Porcelain heart

Abstract 

Rheumatic heart disease (RHD) was the leading-cause of death in individual aged 5–20 years a century ago. Developments in diagnosis and treatment, decreased the incidence of RHD and dropped its mortality-rate to less than 10% since the 1960s. Despite the existence of proven preventive strategies in early detection and management of rheumatic fever (RF), RHD remained the most common cause of cardiovascular-mortality and morbidity in patients with RF. Previous studies have showed that Jones criteria may have insufficient support to diagnose patients with RF. Patients with subclinical, ongoing, and unrecognized episodes of RF may present late to medical attention with complication of RF such as indolent carditis. Recent studies revealed the superior role of echocardiography, as compared with clinical screening to diagnose subclinical RHD. While valvular involvement and ventricular dysfunction of RHD can be easily detected with echocardiography and magnetic resonance imaging (MRI), it remains problematic to determine the presence of whether there is myocardial-calcification after rheumatic heart carditis and if yes, how much extent it involves. The current case-report suggests the superior role of Computed tomography angiography (CTA), as compared with echocardiography and MRI, to diagnose RHD in individuals without known history of RF. CTA with high spatial-resolution accurately evaluates tissue characterization and simultaneous assessment of the anatomy and function of heart and coronaries, and can precisely differentiate RHD from other cause of porcelain heart. The use of CTA in RHD screening provides the opportunity to initiate secondary antibiotic prophylaxis to prevent the poor outcome of rheumatic heart disease.

Keywords: Rheumatic fever, Rheumatic heart disease, Computed tomography angiography