Systemic and pulmonary venous connections in visceral heterotaxy with asplenia. Diagnostic and surgical considerations based on seventy-two autopsied cases.

Abstract

To facilitate the preoperative diagnosis and surgical management of visceral heterotaxy and asplenia, 72 postmortem cases were reviewed with particular attention focused on the systemic and pulmonary venous connections. The superior vena cava was bilateral in 51 cases (71%), but in 9 cases one of the superior venae cavae was partly or totally atretic. Patent bilateral superior venae cavae were found in 42 cases (58%) and the superior vena cava was unilateral in 21 (29%). Although the inferior vena cava was never interrupted, a prominent azygos vein was found in 6 cases (8%). Some hepatic veins drained separately from the inferior vena cava in 20 cases (28%). An intact coronary sinus was rare (2 cases, 3%). Anomalous pulmonary venous connection to a systemic vein was total in 42 (58%) of 72 and partial in 2 (3%) of 72, with obstruction in 24 (55%) of 44. Abnormal pulmonary artery branches (severe hypoplasia, localized stenosis, or discontinuity) were present in 21 (29%), and these obstructive arterial anomalies were associated with a significantly higher prevalence of anomalous pulmonary venous connection (p < 0.01) and of pulmonary venous obstruction (p < 0.01). Cardiac pulmonary venous connections were found in 28 (39%), with the pulmonary veins and the inferior vena cava entering the same atrium in 10 (36%) of 28.