The purpose is to provide a broad overview of the current state of knowledge of pathogenesis, diagnosis, and management of rheumatic heart disease (RHD).
Studies on pathogenesis of RHD have focused on autoimmunity because of molecular mimicry between the streptococcal M antigen α-helical coiled-coil structure and sarcomeric proteins such as myosin and tropomyosin. More recently, nonsarcomeric autoantigens, endothelial injury and the innate immune system have been proposed to play key roles in the pathogenesis of RHD. In the 2015 revised Jones Criteria, the importance of echocardiography and subclinical carditis in the diagnosis of acute rheumatic fever is highlighted. Experimental studies with targeted anti-inflammatory therapeutics have been largely unsuccessful and the only established treatment is still lifelong antibiotics. Efforts to improve patient selection and outcomes with percutaneous mitral balloon valvuloplasty are ongoing. With regard to surgical management, several groups have demonstrated excellent operative and midterm outcomes from valve repair as opposed to valve replacement.
There are still many unanswered questions regarding RHD pathogenesis. The only accepted medical treatment is still long-term antibiotic therapy, whereas advances in mitral repair techniques have led to successful durable repairs being performed in high-volume, expert centers.
aDivision of Cardiac Surgery, St Michael's Hospital
bDivision of Pathology, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada
Correspondence to Bobby Yanagawa, MD, PhD, FRCSC, Department of Surgery, University of Toronto, Division of Cardiac Surgery, St. Michael's Hospital, 30 Bond Street, 8th Floor, Bond Wing, Toronto, ON M5B 1W8, Canada. Tel: +1 416 864 5706; fax: +1 416 864 5031; e-mail: firstname.lastname@example.org