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Annual Review of Medicine

Volume 75, 2024

Complex Congenital Heart Disease in the Adult

Abstract

Congenital heart disease (CHD), a heterogeneous group of structural abnormalities of the cardiovascular system, is the most frequent cause of severe birth defects. Related to improved pediatric outcomes, there are now more adults living with CHD, including complex lesions, than children. Adults with CHD are at high risk for complications related to their underlying anatomy and past surgical palliative interventions. Adults with CHD require close monitoring and proactive management strategies to improve outcomes.

Keyword(s): complications, congenital heart disease, outcomes
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/content/journals/10.1146/annurev-med-050922-052324
2024-01-29
2024-05-01
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Literature Cited

  1. 1.
    van der Linde D, Konings EE, Slager MA et al. 2011. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J. Am. Coll. Cardiol. 58:2241–47
     [Google Scholar]
  2. 2.
    Tutarel O, Kempny A, Alonso-Gonzalez R et al. 2014. Congenital heart disease beyond the age of 60: emergence of a new population with high resource utilization, high morbidity, and high mortality. Eur. Heart J. 35:725–32
     [Google Scholar]
  3. 3.
    Benjamin EJ, Blaha MJ, Chiuve SE et al. 2017. Heart disease and stroke statistics—2017 update: a report from the American Heart Association. Circulation 135:e146–603
     [Google Scholar]
  4. 4.
    Khairy P, Ionescu-Ittu R, Mackie AS et al. 2010. Changing mortality in congenital heart disease. J. Am. Coll. Cardiol. 56:1149–57
     [Google Scholar]
  5. 5.
    Goldstein SA, D'Ottavio A, Spears T et al. 2020. Causes of death and cardiovascular comorbidities in adults with congenital heart disease. J. Am. Heart Assoc. 9:e016400
     [Google Scholar]
  6. 6.
    Marelli AJ, Mackie AS, Ionescu-Ittu R et al. 2007. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation 115:163–72
     [Google Scholar]
  7. 7.
    Marelli AJ, Ionescu-Ittu R, Mackie AS et al. 2014. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation 130:749–56
     [Google Scholar]
  8. 8.
    Stout KK, Daniels CJ, Aboulhosn JA et al. 2019. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation 139:e698–800
     [Google Scholar]
  9. 9.
    Apitz C, Webb GD, Redington AN. 2009. Tetralogy of Fallot. Lancet 374:1462–71
     [Google Scholar]
  10. 10.
    Lillehei CW, Varco RL, Cohen M et al. 1986. The first open heart corrections of tetralogy of Fallot. A 26–31 year follow-up of 106 patients. Ann. Surg. 204:490–502
     [Google Scholar]
  11. 11.
    Smith CA, McCracken C, Thomas AS et al. 2019. Long-term outcomes of tetralogy of Fallot: a study from the Pediatric Cardiac Care Consortium. JAMA Cardiol. 4:34–41
     [Google Scholar]
  12. 12.
    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 356:975–81
     [Google Scholar]
  13. 13.
    Khairy P, Van Hare GF, Balaji S et al. 2014. PACES/HRS expert consensus statement on the recognition and management of arrhythmias in adult congenital heart disease: developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS), and the International Society for Adult Congenital Heart Disease (ISACHD). Can. J. Cardiol. 30:e1–63
     [Google Scholar]
  14. 14.
    Hoffman JI, Kaplan S. 2002. The incidence of congenital heart disease. J. Am. Coll. Cardiol. 39:1890–900
     [Google Scholar]
  15. 15.
    Senning Å. 1959. Surgical correction of transposition of the great vessels. Surgery 45:966–80
     [Google Scholar]
  16. 16.
    Mustard WT, Keith JD, Trusler GA et al. 1964. The surgical management of transposition of the great vessels. J. Thorac. Cardiovasc. Surg. 48:953–58
     [Google Scholar]
  17. 17.
    Jatene AD, Fontes VF, Paulista P et al. 1976. Anatomic correction of transposition of the great vessels. J. Thorac. Cardiovasc. Surg. 72:364–70
     [Google Scholar]
  18. 18.
    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
     [Google Scholar]
  19. 19.
    Raissadati A, Nieminen H, Sairanen H, Jokinen E. 2017. Outcomes after the Mustard, Senning and arterial switch operation for treatment of transposition of the great arteries in Finland: a nationwide 4-decade perspective. Eur. J. Cardiothorac. Surg. 52:573–80
     [Google Scholar]
  20. 20.
    Cuypers JA, Eindhoven JA, Slager MA et al. 2014. The natural and unnatural history of the Mustard procedure: long-term outcome up to 40 years. Eur. Heart J. 35:1666–74
     [Google Scholar]
  21. 21.
    Vejlstrup N, Sørensen K, Mattsson E et al. 2015. Long-term outcome of Mustard/Senning correction for transposition of the great arteries in Sweden and Denmark. Circulation 132:633–38
     [Google Scholar]
  22. 22.
    Warnes CA. 2006. Transposition of the great arteries. Circulation 114:2699–709
     [Google Scholar]
  23. 23.
    Baumgartner H, De Backer J, Babu-Narayan SV et al. 2020. 2020 ESC guidelines for the management of adult congenital heart disease. Eur. Heart J. 42:563–645
     [Google Scholar]
  24. 24.
    Kammeraad JA, van Deurzen CH, Sreeram N et al. 2004. Predictors of sudden cardiac death after Mustard or Senning repair for transposition of the great arteries. J. Am. Coll. Cardiol. 44:1095–102
     [Google Scholar]
  25. 25.
    Al-Khatib SM, Stevenson WG, Ackerman MJ et al. 2018. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation 138:e210–71
     [Google Scholar]
  26. 26.
    Bonnet D, Bonhoeffer P, Piéchaud JF et al. 1996. Long-term fate of the coronary arteries after the arterial switch operation in newborns with transposition of the great arteries. Heart 76:274–79
     [Google Scholar]
  27. 27.
    Legendre A, Losay J, Touchot-Koné A et al. 2003. Coronary events after arterial switch operation for transposition of the great arteries. Circulation 108:Suppl. 1II186–90
     [Google Scholar]
  28. 28.
    Hornung TS, Calder L. 2010. Congenitally corrected transposition of the great arteries. Heart 96:1154–61
     [Google Scholar]
  29. 29.
    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. 36:255–61
     [Google Scholar]
  30. 30.
    Hornung TS, Bernard EJ, Jaeggi ET et al. 1998. Myocardial perfusion defects and associated systemic ventricular dysfunction in congenitally corrected transposition of the great arteries. Heart 80:322–26
     [Google Scholar]
  31. 31.
    Hauser M, Bengel FM, Hager A et al. 2003. Impaired myocardial blood flow and coronary flow reserve of the anatomical right systemic ventricle in patients with congenitally corrected transposition of the great arteries. Heart 89:1231–35
     [Google Scholar]
  32. 32.
    Sabbah BN, Arabi TZ, Shafqat A et al. 2022. Heart failure in systemic right ventricle: mechanisms and therapeutic options. Front. Cardiovasc. Med. 9:1064196
     [Google Scholar]
  33. 33.
    Scherptong RW, Vliegen HW, Winter MM et al. 2009. Tricuspid valve surgery in adults with a dysfunctional systemic right ventricle: repair or replace?. Circulation 119:1467–72
     [Google Scholar]
  34. 34.
    van Dissel AC, Winter MM, van der Bom T et al. 2019. Long-term clinical outcomes of valsartan in patients with a systemic right ventricle: follow-up of a multicenter randomized controlled trial. Int. J. Cardiol. 278:84–87
     [Google Scholar]
  35. 35.
    Zandstra TE, Nederend M, Jongbloed MRM et al. 2021. Sacubitril/valsartan in the treatment of systemic right ventricular failure. Heart 107:1725–30
     [Google Scholar]
  36. 36.
    Fontan F, Baudet E. 1971. Surgical repair of tricuspid atresia. Thorax 26:240–48
     [Google Scholar]
  37. 37.
    Giannakoulas G, Dimopoulos K, Yuksel S et al. 2012. Atrial tachyarrhythmias late after Fontan operation are related to increase in mortality and hospitalization. Int. J. Cardiol. 157:221–26
     [Google Scholar]
  38. 38.
    Stephenson EA, Lu M, Berul CI et al. 2010. Arrhythmias in a contemporary Fontan cohort: prevalence and clinical associations in a multicenter cross-sectional study. J. Am. Coll. Cardiol. 56:890–96
     [Google Scholar]
  39. 39.
    d'Udekem Y, Iyengar AJ, Cochrane AD et al. 2007. The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation 116:I–157I-64
     [Google Scholar]
  40. 40.
    Nürnberg JH, Ovroutski S, Alexi-Meskishvili V et al. 2004. New onset arrhythmias after the extracardiac conduit Fontan operation compared with the intraatrial lateral tunnel procedure: early and midterm results. Ann. Thorac. Surg. 78:1979–88
     [Google Scholar]
  41. 41.
    Ovroutski S, Dähnert I, Alexi-Meskishvili V et al. 2001. Preliminary analysis of arrhythmias after the Fontan operation with extracardiac conduit compared with intra-atrial lateral tunnel. Thorac. Cardiovasc. Surg. 49:334–37
     [Google Scholar]
  42. 42.
    Collins KK. 2009. The spectrum of long-term electrophysiologic abnormalities in patients with univentricular hearts. Congenit. Heart Dis. 4:310–17
     [Google Scholar]
  43. 43.
    Ghaferi AA, Hutchins GM. 2005. Progression of liver pathology in patients undergoing the Fontan procedure: chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma. J. Thorac. Cardiovasc. Surg. 129:1348–52
     [Google Scholar]
  44. 44.
    Wu FM, Ukomadu C, Odze RD et al. 2011. Liver disease in the patient with Fontan circulation. Congenit. Heart Dis. 6:190–201
     [Google Scholar]
  45. 45.
    Kogiso T, Sagawa T, Taniai M et al. 2022. Risk factors for Fontan-associated hepatocellular carcinoma. PLOS ONE 17:e0270230
     [Google Scholar]
  46. 46.
    Possner M, Gordon-Walker T, Egbe AC et al. 2021. Hepatocellular carcinoma and the Fontan circulation: clinical presentation and outcomes. Int. J. Cardiol. 322:142–48
     [Google Scholar]
  47. 47.
    Mertens L, Hagler DJ, Sauer U et al. 1998. Protein-losing enteropathy after the Fontan operation: an international multicenter study. J. Thorac. Cardiovasc. Surg. 115:1063–73
     [Google Scholar]
  48. 48.
    Van den Eynde J, Possner M, Alahdab F et al. 2023. Thromboprophylaxis in patients with Fontan circulation. J. Am. Coll. Cardiol. 81:374–89
     [Google Scholar]
  49. 49.
    Assaad IE, Pastor T, O'Leary E et al. 2021. Atrial pacing in Fontan patients: the effect of transvenous lead on clot burden. Heart Rhythm 18:1860–67
     [Google Scholar]
  50. 50.
    John AS, Driscoll DJ, Warnes CA et al. 2011. The use of oral budesonide in adolescents and adults with protein-losing enteropathy after the Fontan operation. Ann. Thorac. Surg. 92:1451–56
     [Google Scholar]
  51. 51.
    Brancaccio G, Carotti A, D'Argenio P et al. 2003. Protein-losing enteropathy after Fontan surgery: resolution after cardiac transplantation. J. Heart Lung Transplant. 22:484–86
     [Google Scholar]
  52. 52.
    Davies RR, Sorabella RA, Yang J et al. 2012. Outcomes after transplantation for “failed” Fontan: a single-institution experience. J. Thorac. Cardiovasc. Surg. 143:1183–92.e4
     [Google Scholar]
  53. 53.
    Goldberg DJ, French B, McBride MG et al. 2011. Impact of oral sildenafil on exercise performance in children and young adults after the Fontan operation: a randomized, double-blind, placebo-controlled, crossover trial. Circulation 123:1185–93
     [Google Scholar]
  54. 54.
    Hebert A, Mikkelsen UR, Thilen U et al. 2014. Bosentan improves exercise capacity in adolescents and adults after Fontan operation: the TEMPO (Treatment With Endothelin Receptor Antagonist in Fontan Patients, a Randomized, Placebo-Controlled, Double-Blind Study Measuring Peak Oxygen Consumption) study. Circulation 130:2021–30
     [Google Scholar]
  55. 55.
    Eisenmenger V. 1897. Die angeborenen defekte der kammerscheidewand des herzens. Z. Klin. Med. 32:1–28
     [Google Scholar]
  56. 56.
    Wood P. 1958. The Eisenmenger syndrome. Br. Med. J. 2:755–62
     [Google Scholar]
  57. 57.
    Hurdman J, Condliffe R, Elliot CA et al. 2012. ASPIRE registry: assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre. Eur. Respir. J. 39:945–55
     [Google Scholar]
  58. 58.
    Barst RJ, Ivy DD, Foreman AJ et al. 2014. Four- and seven-year outcomes of patients with congenital heart disease–associated pulmonary arterial hypertension (from the REVEAL Registry). Am. J. Cardiol. 113:147–55
     [Google Scholar]
  59. 59.
    Diller GP, Körten MA, Bauer UM et al. 2016. Current therapy and outcome of Eisenmenger syndrome: data of the German National Register for congenital heart defects. Eur. Heart J. 37:1449–55
     [Google Scholar]
  60. 60.
    Arvanitaki A, Gatzoulis MA, Opotowsky AR et al. 2022. Eisenmenger syndrome: JACC state-of-the-art review. J. Am. Coll. Cardiol. 79:1183–98
     [Google Scholar]
  61. 61.
    Diller G-P, Dimopoulos K, Broberg CS et al. 2006. Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case–control study. Eur. Heart J. 27:1737–42
     [Google Scholar]
  62. 62.
    Takeshita M, Kagawa M, Yato S et al. 1997. Current treatment of brain abscess in patients with congenital cyanotic heart disease. Neurosurgery 41:1270–78
     [Google Scholar]
  63. 63.
    Hjortshøj CMS, Kempny A, Jensen AS et al. 2017. Past and current cause-specific mortality in Eisenmenger syndrome. Eur. Heart J. 38:2060–67
     [Google Scholar]
  64. 64.
    Broberg CS, Ujita M, Prasad S et al. 2007. Pulmonary arterial thrombosis in Eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity. J. Am. Coll. Cardiol. 50:634–42
     [Google Scholar]
  65. 65.
    Dimopoulos K, Inuzuka R, Goletto S et al. 2010. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation 121:20–25
     [Google Scholar]
  66. 66.
    He B, Zhang F, Li X et al. 2010. Meta-analysis of randomized controlled trials on treatment of pulmonary arterial hypertension. Circ. J. 74:1458–64
     [Google Scholar]
  67. 67.
    Ammash N, Warnes CA. 1996. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J. Am. Coll. Cardiol. 28:768–72
     [Google Scholar]
  68. 68.
    Freisinger E, Gerß J, Makowski L et al. 2020. Current use and safety of novel oral anticoagulants in adults with congenital heart disease: results of a nationwide analysis including more than 44,000 patients. Eur. Heart J. 41:4168–77
     [Google Scholar]
  69. 69.
    Pizula J, Devera J, Ng TMH et al. 2022. Outcome of pregnancy in women with D-transposition of the great arteries: a systematic review. J. Am. Heart Assoc. 11:e026862
     [Google Scholar]
  70. 70.
    Bowater SE, Selman TJ, Hudsmith LE et al. 2013. Long-term outcome following pregnancy in women with a systemic right ventricle: is the deterioration due to pregnancy or a consequence of time?. Congenit. Heart Dis. 8:302–7
     [Google Scholar]
  71. 71.
    Cataldo S, Doohan M, Rice K et al. 2016. Pregnancy following Mustard or Senning correction of transposition of the great arteries: a retrospective study. BJOG Int. J. Obstet. Gynaecol. 123:807–13
     [Google Scholar]
  72. 72.
    Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs J et al. 2018. 2018 ESC guidelines for the management of cardiovascular diseases during pregnancy: the Task Force for the Management of Cardiovascular Diseases During Pregnancy of the European Society of Cardiology (ESC). Eur. Heart J. 39:3165–241
     [Google Scholar]
  73. 73.
    Garcia Ropero A, Baskar S, Roos Hesselink JW et al. 2018. Pregnancy in women with a Fontan circulation: a systematic review of the literature. Circ. Cardiovasc. Qual. Outcomes 11:e004575
     [Google Scholar]
  74. 74.
    Gouton M, Nizard J, Patel M et al. 2015. Maternal and fetal outcomes of pregnancy with Fontan circulation: a multicentric observational study. Int. J. Cardiol. 187:84–89
     [Google Scholar]
  75. 75.
    Putman R, Goldstein SA, Boyd B et al. 2022. Sonographic evaluation of the placenta in Fontan patients: a case series. J. Ultrasound Med. 42:1361–65
     [Google Scholar]
  76. 76.
    Duan R, Xu X, Wang X et al. 2016. Pregnancy outcome in women with Eisenmenger's syndrome: a case series from west China. BMC Pregnancy Childbirth 16:356
     [Google Scholar]
  77. 77.
    Drenthen W, Pieper PG, Roos-Hesselink JW et al. 2007. Outcome of pregnancy in women with congenital heart disease: a literature review. J. Am. Coll. Cardiol. 49:2303–11
     [Google Scholar]
  78. 78.
    Van De Bruaene A, Hickey EJ, Kovacs AH et al. 2018. Phenotype, management and predictors of outcome in a large cohort of adult congenital heart disease patients with heart failure. Int. J. Cardiol. 252:80–87
     [Google Scholar]
  79. 79.
    Crossland DS, Van De Bruaene A, Silversides CK et al. 2019. Heart failure in adult congenital heart disease: from advanced therapies to end-of-life care. Can. J. Cardiol. 35:1723–39
     [Google Scholar]
  80. 80.
    Crossland DS, Jansen K, Parry G et al. 2019. Outcome following heart transplant assessment in adults with congenital heart disease. Heart 105:1741–47
     [Google Scholar]
  81. 81.
    Badger JM. 2005. Factors that enable or complicate end-of-life transitions in critical care. Am. J. Crit. Care 14:513–21
     [Google Scholar]
  82. 82.
    Tobler D, Greutmann M, Colman JM et al. 2012. End-of-life in adults with congenital heart disease: a call for early communication. Int. J. Cardiol. 155:383–87
     [Google Scholar]
  83. 83.
    Tobler D, Greutmann M, Colman JM et al. 2012. End-of-life care in hospitalized adults with complex congenital heart disease: care delayed, care denied. Palliat. Med. 26:72–79
     [Google Scholar]
/content/journals/10.1146/annurev-med-050922-052324
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Complex Congenital Heart Disease in the Adult
Annual Review of Medicine 75, 493 (2024); https://doi.org/10.1146/annurev-med-050922-052324
/content/journals/10.1146/annurev-med-050922-052324
/content/journals/10.1146/annurev-med-050922-052324
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