Unit 4:

Diagnosis and Management of

Rheumatic Heart disease
 Course Objectives
• Describe burden of RHD
• Describe pathogenesis of RHD
• Discuss the complications of rheumatic heart
disease
• Discuss the clinical features of RHD
• Describe the methods of diagnosis of RHD
 Introduction
• RHD is inflammation of heart valves that follows infection with
Group A beta hemolytic streptococcus, commonly pharyngitis.
• It is thought that 40-60% of patients with ARF will go on to
developing RHD.
• Rheumatic disease(RHD) is the only long term sequel of ARF
which can lead to death or disability
• Rheumatic carditis affects mainly the heart valves.
• Histopathology may show the Aschoff nodules (the characteristic
lesions of RHD) the myocardium and pericardium clinical
symptoms and signs are mainly due to valvular involvement.
 Pathophysiology of RHD
• Involvement of the valves is the fundamental issue of
RHD which affects mainly the mitral and aortic
valves.
• In most patients RHD remains clinically silent during
which the valve lesion progresses to cause significant
valvular abnormalities due to recurrent attacks of
unrecognized Acute Rheumatic Fever.
• Heart valves normally allow easy forward blood
flow from one chamber of the heart to another by
adequate opening and prevent back flow by
appropriate complete closure.
 Pathophysiology of RHD…
• In patients with RHD permanent damage of the valve
apparatus leads to scarring resulting in poor opening
and closure of the valves.
• If valves fail to properly open to allow adequate
forward flow narrowing (stenosis ) of the valves is said
to have occurred.
• If the valves fail to properly close backward flow or
leakage of blood( regurgitation) will develop.
• Patients can have isolated stenois or regurgitation or
combined stenosis and regurgitation.
 Symptoms of RHD
• Symptoms of RHD may not develop for many years
 A murmur but no symptoms usually suggests mild-
moderate disease
 Symptoms usually suggest moderate-severe disease
• Symptoms depend upon the type and severity of disease,
and may include
 Breathlessness with exertion or when lying down flat
 Waking at night feeling breathless
 Feeling tired
 General weakness
 Peripheral oedema
 Heart valve involvement
Mitral valve is affected in over 90% of cases of RHD
(isolated or combined with other valves)
• Mitral regurgitation most commonly found in children &
adolescents
• Mitral stenosis represents longer term chronic disease,
commonly in adults
• Most common complication of mitral stenosis is atrial
fibrillation
Aortic valve is the next most commonly affected valve.
• Generally associated with disease of the mitral valve.
• Tends to develop as a long term complication of aortic
regurgitation
Tricuspid and pulmonary valves are much less commonly
affected
• Usually affected in very severe RHD when all valves are
affected
 Signs of RHD
Mitral regurgitation
A pansystolic murmur heard loudest at the apex and radiating
laterally to the axilla
Mitral stenosis
A low-pitched, diastolic rumble heard best at the apex with the bell
of the stethoscope and with the person lying in the left lateral
position.
Aortic regurgitation
A diastolic blowing decrescendo murmur best heard at the left
sternal border with the person sitting up and leaning forward in full
expiration.
Aortic stenosis
A loud, low pitched mid-systolic ejection murmur best heard in the
aortic area, radiating to the neck.
Typical Signs of Severe MR
Typical Signs of Severe AR
Typical Signs of Severe MS
Typical Signs of Aortic Stenosis
 RHD Screening Recommendations
• Screening for RHD is recommended for children and young
adults from communities and countries where RHD is endemic.
• We recommend the following sequence for screening: taking a
history for prior ARF, performing echocardiography in all, and
then clinical cardiac evaluation of cases with abnormal
echocardiography.
• Although there are no guidelines for the timing and frequency of
screening, the evidence on the natural history of RHD suggests
that at least two echocardiography-based screening tests are
indicated: one before age 18 and another by 35 years of age.
• Cases of probable or possible RHD require a repeat evaluation
within a year to confirm the diagnosis prior to embarking upon
long-term prophylaxis
 Investigations
• First step is to make sure that there is no rheumatic fever
recurrence or infective endocarditis: ask for fever, joint pains,
palpitations, shortness of breath/cough or leg swelling.
• CBC and ESR
• Electrocardiogram (ECG)
– To check for rate and rhythm
• Chest X-ray (CXR)
– To determine size and placement of heart
– To identify cardiac failure (pulmonary congestion)
• Echocardiography
– To identify heart valve damage
– To estimate severity of disease
– Useful to compare results with future echocardiogram results
 Management of RHD
Principles of Management of RHD Includes:
• Treatment of cardiac and other symptoms
• Long-term secondary prophylaxis (to prevent recurrent
ARF)
• Regular medical and cardiology review including
echocardiography
• Appropriate and timely surgical interventions
• Dental assessment and care
• Family planning referral
• Management of RHD in special situations(e.g . pregnancy)
 Medical Management of RHD
It encompasses the identification and
management of complications of RHD and
management of patient after cardiac
interventions:
– Heart failure
– Atrial fibrillation
– Infective endocarditis
– Stroke
– Post cardiac intervention care
 Surgery for RHD
The need for surgery depends on
– Severity of symptoms
– Evidence that the heart valves are severely damaged
– Left ventricular chamber size and function
– Availability of long-term management after surgery (i.e.
anticoagulation)
Heart valves can be repaired or replaced
Assessment before surgery includes
– Echocardiogram to assess severity of heart valve damage
– Complete dental assessment and treatment (if required)
– Review and management of other health problems (e.g. Kidney
disease, vascular and chronic respiratory disease, cancers,
malnutrition or obesity)
 Indications for interventions

1. Asymptomatic Patients:
Asymptomatic patients with severe valvular lesion (see Echo criteria for severity)
should be closely monitored to decide the appropriate time for intervention.
The following are indications for intervention:
- Severe Pulmonary hypertension.
- For regurgitant lesions (MR and AR), decrease in LV ejection fraction or
Increasing in LV dimensions especially LV end systolic dimension.
- Importantly, when the ejection fraction approaches 50% progression of heart
failure and death occur in up to 25% of these patients per year if surgery is not
performed (cardiomyopathy secondary to MR). When surgery is performed in
patients with ejection fraction less than 50% the 10 year survival is only 30%
compared to 100% for those whose ejection fraction is 60%.(6)
- Severe MS.
2. Symptomatic patients:
Symptomatic patients with severe valve dysfunction should be referred for
intervention.
 Cardiac Intervention options
1- Valve repair
• This is done mainly for MR but needs careful assessment by Echo
to select the suitable candidates.
2- Percutaneous transvalvular mitral commissurotomy (PTMC):
• Patients with severe MS and suitable valve anatomy should be
considered for PTMC or for closed mitral valvotomy (CMV), if
PTMC is not available.
3- Valve replacement:
• Prosthetic valve replacement is the option when valve repair or
commissurotomy is not feasible for MV disease and it is the main
procedure for AV disease.
• Valve replacement can be either mechanical valve or bioprosthetic
valve
Advantages and Disadvantages valve
replacement versus valve repair
Heart valve Heart valve
REPLACEMENT REPAIR

Anticoagulation required No Anticoagulation

Longer time before re-operation Shorter time before re-operation

RHD
 Long-term complications
Long-term complications of valve replacement
include :
– structural valve deterioration (this is only a concern
for biological and bioprosthetic valves and the
deterioration is time-dependent);
– valve thrombosis (0.01–0.5% per year);
– thromboembolism (2–5% per year);
– prosthetic endocarditis (0.2–1.2% per year);
– major bleeding (conventionally attributed to
anticoagulation), 1–4% per year;
– paravalvular leak (0.1–1.5% per year).
 Post Valve Surgery
• Continuous follow up
• Don’t stop Benzathine Penicillin
• In patients with metalic prosthetic valves:
– Warfarin should not be stopped
– INR control: target 2.5-3
• Endocarditis prophylaxis before high risk
procedures
• Dental hygiene
Recommended RHD Care
based on Severity
 Guidelines for managing Mild RHD
Definition - RHD with any trivial to mild valve lesion.
Secondary Prophylaxis Long-term prevention of recurrent ARF
BPG injection at local health center
Primary care management By local Health Officer/BSc Nurse
Specialist medical review Every 12 months
for children aged to 18
Earlier if clinical deterioration
years
Echocardiogram (if Every 2 years for children
available) Every 3 years for adults
Dental review following With appropriate endocarditis
diagnosis prevention
 Guidelines for managing Moderate RHD
Definition - Any moderate valve lesion, no symptoms, and normal
LV function with stable metallic prosthetic valves
Secondary Prophylaxis Long-term prevention of recurrent ARF
BPG Injection at local health center
Primary care management By local Health Officer/BSc Nurse
Specialist medical review Every 12 months
Earlier if clinical deterioration

Echocardiogram (if available) Every 1 years for children

Every 2 years for adults

Dental review following With appropriate endocarditis prevention

diagnosis
 Guidelines for managing Severe RHD
Definition - Any severe valve lesion with or without symptoms or a
history of valve surgery including mitral valvotomy, any valve repair
and bio-prosthetic valves.
Secondary Prophylaxis Long-term prevention of recurrent
ARF. BPG injection at local health
center
Primary care management By local GP/Health Officer/BSc
Nurse
Specialist medical review Every 3-6 months
Refer to Heart Specialist Annually for surgery assessment or
post surgery valve status assessment
Dental review following With appropriate endocarditis prevention
diagnosis
 Summary
RHD presents as damage to the heart valves
The mitral valve is most commonly affected, followed by Aortic,
Pulmonary and Tricuspid
RHD can be mild, moderate or severe
RHD may be asymptomatic
Management of RHD includes
– Treatment of cardiac and other symptoms
– Long-term secondary prophylaxis (to prevent recurrent ARF)
– Regular medical and cardiology review
– Management of existing pregnancy
– Dental assessment, family planning referral