Anesthetic management and outcomes for patients with pulmonary hypertension and intracardiac shunts and Eisenmenger syndrome: a review of institutional experience

J Clin Anesth. 2014 Jun;26(4):286-93. doi: 10.1016/j.jclinane.2013.11.022. Epub 2014 Jun 6.


Study objective: To propose a set of recommendations for the perioperative management of patients with Eisenmenger syndrome and similar physiology, based on 20 years of experience at a single institution.

Design: Retrospective study of institutional outcomes of Eisenmenger syndrome patients and patients with balanced or fixed right-to-left intracardiac shunts with pulmonary hypertension undergoing noncardiac surgery.

Setting: Single center, university-affiliated hospital.

Measurements: Measurements included data from patients with Eisenmenger syndrome or similar physiology, shunt direction, right ventricular systolic pressure, congestive heart failure classification, noncardiac surgery, type of anesthesia, echocardiographic and catheterization data, mortality within 30 days of surgery, choice of monitoring, and vasopressor use.

Main results: 33 patients with Eisenmenger syndrome or similar physiology undergoing 53 general, regional and/or monitored anesthetic procedures were identified. Significant systemic arterial hypotension occurred in 14 individuals (26%) and oxygen desaturation in 9 (17%) patients. Administration of an intravenous (IV) vasopressor agent during induction significantly decreased the incidence of hypotension. The type of IV induction agent did not influence hemodynamic alterations, though patients who received propofol experienced a trend towards increased hypotension (83% of pts) when a vasopressor was not used. Inhalational induction, regardless of vasopressor use, was more likely to result in hypotension (60% of pts). The 30-day mortality was 3.8% (two pts). Both patients had minor elective procedures with monitored anesthesia care (MAC).

Conclusions: Hypotension is more common in patients with Eisenmenger syndrome and similar physiology when a vasopressor is not used during the peri-induction period, regardless of induction agent. Etomidate tended to have better hemodynamic stability than other induction agents. The use of a vasopressor is recommended. We present general recommendations for anesthesiologists and strongly recommend use of a vasopressor before or during induction to reduce hypotension along with complete avoidance of inhalational induction. Further, MAC anesthesia has been associated with perioperative and 30-day mortality.

Keywords: Congenital heart disease; Eisenmenger syndrome; Etomidate; Hypotension; Monitored anesthesia care; Vasopressors.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Anesthesia / methods*
  • Anesthetics / administration & dosage*
  • Eisenmenger Complex / complications*
  • Eisenmenger Complex / physiopathology
  • Etomidate / administration & dosage
  • Hospitals, University
  • Humans
  • Hypertension, Pulmonary / complications*
  • Hypotension / epidemiology
  • Hypotension / etiology
  • Hypotension / prevention & control
  • Incidence
  • Middle Aged
  • Perioperative Care / methods
  • Propofol / administration & dosage
  • Retrospective Studies
  • Surgical Procedures, Operative / methods
  • Vasoconstrictor Agents / administration & dosage
  • Young Adult


  • Anesthetics
  • Vasoconstrictor Agents
  • Propofol
  • Etomidate