Skip to main content
Key Specialties
Anesthesiology Cardiology Dermatology Diabetology Endocrinology Gastroenterology and Hepatology General Medicine Geriatrics Hematology Infectious Diseases Internal Medicine Nephrology Neurology Obstetrics and Gynecology Oncology Ophthalmology Pediatrics Psychiatry Radiology Respiratory Medicine Rheumatology Surgery Urology
Congress Coverage
ASH 2024 Annual Meeting 2024 ESMO Congress 2024 ERS Congress 2024 EASD Congress 2024 ESC Congress 2024 EAN Congress 2024 ASCO Annual Meeting 2024 ACC Congress
Clinical Collections
Women’s Health Knowledge Hub Advances in Alzheimer’s

Navigating neuroimaging in Alzheimer disease care

PET imaging is playing an increasingly critical role in managing AD, but how can you effectively integrate it into clinical practice? Our expert-led program will empower you with practical strategies and real-world case studies.

This content is intended for healthcare professionals outside of the UK.
ADVANCED SEARCH
Log in
Top
Pediatric Cardiology
Published in:

09-05-2024 | Cardiac Resynchronization Therapy | Research

Adverse Effect of Bundle Branch Block on Exercise Performance in Patients with Fontan Physiology: From the Pediatric Heart Network Fontan Public Data Set

Authors: Omar Meziab, Emily Dereszkiewicz, Claudia E. Guerrero, Andrew W. Hoyer, Brent J. Barber, Scott E. Klewer, Michael D. Seckeler

Published in: Pediatric Cardiology | Issue 4/2025

Login to get access

Abstract

Patients with Fontan physiology have reduced exercise performance compared to their peers as well as a higher incidence of bundle branch block (BBB). This study aims to investigate the association between BBB and exercise performance in the Fontan population through a retrospective review of the Pediatric Heart Network Fontan study public use dataset. “Low Performers” were defined as ≤ 25th percentile (for Fontan patients) for each exercise parameter at anaerobic threshold (AT) for gender and age and “Normal Performers” were all other patients. A total of 303 patients with Fontan physiology who underwent exercise testing reached AT and had complete data for BBB. BBB occurred more frequently in Low Performers for VO2 [OR (95% CI): 2.6 (1.4, 4.8)] and Work [OR (95% CI): 2.7 (1.4, 5.1)], suggesting that BBB in the Fontan population is associated with reduced exercise performance. This data adds to the existing clinical evidence of the adverse effects of conduction abnormalities on single ventricle cardiac output and adds support for consideration of cardiac resynchronization and multi-site ventricular pacing in this patient population.
Literature
1.
O’Leary PW (2002) Prevalence, clinical presentation and natural history of patients with single ventricle. Prog Pediatr Cardiol 16:31–38CrossRef
2.
Hoffman JI, Kaplan S (2002) The Incidence of congenital heart disease. J Am Coll Cardiol 39(12):1890–1900CrossRefPubMed
3.
Pundi KN, Johnson JN, Dearani JA, Pundi KN, Li Z, Hinck CA, Dahl SH, Cannon BC, O’Leary PW, Driscoll DJ, Cetta F (2015) 40-year follow-up after the Fontan operation: long-term outcomes of 1,052 patients. J Am Coll Cardiol 66(15):1700–1710CrossRefPubMed
4.
Rychik J, Atz AM, Celermajer DS, Deal BJ, Gatzoulis MA, Gewillig MH, Hsia T, Hsu DT, Kovacs AH, McCrindle BW, Newburger JW, Pike NA, Rodefeld M, Rosenthal DN, Schumacher KR, Marino BS, Stout K, Veldtman G, Younoszai AK, d’Udekem Y (2019) Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association. Circulation 150(6):e234–e284
5.
Seckeler MD, Moe TG, Thomas ID, Meziab O, Andrews J, Heller E, Klewer SE (2015) Hospital resource utilization for common noncardiac diagnoses in adult survivors of single cardiac ventricle. Am J Cardiol 116(11):1756–1761CrossRefPubMed
6.
Graham EM, Scheurer MA, Saul JP, Bradley SM, Atz AM (2007) QRS duration following the Norwood procedure: Blalock-taussig shunt versus right ventricle to pulmonary artery shunt. Pacing Clin Electrophysiol 30(11):1336–1338CrossRefPubMed
7.
Khan S, Nadorlik H, Kertesz N, Nicholson L, Ro PS, Cua CL (2015) QRS duration changes in patients with single ventricle physiology: birth to 10 years. Pacing Clin Electrophysiol 38(10):1159–1165CrossRefPubMed
8.
Talreja D, Gruver C, Sklenar J, Dent J, Kaul S (2000) Efficient utilization of echocardiography for the assessment of left ventricular systolic function. Am Heart J 139(3):394–398CrossRefPubMed
9.
Dhingra R, Nam BH, Benjamin EJ, Wang TJ, Larson MG, D’Agostino RB Sr, Levy D, Vasan RS (2005) Cross-sectional relations of electrocardiographic QRS duration to left ventricular dimensions: the Framingham Heart Study. J Am Coll Cardiol 45(5):685–689CrossRefPubMed
10.
Bristow MR, Saxon LA, Boehmer J, Krueger S, Kass DA, De Marco T, Carson P, DiCarlo L, DeMets D, White BG, DeVries DW, Feldman AM (2004) Cardiac-resynchronization therapy with or without an implantable defibrillator in advanced chronic heart failure. N Engl J Med 350(21):2140–2150CrossRefPubMed
11.
Cleland JG, Daubert J, Erdmann E, Freemantle N, Gras D, Kappenberger L, Tavazzi L (2005) The effect of cardiac resynchronization on morbidity and mortality in heart failure. N Engl J Med 352(15):1539–1549CrossRefPubMed
12.
Kusumoto FM, Schoenfeld MH, Barrett C, Edgerton JR, Ellenbogen KA, Gold MR, Goldschlager NF, Hamilton RM, Joglar JA, Kim RJ, Lee R, Marine JE, McLeod CJ, Oken KR, Patton KK, Pellegrini CN, Selzman KA, Thompson A, Varosy PD (2019) 2018 ACC/AHA/HRS guideline on the evaluation and management of patients with bradycardia and cardiac conduction delay: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation 140(8):e382–e482PubMed
13.
Janousek J, Gebauer RA, Abdul-Khaliq H, Turner M, Kornyei L, Grollmuss O, Rosenthal E, Villain E, Fruh A, Paul T, Blom NA, Happonen J, Bauersfeld U, Jacobsen JR, van den Heuvel F, Delhaas T, Papagiannis J, Trigo C (2009) Cardiac resynchronization therapy in paediatric and congenital heart disease: differential effects in various anatomical and functional substrates. Heart 95(14):1165–1171CrossRefPubMed
14.
Gokhale J, Husain N, Nicholson L, Texter KM, Zaidi AN, Cua CL (2013) QRS duration and mechanical dyssynchrony correlations with right ventricular function after Fontan procedure. J Am Soc Echocardiogr 26(2):154–159CrossRefPubMed
15.
Zaidi SJ, Penk J, Cui VW, Roberson DA (2019) Right ventricular mechanical dyssynchrony in hypoplastic left heart syndrome: correlation with systolic function and QRS duration. Pediatric Cardiol 40(5):934–942CrossRef
16.
Gearhart A, Bassi S, Rathod RH, Beroukhim RS, Lipsitz S, Gold MP, Harrild DM, Dionne A, Ghelani SJ (2023) Ventricular dyssynchrony late after the Fontan operation is associated with decreased survival. J Cardiovasc Magn Reason 25:66CrossRef
17.
Rosner A, Khalapyan T, Dalen H, McElhinney DB, Friedberg MK, Lui GK (2018) Classic-pattern dyssynchrony in adolescents and adults with a Fontan circulation. J Am Soc Echocardiogr 31(2):211–219CrossRefPubMed
18.
Rosner A, McElhinney DB, Goa Diab S, Friedberg MK, Lui GK (2022) Classic-pattern dyssynchrony is associated with outcome in patients with Fontan circulation. J Am Soc Echocardiogr 35(5):513–522CrossRefPubMed
19.
Gewillig MH, Lundstrom UR, Bull C, Wyse RK, Deanfield JE (1990) Exercise responses in patients with congenital heart disease after Fontan repair: patterns and determinants of performance. J Am Coll Cardiol 15(6):1424–1432CrossRefPubMed
20.
Paridon SM, Mitchell PD, Colan SD, Williams RV, Blaufox A, Li JS, Margossian R, Mital S, Russell J, Rhodes J (2008) A cross-sectional study of exercise performance during the first 2 decades of life after the Fontan operation. J Am Coll Cardiol 52(2):99–107CrossRefPubMed
21.
Ohuchi H, Miyazaki A, Wakisaka Y, Watanabe K, Kishiki K, Yamada O, Yagihara T, Echigo S (2009) Systemic ventricular morphology-associated increased QRS duration compromises the ventricular mechano-electrical and energetic properties long-term after the Fontan operation. Int J Cardiol 133(3):371–380CrossRefPubMed
22.
Westhoff-Bleck M, Norozi K, Schoof S, Ruchs M, Tutarel O, Drexler H, Wessel A, Meyer GP (2009) QRS duration in Fontan circulation in adults: a predictor of aerobic capacity. Int J Cardiol 132(3):375–381CrossRefPubMed
23.
Wilson LH, Chowdhury SM, Jackson LB (2022) QRS fragmentation and prolongation as predictors of exercise capacity in patients after Fontan palliation. Pacing Clin Electrophysiol 45(6):786–796CrossRefPubMedPubMedCentral
24.
Goldberg DJ, Zak V, Goldstein BH, Schumacher KR, Rhodes J, Penny DJ, Petit CJ, Ginde S, Menon SC, Kim S, Kim GB, Nowlen TT, DiMaria MV, Frischhertz BP, Wagner JB, McHugh KE, McCrindle BW, Shillingford AJ, Sabati AA, Yetman AT, John AS, Richmond ME, Files MD, Payne RM, Mackie AS, Davis CK, Shahanavaz S, Hill KD, Garg R, Jacobs JP, Hamstra MS, Woyciechowski S, Rathge KA, McBride MG, Frommelt PC, Russell MW, Urbina EM, Yeager JL, Pemberton VL, Stylianou MP, Pearson GD, Paridon SM (2020) Results of the FUEL Trial. Circulation 141(8):641–651CrossRefPubMed
25.
Seckeler MD, Barber BJ, Colombo JN, Bernardi AM, Hoyer AW, Andrews JG, Klewer SE (2021) Exercise performance in adolescents with fontan physiology (from the Pediatric Heart Network Fontan Public Data Set). Am J Cardiol 149:119–125CrossRefPubMed
26.
Diller GP, Dimopoulos K, Okonko D, Li W, Babu-Narayan SV, Brogerg CS, Johansson B, Bouzas B, Mullen MJ, Poole-Wilson PA, Francis DP, Gatzoulis MA (2005) Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation 112(6):828–835CrossRefPubMed
27.
Diller GP, Giardini A, Dimopoulos K, Gargiulo G, Muller J, Derrick G, Giannakoulas G, Khambadkone S, Lammers AE, Picchio FM, Gatzoulis MA, Hager A (2010) Predictors of morbidity and mortality in contemporary Fontan patients: results from a multicentre study including cardiopulmonary exercise testing in 321 patients. Eur Heart J 31(24):3073–3083CrossRefPubMed
28.
Bossers SS, Helbing WA, Duppen N, Kuipers IM, Schokking M, Hazekamp MG, Bogers AJJC, Ten Harkel ADJ, Takken T (2014) Exercise capacity in children after total cavopulmonary connection: lateral tunnel versus extracardiac conduit technique. J Thorac Cardiovasc Surg 148(4):1490–1497CrossRefPubMed
29.
Egbe AC, Driscoll DJ, Khan AR, Said SS, Akintoye E, Berganza FM, Connolly HM (2017) Cardiopulmonary exercise test in adults with prior Fontan operation: the prognostic value of serial testing. Int J Cardiol 235:6–10CrossRefPubMed
30.
Glikson M, Nielsen JC, Kronborg MB, Michowitz Y, Auricchio A, Barbash IM, Barrabes JA, Boriani G, Braunschweig F, Brignole M, Burri H, Coats AJS, Deharo J, Delgado V, Diller G, Israel CW, Keren A, Knops RE, Kotecha D, Leclercq C, Merkely B, Starck C, Thylen I, Tolosana JM (2021) 2021 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy. Eur Heart J 42(35):3427–3520CrossRefPubMed
31.
Tracy CM, Epstein AE, Darbar D, Dimarco JP, Dunbar SB, Estes NAM, Ferguson TB Jr, Hammill SC, Karasik PE, Link MS, Marine JE, Schoenfeld MH, Shanker AJ, Silka MJ, Stevenson LW, Stevenson WG, Varosy PD (2012) 2012 ACCF/AHA/HRS focused update of the 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Heart Rhythm 9(10):1737–1753CrossRefPubMed
32.
Dubin AM, Janousek J, Rhee E, Strieper MJ, Cecchin F, Law KH, Shannon KM, Temple J, Rosenthal E, Zimmerman FJ, Davis A, Karpawich PP, Al Ahmad A, Vetter VL, Kertesz NJ, Shah M, Snyder C, Stephenson E, Emmel M, Sanatani S, Kanter R, Batra A, Collins KK (2005) Resynchronization therapy in pediatric and congenital heart disease patients: an international multicenter study. J Am Coll Cardiol 46(12):2277–2283CrossRefPubMed
33.
Cecchin F, Frangini PA, Brown DW, Fynn-Thompson F, Alexander ME, Triedman JK, Gauvreau K, Walsh EP, Berul CI (2009) Cardiac resynchronization therapy (and multisite pacing) in pediatrics and congenital heart disease: five years experience in a single institution). J Cardiovasc Electrophsyiol 20(1):58–65CrossRef
34.
Chubb H, Rosenthal DN, Almond CS, Ceresnak SR, Motonaga KS, Arunamata AA, Long J, Trela AV, Hanisch D, McElhinney DB, Dubin AM (2020) Impact of cardiac resynchronization therapy on heart transplant-free survival in pediatric and congenital heart disease patients. Circ Arrhythm Electrophysiol 13(4):e007925PubMed
35.
O’Leary ET, Gauvreau K, Alexander ME, Banka P, Bezzerides VJ, Fynn-Thompson F, Triedman JK, Walsh EP, Mah DY (2018) Dual-site ventricular pacing in patients with Fontan physiology and heart block: does it mitigate the detrimental effects of single-site ventricular pacing? JACC Clin Electrophysiol 4(10):1289–1297CrossRefPubMed
36.
Joyce J, O’Leary ET, Mah EY, Harrild DM, Rhodes J (2020) Cardiac resynchronization therapy improves the ventricular function of patients with Fontan physiology. Am Heart J 230:82–92CrossRefPubMed
37.
Bacha EA, Zimmerman FJ, Mor-Avi V, Weinert L, Starr JP, Sugeng L, Lang RM (2004) Ventricular resynchronization by multisite pacing improves myocardial performance in the postoperative single-ventricle patient. Ann Thorac Surg 78(5):1678–1683CrossRefPubMed
Metadata
Title
Adverse Effect of Bundle Branch Block on Exercise Performance in Patients with Fontan Physiology: From the Pediatric Heart Network Fontan Public Data Set
Authors
Omar Meziab
Emily Dereszkiewicz
Claudia E. Guerrero
Andrew W. Hoyer
Brent J. Barber
Scott E. Klewer
Michael D. Seckeler
Publication date
09-05-2024
Publisher
Springer US
Published in
Pediatric Cardiology / Issue 4/2025
Print ISSN: 0172-0643
Electronic ISSN: 1432-1971
DOI
https://doi.org/10.1007/s00246-024-03500-1

Other articles of this Issue 4/2025

Comparison and Agreement between Cardiovascular Computed Tomography-Derived Mid-Diastolic and End-Diastolic Ventricular Volume in Patients with Congenital Heart Disease

Response to “Health-Related Quality of Life After Neonatal Treatment of Symptomatic Tetralogy of Fallot: Insights from the Congenital Cardiac Research Collaborative”

The Effects of Aspirin dose in Children with Congenital and Acquired Heart Disease. Results from the Paediatric Study of Aspirin Efficacy using Diagnostic and Monitoring Tools (PAED-M)

Transcatheter Closure of Perimembranous Ventricular Septal Defect Using KONAR-MF™: A Multicenter Experience

Quantitative Evaluation of Right Ventricular Workload Based on the Stroke Work Index in Patients after Right Ventricular Outflow Tract Reconstruction

Brachiocephalic Vein Duplication: Case Report of a Double Left Brachiocephalic Vein in an Infant

Keynote series | Spotlight on menopause

Menopause can have a significant impact on the body, with effects ranging beyond the endocrine and reproductive systems. Learn about the systemic effects of menopause, so you can help patients in your clinics through the transition.   

Prof. Martha Hickey
Dr. Claudia Barth
Dr. Samar El Khoudary
Developed by: Springer Medicine
Watch now
Video

A quick guide to ECGs

Improve your ECG interpretation skills with this comprehensive, rapid, interactive course. Expert advice provides detailed feedback as you work through 50 ECGs covering the most common cardiac presentations to ensure your practice stays up to date. 

PD Dr. Carsten W. Israel
Developed by: Springer Medizin
Start the cases
Image Credits