Natural history of ventricular septal defect with right-sided aortic arch

The natural history of 14 patients with ventricular septal defect and right-sided aortic arch is reviewed. Patients are divided into two groups. Group A consisted of 10 patients, all of whom developed progressive infundibular stenosis; group B consisted of 4 patients, none of whom developed infundibular stenosis. The clinical features, x-rays, electrocardiograms, haemodynamic data, and angiocardiograms in these two groups are compared. The right ventricular cine-angiocardiogram seems to be helpful in differentiating the course of the two groups. The patients who developed progressive infundibular stenosis showed an abnormal right ventricular cine-angiocardiogram even in the absence of obstruction. Various aetiological factors for the development of infundibular stenosis are discussed. The importance of medical management in group A patients is emphasized, even though all of them presented in infancy in congestive heart failure. The role of propranolol in preventing cyanotic spells in group A patients is also discussed.