Chronic Rheumatic Valvular Heart

Disease

B Y: A B D U L H A K I M Z E K E R I YA
 Outline
Introduction
Epidimology
Etiology
Pathophysiology
Clinical presentation
Management
Complications
Reference

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 Introduction
❖Rheumatic heart disease is a condition where the heart valves have
been permanently damaged by rheumatic fever. The heart valve
damage may start shortly after untreated or under-treated streptococcal
infection such as strep throat or scarlet fever. An immune response
causes an inflammatory condition in the body. This can result in
ongoing valve damage.
❖Rheumatic heart disease is a chronic condition resulting from
rheumatic fever which involves all the layers of the heart (i.e. pan-
carditis) and is characterized by scarring and deformity of the heart
valves.
❖It is an inflammatory disease characterized by a delayed response to
an infection by Group 'A' Beta hemolytic streptococci (GAS) in the
tonsillopharyngeal.
❖It is a chronic inflammatory disease process resulting from a delayed
(Months to Years) sequel of rheumatic fever, causing scaring and
deformity of the heart valves

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 Cont…
Acute rheumatic fever (ARF) is a multi system disease
resulting from an autoimmune reaction to infection with
group A Streptococcus.
Although many parts of the body may be affected, almost all
of the manifestations resolve completely. The major
exception is cardiac valvular damage (rheumatic heart
disease [RHD]), which may persist after the other features
have disappeared.

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 Epidemology
❖ARF is mainly a disease of children age 5–14 years. Initial episodes
become less common in older adolescents and young adults and are rare
in persons aged >30 years. 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.
❖In Ethiopia, various studies have been conducted to estimate the
prevalence of RHD in different contexts. A community-based study
done in rural Ethiopia using trans-thoracic echocardiography
indicated that 37.5% of the participants were found to have RHD .
Another multisite echocardiography-based study done among
schoolchildren shows that 19 cases per 1000 children have
evidence of the disease.

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 Etiology
Conventional teaching has it that CRVHD is exclusively caused
by infection of the upper respiratory tract infection with group A
beta hemolytic streptococci and rheumatic fever
The disease is seen more commonly in poor socio-economic
stratification of the society living in damp and overcrowded place.

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 Risk Factors
❖Age commonly 5-15 years
❖Over crowded living condition
❖Poverty
❖Rural resident
❖Urban slum resident
❖Genetic predisposition
❖Failure to seek health care for sore throat
❖Inadequate diagnosis and treatment of
streptococcal pharyngitis
❖Poor delivery of secondary prophylaxis
❖Asymptomatic or undiagnosed acute rheumatic
fever

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 PATHOPHYSIOLOGY
❖The pathophysiology of rheumatic heart disease is related to the
delayed response of the immune system to an infection caused by
Group A Beta-hemolytic streptococci (GAS) in the tonsillopharyngeal
region. This delayed response can occur months to years after the
initial infection. The immune response triggers an inflammatory
process that leads to the formation of scar tissue on the heart valves,
causing them to become thickened and rigid.
❖As the disease progresses, the scar tissue can lead to valve stenosis,
where the valve opening becomes narrowed, impeding the flow of
blood. It can also result in valve regurgitation, where the valve does
not close properly, causing blood to leak backward. Both stenosis and
regurgitation can put a strain on the heart and lead to symptoms such as
shortness of breath, fatigue, and chest pain.
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 PATHOPHYSIOLOGY

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 Clinical Presentation
❖Fever
❖Heart palpitation
❖Chest pain
❖Difficulty breathing
❖Fatigue
❖Polyarthritis Swollen joints like wrist and ankles
❖Chorea:- jerky, uncontrollable movements of arms, legs or
facial muscles
❖Erythema marginatum
❖Nodules on the skin

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 Major Criteria
1. Carditis
❖Presents as palpitation, chest pain or breathlessness.
Found in 60%-90% of cases, mainly valvulitis. Rheumatic
carditis may have 30-70% long term morbidity and ends
up in CRVHD).
❖ In chronic rheumatic heart disease, the valves involved
are mitral, aortic and tricuspid valve with mitral valve
being the most commonly affected valve

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 Cont…
Carditis should be expected if there is
❖ High sleeping pulse rate
❖ Cardiac enlargement
❖ Soft or muffled S1
❖ Diastolic apical murmur or changing murmur
❖ Pericardial friction rub

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 Cont…
2. Polyarthritis
❖It is an acute painful migratory swelling of several joints.
Larger joints such as the elbow, knee, ankle and wrist joins
are involved. The joints are hot, red and swollen causing
inability to move the joints.
❖It responds immediately to salicylates and there is no
residual damage to the joint after recovery.

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 Cont…
3. Sydenham’s Chorea ( St. Vitus dance)
❖It is an inflammation involving the basal ganglia, cerebral
cortex and cerebellum. It involves jerky involuntary
purposeless movements that are mainly found in the
proximal limb muscles or trunk. It is a late manifestation
and commonly seen in girls
❖It is self-limiting disease, which can last from 1 week to
more than 2 years.

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 Cont…
4. Erythema Marginatum
❖It is skin lesions, which starts as raised red macules with
pale center mainly over the trunk and proximal part of the
limbs.

5. Subcutaneous Nodules
❖ Firm painless nodules felt over bones or tendons that
indicate severe carditis. They occur on the extensor
surfaces and bony prominences.

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 Diagnosis
Requires the presence of supporting evidence for preceding
streptococcal infection
and the following:
1. Primary episode of Rheumatic Fever or recurrence
without established rheumatic heart disease: Two major, or
one major and two minor manifestations.
2. Recurrent attack of Rheumatic fever with established
rheumatic heart disease-two minor manifestations.
3. Rheumatic chorea or insidious onset carditis-neither
evidence of preceding streptococcal infection nor other
major manifestation needed.

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 Diagnosis
Assessment of the patient’s status should focus on the search
for risk factors, previous history of rheumatic fever, strep
throat
Non imaging Modalities
Blood Test – WBC count & ESR is elevated, C- reactive
protein is elevated, Cardiac enzymes levels (Troponin T)
may increase in sever carditis.
Throat culture – for presence of GABS
ECG – reveals to show PR intervals

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 Cont…
Imaging Modalities
Chest X-ray Cardiomegaly - Sign of pulmonary venous
congestion.
ECHO to check the heart valves for any damage or infection
and assessing if there is heart failure. This is the most useful
test for finding out if RHD is present.

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 Goal Of Therapy
Treatment Objectives
❖Eradicate streptococcal throat infection
❖Prevent recurrent episodes of rheumatic fever and further
valvular damage
❖Treat Heart Failure, if co-existent
❖Control inflammation and relive symptoms of arthritis

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 Non pharmacological prevention

❖Bed rest if the patient has severe rheumatic carditis or

arthritis/arthralgia only.
❖Salt restriction if there is associated Heart Failure.

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 Pharmacological prevention

❖Antibiotic (primary prevention)

❖Conventional therapy for Heart Failure
❖Anti-inflammatory

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 Treatment
Aspirin, 50–60 mg/kg per day, up to a maximum of 80–100 mg/kg per day (4-8
grams per day P.O). in 4 divided doses
Add a GI prophylaxis – PPI (e.g., Omeprazole 20mg, P.O., BID)
Alternative
Prednisolone (consider its use in severe carditis only), 1–2mg/kg per
day (maximum, 80mg); only required for a few days or up to a maximum
of 3 weeks.

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 Treatment
An injection of 0.6 (600,000 units for children ≤27 kg) or 1.2 million
units for patient >27 kg of benzathine penicillin G intramuscularly every
4 weeks is the recommended regimen for secondary prophylaxis. It can
be given every 3 weeks, to persons considered to be at particularly high
risk.
Alternative (if penicillin allergic) Erythromycin, 250mg, P.O. BID

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 Treatment of Cardiac
❖Corticosteroids are used to treat carditis, especially if heart
failure is evident.
❖If heart failure develops, treatment, including ACE
inhibitors, beta blockers and diuretics, is effective.
❖For treating Chorea:- carbamazepine and valproic acid
NB: There no therapy that slows progression of valvular
damage in setting of ARF.

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 Pattern Of Valvular Disease
1. Mitral Regurgitation
➢Resulted from structural changes such as, loss of valvular
substances and/or sub valvular apparatus like elongation
of chordae tendinea.
➢Results HF by coupling with pan-carditis during the
severe carditis of ARF.
➢Involve dilation of LV and enlarging LA by increased
pressure in it and bring pulmonary congestion finally.

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 Cont…
2. Mitral stenosis
➢It is the narrowing of the mitral valve, which is the heart
valve that controls the flow of blood from the heart's left
atrium to the left ventricle.
➢Is chronic process (≥10 yrs.), but it may be accelerated.
➢Resulted from fibrosis of mitral ring, commissural
adhesion & contraction of the leaflets.

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 Cont…
3. Aortic regurgitation
➢is the inadequate closure of the aortic valve during
diastole that results in reverse blood flow through the
aortic valve. This condition can occur as a native valvular
disease or as a result of aortic root dilatation.
➢is acute and show poor coaptation of leaflets or leaflet
prolapse.
➢Can occur in isolation 1-2 yrs after initially combined
with mitral

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 Cont…
4. Tricuspid valve disease
➢Primary involvement is rare, but resulted from Left side HF
➢Is usually TR and On Physical Examination: characterized by
prominent
➢pulsation of Jugular vein, systolic pulsation of liver, blowing
HSM at LLSB increase with inspiration.
➢Concomitantly found with mitral or aortic valve disease
with/out AF.

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 Complications OF VHD
Valvular heart disease can lead to various complications, including:
✓Heart failure: The impaired valve function can result in the heart
being unable to pump blood effectively, leading to symptoms of
heart failure.
✓Infective endocarditis: The damaged valves are more prone to
bacterial infection, which can cause a serious condition called
infective endocarditis.
✓Arrhythmias: The abnormal blood flow caused by the faulty
valves can disrupt the heart's electrical system, leading to irregular
heart rhythms.
✓Stroke: In some cases, blood clots can form on the damaged valves
and travel to the brain, causing a stroke.

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 Treatment
➢In mild: typical Rx of ARF plus prophylaxis against recurrence.
➢In severe:
❖Corticosteroids,
❖Rx of HF, arrhythmia, Infective endocarditis and Stroke.
ACE inhibitors, diuretics, beta blockers.
➢Surgery is indicated for persistent HF, dyspnea on mild activities &
progressive cardiomegaly. Patients with prosthetic valve need
prophylaxis during dental procedures to prevent.

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 Reference
1. HARRISON’S PRINCIPLES OF INTERNAL MEDICINE
21st Edition
2. PHARMACOTHERAPY PRINCIPLE AND PRACTICE
SIXTH EDITION 2022.
3. STANDARD TREATMENT GUIDELINES FOR
GENERAL HOSPITALS FOURTH EDITION 2021.
4. MEDSTAR Clinical guide and synopsis 2nd EDITION
PEDIATRICS December, 2022 JIMMA, ETHIOPIA.
5. https://www.rhdaustralia.org.au/arf-rhd-guidelines.
6. FANOS’ PEDIATRICS Approach to the Patient Review 1st
Edition October, 2020 G.C.

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 Thank You!
Any Questions ?

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