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The Patient with Congenital Cardiac Disease

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Anesthesia in High-Risk Patients

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Abstract

Until a few decades ago, congenital heart disease (CHD) caregivers focused on pediatrics. As a result of medical and surgical advances in the management of children born with heart disease, there are an increasing number of adults with CHD surviving into adulthood. Thus, there is an increasing need for specialized care for this challenging population.

In addition, some of these CHD are uncorrectable despite the primary surgical repair and will require lifelong and specialized follow-up and medical care. As these patients grow older, the risk of complications increases. It is important to know that arrhythmia, heart failure, pulmonary hypertension (PHT), endocarditis, and thromboembolism may complicate the progression of these adults. Women represent half of these patients, and pregnancy in this context constitutes a major challenge for the multidisciplinary staff and sometimes a significant risk for mothers and babies.

ACDH patients can undergo cardiac and noncardiac surgeries. And they will have to be managed and approached differently from the normal population. For cardiac surgeries that are particularly complex, anesthesiologists and intensivists have a specific training for the management of these challenging but rewarding patients. Noncardiac surgery for ACHD patients represents a challenge for non-experienced medical team. The medical advice and consultation of referent cardiologists and anesthesiologists are necessary for a stable ACHD patient. When the CHD is complex and not well tolerated, it is essential to transfer the patient to a multidisciplinary specialized referent center, especially ACHD pregnant women with Fontan procedure or severe PHT.

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References

  1. Ávila P, Mercier LA, Dore A, Marcotte F, Mongeon FP, Ibrahim R, Asgar A, Miro J, Andelfinger G, Mondesert B, de Guise P, Poirier N, Khairy P. Adult congenital heart disease: a growing epidemic. Can J Cardiol 2014;30(12 Suppl):S410–9.

    Google Scholar 

  2. Mylotte D, Pilote L, Ionescu-Ittu R, Abrahamowicz M, Khairy P, Therrien J, Mackie AS, Marelli A. Specialized adult congenital heart disease care: the impact of policy on mortality. Circulation. 2014;129(18):1804–1812.

    Google Scholar 

  3. Marelli AJ, Mackie AS, Ionescu-Ittu R, et al. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation. 2007;115:163–72.

    Article  PubMed  Google Scholar 

  4. Agarwal S, Sud K, Khera S, Kolte D, Fonarow GC, Panza JA, Menon V. Trends in the burden of adult congenital heart disease in US emergency departments. Clin Cardiol 2016;39(7):391–8.

    Google Scholar 

  5. Mazor Dray E, Marelli AJ. Adult congenital heart disease: scope of the problem. Cardiol Clin. 2015;33(4):503–12. vii

    Article  PubMed  Google Scholar 

  6. Ntiloudi D, Giannakoulas G, Parcharidou D, Panagiotidis T, Gatzoulis MA, Karvounis H. Adult congenital heart disease: a paradigm of epidemiological change. Int J Cardiol. 2016;218:269–74.

    Article  PubMed  Google Scholar 

  7. Greutmann M, Tobler D, Kovacs AH, et al. Increasing mortality burden among adults with complex congenital heart disease. Congenit Heart Dis. 2015;10(2):117–27.

    Article  PubMed  Google Scholar 

  8. Diller GP, Kempny A, Alonso-Gonzalez R, et al. Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow-up at a large tertiary centre. Circulation. 2015;132:2118–25.

    Article  PubMed  Google Scholar 

  9. Drenthen W, Pieper PG, Roos-Hesselink JW, et al. Outcome of pregnancy in women with congenital heart disease: a literature review. J Am Coll Cardiol. 2007;49(24):2303–11.

    Article  PubMed  Google Scholar 

  10. Walsh EP, Cecchin F. Arrhythmias in adult patients with congenital heart disease. Circulation. 2007;115:534–45.

    Article  PubMed  Google Scholar 

  11. Holst KA, Dearani JA, Burkhart HM, et al. Risk factors and early outcomes of multiple reoperations in adults with congenital heart disease. Ann Thorac Surg. 2011;92:122–8.

    Article  PubMed  Google Scholar 

  12. Dos L, Teruel L, Ferreira IJ, et al. Late outcome of senning and mustard procedures for correction of transposition of the great arteries. Heart. 2005;91:652–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Lewis MJ, Whang W, Biviano A, Hickey K, Garan H, Rosenbaum M. Rate of arrhythmia recurrence post-ablation in adult congenital heart disease. J Am Coll Cardiol. 2016;67:910.

    Article  Google Scholar 

  14. Szili-Torok T, Kornyei L, Jordaens LJ. Transcatheter ablation of arrhythmias associated with congenital heart disease. J Interv Card Electrophysiol. 2008;22:161–6.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Bhatt AB, Foster E, Kuehl K, et al. Congenital heart disease in the older adult: a scientific statement from the American Heart Association. Circulation. 2015;131(21):1884–931.

    Article  PubMed  Google Scholar 

  16. Silka MJ, Bar-Cohen Y. A contemporary assessment of the risk for sudden cardiac death in patients with congenital heart disease. Pediatr Cardiol. 2012;33(3):452–60.

    Article  PubMed  Google Scholar 

  17. Khairy P, Van Hare GF, Balaji S, et al. 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). Heart Rhythm. 2014;11:e102–65.

    Article  PubMed  Google Scholar 

  18. Verheugt CL, Uiterwaal CS, van der Velde ET, et al. Mortality in adult congenital heart disease. Eur Heart J. 2010;31:1220–9.

    Article  PubMed  Google Scholar 

  19. Diller GP, Dimopoulos K, Okonko D, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005;112:828–35.

    Article  PubMed  Google Scholar 

  20. Humbert M, Morrell NW, Archer SL, et al. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43:13S–24S.

    Article  CAS  PubMed  Google Scholar 

  21. D’Alto M, Diller GP. Pulmonary hypertension in adults with congenital heart disease and Eisenmenger syndrome: current advanced management strategies. Heart. 2014;100:1322–8.

    Article  PubMed  Google Scholar 

  22. Ministeri M, Alonso-Gonzalez R, Swan L, Dimopoulos K. Common long-term complications of adult congenital heart disease: avoid falling in a H.E.A.P. Expert Rev Cardiovasc Ther. 2016;14(4):445–62.

    Article  CAS  PubMed  Google Scholar 

  23. Schneider F, Kelleher A. Adult congenital heart disease. Anes Inten Care Med. 2012;13(10):513–8.

    Article  Google Scholar 

  24. Harris RC, Fries MH, Boyle A, et al. Multidisciplinary management of pregnancy in complex congenital heart disease: a model for coordination of care. Congenit Heart Dis. 2014;9:E204–11. doi:10.1111/chd.12163.

    Article  PubMed  Google Scholar 

  25. Deanfield J, Thaulow E, Warnes C, et al. Management of grown up congenital heart disease. Eur Heart J. 2003;24:1035–84.

    Article  PubMed  Google Scholar 

  26. Drenthen W, Boersma E, Balci A, et al. Predictors of pregnancy complications in women with congenital heart disease. Eur Heart J. 2010;31(17):2124–32.

    Article  PubMed  Google Scholar 

  27. Giannakoulas G, Boutsikou M. The Gordian knot of thromboembolism in congenital heart disease. Heart. 2015;101(19):1523–4.

    Article  CAS  PubMed  Google Scholar 

  28. Andropoulos DB, Stayer SA, Skjonsby BS, East DL, McKenzie ED, Fraser CD. Anesthetic and perioperative outcome of teenagers and adults with congenital heart disease. J Cardiothorac Vasc Anesth. 2002;16(6):731–6.

    Article  CAS  PubMed  Google Scholar 

  29. Seal R. Adult congenital heart disease. Paediatr Anaesth. 2011;21(5):615–22.

    Article  PubMed  Google Scholar 

  30. Chassot PG, Bettex DA. Anesthesia and adult congenital heart disease. J Cardiothorac Vasc Anesth. 2006;20(3):414–37.

    Article  PubMed  Google Scholar 

  31. Acheampong B, Johnson JN, Stulak JM, Dearani JA, Kushwaha SS, Daly RC, Haile DT, Schears GJ. Postcardiotomy ECMO support after high-risk operations in adult congenital heart disease. Congenit Heart Dis. 2016;11:751–755.

    Google Scholar 

  32. Hyldebrandt JA, Sivén E, Agger P, Frederiksen CA, Heiberg J, Wemmelund KB, Ravn HB. Effects of milrinone and epinephrine or dopamine on biventricular function and hemodynamics in an animal model with right ventricular failure after pulmonary artery banding. Am J Physiol Heart Circ Physiol 2015;309(1):H206–12.

    Google Scholar 

  33. Siehr SL, Feinstein JA, Yang W, Peng LF, Ogawa MT, Ramamoorthy C. Hemodynamic effects of phenylephrine, vasopressin, and epinephrine in children with pulmonary hypertension: a pilot study. Pediatr Crit Care Med. 2016;17(5):428–37.

    Article  PubMed  Google Scholar 

  34. Barnett CF, Alvarez P, Park MH. Pulmonary arterial hypertension: diagnosis and treatment. Cardiol Clin. 2016;34(3):375–89.

    Article  PubMed  Google Scholar 

  35. Ghanayem NS, Berger S, Tweddell JS. Medical management of the failing Fontan. Pediatr Cardiol. 2007;28:465–71.

    Article  CAS  PubMed  Google Scholar 

  36. Verghese S, Jackson M, Vaughns J, et al. Plastic bronchitis in a child with Fontan’s physiology presenting for urgent rigid bronchoscopy. Anesth Analg. 2008;107:1446–7.

    Article  PubMed  Google Scholar 

  37. Deal BJ, Mavroudis C, Backer CL, et al. Arrhythmia management in the Fontan patient. Pediatr Cardiol. 2007;28:448–56.

    Article  CAS  PubMed  Google Scholar 

  38. McHugh KE, Hillman DG, Gurka MJ, et al. Three-stage palliation of hypoplastic left heart syndrome in the University Health System Consortium. Congenit Heart Dis. 2010;5:8–15.

    Article  PubMed  Google Scholar 

  39. Wald RM, Valente AM, Marelli A. Heart failure in adult congenital heart disease: emerging concepts with a focus on tetralogy of Fallot. Trends Cardiovasc Med. 2015;25(5):422–32.

    Article  PubMed  Google Scholar 

  40. Therrien J, Siu SC, McLaughlin PR, et al. Pulmonary valve replacement in adults late after repair of tetralogy of Fallot: are we operating too late? J Am Coll Cardiol. 2000;36:1670–5.

    Article  CAS  PubMed  Google Scholar 

  41. Selly JB, Iriart X, Roubertie F, Mauriat P, Marek J, Guilhon E, Jamal-Bey K, Thambo JB. Multivariable assessment of the right ventricle by echocardiography in patients with repaired tetralogy of Fallot undergoing pulmonary valve replacement: a comparative study with magnetic resonance imaging. Arch Cardiovasc Dis 2015;108(1):5–15.

    Google Scholar 

  42. Jaquiss RD, Aziz H. Is four stage management the future of univentricular hearts? Destination therapy in the young. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2016;19(1):50–4.

    Article  PubMed  Google Scholar 

  43. Cohen S, Houyel L, Guillemain R, Varnous S, Jannot AS, Ladouceur M, Boudjemline Y, Bonnet D, Iserin L. Temporal trends and changing profile of adults with congenital heart disease undergoing heart transplantation. Eur Heart J 2016;37(9):783–9.

    Google Scholar 

  44. Krishnamurthy Y, Cooper LB, Lu D, Schroder JN, Daneshmand MA, Rogers JG, Milano CA, Hernandez AF, Patel CB. Trends and outcomes of patients with adult congenital heart disease and pulmonary hypertension listed for orthotopic heart transplantation in the United States. J Heart Lung Transplant 2016;35(5):619–24.

    Google Scholar 

  45. Naguib MA, Dob DP, Gatzoulis MA. A functional understanding of moderate to complex congenital heart disease and the impact of pregnancy. Part II: tetralogy of Fallot, Eisenmenger’s syndrome and the Fontan operation. Int J Obstet Anesth. 2010;19(3):306–12.

    Article  CAS  PubMed  Google Scholar 

  46. Mauriat P, Tafer N. Anesthesia for non-cardiac surgery after Fontan repair. Ann Fr Anesth Reanim. 2013;32(1):e31–6.

    Article  CAS  PubMed  Google Scholar 

  47. Verheugt CL, Uiterwaal CS, van der Velde ET, et al. Turning 18 with congenital heart disease: prediction of infective endocarditis based on a large population. Eur Heart J. 2011;32:1926–34.

    Article  PubMed  Google Scholar 

  48. Hoffmann A, Chockalingam P, Balint OH, et al. Cerebrovascular accidents in adult patients with congenital heart disease. Heart. 2010;96(15):1223–6.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Pediatric and Adult Congenital Heart disease Intensive Care, Haut-Lévèque Hospital, Avenue Magellan, 33604, Pessac, France

    Philippe Mauriat

  2. Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Centre, PO Box: 11-0236, Riad El Solh, Beirut, 1107 2020, Lebanon

    Jana Assy

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  1. Philippe Mauriat
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Correspondence to Philippe Mauriat .

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Editors and Affiliations

  1. Department of Anaesthesiology and Critical Care Medicine, Louis Pradel Hosp, Hospices Civils Lyon, Lyon, Rhône, France

    Jean-Luc Fellahi

  2. Service d'Anesthésie-Réanimation, Hôpital Nord, Marseille cedex 20, France

    Marc Leone

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Mauriat, P., Assy, J. (2018). The Patient with Congenital Cardiac Disease. In: Fellahi, JL., Leone, M. (eds) Anesthesia in High-Risk Patients. Springer, Cham. https://doi.org/10.1007/978-3-319-60804-4_5

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  • DOI: https://doi.org/10.1007/978-3-319-60804-4_5

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  • Publisher Name: Springer, Cham

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  • Online ISBN: 978-3-319-60804-4

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