Total pulmonary venous drainage through a persistent left superior vena cava.

When Parsons et al. (1952) reported two patients with total pulmonary venous drainage into the right heart through a persistent left superior vena cava and the left innominate vein they were able to find accounts of only nine other patients, six of whom were babies diagnosed at autopsy. Another, although only diagnosed in life as having abnormal pulmonary venous drainage into the right superior vena cava by Johnson and McRae (1948), had a teleradiogram similar to those now thought to be characteristic of total pulmonary venous drainage through a persistent left superior vena cava. Two others, reported by Friedlich et al. (1950), were the first patients with this congenital anomaly fully diagnosed in life. Adding Taussig's (1947) Case 33, the four patients of Snellen and Albers (1952), and the three patients of Gardner and Oram (1953) the number of published cases is nineteen. From these records it is apparent that before the advent of angiocardiography and cardiac catheterization this congenital anomaly was recognized only at autopsy. Following the introduction of these special methods of investigation it was possible to diagnose the anomaly in life and to recognize it more frequently. The patients reported by Taussig (1947), Parsons et al. (1952), Snellen and Albers (1952), and Gardner and Oram (1953) have similar teleradiograms in which the striking feature is a " cottageloaf" shaped cardiovascular shadow, formed by an ovoid upper mediastinal mass and an enlarged heart. The two latter groups of observers demonstrated by angiocardiography that the ovoid mediastinal enlargement is produced on the left by a persistent left superior vena cava and on the right by the dilated right superior vena cava, and they supported Taussig's (1947) suggestion that the cardiovascular contour provides a distinctive radiological sign of total pulmonary venous drainage through a left superior vena cava into the left innominate vein. Brown (1950), who was already aware of the radiological appearance of a persistent left superior vena cava, independently recognized the significance of these distinctive radiological features and diagnosed this anomaly on six occasions at routine clinical and radiological examination of patients in cardiac clinics in Hull and in the Sheffield Region. Three patients (Cases 4, 5, and 6) were already under observation but the others (Cases 1, 2, and 3) were seen for the first time early in 1953, which suggests that this anomaly is not so rare as the published cases indicate. When it is widely known that the radiological features are sufficiently characteristic to allow the anomaly to be recognized at routine examination there will probably be an increase in the frequency of its diagnosis similar to that following the introduction of the special methods of investigation.