| Snapshot |
| A 25-year-old woman presents to her primary care physician’s office for her annual physical. She recently immigrated from a developing country and reports having multiple episodes of pharyngitis in the past. On physical exam, there is a holosystolic murmur at the apex. A follow-up echocardiogram shows mitral regurgitation, concerning for rheumatic heart disease. |
| Introduction |
| Clinical definitiona consequence of rheumatic fever characterized by inflammation and scarring of the heart valvesEpidemiologydemographicsfemale > malemost common in developing nationsleading cause of pediatric heart diseaselocationmitral valve > aortic valve > tricuspid valvemost commonly affects the high-pressure valvesrisk factorspoverty and overcrowdingrecurrent acute rheumatic fevergroup A streptococcal pharyngitisEtiologyat least 1 episode of acute rheumatic fever from group A streptococciPathogenesiscumulative inflammation and scarring of the heart valves resulting from an abnormal immune response to group A streptococcimolecular mimicry between streptococcal M protein and cardiac proteins cross-reaction of antibodies to streptococcal M protein with self-antigensimmune-mediated (type II) hypersensitivitydisease is characterized byearly stagevalve regurgitation, most commonly of the mitral valvelate stagevalve stenosis, most commonly of the mitral valvemitral valve stenosis leads to increased end-diastolic left atrial pressure, which causes left atrial enlargementleft atrial enlargement can manifest as atrial fibrillation or dysphagia Associated conditionsrheumatic feverSydenham chorea carditisarthritiserythema marginatumsubcutaneous nodulesPrognosisthe early stage may last for years and may be asymptomaticonset of symptoms usually occurs 10-20 years after acute rheumatic fever |
| Presentation |
| Symptomspalpitations (most common)fatiguechest painPhysical exammay have dyspneacardiac exammitral regurgitationholosystolic murmurmay have a systolic thrillmitral stenosisdiastolic murmur following opening snapspecific to rheumatic heart diseaseaortic regurgitationearly diastolic decrescendo murmuraortic stenosiscrescendo-decrescendo systolic ejection murmur |
| Imaging |
| Echocardiography indicationswhen the murmur auscultated on examination is suspicious for rheumatic heart diseaseto confirm the diagnosisfindingsvalvular abnormalities, including regurgitation or stenosis |
| Studies |
| Labs↑ anti-streptolysin O (ASO) titers Histology Aschoff bodies (granulomas with giant cells) on heart valves Making the diagnosisbased on clinical presentation and confirmed with echocardiography |
| Differential |
| Infective endocarditisdistinguishing factorsno association with group A streptococcal infectionother findings including Roth spots, Osler nodes, Janway lesions, and splinter hemorrhages on the nail bedvegetations seen on valves on imaging |
| Treatment |
| Management approachprophylaxis all patients with rheumatic heart disease should undergo prophylaxis with penicillin for yearsto prevent recurrence or worsening of rheumatic heart diseasetreatmentdepends on type and severity of valve involvementMedicalpenicillinsindicationfor all patients in need of prophylaxissulfadiazineindicationsfor all patients in need of prophylaxisif patients are allergic to penicillinOperativevalve repair or replacementindicationdepending on type and severity of valve pathologymodalitiessurgical repairpercutaneous intervention |
| Complications |
| Aortic regurgitation Cardiac arrhythmias Heart failure |
