Acute rheumatic fever and rheumatic heart disease

Abstract

Acute rheumatic fever (ARF), the acute autoimmune sequela of group A Streptococcus infection, and its chronic life-threatening manifestation, rheumatic heart disease (RHD), which causes permanent heart valve damage, remain major drivers of preventable cardiovascular mortality and disability in low-income and middle-income countries. Since the mid-1990s, multidisciplinary and international collaborative research has yielded new insights into the role of superficial skin infections such as impetigo in ARF pathogenesis and the disease mechanisms that underlie the progression from ARF to RHD, and has explored novel treatments and delivery mechanisms in clinical trials and progressed the development of Strep A vaccines. Early detection and patient care have advanced, with revised echocardiographic screening criteria and the 2024 WHO guidelines, which provide a framework for evidence-based management and therapeutic strategies. Notwithstanding these advancements, considerable disparity persists in the care, control and treatment of advanced disease, necessitating coordinated programmes, prioritization by global agencies and local governments, and substantial involvement of those with RHD. Of note, few to no cardiac surgery programmes exist in some countries most burdened by RHD. Future research must encompass implementation studies for validated evidence-based therapies, translational technologies and applications, together with a definitive pathway to direct patient effect, to achieve the WHO target of eliminating RHD as a public health problem.