Official reprint from UpToDate®
www.uptodate.com ©2018 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Anesthesia for adults with congenital heart disease undergoing noncardiac surgery

Edmund Jooste, MB, ChB
Kelly Machovec, MD, MPH
Section Editors
Jonathan B Mark, MD
Lena S Sun, MD
Heidi M Connolly, MD, FASE
Deputy Editors
Nancy A Nussmeier, MD, FAHA
Susan B Yeon, MD, JD, FACC


Congenital heart disease (CHD) is present in approximately 6 to 19 of 1000 live births [1]. In the United States, approximately 1,000,000 pediatric and adult patients have CHD, with a growing number surviving into middle age and beyond [1-3]. Many of these patients require anesthetic care for either cardiac or noncardiac surgery. The anesthesiologist should understand the patient's native CHD lesion and prior palliation or repair, current cardiopulmonary reserve, and potential adverse effects of the planned surgical procedure in order to assess risk and develop an anesthetic plan appropriate for lesion-specific hemodynamic goals.

This topic will discuss the anesthetic management of adult CHD patients undergoing noncardiac surgery. Anesthetic management during labor and delivery for women with congenital and other high-risk heart disease is discussed separately. (See "Anesthesia for labor and delivery in high-risk heart disease: General considerations" and "Anesthesia for labor and delivery in high-risk heart disease: Specific lesions".)


The history focuses on the native congenital heart disease (CHD) lesion, prior palliative or reparative procedures, and current functional status (eg, exercise tolerance and symptoms of heart failure). The physical examination focuses on signs of cyanosis or heart failure.

Also noted are noncardiac sequelae of CHD such as developmental delay and disability, or presence of an associated genetic syndrome (eg, Down or Williams syndrome).

High- and moderate-risk lesions — CHD patients with high and moderate risk are ideally managed at a center with expertise in the care of adult patients with CHD, particularly if a major surgical procedure is planned [4]. Such patients may require advanced monitoring and other specialized management during the procedure. (See 'Monitoring' below and 'Anesthetic management' below.)

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Dec 2017. | This topic last updated: Jun 09, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2018 UpToDate, Inc.
  1. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002; 39:1890.
  2. Wren C. The epidemiology of cardiovascular malformations. In: Pediatric Cardiovascular Medicine, Moller JH, Hoffman JIE, Benson DW, van Hare GF, Wren C (Eds), Wiley-Blackwell, Oxford 2012. p.268.
  3. Baehner T, Ellerkmann RK. Anesthesia in adults with congenital heart disease. Curr Opin Anaesthesiol 2017; 30:418.
  4. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease). Circulation 2008; 118:2395.
  5. Ammash NM, Connolly HM, Abel MD, Warnes CA. Noncardiac surgery in Eisenmenger syndrome. J Am Coll Cardiol 1999; 33:222.
  6. Currigan DA, Hughes RJ, Wright CE, et al. Vasoconstrictor responses to vasopressor agents in human pulmonary and radial arteries: an in vitro study. Anesthesiology 2014; 121:930.
  7. Eagle SS, Daves SM. The adult with Fontan physiology: systematic approach to perioperative management for noncardiac surgery. J Cardiothorac Vasc Anesth 2011; 25:320.
  8. Yuki K, Casta A, Uezono S. Anesthetic management of noncardiac surgery for patients with single ventricle physiology. J Anesth 2011; 25:247.
  9. Krishnan US, Taneja I, Gewitz M, et al. Peripheral vascular adaptation and orthostatic tolerance in Fontan physiology. Circulation 2009; 120:1775.
  10. Cai J, Su Z, Shi Z, et al. Nitric oxide and milrinone: combined effect on pulmonary circulation after Fontan-type procedure: a prospective, randomized study. Ann Thorac Surg 2008; 86:882.
  11. Taylor K, Moulton D, Zhao XY, Laussen P. The impact of targeted therapies for pulmonary hypertension on pediatric intraoperative morbidity or mortality. Anesth Analg 2015; 120:420.
  12. Friesen RH, Williams GD. Anesthetic management of children with pulmonary arterial hypertension. Paediatr Anaesth 2008; 18:208.
  13. Galante D. Intraoperative management of pulmonary arterial hypertension in infants and children--corrected and republished article. Curr Opin Anaesthesiol 2011; 24:468.
  14. Williams GD, Maan H, Ramamoorthy C, et al. Perioperative complications in children with pulmonary hypertension undergoing general anesthesia with ketamine. Paediatr Anaesth 2010; 20:28.
  15. Ramakrishna G, Sprung J, Ravi BS, et al. Impact of pulmonary hypertension on the outcomes of noncardiac surgery: predictors of perioperative morbidity and mortality. J Am Coll Cardiol 2005; 45:1691.
  16. Ashary N, Kaye AD, Hegazi AR, M Frost EA. Anesthetic considerations in the patient with a heart transplant. Heart Dis 2002; 4:191.
  17. Williams GD, Ramamoorthy C. Anesthesia considerations for pediatric thoracic solid organ transplant. Anesthesiol Clin North America 2005; 23:709.
  18. Lill MC, Perloff JK, Child JS. Pathogenesis of thrombocytopenia in cyanotic congenital heart disease. Am J Cardiol 2006; 98:254.
  19. Hunyady AI, Ehlers MA. Severe polycythemia in an infant with uncorrected tetralogy of Fallot presenting for noncardiac surgery. J Clin Anesth 2006; 18:221.
  20. DeFilippis AP, Law K, Curtin S, Eckman JR. Blood is thicker than water: the management of hyperviscosity in adults with cyanotic heart disease. Cardiol Rev 2007; 15:31.
  21. Frankville D. Anesthesia for children and adults with congenital heart disease. In: Pediatric Cardiac Anesthesia, Lake CL, Booker PD (Eds), Lippincott Williams & Wilkins, Philadelphia 2005. p.601.
  22. Franchini M, Lippi G. Acquired von Willebrand syndrome: an update. Am J Hematol 2007; 82:368.
  23. Voges I, Burstein C, Budde U, Lenschow U. [Acquired von Willebrand syndrome in two children with congenital heart defects and abnormal haemodynamics]. Hamostaseologie 2006; 26:345.
  24. Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol 2014; 63:e57.
  25. Burrows FA. Physiologic dead space, venous admixture, and the arterial to end-tidal carbon dioxide difference in infants and children undergoing cardiac surgery. Anesthesiology 1989; 70:219.
  26. Wilson J, Russo P, Russo J, Tobias JD. Noninvasive monitoring of carbon dioxide in infants and children with congenital heart disease: end-tidal versus transcutaneous techniques. J Intensive Care Med 2005; 20:291.
  27. Jensen AS, Idorn L, Thomsen C, et al. Prevalence of cerebral and pulmonary thrombosis in patients with cyanotic congenital heart disease. Heart 2015; 101:1540.
  28. Ammash N, Warnes CA. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J Am Coll Cardiol 1996; 28:768.
  29. Tanidir IC, Ozturk E, Ozyilmaz I, et al. Near infrared spectroscopy monitoring in the pediatric cardiac catheterization laboratory. Artif Organs 2014; 38:838.
  30. Zacharias DG, Lilly K, Shaw CL, et al. Survey of the clinical assessment and utility of near-infrared cerebral oximetry in cardiac surgery. J Cardiothorac Vasc Anesth 2014; 28:308.
  31. Nielsen HB. Systematic review of near-infrared spectroscopy determined cerebral oxygenation during non-cardiac surgery. Front Physiol 2014; 5:93.
  32. Gray LD, Morris C. The principles and conduct of anaesthesia for emergency surgery. Anaesthesia 2013; 68 Suppl 1:14.
  33. Morris C, Perris A, Klein J, Mahoney P. Anaesthesia in haemodynamically compromised emergency patients: does ketamine represent the best choice of induction agent? Anaesthesia 2009; 64:532.
  34. Pandit JJ. Intravenous anaesthetic agents. Anaesthesia and Intensive Care Medicine 2007; 9:154.
  35. Bovill JG. Intravenous anesthesia for the patient with left ventricular dysfunction. Semin Cardiothorac Vasc Anesth 2006; 10:43.
  36. Scheffer GJ, Ten Voorde BJ, Karemaker JM, et al. Effects of thiopentone, etomidate and propofol on beat-to-beat cardiovascular signals in man. Anaesthesia 1993; 48:849.
  37. Shafer SL. Shock values. Anesthesiology 2004; 101:567.
  38. Bai W, Voepel-Lewis T, Malviya S. Hemodynamic changes in children with Down syndrome during and following inhalation induction of anesthesia with sevoflurane. J Clin Anesth 2010; 22:592.
  39. Kraemer FW, Stricker PA, Gurnaney HG, et al. Bradycardia during induction of anesthesia with sevoflurane in children with Down syndrome. Anesth Analg 2010; 111:1259.
  40. Ikemba CM, Su JT, Stayer SA, et al. Myocardial performance index with sevoflurane-pancuronium versus fentanyl-midazolam-pancuronium in infants with a functional single ventricle. Anesthesiology 2004; 101:1298.
  41. Rivenes SM, Lewin MB, Stayer SA, et al. Cardiovascular effects of sevoflurane, isoflurane, halothane, and fentanyl-midazolam in children with congenital heart disease: an echocardiographic study of myocardial contractility and hemodynamics. Anesthesiology 2001; 94:223.
  42. Laird TH, Stayer SA, Rivenes SM, et al. Pulmonary-to-systemic blood flow ratio effects of sevoflurane, isoflurane, halothane, and fentanyl/midazolam with 100% oxygen in children with congenital heart disease. Anesth Analg 2002; 95:1200.
  43. Vischoff D, Fortier LP, Villeneuve E, et al. Anaesthetic management of an adolescent for scoliosis surgery with a Fontan circulation. Paediatr Anaesth 2001; 11:607.
  44. Walker SG, Stuth EA. Single-ventricle physiology: perioperative implications. Semin Pediatr Surg 2004; 13:188.
  45. Burgmeier C, Schier F. Hemodynamic effects of laparoscopic surgery in term and preterm infants with cardiac anomalies. Pediatr Surg Int 2013; 29:587.
  46. Gillory LA, Megison ML, Harmon CM, et al. Laparoscopic surgery in children with congenital heart disease. J Pediatr Surg 2012; 47:1084.
  47. Zach KJ, Ramakrishna H, Chandrasekaran K, Weis RA. Laparoscopic colectomy in an adult with single ventricle physiology: anesthetic implications and management. Ann Card Anaesth 2015; 18:252.
  48. McClain CD, McGowan FX, Kovatsis PG. Laparoscopic surgery in a patient with Fontan physiology. Anesth Analg 2006; 103:856.