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Research Article| Volume 168, ISSUE 4, P3230-3235, October 09, 2013

Quality of life and cognitive function in Fontan patients, a population-based study

      Abstract

      Background

      After the Fontan procedure patients are at risk for reduced quality of life (QoL) and cognitive function. We aimed to assess these important factors in Danish Fontan patients and to compare the results with a group of healthy controls.

      Methods

      All Fontan patients living in Denmark were identified and invited to participate. QoL was evaluated using the Pediatric Quality of Life Inventory (PedsQL) version 4.0 generic core module in patients <16 years and the Short Form 36 questionnaire (SF-36) in patients ≥16 years. Cognitive function was evaluated in all patients ≥6 years using the Quick Test of Cognitive Speed. To evaluate if QoL correlated with exercise capacity, patients performed a symptom-limited bicycle test.

      Results

      158 of 179 eligible patients (88%) consented to participate. Median age was 13.9 years (IQR: 10.2–19.3). PedsQL scores increased with age but were significantly lower among patients than among controls. SF-36 physical scores were significantly lower in patients compared to controls while psychosocial scores were similar. Cognitive speed was significantly reduced in patients at all ages compared to controls. No significant difference in PedsQL-/SF-36 scores or cognitive speed was found between hypoplastic left heart syndrome (HLHS) and non-HLHS Fontan patient. PedsQL-/SF-36 scores in patients ≥10 years correlated significantly to cognitive speed but not to peak exercise capacity.

      Conclusion

      QoL is reduced in Fontan children compared to their healthy counterparts whereas in patients ≥16 years only physical, but not psychosocial QoL is reduced. Cognitive speed was significantly lower in patients at all ages compared to controls.

      Keywords

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      References

        • Moons P.
        • Sluysmans T.
        • De W.D.
        • et al.
        Congenital heart disease in 111,225 births in Belgium: birth prevalence, treatment and survival in the 21st century.
        Acta Paediatr. March 2009; 98: 472-477
        • Idorn L.
        • Olsen M.
        • Jensen A.S.
        • et al.
        Univentricular hearts in Denmark 1977 to 2009: incidence and survival.
        Int J Cardiol. 2013 Aug 20; 167: 1311-1316
        • Fontan F.
        • Baudet E.
        Surgical repair of tricuspid atresia.
        Thorax. May 1971; 26: 240-248
        • Fixler D.E.
        • Nembhard W.N.
        • Salemi J.L.
        • Ethen M.K.
        • Canfield M.A.
        Mortality in first 5 years in infants with functional single ventricle born in Texas, 1996 to 2003.
        Circulation. February 9 2010; 121: 644-650
        • d'Udekem Y.
        • Iyengar A.J.
        • Cochrane A.D.
        • et al.
        The Fontan procedure: contemporary techniques have improved long-term outcomes.
        Circulation. September 11 2007; 116: I157-I164
        • Driscoll D.J.
        Long-term results of the Fontan operation.
        Pediatr Cardiol. November 2007; 28: 438-442
        • Pike N.A.
        • Evangelista L.S.
        • Doering L.V.
        • Eastwood J.A.
        • Lewis A.B.
        • Child J.S.
        Quality of life, health status, and depression: comparison between adolescents and adults after the Fontan procedure with healthy counterparts.
        J Cardiovasc Nurs. 2011; 27: 539-546
        • d'Udekem Y.
        • Cheung M.M.
        • Setyapranata S.
        • et al.
        How good is a good Fontan? Quality of life and exercise capacity of Fontans without arrhythmias.
        Ann Thorac Surg. December 2009; 88: 1961-1969
        • Marino B.S.
        • 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. May 2008; 17: 613-626
        • McCrindle B.W.
        • Williams R.V.
        • Mitchell P.D.
        • et al.
        Relationship of patient and medical characteristics to health status in children and adolescents after the Fontan procedure.
        Circulation. February 28 2006; 113: 1123-1129
        • Ferry P.C.
        Neurologic sequelae of open-heart surgery in children. An ‘irritating question’.
        Am J Dis Child. March 1990; 144: 369-373
        • Donofrio M.T.
        • Bremer Y.A.
        • Schieken R.M.
        • et al.
        Autoregulation of cerebral blood flow in fetuses with congenital heart disease: the brain sparing effect.
        Pediatr Cardiol. September 2003; 24: 436-443
        • Kaltman J.R.
        • Di H.
        • Tian Z.
        • Rychik J.
        Impact of congenital heart disease on cerebrovascular blood flow dynamics in the fetus.
        Ultrasound Obstet Gynecol. January 2005; 25: 32-36
        • Goldberg C.
        Neurocognitive outcomes for children with functional single ventricle malformations.
        Pediatr Cardiol. November 2007; 28: 443-447
        • Varni J.W.
        • Burwinkle T.M.
        • Seid M.
        • Skarr D.
        The PedsQL 4.0 as a pediatric population health measure: feasibility, reliability, and validity.
        Ambul Pediatr. November 2003; 3: 329-341
        • Varni J.W.
        PedsQLTM translation methodology.
        • Varni J.W.
        • Seid M.
        • Kurtin P.S.
        PedsQL 4.0: reliability and validity of the Pediatric Quality of Life Inventory version 4.0 generic core scales in healthy and patient populations.
        Med Care. August 2001; 39: 800-812
        • McHorney C.A.
        • Ware Jr., J.E.
        • Raczek A.E.
        The MOS 36-Item Short-Form Health Survey (SF-36): II. Psychometric and clinical tests of validity in measuring physical and mental health constructs.
        Med Care. 1993 March; 31: 247-263
        • Overgaard D.
        • Schrader A.M.
        • Lisby K.H.
        • et al.
        Patient-reported outcomes in adult survivors with single-ventricle physiology.
        Cardiology. 2011; 120: 36-42
        • Wiig E.H.
        • Nielsen N.P.
        • Minthon L.
        • Warkentin S.
        A quick test of cognitive speed.
        Harcourt/Psych Corp., San Antonio, TX2002
        • Nielsen N.P.
        • Ringstrom R.
        • Wiig E.H.
        • Minthon L.
        Associations between AQT processing speed and neuropsychological tests in neuropsychiatric patients.
        Am J Alzheimers Dis Other Demen. 2007 June; 22: 202-210
        • Nielsen N.P.
        • Wiig E.H.
        AQT cognitive speed and processing efficiency differentiate adults with and without ADHD: a preliminary study.
        Int J Psychiatry Clin Pract. September 2011; 15: 219-227
        • Wiig E.H.
        • Zureich P.
        • Chan H.N.
        A clinical rationale for assessing rapid automatized naming in children with language disorders.
        J Learn Disabil. July 2000; 33: 359-374
        • Georgiou G.K.
        • Papadopoulos T.C.
        • Fella A.
        • Parrila R.
        Rapid naming speed components and reading development in a consistent orthography.
        J Exp Child Psychol. May 2012; 112: 1-17
        • Jacobson J.M.
        • Nielsen N.P.
        • Minthon L.
        • Warkentin S.
        • Wiig E.H.
        Multiple rapid automatic naming measures of cognition: normal performance and effects of aging.
        Percept Mot Skills. June 2004; 98: 739-753
        • Lang C.C.
        • Karlin P.
        • Haythe J.
        • Tsao L.
        • Mancini D.M.
        Ease of noninvasive measurement of cardiac output coupled with peak VO2 determination at rest and during exercise in patients with heart failure.
        Am J Cardiol. February 1 2007; 99: 404-405
        • Cooper D.M.
        • Weiler-Ravell D.
        Gas exchange response to exercise in children.
        Am Rev Respir Dis. February 1984; 129: S47-S48
        • Cooper C.B.
        • Storer T.W.
        Exercise testing and interpretation; a practical approach.
        University Press, Cambridge, UK2001: 278
        • Driscoll D.J.
        • Offord K.P.
        • Feldt R.H.
        • Schaff H.V.
        • Puga F.J.
        • Danielson G.K.
        Five- to fifteen-year follow-up after Fontan operation.
        Circulation. February 1992; 85: 469-496
        • Gentles T.L.
        • Mayer Jr., J.E.
        • Gauvreau K.
        • et al.
        Fontan operation in five hundred consecutive patients: factors influencing early and late outcome.
        J Thorac Cardiovasc Surg. September 1997; 114: 376-391
        • McCrindle B.W.
        • Zak V.
        • Sleeper L.A.
        • et al.
        Laboratory measures of exercise capacity and ventricular characteristics and function are weakly associated with functional health status after Fontan procedure.
        Circulation. January 5 2010; 121: 34-42
        • Muller 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. December 2009; 30: 2915-2920
        • Latal B.
        • Helfricht S.
        • Fischer J.E.
        • Bauersfeld U.
        • Landolt M.A.
        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: 6
        • Moons P.
        • Van D.K.
        • Budts W.
        • De G.S.
        Caliber of quality-of-life assessments in congenital heart disease: a plea for more conceptual and methodological rigor.
        Arch Pediatr Adolesc Med. November 2004; 158: 1062-1069
        • Rose M.
        • Kohler K.
        • Kohler F.
        • Sawitzky B.
        • Fliege H.
        • Klapp B.F.
        Determinants of the quality of life of patients with congenital heart disease.
        Qual Life Res. February 2005; 14: 35-43
        • Wallander J.
        • Thompson R.
        • Alriksson-Schmidt A.
        Psychosocial adjustment of children with chronic physical conditions.
        in: Roberts M. Handbook of pediatric psychology. New York, NY, Guilford Press2003: 772
        • Moons P.
        • Van D.K.
        • De G.S.
        • Gewillig M.
        • Budts W.
        Is the severity of congenital heart disease associated with the quality of life and perceived health of adult patients?.
        Heart. September 2005; 91: 1193-1198
        • Saliba Z.
        • Butera G.
        • Bonnet D.
        • et al.
        Quality of life and perceived health status in surviving adults with univentricular heart.
        Heart. July 2001; 86: 69-73
        • Diener E.
        • Emmons R.A.
        • Larsen R.J.
        • Griffin S.
        The satisfaction with life scale.
        J Pers Assess. February 1985; 49: 71-75
        • Goldberg C.S.
        • Schwartz E.M.
        • Brunberg J.A.
        • et al.
        Neurodevelopmental outcome of patients after the Fontan operation: a comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions.
        J Pediatr. November 2000; 137: 646-652
        • Wernovsky G.
        • Stiles K.M.
        • Gauvreau K.
        • et al.
        Cognitive development after the Fontan operation.
        Circulation. August 22 2000; 102: 883-889
        • Aisenberg R.B.
        • Rosenthal A.
        • Nadas A.S.
        • Wolff P.H.
        Developmental delay in infants with congenital heart disease. Correlation with hypoxemia and congestive heart failure.
        Pediatr Cardiol. 1982; 3: 133-137
        • Chun D.S.
        • Schamberger M.S.
        • Flaspohler T.
        • et al.
        Incidence, outcome, and risk factors for stroke after the Fontan procedure.
        Am J Cardiol. January 1 2004; 93: 117-119
        • Giardini A.
        • Hager A.
        • Pace N.C.
        • Picchio F.M.
        Natural history of exercise capacity after the Fontan operation: a longitudinal study.
        Ann Thorac Surg. March 2008; 85: 818-821