Updated: 2/1/2018

Rheumatic Heart Disease

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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
  • Clinical definition
    • 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
    • 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
  • Prognosis
    • the early stage may last for years and may be asymptomatic
    • onset of symptoms usually occurs 10-20 years after acute 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
  • Making the diagnosis
    • based on clinical presentation and confirmed with echocardiography
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
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
 

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Questions (3)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(M2.CV.15.36) A 15-year-old girl presents with four days of malaise, painful joints, nodular swelling over her elbows, low-grade fever, and a rash on her chest and left shoulder. Two weeks ago, she complained of a sore throat that gradually improved but was not worked up. She was seen for a follow-up approximately one week later. At this visit her cardiac exam was notable for a late diastolic murmur heard best at the apex in the left lateral decubitus position with no radiation. Which of the following is the best step in the management of this patient? Review Topic | Tested Concept

QID: 104042
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Penicillin therapy

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NSAIDS for symptomatic relief

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Aortic valve replacement

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Mitral valve repair

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Reassurance that this is a benign murmur and send home

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L 3 A

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(M2.CV.15.4662) A 21-year-old Cambodian patient with a history of rheumatic heart disease presents to his primary care physician for a routine check-up. He reports being compliant with monthly penicillin G injections since being diagnosed with rheumatic fever at age 15. He denies any major side effects from the treatment, except for the inconvenience of organizing transportation to a physician's office every month. On exam, the patient is found to have a loud first heart sound and a mid-diastolic rumble that is best heard at the apex. Which of the following is the next best step? Review Topic | Tested Concept

QID: 107050
1

Stop penicillin therapy

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(2/20)

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Stop penicillin therapy in 4 years

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Decrease frequency of injections to bimonthly

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Switch to intramuscular cefotaxime, which has fewer side effects

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5

Continue intramuscular penicillin therapy

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