Co-existence of congenital and rheumatic heart disease in 404 post-mortem cases—comparison with findings in 2,352 cases with isolated rheumatic disease

Abstract

SummaryWe reviewed 2,756 necropsy studies of patients who died because of rheumatic heart disease. The anatomic material was divided into two groups. The first group was made up of 404 cases (14.6%) having the association of rheumatic and congenital heartdisease. The second group comprised 2,352 cases with isolated rheumatic heart disease. The analysis of the site and types of congenital malformations in the first group showed 160 cases (39.6%) with aortic, 112 (27.7%) with pulmonary, 15(3.7%) with tricuspid and six (1.5%) with mitral lesions. Twenty patients (4.9%) had an atrial septal defect whilenine (2.2%) had patency of the arterial duct and four (1 %) a ventricular septal defect. The incidence of rheumatic aortic valvar disease in cases also having congenital lesions of the aortic valve (83.7%) was higher than in patients with isolated rheumatic involvement of the aortic valve (67.7%) (p<0.001). The difference in the incidence of rheumatic lesions found in patients with congenital pulmonary lesions (9.8%) and in the patients with isolated rheumatic disease (3.3%) was also significant (p<0.001). No differences were observed in rheumatic lesions to the mitral and tricuspid valves in those with or without associated congenital lesions. In patientswith an atrial septal defect, there was a higher incidence of combined mitral and tricuspid lesions (35% vs. 18%, p<0.02). Patients with patency of the arterial duct andco-existing rheumatic heart disease had a greater incidence of isolated rheumatic aortic disease (11% vs. 0.7%), and also of combined aortic and mitral lesions (33% vs. 17%), but the differences were not significant. The incidence of involvement of all four valves was 5.4% in those with associated congenital lesions and 3% in those in which rheumatic involvement was isolated (p<0.O2). We conclude that rheumatic heart disease has a tendency to involve preferentially cardiac valves that are the sites of congenital malformations.