Cor-triatriatum dexter with pulmonary hypertension in a neonate

Abstract

Cor-triatriatum dexter is an extremely rare congenital anomaly, in which right atrium is divided into 2 halves by a septum giving rise to 3 atrial chambers. We report here a case of cor-triatriatum dexter who presented at 3 days of life with tachypnea and did not respond to antibiotics. Echocardiography revealed ostium secundum atrial septal defect (ASD) with right to left flow, intact interventricular septum (IVS), severe tricuspid regurgitation with pulmonary arterial hypertension and a membranous structure dividing right atrium into 2 halves with good biventricular function. The patient improved with sildenafil and anti-failure medication. This case of cor-triatriatum dexter presenting with pulmonary hypertension, tricuspid regurgitation and ASD added to the new spectrum of cor-triatriatum dexter.

Schinzel-Giedion syndrome: a case with sacrococcygeal teratoma and cor-triatriatum dexter

“…He was however asymptomatic on cardiovascular evaluation. Cor-triatriatum dexter is an extremely rare congenital heart defect which represents approximately 0.1% of all congenital heart anomalies [ 10 , 11 ]. In this anomaly, the right atrium is divided into two by a septum, resulting in three atrial chambers.…”

“…Cor-triatriatum dexter is believed to result from lack of normal regression of the embryonic right valve of the sinus venosus [ 10 ]. Mild septation of the right atrium is often asymptomatic and is mostly documented as an incidental finding during surgery to correct other cardiac abnormalities or during echocardiography, although more severe septation can cause right sided heart failure and elevated central venous pressure due to obstruction of the tricuspid valve, the right ventricular outflow tract, or the inferior vena cava [ 11 ]. The clinical features in SGS seem to overlap with those seen in children with peroxisomal disorders [ 7 ].…”