Pulmonary-Esophageal Variceal Bleeding: A Unique Presentation of Partial Cor Triatriatum Sinistrum

Cor triatriatum sinistrum is a rare congenital disorder defined as a division of the left atrium by a diaphragmatic membrane resulting in two left atrial chambers. The membranous division of the atrium can be partial or complete and can affect either atrium, with involvement of the right atrium referred to as cor triatriatum dexter. The presence of fenestrations within the membrane allows for communication and forward passage of blood into the true atrium. Absence of fenestrations leads to early symptomatic engorgement of the lungs. We report the case of a young adult male presenting with recurrent hematemesis due to variceal bleeding. On CT imaging the patient was found to have cor triatriatum sinistrum, with a vertical membrane resulting in total obstruction of the pulmonary venous drainage on the right, with normal pulmonary venous drainage on the left. There was extensive pulmonary-systemic arterial collateralization to the right lung suggesting retrograde filling of the right pulmonary artery with effective flow reversal in the right lung.