Abstract
The number of adult patients is continuously growing. However, although improving surgical and interventional techniques in childhood, a substantial number of patients carry persistent or residual lesions for the rest of their life. These lesions might predispose for late electrical, mechanical, or haemodynamic complications: arrhythmia, heart failure (HF), infective endocarditis, pulmonary hypertension, and re-interventions. Therefore, life-long follow-up is needed for most patients.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
References
Wren C, O’Sullivan JJ (2001) Survival with congenital heart disease and need for follow up in adult life. Heart 85:438–443
Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L (2007) Congenital heart disease in the general population: changing prevalence and age distribution. Circulation 115:163–172
Shiina Y, Toyoda T, Kawasoe Y, Tateno S, Shirai T, Wakisaka Y, Matsuo K, Mizuno Y, Terai M, Hamada H, Niwa K (2011) Prevalence of adult patients with congenital heart disease in Japan. Int J Cardiol 146:13–16
van der Bom T, Zomer AC, Zwinderman AH, Meijboom FJ, Bouma BJ, Mulder BJ (2011) The changing epidemiology of congenital heart disease. Nat Rev Cardiol 8:50–60
Baumgartner H, Budts W, Chessa M, Deanfield J, Eicken A, Holm J, Iserin L, Meijboom F, Stein J, Szatmari A, Trindade PT, Walker F, Cardiology WGoG-uCHDotESo (2014) Recommendations for organization of care for adults with congenital heart disease and for training in the subspecialty of ‘grown-up congenital heart disease’ in Europe: a position paper of the working group on grown-up congenital heart disease of the European society of cardiology. Eur Heart J 35:686–690
Walsh EP, Cecchin F (2007) Arrhythmias in adult patients with congenital heart disease. Circulation 115:534–545
van der Velde ET, Vander VE, Vriend JW, Mannens MM, Uiterwaal CS, Brand R, Mulder BJ (2005) Concor, an initiative towards a national registry and dna-bank of patients with congenital heart disease in the Netherlands: rationale, design, and first results. Eur J Epidemiol 20:549–557
Khairy P, Dore A, Talajic M, Dubuc M, Poirier N, Roy D, Mercier LA (2006) Arrhythmias in adult congenital heart disease. Expert Rev Cardiovasc Ther 4:83–95
Koyak Z, Harris L, de Groot JR, Silversides CK, Oechslin EN, Bouma BJ, Budts W, Zwinderman AH, Van Gelder IC, Mulder BJ (2012) Sudden cardiac death in adult congenital heart disease. Circulation 126:1944–1954
Khairy P, Aboulhosn J, Gurvitz MZ, Opotowsky AR, Mongeon FP, Kay J, Valente AM, Earing MG, Lui G, Gersony DR, Cook S, Ting JG, Nickolaus MJ, Webb G, Landzberg MJ, Broberg CS, (AARCC) AfARiCC (2010) Arrhythmia burden in adults with surgically repaired tetralogy of fallot: a multi-institutional study. Circulation 122:868–875
Diller GP, Kempny A, Liodakis E, Alonso-Gonzalez R, Inuzuka R, Uebing A, Orwat S, Dimopoulos K, Swan L, Li W, Gatzoulis MA, Baumgartner H (2012) Left ventricular longitudinal function predicts life-threatening ventricular arrhythmia and death in adults with repaired tetralogy of fallot. Circulation 125:2440–2446
Gelatt M, Hamilton RM, McCrindle BW, Connelly M, Davis A, Harris L, Gow RM, Williams WG, Trusler GA, Freedom RM (1997) Arrhythmia and mortality after the mustard procedure: a 30-year single-center experience. J Am Coll Cardiol 29:194–201
Rhodes LA, Wernovsky G, Keane JF, Mayer JE, Shuren A, Dindy C, Colan SD, Walsh EP (1995) Arrhythmias and intracardiac conduction after the arterial switch operation. J Thorac Cardiovasc Surg 109:303–310
van den Bosch AE, Roos-Hesselink JW, Van Domburg R, Bogers AJ, Simoons ML, Meijboom FJ (2004) Long-term outcome and quality of life in adult patients after the fontan operation. Am J Cardiol 93:1141–1145
Mavroudis C, Deal BJ, Backer CL, Johnsrude CL (1999) The favorable impact of arrhythmia surgery on total cavopulmonary artery fontan conversion. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2:143–156
Mavroudis C, Backer CL, Deal BJ, Johnsrude C, Strasburger J (2001) Total cavopulmonary conversion and maze procedure for patients with failure of the fontan operation. J Thorac Cardiovasc Surg 122:863–871
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
Van De Bruaene A, De Meester P, Buys R, Vanhees L, Delcroix M, Voigt JU, Budts W (2013) Right ventricular load and function during exercise in patients with open and closed atrial septal defect type secundum. Eur J Prev Cardiol 20:597–604
Van De Bruaene A, Delcroix M, Pasquet A, De Backer J, Paelinck B, Morissens M, Budts W (2011) The importance of pulmonary artery pressures on late atrial arrhythmia in transcatheter and surgically closed ASD type secundum. Int J Cardiol 152:192–195
Kantor PF, Redington AN (2010) Pathophysiology and management of heart failure in repaired congenital heart disease. Heart Fail Clin 6:497–506, ix
Verheugt CL, Uiterwaal CS, van der Velde ET, Meijboom FJ, Pieper PG, van Dijk AP, Vliegen HW, Grobbee DE, Mulder BJ (2010) Mortality in adult congenital heart disease. Eur Heart J 31:1220–1229
Oechslin EN, Harrison DA, Connelly MS, Webb GD, Siu SC (2000) Mode of death in adults with congenital heart disease. Am J Cardiol 86:1111–1116
Piran S, Veldtman G, Siu S, Webb GD, Liu PP (2002) Heart failure and ventricular dysfunction in patients with single or systemic right ventricles. Circulation 105:1189–1194
Wald RM, Marie Valente A, Marelli A (2015) Heart failure in adult congenital heart disease: emerging concepts with a focus on tetralogy of fallot. Trends Cardiovasc Med 25(5):422–432
Broberg CS, Aboulhosn J, Mongeon FP, Kay J, Valente AM, Khairy P, Earing MG, Opotowsky AR, Lui G, Gersony DR, Cook S, Ting JG, Webb G, Gurvitz MZ, (AARCC) AfARiCC (2011) Prevalence of left ventricular systolic dysfunction in adults with repaired tetralogy of fallot. Am J Cardiol 107:1215–1220
Cuypers JA, Eindhoven JA, Slager MA, Opić P, Utens EM, Helbing WA, Witsenburg M, van den Bosch AE, Ouhlous M, van Domburg RT, Rizopoulos D, Meijboom FJ, Bogers AJ, Roos-Hesselink JW (2014) The natural and unnatural history of the mustard procedure: long-term outcome up to 40 years. Eur Heart J 35:1666–1674
Gewillig M (2005) The fontan circulation. Heart 91:839–846
Deal BJ, Jacobs ML (2012) Management of the failing fontan circulation. Heart 98:1098–1104
Broberg CS, Prasad SK, Carr C, Babu-Narayan SV, Dimopoulos K, Gatzoulis MA (2014) Myocardial fibrosis in Eisenmenger syndrome: a descriptive cohort study exploring associations of late gadolinium enhancement with clinical status and survival. J Cardiovasc Magn Reson 16:32
Bhatt AB, Foster E, Kuehl K, Alpert J, Brabeck S, Crumb S, Davidson WR, Earing MG, Ghoshhajra BB, Karamlou T, Mital S, Ting J, Tseng ZH (2015) Congenital heart disease in the older adult: a scientific statement from the American heart association. Circulation 131(21):1884–1931
Morris CD, Reller MD, Menashe VD (1998) Thirty-year incidence of infective endocarditis after surgery for congenital heart defect. JAMA 279:599–603
Gersony WM, Hayes CJ, Driscoll DJ, Keane JF, Kidd L, O’Fallon WM, Pieroni DR, Wolfe RR, Weidman WH (1993) Bacterial endocarditis in patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect. Circulation 87:I121–I126
Tleyjeh IM, Steckelberg JM, Murad HS, Anavekar NS, Ghomrawi HM, Mirzoyev Z, Moustafa SE, Hoskin TL, Mandrekar JN, Wilson WR, Baddour LM (2005) Temporal trends in infective endocarditis: a population-based study in Olmsted county, Minnesota. JAMA 293:3022–3028
Van Dijck I, Budts W, Cools B, Eyskens B, Boshoff DE, Heying R, Frerich S, Vanagt WY, Troost E, Gewillig M (2015) Infective endocarditis of a transcatheter pulmonary valve in comparison with surgical implants. Heart 101:788–793
Thanavaro KL, Nixon JV (2014) Endocarditis 2014: an update. Heart Lung 43:334–337
Knirsch W, Nadal D (2011) Infective endocarditis in congenital heart disease. Eur J Pediatr 170:1111–1127
Di Filippo S, Delahaye F, Semiond B, Celard M, Henaine R, Ninet J, Sassolas F, Bozio A (2006) Current patterns of infective endocarditis in congenital heart disease. Heart 92:1490–1495
Wilson W, Taubert KA, Gewitz M, Lockhart PB, Baddour LM, Levison M, Bolger A, Cabell CH, Takahashi M, Baltimore RS, Newburger JW, Strom BL, Tani LY, Gerber M, Bonow RO, Pallasch T, Shulman ST, Rowley AH, Burns JC, Ferrieri P, Gardner T, Goff D, Durack DT, American Heart Association Rheumatic Fever Ed, and Kawasaki Disease Committee, Young AHACoCDit, Cardiology AHACoC, Anesthesia AHACoCSa, Group QoCaORIW (2007) Prevention of infective endocarditis: guidelines from the american heart association: a guideline from the american heart association rheumatic fever, endocarditis, and Kawasaki disease committee, council on cardiovascular disease in the young, and the council on clinical cardiology, council on cardiovascular surgery and anesthesia, and the quality of care and outcomes research interdisciplinary working group. Circulation 116:1736–1754
Day MD, Gauvreau K, Shulman S, Newburger JW (2009) Characteristics of children hospitalized with infective endocarditis. Circulation 119:865–870
Armstrong ML, DeBoer S, Cetta F (2008) Infective endocarditis after body art: a review of the literature and concerns. J Adolesc Health 43:217–225
Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, del Nido P, Fasules JW, Graham TP, Hijazi ZM, Hunt SA, King ME, Landzberg MJ, Miner PD, Radford MJ, Walsh EP, Webb GD (2008) Acc/aha 2008 guidelines for the management of adults with congenital heart disease: a report of the American college of cardiology/American heart association task force on practice guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation 118:e714–e833
Habib G, Hoen B, Tornos P, Thuny F, Prendergast B, Vilacosta I, Moreillon P, de Jesus Antunes M, Thilen U, Lekakis J, Lengyel M, Müller L, Naber CK, Nihoyannopoulos P, Moritz A, Zamorano JL, Guidelines ECfP (2009) Guidelines on the prevention, diagnosis, and treatment of infective endocarditis (new version 2009): the Task Force on the Prevention, Diagnosis, and Treatment of Infective Endocarditis of the European Society of Cardiology (ESC). Endorsed by the European Society of Clinical Microbiology and Infectious Diseases (ESCMID) and the International Society of Chemotherapy (ISC) for Infection and Cancer. Eur Heart J 30:2369–2413
Habib G, Lancellotti P, Antunes MJ, Bongiorni MG, Casalta JP, Del Zotti F, Dulgheru R, El Khoury G, Erba PA, Iung B, Miro JM, Mulder BJ, Plonska-Gosciniak E, Price S, Roos-Hesselink J, Snygg-Martin U, Thuny F, Tornos Mas P, Vilacosta I, Zamorano JL, Erol Ç, Nihoyannopoulos P, Aboyans V, Agewall S, Athanassopoulos G, Aytekin S, Benzer W, Bueno H, Broekhuizen L, Carerj S, Cosyns B, De Backer J, De Bonis M, Dimopoulos K, Donal E, Drexel H, Flachskampf FA, Hall R, Halvorsen S, Hoen B, Kirchhof P, Lainscak M, Leite-Moreira AF, Lip GY, Mestres CA, Piepoli MF, Punjabi PP, Rapezzi C, Rosenhek R, Siebens K, Tamargo J, Walker DM, Members ATF, Reviewers D (2015) 2015 ESC guidelines for the management of infective endocarditis: the Task Force for the Management of Infective Endocarditis of the European Society of Cardiology (ESC). Endorsed by: European Association for Cardio-thoracic Surgery (EACTS), the European Association of Nuclear Medicine (EANM). Eur Heart J 36:3075–3128
Baumgartner H, Bonhoeffer P, De Groot NM, de Haan F, Deanfield JE, Galie N, Gatzoulis MA, Gohlke-Baerwolf C, Kaemmerer H, Kilner P, Meijboom F, Mulder BJ, Oechslin E, Oliver JM, Serraf A, Szatmari A, Thaulow E, Vouhe PR, Walma E, (ESC) TFotMoG-uCHDotESoC, (AEPC) AfEPC, (CPG) ECfPG (2010) Esc guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 31:2915–2957
Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, Simonneau G, Peacock A, Vonk Noordegraaf A, Beghetti M, Ghofrani A, Gomez Sanchez MA, Hansmann G, Klepetko W, Lancellotti P, Matucci M, McDonagh T, Pierard LA, Trindade PT, Zompatori M, Hoeper M, Members: ATF, Members ATF (2016) 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 37(1):67–119
Barst RJ, McGoon M, Torbicki A, Sitbon O, Krowka MJ, Olschewski H, Gaine S (2004) Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol 43:40S–47S
Duffels MG, Engelfriet PM, Berger RM, van Loon RL, Hoendermis E, Vriend JW, van der Velde ET, Bresser P, Mulder BJ (2007) Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol 120:198–204
Diller GP, Gatzoulis MA (2007) Pulmonary vascular disease in adults with congenital heart disease. Circulation 115:1039–1050
Gabriels C, De Meester P, Pasquet A, De Backer J, Paelinck BP, Morissens M, Van De Bruaene A, Delcroix M, Budts W (2014) A different view on predictors of pulmonary hypertension in secundum atrial septal defect. Int J Cardiol 176:833–840
Schuuring MJ, Bouma BJ, Cordina R, Gatzoulis MA, Budts W, Mullen MP, Vis JC, Celermajer D, Mulder BJ (2013) Treatment of segmental pulmonary artery hypertension in adults with congenital heart disease. Int J Cardiol 164:106–110
Engelfriet PM, Duffels MG, Möller T, Boersma E, Tijssen JG, Thaulow E, Gatzoulis MA, Mulder BJ (2007) Pulmonary arterial hypertension in adults born with a heart septal defect: the euro heart survey on adult congenital heart disease. Heart 93:682–687
Voet A, Rega F, de Bruaene AV, Troost E, Gewillig M, Van Damme S, Budts W (2012) Long-term outcome after treatment of isolated pulmonary valve stenosis. Int J Cardiol 156:11–15
Jonas RA (2010) Aortic valve repair for congenital and balloon-induced aortic regurgitation. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 13:60–65
Troost E, Meyns B, Daenen W, Van de Werf F, Gewillig M, Van Deyk K, Moons P, Budts W (2007) Homograft survival after tetralogy of fallot repair: determinants of accelerated homograft degeneration. Eur Heart J 28:2503–2509
Meyns B, Jashari R, Gewillig M, Mertens L, Komárek A, Lesaffre E, Budts W, Daenen W (2005) Factors influencing the survival of cryopreserved homografts. The second homograft performs as well as the first. Eur J Cardiothorac Surg 28:211–216; discussion 216
Boshoff DE, Cools BL, Heying R, Troost E, Kefer J, Budts W, Gewillig M (2013) Off-label use of percutaneous pulmonary valved stents in the right ventricular outflow tract: time to rewrite the label? Catheter Cardiovasc Interv 81:987–995
Cools B, Brown SC, Heying R, Jansen K, Boshoff DE, Budts W, Gewillig M (2015) Percutaneous pulmonary valve implantation for free pulmonary regurgitation following conduit-free surgery of the right ventricular outflow tract. Int J Cardiol 186:129–135
Soufflet V, Daenen W, Van Deyk K, Troost E, Budts W (2005) Repair for partial and complete atrioventricular septal defect: single centre experience and long-term results. Acta Clin Belg 60:236–242
Fuller S (2014) Tetralogy of fallot and pulmonary valve replacement: timing and techniques in the asymptomatic patient. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 17:30–37
Khono K, Tamai A, Kobayashi T, Senzaki H (2011) Effects of stent implantation for peripheral pulmonary artery stenosis on pulmonary vascular hemodynamics and right ventricular function in a patient with repaired tetralogy of fallot. Heart Vessels 26:672–676
Troost E, Gewillig M, Daenen W, Meyns B, Bogaert J, Van Deyk K, Budts W (2009) Behaviour of polyester grafts in adult patients with repaired coarctation of the aorta. Eur Heart J 30:1136–1141
Luijendijk P, Bouma BJ, Groenink M, Boekholdt M, Hazekamp MG, Blom NA, Koolbergen DR, de Winter RJ, Mulder BJ (2012) Surgical versus percutaneous treatment of aortic coarctation: new standards in an era of transcatheter repair. Expert Rev Cardiovasc Ther 10:1517–1531
Patel S, Shah D, Chintala K, Karpawich PP (2011) Atrial baffle problems following the mustard operation in children and young adults with dextro-transposition of the great arteries: the need for improved clinical detection in the current era. Congenit Heart Dis 6:466–474
Berdat PA, Immer F, Pfammatter JP, Carrel T (2004) Reoperations in adults with congenital heart disease: analysis of early outcome. Int J Cardiol 93:239–245
Vida VL, Berggren H, Brawn WJ, Daenen W, Di Carlo D, Di Donato R, Lindberg HL, Corno AF, Fragata J, Elliott MJ, Hraska V, Kiraly L, Lacour-Gayet F, Maruszewski B, Rubay J, Sairanen H, Sarris G, Urban A, Van Doorn C, Ziemer G, Stellin G (2007) Risk of surgery for congenital heart disease in the adult: a multicentered European study. Ann Thorac Surg 83:161–168
Holst KA, Dearani JA, Burkhart HM, Connolly HM, Warnes CA, Li Z, Schaff HV (2011) Risk factors and early outcomes of multiple reoperations in adults with congenital heart disease. Ann Thorac Surg 92:122–128; discussion 129–130
Kirshbom PM, Myung RJ, Simsic JM, Kramer ZB, Leong T, Kogon BE, Kanter KR (2009) One thousand repeat sternotomies for congenital cardiac surgery: risk factors for reentry injury. Ann Thorac Surg 88:158–161
Jordan SC, Scott O (1989) Incidence, aetiology and recurrence of congenital heart disease. In: Jordan SC, Scott O (eds) Heart Disease in Paediatrics, 3rd ed. Butterworth Heniemann, Oxford, pp 3–9
Gomes-Neto M, Saquetto MB, da Silva E Silva CM, Conceição CS, Carvalho VO (2016) Impact of exercise training in aerobic capacity and pulmonary function in children and adolescents after congenital heart disease surgery: a systematic review with meta-analysis. Pediatr Cardiol 37(2):217–224
Lindeman JH, Ashcroft BA, Beenakker JW, van Es M, Koekkoek NB, Prins FA, Tielemans JF, Abdul-Hussien H, Bank RA, Oosterkamp TH (2010) Distinct defects in collagen microarchitecture underlie vessel-wall failure in advanced abdominal aneurysms and aneurysms in Marfan syndrome. Proc Natl Acad Sci U S A 107:862–865
Ostberg JE, Donald AE, Halcox JP, Storry C, McCarthy C, Conway GS (2005) Vasculopathy in turner syndrome: arterial dilatation and intimal thickening without endothelial dysfunction. J Clin Endocrinol Metab 90:5161–5166
Tandonnet J, Clavel J, Baruchel A, Nacka F, Pérel Y (2010) Myeloid leukaemia in children with down syndrome: report of the registry-based french experience between 1990 and 2003. Pediatr Blood Cancer 54:927–933
Derbent M, Öncel Y, Tokel K, Varan B, Haberal A, Yazici AC, Legius E, Özbek N (2010) Clinical and hematologic findings in Noonan syndrome patients with PTPN11 gene mutations. Am J Med Genet A 152A:2768–2774
Adachi I, Ogino H, Imanaka H, Matsuda H, Minatoya K, Sasaki H (2005) Aortic root replacement in a patient with pulmonary dysfunction caused by severe chest deformity associated with Marfan syndrome. J Thorac Cardiovasc Surg 130:213–215
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2016 Springer International Publishing Switzerland
About this chapter
Cite this chapter
Budts, W., Frogoudaki, A. (2016). Cardiac Issues in Adolescents with Congenital Heart Disease. In: Schwerzmann, M., Thomet, C., Moons, P. (eds) Congenital Heart Disease and Adolescence. Congenital Heart Disease in Adolescents and Adults. Springer, Cham. https://doi.org/10.1007/978-3-319-31139-5_2
Download citation
DOI: https://doi.org/10.1007/978-3-319-31139-5_2
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-31137-1
Online ISBN: 978-3-319-31139-5
eBook Packages: MedicineMedicine (R0)