Partial anomalous pulmonary venous drainage associated with mitral stenosis with an intact atrial septum: A distinctive hemodynamic syndrome

A case of partial anomalous venous drainage associated with mitral stenosis and intact interatrial septum is presented. The hemodynamic findings consisted of pulmonary hypertension, markedly elevated pulmonary artery wedge pressure and large left-to-right shunt at the atrial level. These findings are distinctive of an intact septum, because in Lutembacher's syndrome there is a tendency for equalization of pressure in the atria and, therefore, the pulmonary artery wedge pressure may be normal or only slightly elevated. The findings of high and low pulmonary artery wedge pressure in different parts of lungs may be additional evidence for presence of partial anomalous venous connection.