Acute rheumatic fever and

rheumatic heart disease

Presented by Umbetzhanova A.
General practice with EBM course department
NonJSC AMU
 Definition, Jones criteria
• Acute Rheumatic Fever- Systemic connective tissue disease occurring after
streptococcal pharyngitis or skin infection;
Major Criteria
•Carditis (clinical or subclinical) •Carditis (clinical or subclinical)

•Arthritis (polyarthritis) •Arthritis (mono or poly) or polyarthralgia

•Chorea •Chorea
•Erythema marginatum •Erythema marginatum
•Subcutaneous nodules •Subcutaneous nodules
Minor Criteria
Low-risk populations Moderate to high-risk populations

•Polyarthralgia •Monoarthralgia
•Fever (>38.5°C) •Fever (>38.0°C)
•ESR>/ = 60 mm/hour and/or CRP>/= 3.0 mg/dL •ESR>/= 30 mm/hour and/or CRP >/= 3.0 mg/dL

•Prolonged PR interval (unless carditis is a major criterion) •Prolonged PR interval (unless carditis is a major criterion)

Diagnosis:
initial ARF: 2 major, or 1 major plus 2 minor manifestations
recurrent ARF: 2 major, or 1 major plus 2 minor, or 3 minor manifestations
 Easy way to remember the major
manifestations of the Jones criteria for ARF?

Remember the word “Jones”, with “O” as a

“heart”:
• J—joints (35%–66%)
• ♥—carditis (50%–70%)
• N—nodules (<10%)
• E—erythema marginatum (EM) (<10%)
• S—Sydenham’s chorea (10%–30%)
 Diagnosis
• establishing evidence of a recent GAS
infection is critical.
• positive throat culture for GAS or a positive rapid streptococcal antigen
test, but only 25% of ARF patients -due to the latent period (average, 18 days; range, 1–4
weeks) between infection and development of ARF symptoms.
• The most useful tests to support a recent GAS infection and diagnosis of
ARF are antibodies, including the antistreptolysin-O (ASO),
antideoxyribonuclease-B (antiDNase-B or ADB), antistreptokinase,
antihyaluronidase, and anti-NADase (anti-DPNase).
• The most commonly used serologies are the ASO and ADB
Supporting Evidence of Streptococcal Infection
• Increased titer of antistreptolysin antibodies (antistreptolysin O in
particular)
• Positive throat culture for group A streptococci
• Recent scarlet fever
The presence of two major or one major and two minor manifestations
suggests a high probability of acute rheumatic fever if supported by
evidence of a preceding group A streptococcal infection. Do not make
the diagnosis on the basis of laboratory findings and two minor
manifestations alone.
 PATIENT DATA
• Fever
• Inflamed swollen joints
• Flat or slightly raised, painless rash with pink margins with pale centers and a
ragged edge (erythema marginatum)
• Aimless jerky movements (Sydenham chorea or St. Vitus dance)
• Small, painless nodules beneath the skin
• Chest pain
• Palpitations
• Fatigue
• Shortness of breath
 Objective Data

• Characterized by a variety of major and

minor manifestations
• Murmurs of mitral regurgitation and aortic
insufficiency
• Cardiomegaly
• Friction rub of pericarditis
• Signs of congestive heart failure
 Acute Rheumatic Fever and course of disease

Acute Rheumatic Fever and Rheumatic Heart Disease

Thacker, Deepika, Netter's Pediatrics, 49, 300-306

Cardiac manifestations of acute rheumatic fever.

Copyright © 2011 Copyright © 2011 by Saunders, an imprint of Elsevier Inc.
Noncardiac manifestations of acute rheumatic
fever.

Acute Rheumatic Fever and Rheumatic Heart Disease

Thacker, Deepika, Netter's Pediatrics, 49, 300-306

Noncardiac manifestations of acute rheumatic fever.

Copyright © 2011 Copyright © 2011 by Saunders, an imprint of Elsevier Inc.
 arthritis of ARF
• usually involves larger joints, particularly the knees (75%) and ankles
(50%), and less commonly elbows/wrists/hips (15%).
• Typically, several joints are involved at a time in a migratory polyarthritis
pattern, along with an acute febrile illness.
• Patients are usually symptomatic for 1 to 2 (rarely longer than 4) weeks.
• ARF does not cause erosions or permanent joint damage, with the rare
exception of Jaccoud deformity (Image), which can occur following
multiple recurrences of ARF.
• Synovial fluid is sterile and inflammatory.
Clinical manifestations of ARF

Basic Sciences
Schmitz, Matthew R., Miller's Review of Orthopaedics, Chapter 1, 1-147.e4

Acute rheumatic fever: acute migratory arthritis with effusions approximately

2 to 4 weeks after streptococcal infection (“A sore throat can lead to a broken
heart.”). Historically, the most common cause of arthritis in children. Pathology
results...

Copyright © 2016 Copyright © 2016 by Elsevier, Inc. All rights reserved.

 Sydenham's chorea (St Vitus' dance)

• Neurologic disorder characterized by emotional lability and uncoordinated,

involuntary, and often asymmetric movements of the face, hands, and feet.
• The “ milkmaid’s grip ” (involuntary, repeated grasp, and release of an examiner’s
finger by a subject attempting to hold a constant grip) is pathognomonic.
• Sensation is not affected.
• The choreiform movements disappear during sleep.
• The latent interval between streptococcal pharyngitis and chorea onset may be
prolonged, frequently >6 to 8 weeks.
• Consequently, ASO titers may be normal, although ADB may still be elevated.
• Brain magnetic resonance imaging shows inflammation in the basal ganglia.
Symptoms can last 2 to 4 months and may be the only manifestation of ARF,
although this manifestation is associated with RHD in up to 33% of patients.
• Patients may also be at risk for future neuropsychiatric disorders such as an
obsessive compulsive disorder (OCD).
 Sydenham's chorea (St Vitus' dance)

Nervous System
Buja, L. Maximilian, MD, Netter's Illustrated Human Pathology, Chapter 13, 441-513

Chorea Chorea is a term applied to rapid, complex, and varied movements of the body, especially
the distal limbs. The differential diagnosis includes Sydenham chorea (acute rheumatic fever),
systemic lupus erythematosus, chorea gravidarum (in pre...

Copyright © 2014 Copyright © 2014, 2005 by Saunders, an imprint of Elsevier Inc.

 Subcutaneous nodules
• (<10%):
• usually associated with
severe carditis but not
arthritis.
• firm, painless,
• a few millimeters to 2 cm
in size,
• and often resolve within
days.
 Erythema marginatum
EM is an irregular, serpiginous,
nonpruritic rash that spreads
centrifugally to the arms and trunk
(never the face).
Strongly associated with carditis.
(<10%)

Rheumatology
Rosé, Carlos D., MD, CIP, Pediatric Secrets, Chapter 17, 617-642

Erythema marginatum demonstrating serpiginous margins and rapid migration: the pen mark shows the location of the rash
about 60 minutes earlier.
(From Dougherty S, Carapetis J, Zühlke L, et al, eds. Acute Rheumatic Fever and Rheumatic Heart Diseas...

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 Differential Diagnosis of Acute Rheumatic
Fever

• Myocarditis
• Pericarditis
• Kawasaki disease
• Rheumatoid arthritis
• Other arthritis: traumatic, septic
• Sickle cell disease
• Infective endocarditis
• Systemic lupus erythematosus
• Lyme disease
A sample treatment algorithm in acute rheumatic fever

Acute Rheumatic Fever and Poststreptococcal Arthritis

Wood, Patrick R., MD, Rheumatology Secrets, Chapter 44, 357-362

A sample treatment algorithm in acute rheumatic

fever. GAS, group A streptococcus; IM,
intramuscularly; NSAID, nonsteroidal
antiinflammatory drug.

Copyright © 2020 Copyright © 2020 by Elsevier, Inc. All rights

reserved.
 Preventing recurrent ARF
• WHO recommends 5 years of secondary prophylaxis after the initial
attack or until 18 years of age, whichever is longer.
• For mild RHD, prophylaxis is recommended for 10 years or until the
age of 25 years.
• For moderate, severe cases or after valve surgery, secondary
prophylaxis is recommended to be lifelong.
• Using prolonged forms of penicillines
 Rheumatic Heart Disease. Cardiovascular System Physiology.

Cardiovascular System Physiology Review

Gottlieb, Armand, Netter's Integrated Review of Medicine, Chapter 56, 227-231

(A) Distribution of Blood Throughout the Circulatory System. Most of the body's blood at any given time lies
in the veins, with smaller portions in arteries, capillaries, the heart, and lungs. (B) Distribution of Vascular
Resistance Throughout the...
Rheumatic Heart Disease. Cardiovascular
System Physiology.

Cardiovascular physiology
McGeown, J.G., BSc MB BCh BAO PhD, Master Medicine: Physiology, Chapter 3, 59-99

Diagrammatic representation of the cardiovascular system (see text).

Copyright © 2007 © 2007, Elsevier Limited. All rights reserved.

 Rheumatic heart disease. Pathology.
Chronic RHD results when a single or multiple attacks of ARF deform and fuse valve cusps,
commissures, or chordae.
Isolated mitral valve involvement occurs in 60% to 70%,
Mitral and aortic involvement in 20%, and isolated aortic involvement is rare.
The tricuspid valve is involved in 5% to 10% but occurs with mitral or aortic disease.
Pulmonary valve involvement is rare.

Heart
Kumar, Vinay, MBBS, MD, FRCPath, Robbins Basic Pathology,
Chapter 11, 399-440

Rheumatic heart disease.

(A) Acute rheumatic mitral valvulitis superimposed
on chronic rheumatic heart disease. Small
vegetations (verrucae) are visible along the line of
closure of the mitral valve leaflet (arrows). Previous
episodes of rheumatic...

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reserved.
 Clinical presentation
• Patients with mitral or aortic valve disease may present with an isolated
heart murmur or palpitations caused by atrial rrhythmias.
• They can present with fatigue, decreased exercise tolerance, dyspnea on
exertion, orthopnea, and paroxysmal nocturnal dyspnea, which can
represent low cardiac output or pulmonary hypertension.
• However, the onset of symptoms can often be so insidious that patients
adapt and are unaware of their significant functional limitations.
 Diagnosis
• Laboratory Studies: evidence of preceding GAS infection. Throat culture or rapid
antigen tests (antistreptolysin (ASO) titer, Anti-DNAse B (anti-deoxyribonuclease B));
• Other baseline laboratory investigations should include a complete blood count, C-
reactive protein (CRP), and erythrocyte sedimentation rate (ESR).
• Cardiac Evaluation:Chest Radiography: identifying cardiomegaly and pulmonary
congestion in patients with heart failure or valvar heart disease.
• Electrocardiography: sinus tachycardia, although children may have sinus
bradycardia because of high vagal tone. First-degree atrioventricular block. Higher
degrees of heart block may occasionally be seen, but they resolve with the resolution
of the acute process. In patients with chronic RHD, ECG may suggest chamber
enlargement or hypertrophy. atrial flutter or fibrillation.
• Two-Dimensional Echocardiography and Color Doppler: signs of acute carditis-
pericardial effusion, decreased ventricular function, and mitral regurgitation. In
patients with chronic RHD, echocardiography along with color Doppler evaluation-
identifying the nature and extent of valvar involvement.
• Cardiac Magnetic Resonance Imaging: useful in defining the mechanism and
severity of valvar disease in greater anatomic and functional detail. It can be
especially useful in planning management strategies and surgical intervention.
• Cardiac Catheterization: Cardiac catheterization is not performed in the setting of
ARF. In patients with chronic RHD, diagnostic catheterization is now reserved for the
few patients in whom the symptoms, clinical findings, and noninvasive imaging are
discrepant. However, therapeutic cardiac catheterization and balloon valvuloplasty
continue to remain central in management of valvar RHD.
Acute Rheumatic Fever and Rheumatic Heart Disease
Thacker, Deepika, Netter's Pediatrics, 49, 300-306

Rheumatic heart disease: clinical presentation.

Copyright © 2011 Copyright © 2011 by Saunders, an imprint of Elsevier Inc.

Cardiology
Bunce, Nicholas H., Kumar and Clark's Clinical Medicine, 30, 1019-1132

Features associated with mitral regurgitation and mitral stenosis.

A2, aortic component of the second heart sound; MDM, mid-diastolic
murmur; OS, opening snap;
P2, pulmonary component of the second heart sound (loud with pulmonary
hypertension); P...

Copyright © 2021 © 2021, Elsevier Limited. All rights reserved.

Valve disorders I : mitral valve
Currie, Graeme P, MBChB, DCH, Pg Dip MEd, MD, FRCP(Edin), Flesh and Bones of Medicine, The, 8, 52-53

Features of mitral regurgitation and mitral stenosis.

S1, first heart sound;
S2, second heart sound;
MDM, mid-diastolic murmur;
OS, opening snap;
PSM, pansystolic murmur
P2, pulmonary valve closure sound.

Copyright © 2011 © 2011, Elsevier Ltd. All rights reserved.

 Mitral stenosis

Cardiology
Newby, DE, Davidson's Principles and Practice of Medicine, 16, 441-544

Mitral stenosis: murmur and the diastolic pressure gradient between left atrium
(LA) and left ventricle (LV).
(Mean gradient is reflected by the area between LA and LV in diastole.)
The first heart sound is loud, and there is an opening snap (OS) ...

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Mitral Stenosis
Kasfiki, Eirini V, MB BCh, MRCP (UK),
PGDipME, FHEA, 250 Cases in Clinical
Medicine, Chapter 43, 169-175

Chest radiographs in
severe mitral stenosis.

(A) Posteroanterior view

shows enlargement of the
left atrium (arrowheads),
prominence of the hilar
vessels, and pulmonary
venous redistribution.
Transverse angle of the
apex suggests right
ventricular ...
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rights reserved.
Echo (mitral stenosis)
Valvular Heart Disease
Carabello, Blase A., Goldman-Cecil Medicine, 66,
406-418.e2

Mitral stenosis.
An en face view of a stenotic
mitral valve in the short axis
view of the left ventricle is shown
on the left.
Planimetry for the mitral valve
orifice yielded an area of
1.09 cm 2. The M-mode
echocardiogram on the right has
been al...
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Inc. All rights reserved.
Thorax
Abrahams, Peter H., MB BS, FRCS (Ed), FRCR, DO
(Hon) FHEA, Abrahams' and McMinn's Clinical Atlas
of Human Anatomy, Chapter 4, 179-226

Mitral valve disease

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rights reserved.
Mitral regurgitation
The cardiovascular system
Mills, Nicholas L, Macleod's Clinical
Examination, 4, 39-74

Mitral regurgitation.
Mitral regurgitation is caused by
dilatation of the left ventricle and
failure of leaflets to co-apt.
The murmur begins at the moment
of valve closure and may obscure
the first heart sound. It varies little in
intensity throu...
Copyright © 2018 © 2018 Elsevier Ltd. All
rights reserved.
Mitral regurgitation
Aortic Regurgitation
Aortic Regurgitation
Khare, Sarthak, MD, Ferri's Clinical Advisor 2021, 143-146.e2

Aortic regurgitation as an example of an early

diastolic murmur.
From Epstein O et al: Pocket guide to clinical examination, ed 4, St Louis,
2009, Mosby.

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 Corrigan's Sign/Quincke’s Pulse in Severe
Aortic Regurgitation
https://youtu.be/adcVq_4LkEY
Aortic Regurgitation
Kasfiki, Eirini V, MB BCh, MRCP (UK),
PGDipME, FHEA, 250 Cases in
Clinical Medicine, Chapter 29, 115-119

LV hypertrophy with
prominent positive
anterior T waves.
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Limited. All rights reserved.
Aortic regirgitation
Cardiology
Newby, DE, Davidson's Principles and Practice of
Medicine, 16, 441-544

Chest X-ray of a patient with

aortic regurgitation,
ventricular enlargement and
dilatation of the ascending
aorta.
Copyright © 2018 © 2018 Elsevier Ltd. All rights
reserved.
Thorax
Abrahams, Peter H., MB BS, FRCS (Ed), FRCR, DO (Hon) FHEA,
Abrahams' and McMinn's Clinical Atlas of Human Anatomy, Chapter 4,
179-226

Left ventricular enlargement

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Aortic Stenosis

Aortic Stenosis
Kasfiki, Eirini V, MB BCh, MRCP (UK), PGDipME, FHEA, 250 Cases in Clinical Medicine,
Chapter 30, 120-125

Chest radiographs in severe aortic stenosis.

(A) Frontal view shows prominent aortic root to the right of
the midline (arrowheads).
(B) Lateral view demonstrates calcification of the aortic
valve leaflets (arrows), suggestive of a bicuspid valve. ...
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Aortic Stenosis
Aortic Stenosis
 MGMT of aortic stenosis
Aortic Stenosis
Daly, Gerard, MD, MSc,
Ferri's Clinical Advisor
2021, 147-151.e1

Management
strategy for
patients with
severe AS.
Periodic
monitoring is
indicated for all
patients in whom
AVR is not yet
indicated,
including those
with asymptomatic
AS (stage B or C)
and those with
low-gradient AS
(stage D2 or D3)
who do not mee...
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Elsevier, Inc. All rights
reserved.