Fallot씨 사징, 반상심내막 섬유증과 신위축증 및 고혈압을 수반한 안전내장 전위증

Abstract

to have situs inversus totalis including dextrocardia. and tetralogy of Fallot. A surgical palliation was tried, but it was found to be of little avail. Post-mortem examination revealed the following: All thoraco-abdominal viscera and vessels were in the mirror-image position of the normal. The heart was in the right hemithorax in the mirror -irnage position of the normal, the cardiac axis directing right-lateralwards. The heart was globular in shape, enormously large, and weighed 500 gms. On section, the heart revealed tetralogy of Fallot in dextrocardiac position, including marked pulmonary stenosis of infundibular type with hypoplasia of pulmonary artery, marked hypertrophy of left-sided right ventricle (venous ventricle), large interventricular septal defect at the pars membranacea, and sinistroposition and over-riding of the aorta. There was a large 6X3cm. localized elongatedrhomboid area of endocardial thickening in the rightsided left ventricle(arterial ventricle), that revealed microscopically markedly increased noninflamed loose 171 fibrous connective tissue with some elastic fibers extending into the adjacent and underlying myocardium. Multiple small fenestration of the aortic valve leaflets and marked dil-atation of bilateral bronchial arteries in the mirror image position were observed. An aberrant parathyroid gland was observed over the trachea, 1.5 em. distal to the right lower pole of the thyroid gland. The right and left kidneys weighed 120 gms, and 100 gms, respectively, and showed bilateral diffuse but mild arteriolar nephrosclerosis. Clinically this patient had mild hypertension. The aorta and its main branches showed marked atherosclerosis. There were moderate generalized passive congestion of all viscera and clubbed fingers and toes. A small confluent nodular lesion of fibrocaseous tuberculosis that was partly calcified was observed in the right upper lobe of the lungs. This is believed to be the first autopsy case of congenital anomaly of the heart of this type reported in the Korean literature.