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Quality of life in children with pulmonary atresia and intact ventricular septum

  • Britt-Marie Ekman-Joelsson (a1), Leeni Berntsson (a2) and Jan Sunnegårdh (a3)


Objective: To measure quality of life in children with pulmonary atresia and intact ventricular septum, and make comparisons with a healthy group of children from the general Swedish population. An additional aim was to compare quality of life in two subgroups, children undergoing biventricular and univentricular repair, the latter by establishment of the Fontan circulation. Methods: Quality of life was measured using a mailed questionnaire, taking into consideration the three spheres of personal, interpersonal, and external living conditions. In all, 52 children received the questionnaire, and the answers were compared with those of a random sample of 1856 healthy Swedish children. Results: We received responses from 42 children and parents. The overall differences between groups were small, and no difference was found when comparing the children undergoing univentricular as opposed to biventricular surgical repair. In terms of personal quality of life, we found a higher level of psychosomatic complaints, and lower satisfaction with their own and parent–child activities, in those with the congenital anomaly than in their normal peers. In terms of the interpersonal sphere, more time was available for those with malformed hearts, but the experience of support from the relatives was significantly lower. Conclusions: Overall, quality of life was equal between children with pulmonary atresia and intact ventricular septum and the children in the reference group. The personal psychological conditions were less favourable for children with pulmonary atresia and intact ventricular septum. The perceived lack of support from relatives was also higher in these families. No difference was found when comparing the children undergoing univentricular and biventricular surgical repair.


Corresponding author

Correspondence to: Britt-Marie Ekman-Joelsson, Paediatric and Adolescent Medical Care, Skaraborg Hospital, 541 85 Skövde, Sweden. Tel: +46 500 431000; Fax: +46 500 432299; E-mail:


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Ekman-Joelsson BM, Sunnegårdh J, Hanséus K, et al. The outcome of children born with pulmonary atresia and intact ventricular septum in Sweden from 1980 to 1999. Scand Cardiovasc J 2001; 35: 192198.
Berntsson L, Köhler L. Quality of life among children aged 2–17 years in the five Nordic countries. Comparison between 1984 and 1996. Eur J Public Health 2001; 11: 437445.
Lindström B, Eriksson B. Quality of life among children in the Nordic countries. Quality of Life Research 1993; 2: 2332.
Pal DK. Quality of life assessment in children: a review of conceptual and methodological issues in multidimensional health status measures. J Epidemiol Community Health 1996; 50: 391396.
Raphael D. Determinants of health of North American adolescents: evolving definitions, recent findings, and proposed research agenda. J Adolesc Health 1996; 19: 616.
Bredow TS, Peterson SJ. Health-related quality of life. In: Peterson SJ, Bredow TS (eds). Middle Range Theories: Application to Nursing Research. Lippincott Williams and Wilkins, Philadelphia, PA, 2004, pp 274287.
Ferrans CE. Development of a conceptual model of quality of life. In: Ferrans CD (ed.). Clarifying Concepts in Nursing Research. Springer Publishing Company, New York, 1997, pp 110121.
Möyen Laane K. Quality of life in children with congenital heart defects. Master of Science in Public Health. NHV-report 2000: 1. Nordic School of Public Health, Göteborg.
Culbert EL, Ashburn DA, Cullen-Dean G, et al. Congenital Heart Surgeons Society. Quality of life of children after repair of transposition of the great arteries. Circulation 2003; 108: 857862.
Casey FA, Craig BG, Mulholland HC. Quality of life in surgically palliated complex congenital heart disease. Arch Dis Child 1994; 70: 382386.
Dunbar-Masterson C, Wypij D, Bellinger DC, et al. General health status of children with D-transposition of the great arteries after the arterial switch operation. Circulation 2001; 104 (12 Suppl 1): 11381142.
Casey FA, Sykes DH, Craig BG, Powe R, Mulholland HC. Behavioral adjustment of children with surgically palliated complex congenital heart disease. J Pediatr Psychol 1996; 21: 335352.
Favarato ME, Romano BW. Heart surgery in childhood. Impact on the quality of life of adolescents. Arq Bras Cardiol 1994; 62: 171174.
Mair DD, Puga FJ, Danielsson GK. The Fontan procedure for tricuspid atresia: early and late results of a 25-year experience with 216 patients. J Am Coll Cardiol 2001; 37: 933939.
Gentles TL, Gauvreau K, Mayer J, et al. Functional outcome after Fontan operation: factors influencing late morbidity. J Thorac Cardiovasc Surg 1997; 114: 392403.
Mahle WT, Clancy RR, Moss EM, Gerdes M, Jobe, Wernovsky G. Neurodevelopmental outcome and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome. Pediatrics 2000; 105: 10821090.
Mair DD, Julsrud PR, Puga FJ, Danielsson GK. The Fontan procedure for pulmonary atresia with intact ventricular septum: operative and late results. J Am Coll Cardiol 1997; 29: 13591364.
Gentles TL, Mayer JE, Gauvreau K, et al. Fontan operation in five hundred consecutive patients: factors influencing early and late outcome. J Thorac Cardiovasc Surg 1997; 114: 376391.
Wallander J, Varni J, Babani L, Banis, H, Wilcox, K. Family resources as resistance for psychological maladjustment in chronically ill and handicapped children. J Pediatr Psychol 1989; 14: 157173.
Köhler L. Children with and without disabilities in the Nordic countries. A Nordic project. Scand J Soc Med 1993; 21: 146149.
Berntson LT, Gustafsson J-E. Determinants of psychosomatic complaints in Swedish schoolchildren aged seven to twelve years. Scand J Public Health 2000; 28: 283293.
Saliba Z, Bustera G, Bonnet D, et al. Quality of life and perceived health status in surviving adults with univentricular hearts. Heart 2001; 86: 6973.
Ternestedt BM, Wall K, Oddsson H, Reisenfeld T, Groth I, Schollin J. Quality of life 20 and 30 years after surgery in patients operated on for tetralogy of Fallot and for atrial septal defect. Pediatr Cardiol 2001; 22: 128132.
Bromberg JI, Beasly PJ, D'angelo EJ, Landzberg M, Demaso BR. Depression and anxiety in adults with congenital heart disease. A pilot study. Heart Lung 2003; 32: 105110.
The WHOQOL Group, 1994. Quality of Life Assessment. What Quality of Life? In: World Health Forum. WHO, Geneva, 1996.



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