Hemodynamic changes during pregnancy can result in cardiovascular decompensation in women with pre-existing
cardiac diseases. Despite optimized medical treatment, some patients with severe structural cardiac abnormalities may need surgical intervention during pregnancy. We describe a woman who presented at 20 weeks of gestation with acute
heart failure due to
cor triatriatum, a rare form of
congenital heart disease. This condition is characterized by a perforated fibromuscular membrane dividing the left atrium into two chambers. The clinical presentation varies from asymptomatic to acute
heart failure depending on the size of the fenestrations in the membrane and the presence of associated cardiac malformations. In our patient, two severely restrictive orifices in a membrane within the left atrium, moderate to severe
pulmonary hypertension and good biventricular function were demonstrated by transthoracic echocardiography. Without surgical resection, the increased blood volume and cardiac output associated with pregnancy could have resulted in cardiovascular decompensation. She underwent urgent corrective open heart surgery with
cardiopulmonary bypass. Perioperative
anesthetic management included prevention of
tachycardia, atrial dysrhythmias and
pulmonary hypertension, close monitoring for and prompt treatment of maternal
hypotension, maintaining euvolemia and good cardiac contractility and avoiding
hemodilution and
hypothermia. These approaches, together with minimizing bypass time, resulted in successful maternal and fetal outcome.