Aila V. Velasco, Stephanie D.
Cor triatriatum is a rare cardiac condition involving the left atrium, representing only 0.1-0.4% of all congenital heart diseases. It presents as one of the atrium being divided into two chambers by a fibromuscular membrane and classically appear on the left atrium. We report the case of a 23-year old Filipino female with orofacial cleft, presenting with symptoms of decompensated heart failure. Clinical examination revealed the presence of diastolic murmur, 5/6, with accentuated pulmonary component of the second heart sound. Cardiomegaly was seen on chest x-ray and right ventricular hypertrophy on electrocardiogram. Transthoracic echocardiogram (TTE) shows Cor triatrium sinistrum; dilated left atrium divided by a membrane into a proximal and distal chamber; dilated right atrium; dilated right ventricle with right ventricular hypertrophy; dilated main pulmonary artery; and severe pulmonic regurgitation with severe pulmonary hypertension. TTE should provide accurate and sufficient characterization of cardiac anatomy for optimal diagnosis but because of its rarity, can be missed. A transesophageal echocardiography (TEE) provides excellent resolution and offers invaluable assistance to surgical intervention. TEE was done upon follow up to rule out associated congenital abnormalities which only showed patent ductus arteriosus. Surgery provides a satisfactory early and long term survival. Therefore, accurate evaluation of cor triatriatum is the key. Our report highlights early diagnosis and the utility of transesophageal echocardiography in the diagnosis of such congenital cardiac abnormalities and associated lesions.