Snapshot A 30-year-old woman presents to her physician’s office for palpitations. She reports that she previously was diagnosed with group A streptococcal pharyngitis as a child and was suspected to have rheumatic fever. She took antibiotics for it, but she was subsequently lost to follow-up. On physical exam, there is a holosystolic murmur at the apex, suspicious for mitral regurgitation. She is sent for further imaging to confirm the diagnosis. Introduction A consequence of rheumatic fever characterized by inflammation and scarring of the heart valves Epidemiology Demographics female > male most common in developing nations leading cause of pediatric heart disease Location mitral valve > aortic valve > tricuspid valve most commonly affects the high-pressure valves Risk factors poverty and overcrowding recurrent acute rheumatic fever group A streptococcal pharyngitis ETIOLOGY Microbiology at least 1 episode of acute rheumatic fever from group A streptococci Pathogenesis cumulative inflammation and scarring of the heart valves resulting from an abnormal immune response to group A streptococci molecular mimicry between streptococcal M protein and cardiac proteins login to view 2 more bullets disease is characterized by early stage login to view 1 more bullet late stage login to view 1 more bullet Associated conditions rheumatic fever Presentation Symptoms palpitations (most common) fatigue chest pain Physical exam may have dyspnea cardiac exam mitral regurgitation login to view 2 more bullets mitral stenosis login to view 2 more bullets aortic regurgitation login to view 1 more bullet aortic stenosis login to view 1 more bullet Imaging Echocardiography indications when the murmur auscultated on examination is suspicious for rheumatic heart disease to confirm diagnosis findings valvular abnormalities, including regurgitation or stenosis Studies Labs ↑ anti-streptolysin O (ASO) titers Histology Aschoff bodies (granulomas with giant cells) on heart valves Differential Infective endocarditis distinguishing factors no association with group A streptococcal infection other findings including Roth spots, Osler nodes, Janway lesions, and splinter hemorrhages on nail bed vegetations seen on valves on imaging Diagnosis Making the diagnosis based on clinical presentation and confirmed with echocardiography Treatment Management approach prophylaxis all patients with rheumatic heart disease should undergo prophylaxis with penicillin for the specified time period below login to view 3 more bullets to prevent recurrence or worsening of rheumatic heart disease treatment depends on type and severity of valve involvement Medical penicillins indication login to view 1 more bullet sulfadiazine indications login to view 2 more bullets Operative valve repair or replacement indication login to view 1 more bullet modalities login to view 2 more bullets Complications Aortic regurgitation Cardiac arrhythmias left atrial dilation and atrial fibrillation Heart failure Prognosis The early-stage may last for years and maybe asymptomatic Onset of symptoms usually occurs 10-20 years after acute rheumatic fever