Background: 

Chronic rheumatic heart disease (RHD) is the major sequel of healing of acute rheumatic fever (ARF), which often results in crippling valvular deformities. The patients are usually young and present with features of mitral and/or aortic valvular dysfunctions. However, at times, the disease may remain asymptomatic and may be seen as incidental findings at autopsy or may even be a cause of sudden cardiac death. 

 

Material and Methods: 

A retrospective 20-year (2000-2019) autopsy data of chronic RHD was reviewed and patients, in whom the valvular deformities had been incidental autopsy findings, were selected. The clinical details of these patients were correlated with morphology of the affected valves. On this pathological analysis, the patients were assigned to a category of stenosis with or without regurgitation and regurgitation. Also the cases were analyzed on the basis of subtle or significant valvular deformity and their clinical diagnosis.

Results: 

Among 475 cases of chronic RHD identified at autopsy in the study period, the disease was diagnosed incidentally in 69 patients (14.5 %). Significant valvular deformity was noted in 61 cases while others had subtle valvular deformity. The most common cause of death was cardiac failure (39 patients, 56%). 11 cases had experienced sudden cardiac death. Amongst the undiagnosed cases, only 5 were clinically diagnosed with cardiac pathology while others had non cardiac overwhelming diseases which most commonly included acute febrile illness (most commonly pneumonia or fever of unclear etiology). The most common misdiagnosis was pulmonary thromboembolism and cardiomyopathy.

Conclusion: 

Our study indicates that mortality and morbidity due to RHD is under-determined. Patients remain undiagnosed due to either insignificant valvular involvement, clinically silent in the presence of significant valvular deformity, presence of other overwhelming diseases or misdiagnosis partly due to resemblance with other pathologies.