Rheumatic Heart Disease – At A Glance

It was one of the major causes of death in 1960s, but now the incidence is declining, even though it prevails more in developing countries.
Importance of this disease lies on the risk of developing recurrent attacks which can lead to heart failure, therefore prophylaxis is a crucial aspect of this disease

Pathogenesis

It is not fully understood. 

Infection with Lancefield group A beta haemolytic streptococci (Pharyngitis / Skin infection)
These bacteria consist of M proteins
                    ↓
Antibodies are formed against these M proteins
                    ↓
These antibodies cross reacts with joints, heart, skin and brain 
                    ↓
Leading to development of symptoms

Risk Factors

  1. Overcrowing
  2. Poor hygiene
  3. Illiteracy – Delay in seeking medical care 
  4. Low socioeconomic status
  5. Genetic susceptibility 



Modified Jones Criteria

A patient is high likelihood of having RF, if the patient fulfills the following criteria

  • Essential criteria (EC)
  • EC + Two major criteria or 
  • EC + One major + Two minor criteria
 
Essential Criteria
  • Evidence of recent streptococcal infection 
    • Hstory of scarlet fever
    • Positive throat swab 
    • Rising or increased antistreptolysin O titre (ASOT) and 200 U/mL or AntiDNase B titre
Major Criteria
  • Arthritis
  • Carditis
  • Chorea
  • Subcutaneous nodules
  • Erythema marginatus 
 
Minor Criteria
  • Fever
  • ESR, CRP highly raised
  • Athralgia 
  • Prolong PR interval


Clinical Presentation
  1. Incidence : 5 – 15 years of age
  2. Retrospective history : Sore throat or skin infection 1 – 5 weeks prior to onset of symptoms
  3. Painful, swollen joints  
  4. Fever
  5. Carditis – SOB on exertion, swelling of legs (Features of heart failure)
  6. Other symptoms of the above mentioned criteria can also be present
 
Arthritis
  • Commonest and earliest presentation
  • Migratory / flitting arthritis
  • Large joint involvement – Knee, ankles, wrist and elbows
  • Inflammation present
  • One or two joints involved at a same time lasting for few hours 
 
Carditis
  • Most serious and second commonest manifestation
  • Causes pancarditis (Pericardium, myocardium and endocardium)
  • Endocarditis leads to valvular problems 
  • Commonly affect mitral valve
  • Acute disease – MR
  • Chronic disease – MS 
  • AR – Common in young adults and older children
  • Characteristic murmurs of RF
    • Carey Coombs’ murmur – Mid diastolic murmur 
    •  Austin Flint’s murmur – Early diastolic murmur of AR
  • Aschoff bodies – Pathognomonic lesion found in severe carditis 
 
Subcutaneous Nodules
  • Rare presentation
  • Seen on the extensor surface of elbows, knees, ankles…
  • Firm, painless lumps, < 2cm in size
 
Chorea
  • Also known as Sydenham’s chorea and ‘St Vitus’ Dance’
  • Late presentation (6 months after onset of the disease)
  • More common in females
  • Presentation – Impaired writing, speech, emotional lability, abnormal involuntary movements 
  • Usually associated with carditis
 
Erythema Marginatum
  • Pale red macules or papules between 1 – 3cm in diameter on the trunk and proximal limbs
  • Exacerbated by heat and reduced when the patient is cool
  • Presents early and lasts even after resolution, but it is a rare presentation


Investigations
  1. FBC – Neutrophlic leukocytosis
  2. CRP, ESR – Elevated 
  3. ASOT < 200U/mL is significant | Anti DNase B
  4. ECG – PR interval
  5. Chest X-Ray – Cardiomegaly, pulmonary oedema (Heart failure)
  6. ECHO 
 
 
Treatment
  1. Primary prophylaxis to eradicate any organisms still present 
    • Oral penicillin for 10 days 
    • Erythromycin for penicillin allergy 
  2. For arthritis
    • Aspirin (High Doses) 
    • But if the patient is having moderate to severe carditis – Substitute prednisolone for aspirin 
      • Because aspirin increases BMR and fluid retention 
  3. For carditis
    • Prop up the patient
    • Oxygen 
    • Frusemide IV
  4. For chorea
    • Haloperidol / diazepam 
  5. Symptomatic management 
    • Fever – Acetaminophen 


Complications
  1. Carditis
  2. Mitral stenosis
  3. CCF


Secondary Prophylaxis / Prevention
  1. Without carditis
    • Continued for at least 5 years or until age 21 years, whichever is longer
  2. With carditis, but no valvular involvement
    • Continued for 10 years or well into adulthood, whichever is longer
  3. With carditis and valvular involvement / with valve surgery 
    • Continued for life (At least ten years or until age 40 years)
 
IM Benzathine Penicillin is given for secondary prophylaxis.
Every 4 weeks – If there is no associated carditis
Every 3 weeks – If there is associated carditis
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skymd
1 year ago

fgujy