Juvenile Rheumatic Mitral Stenosis with Multiple Ventricular Septal Defects

Abstract

Congenital heart disease is related to events occurring in the embryonal stage, while rheumatic heart disease is a sequela of immune-mediated damage following streptococcal infection. We report an unusual association of multiple ventricular septal defects and severe pulmonary arterial hypertension with rheumatic mitral stenosis in a 7-year-old girl. This case highlights the need for careful examination for coexisting rheumatic disease in late presentations of congenital heart disease.

Juvenile severe mitral stenosis predisposing Eisenmenger syndrome in a case with ventricular septal defect, patent ductus arteriosus, coarctation of aorta & hypoplastic aortic arch: Report of first case of rare association

“…Among the previous cases, both the cases had undergone surgical correction of VSD and MVR. 5,6 But, in our case surgical correction was not possible due to irreversibility of PAP & PVRI after O2 administration though PTMC could be done as mitral valve was suitable for it. 8 But due to refusal by parents we couldn't do the procedure in our case and she was managed medically only.…”

“…4 There are few case reported in the literature where RHD with severe MS was associated with VSD. 5,6 Another case reported in 1956, which showed association between RHD and Eisenmenger syndrome. 7 But, our case was very peculiar because she had presented with the clinical features Eisenmenger syndrome as the primary Fig.…”

Severe Pulmonary Hypertension From Combined Ventricular Septal Defect and Rheumatic MIitral Valve Disease: A Case Report and Literature Review

“… 30 When septal defects co-exist with valvular heart disease, there is an increases risk of developing pulmonary hypertension and symptoms of heart failure tend to occur at an earlier age. 14 …”

“…Patients with VSD who again develop mitral valve disease are at dual risk of developing severe pulmonary hypertension from both precapillary and post capillary physiologies increasing the risk of adverse outcomes. In sub-Saharra Africa where rheumatic heart disease is common, the association of rheumatic mitral stenosis with atrial septal defect (Lutembacher syndrome) 10 - 14 and that of VSD have also been reported. 14 However, the occurrence of VSD and rheumatic mitral valve disease with isolated regurgitation has not been reported.…”

“…In sub-Saharra Africa where rheumatic heart disease is common, the association of rheumatic mitral stenosis with atrial septal defect (Lutembacher syndrome) 10 - 14 and that of VSD have also been reported. 14 However, the occurrence of VSD and rheumatic mitral valve disease with isolated regurgitation has not been reported. We report a case of a young man with a large VSD and rheumatic mitral regurgitation which became complicated with very severe pulmonary hypertension, paroxysmal atrial fibrillation and death.…”