The Ideal Intensive Care Unit for Adults with Congenital Heart Disease

  • David Briston
  • Curt DanielsEmail author
Part of the Congenital Heart Disease in Adolescents and Adults book series (CHDAA)


Adults with congenital heart disease (ACHD) represent a diverse cohort with specific and wide-ranging healthcare needs especially within the intensive care unit (ICU). They require coordinated care with expertise from both pediatric and internal medicine realms in both primary and consultative roles. With data showing increased morbidity and mortality as compared to other populations, the details regarding care are especially important. Cardiologists with specific training in ACHD and a team of subspecialists able to care for specific needs are necessary to providing adequate care. Consultative services with multiple subspecialties should be available to deal with litany of other healthcare needs that may become relevant. With increased morbidity and mortality and as a growing population, this cohort has unique healthcare needs within the ICU. While no perfect ICU exists for ACHD patients, combining the expertise of pediatric and internal medicine knowledge with well-trained staff together constitutes the most ideal environment to care the ACHD population.


Adult congenital heart disease Congenital heart disease Intensive care unit 


  1. 1.
    Bhatt AB, Rajabali A, He W, Benavidez OJ. High resource use among adult congenital heart surgery admissions in adult hospitals: risk factors and association with death and comorbidities. Congenit Heart Dis. 2015;10(1):13–20.CrossRefGoogle Scholar
  2. 2.
    Marelli AJ, Ionescu-Ittu R, Mackie AS, Guo L, Dendukuri N, Kaouache M. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014;130(9):749–56.CrossRefGoogle Scholar
  3. 3.
    Gilboa SM, Devine OJ, Kucik JE, Oster ME, Riehle-Colarusso T, Nembhard WN, Xu P, Correa A, Jenkins K, Marelli AJ. Congenital heart defects in the United States: estimating the magnitude of the affected population in 2010. Circulation. 2016;134(2):101–9.CrossRefGoogle Scholar
  4. 4.
    Webb G, Mulder BJ, Aboulhosn J, Daniels CJ, Elizari MA, Hong G, Horlick E, Landzberg MJ, Marelli AJ, O'Donnell CP, Oechslin EN, Pearson DD, Pieper EP, Saxena A, Schwerzmann M, Stout KK, Warnes CA, Khairy P. The care of adults with congenital heart disease across the globe: current assessment and future perspective: a position statement from the International Society for Adult Congenital Heart Disease (ISACHD). Int J Cardiol. 2015;195:326–33.CrossRefGoogle Scholar
  5. 5.
    Holst KA, Dearani JA, Burkhart HM, Connolly HM, Warnes CA, Li Z, Schaff HV. Risk factors and early outcomes of multiple reoperations in adults with congenital heart disease. Ann Thorac Surg. 2011;92(1):122–8.CrossRefGoogle Scholar
  6. 6.
    Berdat PA, Immer F, Pfammatter JP, Carrel T. Reoperations in adults with congenital heart disease: analysis of early outcome. Int J Cardiol. 2004;93(2–3):239–45.CrossRefGoogle Scholar
  7. 7.
    Engelings CC, Helm PC, Abdul-Khaliq H, Asfour B, Bauer UM, Baumgartner H, Kececioglu D, Körten MA, Diller GP, Tutarel O. Cause of death in adults with congenital heart disease—an analysis of the German National Register for Congenital Heart Defects. Int J Cardiol. 2016;211:31–6.CrossRefGoogle Scholar
  8. 8.
    Allan C. Intensive care of the adult patient with congenital heart disease. Prog Cardiovasc Dis. 2011;53(4):274–80.CrossRefGoogle Scholar
  9. 9.
    Mackie AS, Pilote L, Ionescu-Ittu R, Rahme E, Marelli AJ. Health care resource utilization in adults with congenital heart disease. Am J Cardiol. 2007;99(6):839–43.CrossRefGoogle Scholar
  10. 10.
    Verheugt CL, Uiterwaal CS, van der Velde ET, Meijboom FJ, Pieper PG, Sieswerda GT, Plokker HW, Grobbee DE, Mulder BJ. The emerging burden of hospital admissions of adults with congenital heart disease. Heart. 2010;96(11):872–8.CrossRefGoogle Scholar
  11. 11.
    Goldsbury DE, O'Connell DL, Girgis A, Wilkinson A, Phillips JL, Davidson PM, Ingham JM. Acute hospital-based services used by adults during the last year of life in New South Wales, Australia: a population-based retrospective cohort study. BMC Health Serv Res. 2015;15:537.CrossRefGoogle Scholar
  12. 12.
    Kogon BE, Plattner C, Leong T, Kirshbom PM, Kanter KR, McConnell M, Book W. Adult congenital heart surgery: adult or pediatric facility? Adult or pediatric surgeon? Ann Thorac Surg. 2009;87(3):833–40.CrossRefGoogle Scholar
  13. 13.
    Maxwell BG, Maxwell TG, Wong JK. Decentralization of care for adults with congenital heart disease in the United States: a geographic analysis of outpatient surgery. PLoS One. 2014;9(9):e106730.CrossRefGoogle Scholar
  14. 14.
    Karamlou T, Diggs BS, Ungerleider RM, Welke KF. Adults or big kids: what is the ideal clinical environment for management of grown-up patients with congenital heart disease? Ann Thorac Surg. 2010;90(2):573–9.CrossRefGoogle Scholar
  15. 15.
    Karamlou T, Diggs BS, Person T, Ungerleider RM, Welke KF. National practice patterns for management of adult congenital heart disease: operation by pediatric heart surgeons decreases in-hospital death. Circulation. 2008;118(23):2345–52.CrossRefGoogle Scholar
  16. 16.
    Redington A. Intensive care in the adult with congenital heart disease. Pediatr Crit Care Med. 2016;17(8 Suppl 1):S377–82.CrossRefGoogle Scholar
  17. 17.
    Schuuring MJ, van Gulik EC, Koolbergen DR, Hazekamp MG, Lagrand WK, Backx AP, Mulder BJ, Bouma BJ. Determinants of clinical right ventricular failure after congenital heart surgery in adults. J Cardiothorac Vasc Anesth. 2013;27(4):723–7.CrossRefGoogle Scholar
  18. 18.
    Perry J, Lanzberg M, Franklin W, Webb G, Chang AC. Cardiac intensive care of the adult with congenital heart disease: basic principles in the management of common problems. World J Pediatr Congenit Heart Surg. 2011;2(3):430–44.CrossRefGoogle Scholar
  19. 19.
    Giannakoulas G, Dimopoulos K, Engel R, Goktekin O, Kucukdurmaz Z, Vatankulu MA, Bedard E, Diller GP, Papaphylactou M, Francis DP, Di Mario C, Gatzoulis MA. Burden of coronary artery disease in adults with congenital heart disease and its relation to congenital and traditional heart risk factors. Am J Cardiol. 2009;103(10):1445–50.CrossRefGoogle Scholar
  20. 20.
    Bouchardy J, Therrien J, Pilote L, Ionescu-Ittu R, Martucci G, Bottega N, Marelli AJ. Atrial arrhythmias in adults with congenital heart disease. Circulation. 2009;120(17):1679–86.CrossRefGoogle Scholar
  21. 21.
    Diller GP, Giardini A, Dimopoulos K, Gargiulo G, Müller J, Derrick G, Giannakoulas G, Khambadkone S, Lammers AE, Picchio FM, Gatzoulis MA, Hager A. Predictors of morbidity and mortality in contemporary Fontan patients: results from a multicenter study including cardiopulmonary exercise testing in 321 patients. Eur Heart J. 2010;31(24):3073–83.CrossRefGoogle Scholar
  22. 22.
    Buelow MW, Dall A, Bartz PJ, Tweddell JS, Sowinski J, Rudd N, Katzmark L, Earing MG. Renal dysfunction is common among adults after palliation for previous tetralogy of Fallot. Pediatr Cardiol. 2013;34(1):165–9.CrossRefGoogle Scholar
  23. 23.
    Buelow MW, Cohen SB, Earing MG. Renal dysfunction in adults with congenital heart disease. Prog Pediatr Cardiol. 2016;41:47–9.CrossRefGoogle Scholar
  24. 24.
    Dimopoulos K, Diller GP, Koltsida E, Pijuan-Domenech A, Papadopoulou SA, Babu-Narayan SV, Salukhe TV, Piepoli MF, Poole-Wilson PA, Best N, Francis DP, Gatzoulis MA. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease. Circulation. 2008;117(18):2320–8.CrossRefGoogle Scholar
  25. 25.
    Waldow HC, Westhoff-Bleck M, Widera C, Templin C, von Depka M. Acquired von Willebrand syndrome in adult patients with congenital heart disease. Int J Cardiol. 2014;176(3):739–45.CrossRefGoogle Scholar
  26. 26.
    lonso-Gonzalez R, Borgia F, Diller GP, Inuzuka R, Kempny A, Martinez-Naharro A, Tutarel O, Marino P, Wustmann K, Charalambides M, Silva M, Swan L, Dimopoulos K, Gatzoulis MA. Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival. Circulation. 2013;127(8):882–90.CrossRefGoogle Scholar
  27. 27.
    Pundi K, Pundi KN, Kamath PS, Cetta F, Li Z, Poterucha JT, Driscoll DJ, Johnson JN. Liver disease in patients after the Fontan operation. Am J Cardiol. 2016;117(3):456–60.CrossRefGoogle Scholar
  28. 28.
    Giamberti A, Chessa M, Abella R, Butera G, Carlucci C, Nuri H, Frigiola A, Ranucci M. Morbidity and mortality risk factors in adults with congenital heart disease undergoing cardiac reoperations. Ann Thorac Surg. 2009;88(4):1284–9.CrossRefGoogle Scholar
  29. 29.
    Wauthy P, Massaut J, Sanoussi A, Demanet H, Morissens M, Damry N, Dessy H, Malekzadeh-Milani SG, Deuvaert FE. Ten-year experience with surgical treatment of adults with congenital cardiac disease. Cardiol Young. 2011;21(1):39–45.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Nationwide Children’s Hospital, The Ohio State UniversityColumbusUSA

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