Septum primum malposition defect: a rare congenital anomaly

Abstract

Displacement of the septum primum-leftward in atrial situs solitus or rightward in atrial situs inversus is termed as septum primum malposition defect. It appears to be responsible for the anomalous pulmonary venous drainage. This abnormality occurs predominantly in patients with visceral heterotaxy, usually with polysplenia, or rarely with asplenia or a normally formed spleen. Poor development and absence of septum secundum are considered to be responsible for the malposition of septum primum. Transthoracic echocardiographic recognition of the displacement of septum primum facilitates surgical management. In this article, we present an 18-year-old male case of an echocardiographic diagnosis of septum primum malposition defect and its repair technique.