Rheumatic heart disease: 15 years of clinical and immunological follow-up

Roney O Sampaio, Kellen C Fae, Lea MF Demarchi, Pablo MA Pomerantzeff, Vera D Aiello, Guilherme S Spina, Ana C Tanaka, Sandra E Oshiro, Max Grinberg, Jorge Kalil, Luiza Guilherme

Heart Institute (InCor), University of São Paulo, Brazil

Abstract: Rheumatic fever (RF) is a sequel of group A streptococcal throat infection and occurs in untreated susceptible children. Rheumatic heart disease (RHD), the major sequel of RF, occurs in 30%–45% of RF patients. RF is still considered endemic in some regions of Brazil and is responsible for approximately 90% of early childhood valvular surgery in the country. In this study, we present a 15-year clinical follow-up of 25 children who underwent surgical valvular repair. Histopathological and immunological features of heart tissue lesions of RHD patients were also evaluated. The patients presented severe forms of RHD with congestive symptoms at a very young age. Many of them had surgery at the acute phase of RF. Histological analysis showed the presence of dense valvular inflammatory infiltrates and Aschoff nodules in the myocardium of 21% of acute RHD patients. Infiltrating T-cells were mainly CD4+ in heart tissue biopsies of patients with rheumatic activity. In addition, CD4+ and CD8+ infiltrating T-cell clones recognized streptococcal M peptides and cardiac tissue proteins. These findings may open the possibilities of new ways of immunotherapy. In addition, we demonstrated that the surgical procedure during acute phase of the disease improved the quality of life of young RHD patients.

Keywords: rheumatic heart disease, Streptococcus pyogenes, heart failure, inflammatory infiltrate, T lymphocytes, molecular mimicry