Cor Triatriatum with Infracardiac Total Anomalous Pulmonary Venous Drainage / 대한흉부외과학회지

ABSTRACT

We report one case of an 18-day-old female patient, weighing 3.4 kg, with severe cyanosis. The diagnosis was made with only transthoracic echocardiography, which revealed cor triatriatum with an atretic small opening of fibromuscular membrane, obstructive infracardiac total anomalous pulmonary venous drainage(TAPVD), severely restrictive interatrial communication, and scanty mitral inflow and aortic forward flow. The preoperative decision-making for biventricular repair was not easy due to collapsed left heart system caused by remarkably reduced blood flow. An emergent operation was performed due to severe cyanosis. All left heart structures were somewhat hypoplastic but thought to be adequate for systemic circulation. Biventricular repair was done without specific intraoperative problems. The postoperative course was uneventful. The patient has been doing well with no evidence of pulmonary vein stenosis or mitral regurgitation for 4 months after operation.