Working Problem 3- Rheumatic Heart Disease

Question 1

What is rheumatic fever?

Answer 1

an autoimmune disease following infection with group A streptococci. It affects multiple systems including the joints, the brain, the skin, and the heart.

Question 2

What is the pathophysiology of rheumatic heart disease?

Answer 2

• Antibodies directed against streptococcal M proteins cross-react with tissue glycoproteins in heart, joints and other tissues

Question 3

How do you assess Rheumatic fever

Answer 3

Five manifestations are considered major manifestations of acute rheumatic fever.
• Carditis.(bigger clues and may present with pansystolic murmur due to mitral regurgitation)
• Polyarthritis.(bigger clues)
• Chorea (involuntary abnormal movements)
• Erythema marginatum
• Subcutaneous nodules.
Four manifestations are considered minor manifestations of acute rheumatic fever.
• Fever.
• Polyarthralgia and monoarthritis
• Elevated inflammatory markers
• Prolonged PR interval on electrocardiogram

Question 4

What are the initial test done?

Answer 4

• ESR - >30mm/hr
• CRP - >30mg/L
• WBCC – may be elevated
• ECG – prolonged PR interval
• CXR – chamber enlargement and congestive cardiac failure
• Echocardiogram - may reveal morphological changes to the mitral and/or aortic valves; severity of regurgitation (mitral, aortic, and tricuspid); pericardial effusion if pericarditis present
• Throat culture – beta-haemolytic group A Strep
• Rapid antigen test for group A strep - positive
• Anti-strep serology – above normal range

Question 5

What is the management of rheumatic fever?

Answer 5

• Monoarthritis – unconfirmed rheumatic fever
o Analgesia
• Confirmed rheumatic fever – antibiotic therapy(give penicillin)
o With arthritis – NSAIDs
o With heart failure- diuretics/ACEi, glucocorticoids
o With atrial fibrillation – digoxin
o With valve leaflet or chordae tendinae rupture – assessment for valve replacement
o With severe chorea – anticonvulsants
• Follow up treatment – secondary antibiotic prophylaxis
(penicillin given as intramuscular injection for 10 years or until the child is 21 years old whichever longer

Question 6

What is mitral regurgitation?

Answer 6

most common valvular lesion in the young patients with rheumatic fever

it is characterised by volume overload and dilation of the left ventricle and atrium

Question 7

What are the signs and symptoms of mitral regurgitation?

Answer 7

symptoms-exertional dyspnea and fatigue and can be asymptomatic for years

signs -Pan systolic murmur

Question 8

what are the Investigations done for mitral regurgitation?

Answer 8

ECG(left atrial enlargement and fibrillation)

CXR(left ventricular enlargement and pulmonary venous congestion)

Echocardiography-LV and LA size and function

Question 9

What is the medical management for mitral regurgitation?

Answer 9

echocardiography: every 6-12 months

drug therapy: digoxin,ACEI / ARB diuretics if CCF or AF

Question 10

What are the surgical procedures?

Answer 10

Indications
-moderate/severe regurgitation with symptoms

-asymptomatic severe mitral regurgitation with: LV ejection fraction

Procedure
a. mitral valve repair
b. mitral valve replacement (only in older patients with a heavily calcified valve)
# bioprosthetic valve # mechanical valve (avoid mechanical prostheses if concerns about anticoaglation adherence or future pregnancy)

Question 11

What is mitral stenosis?

Answer 11

less common than mitral regurgitation
is usually seen ~30 years after rheumatic fever
characterised by: left atrial enlargement pulmonary hypertension right ventricular dilatation tricuspid regurgitation

Question 12

Symptoms and signs of mitral stenosis?

Answer 12

Symptoms:
 exertional dyspnoea  		( especially at faster heart rates )
haemoptysis
pulmonary oedema 
palpitations  
arterial emboli

Signs:
low-pitched mid-diastolic “rumble” at apex
signs of pulmonary hypertension

Question 13

What are tests done for mitral stenosis?

Answer 13

ECG:
left atrial enlargement atrial fibrillation

CXR:
left atrial enlargement calcification of mitral valve
pulmonary venous congestion

Echocardiography:
LA size visualisation of mitral valve

Question 14

How do you treat atrial fibrillation which arise as a result of MS?

Answer 14

when paroxysmal, consider cardioversion (beware of risk of systemic embolism)
anticoagulation
rate control with: digoxin
β-blocker
non-dihydropyridine calcium-channel antagonist

Question 15

How do you treat mitral stenosis?

Answer 15

echocardiography: every 6-12 months
pulmonary congestion - diuretics
In general terms, once a patient becomes symptomatic, the treatment of choice is interventional therapy

Indications for intervention
progressive exertional dyspnoea
mitral valve orifice    50 mmHg)
paroxysmal atrial fibrillation
thromboembolism

Question 16

What is the interventional therapy for MS ?

Answer 16

a. percutaneous balloon mitral valvuloplasty(tx of choice)
b. surgical repair of the mitral valve
c. mitral valve replacement

Question 17

How does rheumatic heart disease affect pregnancy?

Answer 17

During pregnancy, blood volume increases 25–100% - exacerbates any pre-existing rheumatic valvular heart disease

Factors that put pregnancy and mother at increased risk: - decreased LV systolic function - significant mitral stenosis(most poorly tolerated - pulmonary hypertension - heart failure ( especially if mother was symptomatic before pregnancy )

Progress should be monitored with regular echocardiography
and tx the CCF with digoxin,diuretics and calcium channel blockers

Question 18

How is acute oedema managed?

Answer 18

Normotensive
Lasix(furosemide)
Morphine
Nitrates(decreases preload by venodilating)
Oxygen
Position(make them sit upright)

Hypotensive
o Intubation and ventilation
o Inotropic support – adrenaline or dopamine