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Quality of life of adult Fontan patients

Published online by Cambridge University Press:  26 October 2020

Alexandra Soufi
Affiliation:
Cardiology Department, Jean Minjoz University Medical Center, Besançon, France Congenital Cardiology Department, Cardiovascular Hospital Louis Pradel, Hospices Civils de Lyon, Lyon, France
Marielle Gouton
Affiliation:
Congenital Cardiology Department, Cardiovascular Hospital Louis Pradel, Hospices Civils de Lyon, Lyon, France
Olivier Metton
Affiliation:
Congenital Cardiology Department, Cardiovascular Hospital Louis Pradel, Hospices Civils de Lyon, Lyon, France
Julia Mitchell
Affiliation:
Congenital Cardiology Department, Cardiovascular Hospital Louis Pradel, Hospices Civils de Lyon, Lyon, France
Yvette F. Bernard
Affiliation:
Cardiology Department, Jean Minjoz University Medical Center, Besançon, France
Andre Bozio
Affiliation:
Congenital Cardiology Department, Cardiovascular Hospital Louis Pradel, Hospices Civils de Lyon, Lyon, France
Jean Ninet
Affiliation:
Congenital Cardiology Department, Cardiovascular Hospital Louis Pradel, Hospices Civils de Lyon, Lyon, France
Sylvie Di Filippo*
Affiliation:
Congenital Cardiology Department, Cardiovascular Hospital Louis Pradel, Hospices Civils de Lyon, Lyon, France
*
Author for correspondence: Sylvie Di Filippo, Department of Congenital Heart Disease, Cardiovascular Hospital Louis Pradel, 28 Avenue Doyen Lépine, 69677 Lyon, France. Tel: +33 (0)6 10 32 15 12; Fax: +33 (0)4 72 35 70 49. E-mail: sylvie.di-filippo@chu-lyon.fr.

Abstract

Background:

Progress in the management of complex congenital heart disease (CHD) led to an improvement in survival rates of adults with a Fontan-like circulation. The objective of this study was to assess the subjective health status and quality of life of this population.

Methods and results:

Patients aged more than 18 years at the time of the study, who underwent a Fontan-like procedure. Subjective health status was assessed by the SF-36 questionnaire and a linear analog scale was used to score patients’ self-perception of their quality of life; cardiac and demographic parameters were collected.

Results:

Among 65 eligible patients, 60 (23 females; mean ± SD age: 25.7 ± 7.2 years) answered the SF-36 questionnaire and 46 of these were interviewed to evaluate their perceived quality of life. Among them, 20 (33.3%) were working full-time and 21 (35%) experienced arrhythmias. The physical SF-36 scores were lower in patients than in the general population (p ≤ 0.05). The New York Hear Association (NYHA) class and occupation were correlated with SF-36 scores of physical activity (respectively, p = 0.0001 and p = 0.025). SF-36 scores of psychological status were associated with the number of drugs and occupation (respectively, p = 0.0001 and p = 0.02). The mean ± SD quality of life score measured using a linear analog scale was 7.02 ± 1.6 and was linked to education and occupation (p ≤ 0.05) but not with cardiac parameters.

Conclusion:

Adult Fontan patients perceive an impaired physical health but report a good overall quality of life. Education and occupation impacts significantly on Fontan patients’ quality of life.

Type
Original Article
Copyright
© The Author(s), 2020. Published by Cambridge University Press

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References

Marelli, AJ, Mackie, AS, Ionescu-Ittu, R, Rahme, E, Pilote, L. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation 2007; 115: 163172.10.1161/CIRCULATIONAHA.106.627224CrossRefGoogle ScholarPubMed
Fontan, F, Baudet, E. Surgical repair of tricuspid atresia. Thorax 1971; 26: 240248.10.1136/thx.26.3.240CrossRefGoogle ScholarPubMed
Walker, F. Pregnancy and the various forms of the Fontan circulation. Heart 2007; 93: 152154.10.1136/hrt.2006.091082CrossRefGoogle ScholarPubMed
Julsrud, PR, Weigel, TJ, Van Son, JA, et al. Influence of ventricular morphology on outcome after the Fontan procedure. Am J Cardiol 2000; 86: 319323.10.1016/S0002-9149(00)00922-XCrossRefGoogle ScholarPubMed
Driscoll, DJ. Long-term results of the Fontan operation. Pediatr Cardiol 2007; 28: 438442.10.1007/s00246-007-9003-4CrossRefGoogle ScholarPubMed
Khairy, P, Fernandes, SM, Mayer, JE Jr, et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation 2008; 117: 8592.10.1161/CIRCULATIONAHA.107.738559CrossRefGoogle ScholarPubMed
d’Udekem, Y, Xu, MY, Galati, JC, et al. Predictors of survival after single-ventricle palliation: the impact of right ventricular dominance. J Am Coll Cardiol 2012; 59: 11781185.CrossRefGoogle ScholarPubMed
Kovacs, AH, Sears, SF, Saidi, AS. Biopsychosocial experiences of adults with congenital heart disease: review of the literature. Am Heart J 2005; 150: 193201.CrossRefGoogle ScholarPubMed
Marino, BS, Shera, D, Wernovsky, G, et al. The development of the pediatric cardiac quality of life inventory: a quality of life measure for children and adolescents with heart disease. Qual Life Res 2008; 17: 613626.10.1007/s11136-008-9323-8CrossRefGoogle ScholarPubMed
McHorney, CA, Ware, JE Jr, Lu, JF, Sherbourne, CD. The MOS 36-item Short-Form Health Survey (SF-36): III. Tests of data quality, scaling assumptions, and reliability across diverse patient groups. Med Care 1994; 32: 4066.CrossRefGoogle ScholarPubMed
Leplege, A, Ecosse, E, Verdier, A, Perneger, TV. The French SF-36 Health Survey: translation, cultural adaptation and preliminary psychometric evaluation. J Clin Epidemiol 1998; 51: 10131023.CrossRefGoogle ScholarPubMed
Rassart, J, Apers, S, Kovacs, AH, et al. Illness perceptions in adult congenital heart disease: a multi-center international study. Int J Cardiol 2017; 244: 130138.CrossRefGoogle ScholarPubMed
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: 16.10.1016/j.ijcard.2015.05.070CrossRefGoogle ScholarPubMed
Moons, P, Van Deyk, K, De Bleser, L, et al. Quality of life and health status in adults with congenital heart disease: a direct comparison with healthy counterparts. Eur J Cardiovasc Prev Rehabil 2006; 13: 407413.10.1097/01.hjr.0000221864.19415.a0CrossRefGoogle ScholarPubMed
Kukreja, M, Bryant, AS, Cleveland, DC, Dabal, R, Hingorani, N, Kirklin, JK. Health-related quality of life in adult survivors after the Fontan operation. Semin Thorac Cardiovasc Surg 2015; 27: 299306.10.1053/j.semtcvs.2015.08.007CrossRefGoogle ScholarPubMed
Holbein, CE, Fogleman, ND, Hommel, K, et al. A multinational observational investigation of illness perceptions and quality of life among patients with a Fontan circulation. Congenit Heart Dis 2018; 13: 392400.CrossRefGoogle ScholarPubMed
Smith, KW, Avis, NE, Assmann, SF. Distinguishing between quality of life and health status in quality of life research: a meta-analysis. Qual Life Res 1999; 8: 447459.10.1023/A:1008928518577CrossRefGoogle ScholarPubMed
Moons, P, Luyckx, K. Quality-of-life research in adult patients with congenital heart disease: current status and the way forward. Acta Paediatr 2019; 108: 17651772.10.1111/apa.14876CrossRefGoogle ScholarPubMed
Gill, TM, Feinstein, AR. A critical appraisal of the quality of quality-of-life measurements. JAMA 1994; 272: 619626.CrossRefGoogle ScholarPubMed
Helm, PC, Kempert, S, Körten, MA, Lesch, W, Specht, K, Bauer, UMM. Congenital heart disease patients’ and parents’ perception of disease-specific knowledge: health and impairments in everyday life. Congenit Heart Dis 2018; 13: 377383.10.1111/chd.12581CrossRefGoogle Scholar
Hickey, A, Barker, M, McGee, H, O’Boyle, C. Measuring health-related quality of life in older patient populations: a review of current approaches. Pharmacoeconomics 2005; 23: 971993.CrossRefGoogle ScholarPubMed
Holbein, CE, Veldtman, GR, Moons, P, et al. Perceived health mediates effects of physical activity on quality of life in patients with a Fontan circulation. Am J Cardiol 2019; 124: 144150.10.1016/j.amjcard.2019.03.039CrossRefGoogle ScholarPubMed
Moons, P, Luyckx, K. Quality-of-life research in adult patients with congenital heart disease: current status and the way forward. Acta Paediatr 2019; 108: 17651772.CrossRefGoogle ScholarPubMed
Fogleman, ND, Apers, S, Moons, P, et al. Regional variation in quality of life in patients with a Fontan circulation: a multinational perspective. Am Heart J 2017; 193: 5562.10.1016/j.ahj.2017.07.019CrossRefGoogle ScholarPubMed
Eaton, SL, Wang, Q, Menahem, S. Determinants of quality of life in adults with CHD: an Australian cohort. Cardiol Young 2017; 27: 15711576.CrossRefGoogle Scholar
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: 22372245.10.1016/j.jacc.2016.03.477CrossRefGoogle ScholarPubMed
Pike, NA, Evangelista, LS, Doering, LV, Koniak-Griffin, D, Lewis, AB, Child, JS. Clinical profile of the adolescent/adult Fontan survivor. Congenit Heart Dis 2011; 6: 917.CrossRefGoogle ScholarPubMed
Bordin, G, Padalino, MA, Perentaler, S, et al. Clinical profile and quality of life of adult patients after the Fontan procedure. Pediatr Cardiol 2015; 36: 12611269.10.1007/s00246-015-1156-yCrossRefGoogle ScholarPubMed
Dulfer, K, Bossers, SS, Utens, EM, et al. Does functional health status predict health-related quality of life in children after Fontan operation? Cardiol Young 2016; 26: 459468.10.1017/S1047951115000426CrossRefGoogle ScholarPubMed
Müller, J, Christov, F, Schreiber, C, Hess, J, Hager, A. Exercise capacity, quality of life, and daily activity in the long-term follow-up of patients with univentricular heart and total cavopulmonary connection. Eur Heart J 2009; 30: 29152920.10.1093/eurheartj/ehp305CrossRefGoogle ScholarPubMed
Lambert, LM, Minich, LL, Newburger, JW, et al. Parent- versus child-reported functional health status after the Fontan procedure. Pediatrics 2009; 124: e942e949.10.1542/peds.2008-1697CrossRefGoogle ScholarPubMed
Gratz, A, Hess, J, Hager, A. Self-estimated physical functioning poorly predicts actual exercise capacity in adolescents and adults with congenital heart disease. Eur Heart J 2009; 30: 497504.10.1093/eurheartj/ehn531CrossRefGoogle ScholarPubMed
Atz, AM, Travison, TG, McCrindle, BW, et al. Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection. Cardiol Young 2013; 23: 335343.10.1017/S1047951112001175CrossRefGoogle ScholarPubMed
Amedro, P, Gavotto, A, Legendre, A, et al. Impact of a centre and home-based cardiac rehabilitation program on the quality of life of teenagers and young adults with congenital heart disease: the QUALI-REHAB study rationale, design and methods. Int J Cardiol 2019; 283: 112118.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: 175182.10.1111/chd.12330CrossRefGoogle ScholarPubMed
McCrindle, BW, Williams, RV, Mitchell, PD, et al. Relationship of patient and medical characteristics to health status in children and adolescents after the Fontan procedure. Circulation 2006; 113: 11231129.10.1161/CIRCULATIONAHA.105.576660CrossRefGoogle ScholarPubMed
Idorn, L, Jensen, AS, Juul, K, et al. Quality of life and cognitive function in Fontan patients, a population-based study. Int J Cardiol 2013; 168: 32303235.CrossRefGoogle ScholarPubMed
Berghammer, MC, Brink, E, Rydberg, AM, Dellborg, M, Ekman, I. Committed to life: adolescents’ and young adults’ experiences of living with Fontan circulation. Congenit Heart Dis 2015; 10: 403412.10.1111/chd.12244CrossRefGoogle Scholar
Van den Bosch, AE, Roos-Hesselink, JW, Van Domburg, R, Bogers, AJ, Simoons, ML, Meijboom, FJ. Long-term outcome and quality of life in adult patients after the Fontan operation. Am J Cardiol 2004; 93: 11411145.10.1016/j.amjcard.2004.01.041CrossRefGoogle ScholarPubMed
Latal, B, Helfricht, S, Fischer, JE, Bauersfeld, U, Landolt, MA. Psychological adjustment and quality of life in children and adolescents following open-heart surgery for congenital heart disease: a systematic review. BMC Pediatr 2009; 9: 616.10.1186/1471-2431-9-6CrossRefGoogle ScholarPubMed
Moons, P, Norekvål, TM. Is sense of coherence a pathway for improving the quality of life of patients who grow up with chronic diseases? A hypothesis. Eur J Cardiovasc Nurs 2006; 5: 1620.10.1016/j.ejcnurse.2005.10.009CrossRefGoogle ScholarPubMed
Rozenblum, R, Gianola, A, Ionescu-Ittu, R, et al. Clinicians’ perspectives on patient satisfaction in adult congenital heart disease clinics – a dimension of health care quality whose time has come. Congenit Heart Dis 2015; 10: 128136.10.1111/chd.12190CrossRefGoogle ScholarPubMed
Reiner, B, Oberhoffer, R, Ewert, P, Müller, J. Quality of life in young people with congenital heart disease is better than expected. Arch Dis Child 2019; 104: 124128.10.1136/archdischild-2017-314211CrossRefGoogle ScholarPubMed
Rometsch, S, Greutmann, M, Latal, B, et al. Predictors of quality of life in young adults with congenital heart disease. Eur Heart J Qual Care Clin Outcomes 2019; 5: 161168.10.1093/ehjqcco/qcy046CrossRefGoogle ScholarPubMed
Popelová, J, Slavík, Z, Skovránek, J. Are cyanosed adults with congenital cardiac malformations depressed? Cardiol Young 2001; 11: 379384.10.1017/S1047951101000488CrossRefGoogle 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: 804809.CrossRefGoogle ScholarPubMed
Kamphuis, M, Vogels, T, Ottenkamp, J, Van Der Wall, EE, Verloove-Vanhorick, SP, Vliegen, HW. Employment in adults with congenital heart disease. Arch Pediat Adolesc Med 2002; 156: 11431148.10.1001/archpedi.156.11.1143CrossRefGoogle ScholarPubMed
Xiang, L, Su, Z, Liu, Y, et al. Impact of family socioeconomic status on health-related quality of life in children with critical congenital heart disease. J Am Heart Assoc 2019; 8: e010616.CrossRefGoogle ScholarPubMed