Abstract
The number of grown-up patients with congenital heart disease (GUCH) is constantly increasing and will equal the number of children requiring surgery for congenital heart disease (CHD). Specialized centers dealing with the medical and paramedical problems of these patients are required. GUCH patients can be divided into the following groups: (1) patients with minor cardiac malformations presenting at adult age for first treatment; (2) patients presenting for correction as adults because they are either naturally balanced or were surgically palliated; (3) patients presenting for expected reoperations after correction in childhood; (4) patients requiring repair of residual defects after correction; (5) patients developing heart failure after correction or palliation of CHD requiring thoracic transplantation; and (6) patients developing acquired heart disease in addition to CHD. Special aspects of malformations frequently occurring in GUCH patients are discussed in detail. Acquired heart disease in this patient population is expected to increase in the coming decades as survivors of CHD grow old.
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Backer CL, Deal BJ, Mavroudis C, Franklin WH, Stewart RD (2006) Conversion of the failed Fontan circulation. Cardiol Young 16(Suppl 1):85–91
Bailey LL, Wood M, Razzouk A, Van Arsdell G, Gundry S (1989) Heart transplantation during the first 12 years of life. Loma Linda University Pediatric Heart Transplant Group. Arch Surg 124:1221–1226
Berdat PA, Immer F, Pfammatter JP, Carrel T (2004) Reoperations in adults with congenital heart disease: analysis of early outcome. Int J Cardiol 93(2–3):239–245
Bernaccio C, Carotti A, D’Argeniio P, Michielson G, Parisi F (2003) Protein-losing enteropathy after Fontan surgery: resolution after cardiac transplantation. J Heart Lung Transplant 22:484–486
Bolger AP, Gatzoulis MA (2004) Towards defining heart failure in adults with congenital heart disease. Int J Cardiol 97(Suppl 1): 15–23
Budts W, Scheurwegs C, Stevens A, et al. (2006) The future of adult patients after Mustard or Senning repair for transposition of the great arteries. Int J Cardiol [Epub ahead of print] 113(2):209–214
Chessa M, Carrozza M, Butera G, et al. (2005) Results and mid-long-term follow-up of stent implantation for native and recurrent coarctation of the aorta. Eur Heart J 26:2728–2732
Chessa M, Cullen S, Deanfield J, et al. (2004) The care of adult patients with congenital heart defects: a new challenge. Ital Heart J 5:178–182
Craig RJ, Selzer A (1968) Natural history and prognosis of atrial septal defect. Circulation 37:805–815
Crossland DS, Jackson SP, Lyall R, Burn J, Sullivan JJ (2005) Employment and advice regarding careers for adults with congenital heart disease. Cardiol Young 15:391–395
Däbritz S, Sachweh JS, Walter M, Messmer BJ (1999) Closure of atrial septal defects via limited right anterolateral thoracotomy as a minimally invasive approach in female patients. Eur J Cardiothorac Surg 15:18–23
Daebritz SH, Nollert GD, Zurakowski D, et al. (2000) Results of Norwood stage I operation: comparison of hypoplastic left heart syndrome with other malformations. J Thorac Cardiovasc Surg 119:358–367
Daebritz SH, Tiete AR, Sachweh JS, et al. (2001) Systemic right ventricular failure after atrial switch operation: midterm results of conversion into an arterial switch. Ann Thorac Surg 71:1225–1259
Daenen W, Lacour-Gayet F, Aberg T, et al. (2003) EACTS Congenital Heart Disease Committee Optimal structure of a congenital heart surgery department in Europe. Eur J Cardiothorac Surg 24:343–351
Deal BJ, Mavroudis C, Backer CL (2003) Beyond Fontan conversion: surgical therapy of arrhythmias including patients with associated complex congenital heart disease. Ann Thorac Surg 76:542–554
Deanfield J, Thaulow E, Warnes C, et al. (2003) Task Force on-the Management of Grown Up Congenital Heart Disease, European Society of Cardiology; ESC Committee for Practice Guidelines. Management of grown up congenital heart disease. Eur Heart J 24:1035–1084
de Leval M (1998) The Fontan circulation: what have we learned? What to expect. Pediatr Cardiol 19:316–320
Diller GP, Dimopoulos K, Okonko D, et al. (2005) Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation 112:828–835
Dore A, Glancy DL, Stone S, Menashe VD, Somerville J (1997) Cardiac surgery for grown-up congenital heart patients: survey of 307 consecutive operations from 1991 to 1994. Am J Cardiol 80:906–913
Dos L, Teruel L, Ferreira IJ, et al. (2005) Late outcome of Senning and Mustard procedures for correction of transposition of the great arteries. Heart 91:652–656
Doty DB, Renlund DG, Caputo GR, Burton NA, Jones KW (1990) Cardiac transplantation in situs inversus. J Thorac Cardiovasc Surg 99:493–499
Engelfriet P, Boersma E, Oechslin E, et al. (2005) The spectrum of adult congenital heart disease in Europe: morbidity and mortality in a 5 year follow-up period. The Euro Heart Survey on Adult Congenital Heart Disease. Eur Heart J 26:2325–2333
Fontan F, Mounicot FB, Baudet E, et al. (1971) “Correction” of tricuspid atresia. 2 cases “corrected” using a new surgical technic. Ann Chir Thorac Cardiovasc 10:39–47
Fredriksen PM, Therrien J, Veldtmann G, et al. (2001) Lung function and aerobic capacity in adult patients following modified Fontan procedure. Heart 85:295–299
Frigiola A, Giamberti A, Chessa M, et al. (2006) the RESTORE group. Right ventricular restoration during pulmonary valve implantation in adults with congenital heart disease. Eur J Cardiothorac Surg 29(Suppl 1):S279–S285
Gamba A, Merlo M, Fiocchi R, et al. (2004) Heart transplantation in patients with previous Fontan operations. J Thorac Cardiovasc Surg 127:555–562
Gates RN, Laks H, Drinkwater DC, et al. (1997) The Fontan procedure in adults. Ann Thorac Surg 63:1085–1090
Gatzoulis MA, Balaji S, Webber SA, et al. (2000) Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet 356:975–981
Gatzoulis MA, Munk MD, Williams WG, Webb GD (2000) Definitive palliation with cavopulmonary or aortopulmonary shunts for adults with single ventricle physiology. Heart 83:51–57
Gelatt M, Hamilton RM, McCrindle BW, et al. (1997) Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience.J Am Coll Cardiol 29:194–201
Gentles TL, Gauvreau K, Mayer JE Jr, et al. (1997) Functional outcome after the Fontan operation: factors influencing late morbidity. J Thorac Cardiovasc Surg 114:392–405
Graham TP Jr, Bernard YD, Mellen BG, et al. (2000) Long-term outcome in congenitally corrected transposition of the great arteries. A multi-institutional study. J Am Coll Cardiol 36:255–261
Groetzner J, Reichart B, Roemer U, et al. (2005) Cardiac transplantation in pediatric patients: fifteen-year experience of a single center. Ann Thorac Surg 79:53–61
Hannoush H, Tamim H, Younes H, et al. (2004) Patterns of congenital heart disease in unoperated adults: a 20-year experience in a developing country. Clin Cardiol 27:236–240
Hasan AUJ, Hamilton JRL, Hunter S, Hilton CJ, Dark JH (1993) orthotopic heart transplantation for congenital heart disease: technical considerations. Eur J Cardiothorac Surg 7:65–70
Hong YM, Moller JH (1993) Ebstein’s anomaly: a long-term study of survival. Am Heart J 125:1419–1424
Hsu DT, Quaegebeur JM, Michler RE, et al. (1995) Heart transplantation in children with congenital heart disease. J Am Coll Cardiol 26:743–749
Humes RA, Mair DD, Porter CJ, et al. (1988) Results of the modified Fontan operation in adults. Am J Cardiol 1:602–604
Imai Y (1997) Double-switch operation for congenitally corrected transposition. Adv Card Surg 9:65–86
Immer FF, Althaus SM, Berdat PA, Saner H, Carrel TP (2005) Quality of life and specific problems after cardiac surgery in adolescents and adults with congenital heart diseases. Eur J Cardiovasc Prev Rehabil 12:138–143
Iserin L, Chua TP, Chambers J, Coats AJ, Somerville J (1997) Dyspnoea and exercise intolerance during cardiopulmonary exercise testing in patients with univentricular heart. The effects of chronic hypoxaemia and Fontan procedure. Eur Heart J 18:1350–1356
ISHLT (2005) J Heart Lung Transpl 24:945–982
Jacobs ML, Pelletier G (2006) Late complications associated with the Fontan circulation. Cardiol Young 16 (Suppl 1):80–84
Jatene AD, Fontes VF, Paulista PP, et al. (1975) Successful anatomic correction of transposition of the great vessels. A preliminary report. Arg Bras Cardiol 28:461–464
Jayakumar KA, Addonizio LJ (2004) Cardiac transplantation after the Fontan or Glenn procedure. J Am Coll Cardiol 44(10):2065–2072
Knirsch W, Haas NA, Uhlemann F, Dietz K, Lange PE (2005) Clinical course and complications of infective endocarditis in patients growing up with congenital heart disease. Int J Cardiol 101:285–291
Laks H, Marelli D (2001) Heart transplantation in the young and elderly. Heart Failure. Rev 6:221–226
Lamour JM, Addonizio LJ, Galantowicz ME, et al. (1999) Outcome after orthotopic cardiac transplantation in adults with congenital heart disease. Circulation 100 (19 Suppl):II200–II205
Li W, Sarubbi B, Sutton R, et al. (2001) Atrial and ventricular electromechanical function in 1-ventricle hearts: influence of atrial flutter and Fontan procedure. J Am Soc Echocardiogr 14:186–193
Li W, Somerville J (2000) Atrial flutter in grown-up congenital heart (GUCH) patients. Clinical characteristics of affected population. Int J Cardiol 75:129–139
Li W, Somerville J, Gibson DG, Henein MY (2002) Effect of atrial flutter on exercise tolerance in patients with grown-up congenital heart (GUCH). Am Heart J 144:173–179
Liang T, XiangJun Z, XiaoJing M, Yun L, Leng CY (2006) New minimally invasive technique to occlude secundum atrial septal defect in 53 patients. Ann Thorac Surg 81:1417–1419
Lubiszewska B, Rozanski J, Szufladowicz M, et al. (1999) Mechanical valve replacement in congenital heart disease in children. J Heart Valve Dis 8:74–79
MacMahon B, McKeown T, Record RG (1953) The incidence and life expectation of children with congenital heart disease. Br Heart J 15:121
Marcelletti CF, Hanley FL, Mavroudis C, et al. (2000) Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: a multicenter experience. J Thorac Cardiovasc Surg 119:340–346
Marelli D, Laks H, Kobashigawa JA, et al. (2002) Seventeen-year experience with 1083 heart transplants at a single institution. Ann Thorac Surg 74:1558–1567
Mattila S, Merikallio E, Tala P (1979) ASD in patients over 40 years of age. Scand J Thorac Cardiovasc Surg 13:21–24
Mavroudis C, Deal BJ, Backer CL (2002) The beneficial effects of total cavopulmonary conversion and arrhythmia surgery for the failed Fontan. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 5:12–24
Mayer JE Jr, Perry S, O’Brien P, et al. (1990) Orthotopic heart transplantation for complex congenital heart disease. J Thorac Cardiovasc Surg 99:484–492
Mee RBB (1986). Severe right ventricular failure after Mustard or Senning operation. Two-stage repair: pulmonary artery banding and switch. J Thorac Cardiovasc Surg 92:385–390
Mertens C, Canter C, Parisi F (1999) The outcome of heart transplantation for protein-losing enteropathy after Fontan operation. Circulation 100 (Suppl 1):1602
Mertens L, Hagler DJ, Sauer U, Somerville J, Gewillig M (1998) Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg 115:1063–1073
Michielson G, Parisi F, Squiteri C, et al. (2003) Orthotopic heart transplantation for congenital heart disease: an alternative for high risk Fontan Candidates? Circulation Suppl 1:II-140
Mitchell MB, Campbell DN, Boucek MM (2004) Heart transplantation for the failing Fontan circulation. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 7:56–64
Moran AM, Daebritz S, Keane JF, Mayer JE (2000) Surgical management of mitral regurgitation after repair of endocardial cushion defects: early and midterm results. Circulation 102(19 Suppl 3):III160–III165
Mott AR, Feltes TF, McKenzie ED, et al. (2004) Improved early results with the Fontan operation in adults with functional single ventricle. Ann Thorac Surg 77:1334–1340
Nollert G, Fischlein T, Bouterwek S, et al. (1997) Long-term survival in patients with repair of tetralogy of fallot: 36-year follow-up of 490 survivors of the first year after surgical repair. J Am Coll Cardiol 30:1374–1383
Nollert GD, Däbritz SH, Schmoekel M, Vicol C, Reichart B (2003) Risk factors for sudden death after repair of tetralogy of Fallot. Ann Thorac Surg 76:1901–1905
Norwood WI, Lang P, Hansen DD (1983) Physiologic repair of aortic atresia–hypoplastic left heart syndrome. N Engl J Med 308:23–26
Petko M, Myung RJ, Wernovsky G, et al. (2003) Surgical reinterventions following the Fontan procedure. Eur J Cardiothorac Surg 24:255–259
Pigula FA, Gandhi SK, Stukus D, et al. (2001) Cardiopulmonary transplantation for congenital heart disease in adult. J Heart Lung Transplant 20:297–303
Rammohan M, Airan B, Bhan A, et al. (1998) Total correction of tetralogy of Fallot in adults—surgical experience. Int J Cardiol 63:121–128
Report of the British Cardiac Society Working Party (2002) Grown-up congenital heart (GUCH) disease: current needs and provision of service for adolescents and adults with congenital heart disease in the UK. Heart 88(Suppl 1):il–i14
Reyes A 2nd, Bove EL, Mosca RS, Kulik TJ, Ludomirsky A (1997) Tricuspid valve repair in children with hypoplastic left heart syndrome during staged surgical reconstruction. Circulation 96 (9 Suppl):II-341–II-345
Rhodes LA, Walsh EP, Gamble WJ, Triedman JK, Saul JP (2003) Benefits and potential risks of atrial antitachycardia pacing after repair of congenital heart disease. Eur J Cardiothorac Surg 24:341–342
Scarso P, Volpe B, Melendugno A, Daliento L (2003) Psychological problems and cognitive impairments in the GUCH community. Ital Heart J Suppl 4:705–711
Schreiber C, Bleiziffer S, Kostolny M, et al. (2005) Minimally invasive midaxillary muscle sparing thoracotomy for atrial septal defect closure in prepubescent patients. Ann Thorac Surg 80:673–676
Senning A (1959) Surgical correction of transposition of the great vessels. Surgery 45:966–980
Shibata Y, Abe T, Kuribayashi R, et al. (1996) Surgical treatment of isolated secundum atrial septal defect in patients more than 50 years old. Ann Thorac Surg 62:1096–1099
Shrinathan SK, Bonser RS, Sthia B, et al. (2005) Changing practice of cardiac surgery in adult patients with congenital heart disease. Heart 91:207–212
Somerville J (1997) Management of adults with congenital heart disease: an increasing problem. Annu Rev Med 48:283–293
Somerville J (1998) The Denolin Lecture: the woman with congenital heart disease. Eur Heart J 19:1766–1775
Somerville J (2001) Grown-up congenital heart disease—medical demands look back, look forward 2000. Thorac Cardiovasc Surg 49:21–26
Srinatlian SK, Bonser RS, Sethia B, et al. (2005) Changing practice of cardiac surgery in adult patients with congenital heart disease. Heart 91:207–212
Stamm C, Friehs I, Mayer JE Jr, et al. (2001) Long-term results of the lateral tunnel Fontan operation. J Thorac Cardiovasc Surg 121:28–41
Stellin G, Vida MA, Padalino MA, Rizzoli G (2004) Surgical outcome for congenital heart malformations in the adult age: a multicenter European study. Semin Thorac Cardiovasc Surg 7:95–101
Swan L, Hillis WS (2000) Exercise prescription in adults with congenital deart disease: a long way to go. Heart 83:685–687
Thilen U, (2005) GUCH—grown-up congenital heart disease—adults with congenital heart defects. The number of adults with congenital heart defects is increasing with the developments in pediatric cardiology. Lakartidningen 102:2304–2306, 2308
Tiete AR, Sachweh JS, Kozlik-Feldmann R, et al. (2002) Minimally invasive surgery for congenital heart defects in paediatric patients. Thorac Cardiovasc Surg 50: 271–275
Toro-Salazar OH, Steinberger J, Thomas W, et al. (2002) Long-term follow-up of patients after coarctation of the aorta repair. Am J Cardiol 89:541–547
Triedman JK (2002) Arrhythmias in adults with congenital heart disease. Heart 87:383–389
Turina M, Siebermann R, Nussbaumer P, Senning A (1988) Long term outlook after atrial correction of transposition of the great arteries. J Thorac Cardiovasc Surg 95:828–835
van Son JA, Danielson GK, Huhta JC, et al. (1995) Late results of systemic atrioventricular valve replacement in corrected transposition. J Thorac Cardiovasc Surg 109:642–653
Walker F, Webb G (2001) Congenital coronary artery anomalies: the adults perspective. Coron Artery Dis 12: 599–604
Warner KG, O’Brien PK, Rhodes J (2003) Expanding the indications for pulmonary valve replacement after repair of tetralogy of Fallot. Ann Thorac Surg 76:1066–1071
Warnes CA (2005) The adult with congenital heart disease: born to be bad? J Am Coll Cardiol 46:1–8
Webb GD (2000) Care of adults with congenital heart disease—a challenge for the new millennium. Thorac Cardiovasc Surg 49:30–34
Wren C, O’Sullivan JJ (2001) Survival with congenital heart disease and need for follow-up in adult life. Heart 85:438–443
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Daebritz, S.H. Update in Adult Congenital Cardiac Surgery. Pediatr Cardiol 28, 96–104 (2007). https://doi.org/10.1007/s00246-006-1446-5
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DOI: https://doi.org/10.1007/s00246-006-1446-5