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 cross-reaction of antibodies to streptococcal M protein with self-antigens immune-mediated (type II) hypersensitivity disease is characterized by early stage valve regurgitation, most commonly of the mitral valve late stage valve stenosis, most commonly of the mitral valve Associated conditions rheumatic fever Presentation Symptoms palpitations (most common) fatigue chest pain Physical exam may have dyspnea cardiac exam mitral regurgitation holosystolic murmur may have systolic thrill mitral stenosis diastolic murmur following opening snap specific to rheumatic heart disease aortic regurgitation early diastolic decrescendo murmur aortic stenosis crescendo-decrescendo systolic ejection murmur 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 no evidence of carditis for 5 years or until age 21 (whichever is longer) evidence of carditis without valvular abnormalities for 10 years or until age 21 (whichever is longer) evidence of carditis and valvular abnormalitis for 10 years or until age 40 (whichever is longer) to prevent recurrence or worsening of rheumatic heart disease treatment depends on type and severity of valve involvement Medical penicillins indication for all patients in need of prophylaxis sulfadiazine indications for all patients in need of prophylaxis if patients are allergic to penicillin Operative valve repair or replacement indication depending on type and severity of valve pathology modalities surgical repair percutaneous intervention 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