Complete Transposition of the Great Arteries with Large Ventricular Septal Defect and Pulmonary Hypertension: Progressive Pulmonary Vascular Disease after Total Correction

Abstract

The postoperative change in pulmonary vascular disease following complete surgical repair in cases of the complete transposition of the great arteries with severe pulmonary hypertension remains an extremely interesting problem. We have performed complete surgical repair on an 8-month-old boy with severe pulmonary hypertension having a pulmonarysystemic pressure ratio of 1.0 and 14 units of pulmonary vascular resistance, but who suddenly died 9 months postoperatively. The results of a comparison of the histometrical measurements of biopsy lung and autopsy lung showed postoperative hypertrophy of the media of the small pulmonary arteries and progressed pulmonary vascular disease. It is thought that this phenomenon was brought about in the following way: The specific factor in this congenital heart disease which suppresses the hypertrophy of the pulmonary arterial media is removed due to the total correction and the media over-reacts to the postoperative pulmonary arterial pressure, resulting in hypertrophy. Repeated vasoconstriction of the abnormally thick pulmonary arterial walls leads to ischemic change in the peripheral blood vessels and obstructive pulmonary vascular disease is progressively brought about.