Co-existent Total Anomalous Pulmonary Venous Drainage into Portal Vein, Drainage of Left-sided Inferior Vena Cava into Left Atrium, and Splenic Agenesis

Abstract

Total anomalous drainage of the pulmonary veins into the portal venous system is rare. Burroughs and Edwards (1960) noted 17 such cases in the 188 examples of total anomalous pulmonary venous drainage which they collected from the literature.Rarer still is the presence of a left-sided inferior vena cava emptying into the left atrium or into the left side of a common atrium. We have traced five such cases.A review of the literature by Gilbert, Nishimura, and Wedum (1958) revealed only 81 cases of coexistent splenic agenesis and cardiac malformation. In three of these the pulmonary veins drained into the portal circulation.The simultaneous presence of all three congenital abnormalities-total anomalous drainage of the pulmonary veins into the portal system, drainage of a left-sided inferior vena cava into the left atrium, and splenic agenesis-must be exceedingly rare. We report such a case, the like of which we have not been able to trace. CASE REPORT T. C. H., a 27-day-old Chinese male infant, was admitted to the General Hospital, Singapore, on 2 May 1964 with a one-week history of breathlessness and cough. He was found to be generally cyanosed: a systolic murmur was heard all over the praecordium, and a few crepitations and wheezes were heard in the lungs. The child died four days after admission. Although congenital heart disease was diagnosed, the nature of the defects was in doubt.NECROPSY The heart measured 4-5 cm. from base to apex; the transverse diameter was 3-8 cm. It was unusually mobile as the intrapericardial portions of the pulmonary veins were absent. Externally, the two atria and their appendages were clearly defined, but there was no interventricular groove.Internally, the heart was seen to consist of two incompletely separated atria and a single common ventricle. The cavity of the left atrium was slightly larger than that of the right. The interatrial septum was incomplete, being represented by a small biconcave dense band of tissue, 0-5 cm. in width at either end and 0-2 cm. in width at the centre, running anteroposteriorly and almost vertically (Figs 1 and 2). This was attached superoanteriorly to the atrioventricular junction and inferoposteriorly to the most inferior portion of the atrial walls. There were thus two elliptical interatrial foramina; one, of the ostium secundum type, 1 8xl1-cm., and a smaller one, of the ostium primum type, 15 x 0 8 cm. (Fig. 1).The right atrium received a right superior vena cava but no inferior vena cava. In the most inferior portion of the medial wall was a small valveless circular opening 0-2 cm. across, into which drained several very small venous channels, which arose from the region of the right atrioventricular groove.The left atrium received a left superior vena cava and left-sided inferior vena cava (Fig. 1). At the junction of the smooth endocardium with the musculi pectinati were several small openings, the largest 0-2 cm. across (Fig. 1). Neither left nor right superior venae cavae received veins from the surface of the heart, nor was there any commu...