Rheumatic Fever /

Rheumatic Heart Disease

BY
Dr. Sohil Elfar , MD
RHEUMATIC FEVER
 Introduction

 Rheumatic fever (RF) and rheumatic heart disease

(RHD) are still major medical and public health
problems mainly in developing countries.

 These diseases in developing areas of the world

affect ~ 20 million people and are the leading causes
of cardiovascular death during the first 5 decades of
life, with more than 400 000 deaths annually and
hundreds of thousands of people disabled.
 Worldwide prevalence of RHD.

Penelope et al, Circulation 2009;119;742-753 .

Epidemiology

• Major public health problem in heavily

populated underdeveloped and developing
countries
 Ages 5-15 yrs, peak at 8 yrs.
 The attack rate ranged from 0.3%to 3%
 Equal sex
 Environmental factors over crowding, poor
hygiene, poverty,
 Incidence more during fall ,winter & early
spring
Etiology and Pathogenesis

 Delayed Autoimmune, multisystem,

inflammatory disorder, following
Group A, (ß-hemolytic) streptococcal
pharyngitis.
 It is a delayed non-suppurative sequelae to
URTI with GABH streptococci.
 a latent period of 1-3 weeks, Antibody against
m-protein ( M types l, 3, 5, 6,18 & 24) cross
react with glycoprotein - susceptible
individuals
Rheumatic fever-pathogenesis
Pathologic Lesions

 Fibrinoid degeneration of connective tissue,

inflammatory edema, inflammatory cell infiltration
& proliferation of specific cells resulting in
formation of Ashcoff nodules, resulting in-
-Pancarditis in the heart
-Arthritis in the joints
-Ashcoff nodules in the subcutaneous
tissue
-Basal gangliar lesions resulting in
chorea
Clinical Features
1.Polyarthritis

 Migratory polyarthritis of major

joints (knee, ankle, elbow &
wrist)
 Occur in 70%, (tender,
swelling, heat, redness, severe
pain, and limitation of motion)
are common in the involved
joints
 Responds to salicylate therapy
 Arthritis do NOT progress to
chronic disease
Clinical Features
2.Carditis

 Manifest as pancarditis (endocarditis,

myocarditis and pericarditis), occur in 50%
of patients
 Carditis is the ONLY manifestation of
rheumatic fever that leaves a sequelae &
permanent damage
 Valvulitis occur in acute phase
 Chronic phase- fibrosis, calcification &
stenosis of heart valves (fishmouth valves)
Clinical Features
3.Sydenham Chorea

 15% of patients
 Mainly in girls of 8-12 yrs age
 2-6 months after the attack of
streptococcal pharyngitis
 Clinically manifest as
neuropsychiatric disorder -
deterioration of handwriting,
emotional lability
Clinical Features
4.Erythema Marginatum

 <10%. Unique, transient, nonpruritic, annular

erthematuous lesions of 1-2 inches in size
 Pale center with red irregular margin
 More on trunks & limbs & spare the face
 Worsens with application of heat
 Often associated with chronic carditis
Clinical Features

5.Subcutaneous nodules
 Occur in 2-10%
 Painless, hard, palpable freely mobile
nodules, and 0.2-2 cm in diameter
 Mainly over extensor surfaces of joints,
spine, scapulae & scalp
 Associated with strong seropositivity
 Always associated with severe carditis
Laboratory Findings

 High ESR
 Anemia, leukocytosis
 Elevated C-reactive protein
 ASO titer >200 Todd units.
(Peak value attained at 3 weeks, then
comes down to normal by 6 weeks)
 Anti-DNAse B test
 Throat culture-GABH streptococci
Laboratory Findings

 ECG- prolonged PR interval, 2nd or 3rd

degree blocks, ST depression, T inversion

 2D Echo-cardiography- valve edema, mitral

regurgitation, LA & LV dilatation, pericardial
effusion, decreased contractility
Diagnosis

 Rheumatic fever is mainly a clinical

diagnosis
 No single diagnostic sign or specific
laboratory test available for diagnosis
 Diagnosis based on MODIFIED JONES
CRITERIA
Jones Criteria (Revised)
Guidance in the Diagnosis of Rheumatic Fever*

• Positive Throat Culture or rapid streptococcal antigen test

• Recent Scarlet Fever
• Increased or rising antibody Titer

Two major criteria OR One major criteria + two minor

Minor Criteria
• Clinical • Lab
• Fever • Acute phase reactants:
• Arthralgia • Erythrocyte sedimentation rate
• C-reactive protein
• Prolonged P-R interval
Recommendations of the American Heart Association
Exceptions to Jones Criteria

 Chorea alone, if other causes have been

excluded
 Insidious or late-onset carditis with no other
explanation
 Patients with documented RHD or prior
rheumatic fever, suggests recurrence
 one major criterion,or
 fever,arthralgia or
 high CRP
management

 Definite diagnosis
 Antibacterial (eradication of streptococci)
 Bed rest
 Anti inflammatory treatment
(aspirin, steroids)
 Supportive management &
management of complications
 Prevention
Step II: Anti inflammatory treatment

Clinical condition Drugs

Arthritis , mild and Aspirin 75-100
moderate carditis mg/kg/day,give as 4
divided doses for 6
weeks
(Attain a blood level 20-
30 mg/dl)
Severe Carditis Prednisolone 2-2.5
mg/kg/day, give as two
divided doses for 2
weeks
Taper over 2 weeks &
while tapering add
Aspirin 75 mg/kg/day
for 2 weeks.
Continue aspirin alone
100 mg/kg/day for
another 4 weeks
3.Step III: Supportive management &
management of complications

 Bed rest
 Treatment of congestive cardiac failure:
-digitalis,diuretics
 Treatment of chorea:
-diazepam or haloperidol
 Rest to joints & supportive splinting
 Preventions of Rheumatic Fever/ RHD
Potential Preventive Measures for Rheumatic Fever and Rheumatic
Heart Disease.

Jonathan & Carapetis, N Engl J Med 357;5 august 2, 2007.

 Prevention of Initial Attacks (Primary
Prevention)

 Prevention of initial episodes of ARF

requires accurate recognition and proper
antibiotic ttt of GAS pharyngitis

 Appropriate antibiotic treatment of

streptococcal pharyngitis prevents ARF in
most cases.

 At least one third of episodes of ARF result

from inapparent streptococcal infections.

 Some symptomatic patients do not seek

medical care.
Clinical and
Epidemiological
Findings and
Diagnosis of GAS
Pharyngitis

Michael, et al. Circulation

2009;119;1541-1551.
 Primary Prevention
Diagnosis of Streptoccocal Infections
Throat Culture
 It is the criterion standard
Antigen Detection Tests
 High degree of specificity
 A negative test does not exclude the
presence of GAS, and a throat culture
should be performed.
Streptococcal Antibody Tests
 The most commonly used
 Elevated or rising antistreptococcal
antibody titers
 Elevated titers may persist for several
months after uncomplicated GAS
infections.
Prevention of Initial Attacks (Primary Prevention)

Treatment of Streptococcal Tonsillopharyngitis

Michael, et al. Circulation 2009;119;1541-1551.

 Secondary Prevention

Duration of Secondary Rheumatic Fever

Prophylaxis

FEIGIN, etMichael,
al. Textbook
et of
al.Pediatric Infectious
Circulation Diseases 2004;236:3029-3040.
2009;119;1541-1551.
 Secondary Prevention of Rheumatic Fever
(Prevention of Recurrent Attacks)

FEIGIN, etMichael,
al. Textbook
et of
al.Pediatric Infectious
Circulation Diseases 2004;236:3029-3040.
2009;119;1541-1551.
 Bacterial Endocarditis Prophylaxis

 The current AHA recommendations NO longer

suggest prophylaxis for patients with rheumatic heart
disease
 Few patients with rheumatic heart disease , such as
those with prosthetic valves or prosthetic material
used in valve repair.
 These recommendations advise the use of an agent
other than a penicillin to prevent infective
endocarditis in those receiving penicillin prophylaxis
for rheumatic fever.

Michael, et al. Circulation 2009;119;1541-1551.

Prognosis

Permanent cardiac damage determine the

prognosis, it is affected by

 Cardiac status at the start of treatment

 Recurrence of rheumatic fever
 Regression of heart disease
 Mitral Stenosis

Rheumatic heart
disease causes
mitral stenosis in
99.8% of
 Mitral Stenosis
 Patients with MS from rheumatic fever, the
pathological process causes leaflet thickening and
calcification, commissural fusion, chordal fusion, or a
combination of these processes.

 The normal MV area is 4.0 to 5.0 cm2. Narrowing of

the valve area to less than 2.5 cm2 typically occurs
before the development of symptoms.
Mitral Stenosis

 The resulting elevation of left atrial pressure is

reflected back into the pulmonary venous
circulation.

 The pulmonary arterioles may react with

vasoconstriction, intimal hyperplasia, and medial
hypertrophy, which lead to pulmonary arterial
hypertension.
Mitral Stenosis
Mitral Stenosis: Symptoms

 Fatigue  Systemic embolism

 Pulmonary infection
 Palpitations
 Hemoptysis
 Cough
 Right sided failure
 SOB  Hepatic
Congestion
 Edema
 Mitral Stenosis :
Physical Exam

Palpation: Auscultation:
 RV lift  Loud S1- as loud as S2 in
 Palpable S2 aortic area
 Diastolic rumble: length
proportional to severity
ECG:
 In severe MS with low flow-
 LAE, AFIB, RVH. S1, OS & rumble may be
inaudible
NATURAL HISTORY

 An MV area greater than 1.5 cm2 usually does

not produce symptoms at rest.
 MS is a continuous, progressive, lifelong
disease, usually consisting of a slow, stable
course in the early years followed by a
progressive acceleration later in life.
 Once symptoms develop, there is another period
of almost a decade before symptoms become
disabling.
 NATURAL HISTORY

 When severe pulmonary hypertension develops,

mean survival drops to less than 3 years.

 The mortality rate of untreated patients with MS is

due to progressive pulmonary and systemic
congestion in 60% to 70%, systemic embolism in
20% to 30%, pulmonary embolism in 10%, and
infection in 1% to 5%.
NATURAL HISTORY
Mitral Stenosis

Diagnosis

 ECG: A Fib, LAE, RAE, RVH

 Echo 2D/color doppler –
 Cardiac Cath – helpful, confirmatory,
Mitral Stenosis

Treatment of Symptomatic Mitral Stenosis

Medical Therapy – treats the symptoms not the
cause
 Diuretics – for congestion
 Digoxin, Beta and Ca Channel Blockers for Afib
rate control
 Anticoagulation – for AFib and LA clots
 SBE Prophylaxix – prevent endocarditis
 Mitral Stenosis
Treatment of Symptomatic Mitral Stenosis

Surgical Therapy – treats the cause

 Percutaneous Ballon Valvulaoplasty – Non-calcified
valve
 Mitral Stenosis

Treatment of Symptomatic Mitral Stenosis

Surgical Therapy – treats the cause
 Open Commisurotomy – valve repair
 Mitral Valve Replacement
Mitral Regurgitation
Mitral Regurgitation

Symptoms

 Fatigue and weakness

 Dyspnea and orthopnea
 Palpitation
 Right sided HF
Mitral Regurgitation

Physical Exam
 Holosystolic Apical Blowing Murmur(
starting with S1,grade 2 to 4/6
 Laterally displaced apical impulse
 Split S2 (but is obscured by the murmur)
 S3 Gallop (increased volume during
diastole)
Mitral Regurgitation

Diagnosis
 ECG – LAE, LVH, Afib.
 X-Ray: LA,LV enlarged. pulmonary venous
congestion.
 Echo 2D/color doppler –
 Cardiac Cath – helpful, confirmatory,
Mitral Regurgitation

- SBE Prophylaxis
Mitral Regurgitation
Aortic Regurgitation
Aortic Regurgitation
Aortic Regurg – pathophysiology
Aortic Regurg – pathophysiology
Aortic Regurgitation
Aortic Regurgitation

Physical Exam
 Diastolic Decrescendo Blowing Murmur
 Hyperdynamic LV apical impulse
 Bounding Pulses
 S4, S3 Gallop-advanced AI
 Apical Rumble –
Aortic Regurgitation

Diagnosis

 ECG – LAE, LVH

 Echo 2D/color doppler –
 Cardiac Cath –
Aortic Regurgitation

Treatment of Asymptomatic Aortic Regurg

Medical Therapy – treats the symptoms not the cause
• Serial Check ups with Echos (Severity AR)
• SBE Prophylaxis
• Vasodialators (Nifedipine, ACE-I)
• Diuretics
Treatment of Symptomatic Aortic Regurg
Aortic Valve Replacement
Bioprosthetic vs Mechanical AVR
AS symptomes

 Cardinal Symptoms
 Chest pain (angina)
• Reduced coronary flow reserve
• Increased demand-high afterload
 Syncope/Dizziness (exertional pre-syncope)
• Fixed cardiac output
• Vasodepressor response
 Dyspnea on exertion & rest
 Impaired exercise tolerance

 Other signs of LV failure

 Diastolic & systolic dysfunction
 © Continuing Medical Implementation …...bridging the care gap

Severity of Stenosis

 Normal aortic valve area 2.5-3.5 cm2

 Mild stenosis 1.5-2.5 cm2
 Moderate stenosis 1.0-1.5 cm2
 Severe stenosis < 1.0 cm2
 Onset of symptoms
~ 0.9 cm2 with CAD
~ 0.7 cm2 without CAD
 Aortic Stenosis: Prognosis

Symptom/Sign Live expectancy

Angina 5 years
Syncope 2-3 years
Congestive Heart Failure 1-2 years

Therapy: Valve replacement for severe aortic stenosis

Operative mortality (elderly) ~ 4-24%/Morbidity ~ 3-11%
Event rate in asymptomatic severe AS ~ 1%/year
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