All issues > Volume 26(11); 1983

Original Article

J Korean Pediatr Soc. 1983;26(11):1070-1080. Published online November 30, 1983.

A Clinical Study of Cor Triatriatriatum.

Jae Ho Lee¹, Il Suck Chang¹, Nam Su Kim¹, Yong Soo Yoon¹, Chang Yee Hong¹

¹Department of Pediatrics，College of Medicine, Seoul National University

Abstract

Cor triatriatum is a rare congenital cardiac anomaly in which the left atrium is divided into two chambers by an abnormal fibro-muscular septum with, a small opening or not, product pulmonary venous obstruction and pulmonary hypertension. Three cases of cor triatriatum were observed at the Dept, of Pediatrics, Seoul National University. Each patient had no specific problem during early infancy, and presented with a history of frequent upper respiratory infection and dyspnea on exertion, later. The clinical symptoms and signs were similar each, other. The common electrocardiographic finding was right vent ricular hypertrophy without anlartralgeiement. Plain radiograph of the chest showed variable heart size from normal to moderate cadiromegaly. Left atrial enlargement wa not sconfirmed n all cases. Pulmonarysvenous congestion was noted in 2 case. Echo cardiographic studies revealed the abnormal membraneous septum with atrial septal defect in tow cases. This non-invasive technic has been found useful as a important complementary method for the investigation of cor triatriatum, because the early and correst preoperative diagnosis of cor triatriatum was related to the prognosis closely, and it can be even be performed in seriously ill patientseasily. Hemodynamic studies revealed aconsiderable increase in the pulmcfiiiry atrial pressure in all cases. In one case, the pulmonary wedge pressure was increased. (24 mmHg systolic and 15 mmHg diastolic). In two cases, we could measure the pressure of left atrium and accessory chamber, which revealed normal pressure through the atrial septal defect. Left ventricular end-diastolic pressure was Rormal. Angiocardiography was performed in two cases, and in each the intra-atrial diaphragm was demonstrated clearly. The single opening in the intra-atrial diaphragm was demonstrated in one case. The combined anomaly of the heart were atrial septal defect and P.D.A.. We thought that atrial septal defect should be contributed to releave the pulmonary venous obstruction. Removal of the left atrial diaphragm was successful in two cases. The third case willjbe operated.

Keywords :Congenital Heart Disease; Cor triatriatum.