Total unilateral anomalous pulmonary venous connection with intact atrial septum: Diagnostic features and a method of surgical correction

The clinical and hemodynamic findings in a patient with total right anomalous pulmonary venous drainage and an intact atrial septum are presented. This diagnosis was suspected when a large left-to-right shunt entering the right atrium was demonstrated by Kr 85 inhalation tests, but the atrial septum could not be traversed by the catheter. It was confirmed by indicator dilution curves which characterized the abnormal drainage pathways of the right pulmonary veins. In addition, the phono-cardiogram revealed greater respiratory variation in the interval between the two components of the second heart sound than that ordinarily found in patients with atrial septal defects. The diagnostic features of the malformation are discussed in detail and the method of surgical correction which was successfully employed, repositioning of the atrial septum, is illustrated.