Situs inversus, subaortic and subpulmonic stenosis, ventricular septal defect, and single coronary artery

Abstract

The unusual occurrence of situs inversus totalis, ventricular septal defect, hypertrophic subaortic and subpulmonic stenosis, and single coronary artery in a 38-year-old man is presented. The clinical course was remarkably mild, as documented by data from 23 years of study including four cardiac catheterizations. At age 35 years, however, syncope, chest pain, and marked elevation of right ventricular pressure prompted complete surgical repair of the left and right ventricular outflow tract obstructions and closure of the septal defect. Three years after surgery the patient continues to lead an active life without symptoms. The unusually mild course can be attributed to the natural banding effects of the subpulmonic stenosis which prevented irreversible pulmonary hypertension.