Cor triatriatum with a left upper anomalous pulmonary venous return to the innominate vein

Abstract

Cor triatriatum sinister is an uncommon congenital cardiac anomaly accounting for 0.1% of all congenital cardiac malformations, with an anomalous venous return being a frequently associated condition. We present the case of a 14-year-old girl misdiagnosed as having an atrial septal defect with pulmonary hypertension who actually had a cor triatriatum with a left upper anomalous pulmonary venous return to the innominate vein.

Cor triatriatum sinister with left anomalous pulmonary venous drainage to innominate vein: what to do with the vertical vein?

“…Galoin-Bertail and Gouton reported a 14-year-old girl with CTS with left anomalous pulmonary venous drainage to the innominate vein in whom the left inferior PV connection to the accessory LA chamber was extremely similar to that in our case [4]. In that case, however, they resected the fibromuscular membrane without involving the PV because of the wide communication between the left PV and the accessory LA chamber.…”

“…The basic surgical management for CTS is the resection of the fibromuscular membrane inside the LA, accompanied if necessarily by surgical intervention for concomitant cardiac malformations. In CTS associated with left anomalous pulmonary venous drainage, the necessity of surgical intervention on the VV varies among patients depending on anatomical and hemodynamic evaluation [3][4][5][6][7].…”

“…Cor triatriatum sinister (CTS) represents only 0.1% of all cardiac malformations [1]. Partial or total anomalous pulmonary venous return (PAPVR/TAPVR) is reported as a coexisting disease in 10-33% of patients with CTS [2], but left anomalous pulmonary venous drainage coexisting with CTS is less frequently described [3][4][5][6][7]. CTS with left anomalous pulmonary venous drainage may be hemodynamically similar to obstructive TAPVR when a common pulmonary venous collector (CPVC) is present, and surgical intervention may be urgently needed [3].…”