Rheumatic heart disease is not over: Cardiac cirrhosis and multivalvular sequelae in an endemic setting - A case...

Rheumatic Heart Disease (RHD) remains a major cause of valvular morbidity in endemic regions despite declining incidence in high-income countries. While early disease manifests as acute rheumatic fever, contemporary presentations increasingly reflect advanced structural sequelae. Congestive hepatopathy and cardiac cirrhosis secondary to chronic right-sided failure remain under-recognized manifestations of advanced rheumatic valvular disease.

To describe three cases of advanced rheumatic multivalvular disease presenting predominantly with ascites and hepatic congestion and to integrate these observations with current literature on pulmonary hypertension, right ventricular remodeling, and cardiac cirrhosis.

We conducted a retrospective case series at a tertiary referral center in North-India. Inclusion criteria comprised adult patients with echocardiographically confirmed rheumatic valvular disease, preserved left ventricular ejection fraction (≥50%), right-sided heart failure manifestations, and evidence of congestive hepatopathy. Comprehensive clinical, echocardiographic, laboratory, and hepatic Doppler assessments were performed. A structured literature review contextualized the findings.

Three middle-aged women (46-52 years) presented with progressive dyspnea and tense ascites. All demonstrated severe left-sided rheumatic lesions (mitral stenosis or regurgitation), severe functional tricuspid regurgitation, pulmonary hypertension (PASP 55-64 mmHg), and preserved left ventricular systolic function (LVEF 58-62%). Marked left atrial enlargement (indexed volume 68-82 mL/m²) and varying degrees of right ventricular dysfunction (TAPSE 13-16 mm) were observed. High serum-ascites albumin gradients and hepatic Doppler abnormalities confirmed post-sinusoidal portal hypertension consistent with stage II-III congestive hepatopathy. All patients responded to diuretic therapy but were unsuitable for percutaneous intervention due to advanced rheumatic morphology and were referred for surgical valve replacement and tricuspid repair.