Human situs inversus of the thoracoabdominal structures

Abstract

Situs inversus totalis is well known, but its comprehensive description has been rare, especially on the internal view of the heart. For discussions based on recent results in developmental biology, the present study demonstrates a region- or part-specific manner of the inverted morphology found in a male donated cadaver and discusses the pathogenetic mechanisms of situs inversus in the human. Therein, clearly inverted morphologies existed in the coronary vessels, the apex position (dextracardia), connections between the heart and great vessels, the internal view of the atrium, the aortic arch with the three major branches, lung and liver segments and abdominal gastrointestinal tract. However, the ventricular internal view suggested incomplete laterality, such as tricuspid atrioventricular valves for both ventricles. The cardiac conductive system appeared not to be inverted but abnormal. The thoracic aorta and pulmonary artery took an L-spiral position with modifications. The inferior vena cava was located on the right side of the abdominal aorta. However, the left-sided kidney was located superior to the right-sided kidney. Similarly, the testicular vessels did not exhibit a clearly inverted morphology, but were almost normal. Therefore, the posterior mediastinal and retroperitoneal structures appeared to exhibit neutral laterality, incomplete inverted morphology or even normal morphology. According to the personal history and present histology, this specimen was unlikely to correspond to Kartagener's syndrome. The present observations seem to be consistent with recent findings in mutant models of laterality disturbances, in which a single gene or molecule is responsible for the changes in a region-specific manner.