The Anomalous Relationship of the Inferior Vena Cava and Abdominal Aorta as a Specific Angiocardiographic Sign in Asplenia

Abstract

The visceral anomaly known as agenesis of the spleen (herein referred to as asplenia) has been adequately described (2). The condition is almost universally associated with multiple and complex anomalies of the heart. These generally include common atrium, common atrioventricular valve, common ventricle, transposed great vessels, severe pulmonary stenosis or atresia, and anomalies of the pulmonary veins. The complex nature of the intracardiac malformations usually precludes correction by surgical technics. Nevertheless, it is important to recognize the syndrome of asplenia in order to exclude other forms of cyanotic heart disease which are amenable to surgical repair. Upon review of the forward angiocardiograms in these cases, it became apparent that the relationship between the inferior vena cava and abdominal aorta was anomalous. Because of this preliminary observation, we were motivated to review the angiocardiograms in several other cases to determine whether or not this anomalous relationship was diagnostic of asplenia. To the best of our knowledge, this sign is unreported. Material Forward venous angiograms (from the saphenous vein) were reviewed in 14 cases of asplenia. Ten of the 14 were proved by necropsy, the pathologic aspects appearing in an earlier communication from this institution (2). The remaining 4 cases were documented clinically on the basis of (a) cyanosis, (b) roentgen evidence of hepatic symmetry, (c) Howell-Jolly bodies and Heinz bodies in the erythrocytes as well as numerous erythroblasts, and (d) angiocardiographic evidence of complex cardiac and pulmonary venous anomalies. The thoracic and abdominal components of the aorta showed simultaneous opacification with the inferior vena cava in each of the 14 cases. This phenomenon occurs because of (a) transposition of the great vessels and (b) defects or absence of the atrial and ventricular septa. Results In the 14 cases, forward venous angiocardiography demonstrated the inferior vena cava ascending on the right side of the spine in 10 cases and on the left side in the remaining 4. An anomalous relationship between the upper abdominal aorta and inferior vena cava (IVC) was observed in each case. The aorta, immediately upon leaving the thorax, failed to follow its usual course in the abdomen and descended in the same position as the IVC, regardless of the side occupied by the IVC. The anomalous relationship of the IVC and abdominal aorta was divided into two major types. In Type I (11 cases), the thoracic aorta (regardless of the side of the IVC) descended on the opposite side of the thorax from the IVC (Figs. 1 and 2). The aorta, upon entering the abdomen, angled across the spine toward the IVC, whereupon it descended in the abdomen immediately adjacent to it (Figs. 1 and 2).