Total Anomalous Pulmonary Venous Drainage into the Portal System

Abstract
Many variations of anomalous pulmonary venous drainage are known. Most of these are supradiaphragmatic, the veins emptying into the right side of the heart directly or via interposed vascular channels. These anomalies are generally compatible with life beyond infancy, dependent, of course, on associated defects of the heart and great vessels. Some of the anomalous venous connections can be suspected from the roentgen appearances because of characteristic contours of the heart and great vessels. Pulmonary venous drainage into infradiaphragmatic vessels is far less common than the supradiaphragmatic type, comprising 10 to 20 per cent of all cases of total anomalous pulmonary venous drainage (2,6,8). With rare exceptions, the drainage is into the portal vein or ductus venosus and it is solely with these cases that this report is concerned. From the recorded examples, especially those of Johnson et al. (7), and from personal experience, it is evident that in this group also there is a characteristic roentgenographic appearance which permits accurate diagnosis. In this instance it is the pulmonary vascular pattern rather than the contour of the heart or great vessels which is distinctive. It is our intention here to correlate the abnormal physiology and pathologic anatomy with the roentgen findings in infradiaphragmatic pulmonary venous drainage. Four cases make up the subject material for this report. Three were proved at autopsy; the fourth was diagnosed by angiocardiography. Case Reports Case I: E. L., a 2-month-old white male infant, was admitted to Children's Memorial Hospital because of intermittent cyanosis since the age of three days. The mother had suffered no illness during her pregnancy, and there was a normal two-year-old sibling. The patient had been born spontaneously at full-term. The child had been kept under observation in the hospital and progressed reasonably well for about three weeks, when he was discharged in fair condition. The heart and abdominal organs were found to be reversed. In the interval between his discharge and admission to Children's Memorial Hospital, the mother noted increasing respiratory difficulty during the cyanotic episodes. On admission the child was well nourished but pale, with slightly cyanotic lips and fingers. There was moderate tachypnea with no retractions while in oxygen. The pulse rate was 144 per minute. The lungs appeared clear. The maximum cardiac impulse was on the right. The liver edge was palpated on the left. Roentgenograms of the chest confirmed the dextrocardia. The stomach was on the right. The lung fields showed a marked reticulated appearance (Fig. 1). Angiocardiography was performed through a catheter placed in the right ventricle. The aorta and pulmonary arteries filled promptly, apparently from a single ventricle.