.

Seminar on
Rheumatic heart
Disease
23/03/2013 by zerihun getachew 1
Content:-
 Introduction
 Epidemiology
 Etiology and risk factor
 Pathophysiology
 Pattern of cardiac valve disease
 Clinical presentation and Diagnosis
 Management/ treatment

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 Introduction
Rheumatic heart disease is a chronic condition in which the heart valves have been
permanently damaged by rheumatic fever which is  an inflammatory disease that can
affect many connective tissues, especially in the heart.

Rheumatic heart disease (RHD) is a chronic heart valve disease caused by a

preventable infection of the bacterium Group A Streptococcus (Strep A) which may
lead to an autoimmune response in the body, namely acute rheumatic fever (ARF).

If Strep A and ARF go untreated, repeated infections are more likely to occur.

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 Cont..
While fever and other rheumatic symptoms often resolve, ARF-associated carditis can
result in permanent damage to at least one of the four heart valves.

The valvular lesions begin as small verrucae composed of fibrin and blood cells along

the borders of one or more of the heart valves.

The mitral valve is affected most often, followed in frequency by the aortic valve; right-

sided heart manifestations are rare.

Long-term consequences of RHD may include stroke, heart failure, and premature

mortality.
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 EPIDEMIOLOGY
ARF is mainly a disease of children age 5–15 years but, rare in persons age >40
and < 4 years.
Initial episodes become less common in older adolescents , young adults.
 By contrast, recurrent episodes of ARF remain relatively common in adolescents
and young adults.
This pattern contrasts with the prevalence of RHD, which peaks between
25 and 40 years.
 There is no clear gender association for ARF, but RHD more commonly affects
females, sometimes up to twice as frequently as males.

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Cont..
Data on RHD related morbidity and mortality are less robust, but the estimate is at
least 260,000-300,000 deaths per year.
In 2017, there were an estimated 38-40.8 million cases of RHD globally, with the
highest prevalence in Oceania, South Asia, and sub-Saharan Africa.
Unfortunately, the greatest burden of disease is found in developing countries,
most of which do not have the resources, capacity, and/or interest to tackle this
multifaceted disease.
Prevalence ranged from 3.4 cases/100,000 in non-endemic regions, to
>1,000/100,000 cases in endemic areas.
Up to 60% of patient with ARF progress to RHD.

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 Etiology
Group A beta hemolytic streptococcal bacteria
Rheumatic fever

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 Risk factor

 poor socioeconomic status

Over crowding

Age

Climate and season

Previous history of ARF

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 Pathophysiology
When a child gets a group A streptococcal infection of the throat (known as strep
throat), their body’s immune system, in trying to fight that infection, produces
antibodies.

Sometimes these antibodies, in addition to killing the strep, can damage the heart
valve.

Acute rheumatic fever can occur following an untreated strep throat infection and
can cause irreversible damage to the major cardiac valves, known as rheumatic
heart disease.
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Pathophysiology
Exposure
pharyngitis
to GABS

RF

Heart
RHD
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 PATTERNS OF VALVULAR DISEASE
1. MITRAL INSUFFICIENCY
Mitral insufficiency is the result of structural changes that usually include some
loss of valvular substance or shortening and thickening of the chordae tendineae.
During acute rheumatic fever with severe cardiac involvement, heart failure is
caused by a combination of mitral insufficiency coupled with inflammatory disease
of the pericardium, myocardium, endocardium, and epicardium.
 Because of the high volume load and inflammatory process, the left ventricle
becomes enlarged.
The left atrium dilates as blood regurgitates into this chamber.
Increased left atrial pressure results in pulmonary congestion and symptoms of
left-sided heart failure

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cont..

A 3rd heart sound is generally prominent.

A holosystolic murmur is heard at the apex with radiation to the axilla.

A short mid-diastolic rumbling murmur is caused by increased blood flow across

the mitral valve as a result of the insufficiency.

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cont..

In patients with severe chronic mitral insufficiency, pulmonary arterial pressure

becomes elevated, the right ventricle and atrium become enlarged, and right-sided

heart failure subsequently develops.

The effects of chronic mitral insufficiency may become manifest after many years and

include right ventricular failure and atrial and ventricular arrhythmias

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 MITRAL STENOSIS
Mitral stenosis of rheumatic origin results from fibrosis of the mitral ring,
commissural adhesions, and contracture of the valve leaflets, chordae, and papillary
muscles over time, it takes 10 yrs. or more for the lesion to become fully
established, although the process may occasionally be accelerated.
Rheumatic mitral stenosis is seldom encountered before adolescence and is not
usually recognized until adult life.
Significant mitral stenosis results in
Enlargement and hypertrophy of the left atrium
Increased pulmonary vascular resistance
 Pulmonary hypertension

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Cont..

More severe degrees of obstruction are associated with exercise intolerance and

dyspnea.

When pulmonary hypertension has developed, right ventricular dilatation may

result in functional tricuspid insufficiency.

Hemoptysis caused by rupture of bronchial veins and, occasionally, by pulmonary

infarction may occur.

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AORTIC INSUFFICIENCY
In chronic rheumatic aortic insufficiency, sclerosis of the aortic valve results in

distortion and retraction of the cusps.

Regurgitation of blood leads to volume overload with dilatation and hypertrophy of

the left ventricle.

Combined mitral and aortic insufficiency is more common than aortic involvement

alone

Systolic blood pressure is elevated, and diastolic pressure is lowered. In severe

aortic insufficiency, the heart is enlarged, with a left ventricular apical heave.
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Cont..
The murmur is heard over the upper and midleft sternal border with radiation to

the apex and the aortic area.

An apical presystolic murmur (Austin Flint murmur) resembling that of mitral

stenosis is sometimes heard, is a result of the large regurgitant aortic flow in

diastole that prevents the mitral valve from opening fully.

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TRICUSPID VALVE DISEASE.
Primary tricuspid involvement is rare after rheumatic fever.

Tricuspid insufficiency is more common secondary to right ventricular dilatation

resulting from unrepaired left-sided lesions.

The signs produced by tricuspid insufficiency include prominent pulsations of the

jugular veins, and a blowing holosystolic murmur at the lower left sternal border that
increases in intensity during inspiration.

Concomitant signs of mitral or aortic valve disease, with or without atrial fibrillation,
are frequent
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 PULMONARY VALVE DISEASE
Pulmonary insufficiency usually occurs on a functional basis secondary to

pulmonary hypertension and is a late finding with severe mitral stenosis.

The murmur (Graham Steell murmur) is similar to that of aortic insufficiency, but

peripheral arterial signs (bounding pulses) are absent.

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cont..

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Clinical presentation and Diagnosis
The symptoms of rheumatic heart disease vary, which depend on the extent and
location of the heart damage.
 Dyspnea
Orthopnea
Palpitation mitral valve stenosis
Chest pain
PND
hemoptysis
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Cont..
Exertional angina

Exertional dyspnea aortic stenosis

Dizziness or exertional syncope

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 DIAGNOSIS
 Because no clinical or laboratory finding is pathognomonic for acute rheumatic fever ,
T.Duckett Jones proposed guidelines in 1944 to aid in diagnosis and to limit over diagnosis.
 There are 5 major and 4 minor criteria and a requirement for diagnosis of ARF following

GAS infection.
Major minor
Carditis Arthralgia

Migratory polyarthritis Fever

Sydenham’s chorea Elevated acute phase reactants (ESR, CRP)

Erythema marginatum Prolonged PR-interval on ECG

Subcutaneous nodules
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Cont..
 Diagnosis of acute rheumatic fever is established with: 2 major criteria or 1 major
and 2 minor criteria and supporting evidence for antecedent streptococcal
pharyngitis (mandatory).
 Strict adherence to Jones criteria is not needed under the following situations:
 Sydenham’s chorea
 Indolent carditis
 Rheumatic fever recurrence

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Cont..
 Investigations
 Culture from throat swab:- positive for strep A
 ASO titer, C-reactive protein (CRP), ESR, leukocytosis :-evidence of strep
A infection
 Chest x-ray :-show enlarged heart
 ECG:-to ass rhythm problem and structural change
 Echocardiography:- identify and quantify valve insufficiency and
dysfunction.
 Magnetic resonance angiography (MRA) :-useful in quantitating
regurgitated volume
 Cardiac catheterization :- when the echocardiographic data are
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equivocal by zerihun getachew 26
TREATMENT
 Treatment goal
 Eradication of infection prevent complication
 Maximize cardiac out put
 Promote patient comfort

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1. Eradication of infection
A. PRIMARY PREVENTION
 Once the diagnosis of acute RF has been established and regardless of the throat

culture results, the patient should receive 10 days of orally administered penicillin

or amoxicillin or a single intramuscular injection of benzathine penicillin to ensure

eradication of GAS from the upper respiratory tract.

 Appropriate antibiotic therapy instituted before the 9 th day of symptoms of acute

GAS pharyngitis is highly effective in preventing first attacks of acute RF.

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Cont..
 If penicillin allergic;
 Erythromycin-40mg/kg/days QID 10 days.

 Azithromycin 10mg/kg for1st day then 5mg/kg/ day for 4 days.

 Clindamycin 20 mg/kg/day for 10 days.

For atrial fibrillation or flutter, anticoagulation with oral vitamin K

antagonists or direct oral anticoagulants is still recommended.

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 B. SECONDARY PREVENTION
Secondary prevention with continuous antibiotic to prevent recurrent
infection with Group A streptococcus.

 Penicillin G benzathine 600,000 IU for children weighing ≤27 kg and 1.2

million IU for children >27 kg, every 4 wk.

 Penicillin V 250 mg, twice daily Oral.

 For People Who Are Allergic to Penicillin give Macrolide or azalide.

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 DURATION OF PROPHYLAXIS
CATEGORY DURA TION

Rheumatic fever without carditis 5 yr or until 21 yr of age, whichever is

longer

Rheumatic fever with carditis but 10 yr or until 21 yr of age, whichever

without residual heart disease (no is longer
valvular disease)

Rheumatic fever with carditis and 10 yr or until 40 yr of age, whichever is

residual heart disease (persistent longer; sometimes lifelong
valvular disease) prophylaxis
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2. Maximize cardiac out put

Corticosteroid are used to treat carditis especially if heart failure is evident

The usual dose of prednisone is 2mg/kg/day in 4 divided doses for 2-3 wk,
followed by half the dose for 2-3 wk. and then tapering of the dose by 5mg/24 hr
every 2-3 days.

When prednisone is being tapered, aspirin should be started at 50 mg/kg/day in 4

divided doses for 6 wk. to prevent rebound of inflammation.

If heart failure develops treatment, include ACEI, BB, and Diuretics.

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3. Promote comfort
Patient with arthritic manifestation should be treated with salicylate.

Migratory polyarthritis and carditis with out cardiomegaly; Aspirin 50-

70mg/kg/day divided in to 4 dose po for 3-5 days then 50mg/kg/day for 4 weeks.

Patients with carditis and more than minimal cardiomegaly and/or congestive
heart failure should receive corticosteroids.

Bed rest is usually advised to reduce cardiac effort until evidence of inflammation
has subside.

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SURGICAL MANAGEMENT
 When heart failure persist or worsen after aggressive medical therapy for
acute rheumatic heart disease, surgery to decrease valve insufficiency may be
life saving.
Surgical valve replacement
1. Mechanical
2. Bioprosthetic
 If patient unable to undergo valve replacement, should goes to valve repair…
i. Balloon valvuloplasty
ii. Transcatheter valve replacement following balloon valvuloplasty
iii. annuloplasty ring

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 cont.…
Cummisurotomy can be done to widen the valve.

In patient with critical stenosis

Valvulotomy
Percutaneous balloon valvuloplasty may be indicated.
Mitral valve replacement

Due to high rate of recurrent symptom after annuloplasty or other repair

procedures, valve replacement appears to be the preferred surgical option.

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 REFERENCE

1. HARRISON'S PRINCIPLES OF INTERNAL MEDICINE, 20 EDITION

2. AMERICAN COLLEGE OF CARDIOLOGY

3. NELSON TEXT BOOK OF PEDATRICS 21 EDITION

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 Thank you

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