Background and Objectives Eisenmenger’s syndrome is characterized by right-to-left or bidirectional shunting and pulmonary hypertension. Perioperative risk is high for noncardiac surgery, and many clinicians avoid regional anesthesia because of the potential deleterious hemodynamic effects. We determined perioperative mortality based on published reports describing anesthetic management in patients with Eisenmenger’s syndrome.

Methods A literature search identified 57 articles describing 103 anesthetics in patients with Eisenmenger’s syndrome. An additional 21 anesthetics were identified in patients receiving regional anesthesia for labor.

Results Overall perioperative mortality was 14%; patients receiving regional anesthesia had a mortality of 5%, whereas those receiving general anesthesia had a mortality of 18%. This trend favored the use of regional anesthesia but was not statistically significant. A better predictor of outcome was the nature of the surgery (and presumably the surgical disease). Patients requiring major surgery had mortality of 24%, whereas those requiring minor surgery had mortality of 5% (P < .05). Patients in labor receiving regional anesthesia had a mortality rate of 24%, and most of these occurred several hours after delivery.

Conclusions This review of anesthesia and surgery in patients with Eisenmenger’s syndrome reveals that most deaths probably occurred as a result of the surgical procedure and disease and not anesthesia. Although perioperative and peripartum mortalities are high, many anesthetic agents and techniques have been used with success.