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1
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Ainab I, Van Ochten N, Suckow E, Pierce K, Arent C, Kay J, Forbes LM, Cornwell WK. Determinants of cardiac output in health and heart failure. Exp Physiol 2025; 110:637-648. [PMID: 40121540 PMCID: PMC12053893 DOI: 10.1113/ep091505] [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: 09/16/2024] [Accepted: 02/20/2025] [Indexed: 03/25/2025]
Abstract
Sustained physical exercise depends on delivery of oxygenated blood to exercising muscle. At least among healthy individuals, bulk transport of blood is tightly matched to metabolic demand, such that cardiac output increases by ∼6 L/min for every 1 L/min increase in oxygen uptake. Multiple factors contribute to the regulation of cardiac output, including central command, the exercise pressor reflex (EPR) and arterial baroreceptors. Pulmonary arterial and left ventricular pressures increase in proportion to the rise in cardiac output and exercise intensity. The right ventricle augments contractility to maintain ventricular-arterial (VA) coupling and lusitropy to facilitate venous return. Among patients with heart failure (HF), however, the ability to deliver blood to exercising muscle is compromised as a result of multiple abnormalities impacting EPR, ventricular contractility, haemodynamics and VA coupling. The purpose of this review is to provide an overview of the factors limiting exercise capacity and cardiac output among patients with HF compared to what is known about normal physiology among healthy individuals.
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Affiliation(s)
- Ibrahim Ainab
- Department of Medicine‐CardiologyUniversity of Colorado Anschutz Medical CampusAuroraColoradoUSA
| | - Natalie Van Ochten
- Department of MedicineUniversity of Colorado Anschutz Medical CampusAuroraColoradoUSA
| | - Emmett Suckow
- Department of Medicine‐CardiologyUniversity of Colorado Anschutz Medical CampusAuroraColoradoUSA
| | - Kathryn Pierce
- Clinical Translational Research CenterUniversity of Colorado Anschutz Medical CampusAuroraColoradoUSA
| | - Chelsea Arent
- Clinical Translational Research CenterUniversity of Colorado Anschutz Medical CampusAuroraColoradoUSA
| | - Joseph Kay
- Department of MedicineUniversity of Colorado Anschutz Medical CampusAuroraColoradoUSA
| | - Lindsey M. Forbes
- Department of Medicine, Division of Pulmonary and Critical Care MedicineUniversity of Colorado Anschutz Medical CampusAuroraColoradoUSA
| | - William K. Cornwell
- Department of MedicineUniversity of Colorado Anschutz Medical CampusAuroraColoradoUSA
- Clinical Translational Research CenterUniversity of Colorado Anschutz Medical CampusAuroraColoradoUSA
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Cifra B, Cordina RL, Gauthier N, Murphy LC, Pham TD, Veldtman GR, Ward K, White DA, Paridon SM, Powell AW. Cardiopulmonary Exercise Test Interpretation Across the Lifespan in Congenital Heart Disease: A Scientific Statement From the American Heart Association. J Am Heart Assoc 2025; 14:e038200. [PMID: 39782908 PMCID: PMC12074744 DOI: 10.1161/jaha.124.038200] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/08/2024] [Accepted: 08/08/2024] [Indexed: 01/12/2025]
Abstract
Survivorship from congenital heart disease has improved rapidly secondary to advances in surgical and medical management. Because these patients are living longer, treatment and disease surveillance targets have shifted toward enhancing quality of life and functional status. Cardiopulmonary exercise testing is a valuable tool for assessing functional capacity, evaluating cardiac and pulmonary pathology, and providing guidance on prognosis and interventional recommendations. Despite the extensive evidence supporting the ability of cardiopulmonary exercise testing to quantitatively evaluate cardiovascular function, there remains confusion on how to properly interpret cardiopulmonary exercise testing in patients with congenital heart disease. The purpose of this statement is to provide a lifespan approach to the interpretation of cardiopulmonary exercise testing in patients with congenital heart disease. This is an updated report of the American Heart Association's previous publications on exercise in children. This evidence-based update on the significance of cardiopulmonary exercise testing findings in pediatric, adolescent, and adult patients with various congenital cardiac pathologies and surgically modified physiology is formatted in a way to guide cardiopulmonary exercise testing interpretation practically for the clinicians and exercise physiologists who care for patients with congenital heart disease. Focus is placed on the indications for exercise testing, expected findings, and how exercise testing should guide the management of patients with various congenital heart disease subtypes. Areas for future intervention that could lead to improved care and outcomes for those with congenital heart disease are noted.
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3
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Carvalho TD, Freitas OGAD, Chalela WA, Hossri CAC, Milani M, Buglia S, Falcão AMGM, Costa RVC, Ritt LEF, Pfeiffer MET, Silva OBE, Imada R, Pena JLB, Avanza Júnior AC, Sellera CAC. Brazilian Guideline for Exercise Testing in Children and Adolescents - 2024. Arq Bras Cardiol 2024; 121:e20240525. [PMID: 39292116 PMCID: PMC11495813 DOI: 10.36660/abc.20240525] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/19/2024] Open
Abstract
CLASSES OF RECOMMENDATION LEVELS OF EVIDENCE
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Affiliation(s)
- Tales de Carvalho
- Clínica de Prevenção e Reabilitação Cardiosport, Florianópolis, SC - Brasil
- Universidade do Estado de Santa Catarina, Florianópolis, SC - Brasil
| | | | - William Azem Chalela
- Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (InCor-HCFMUSP), São Paulo, SP - Brasil
- Sociedade Beneficente de Senhoras do Hospital Sírio-Libanês, São Paulo, SP - Brasil
| | | | - Mauricio Milani
- Universidade de Brasília (UnB), Brasília, DF - Brasil
- Hasselt University, Hasselt - Bélgica
- Jessa Ziekenhuis, Hasselt - Bélgica
| | - Susimeire Buglia
- Instituto Dante Pazzanese de Cardiologia, São Paulo, SP - Brasil
| | - Andréa Maria Gomes Marinho Falcão
- Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (InCor-HCFMUSP), São Paulo, SP - Brasil
| | | | - Luiz Eduardo Fonteles Ritt
- Escola Bahiana de Medicina e Saúde Pública, Salvador, BA - Brasil
- Instituto D'Or de Pesquisa e Ensino, Salvador, BA - Brasil
- Hospital Cárdio Pulmonar, Salvador, BA - Brasil
| | | | | | - Rodrigo Imada
- Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (InCor-HCFMUSP), São Paulo, SP - Brasil
- Hospital Sírio-Libanês, São Paulo, SP - Brasil
| | - José Luiz Barros Pena
- Faculdade Ciências Médicas de Minas Gerais, Belo Horizonte, MG - Brasil
- Hospital Felício Rocho, Belo Horizonte, MG - Brasil
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Bredy C, Werner O, Helena H, Picot MC, Amedro P, Adda J. Cardiac magnetic resonance ventricular parameters correlate with cardiopulmonary fitness in patients with functional single ventricle. THE INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING 2024; 40:1041-1048. [PMID: 38546925 DOI: 10.1007/s10554-024-03072-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 08/25/2023] [Accepted: 02/19/2024] [Indexed: 06/05/2024]
Abstract
Owing to advances in medical and surgical fields, patients with single ventricle (SV) have a greatly improved life expectancy. However, progressive functional deterioration is observed over time, with a decrease in cardiopulmonary fitness. This study aimed to identify, in patients with SV, the association between cardiac magnetic resonance imaging (CMR) parameters and change in cardiopulmonary fitness assessed by cardiopulmonary exercise test (CPET), and if certain thresholds could anticipate a decline in aerobic fitness. Patients with an SV physiology were retrospectively screened from 2011 and 2021 in a single-centre observational study. We evaluated (1) the correlation between baseline CMR and CPET parameters, (2) the association between baseline CMR results and change in peak oxygen uptake (peak VO2), and (3) the cut-off values of end-diastolic and end-systolic volume index in patients with an impaired cardiopulmonary fitness (low peak VO2 and/or high VE/VCO2 slope). 32 patients were included in the study. End-systolic volume index (r = 0.37, p = 0.03), end-diastolic volume index (r = 0.45, p = 0.01), and cardiac index (r = 0.46, p = 0.01) correlated with the VE/VCO2 slope. End-systolic ventricular volume (r = - 0.39, p = 0.01), end-diastolic ventricular volume (r = - 0.38, p = 0.01), and cardiac output (r = - 0.45, p < 0.01) inversely correlated with the peak VO2. In multivariate analysis, the cardiac index obtained from baseline CMR was inversely associated with the change in peak VO2 (p < 0.01). An end-diastolic volume index > 101 ml/m2 and an end-systolic volume index > 47 ml/m2 discriminated patients with impaired cardiopulmonary fitness. CMR parameters correlate with cardiopulmonary fitness in patients with SV and can therefore be useful for follow-up and therapeutic management of these patients.
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Affiliation(s)
- Charlene Bredy
- Paediatric and Congenital Cardiology Department, M3C Regional Reference CHD Centre, Montpellier University Hospital, Montpellier, France
| | - Oscar Werner
- Paediatric and Congenital Cardiology Department, M3C Regional Reference CHD Centre, Montpellier University Hospital, Montpellier, France
- Pediatric Imaging Department, Montpellier University Hospital, Montpellier, France
| | - Huguet Helena
- Epidemiology and Clinical Research Department, University Hospital, Montpellier, France
- Clinical Investigation Centre, INSERM U1411, Montpellier University Hospital, University of Montpellier, Montpellier, France
| | - Marie-Christine Picot
- Epidemiology and Clinical Research Department, University Hospital, Montpellier, France
- Clinical Investigation Centre, INSERM U1411, Montpellier University Hospital, University of Montpellier, Montpellier, France
| | - Pascal Amedro
- Paediatric and Congenital Cardiology Department, M3C National Reference Centre, Bordeaux University Hospital, Bordeaux, France
- IHU Liryc, Electrophysiology and Heart Modelling Institute, Bordeaux University Foundation, Pessac, France
| | - Jerome Adda
- Cardiology Department, Montpellier University Hospital, Montpellier, France.
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Cunha G, Apostolo A, De Martino F, Salvioni E, Matavelli I, Agostoni P. Exercise oscillatory ventilation: the past, present, and future. Eur J Prev Cardiol 2023; 30:ii22-ii27. [PMID: 37819229 DOI: 10.1093/eurjpc/zwad205] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/11/2023] [Revised: 05/22/2023] [Accepted: 06/12/2023] [Indexed: 10/13/2023]
Abstract
Exercise oscillatory ventilation (EOV) is a fascinating event that can be appreciated in the cardiopulmonary exercise test and is characterized by a cyclic fluctuation of minute ventilation, tidal volume, oxygen uptake, carbon dioxide production, and end-tidal pressure for oxygen and carbon dioxide. Its mechanisms stem from a dysregulation of the normal control feedback of ventilation involving one or more of its components, namely, chemoreflex delay, chemoreflex gain, plant delay, and plant gain. In this review, we intend to breakdown therapeutic targets according to pathophysiology and revise the prognostic value of exercise oscillatory ventilation in the setting of heart failure and other diagnoses.
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Affiliation(s)
- Gonçalo Cunha
- Cardiology department, Hospital de Santa Cruz, Centro Hospitalar Lisboa Ocidental, Av. Prof. Dr. Reinaldo dos Santos, 2790-134 Carnaxide, Portugal
| | - Anna Apostolo
- Heart failure department, Centro Cardiologico Monzino, IRCCS, Milan, Italy
| | | | | | - Irene Matavelli
- Heart failure department, Centro Cardiologico Monzino, IRCCS, Milan, Italy
| | - Piergiuseppe Agostoni
- Heart failure department, Centro Cardiologico Monzino, IRCCS, Milan, Italy
- Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
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Goulart CDL, Silva RN, Agostoni P, Franssen FME, Myers J, Arena R, Borghi-Silva A. Exercise oscillatory ventilation in patients with coexisting chronic obstructive pulmonary disease and heart failure: Clinical implications. Respir Med 2023; 217:107332. [PMID: 37356636 DOI: 10.1016/j.rmed.2023.107332] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/23/2023] [Revised: 06/14/2023] [Accepted: 06/15/2023] [Indexed: 06/27/2023]
Abstract
BACKGROUND Exercise oscillatory ventilation (EOV) is considered an important variable for predicting poor prognosis in patients with heart failure (HF) with reduced left ventricular ejection fraction (HFrEF). However, there are no studies evaluating EOV presence in the coexistence chronic obstructive pulmonary disease (COPD) and HFrEF. AIMS I) To compare the clinical characteristics of participants with coexisting HFrEF-COPD with and without EOV during cardiopulmonary exercise testing (CPET); and II) to identify the impact of EOV on mortality during follow-up for 35 months. METHODS 50 stable HFrEF-COPD (EF<50%) participants underwent CPET and were followed for 35 months. The parametric Student's t-test, chi-square tests, linear regression model and Kaplan-Meier analysis were applied. RESULTS We identified 13 (26%) participants with EOV and 37 (74%) without EOV (N-EOV) during exercise. The EOV group had worse cardiac function (LVEF: 30 ± 6% vs. N-EOV 40 ± 9%, p = 0.007), worse pulmonary function (FEV1: 1.04 ± 0.7 L vs. N-EOV 1.88 ± 0.7 L, p = 0.007), a higher mortality rate [7 (54%) vs. N-EOV 8 (27%), p = 0.02], higher minute ventilation/carbon dioxide production (V̇˙E/ V̇˙ CO2) slope (42 ± 7 vs. N-EOV 36 ± 8, p = 0.04), reduced peak ventilation (L/min) (26.2 ± 16.7 vs. N-EOV 40.3 ± 16.4, p = 0.01) and peak oxygen uptake (mlO2 kg-1 min-1) (11.0 ± 4.0 vs. N-EOV 13.5 ± 3.4 ml●kg-1●min-1, p = 0.04) when compared with N-EOV group. We found that EOV group had a higher risk of mortality during follow-up (long-rank p = 0.001) than patients with N-EOV group. CONCLUSION The presence of EOV is associated with greater severity of coexisting HFrEF and COPD and a reduced prognosis. Assessment of EOV in participants with coexisting HFrEF-COPD, as a biomarker for both clinical status and prognosis may therefore be warranted.
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Affiliation(s)
- Cássia da Luz Goulart
- Cardiopulmonary Physiotherapy Laboratory, Physiotherapy Department, Federal University of Sao Carlos, UFSCar, Sao Carlos, SP, Brazil
| | - Rebeca Nunes Silva
- Cardiopulmonary Physiotherapy Laboratory, Physiotherapy Department, Federal University of Sao Carlos, UFSCar, Sao Carlos, SP, Brazil
| | - Piergiuseppe Agostoni
- Centro Cardiologico Monzino, IRCCS, Via Parea 4, Milan, 20138, Italy; Department of Clinical Sciences and Community Health, Cardiovascular Section, University of Milano, Milano, Italy
| | - Frits M E Franssen
- Department of Respiratory Medicine, Maastricht University Medical Center, Maastricht, the Netherlands
| | - Jonathan Myers
- Division of Cardiovascular Medicine, VA Palo Alto Health Care System, USA; Department of Medicine, Stanford University, Stanford, CA, USA
| | - Ross Arena
- Department of Physical Therapy, College of Applied Health Sciences, University of Illinois Chicago, Chicago, IL, USA
| | - Audrey Borghi-Silva
- Cardiopulmonary Physiotherapy Laboratory, Physiotherapy Department, Federal University of Sao Carlos, UFSCar, Sao Carlos, SP, Brazil.
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Mahendran AK, Katz D, Opotowsky AR, Lubert AM. Exercise Pathophysiology and Testing in Individuals With a Fontan Circulation. CJC PEDIATRIC AND CONGENITAL HEART DISEASE 2023; 2:112-123. [PMID: 37969357 PMCID: PMC10642126 DOI: 10.1016/j.cjcpc.2023.01.001] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 11/03/2022] [Accepted: 01/07/2023] [Indexed: 11/17/2023]
Abstract
The Fontan circulation, a surgical palliation for single-ventricle congenital heart disease, profoundly impacts the cardiopulmonary response to exercise. Reliant on passive pulmonary blood flow, the Fontan circulation has limited capacity to augment cardiac output as necessary to supply working muscles during exercise. Cardiopulmonary exercise testing (CPET) objectively assesses cardiorespiratory fitness and provides insight into the etiology of exercise intolerance. Furthermore, CPET variables, such as peak oxygen consumption and submaximal variables, have prognostic value and may be used as meaningful endpoints in research studies. CPET is also useful in clinical research applications to assess the effect of pharmacologic or other interventions. Medical therapies to improve exercise tolerance in individuals with a Fontan circulation, such as pulmonary vasodilators, may modestly improve peak oxygen consumption. Exercise training focused on aerobic fitness and lower extremity strength may have a more consistent and larger impact on these measures of aerobic fitness. CPET is a valuable diagnostic and prognostic tool for those with a Fontan circulation. Newer ancillary assessments, such as noninvasive peripheral venous pressure monitoring and cardiac output measurements, hold promise to provide a more nuanced insight into the underlying pathophysiology.
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Affiliation(s)
- Arjun K. Mahendran
- Department of Pediatrics, Heart Institute, Cincinnati Children’s Hospital, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
| | - David Katz
- Department of Pediatrics, Heart Institute, Cincinnati Children’s Hospital, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
| | - Alexander R. Opotowsky
- Department of Pediatrics, Heart Institute, Cincinnati Children’s Hospital, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
| | - Adam M. Lubert
- Department of Pediatrics, Heart Institute, Cincinnati Children’s Hospital, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
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Wadey CA, Barker AR, Stuart G, Tran DL, Laohachai K, Ayer J, Cordina R, Williams CA. Scaling Peak Oxygen Consumption for Body Size and Composition in People With a Fontan Circulation. J Am Heart Assoc 2022; 11:e026181. [PMID: 36515232 PMCID: PMC9798799 DOI: 10.1161/jaha.122.026181] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
Background Peak oxygen consumption (peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$) is traditionally divided ("ratio-scaled") by body mass (BM) for clinical interpretation. Yet, it is unknown whether ratio-scaling to BM can produce a valid size-independent expression of peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ in people with a Fontan circulation. Furthermore, people with a Fontan circulation have deficits in lean mass, and it is unexplored whether using different measures of body composition may improve scaling validity. The objective was to assess the validity of different scaling denominators (BM, stature, body surface area, fat-free mass, lean mass, and appendicular lean mass using ratio and allometric scaling). Methods and Results Eighty-nine participants (age: 23.3±6.7 years; 53% female) with a Fontan circulation had their cardiorespiratory fitness and body composition measured by cardiopulmonary exercise testing and dual-energy x-ray absorptiometry. Ratio and allometric (log-linear regression) scaling was performed and Pearson correlations assessed scaling validity. Scaling denominators BM (r=-0.25, P=0.02), stature (r=0.46, P<0.001), and body surface area (0.23, P=0.03) were significantly correlated with their respective ratio-scaled expressions of peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$, but fat-free mass, lean mass, or appendicular lean mass were not (r≤0.11; R2=1%). Allometrically expressed peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ resulted in no significant correlation with any scaling denominator (r=≤0.23; R2=≤4%). Conclusions The traditional and accepted method of ratio-scaling to BM is invalid because it fails to create a size-independent expression of peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ in people with a Fontan circulation. However, ratio-scaling to measures of body composition (fat-free mass, lean mass, and appendicular lean mass) and allometric techniques can produce size-independent expressions of peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ in people with a Fontan circulation.
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Affiliation(s)
- Curtis A. Wadey
- Children’s Health & Exercise Research Centre (CHERC)Public Health and Sport Science, Faculty of Health and Life Sciences, University of ExeterExeterUnited Kingdom
| | - Alan R. Barker
- Children’s Health & Exercise Research Centre (CHERC)Public Health and Sport Science, Faculty of Health and Life Sciences, University of ExeterExeterUnited Kingdom
| | - Graham Stuart
- Bristol Congenital Heart Centre, The Bristol Heart Institute, University Hospitals Bristol NHS Foundation TrustBristolUnited Kingdom
| | - Derek L. Tran
- Central Clinical School, The University of SydneyCamperdownNew South Wales,Department of CardiologyRoyal Prince Alfred HospitalCamperdownNew South Wales,Heart Research Institute, Charles Perkins Centre, The University of SydneyCamperdownNew South Wales
| | - Karina Laohachai
- Central Clinical School, The University of SydneyCamperdownNew South Wales,Department of CardiologyRoyal Prince Alfred HospitalCamperdownNew South Wales,Heart Research Institute, Charles Perkins Centre, The University of SydneyCamperdownNew South Wales
| | - Julian Ayer
- Central Clinical School, The University of SydneyCamperdownNew South Wales,Department of CardiologyRoyal Prince Alfred HospitalCamperdownNew South Wales,Heart Research Institute, Charles Perkins Centre, The University of SydneyCamperdownNew South Wales
| | - Rachael Cordina
- Central Clinical School, The University of SydneyCamperdownNew South Wales,Department of CardiologyRoyal Prince Alfred HospitalCamperdownNew South Wales,Heart Research Institute, Charles Perkins Centre, The University of SydneyCamperdownNew South Wales
| | - Craig A. Williams
- Children’s Health & Exercise Research Centre (CHERC)Public Health and Sport Science, Faculty of Health and Life Sciences, University of ExeterExeterUnited Kingdom
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9
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Hager A. Minute ventilation/carbon dioxide production in congenital heart disease. Eur Respir Rev 2021; 30:30/161/200178. [PMID: 34526311 PMCID: PMC9488851 DOI: 10.1183/16000617.0178-2020] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2020] [Accepted: 11/17/2020] [Indexed: 11/24/2022] Open
Abstract
This review summarises various applications of how ventilatory equivalent (ventilatory efficiency or better still ventilatory inefficiency) and the minute ventilation (VʹE)/carbon dioxide production (VʹCO2) slope obtained from cardiopulmonary exercise testing (CPET) can be used in the diagnostic or prognostic workup of patients with congenital heart disease. The field of congenital heart disease comprises not only a very heterogeneous patient group with various heart diseases, but also various conditions in different stages of repair, as well as the different residuals seen in long-term follow-up. As such, various physiologic disarrangements must be considered in the analysis of increased VʹE/VʹCO2 slope from CPET in patients with congenital heart disease. In addition to congestive heart failure (CHF), cyanosis, unilateral pulmonary stenosis and pulmonary hypertension (PH) provide the background for this finding. The predictive value of increased VʹE/VʹCO2 slope on prognosis seems to be more important in conditions where circulatory failure is associated with failure of the systemic ventricle. In cyanotic patients, those with Fontan circulation, or those with substantial mortality from arrhythmia, the impact of VʹE/VʹCO2 on prognosis is not that important. VʹE/VʹCO2 elevation is a common finding in patients with congenital heart disease. It can be used as a sign for right-to-left shunting, unilateral pulmonary stenosis, pulmonary hypertension and circulatory failure. It is predictive for clinical worsening.https://bit.ly/33gj3NQ
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Affiliation(s)
- Alfred Hager
- Dept of Pediatric Congenital Heart Disease and Pediatric Cardiology, Deutsches Herzzentrum München, Technical University of Munich, Munich, Germany
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10
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Wadey CA, Weston ME, Dorobantu DM, Pieles GE, Stuart G, Barker AR, Taylor RS, Williams CA. The role of cardiopulmonary exercise testing in predicting mortality and morbidity in people with congenital heart disease: a systematic review and meta-analysis. Eur J Prev Cardiol 2021; 29:513-533. [PMID: 34405863 DOI: 10.1093/eurjpc/zwab125] [Citation(s) in RCA: 12] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/26/2021] [Revised: 06/10/2021] [Indexed: 12/16/2022]
Abstract
AIMS The role of cardiopulmonary exercise testing (CPET) in predicting major adverse cardiovascular events (MACE) in people with congenital heart disease (ConHD) is unknown. A systematic review with meta-analysis was conducted to report the associations between CPET parameters and MACE in people with ConHD. METHODS AND RESULTS Electronic databases were systematically searched on 30 April 2020 for eligible publications. Two authors independently screened publications for inclusion, extracted study data, and performed risk of bias assessment. Primary meta-analysis pooled univariate hazard ratios across studies. A total of 34 studies (18 335 participants; 26.2 ± 10.1 years; 54% ± 16% male) were pooled into a meta-analysis. More than 20 different CPET prognostic factors were reported across 6 ConHD types. Of the 34 studies included in the meta-analysis, 10 (29%), 23 (68%), and 1 (3%) were judged as a low, medium, and high risk of bias, respectively. Primary univariate meta-analysis showed consistent evidence that improved peak and submaximal CPET measures are associated with a reduce risk of MACE. This association was supported by a secondary meta-analysis of multivariate estimates and individual studies that could not be numerically pooled. CONCLUSION Various maximal and submaximal CPET measures are prognostic of MACE across a variety of ConHD diagnoses. Further well-conducted prospective multicentre cohort studies are needed to confirm these findings.
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Affiliation(s)
- Curtis A Wadey
- Children's Health & Exercise Research Centre (CHERC), College of Life and Environmental Sciences, St. Luke's Campus, University of Exeter, Heavitree Road, Exeter EX1 2LU, UK
| | - Max E Weston
- Children's Health & Exercise Research Centre (CHERC), College of Life and Environmental Sciences, St. Luke's Campus, University of Exeter, Heavitree Road, Exeter EX1 2LU, UK.,School of Human Movement and Nutrition Sciences, Human Movement Studies Building, University of Queensland, QLD 4067, Brisbane, Australia
| | - Dan Mihai Dorobantu
- Children's Health & Exercise Research Centre (CHERC), College of Life and Environmental Sciences, St. Luke's Campus, University of Exeter, Heavitree Road, Exeter EX1 2LU, UK.,School of Population Health Sciences, University of Bristol, BS8 1QU, Bristol, UK
| | - Guido E Pieles
- National Institute for Health Research (NIHR) Cardiovascular Biomedical Research Centre, Bristol Heart Institute, Terrell St, BS2 8ED, Bristol, UK.,Bristol Congenital Heart Centre, The Bristol Heart Institute, University Hospitals Bristol NHS Foundation Trust, Upper Maudlin Street, BS2 8HW, Bristol, UK.,Institute of Sport Exercise and Health (ISEH), University College London, 170 Tottenham Court Rd, W1T 7HA, London, UK
| | - Graham Stuart
- National Institute for Health Research (NIHR) Cardiovascular Biomedical Research Centre, Bristol Heart Institute, Terrell St, BS2 8ED, Bristol, UK.,Bristol Congenital Heart Centre, The Bristol Heart Institute, University Hospitals Bristol NHS Foundation Trust, Upper Maudlin Street, BS2 8HW, Bristol, UK
| | - Alan R Barker
- Children's Health & Exercise Research Centre (CHERC), College of Life and Environmental Sciences, St. Luke's Campus, University of Exeter, Heavitree Road, Exeter EX1 2LU, UK
| | - Rod S Taylor
- MRC/CSO Social and Public Health Sciences Unit & Robertson Centre for Biostatistics, Institute of Health and Well Being, University of Glasgow, 99 Berkeley Street, G3 7HR, Glasgow, UK
| | - Craig A Williams
- Children's Health & Exercise Research Centre (CHERC), College of Life and Environmental Sciences, St. Luke's Campus, University of Exeter, Heavitree Road, Exeter EX1 2LU, UK
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11
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The pulmonary vascular bed in patients with functionally univentricular physiology and a Fontan circulation. Cardiol Young 2021; 31:1241-1250. [PMID: 34378498 DOI: 10.1017/s104795112100192x] [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] [Indexed: 02/03/2023]
Abstract
Fontan palliation represents one of the most remarkable surgical advances in the management of individuals born with functionally univentricular physiology. The operation secures adult survival for all but a few with unfavourable anatomy and/or physiology. Inherent to the physiology is passive transpulmonary blood flow, which produces a vulnerability to adequate filling of the systemic ventricle at rest and during exertion. Similarly, the upstream effects of passive flow in the lungs are venous congestion and venous hypertension, especially marked during physical activity. The pulmonary vascular bed has emerged as a defining character on the stage of Fontan circulatory behaviour and clinical outcomes. Its pharmacologic regulation and anatomic rehabilitation therefore seem important strategic therapeutic targets. This review seeks to delineate the important aspects of pulmonary artery development and maturation in functionally univentricular physiology patients, pulmonary artery biology, pulmonary vascular reserve with exercise, and pulmonary artery morphologic and pharmacologic rehabilitation.
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12
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Welch R, Kolbe J, Lardenoye M, Ellyett K. Novel application of Poincaré analysis to detect and quantify exercise oscillatory ventilation. Physiol Meas 2021; 42. [PMID: 33740782 DOI: 10.1088/1361-6579/abf05d] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/19/2020] [Accepted: 03/19/2021] [Indexed: 11/12/2022]
Abstract
Objective.Exercise oscillatory ventilation (EOV) is frequently observed in individuals with cardiac disease. Assessment of EOV relies on pattern recognition and this subjectivity and lack of quantification limits the widespread clinical use of EOV as a prognostic marker. Poincaré analysis quantifies the short (SD1) and long-term (SD2) variability of a signal and may provide an alternative means to identify and quantify unstable exercise breathing patterns. This study aimed to determine if Poincaré analysis can distinguish between the breathing patterns of healthy control subjects and individuals being assessed for heart transplantation with and without EOV.Approach.Thirty-nine subjects performed a cardiopulmonary exercise test as part of heart transplant assessment and were subjectively classified into two groups according to the presence of EOV: non-EOV (n = 19) and EOV (n = 20). The control group (n = 24) consisted of healthy adults. Poincaré analysis (SD1 and SD2) was performed for minute ventilation (V̇E) and tidal volume (VT) normalized to forced vital capacity (V̇EnandV̇Tn), and breathing frequency (BF) for breath-by-breath data over the 10-15 ml · min-1 · kg-1V̇O2range.Main results.Poincaré analysis showed similar exercise ventilatory responses between the non-EOV and control group. BF was found to discriminate between subjects with stable and unstable ventilation. BF SD1 was significantly higher in the EOV group compared to the non-EOV (7.9 versus 4.6,p < 0.01) and control (7.9 versus 4.2,p < 0.01) groups. The EOV group had significantly greater BF SD2 compared to the non-EOV (5.7 versus 3.5,p < 0.01) and control (5.7 versus 3.5,p < 0.01) groups.Significance.We demonstrated that this novel application of Poincaré analysis can objectively distinguish and quantify unstable from stable breathing patterns during exercise. In subjects being assessed for heart transplantation the presence of EOV is associated with greater BF variability. Poincaré analysis provides an objective measure to identify and quantify EOV.Summary at a glance.As EOV may indicate abnormal ventilatory control, there is a need for an objective measure to identify and quantify unstable from stable ventilation during exercise. We developed a method of quantifying BF variation by the application of Poincaré analysis and demonstrated higher than normal variability of BF in subjects being assessed for heart transplantation who demonstrated EOV.
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Affiliation(s)
- Ryan Welch
- Respiratory Services, Auckland District Health Board, Auckland, New Zealand.,The University of Auckland, Auckland, New Zealand
| | - John Kolbe
- Respiratory Services, Auckland District Health Board, Auckland, New Zealand.,The University of Auckland, Auckland, New Zealand
| | - Mandy Lardenoye
- Respiratory Services, Auckland District Health Board, Auckland, New Zealand
| | - Kevin Ellyett
- Respiratory Services, Auckland District Health Board, Auckland, New Zealand.,The University of Auckland, Auckland, New Zealand
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13
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Abstract
As the population of adult congenital heart disease patients ages and grows, so too does the burden of heart failure in this population. Despite the advances in medical and surgical therapies over the last decades, heart failure in adult congenital heart disease remains a formidable complication with high morbidity and mortality. This review focuses on the challenges in determining the true burden and management of heart failure in adult congenital heart disease. There is a particular focus on the need for developing a common language for classifying and reporting heart failure in adult congenital heart disease, the clinical presentation and prognostication of heart failure in adult congenital heart disease, the application of hemodynamic evaluation, and advanced heart failure treatment. A common case study of heart failure in adult congenital heart disease is utilized to illustrate these key concepts.
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Affiliation(s)
- Luke J Burchill
- Department of Cardiology, The Royal Melbourne Hospital, Grattan Street, Parkville, Melbourne, VIC 3050, Australia; Department of Medicine, The University of Melbourne, Melbourne, Australia.
| | - Melissa G Y Lee
- Department of Cardiology, The Royal Melbourne Hospital, Grattan Street, Parkville, Melbourne, VIC 3050, Australia; Heart Research, Clinical Sciences, Murdoch Children's Research Institute, Melbourne, Australia; Department of Paediatrics, The University of Melbourne, Melbourne, Australia
| | - Vidang P Nguyen
- Department of Cardiology, Cedars-Sinai Heart Institute, 127 S San Vicente Boulevard a3600, Los Angeles, CA 90048, USA
| | - Karen K Stout
- Division of Cardiology, Department of Medicine, University of Washington, Seattle, WA 98195, USA
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14
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Tran DL, Gibson H, Maiorana AJ, Verrall CE, Baker DW, Clode M, Lubans DR, Zannino D, Bullock A, Ferrie S, Briody J, Simm P, Wijesekera V, D'Almeida M, Gosbell SE, Davis GM, Weintraub R, Keech AC, Puranik R, Ugander M, Justo R, Zentner D, Majumdar A, Grigg L, Coombes JS, d'Udekem Y, Morris NR, Ayer J, Celermajer DS, Cordina R. Exercise Intolerance, Benefits, and Prescription for People Living With a Fontan Circulation: The Fontan Fitness Intervention Trial (F-FIT)-Rationale and Design. Front Pediatr 2021; 9:799125. [PMID: 35071139 PMCID: PMC8771702 DOI: 10.3389/fped.2021.799125] [Citation(s) in RCA: 14] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/21/2021] [Accepted: 12/07/2021] [Indexed: 12/16/2022] Open
Abstract
Background: Despite developments in surgical techniques and medical care, people with a Fontan circulation still experience long-term complications; non-invasive therapies to optimize the circulation have not been established. Exercise intolerance affects the majority of the population and is associated with worse prognosis. Historically, people living with a Fontan circulation were advised to avoid physical activity, but a small number of heterogenous, predominantly uncontrolled studies have shown that exercise training is safe-and for unique reasons, may even be of heightened importance in the setting of Fontan physiology. The mechanisms underlying improvements in aerobic exercise capacity and the effects of exercise training on circulatory and end-organ function remain incompletely understood. Furthermore, the optimal methods of exercise prescription are poorly characterized. This highlights the need for large, well-designed, multi-center, randomized, controlled trials. Aims and Methods: The Fontan Fitness Intervention Trial (F-FIT)-a phase III clinical trial-aims to optimize exercise prescription and delivery in people with a Fontan circulation. In this multi-center, randomized, controlled study, eligible Fontan participants will be randomized to either a 4-month supervised aerobic and resistance exercise training program of moderate-to-vigorous intensity followed by an 8-month maintenance phase; or usual care (control group). Adolescent and adult (≥16 years) Fontan participants will be randomized to either traditional face-to-face exercise training, telehealth exercise training, or usual care in a three-arm trial with an allocation of 2:2:1 (traditional:telehealth:control). Children (<16 years) will be randomized to either a physical activity and exercise program of moderate-to-vigorous intensity or usual care in a two-arm trial with a 1:1 allocation. The primary outcome is a change in aerobic exercise capacity (peak oxygen uptake) at 4-months. Secondary outcomes include safety, and changes in cardiopulmonary exercise testing measures, peripheral venous pressure, respiratory muscle and lung function, body composition, liver stiffness, neuropsychological and neurocognitive function, physical activity levels, dietary and nutritional status, vascular function, neurohormonal activation, metabolites, cardiac function, quality of life, musculoskeletal fitness, and health care utilization. Outcome measures will be assessed at baseline, 4-months, and 12-months. This manuscript will describe the pathophysiology of exercise intolerance in the Fontan circulation and the rationale and protocol for the F-FIT.
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Affiliation(s)
- Derek L Tran
- Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.,Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia.,Charles Perkins Centre, Heart Research Institute, Sydney, NSW, Australia.,Faculty of Medicine and Health, The University of Sydney, Sydney, NSW, Australia
| | - Hannah Gibson
- Charles Perkins Centre, Heart Research Institute, Sydney, NSW, Australia
| | - Andrew J Maiorana
- School of Allied Health, Curtin University, Perth, WA, Australia.,Allied Health Department, Fiona Stanley Hospital, Perth, WA, Australia
| | - Charlotte E Verrall
- The University of Sydney Westmead Clinical School, Sydney, NSW, Australia.,Heart Centre for Children, The Children's Hospital at Westmead, Sydney, NSW, Australia
| | - David W Baker
- Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.,Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia
| | - Melanie Clode
- Heart Research Group, Murdoch Children's Research Institute, Melbourne, VIC, Australia
| | - David R Lubans
- School of Education, Priority Research Centre for Physical Activity and Nutrition, The University of Newcastle, Newcastle, NSW, Australia
| | - Diana Zannino
- Heart Research Group, Murdoch Children's Research Institute, Melbourne, VIC, Australia
| | - Andrew Bullock
- Paediatric and Adult Congenital Cardiology, Perth Children's Hospital, Perth, WA, Australia
| | - Suzie Ferrie
- Department of Nutrition and Dietetics, Royal Prince Alfred Hospital, Sydney, NSW, Australia
| | - Julie Briody
- Department of Nuclear Medicine, The Children's Hospital at Westmead, Sydney, NSW, Australia
| | - Peter Simm
- Heart Research Group, Murdoch Children's Research Institute, Melbourne, VIC, Australia
| | - Vishva Wijesekera
- Department of Cardiology, The Prince Charles Hospital, Brisbane, QLD, Australia
| | - Michelle D'Almeida
- Charles Perkins Centre, Heart Research Institute, Sydney, NSW, Australia
| | - Sally E Gosbell
- Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia.,Charles Perkins Centre, Heart Research Institute, Sydney, NSW, Australia.,Heart Centre for Children, The Children's Hospital at Westmead, Sydney, NSW, Australia
| | - Glen M Davis
- Faculty of Medicine and Health, The University of Sydney, Sydney, NSW, Australia
| | - Robert Weintraub
- Heart Research Group, Murdoch Children's Research Institute, Melbourne, VIC, Australia.,Department of Cardiology, The Royal Children's Hospital, Melbourne, VIC, Australia
| | - Anthony C Keech
- Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.,Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia.,NHMRC Clinical Trials Centre, The University of Sydney, Sydney, NSW, Australia
| | - Rajesh Puranik
- Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.,Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia
| | - Martin Ugander
- Royal North Shore Hospital, The Kolling Institute, Sydney, NSW, Australia
| | - Robert Justo
- Paediatric Cardiac Service, Queensland Children's Hospital, Brisbane, QLD, Australia
| | - Dominica Zentner
- The University of Melbourne Medical School, Melbourne, VIC, Australia.,Department of Cardiology, The Royal Melbourne Hospital, Melbourne, VIC, Australia
| | - Avik Majumdar
- Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia.,Australian National Liver Transplant Unit, AW Morrow Gastroenterology and Liver Centre, Royal Prince Alfred Hospital, Camperdown, NSW, Australia
| | - Leeanne Grigg
- The University of Melbourne Medical School, Melbourne, VIC, Australia.,Department of Cardiology, The Royal Melbourne Hospital, Melbourne, VIC, Australia
| | - Jeff S Coombes
- School of Human Movement and Nutrition Sciences, Centre for Research on Exercise, Physical Activity, and Health, The University of Queensland, Brisbane, QLD, Australia
| | - Yves d'Udekem
- Division of Cardiac Surgery, Children's National Hospital, Washington, DC, United States
| | - Norman R Morris
- Allied Health Collaborative and Queensland Lung Transplant Service, The Prince Charles Hospital, Brisbane, QLD, Australia.,School of Health Sciences and Social Work, Griffith University, Gold Coast, QLD, Australia
| | - Julian Ayer
- The University of Sydney Westmead Clinical School, Sydney, NSW, Australia.,Heart Centre for Children, The Children's Hospital at Westmead, Sydney, NSW, Australia
| | - David S Celermajer
- Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.,Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia.,Charles Perkins Centre, Heart Research Institute, Sydney, NSW, Australia
| | - Rachael Cordina
- Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.,Central Clinical School, The University of Sydney School of Medicine, Sydney, NSW, Australia.,Charles Perkins Centre, Heart Research Institute, Sydney, NSW, Australia.,Heart Research Group, Murdoch Children's Research Institute, Melbourne, VIC, Australia
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15
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Das BB, Godoy A, Kadish T, Niu J. Maximal versus sub-maximal effort during cardiopulmonary exercise testing in adults with congenital heart disease: outcome analysis of short-term cardiac-related events. Cardiol Young 2021; 31:91-96. [PMID: 33070792 DOI: 10.1017/s104795112000339x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
Peak respiratory exchange ratio is an objective marker of patient effort during cardiopulmonary exercise testing. We evaluated exercise variables in 175 adult congenital heart disease patients and the impact of respiratory exchange ratio on the prognostic value of exercise variables for short-term cardiac-related events. Of 175 patients, 110 completed the exercise test with a peak respiratory exchange ratio of ≥1.10 and the remaining 65 had a peak respiratory exchange ratio of <1.10. Peak oxygen consumption, the percentage of oxygen consumption at the ventilatory threshold, peak heart rate, percentage predicted peak heart rate, double product, oxygen uptake efficiency slope, and the number of patients with exercise oscillatory ventilation were reduced significantly in patients with a respiratory exchange ratio of <1.10 compared to those with a respiratory exchange ratio of ≥1.10. After a median follow-up of 21 months, total cardiac-related events occurred in 37 (21%) patients. Multivariate Cox proportional hazard analysis showed that the percentage predicted peak oxygen consumption, and oxygen uptake efficiency slope were independent predictors of cardiac-related events only in patients with a peak respiratory exchange ratio of ≥1.10. Sub-maximal exercise performance can be preserved in adult congenital heart disease patients. The percentage predicted oxygen consumption and the oxygen uptake efficiency slope are two independent predictors for short-term cardiac-related events in adult congenital heart disease patients.
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Affiliation(s)
- Bibhuti B Das
- Office of Human Research, Memorial Cardiac and Vascular Institute, Memorial Healthcare System, Hollywood, FL, USA
- Baylor College of Medicine, Texas Children's Hospital Austin Specialty Care, Austin, TX, USA
| | - Aliana Godoy
- Office of Human Research, Memorial Cardiac and Vascular Institute, Memorial Healthcare System, Hollywood, FL, USA
| | - Talya Kadish
- Office of Human Research, Memorial Cardiac and Vascular Institute, Memorial Healthcare System, Hollywood, FL, USA
| | - Jianli Niu
- Office of Human Research, Memorial Cardiac and Vascular Institute, Memorial Healthcare System, Hollywood, FL, USA
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16
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Abstract
The authors summarize the most important anatomic and physiologic substrates of Fontan circulation. Common anatomic substrates include hypoplastic left heart syndrome, tricuspid atresia, double inlet left ventricle, and unbalanced atrioventricular septal defects. After the Fontan operation exercise capacity is limited and the key hemodynamic drivers is limited preload due to a relatively fixed pulmonary vascular resistance. The authors provide contemporary data on survival, morbidity, and need for reintervention. Operative morality is now expected to be less than 1% and 30 year survival approximately 89%. The authors delineate potential therapeutic approaches for the potential late complications.
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Affiliation(s)
- Ahmed AlZahrani
- Adult Congenital Heart Disease Program, Paediatric Cardiology, Prince Sultan Cardiac Centre, PO Box 7897 - G352, Riyadh 11159, Saudi Arabia
| | - Rahul Rathod
- Department of Pediatrics, Harvard Medical School, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA
| | - Ahmed Krimly
- Department of Cardiology, King Faisal Cardiac Center, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, 6412 ibn Mashhur Street, Alsalama District, Jeddah 23436 2946, Saudi Arabia; Department of Medical Research, King Abdullah International Medical Research Center, Jeddah, Saudi Arabia; Department of Medical Research, King Saud Bin Abdulaziz University for Health Science, Jeddah, Saudi Arabia
| | - Yezan Salam
- College of Medicine, Alfaisal University, Takhassusi Street, Riyadh-11533, Saudi Arabia
| | - AlJuhara Thaar AlMarzoog
- Adult Congenital Heart Disease Service, King Faisal Specialist Hospital and Research Centre, Zahrawi Street, Al Maather, Al Maazer, Riyadh 12713, Saudi Arabia
| | - Gruschen R Veldtman
- Adult Congenital Heart Disease, Heart Centre, King Faisal Specialist Hospital and Research Centre, Zahrawi Street, Al Maather, Al Maazer, Riyadh 12713, Saudi Arabia.
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17
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Hoashi T, Shimada M, Imai K, Komori M, Kurosaki K, Ohuchi H, Ichikawa H. Long-term therapeutic effect of Fontan conversion with an extracardiac conduit. Eur J Cardiothorac Surg 2020; 57:951-957. [PMID: 31883324 DOI: 10.1093/ejcts/ezz355] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/30/2019] [Revised: 11/08/2019] [Accepted: 11/29/2019] [Indexed: 11/14/2022] Open
Abstract
OBJECTIVES The aim of this study was to identify the long-term therapeutic effect of total cavopulmonary connection (TCPC) conversion with an extracardiac conduit. METHODS Between 1991 and 2014, 36 patients underwent TCPC conversion with an extracardiac conduit. Half of these patients were diagnosed with tricuspid atresia or its variant. The left ventricle was dominant in 26 patients (72.2%). Median age at conversion and interval from initial Fontan operation to conversion were 24.1 years (interquartile range 18.9-29.2) and 17.8 years (15.4-20.9), respectively. Surgical cryoablation was concomitantly performed in 32 patients (88.9%). Cardiac catheter examination was performed preoperatively (36 patients, 100%) and at 1 year (31 patients, 86%), 5 years (25 patients, 69%) and 10 years (13 patients, 36%) after TCPC conversion. Symptom-limited treadmill exercise with expired gas analysis was performed preoperatively (32 patients, 88.9%) and at 1 year (27 patients, 75.0%), 5 years (20 patients, 55.6%) and 10 years (12 patients, 33.3%) after conversion. RESULTS All patients received follow-up; the mean follow-up period was 8.2 ± 4.8 years. Actuarial survival rate, protein-losing enteropathy-free survival rate and rate of survival with sinus rhythm maintenance at 10 years were 79.2%, 67.8% and 48.5%, respectively. The survival curve declined steeply when the duration of Fontan circulation exceeded 25 years. New cases of protein-losing enteropathy developed postoperatively in 2 patients. Permanent pacemakers were implanted in 12 patients (33%), but atrial tachyarrhythmia was not sustained in any of the remaining patients. Pulmonary arterial pressure (11.0 ± 3.1 to 9.5 ± 3.6 mmHg, P = 0.003), pulmonary vascular resistance (2.1 ± 0.7 to 1.3 ± 0.5 WU/m2, P < 0.0001) and cardiac index (2.0 ± 0.3 to 2.9 ± 0.6 l/min/m2, P < 0.0001) significantly improved from preoperative evaluation to 1 year after the conversion, and these improvements were maintained during the entire follow-up period. Peak oxygen uptake remained unchanged from the preoperative evaluation (49.7 ± 11.5% predicted) to 1 year (52.5 ± 12.0%), 5 years (56.2 ± 9.6%) and 10 years (51.2 ± 9.4%) after conversion (P = 0.19). CONCLUSIONS Owing to its anti-arrhythmic effect and Fontan pathway recruitment effect, TCPC conversion with an extracardiac conduit prevented the natural decline of exercise tolerance that is seen in classic Fontan patients.
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Affiliation(s)
- Takaya Hoashi
- Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan
| | - Masatoshi Shimada
- Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan
| | - Kenta Imai
- Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan
| | - Motoki Komori
- Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan
| | - Kenichi Kurosaki
- Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan
| | - Hideo Ohuchi
- Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan
| | - Hajime Ichikawa
- Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan
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19
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Callegari A, Neidenbach R, Milanesi O, Castaldi B, Christmann M, Ono M, Müller J, Ewert P, Hager A. A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life. CONGENIT HEART DIS 2018; 14:147-155. [PMID: 30378270 DOI: 10.1111/chd.12694] [Citation(s) in RCA: 36] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/15/2018] [Revised: 10/04/2018] [Accepted: 10/07/2018] [Indexed: 11/30/2022]
Abstract
AIM The Fontan circulation is highly dependent on ventilation, improving pulmonary blood flow and cardiac output. A reduced ventilatory function is reported in these patients. The extent of this impairment and its relation to exercise capacity and quality of life is unknown and objective of this study. METHODS This multicenter retrospective/cross-sectional study included 232 patients (140 females, age 25.6 ± 10.8 years) after Fontan palliation (19.8% atrioventricular connection; 20.3% atriopulmonary connection; 59.9% total cavopulmonary connection). Resting spirometry, cardiopulmonary exercise tests, and quality-of-life assessment (SF-36 questionnaire) were performed between 2003 and 2015. RESULTS Overall, mean forced expiratory volume in one second (FEV1 ) was 74.7 ± 17.8%predicted (%pred). In 59.5% of the patients, FEV1 was <80%pred., and all of these patients had FEV1 /forced vital capacity (FVC) > 80%, suggestive of a restrictive ventilatory pattern. Reduced FEV1 was associated with a reduced peakVO2 of 67.0 ± 17.6%pred. (r = 0.43, P < .0001), even if analyzed together with possible confounding factors (sex, BMI, age, years after palliation, number of interventions, scoliosis, diaphragmatic paralysis). Synergistically to exercise capacity, FEV1 was associated to quality of life in terms of physical component summary (r = 0.30, P = .002), physical functioning (r = 0.25, P = .008), bodily pain (r = 0.22, P = .02), and general health (r = 0.16, P = .024). Lower FEV1 was associated with diaphragmatic paralysis (P = .001), scoliosis (P = .001), higher number of interventions (P = .002), and lower BMI (P = .01). No correlation was found to ventricular morphology, type of surgeries, or other perioperative/long-term complications. CONCLUSIONS This study shows that the common restrictive ventilatory pattern in Fontan patients is associated with lower exercise capacity and quality of life. Risk factors are diaphragmatic paralysis, scoliosis, a high total number of interventions and low BMI.
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Affiliation(s)
- Alessia Callegari
- Department of Women's and Children's Health, Pediatric Cardiology, University of Padua, Padua, Italy.,Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technical University of Munich, München, Germany.,Pediatric Cardiology, Pediatric Heart Center, Department of Surgery, University Children's Hospital Zurich, Zurich, Switzerland
| | - Rhoia Neidenbach
- Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technical University of Munich, München, Germany
| | - Ornella Milanesi
- Department of Women's and Children's Health, Pediatric Cardiology, University of Padua, Padua, Italy
| | - Biagio Castaldi
- Department of Women's and Children's Health, Pediatric Cardiology, University of Padua, Padua, Italy
| | - Martin Christmann
- Pediatric Cardiology, Pediatric Heart Center, Department of Surgery, University Children's Hospital Zurich, Zurich, Switzerland
| | - Masamichi Ono
- Department of Cardiovascular Surgery, Deutsches Herzzentrum München, Technical University of Munich, München, Germany
| | - Jan Müller
- Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technical University of Munich, München, Germany
| | - Peter Ewert
- Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technical University of Munich, München, Germany
| | - Alfred Hager
- Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technical University of Munich, München, Germany
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20
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Shafer KM, Opotowsky AR, Rhodes J. Exercise testing and spirometry as predictors of mortality in congenital heart disease: Contrasting Fontan physiology with repaired tetralogy of Fallot. CONGENIT HEART DIS 2018; 13:903-910. [DOI: 10.1111/chd.12661] [Citation(s) in RCA: 17] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/19/2018] [Revised: 06/17/2018] [Accepted: 07/12/2018] [Indexed: 11/29/2022]
Affiliation(s)
- Keri M. Shafer
- Department of Cardiology; Boston Children’s Hospital; Boston Massachusetts
- Division of Cardiology; Brigham and Women’s Hospital; Boston Massachusetts
| | - Alexander R. Opotowsky
- Department of Cardiology; Boston Children’s Hospital; Boston Massachusetts
- Division of Cardiology; Brigham and Women’s Hospital; Boston Massachusetts
| | - Jonathan Rhodes
- Department of Cardiology; Boston Children’s Hospital; Boston Massachusetts
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21
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Udholm S, Aldweib N, Hjortdal VE, Veldtman GR. Prognostic power of cardiopulmonary exercise testing in Fontan patients: a systematic review. Open Heart 2018; 5:e000812. [PMID: 30057765 PMCID: PMC6059270 DOI: 10.1136/openhrt-2018-000812] [Citation(s) in RCA: 53] [Impact Index Per Article: 7.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/23/2018] [Revised: 04/03/2018] [Accepted: 04/24/2018] [Indexed: 11/04/2022] Open
Abstract
Objective Exercise impairment is common in Fontan patients. Our aim is to systematically review previous literature to determine the prognostic value of exercise capacity in older adolescent and adult Fontan patients with respect to late outcome. Additionally, we reviewed the determinants of exercise capacity in Fontan patients and changes in exercise capacity over time. Methods PubMed, CINAHL, Embase, The Cochrane Library and Scopus were searched systematically for studies reporting exercise capacity and late outcome such as mortality, cardiac transplantation and hospitalisation. Studies were eligible for inclusion if more than 30 patients were included and mean age was ≥16 years. Results Four thousand and seven hundred and twenty-two studies were identified by the systematic search. Seven studies fulfilled the inclusion and exclusion criteria. The total number of patients was 1664 adult Fontan patients. There were 149 deaths and 35 heart transplantations. All eligible studies were retrospective cohort studies. The correlation between exercise capacity and late outcome was identified, and HRs were reported. Conclusion In Fontan patients, the best predictors of death and transplantation were a decline in peak VO2, heart rate variables and exercise oscillatory ventilation. Peak VO2 was not strongly predictive of mortality or hospitalisation in Fontan patients. Several variables were strong and independent predictors of hospitalisation and morbidity.
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Affiliation(s)
- Sebastian Udholm
- Department of Cardiothoracic and Vascular Surgery, Aarhus University Hospital, Aarhus, Denmark
| | - Nael Aldweib
- Cardiovascular Medicine Department, Allegheny General Hospital, Pittsburgh, Pennsylvania, USA
| | | | - Gruschen R Veldtman
- Adult Congenital Heart Disease Program, Heart Institute, Cincinnati Children's Hospital Medical Centre, Cincinnati, Ohio, USA
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22
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Muneuchi J, Watanabe M. Absence of Exertional Oscillatory Ventilation During Exercise Related to Late Adverse Outcome in Fontan Patients. Pediatr Cardiol 2018; 39:640-641. [PMID: 29417171 DOI: 10.1007/s00246-018-1826-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/23/2016] [Accepted: 02/01/2018] [Indexed: 11/30/2022]
Affiliation(s)
- Jun Muneuchi
- Department of Pediatrics, Japan Community Healthcare Organization, Kyushu Hospital, Kitakyushu, Japan.
| | - Mamie Watanabe
- Department of Pediatrics, Japan Community Healthcare Organization, Kyushu Hospital, Kitakyushu, Japan
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Wu FM, Opotowsky AR, Denhoff ER, Gongwer R, Gurvitz MZ, Landzberg MJ, Shafer KM, Valente AM, Uluer AZ, Rhodes J. A Pilot Study of Inspiratory Muscle Training to Improve Exercise Capacity in Patients with Fontan Physiology. Semin Thorac Cardiovasc Surg 2018; 30:462-469. [DOI: 10.1053/j.semtcvs.2018.07.014] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/16/2018] [Accepted: 07/24/2018] [Indexed: 11/11/2022]
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Cunningham JW, Nathan AS, Rhodes J, Shafer K, Landzberg MJ, Opotowsky AR. Decline in peak oxygen consumption over time predicts death or transplantation in adults with a Fontan circulation. Am Heart J 2017. [PMID: 28625375 DOI: 10.1016/j.ahj.2017.04.009] [Citation(s) in RCA: 38] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
Abstract
Peak oxygen consumption (pVO2) measured by cardiopulmonary exercise test (CPET) predicts mortality in adults with a Fontan circulation. The purpose of this study was to assess the additive prognostic value of change in pVO2 over time. METHODS We analyzed a cohort of adults (≥18 years old) with a Fontan circulation who underwent at least 2 maximal CPETs separated by 6-30 months at Boston Children's Hospital between 2000 and 2015. Survival analysis was performed to determine whether changes in CPET variables, including pVO2 between consecutive tests, were associated with subsequent clinical events. The primary outcome was transplant-free survival. RESULTS The study included 130 patients with 287 CPET test pairs. Average age was 26.6±9.5 years. Baseline pVO2 averaged 22.0±5.7 mL/kg/min or 60.9%±13.7% predicted. In the cohort overall, there was no change in mean pVO2 between sequential CPETs. Eleven patients died and 2 underwent transplant. On average, pVO2 declined for patients who subsequently died or underwent transplant but remained stable among those who did not (-9.8%±14.6% vs 0.0±13.0%, P<.01). Those with a decline in pVO2 between CPETs were at greater risk of death or transplantation (per 10% decrease in pVO2: HR=2.0, 95% CI 1.2-3.1, P=.004). Change in pVO2 remained a significant predictor of death or transplant after adjusting for pVO2 at first CPET (per 10% decline in pVO2: HR=2.5, 95% CI 1.5-4.2, P<.001). CONCLUSIONS A decline in pVO2 between consecutive CPETs predicts increased risk for death or transplant in adults with a Fontan circulation independent of baseline pVO2. These results support the additive clinical value of serial CPET in this population.
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Watrous RL, Chin AJ. Model-Based Comparison of the Normal and Fontan Circulatory Systems-Part III. World J Pediatr Congenit Heart Surg 2017; 8:148-160. [PMID: 28329460 DOI: 10.1177/2150135116679831] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
BACKGROUND For patients with the Fontan circulatory arrangement, angiotensin-converting enzyme inhibition, guanylate cyclase activation, phosphodiesterase 5 inhibition, and endothelin receptor antagonism have so far resulted in little or no improvement in [Formula: see text] or peak cardiac index (CI), suggesting that our understanding of the factors that most impact the exercise hemodynamics is incomplete. METHODS To facilitate comparisons with clinical reports of the exercise performance of preadolescent Fontan patients, we rescaled our previously reported computational models of a two-year-old normal child and similarly aged Fontan patient, extended our Fontan model to capture the nonlinear relationship between flow and resistance quantified from previous computational fluid dynamic analyses of the total cavopulmonary connection (TCPC), and added respiration as well as skeletal muscle contraction. RESULTS (1) Without respiration, the computational model for both the normal and the Fontan cannot attain the values for CI at peak exercise reported in the clinical literature, (2) because flow through the TCPC is much greater during inspiration than during expiration, the effect on the CI of the dynamic (flow-related) TCPC resistance is much more dramatic during exercise than it is in breath-hold mode at rest, and (3) coupling breathing with skeletal muscle contraction leads to the highest augmentation of cardiac output, that is, the skeletal muscle pump is most effective when the intrathoracic pressure is at a minimum-at peak inspiration. CONCLUSIONS Novel insights emerge when a Fontan model incorporating dynamic TCPC resistance, full respiration, and skeletal muscle contraction can be compared to the model of the normal.
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Affiliation(s)
- Raymond L Watrous
- 1 Division of Cardiology, Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
| | - Alvin J Chin
- 1 Division of Cardiology, Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.,2 Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
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Dhakal BP, Lewis GD. Exercise oscillatory ventilation: Mechanisms and prognostic significance. World J Cardiol 2016; 8:258-266. [PMID: 27022457 PMCID: PMC4807314 DOI: 10.4330/wjc.v8.i3.258] [Citation(s) in RCA: 26] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/02/2015] [Accepted: 12/18/2015] [Indexed: 02/06/2023] Open
Abstract
Alteration in breathing patterns characterized by cyclic variation of ventilation during rest and during exercise has been recognized in patients with advanced heart failure (HF) for nearly two centuries. Periodic breathing (PB) during exercise is known as exercise oscillatory ventilation (EOV) and is characterized by the periods of hyperpnea and hypopnea without interposed apnea. EOV is a non-invasive parameter detected during submaximal cardiopulmonary exercise testing. Presence of EOV during exercise in HF patients indicates significant impairment in resting and exercise hemodynamic parameters. EOV is also an independent risk factor for poor prognosis in HF patients both with reduced and preserved ejection fraction irrespective of other gas exchange variables. Circulatory delay, increased chemosensitivity, pulmonary congestion and increased ergoreflex signaling have been proposed as the mechanisms underlying the generation of EOV in HF patients. There is no proven treatment of EOV but its reversal has been noted with phosphodiesterase inhibitors, exercise training and acetazolamide in relatively small studies. In this review, we discuss the mechanistic basis of PB during exercise and the clinical implications of recognizing PB patterns in patients with HF.
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Budts W, Roos-Hesselink J, Rädle-Hurst T, Eicken A, McDonagh TA, Lambrinou E, Crespo-Leiro MG, Walker F, Frogoudaki AA. Treatment of heart failure in adult congenital heart disease: a position paper of the Working Group of Grown-Up Congenital Heart Disease and the Heart Failure Association of the European Society of Cardiology. Eur Heart J 2016; 37:1419-27. [PMID: 26787434 DOI: 10.1093/eurheartj/ehv741] [Citation(s) in RCA: 169] [Impact Index Per Article: 18.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/09/2015] [Accepted: 12/14/2015] [Indexed: 12/11/2022] Open
Affiliation(s)
- Werner Budts
- Congenital and Structural Cardiology, University Hospitals Leuven, Herestraat 49, B-3000 Leuven, Belgium
| | - Jolien Roos-Hesselink
- Department of Cardiology, Erasmus Medical Center Rotterdam, Rotterdam, The Netherlands
| | - Tanja Rädle-Hurst
- Department of Pediatric Cardiology, Saarland University Medical Center, Homburg, Germany
| | | | | | - Ekaterini Lambrinou
- Department of Nursing, School of Health Sciences Cyprus University of Technology, Limassol, Cyprus
| | - Maria G Crespo-Leiro
- Advanced Heart Failure and Heart Transplantation Unit, Cardiology Service, Hospital Universitario A Coruña, La Coruña, Spain
| | - Fiona Walker
- Centre for Grown-Up Congenital Heart Disease, St Bartholomews Hospital, London, UK
| | - Alexandra A Frogoudaki
- Adult Congenital Heart Clinic, Second Cardiology Department, ATTIKON University Hospital and Athens University, Athens, Greece
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