rheumatic heart disease Flashcards
Major manifestations of RHD
- Joints- Polyarthritis
- Carditis
- Nodules (subcutaneous)
- Erythema Marginatum
- Sydenhams Chorea
Minor
Clinical- Arthralgia and Fever
Laboratory- Elevated ESR/CRP and Prolonged PR
Features of arthritis
- Most common manifestation -70%
- Large joints
- More than one usually affected and migrates
- Respond dramatically to salicylates (if present >48/24 question the diagnosis of ARF)
- Subsides in days to weeks, doesn’t cause permanent damage
Features of Carditis
- Occurs in 50%
- Tachycardia out of proportion of degree of fever
- Heart murmur is always present
- Echo may detect pericardial effusion, increased LV dimension, or impaired LV function - Pericarditis may be present (always occurs with MV involvement)
- Cardiomegaly on CXR indicative of severity of disease
- Signs of CHF indicate severe cardiac dysfunction
- Can cause permanent damage
Features of Erythema marginatum
- Fewer than 10%
- Non pruritic serpiginous or annular erythema
- Mostly on trunk and inner proximal portions of limbs, never on face
- Evanescent- appear when warm, disappear in cold
Features of subcutaneous nodules
- Found in 2-10% of patients and usually in recurrence
- Hard, painless, non pruritic, mobile swellings 0.2-2cm
- Symmetrical, single or clusters on extensor surfaces of small jnts, scalp and spine
- Not transient, last for weeks
- Not exclusive to ARF (10% with rheumatoid arthritis, and SLE)
Features of Sydenham’s Chorea
- 15% of patients
- prepubertal girls >boys
- Neuro- choreic movements and hypotonia
- Psychiatric- Lability, hyperactivity, sep anxiety, Obs/compulsions
- Elevated titres of antineuronal antibodies targeting basal ganglia found in 90%. Levels proportional to severity of chorea
- Gradually subsides in 6-7 months
- Usually doesn’t cause permanent neuro sequelae
What is most reliable laboratory evidence of antecedent streptococcal infection?
Streptococcal antibodies.
Antistreptolysin O titres elevated in 80% of pts with ARF and 20% of normal. A single low ASOT doesn’t exclude ARF.
If 3 Antistrep Abs are obtained (ASOT, Antideoxyribonuclease B, antihyaluronidase) at least 1 is elevated in 95%
How is ARF diagnosed?
Revised Jones criteria.
- Either 2 major, or
- one major and 2 minor + evidence of antecedant strep infection
Absence of evidence of recent strep infection make Dx unlikely
Stills murmur often mistaken for MR
differentials for ARF
- JRA (involvement of peripheral small joints, symmetrical involvement of large joints without migration, pallor, indolent course, no recent strep or response to salicylates)
- Other collagen vascular disorders (SLE); infective and reactive arthritis including post strep arthritis; serum sickness
- Virus associated acute arthritis (Rubella, parvo, Hep B, herpes, entero) more common in adults
- Haematological disorders; sickleamia and leukaemia
When suspecting ARF on history and exam, what investigations are warranted?
FBP, CRP, ESR, throat swab, ASOT (and a second Ab titre), CXR, ECG
Cardiology consult for ECHO and doppler
How is ARF treated acutely?
- Benzathine penicillin G (erythromycin in penicillin allergy)
- Anti-inflammatory with salicylates or steroids must not be started until definitive diagnosis
- Education and continued streptococcal prophylaxis
- Bed rest until the ESR is normalized (unless CHF present)
How is CHF treated in ARF?
- Complete bed rest with orthopnoeic position and moist cool O2
- Prednisone with severe carditis of recent onset
- Digoxin with caution
- Frusemide if indicated
How is Sydenhams treated?
- Reduce physical and emotional stress
- Benzathine penicillin G (to prevent valvular disease in isolated SC)
- Anti-inflammatory agents are NOT needed with isolated SC
- In severe cases; Phenobarb, haloperidol, Valproate, Chlorpromazine, diazepam or steroids.
- Plasma exchange and IVIg work better than steroids
What determines prognosis in ARF
- Cardiac status at start of Rx
- Recurrence of RF. Severity of valvular disease increases each time
- Regression of heart disease; in 10-25% of pts cardiac involvement disappears after 10 yrs from initial attack. PROPHYLAXIS