] Open
Abstract
Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs. Women with SS often experience gynecological symptoms due to the disease and need extra care regarding their sexual activity, reproductive health and during pregnancy, conditions that are not properly conducted in the clinical practice. To cover this gap, a panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis on the identification of symptoms, diagnosis, monitoring, prognosis, and treatment of these manifestations. A Focus Group meeting was held and included experts in the field and methodologists, based on a previously developed script, with themes related to the objective of the study. The most important topics were summarized and 11 recommendations were provided.
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Affiliation(s)
- Fabiola Reis Oliveira
- Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto (HCFMRP-USP), Av. Bandeirantes, 3900, Vila Monte Alegre, Ribeirão Preto, SP, CEP: 14049-900, Brazil
| | - Valeria Valim
- Serviço de Reumatologia, Hospital Universitário Cassiano Antônio de Moraes, Universidade Federal do Espírito Santo, Av. Marechal Campos, 1468, Maruípe, Vitória, ES, CEP: 29075-910, Brazil
| | - Sandra Gofinet Pasoto
- Disciplina de Reumatologia, Laboratório de Autoimunidade (DLC + LIM17), Hospital das Clínicas da Faculdade de Medicina da USP (HCFMUSP), R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo, SP, CEP: 05403-010, Brazil
| | | | - Maria Lucia Lemos Lopes
- Disciplina de Reumatologia Departamento de Clínica Médica, Universidade Federal de Ciências da Saúde de Porto Alegre (UFCSPA),, R. Sarmento Leite, 245 - Centro Histórico de Porto Alegre, Porto Alegre, RS, CEP: 90050-170, Brazil
| | | | - Aysa César Pinheiro
- Disciplina de Reumatologia, Departamento de Clínica Médica, Universidade Federal de Pernambuco, Av. Prof. Moraes Rego, 1235, Cidade Universitária, Recife, PE, CEP: 50670-901, Brazil
| | - Laura Caldas Dos Santos
- Departamento de Oftalmologia, Escola Paulista de Medicina-Universidade Federal de São Paulo (EPM-UNIFESP), Rua Botucatu, 820, Vila Clementino, Sao Paulo, SP, CEP: 04023-062, Brazil
| | - Simone Appenzeller
- Departamento de Ortopedia, Reumatologia e Traumatologia da Faculdade de Ciências Médicas da Universidade Estadual de Campinas (UNICAMP), R. Tessália Vieira de Camargo, 126 - Cidade Universitária, Campinas, SP, CEP: 13083-887, Brazil
| | - Tania Fidelix
- Departamento de Oftalmologia, Escola Paulista de Medicina-Universidade Federal de São Paulo (EPM-UNIFESP), Rua Botucatu, 820, Vila Clementino, Sao Paulo, SP, CEP: 04023-062, Brazil
| | - Sandra Lucia Euzébio Ribeiro
- Disciplina de Reumatologia, Universidade Federal do Amazonas, Rua Afonso Pena, 1053, Manaus, AM, CEP: 69020-160, Brazil
| | - Danielle Christinne Soares Egypto de Brito
- Disciplina de Reumatologia, Departamento de Medicina Interna, Centro de Ciências Médicas, Universidade Federal de Paraíba (UFPB), Campus I - Lot. Cidade Universitária, Paraíba, PB, CEP: 58051-900, Brazil
| | - Tatiana Libório
- Disciplina de Reumatologia, Universidade Federal do Amazonas, Rua Afonso Pena, 1053, Manaus, AM, CEP: 69020-160, Brazil
| | - Maria Carmen Lopes Ferreira Silva Santos
- Departamento de Patologia, Hospital Universitário Cassiano Antônio de Moraes, Universidade Federal do Espírito Santo, Av. Marechal Campos, 1468, Maruípe, Vitória, ES, CEP: 29075-910, Brazil
| | - Leandro Tanure
- Disciplina de Reumatologia, Universidade Federal de Minas Gerais, Av. Pres. Antônio Carlos, 6627, Pampulha, Belo Horizonte, MG, CEP: 31270-901, Brazil
| | - Juliana DAgostino Gennari
- Serviço de Reumatologia da Santa Casa de São Paulo, R. Dr. Cesário Mota Júnior, 112, Vila Buarque, São Paulo, SP, CEP: 01221-020, Brazil
| | - Vinicius Tassoni Civile
- Disciplina de Medicina de Urgência e Medicina Baseada em Evidências, Escola Paulista de Medicina-Universidade Federal de São Paulo (EPM-UNIFESP), Rua Botucatu 740 Vila Clementino, São Paulo, SP, CEP: 04023-062, Brazil
| | - Ana Carolina Pereira Nunes Pinto
- Disciplina de Medicina de Urgência e Medicina Baseada em Evidências, Escola Paulista de Medicina-Universidade Federal de São Paulo (EPM-UNIFESP), Rua Botucatu 740 Vila Clementino, São Paulo, SP, CEP: 04023-062, Brazil
| | - César Ramos Rocha-Filho
- Disciplina de Medicina de Urgência e Medicina Baseada em Evidências, Escola Paulista de Medicina-Universidade Federal de São Paulo (EPM-UNIFESP), Rua Botucatu 740 Vila Clementino, São Paulo, SP, CEP: 04023-062, Brazil
| | - Samira Tatiyama Miyamoto
- Departamento de Educação Integrada em Saúde, Universidade Federal do Espírito Santo (UFES), Av. Marechal Campos, 1468, Maruípe, Vitória, ES, CEP: 29040-090, Brazil
| | - Lissiane Karine Noronha Guedes
- Disciplina de Reumatologia, Laboratório de Autoimunidade (DLC + LIM17), Hospital das Clínicas da Faculdade de Medicina da USP (HCFMUSP), R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo, SP, CEP: 05403-010, Brazil
| | - Alisson Pugliesi
- Departamento de Ortopedia, Reumatologia e Traumatologia da Faculdade de Ciências Médicas da Universidade Estadual de Campinas (UNICAMP), R. Tessália Vieira de Camargo, 126 - Cidade Universitária, Campinas, SP, CEP: 13083-887, Brazil.
| | - Virginia Fernandes Moça Trevisani
- Disciplina de Medicina de Urgência e Medicina Baseada em Evidências, Escola Paulista de Medicina-Universidade Federal de São Paulo (EPM-UNIFESP), Rua Botucatu 740 Vila Clementino, São Paulo, SP, CEP: 04023-062, Brazil.,Disciplina de Reumatologia, Universidade de Santo Amaro, Rua Enéas Siqueira Neto, Jardim das Imbuias, São Paulo, SP, CEP: 04829-300, Brazil
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21
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Abou Ziki MD, Bhat N, Neogi A, Driscoll TP, Ugwu N, Liu Y, Smith E, Abboud JM, Chouairi S, Schwartz MA, Akar JG, Mani A. Epistatic interaction of PDE4DIP and DES mutations in familial atrial fibrillation with slow conduction. Hum Mutat 2021; 42:1279-1293. [PMID: 34289528 DOI: 10.1002/humu.24265] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/13/2021] [Revised: 06/29/2021] [Accepted: 07/06/2021] [Indexed: 11/09/2022]
Abstract
The genetic causes of atrial fibrillation (AF) with slow conduction are unknown. Eight kindreds with familial AF and slow conduction, including a family affected by early-onset AF, heart block, and incompletely penetrant nonischemic dilated cardiomyopathy (DCM) underwent whole exome sequencing. A known pathogenic mutation in the desmin (DES) gene resulting in p.S13F substitution (NM_001927.3:c.38C>T) at a PKC phosphorylation site was identified in all four members of the kindred with early-onset AF and heart block, while only two developed DCM. Higher penetrance for AF and heart block prompted a genetic screening for DES modifier(s). A deleterious mutation in the phosphodiesterase-4D-interacting-protein (PDE4DIP) gene resulting in p.A123T substitution (NM_001002811:c.367G>A) was identified that segregated with early-onset AF, heart block, and the DES mutation. Three additional novel deleterious PDE4DIP mutations were identified in four other unrelated kindreds. Characterization of PDE4DIPA123T in vitro suggested impaired compartmentalization of PKA and PDE4D characterized by reduced colocalization with PDE4D, increased cAMP activation leading to higher PKA phosphorylation of the β2-adrenergic-receptor, and decreased PKA phosphorylation of desmin after isoproterenol stimulation. Our findings identify PDE4DIP as a novel gene for slow AF and unravel its epistatic interaction with DES mutations in development of conduction disease and arrhythmia.
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Affiliation(s)
- Maen D Abou Ziki
- Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA
| | - Neha Bhat
- Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA
| | - Arpita Neogi
- Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA
| | - Tristan P Driscoll
- Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA.,Chemical and Biomedical Engineering, Florida A&M University-Florida State University College of Engineering, Tallahassee, Florida, USA
| | - Nelson Ugwu
- Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA
| | - Ya Liu
- Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA
| | - Emily Smith
- Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA
| | - Johny M Abboud
- Saint George Hospital University Medical Center, Beirut, Lebanon
| | - Salah Chouairi
- Saint George Hospital University Medical Center, Beirut, Lebanon
| | - Martin A Schwartz
- Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA
| | - Joseph G Akar
- Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA
| | - Arya Mani
- Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA.,Department of Genetics, Yale University School of Medicine, New Haven, Connecticut, USA
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22
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His Bundle Pacing in Congenital Complete Heart Block: Making HIStory? JACC Clin Electrophysiol 2021; 7:530-532. [PMID: 33888270 DOI: 10.1016/j.jacep.2020.12.008] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/10/2020] [Accepted: 12/11/2020] [Indexed: 11/21/2022]
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23
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Butt S, Kiran S, Qadir N, Menghani D, Tanzeem H. Cardiac Conduction Defects in Systemic Lupus Erythematosus. Cureus 2020; 12:e10882. [PMID: 33178534 PMCID: PMC7652344 DOI: 10.7759/cureus.10882] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022] Open
Abstract
Systemic autoimmune conditions may cause morbidity and mortality. Systemic lupus erythematosus (SLE) is a prominent example of such diseases. It can result in conduction abnormalities due to accelerated atherosclerosis, vasculitis, or autoantibodies-induced myocarditis. Cardiac conduction abnormalities may produce sinus tachycardia, sinus bradycardia, prolonged QT intervals, atrial fibrillation, or atrioventricular (AV) nodal blocks. Neonatal lupus is sometimes associated with anti-Ro/SSA and anti-La/SSB antibodies, but their role remains a matter of controversy in adults.
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Affiliation(s)
- Shayan Butt
- Internal Medicine, Baptist Memorial Hospital, Oxford, USA
| | - Simra Kiran
- Pulmonology and Critical Care, University of Cincinnati Medical Center, Cincinnati, USA
| | - Nida Qadir
- Infectious Diseases, University of Louisville, Louisville, USA
| | - Divya Menghani
- Infectious Diseases, University of Louisville, Louisville, USA
| | - Hammad Tanzeem
- Medicine, Dow Medical College/Civil Hospital, Karachi, PAK
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Neonatal lupus with left bundle branch block and cardiomyopathy: a case report. BMC Cardiovasc Disord 2020; 20:352. [PMID: 32727396 PMCID: PMC7391615 DOI: 10.1186/s12872-020-01637-4] [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: 05/12/2020] [Accepted: 07/20/2020] [Indexed: 11/18/2022] Open
Abstract
Background Cardiac manifestations of neonatal lupus include an array of structural and conduction abnormalities due to placental transference of maternal anti-SSA/Ro and anti-SSB/La autoantibodies. Late-onset neonatal lupus cardiomyopathies, occurring outside the neonatal period, is an infrequently reported manifestation with unknown pathophysiology and poorly defined treatment regimens. Due to the rarity of this condition, additional studies and case reports are required to better understand and manage late-onset neonatal lupus cardiomyopathies. Case presentation A 4-week-old female, born to a mother with known anti-SSA/Ro and anti-SSB/La autoantibodies, presents with classic cutaneous manifestations for neonatal lupus and is found to have left bundle branch block, severely dilated cardiomyopathy with an ejection fraction of 25%, and a thin echogenic dyskinetic ventricular septum. Weekly second trimester and 30-week fetal echocardiograms showed no signs of structural or conduction abnormalities. There were no histologic signs of inflammation on cardiac tissue biopsy. After a complicated hospital course, she was successfully treated with biventricular pacemaker, intravenous immunoglobulin, and plasmapheresis. Conclusions We present a case of late-onset neonatal lupus with severe dilated cardiomyopathy, a dyskinetic ventricular septum, and left bundle branch block. To our knowledge, the dyskinetic ventricular septum has never been reported and left bundle branch block is rarely reported in NL. This case further validates the need for long term cardiac follow up for patients born with NL, even if lacking cardiac manifestations in the peripartum period. We characterize a unique presentation of a rare clinical entity, highlighting the diagnostic challenges, and describe a successful treatment course.
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Popescu MR, Dudu A, Jurcut C, Ciobanu AM, Zagrean AM, Panaitescu AM. A Broader Perspective on Anti-Ro Antibodies and Their Fetal Consequences-A Case Report and Literature Review. Diagnostics (Basel) 2020; 10:E478. [PMID: 32674462 PMCID: PMC7399931 DOI: 10.3390/diagnostics10070478] [Citation(s) in RCA: 16] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/31/2020] [Revised: 07/11/2020] [Accepted: 07/12/2020] [Indexed: 12/20/2022] Open
Abstract
The presence of maternal Anti-Ro/Anti-La antibodies causes a passively acquired autoimmunity that may be associated with serious fetal complications. The classic example is the autoimmune-mediated congenital heart block (CHB) which is due in most cases to the transplacental passage of Anti-Ro/Anti-La antibodies. The exact mechanisms through which these pathologic events arise are linked to disturbances in calcium channels function, impairment of calcium homeostasis and ultimately apoptosis, inflammation and fibrosis. CHB still represents a challenging diagnosis and a source of debate regarding the best management. As the third-degree block is usually irreversible, the best strategy is risk awareness and prevention. Although CHB is a rare occurrence, it affects one in 20,000 live births, with a high overall mortality rate (up to 20%, with 70% of in utero deaths). There is also concern over the lifelong consequences, as most babies need a pacemaker. This review aims to offer, apart from the data needed for a better understanding of the issue at hand, a broader perspective of the specialists directly involved in managing this pathology: the rheumatologist, the maternal-fetal specialist and the cardiologist. To better illustrate the theoretical facts presented, we also include a representative clinical case.
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Affiliation(s)
- Mihaela Roxana Popescu
- Cardiology Department, Elias University Hospital, “Carol Davila” University of Medicine and Pharmacy, 011461 Bucharest, Romania
| | - Andreea Dudu
- Internal Medicine Department, “Dr Carol Davila” Central Emergency University Military Hospital, 010825 Bucharest, Romania; (A.D.); (C.J.)
| | - Ciprian Jurcut
- Internal Medicine Department, “Dr Carol Davila” Central Emergency University Military Hospital, 010825 Bucharest, Romania; (A.D.); (C.J.)
| | - Anca Marina Ciobanu
- Department of Obstetrics and Gynecology, Filantropia Clinical Hospital, “Carol Davila” University of Medicine and Pharmacy, 011171 Bucharest, Romania; (A.M.C.); (A.M.P.)
| | - Ana-Maria Zagrean
- Division of Physiology and Neuroscience, Department of Functional Sciences, “Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, Romania;
| | - Anca Maria Panaitescu
- Department of Obstetrics and Gynecology, Filantropia Clinical Hospital, “Carol Davila” University of Medicine and Pharmacy, 011171 Bucharest, Romania; (A.M.C.); (A.M.P.)
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26
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Song MK, Kim NY, Bae EJ, Kim GB, Kwak JG, Kim WH, Lee JR. Long-term Follow-up of Epicardial Pacing and Left Ventricular Dysfunction in Children With Congenital Heart Block. Ann Thorac Surg 2020; 109:1913-1920. [DOI: 10.1016/j.athoracsur.2019.09.063] [Citation(s) in RCA: 16] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/10/2019] [Revised: 09/03/2019] [Accepted: 09/18/2019] [Indexed: 01/21/2023]
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Silvetti MS, Muzi G, Unolt M, D'Anna C, Saputo FA, Di Mambro C, Albanese S, Ammirati A, Ravà L, Drago F. Left ventricular (LV) pacing in newborns and infants: Echo assessment of LV systolic function and synchrony at 5‐year follow‐up. PACING AND CLINICAL ELECTROPHYSIOLOGY: PACE 2020; 43:535-541. [DOI: 10.1111/pace.13908] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/07/2019] [Revised: 02/10/2020] [Accepted: 03/15/2020] [Indexed: 11/29/2022]
Affiliation(s)
- Massimo Stefano Silvetti
- Pediatric Cardiology and Cardiac Arrhythmias UnitDepartment of Pediatric Cardiology and Cardiac SurgeryBambino Gesù Children's Hospital and Research Institute Rome Italy
| | - Giulia Muzi
- Pediatric Cardiology and Cardiac Arrhythmias UnitDepartment of Pediatric Cardiology and Cardiac SurgeryBambino Gesù Children's Hospital and Research Institute Rome Italy
| | - Marta Unolt
- Pediatric Cardiology and Cardiac Arrhythmias UnitDepartment of Pediatric Cardiology and Cardiac SurgeryBambino Gesù Children's Hospital and Research Institute Rome Italy
| | - Carolina D'Anna
- Pediatric Cardiology and Cardiac Arrhythmias UnitDepartment of Pediatric Cardiology and Cardiac SurgeryBambino Gesù Children's Hospital and Research Institute Rome Italy
| | - Fabio Anselmo Saputo
- Pediatric Cardiology and Cardiac Arrhythmias UnitDepartment of Pediatric Cardiology and Cardiac SurgeryBambino Gesù Children's Hospital and Research Institute Rome Italy
| | - Corrado Di Mambro
- Pediatric Cardiology and Cardiac Arrhythmias UnitDepartment of Pediatric Cardiology and Cardiac SurgeryBambino Gesù Children's Hospital and Research Institute Rome Italy
| | - Sonia Albanese
- Cardiac Surgery UnitBambino Gesù Children's Hospital and Research Institute Rome Italy
| | - Antonio Ammirati
- Pediatric Cardiology and Cardiac Arrhythmias UnitDepartment of Pediatric Cardiology and Cardiac SurgeryBambino Gesù Children's Hospital and Research Institute Rome Italy
| | - Lucilla Ravà
- Epidemiology UnitBambino Gesù Children's Hospital and Research Institute Rome Italy
| | - Fabrizio Drago
- Pediatric Cardiology and Cardiac Arrhythmias UnitDepartment of Pediatric Cardiology and Cardiac SurgeryBambino Gesù Children's Hospital and Research Institute Rome Italy
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Saxena A, Izmirly PM, Bomar RP, Golpanian RS, Friedman DM, Eisenberg R, Kim MY, Buyon JP. Factors associated with long-term cardiac dysfunction in neonatal lupus. Ann Rheum Dis 2020; 79:217-224. [PMID: 31672776 PMCID: PMC11545888 DOI: 10.1136/annrheumdis-2019-215900] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2019] [Revised: 10/13/2019] [Accepted: 10/14/2019] [Indexed: 11/04/2022]
Abstract
OBJECTIVES Cardiac manifestations of neonatal lupus (NL) have been associated with significant morbidity and mortality; however, there is minimal information on long-term outcomes of affected individuals. This study was initiated to evaluate the presence of and the risk factors associated with cardiac dysfunction in NL after birth in multiple age groups to improve counselling, to further understand pathogenesis and to provide potential preventative strategies. METHODS Echocardiogram reports were evaluated in 239 individuals with cardiac NL: 143 from age 0-1 year, 176 from age >1-17 years and 64 from age >17 years. Logistic regression analyses evaluated associations of cardiac dysfunction at each age group with demographic, fetal and postnatal factors, using imputation to address missing data. RESULTS Cardiac dysfunction was identified in 22.4% at age 0-1 year, 14.8% at age >1-17 years and 28.1% at age >17 years. Dysfunction in various age groups was significantly associated with male sex, black race, lower fetal heart rates, fetal extranodal cardiac disease and length of time paced. In 106 children with echocardiograms at ages 0-1 year and >1-17 years, 43.8% with dysfunction at age 0-1 year were also affected at age >1-17 years, while the others reverted to normal. Of children without dysfunction at age 0-1 year, 8.9% developed new dysfunction between ages >1 and 17 years. Among 34 with echocardiograms at ages >1-17 years and >17 years, 6.5% with normal function at age >1-17 years developed dysfunction in adulthood. CONCLUSIONS Risk factors in fetal life can influence cardiac morbidity into adulthood.Although limited by a small number of cases, cardiac dysfunction in the first year often normalises by later childhood. New-onset dysfunction, although rare, can occur de novo after the first year.
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Affiliation(s)
- Amit Saxena
- Medicine, NYU School of Medicine, New York, New York, United States
| | - Peter M Izmirly
- Medicine, NYU School of Medicine, New York, New York, United States
| | - Rebecca P Bomar
- Medicine, NYU School of Medicine, New York, New York, United States
| | | | | | - Ruth Eisenberg
- Epidemiology and Population Health, Albert Einstein College of Medicine, Bronx, New York, USA
| | - Mimi Y Kim
- Epidemiology and Population Health, Albert Einstein College of Medicine, Bronx, New York, USA
| | - Jill P Buyon
- Medicine, NYU School of Medicine, New York, New York, United States
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29
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Nalli C, Galli J, Lazzaroni MG, Andreoli L, Fazzi E, Tincani A. Long-term outcome of children born from mothers with autoimmune diseases. Best Pract Res Clin Obstet Gynaecol 2019; 64:107-116. [PMID: 31787531 DOI: 10.1016/j.bpobgyn.2019.11.003] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/14/2019] [Revised: 10/28/2019] [Accepted: 11/04/2019] [Indexed: 12/13/2022]
Abstract
Autoimmune diseases often affect young women and this may represent a problem in family planning. Pregnancies in these patients may carry several complications but nowadays the continued amelioration in treatment and management has greatly improved the pregnancy outcome. The main concern of these women obviously is the short- and long-term outcome of their children. A child born from a woman with autoimmune disease is potentially exposed in utero to maternal autoantibodies, cytokines, and drugs, and each item could impair his or her development. In addition, the maternal genetic heritage can favor autoimmunity. All these items could have a role, for example, in the development of autoimmune diseases (the same as the mother or different ones) or neurological disorders. Data in literature are controversial. This review will gather the available data possibly providing a useful tool for counseling future mothers.
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Affiliation(s)
- Cecilia Nalli
- Rheumatology and Immunology Unit, Department of Clinical and Experimental Sciences, University of Brescia and ASST Spedali Civili of Brescia, Piazzale Spedali Civili 1, 25123, Brescia, Italy.
| | - Jessica Galli
- Child Neurology and Psychiatry Unit, Clinical and Experimental Sciences Department, University of Brescia and ASST Spedali Civili Brescia, Piazzale Spedali Civili 1, 25123, Brescia, Italy
| | - Maria Grazia Lazzaroni
- Rheumatology and Immunology Unit, Department of Clinical and Experimental Sciences, University of Brescia and ASST Spedali Civili of Brescia, Piazzale Spedali Civili 1, 25123, Brescia, Italy
| | - Laura Andreoli
- Rheumatology and Immunology Unit, Department of Clinical and Experimental Sciences, University of Brescia and ASST Spedali Civili of Brescia, Piazzale Spedali Civili 1, 25123, Brescia, Italy
| | - Elisa Fazzi
- Child Neurology and Psychiatry Unit, Clinical and Experimental Sciences Department, University of Brescia and ASST Spedali Civili Brescia, Piazzale Spedali Civili 1, 25123, Brescia, Italy
| | - Angela Tincani
- Rheumatology and Immunology Unit, Department of Clinical and Experimental Sciences, University of Brescia and ASST Spedali Civili of Brescia, Piazzale Spedali Civili 1, 25123, Brescia, Italy
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30
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Hamilton RM. Editorial commentary: Live better electrically? Optimizing the timing and application of pacing in congenital heart block. Trends Cardiovasc Med 2019; 30:287-288. [PMID: 31395307 DOI: 10.1016/j.tcm.2019.07.009] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/23/2019] [Accepted: 07/25/2019] [Indexed: 01/13/2023]
Affiliation(s)
- Robert M Hamilton
- The Hospital for Sick Children, Pediatrics (Cardiology), 555 University Ave Rm 1725D, Toronto, Ontario M5G1X8, Canada.
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31
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Congenital heart block: Pace earlier (Childhood) than later (Adulthood). Trends Cardiovasc Med 2019; 30:275-286. [PMID: 31262557 DOI: 10.1016/j.tcm.2019.06.006] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/07/2019] [Revised: 06/16/2019] [Accepted: 06/17/2019] [Indexed: 12/22/2022]
Abstract
Congenital complete heart block (CCHB) occurs in 2-5% of pregnancies with positive anti-Ro/SSA and/or anti-La/SSB antibodies, and has a recurrence rate of 12-25% in a subsequent pregnancy. After trans-placental passage, these autoantibodies attack and destroy the atrioventricular (AV) node in susceptible fetuses with the highest-risk period observed between 16 and 28 weeks' gestational age. Many mothers are asymptomatic carriers, while <1/3 have a preexisting diagnosis of a rheumatic disease. The mortality of CCHB is predominant in utero and in the first months of life, reaching 15-30%. The diagnosis of CCHB can be confirmed by fetal echocardiography before birth and by electrocardiography after birth. Whether early in-utero detection and treatment might prevent or reverse this condition remains controversial. In addition to autoantibody-associated CCHB, there is also an isolated (absent structural heart disease) nonimmune early- or late-onset heart block detected later in childhood that may be associated with specific genetic markers or other pathogenic mechanisms. In isolated immune or non-immune CCHB, cardiac pacemakers are implanted in symptomatic patients, however, data on the natural history of CCHB in the adult life indicate that all patients, even if asymptomatic, should receive a pacemaker when first diagnosed. However, important issues have emerged in these patients wherein life-long conventional right ventricular apical pacing may produce left ventricular dysfunction (pacing-induced cardiomyopathy) necessitating a priori alternate site pacing or subsequent upgrading to biventricular pacing. All these issues are herein reviewed and two algorithms are proposed for diagnosis and management of CCHB in the fetus and in the older individual.
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Lipshultz SE, Law YM, Asante-Korang A, Austin ED, Dipchand AI, Everitt MD, Hsu DT, Lin KY, Price JF, Wilkinson JD, Colan SD. Cardiomyopathy in Children: Classification and Diagnosis: A Scientific Statement From the American Heart Association. Circulation 2019; 140:e9-e68. [PMID: 31132865 DOI: 10.1161/cir.0000000000000682] [Citation(s) in RCA: 203] [Impact Index Per Article: 33.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
In this scientific statement from the American Heart Association, experts in the field of cardiomyopathy (heart muscle disease) in children address 2 issues: the most current understanding of the causes of cardiomyopathy in children and the optimal approaches to diagnosis cardiomyopathy in children. Cardiomyopathies result in some of the worst pediatric cardiology outcomes; nearly 40% of children who present with symptomatic cardiomyopathy undergo a heart transplantation or die within the first 2 years after diagnosis. The percentage of children with cardiomyopathy who underwent a heart transplantation has not declined over the past 10 years, and cardiomyopathy remains the leading cause of transplantation for children >1 year of age. Studies from the National Heart, Lung, and Blood Institute-funded Pediatric Cardiomyopathy Registry have shown that causes are established in very few children with cardiomyopathy, yet genetic causes are likely to be present in most. The incidence of pediatric cardiomyopathy is ≈1 per 100 000 children. This is comparable to the incidence of such childhood cancers as lymphoma, Wilms tumor, and neuroblastoma. However, the published research and scientific conferences focused on pediatric cardiomyopathy are sparcer than for those cancers. The aim of the statement is to focus on the diagnosis and classification of cardiomyopathy. We anticipate that this report will help shape the future research priorities in this set of diseases to achieve earlier diagnosis, improved clinical outcomes, and better quality of life for these children and their families.
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Nakashima A, Miyoshi T, Aoki-Kamiya C, Nishio M, Horiuchi C, Tsuritani M, Iwanaga N, Katsuragi S, Neki R, Ikeda T, Yoshimatsu J. Predicting postpartum cardiac events in pregnant women with complete atrioventricular block. J Cardiol 2019; 74:347-352. [PMID: 31060956 DOI: 10.1016/j.jjcc.2019.04.002] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/26/2018] [Revised: 03/12/2019] [Accepted: 04/01/2019] [Indexed: 11/26/2022]
Abstract
BACKGROUND Women with complete atrioventricular block (CAVB) can tolerate hemodynamic changes during pregnancy; however, the incidence of cardiac events in women with CAVB may increase after delivery. The aim of this study was to investigate predictive factors for postpartum cardiac events in pregnant women with CAVB. METHODS AND RESULTS Pregnant women with CAVB who received perinatal management at a tertiary cardiac center from 1981 to 2015 were retrospectively reviewed. Univariate and multivariate logistic analyses of postpartum cardiac events were performed. Postpartum cardiac event was defined as cardiopulmonary arrest, cardiac failure, or the need for permanent pacemaker implantation (p-PMI) within 3 months after delivery. A total of 63 pregnancies in 36 women with CAVB were included in this study; 25 had undergone p-PMI before pregnancy. Regardless of p-PMI status, women with CAVB had no further increases in heart rate during the second and third trimesters. No heart failure was found during pregnancy and delivery. Postpartum cardiac events occurred in 9 pregnancies (14.3%) in 8 women with CAVB; 3 had cardiac failure and p-PMI, 3 had cardiac failure, 2 required p-PMI, and 1 had cardiopulmonary arrest. Multivariate analysis showed that perinatal ventricular pause (odds ratio 11.60, 95% confidence interval 1.90-82.18, p<0.01) and family history of CAVB (odds ratio 10.59, 95% confidence interval 1.36-90.56, p=0.03) were associated with postpartum cardiac events. CONCLUSIONS All cardiac events occurred during the postpartum period among women with CAVB, and ventricular pause during the perinatal period and a family history of CAVB were predictors of postpartum cardiac events. Close follow-up should be considered during the postpartum period for women with high-risk CAVB.
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Affiliation(s)
- Ayaka Nakashima
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, Suita, Japan
| | - Takekazu Miyoshi
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, Suita, Japan; Department of Obstetrics and Gynecology, Mie University, Tsu, Japan.
| | - Chizuko Aoki-Kamiya
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, Suita, Japan
| | - Miho Nishio
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, Suita, Japan
| | - Chinami Horiuchi
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, Suita, Japan; Department of Obstetrics and Gynecology, Mie University, Tsu, Japan
| | - Mitsuhiro Tsuritani
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, Suita, Japan
| | - Naoko Iwanaga
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, Suita, Japan
| | - Shinji Katsuragi
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, Suita, Japan
| | - Reiko Neki
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, Suita, Japan
| | - Tomoaki Ikeda
- Department of Obstetrics and Gynecology, Mie University, Tsu, Japan
| | - Jun Yoshimatsu
- Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, Suita, Japan
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Jan SL, Lin MC, Chan SC. Spontaneous Return of Sinus Rhythm in a Child Born With Complete Heart Block. J Emerg Med 2019; 56:e81-e82. [PMID: 30922708 DOI: 10.1016/j.jemermed.2019.01.032] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/04/2018] [Revised: 01/04/2019] [Accepted: 07/25/2019] [Indexed: 06/09/2023]
Affiliation(s)
- Sheng-Ling Jan
- Department of Pediatrics, Children's Medical Center, Taichung Veterans General Hospital, Taichung, Taiwan; Department of Pediatrics, School of Medicine, National Yang-Ming University, Taipei, Taiwan; Department of Pediatrics, School of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Ming-Chih Lin
- Department of Pediatrics, Children's Medical Center, Taichung Veterans General Hospital, Taichung, Taiwan; Department of Pediatrics, School of Medicine, National Yang-Ming University, Taipei, Taiwan
| | - Sheng-Ching Chan
- Department of Nursing, Ta-Jen University, Pingtung County, Taiwan
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Mofors J, Eliasson H, Ambrosi A, Salomonsson S, Skog A, Fored M, Ekbom A, Bergman G, Sonesson SE, Wahren-Herlenius M. Comorbidity and long-term outcome in patients with congenital heart block and their siblings exposed to Ro/SSA autoantibodies in utero. Ann Rheum Dis 2019; 78:696-703. [PMID: 30808622 DOI: 10.1136/annrheumdis-2018-214406] [Citation(s) in RCA: 19] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/06/2018] [Revised: 01/26/2019] [Accepted: 02/01/2019] [Indexed: 11/04/2022]
Abstract
OBJECTIVE Congenital heart block (CHB) may develop in fetuses of Ro/SSA autoantibody-positive women. Given the rarity of CHB, information on comorbidity and complications later in life is difficult to systematically collect for large groups of patients. We therefore used nation-wide healthcare registers to investigate comorbidity and outcomes in patients with CHB and their siblings. METHODS Data from patients with CHB (n= 119) and their siblings (n= 128), all born to anti-Ro/SSA-positive mothers, and from matched healthy controls (n= 1,190) and their siblings (n= 1,071), were retrieved from the Swedish National Patient Register. Analyses were performed by Cox proportional hazard modelling. RESULTS Individuals with CHB had a significantly increased risk of cardiovascular comorbidity, with cardiomyopathy and/or heart failure observed in 20 (16.8%) patients versus 3 (0.3%) controls, yielding a HR of 70.0 (95% CI 20.8 to 235.4), and with a HR for cerebral infarction of 39.9 (95% CI 4.5 to 357.3). Patients with CHB also had a higher risk of infections. Pacemaker treatment was associated with a decreased risk of cerebral infarction but increased risks of cardiomyopathy/heart failure and infection. The risk of systemic connective tissue disorder was also increased in patients with CHB (HR 11.8, 95% CI 4.0 to 11.8), and both patients with CHB and their siblings had an increased risk to develop any of 15 common autoimmune conditions (HR 5.7, 95% CI 2.83 to 11.69 and 3.6, 95% CI 1.7 to 8.0, respectively). CONCLUSIONS The data indicate an increased risk of several cardiovascular, infectious and autoimmune diseases in patients with CHB, with the latter risk shared by their siblings.
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Affiliation(s)
- Johannes Mofors
- Department of Medicine, Karolinska Institutet, Stockholm, Sweden
| | - Håkan Eliasson
- Department of Women's and Children's Health, Karolinska Universitetssjukhuset, Stockholm, Sweden
| | - Aurelie Ambrosi
- Department of Medicine, Karolinska Institutet, Stockholm, Sweden
| | | | - Amanda Skog
- Department of Medicine, Karolinska Institutet, Stockholm, Sweden
| | - Michael Fored
- Department of Medicine, Karolinska Institutet, Stockholm, Sweden
| | - Anders Ekbom
- Department of Medicine, Karolinska Institutet, Stockholm, Sweden
| | - Gunnar Bergman
- Department of Women's and Children's Health, Karolinska Universitetssjukhuset, Stockholm, Sweden
| | - Sven-Erik Sonesson
- Department of Women's and Children's Health, Karolinska Universitetssjukhuset, Stockholm, Sweden
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Yu S, Wu Q, Chen BL, An YP, Bu J, Zhou S, Wang YM. Biventricular pacing for treating heart failure in children: A case report and review of the literature. World J Clin Cases 2019; 7:396-404. [PMID: 30746382 PMCID: PMC6369388 DOI: 10.12998/wjcc.v7.i3.396] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/21/2018] [Revised: 12/27/2018] [Accepted: 01/08/2019] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Cardiac resynchronization therapy (CRT) can be used as an escalated therapy to improve heart function in patients with cardiac dysfunction due to long-term right ventricular pacing. However, guidelines are only targeted at adults. CRT is rarely used in children.
CASE SUMMARY This case aimed to implement biventricular pacing in one child with heart failure who had a left ventricular ejection fraction < 35% at 4 years after implantation of an atrioventricular sequential pacemaker due to atrioventricular block. Postoperatively, echocardiography showed atrial sensing ventricular pacing and QRS wave duration of 120-130 ms, and cardiac function significantly improved after upgrading pacemaker.
CONCLUSION Patients whose cardiac function is deteriorated to a level to upgrade to CRT should be upgraded to reverse myocardial remodeling as soon as possible.
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Affiliation(s)
- Shan Yu
- Department of Cardiology, Guizhou Provincial People’s Hospital, Guiyang 550002, Guizhou Province, China
| | - Qiang Wu
- Department of Cardiology, Guizhou Provincial People’s Hospital, Guiyang 550002, Guizhou Province, China
| | - Bao-Lin Chen
- Department of Cardiology, Guizhou Provincial People’s Hospital, Guiyang 550002, Guizhou Province, China
| | - Ya-Ping An
- Department of Cardiology, Guizhou Provincial People’s Hospital, Guiyang 550002, Guizhou Province, China
| | - Jie Bu
- Department of Cardiology, Guizhou Provincial People’s Hospital, Guiyang 550002, Guizhou Province, China
| | - Song Zhou
- Department of Radiology, Guizhou Provincial People’s Hospital, Guiyang 550002, Guizhou Province, China
| | - Yong-Mei Wang
- Department of Cardiology, Guizhou Provincial People’s Hospital, Guiyang 550002, Guizhou Province, China
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37
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Wang B, Hu S, Shi D, Bing Z, Li Z. Arrhythmia and/or Cardiomyopathy Related to Maternal Autoantibodies: Descriptive Analysis of a Series of 16 Cases From a Single Center. Front Pediatr 2019; 7:465. [PMID: 31824893 PMCID: PMC6879546 DOI: 10.3389/fped.2019.00465] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/09/2019] [Accepted: 10/24/2019] [Indexed: 11/13/2022] Open
Abstract
Objective: To describe the clinical characteristics of maternal autoantibody-mediated arrhythmia and/or cardiomyopathy, and to explore the therapeutic role of glucocorticoids in these diseases. Methods: This was a retrospective observational study of 2 fetuses and 14 children who presented with autoantibody-mediated arrhythmia and/or cardiomyopathy in our hospital from September 2010 to December 2018. Results: In total, 16 patients were identified, including 2 fetuses, and 14 children. One mother suffered from Sjogren's syndrome, two suffered from systemic lupus erythematosus (SLE), and the remaining 13 were asymptomatic carriers of autoantibodies. Two fetuses were diagnosed with complete congenital heart block (CHB) and had mean heart rates of 45 and 50 bpm. In the 14 surviving children, third-degree CHB was detected in 4 children, second- to third-degree CHB in 4, corrected QT interval (QTc) prolongation in 1, atrioventricular dissociation, and junctional ectopic tachycardia in 1, complete left bundle branch block (CLBBB) with dilated cardiomyopathy (DCM) in 3, and endocardial fibroelastosis (EFE) in 1. All of the 14 surviving babies received intravenous immunoglobulin and glucocorticoids. None of the children received pacemaker implantation. During the follow-up, one 3-month-old girl who had complete CHB, DCM, and Torsades de pointes almost recovered after the administration of prednisone for ~8 years. Three cases with complete CHB had no improvement after 3-5 years of follow-up. One case with EFE and three cases with CLBBB and DCM were in stable condition now. Children with QTc prolongation and junctional ectopic tachycardia returned to a regular rhythm. Conclusions: Autoantibody-mediated arrhythmias and/or cardiomyopathy are severe complications related to maternal autoantibodies, and the administration of steroid may be beneficial in reversing complete CHB.
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Affiliation(s)
- Benzhen Wang
- School of Medicine, Shandong University, Jinan, China.,Heart Center, Qingdao Women and Children's Hospital, Affiliated to Qingdao University, Qingdao, China
| | - Sujuan Hu
- Department of Pediatrics, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Degong Shi
- Department of Pediatrics, The Traditional Chinese Medical Hospital of Huangdao District, Qingdao, China
| | - Zhen Bing
- Heart Center, Qingdao Women and Children's Hospital, Affiliated to Qingdao University, Qingdao, China
| | - Zipu Li
- School of Medicine, Shandong University, Jinan, China.,Heart Center, Qingdao Women and Children's Hospital, Affiliated to Qingdao University, Qingdao, China
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38
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Clowse MEB, Eudy AM, Kiernan E, Williams MR, Bermas B, Chakravarty E, Sammaritano LR, Chambers CD, Buyon J. The prevention, screening and treatment of congenital heart block from neonatal lupus: a survey of provider practices. Rheumatology (Oxford) 2018; 57:v9-v17. [PMID: 30137589 PMCID: PMC6099126 DOI: 10.1093/rheumatology/key141] [Citation(s) in RCA: 40] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2017] [Revised: 04/20/2018] [Indexed: 11/12/2022] Open
Abstract
Objective To survey an international sample of providers to determine their current practices for the prevention, screening, and treatment of congenital heart block (CHB) due to maternal Ro/SSA antibodies. Methods A survey was designed by the organizing committee of the 9th International Conference of Reproduction, Pregnancy and Rheumatic Diseases. It was sent to attendants of the conference and authors of recent publications or abstracts at ACR 2012, 2013 or 2014 on rheumatic diseases and pregnancy. Results In anti-Ro/SSA positive women, 80% of 49 respondents recommended screening by serial fetal echocardiogram (ECHO), with most starting at week 16 (59%) and stopping at week 28 (25%), although the time to stop varied widely. For women without a prior infant with neonatal lupus, respondents recommend every other week (44%) or weekly (28%) fetal ECHOs. For women with a prior infant with neonatal lupus, 80% recommend weekly fetal ECHOs. To prevent CHB, HCQ was recommended by 67% of respondents and most would start pre-pregnancy (62%). Respondents were asked about medications to treat varying degrees of CHB in a 20-week pregnant, anti-Ro and La positive SLE patient. For first degree, respondents recommended starting dexamethasone (53%) or HCQ (43%). For second degree, respondents recommended starting dexamethasone (88%). For third degree, respondents recommended starting dexamethasone (55%) or IVIg (33%), although 27% would not start treatment. Conclusion Despite the absence of official guidelines, many physicians with a focus on pregnancy and rheumatic disease have developed similar patterns in the screening, prevention and treatment of CHB.
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Affiliation(s)
- Megan E B Clowse
- Division of Rheumatology and Immunology, Duke University Medical Center, Durham, NC
| | - Amanda M Eudy
- Division of Rheumatology and Immunology, Duke University Medical Center, Durham, NC
| | - Elizabeth Kiernan
- Department of Pediatrics, Division of Dysmorphology and Teratology, University of California San Diego, San Diego, CA
| | - Matthew R Williams
- Pediatric Cardiology and Cardiovascular Surgery, Rady Children's Hospital, University of California San Diego, San Diego, CA
| | - Bonnie Bermas
- Division of Rheumatic Diseases, UT Southwestern, Dallas, TX
| | - Eliza Chakravarty
- Department of Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, OK
| | - Lisa R Sammaritano
- Division of Rheumatology, Hospital for Special Surgery, Weill Cornell Medicine, New York, NY
| | | | - Jill Buyon
- Division of Rheumatology, New York University School of Medicine, NY, USA
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Baggio JM, Afiune CMC, Afiune JY, Sarabanda AV, Atik FA. Transvenous dual-chamber pacemaker after paediatric heart transplantation using left ventricle pacing through the coronary sinus. ESC Heart Fail 2018; 5:204-207. [PMID: 29356392 PMCID: PMC5793968 DOI: 10.1002/ehf2.12254] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2017] [Revised: 10/28/2017] [Accepted: 11/27/2017] [Indexed: 11/11/2022] Open
Abstract
A 12‐year‐old child with end‐stage heart failure due to restrictive cardiomyopathy was submitted to orthotopic heart transplantation. Primary graft dysfunction required venous arterial extra‐corporeal membrane oxygenation. Heart function normalized, but complete atrioventricular block remained after 3 weeks. A dual‐chamber pacing with transvenous left ventricle pacing through the coronary sinus was performed. At 5‐year follow‐up, the patient is stable with the same pacing system and with preserved ventricular function.
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Affiliation(s)
- José Mario Baggio
- Division of Electrophysiology and Cardiac Device Unit, Instituto de Cardiologia do Distrito Federal, Brasília, Brazil
| | | | - Jorge Y Afiune
- Division of Pediatric Cardiology, Instituto de Cardiologia do Distrito Federal, Brasília, Brazil
| | - Alvaro V Sarabanda
- Division of Electrophysiology and Cardiac Device Unit, Instituto de Cardiologia do Distrito Federal, Brasília, Brazil
| | - Fernando A Atik
- Division of Cardiovascular Surgery and Transplant Unit, Instituto de Cardiologia do Distrito Federal, St Sudoeste Cruzeiro Novo, Brasília, 70658-700, Brazil
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40
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Incidence, risk factors, and mortality of neonatal and late-onset dilated cardiomyopathy associated with cardiac neonatal lupus. Int J Cardiol 2017; 248:263-269. [DOI: 10.1016/j.ijcard.2017.07.100] [Citation(s) in RCA: 28] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/14/2017] [Revised: 07/19/2017] [Accepted: 07/26/2017] [Indexed: 01/12/2023]
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Abstract
INTRODUCTION Congenital complete heart block affects 1/15,000 live-born infants, predominantly due to atrioventricular nodal injury from maternal antibodies of mothers with systemic lupus erythermatosus or Sjogren's syndrome. The majority of these children will need a pacemaker implanted prior to becoming young adults. This article will review the various patient and technical factors that influence the type of pacemaker implanted, and the current literature on optimal pacing practices. Areas covered: A literature search was performed using PubMed, Embase and Web of Science. Data regarding epicardial versus transvenous implants, pacing-induced ventricular dysfunction, alternative pacing strategies (including biventricular pacing, left ventricular pacing, and His bundle pacing), and complications with pacemakers in the pediatric population were reviewed. Expert commentary: There are numerous pacing strategies available to children with congenital complete heart block. The risks and benefits of the initial implant should be weighed against the long-term issues inherent with a life-time of pacing.
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Affiliation(s)
- Stephanie F Chandler
- a Department of Cardiology , Boston Children's Hospital , Boston , MA , USA.,b Department of Pediatrics , Harvard Medical School , Boston , MA , USA
| | - Francis Fynn-Thompson
- c Department of Cardiovascular Surgery , Boston Children's Hospital , Boston , MA , USA.,d Department of Surgery , Harvard Medical School , Boston , MA , USA
| | - Douglas Y Mah
- a Department of Cardiology , Boston Children's Hospital , Boston , MA , USA.,b Department of Pediatrics , Harvard Medical School , Boston , MA , USA
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42
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43
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Nouvelles techniques de stimulation dans le domaine des cardiopathies congénitales. Presse Med 2017; 46:594-605. [DOI: 10.1016/j.lpm.2017.05.015] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/10/2017] [Revised: 04/25/2017] [Accepted: 05/11/2017] [Indexed: 11/30/2022] Open
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44
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Neonatal dilated cardiomyopathy. Rev Port Cardiol 2017; 36:201-214. [DOI: 10.1016/j.repc.2016.10.007] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/19/2016] [Revised: 09/25/2016] [Accepted: 10/06/2016] [Indexed: 01/09/2023] Open
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45
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Neonatal dilated cardiomyopathy. REVISTA PORTUGUESA DE CARDIOLOGIA (ENGLISH EDITION) 2017. [DOI: 10.1016/j.repce.2016.10.017] [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] Open
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46
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Assessment of left ventricular electromechanical activation during right ventricular apical and outflow tract pacing. Egypt Heart J 2016. [DOI: 10.1016/j.ehj.2016.04.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
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Van den Berg N, Slieker M, van Beynum I, Bilardo C, de Bruijn D, Clur S, Cornette J, Frohn-Mulder I, Haak M, van Loo-Maurus K, Manten G, Rackowitz A, Rammeloo L, Reimer A, Rijlaarsdam M, Freund M. Fluorinated steroids do not improve outcome of isolated atrioventricular block. Int J Cardiol 2016; 225:167-171. [DOI: 10.1016/j.ijcard.2016.09.119] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/23/2016] [Revised: 09/25/2016] [Accepted: 09/29/2016] [Indexed: 10/20/2022]
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Late Outcome and Predictors of Adverse Events Related to the Implantation of a Permanent Pacemaker in Patients with Isolated Congenital Atrioventricular Block. Pediatr Cardiol 2016; 37:1319-27. [PMID: 27335082 DOI: 10.1007/s00246-016-1437-0] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/28/2016] [Accepted: 06/17/2016] [Indexed: 10/21/2022]
Abstract
Isolated congenital atrioventricular block (ICAVB) is a rare, and pacemaker implantation is the only effective treatment. We sought to identify the predictive factors of adverse events related to pacemaker implantation in ICAVB. This is a cohort study of patients diagnosed with ICAVB who underwent pacemaker implantation from 1980 to 2014 in a single center. During the studied period, a total of 647 patients underwent implantation of their first permanent cardiac pacemaker before 30 years of age. Of these, only 62 (9.5 %) were diagnosed with ICAVB. This condition was diagnosed in utero in 15 (24.2 %) cases, 5 (8.1 %) in the neonatal period, 32 (51.6 %) during childhood, and 10 (16.1 %) during adolescence and young adulthood. The presence of autoantibodies (anti-Ro/SSA) was observed in 41 % of mothers who underwent serological evaluation. Age at the time of the initial pacemaker implant was 9.8 ± 9 years. During a mean follow-up time of 15 years, 1 (1.7 %) death occurred due to infectious endocarditis. Complications related to pacemaker implant were reported in 24 patients (38.7 %). The number of complications was significantly higher in the group with an epimyocardial implantation site (HR 6; CI 2.45-14.95). Ventricular dysfunction occurred in 6 (11.7 %) patients; however, we were not able to identify any predictors of it. Our results showed a low mortality rate after permanent therapy. However, these patients exhibited high morbidity related to the pacemaker system, and the epimyocardial implant site was an independent predictor of complications. Predictors of left ventricular dysfunction were not found in the present study.
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Baruteau AE, Pass RH, Thambo JB, Behaghel A, Le Pennec S, Perdreau E, Combes N, Liberman L, McLeod CJ. Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management. Eur J Pediatr 2016; 175:1235-1248. [PMID: 27351174 PMCID: PMC5005411 DOI: 10.1007/s00431-016-2748-0] [Citation(s) in RCA: 75] [Impact Index Per Article: 8.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/28/2016] [Revised: 06/13/2016] [Accepted: 06/16/2016] [Indexed: 02/07/2023]
Abstract
UNLABELLED Atrioventricular block is classified as congenital if diagnosed in utero, at birth, or within the first month of life. The pathophysiological process is believed to be due to immune-mediated injury of the conduction system, which occurs as a result of transplacental passage of maternal anti-SSA/Ro-SSB/La antibodies. Childhood atrioventricular block is therefore diagnosed between the first month and the 18th year of life. Genetic variants in multiple genes have been described to date in the pathogenesis of inherited progressive cardiac conduction disorders. Indications and techniques of cardiac pacing have also evolved to allow safe permanent cardiac pacing in almost all patients, including those with structural heart abnormalities. CONCLUSION Early diagnosis and appropriate management are critical in many cases in order to prevent sudden death, and this review critically assesses our current understanding of the pathogenetic mechanisms, clinical course, and optimal management of congenital and childhood AV block. WHAT IS KNOWN • Prevalence of congenital heart block of 1 per 15,000 to 20,000 live births. AV block is defined as congenital if diagnosed in utero, at birth, or within the first month of life, whereas childhood AV block is diagnosed between the first month and the 18th year of life. As a result of several different etiologies, congenital and childhood atrioventricular block may occur in an entirely structurally normal heart or in association with concomitant congenital heart disease. Cardiac pacing is indicated in symptomatic patients and has several prophylactic indications in asymptomatic patients to prevent sudden death. • Autoimmune, congenital AV block is associated with a high neonatal mortality rate and development of dilated cardiomyopathy in 5 to 30 % cases. What is New: • Several genes including SCN5A have been implicated in autosomal dominant forms of familial progressive cardiac conduction disorders. • Leadless pacemaker technology and gene therapy for biological pacing are promising research fields. In utero percutaneous pacing appears to be at high risk and needs further development before it can be adopted into routine clinical practice. Cardiac resynchronization therapy is of proven value in case of pacing-induced cardiomyopathy.
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Affiliation(s)
- Alban-Elouen Baruteau
- Cardiovascular and Cell Sciences Research Center, St George’s University of London, London, UK
- LIRYC Institute, CHU Bordeaux, Department of Pediatric Cardiology, Bordeaux-II University, Bordeaux, France
- Service de Cardiologie Pédiatrique, Hôpital du Haut Lévèque, Institut Hospitalo-Universitaire LIRYC (Electrophysiology and Heart Modeling Institute), 5 avenue de Magellan, 33600 Pessac, France
| | - Robert H. Pass
- Division of Pediatric Electrophysiology, Albert Einstein College of Medicine, Montefiore Children’s Hospital, Bronx, NY USA
| | - Jean-Benoit Thambo
- LIRYC Institute, CHU Bordeaux, Department of Pediatric Cardiology, Bordeaux-II University, Bordeaux, France
| | - Albin Behaghel
- CHU Rennes, Department of Cardiology, LTSI, INSERM 1099, Rennes-1 University, Rennes, France
| | - Solène Le Pennec
- CHU Rennes, Department of Cardiology, LTSI, INSERM 1099, Rennes-1 University, Rennes, France
| | - Elodie Perdreau
- LIRYC Institute, CHU Bordeaux, Department of Pediatric Cardiology, Bordeaux-II University, Bordeaux, France
| | - Nicolas Combes
- Department of Cardiology, Clinique Pasteur, Toulouse, France
| | - Leonardo Liberman
- Morgan Stanley Children’s Hospital, Division of Pediatric Cardiology, New York Presbyterian Hospital, Columbia University Medical Center, New York, NY USA
| | - Christopher J. McLeod
- Mayo Clinic, Division of Cardiovascular Diseases, Mayo Clinic College of Medicine, Rochester, MN USA
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Motta M, Tincani A, Lojacono A, Faden D, Gorla R, Airò P, Neri F, Gasparoni A, Ciardelli L, de Silvestri A, Marconi M, Chirico G. Neonatal outcome in patients with rheumatic disease. Lupus 2016; 13:718-23. [PMID: 15485111 DOI: 10.1191/0961203403lu2002oa] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
Rheumatic autoimmune diseases have a higher prevalence in women, particularly during their childbearing age. Due to improved management, an increasing number of patients plan and carry out one or more pregnancies. Therefore, a growing interest is being paid to the possible consequences of maternal disease and associated treatment on the fetus and newborn infant. If maternal disease is characterized by the presence of IgG isotype autoantibodies, these can cross the placenta with possible antibody-mediated damage to the fetus. This is typically the case of the so called neonatal lupus erythematosus (NLE); a similar mechanism has been shown in infants of patients with immune thrombocytopenic purpura (ITP) and, less frequently, in those from mothers with antiphospholipid syndrome (APS). Indeed, this last condition is often responsible for placental, rather than neonatal, pathology. In addition, immunosuppressive and other drugs administered to the mothers during pregnancy and lactation might affect the fetal and neonatal immune system development. Finally, mothers disease and/or treatment could be related to neuropsychological alteration reported in some of their children.
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Affiliation(s)
- M Motta
- Neonatology and Neonatal Intensive Care, Spedali Civili, Brescia, Italy.
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