Diagnosis and surgical correction of cor triatriatum in an adult: combined use of transesophageal and contrast echocardiography, and a review of literature

Abstract

Background: Cor triatriatum is rarely found in adults and its diagnosis is mostly dependent on transesophageal echocardiogram. We present a case of an adult male with cor triatriatum and discuss our approach to diagnosis with a review of literature.

Case: A 49-year-old male presented with progressive exertional dyspnea for the past two-and-half years. A transthoracic echocardiogram revealed a thin linear echo-dense structure traversing the left atrium (LA). A transesophageal echocardiogram demonstrated a thin immobile membrane in the LA attached medially to the interatrial septum. No fenestration of the membrane was seen. A swirling spontaneous contrast was noted in the posterior chamber. Color Doppler did not reveal any flow across the membrane. A CT scan of the chest was unhelpful to differentiate extracardiac versus intracardiac origin. Contrast echocardiogram with Optison was performed, which showed a differential opacification of the two atrial chambers and delayed emptying of contrast into true LA establishing communication between the chambers. Left and right heart catheterization were performed with simultaneous pulmonary capillary wedge and left ventricular end diastolic pressure measurement, revealing a mean gradient of 17 mmHg. Surgical correction of the membrane was recommended. Intraoperatively, an orifice of 0.7 cm was identified in an eccentric position with mosaic pattern of continuous turbulent flow across the membrane by color Doppler.

Discussion: Our case demonstrates the impact of contrast echocardiography in a situation where the transesophageal approaches were limited even with the aid of color Doppler.