open access
What determines the quality of life of adult patients after Fontan procedure?
open access
Abstract
Background: Despite the low early mortality of Fontan procedures, Fontan patients are prone to various cardiac and extra-cardiac complications in the long term. This may influence patient perception of their health and outcome. The aim of the study was to assess the relationship of multi-organ complications and physical efficiency with self-reported health-related quality of life (QOL) in adult Fontan patients.
Methods: Quality of life was assessed with the Short Form-36 questionnaire. Laboratory tests were done together with echocardiography, plethysmography, and cardiopulmonary exercise test.
Results: The QOL was poorer in patients than in control subjects. The physical characteristics of patients correlated with dynamic ventilatory parameters, heart rate at the peak of exercise, alanine aminotransferase and albumin level.
Conclusions: Liver impairment and chronotropic incompetence during exercise are associated with poor QOL in patients after Fontan procedure. In these patient, hepatic, pulmonary and cardiac functions should be carefully monitored. (Cardiol J 2018; 25, 1: 72–80)
Abstract
Background: Despite the low early mortality of Fontan procedures, Fontan patients are prone to various cardiac and extra-cardiac complications in the long term. This may influence patient perception of their health and outcome. The aim of the study was to assess the relationship of multi-organ complications and physical efficiency with self-reported health-related quality of life (QOL) in adult Fontan patients.
Methods: Quality of life was assessed with the Short Form-36 questionnaire. Laboratory tests were done together with echocardiography, plethysmography, and cardiopulmonary exercise test.
Results: The QOL was poorer in patients than in control subjects. The physical characteristics of patients correlated with dynamic ventilatory parameters, heart rate at the peak of exercise, alanine aminotransferase and albumin level.
Conclusions: Liver impairment and chronotropic incompetence during exercise are associated with poor QOL in patients after Fontan procedure. In these patient, hepatic, pulmonary and cardiac functions should be carefully monitored. (Cardiol J 2018; 25, 1: 72–80)
Keywords
adult Fontan patients, quality of life, SF-36, exercise tolerance, pulmonary function test, multiorgan complications
About this articleTitle
What determines the quality of life of adult patients after Fontan procedure?
Journal
Issue
Pages
72-80
Published online
2017-07-10
Page views
1980
Article views/downloads
1798
DOI
Pubmed
Bibliographic record
Cardiol J 2018;25(1):72-80.
Keywords
adult Fontan patients
quality of life
SF-36
exercise tolerance
pulmonary function test
multiorgan complications
Authors
Monika Smaś-Suska
Natalia Dłużniewska
Piotr Weryński
Jacek Pająk
Wojciech Płazak
Maria Olszowska
Piotr Podolec
Lidia Tomkiewicz-Pająk
References (40)- Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971; 26(3): 240–248.
- Loup O, von Weissenfluh C, Gahl B, et al. Quality of life of grown-up congenital heart disease patients after congenital cardiac surgery. Eur J Cardiothorac Surg. 2009; 36(1): 105–11; discussion 111.
- Somerville J. Management of adults with congenital heart disease: an increasing problem. Annu Rev Med. 1997; 48: 283–293.
- Webb GD. Care of adults with congenital heart disease--a challenge for the new millennium. Thorac Cardiovasc Surg. 2001; 49(1): 30–34.
- Rychik J, Goldberg DJ. Late consequences of the Fontan operation. Circulation. 2014; 130(17): 1525–1528.
- de Leval MR, Deanfield JE. Four decades of Fontan palliation. Nat Rev Cardiol. 2010; 7(9): 520–527.
- Schumacher KR, Stringer KA, Donohue JE, et al. Fontan-associated protein-losing enteropathy and plastic bronchitis. J Pediatr. 2015; 166(4): 970–977.
- Tomkiewicz-Pająk L, Hoffman P, Trojnarska O, et al. Cardiac surgery Long-term follow-up in adult patients after Fontan operations. Pol J Cardio-Thorac Surg. 2013; 4: 357–363.
- Tomkiewicz-Pająk L, Lelakowski J, Pająk J, et al. Ablation of arrhythmias in adult patients after Fontan operation. Pol Arch Med Wewn. 2013; 123(12): 723–725.
- Dluzniewska N, Knap K, Tomkiewicz-Pająk L, et al. Cardiopulmonary exercise tests in rare cardiovascular heart diseases. J Rare Cardiovasc Dis. 2015; 2(5).
- Marino BS, Tomlinson RS, Drotar D, et al. Quality-of-life concerns differ among patients, parents, and medical providers in children and adolescents with congenital and acquired heart disease. Pediatrics. 2009; 123(4): e708–e715.
- Moons P, Van Deyk K, De Geest S, et al. Is the severity of congenital heart disease associated with the quality of life and perceived health of adult patients? Heart. 2005; 91(9): 1193–1198.
- 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(6): 1261–1269.
- Moons P. Patient-reported outcomes in congenital cardiac disease: are they as good as you think they are? Cardiol Young. 2010; 20 (Suppl 3): 143–148.
- Wanger J, Clausen JL, Coates A, et al. Standardisation of the measurement of lung volumes. Eur Respir J. 2005; 26(3): 511–522.
- Tylka J, Piotrowicz R. [Quality of life questionnaire SF-36 -- Polish version]. Kardiol Pol. 2009; 67(10): 1166–1169.
- Drakouli M, Petsios K, Giannakopoulou M, et al. Determinants of quality of life in children and adolescents with CHD: a systematic review. Cardiol Young. 2015; 25(6): 1027–1036.
- Areias MEG, Pinto CI, Vieira PF, et al. Living with CHD: quality of life (QOL) in early adult life. Cardiol Young. 2014; 24 (Suppl 2): 60–65.
- Gewillig M, Brown SC, Eyskens B, et al. The Fontan circulation: who controls cardiac output? Interact Cardiovasc Thorac Surg. 2010; 10(3): 428–433.
- Takken T, Tacken MHP, Blank AC, et al. Exercise limitation in patients with Fontan circulation: a review. J Cardiovasc Med (Hagerstown). 2007; 8(10): 775–781.
- Khairy P, Poirier N, Mercier LA. Univentricular heart. Circulation. 2007; 115(6): 800–812.
- Zajac A, Tomkiewicz L, Podolec P, et al. Cardiorespiratory response to exercise in children after modified fontan operation. Scand Cardiovasc J. 2002; 36(2): 80–85.
- Fredriksen PM, Therrien J, Veldtman G, et al. Lung function and aerobic capacity in adult patients following modified Fontan procedure. Heart. 2001; 85(3): 295–299.
- Heying R, Seghaye MC, Grabitz RG, et al. Mid-term follow-up after multiple system organ failure following cardiac surgery in children. Acta Paediatr. 1999; 88(11): 1238–1243.
- Cohen SB, Ginde S, Bartz PJ, et al. Extracardiac complications in adults with congenital heart disease. Congenit Heart Dis. 2013; 8(5): 370–380.
- Tomkiewicz-Pajak L, Olszowska M, Komnata K, et al. Lung function and exercise tolerance in adults after Fontan procedure. Exp Clin Cardiol. 2014; 20(1): 2606–2614.
- Winkelmann ER, Chiappa GR, Lima COC, et al. Addition of inspiratory muscle training to aerobic training improves cardiorespiratory responses to exercise in patients with heart failure and inspiratory muscle weakness. Am Heart J. 2009; 158(5): 768.e1–768.e7.
- Mancini DM, Henson D, La Manca J, et al. Benefit of selective respiratory muscle training on exercise capacity in patients with chronic congestive heart failure. Circulation. 1995; 91(2): 320–329.
- Cordina RL, O'Meagher S, Karmali A, et al. Resistance training improves cardiac output, exercise capacity and tolerance to positive airway pressure in Fontan physiology. Int J Cardiol. 2013; 168(2): 780–788.
- Greutmann M, Le TL, Tobler D, et al. Generalised muscle weakness in young adults with congenital heart disease. Heart. 2011; 97(14): 1164–1168.
- Stein R, Chiappa GR, Güths H, et al. Inspiratory muscle training improves oxygen uptake efficiency slope in patients with chronic heart failure. J Cardiopulm Rehabil Prev. 2009; 29(6): 392–395.
- Oka T, Kato R, Fumino S, et al. Noninvasive estimation of central venous pressure after Fontan procedure using biochemical markers and abdominal echography. J Thorac Cardiovasc Surg. 2013; 146(1): 153–157.
- Bouliaris K, Christodoulidis G, Symeonidis D, et al. Damage control surgery for hepatocellular cancer rupture in an elderly patient: survival and quality of life. Case Rep Emerg Med. 2015; 2015: 536029.
- Alkatheri A, Al Bekairy A, Aburuz S, et al. Exploring quality of life among renal and liver transplant recipients. Ann Saudi Med. 2015; 35(5): 368–376.
- Fournier E, Jooste V, Woronoff AS, et al. Health-related quality of life is a prognostic factor for survival in older patients after colorectal cancer diagnosis: A population-based study. Dig Liver Dis. 2016; 48(1): 87–93.
- Polis S, Fernandez R. Impact of physical and psychological factors on health-related quality of life in adult patients with liver cirrhosis: a systematic review protocol. JBI Database System Rev Implement Rep. 2015; 13(1): 39–51.
- Silvilairat S, Cabalka AK, Cetta F, et al. Protein-losing enteropathy after the Fontan operation: associations and predictors of clinical outcome. Congenit Heart Dis. 2008; 3(4): 262–268.
- Mertens L, Hagler DJ, Sauer U, et al. Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg. 1998; 115(5): 1063–1073.
- Kukreja M, Bryant AS, Cleveland DC, et al. Health-Related quality of life in adult survivors after the Fontan operation. Semin Thorac Cardiovasc Surg. 2015; 27(3): 299–306.
- 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(6): 1261–1269.
