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Does reduced cardiopulmonary exercise testing performance predict poorer quality of life in adult patients with Fontan physiology?

Published online by Cambridge University Press:  21 October 2020

Blair Suter Open the ORCID record for Blair Suter [Opens in a new window] ,
William A. Kay ,
Alisha M. Kuhlenhoelter  and
Eric S. Ebenroth
Blair Suter*
Affiliation:
Department of Internal Medicine, Indiana University School of Medicine, Indianapolis, IN, USA Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA
William A. Kay
Affiliation:
Kay Cardiology Consulting, LLC, Zionsville, IN, USA
Alisha M. Kuhlenhoelter
Affiliation:
Department of Pediatrics, Section of Pediatric Cardiology, Indiana University School of Medicine, Indianapolis, IN, USA
Eric S. Ebenroth
Affiliation:
Department of Pediatrics, Section of Pediatric Cardiology, Indiana University School of Medicine, Indianapolis, IN, USA
*
Author for correspondence: Blair Suter, Division of Cardiovascular Health and Disease, 231 Albert Sabin Way, MLC 0542, Cincinnati, OH45267, USA. Tel: 513-558-3070; Fax: 513-558-1255. E-mail: [email protected]
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Abstract

Background:

Cardiopulmonary exercise testing performance has been shown to be a predictor of morbidity, mortality, and quality of life in patients with Fontan physiology; however, the role of exercise performance along with other diagnostics is not fully understood. We evaluated the hypothesis that reduced exercise performance correlates with poorer quality of life in Fontan patients as they continue to age.

Methods:

Chart review was performed on patients 12 years and older with Fontan who had completed cardiopulmonary exercise testing and age-appropriate quality of life surveys. Quality of life outcomes were analysed against exercise performance and other descriptive data.

Results:

For the younger cohort (n = 22), exercise performance predicted quality of life with different measures across domains and had a stronger correlation than echocardiographic parameters. For the older cohort (n = 34), exercise performance did not predict quality of life.

Conclusions:

Objective exercise performance was a useful marker for general, physical, emotional, social, and school quality of life in a younger cohort but less helpful in older adults. This is perhaps due to older patients accommodating to their conditions over time. The role of exercise performance and objective data in predicting quality of life in patients with Fontan physiology is incompletely understood and additional prospective evaluation should be undertaken.

Keywords

Quality of lifeFontan procedureuniventricular heartheart defects
Type
Original Article
Information
Cardiology in the Young , Volume 31 , Issue 1 , January 2021 , pp. 84 - 90
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Copyright
© The Author(s), 2020. Published by Cambridge University Press

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References

Alsaied, T, Bokma, JP, Engel, ME, et al. Factors associated with long-term mortality after Fontan procedures: a systematic review. Heart 2017; 103: 104–110. doi: 10.1136/heartjnl-2016-310108CrossRefGoogle ScholarPubMed
Ohuchi, H, Negishi, J, Noritake, K, et al. Prognostic value of exercise variables in 335 patients after the Fontan operation: a 23-year single-center experiences of cardiopulmonary exercise testing. Congenit Heart Dis 2015; 10: 105–116. doi: 10.1111/chd.12222 CrossRefGoogle Scholar
Bratt, EL, Moons, P. Forty years of quality-of-life research in congenital heart disease: temporal trends in conceptual and methodological rigor. Int J Cardiol 2015; 195: 1–6. doi: 10.1016/j.ijcard.2015.05.070 CrossRefGoogle ScholarPubMed
Apers, S, Kovacs, AH, Luyckx, K, et al. Quality of life of adults with congenital heart disease in 15 countries: evaluating country-specific characteristics. J Am Coll Cardiol 2016; 67: 2237–2245. doi: 10.1016/j.jacc.2016.03.477 CrossRefGoogle ScholarPubMed
While, AE, Heery, E, Sheehan, AM, Coyne, I. Health-related quality of life with long-term illnesses before and after transfer from child to adult healthcare. Child Care Health Dev 2017; 43: 144–151. doi: 10.1111/cch.12410 CrossRefGoogle ScholarPubMed
Uzark, K, Zak, V, Shrader, P, et al. Assessment of quality of life in young patient with single ventricle after the Fontan operation. J Pediatr 2016; 170: 166–172.e1. doi: 10.1016/j.jpeds.2015.11.016 CrossRefGoogle ScholarPubMed
Hager, A, Hess, J. Comparison of health-related quality of life with cardiopulmonary exercise testing in adolescents and adults with congenital heart disease. Heart 2005: 515–520. doi: 10.1136/hrt.2003.032722 Google ScholarPubMed
Atz, AM, Zak, V, Mahony, L, et al. Longitudinal outcomes of patients with single ventricle after the Fontan procedure. J Am Coll Cardiol 2017; 69: 2735–2744. doi: 10.1016/j.jacc.2017.03.582 CrossRefGoogle ScholarPubMed
Varni, JW, Burwinkle, TM, Seid, M, Skarr, D. The PedsQL 4.0 as a pediatric population health measure: feasibility, reliability, and validity. Ambul Pediatr 2003; 3: 329–341. doi: 10.1367/1539-4409(2003)003<0329:tpaapp>2.0.co;2 2.0.CO;2>CrossRefGoogle ScholarPubMed
Hays, RD, Morales, LS. The RAND-36 measure of health-related quality of life. Ann Med 33; 350–357. doi: 10.3109/07853890109002089 Google Scholar
Lewis, EF, Lamas, GA, O’Meara, E, et al. Characterization of health-related quality of life in heart failure patients with preserved versus low ejections fraction in CHARM. Eur J Heart Fail 2007; 9: 83–91. doi: 10.1016/j.ejheart.2006.10.012 Google Scholar
Chandra, A, Vaduganathan, M, Lewis, EF, et al. Health-related quality of life in heart failure with preserved ejection fraction: the PARAGON-HF trial. JACC Heart Fail 2019; 7: 862–874. doi: 10.1016/j.jchf.2019.05.015 CrossRefGoogle ScholarPubMed
D’Udekem, Y, Cheung, MM, Setyapranata, S, et al. How good is a good Fontan? Quality of life and exercise capacity of Fontans without arrhythmias. Ann Thoracic Surg 2009; 88: 1961–1969. doi: 10.1016/j.athoracsur.2009.07.079 CrossRefGoogle ScholarPubMed
Das, BB, Young, M, Niu, J, Veldtman, G. Relation between New York Heart Association functional class and objective measures of cardiopulmonary exercise in adults with congenital heart disease. Am J Cardiol 2019; 123: 1868–1873. doi: 10.1016/j.amjcard.2019.02.053 CrossRefGoogle ScholarPubMed
Diller, GP, Dimopoulous, K, Okonko, D, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation 2005; 112: 828–835. doi: 10.1161/CIRCULATIONAHA.104.529800 CrossRefGoogle ScholarPubMed
Bredy, C, Ministeri, M, Kempny, A, et al. New York Heart Association (NYHA) classification in adults with congenital heart disease: relation to objective measures of exercise and outcome. Eur Heart J 2018; 4, 51–58. doi: doi.org/10.1093/ehjqcco/qcx031 Google ScholarPubMed
Jackson, JL, Hassen, L, Gerardo, GM, Vannatta, K, Daniels, CJ. Medical factors that predict quality of life for young adults with congenital heart disease: what matters most? Int J Cardiol 2016; 202: 804–809. doi: 10.1016/j.ijcard.2015.09.116 CrossRefGoogle ScholarPubMed
Mackie, AS, Ionescu-Ittu, R, Therrien, J, Pilote, L, Abrahamowicz, M, Marelli, AJ. Children and adults with congenital heart disease lost to follow-up: who and when? Circulation 2009; 120: 302–309. doi: 10.1161/CIRCULATIONAHA.108.839464 Google ScholarPubMed
Duffer, K, Suppen, N, Kuipers, IM, et al. Aerobic exercise influences quality of life in children and youngsters with congenital heart disease: a randomized controlled trial. J Adolesc Health 2014; 55: 65–72. doi: 10.1016/j.jadohealth.2013.12.010 Google Scholar
Gierat-Haponiuk, K, Haponiuk, I, Szalewska, D, et al. Effect of complex cardiac rehabilitation on physical activity and quality of life during long-term follow-up after surgical correction of congenital heart disease. Kardiol Pol 2015; 73: 267–273. doi: 10.5603/KP.a2014.0206 CrossRefGoogle ScholarPubMed
Optowsky, AR, Rhodes, J, Landzberg, MJ, et al. A randomized trial comparing cardiac rehabilitation to standard of care for adults with congenital heart disease. World J Pediatr Congenit Heart Surg 2018; 9: 185–193. doi: 10.1177/2150135117752123 CrossRefGoogle Scholar
Wittekind, S, Mays, W, Gerdes, Y, et al. A novel mechanism for improved exercise performance in pediatrics Fontan patients after cardiac rehabilitations. Ped Cardiol 2018; 39: 1023–1030. doi: 10.1007/s00246-018-1854-3 CrossRefGoogle Scholar
Jacobsen, RM, Ginde, S, Mussatto, K, Neubauer, J, Earing, M, Danduran, M. Can a home-based cardiac physical activity program improve the physical function quality of life in children with Fontan circulation? Congenit Heart Dis 2016; 11: 175–182. doi: 10.1111/chd.12330 CrossRefGoogle ScholarPubMed
Sanberg, C, Hedstrom, M, Wadell, K, et al. Home-based interval training increases endurance capacity in adults with complex congenital heart disease. Congenit Heart Dis 2017. doi: 10.1111/chd.12562 Google Scholar