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Published in:

01-08-2015 | Review Article

Perioperative Care of Children with Eisenmenger Syndrome Undergoing Non-cardiac Surgery

Author: Bibhuti B. Das

Published in: Pediatric Cardiology | Issue 6/2015

Abstract

The Eisenmenger syndrome (ES) is a severe form of pulmonary arterial hypertension and arises in congenital heart disease with a systemic-to-pulmonary shunt. Patients with ES have multisystem involvement as a result of chronic hypoxemia with hematologic, skeletal, renal, and neurologic systems, causing significant morbidity and mortality. In contrast to pulmonary arterial hypertension, survival prospects are far superior in patients with ES and a growing number of ES patients are surviving into adulthood. As a result, many face the prospect of incidental surgery. To date, there is no guideline for the perioperative care of ES patients in children and limited data available for adult patients. This review provides an overview of appropriate measures for the safe perioperative care of patients, based on an understanding of the pathophysiological changes that occur in ES.

Ammash NM, Connolly HM, Abel MD, Warnes CA (1999) Noncardiac surgery in Eisenmenger syndrome. J Am Coll Cardiol 33:222–227PubMedCrossRef

Barry Lyons, Motherway C, Casey W, Doherty P (1995) The anaesthetic management of the child with Eisenmenger’s syndrome. Can J Anaesth 42:904–909CrossRef

Blais G, Langlebe D, Hubert B (2003) Pulmonary arterial hypertension: pathophysiology and anesthetic approach. Anesthesiology 99:1415–1432CrossRef

Bonello B, Renard S, Mancini J, Hubert S, Habbib G, Fraise A (2004) Life span of patients with Eisenmenger syndrome is not superior to that of patients with other causes of pulmonary hypertension. Cardiovasc Diagn Therapy 4:341–349

Brammell HL, Vogel JHK, Pryor RI, Blount SG Jr (1971) The Eisenmenger syndrome-clinical and physiologic reappraisal. Am J Cardiol 28:679–691PubMedCrossRef

Cantor WJ, Harrison DA, Moussadii JS et al (1999) Determinants of survival in adults with Eisenmenger syndrome. Am J Cardiol 84:677–681PubMedCrossRef

Carmosino MJ, Friesen RH, Doran A, Ivy DD (2007) Perioperative complications in children with pulmonary hypertension undergoing surgery or cardiac catheterization. Anesth Analg 104:521–527PubMedCentralPubMedCrossRef

Clarkson PM, Frye RL, DuShane JW et al (1968) Prognosis for patients with ventricular septal defect and severe pulmonary vascular obstructive disease. Circulation 8:129–135CrossRef

Daliento L, Somerville J, Presbitero P et al (1998) Eisenmenger syndrome factors relating to deterioration and death. Eur Heart J 19:1845–1855PubMedCrossRef

10.

Deanfield J, Thaulow E, Warens C et al (2003) Management of grown up congenital heart disease. Eur Heart J 24:1035–1084PubMedCrossRef

11.

Devitt JH, Noble WH, Byrick RJ (1982) A Swan-Ganz catheter related complication in a patient with Eisenmenger’s syndrome. Anesthesiology 57:335–337PubMedCrossRef

12.

Diller GP, Kempny A, Inuzuka R et al (2014) Survival prospects of treatment naïve patients with Eisenmenger: a systematic review of the literature and report of own experience. Heart 100:1366–1372PubMedCrossRef

13.

Eisenmenger V (1897) Die angeborenon defect der kammerscheiderwand des herzeas. Z Klin Med 32:1–28

14.

Farber HW, Loscalzo J (2004) Pulmonary arterial hypertension. N Eng J Med 351:1655–1665CrossRef

15.

Fernandez SM, Newburger JW, Lang P et al (2003) Usefulness of epoprostenol therapy in the severely ill adolescent/adult with Eisenmenger physiology. Am J Cardiol 91:632–635CrossRef

16.

Flick RP, Sprung J, Harrison TE et al (2007) Anesthesia-related cardiac arrest in children: initial findings of the pediatric perioperative cardiac arrest (POCA) registry. Anesthesiology 106:226–237PubMedCrossRef

17.

Frisen RH, Williams GD (2006) Anesthetic management of children with pulmonary hypertension. Pediatr Anesth 18:108–216

18.

Galie N, Beghetti M, Gatzoulis MA et al (2006) Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation 114:48–54PubMedCrossRef

19.

Haworth SG (2006) Role of the endothelium in pulmonary arterial hypertension. Vasc Pharmacol 45:317–325CrossRef

20.

Hoeper MM, Granton J (2011) Intensive care unit management of patients with severe pulmonary hypertension and right heart failure. Am J Respir Crit Care Med 184:1114–1124PubMedCrossRef

21.

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

22.

Iversen K, Jensen AS, Jensen TV, Vejlstrup NG, Sondergaard L (2010) Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: a randomized, placebo-controlled, double-blinded trial. Eur Heart J 31:344–349CrossRef

23.

Ivy DD, Abman SH, Barst RJ et al (2013) Pediatric pulmonary hypertension. J Am Col Cardiol 62(25 Suppl):D117–D126CrossRef

24.

Kahn ML (1993) Eisenmenger’s syndrome in pregnancy. N Eng J Med 329:887CrossRef

25.

Kaw R, Pasupuleti V, Deshpande A, Hamieh T, Walker E, Minai OA (2011) Pulmonary hypertension: an important predictor of outcomes in patients undergoing non-cardiac surgery. Respir Med 5:619–624CrossRef

26.

Kidd L, Driscol DJ, Gersony WH et al (1993) Second natural history study of congenital heart defects: results of treatment of patients with ventricular septal defects. Circulation 87:138–151

27.

Kleinman ME, Chameides L, Schexnayder SM et al (2010) Part 14: pediatric advanced life support: 2010 American Heart Association guidelines for cardiopulmonary resuscitation and emergency cardiovascular care. Circulation 122:S876–S908PubMedCrossRef

28.

Lai HC, Lai HC, Wang KY, Lee WL, Ting CT, Liu TJ (2007) Severe pulmonary hypertension complicates postoperative outcome of non-cardiac surgery. Br J Anaesth 99:184–190PubMedCrossRef

29.

Lumley J, Whitman JG, Morgan M (1997) General anesthesia in Eisenmenger’s syndrome. Anaesth Analg Curr Res 56:543–547

30.

Masen N, Maessen J, Allesie M, Shotten U (2012) Post-operative atrial fibrillation: a maze of mechanism. Europace 14:159–174CrossRef

31.

Meyer S, McLaughlin VV, Seyfarth HJ (2013) Outcome of noncardiac, nonobstetric surgery in patients with PAH: an international prospective survey. Eur Respir J 41:1302–1307PubMedCrossRef

32.

Mooray JP, Geiduscheck JM, Ramamoorthy C et al (2009) Perioperative cardiac arrest in children between 1988 and 2005 at tertiary referral center: a study of 92,881 patients. Anesthesiology 93:6–14CrossRef

33.

Niwa K, Perloff JK, Kaplan S, Child JS (1999) Miner PD (1999) Eisenmeneger syndrome in adults: ventricular septal defect, truncus arteriosus, univentricular heart. J Am Coll Cardiol 34:223–232PubMedCrossRef

34.

Oechslin E (2004) Hematological management of the cyanotic adult with congenital heart disease. Int J Cardiol 97(Suppl 1):109–115PubMedCrossRef

35.

Perloff JK (2003) The clinical recognition of congenital heart disease, 5th Ed. Philadelphia, WB Saunders, pp 318–321

36.

Placik B, Rodbard S, McMohan J, Swaroop S (1976) Pulmonary artery dissection and rupture in Eisenmenger’s syndrome. Vasc Surg 10:72–80PubMedCrossRef

37.

Raines DE, Liberthson RR, Murray JR (1996) Anesthetic management and outcome following noncardiac surgery in nonparturients with Eisenmenger’s physiology. J Clin Anesth 30:341–347CrossRef

38.

Ramakrishna G, Sprung J, Ravi B, Chandrasekaran K, McGoon M (2005) Impact of pulmonary hypertension on the outcomes of noncardiac surgery: predictors of perioperative morbidity and mortality. J Am Coll Cardiol 45:1691–1699PubMedCrossRef

39.

Raposo-Sonnenfeld I, Otero-Gonzalez I, Blanco-Aparicio M, Ferrer-Barba A, Medrano-Lopez C (2007) Treatment with sildenafil, bosentan or both in children and young people with idiopathic pulmonary arterial hypertension and Eisenmenger’s syndrome. Rev Esp Cardiol 60:366–372PubMedCrossRef

40.

Rosenzweig EB, Kerstein D, Barst RJ (1999) Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 99:1858–1865PubMedCrossRef

41.

Sandoval J, Rothman A, Pulido T (2001) Atrial septostomy for pulmonary hypertension. Clin Chest Med 22:547–560PubMedCrossRef

42.

Simonneau G, Gatzoulis MA, Adatia I et al (2013) Updated clinical classification of pulmonary hypertension. J Am Col Cardiol 62(25 Suppl):D34–D41CrossRef

43.

Spinnato JA, Karynack BJ, Cooper MW (1981) Eisenmenger’s syndrome in pregnancy: epidural anesthesia for elective cesarean section. N Eng J Med 304:1215–1217CrossRef

44.

Steele PM, Fuster V, Cohen M et al (1987) Isolated atrial septal defect with pulmonary vascular obstructive disease: long-term follow-up and prediction of outcome after surgical correction. Circulation 76:1037–1042PubMedCrossRef

45.

Tapson VF, Gomberg-Maitland M, McLaughlin VV et al (2006) Safety and efficacy of IV treprostinil for pulmonary hypertension: a prospective, multicenter, open-label, 12 week trial. Chest 129:683–688PubMedCrossRef

46.

Territo MC, Perloff JK, Rosove MH et al (1998) Acquired von Willebrand factor abnormalities in adults with congenital heart disease: dependence upon cardiopulmonary pathophysiologic subtype. Clin Appl Thromb/Hemost 4:257–261CrossRef

47.

Twite MD, Friesen RH (2014) The anesthetic management of children with pulmonary hypertension in the cardiac catheterization laboratory. Anesthesiol Clin 32:157–173PubMedCrossRef

48.

Warnes CA, Williams RG, Bashore TM et al (2008) 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. Circulation 118:2395–2451PubMedCrossRef

49.

Wilson W, Taubert KA, Gewitz M et al (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 116:1736–1754PubMedCrossRef

50.

Wood P (1958) The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J 2:701–709PubMedCentralPubMedCrossRef

51.

Yao A (2012) Recent advances and future perspectives in therapeutic strategies for pulmonary arterial hypertension. J Cardiol 31:1124–1131

52.

Zuckerman WA, Leaderer D, Rowan CA, Mitunewicz JD, Rosenweig EB (2011) Ambrisentan for pulmonary arterial hypertension due to congenital heart disease. Am J Cardiol 107:1381–1385PubMedCrossRef

Title: Perioperative Care of Children with Eisenmenger Syndrome Undergoing Non-cardiac Surgery
Author: Bibhuti B. Das
Publication date: 01-08-2015
Publisher: Springer US
Published in: Pediatric Cardiology / Issue 6/2015
Print ISSN: 0172-0643
Electronic ISSN: 1432-1971
DOI: https://doi.org/10.1007/s00246-015-1184-7

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