Abstract
Introduction
Cor triatriatum sinistrum is a rare congenital condition, diagnosed in 0.4% of cases at autopsy, in which the left atrium is divided in two chambers by a fibromuscular membrane. In 80% of the cases it is associated with other cardiac abnormalities, the most common being ostium secundum atrial septal defect.
Case report
A 71-year-old man with a history of congestive heart failure was admitted to the hospital for dyspnoea, fatigue and ankle swelling. The physical examination revealed jaundice, severe pretibial oedema, bibasal crackles, irregular heart sounds, a 3/6 mitral and tricuspid systolic murmur, jugular vein distension, hepatomegaly. Electrocardiographical findings included atrial fibrillation and right bundle branch block. Transthoracic echocardiography showed severely enlarged right cavities, an ostium secundum atrial septal defect of 23 mm with right to left shunt, a dilated left atrium divided by a membrane and severe pulmonary arterial hypertension. Cardiac magnetic resonance imaging revealed nodular late gadolinium enhancement (LGE) areas and confirmed ostium secundum atrial septal defect and also a fibromuscular membrane, dividing the left atrium into a proximal and distal chamber. Despite appropriate therapy, the patient’s clinical state altered in the following days and he eventually died, due to ventricular fibrillation. The autopsy confirmed the ostium secundum atrial septal defect and the cor triatriatum sinister and also revealed an abnormal emergence of the circumflex artery from the left coronary sinus (Figure).
Conclusion
Cor triatriatum sinistrum and also ostium secundum atrial septal defect are rare abnormalities usually recognized early in lifetime due to unspecified symptoms of heart failure. In adults it can become symptomatic later in time, like in our case, with the development of multiple associated conditions: atrial fibrillation, pulmonary hypertension, left ventricle dysfunction, with a poor prognosis.
Abstract P1341 Figure
Intraoperative 2D and 3D transesophageal echocardiographic assessment of cor triatriatum with ostium secundum atrial septal defect in an adult patient