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.
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