SESSION TITLE: Pulmonary Vascular Disease Cases II
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Monday, October 26, 2015 at 03:15 PM - 04:15 PM
INTRODUCTION: Cor triatriatum sinistrum (Cor = heart, Tri = three, Atriatum = atria, sinistrum = left) is a congenital anomaly in which left atrium is divided into 2 chambers by a fold of tissue, usually a membrane. The subtotal variant consists of a small accessory proximal chamber draining part of pulmonary venous return and the rest is drained into distal main left atrium. A flow gradient is created in the left atrium across the membrane leading to pulmonary venous hypertension.
CASE PRESENTATION: 52 year-old African American lady with history of diabetes mellitus, morbid obesity and obstructive sleep apnea presented for progressive dyspnea and episodes of near-syncope. Transthoracic echocardiogram revealed normal left ventricular size and function with right atrial and right ventricular enlargement and severe tricuspid regurgitation. Pulmonary artery systolic pressures (PASP) were estimated to be 105 mmHg. This study was very limited due to poor acoustic windows. CT angiogram was performed to rule out pulmonary embolism. The CT scan showed partially divided left atrium with proximal chamber draining right pulmonary veins and the main chamber draining left pulmonary veins as depicted in the image. Right heart catheterization showed PASP of 117 mmHg. Other causes of pulmonary hypertension were ruled out. Patient was referred for further evaluation for septoplasty but unfortunately passed away due to intracranial hemorrhage.
DISCUSSION: Cor triatriatum sinistrum usually presents in pediatric population and may be associated with other congenital cardiac anomalies. Multiple anatomical variants have been noted. This presentation at such an advanced age has not been reported in literature. Treatment options include balloon dilatation of defect in the membrane or surgical excision of the dividing membrane. Due to severe pulmonary hypertension, complex anatomy and late diagnosis, chances of surgical correction and resolution of pulmonary hypertension were extremely low in our case.
CONCLUSIONS: Subtotal cor triatriatum sinistrum is a rare cause of severe pulmonary hypertension. Treatment is surgical correction if feasible. If untreated and associated with severe pulmonary hypertension, the disease can be fatal.
Reference #1: Villa E, Le Bret E, Batisse A, Vouhé P. Obstructed right pulmonary venous drainage and ipsilateral lung hypoplasia. Eur J Cardiothorac Surg. 2004;25(5):899-901.
Reference #2: Sivakumar K, Satish R, Tailor K, Coelho R. Transcatheter management of subtotal cor triatriatum sinister: a rare anomaly. Pediatr Cardiol. 2008;29(4):812-5
DISCLOSURE: The following authors have nothing to disclose: Sandeep Chennadi, Sikander Zulqarnain
No Product/Research Disclosure Information