Rheumatic fever & Rheumatic

Heart Disease
 OBJECTIVES
 Define rheumatic fever and rheumatic heart disease.

Describe the aetiology, risk factors, clinical manifestations,

diagnostic criteria and management of RF

 Explain the pathophysiology and symptoms in RF & RHD.

 Explain the management of RF & RHD.

 Describe about preventive methods.

 INTRODUCTION
• Rheumatic heart disease (RHD) is the most common
acquired heart disease in children, especially in
developing countries.

• RHD is a chronic heart condition caused by rheumatic

fever that can be prevented and controlled.
 WHAT IS
RHEUMATIC FEVER ?
 RHEUMATIC FEVER
• Rheumatic fever is an immunologically mediated
inflammatory disorder, which occurs as a sequel to
group A streptococcal pharyngeal infection.

• Multisystem disease affecting connective tissue

particularly of the heart, joints, brain, cutaneous and
subcutaneous tissues

• RF – not a communicable disease but results from

a communicable disease (streptococcal
pharyngitis).
• RF  RHD (rheumatic heart disease);
a crippling disease.

• Epidemiological point of view these cannot be

separated.
[WHO CHRONICLE 1969]

• RF and RHD  diseases of the poor most

prevalent in underdeveloped and developing countries.

• The clinical course of rheumatic fever involves a

childhood infection with complications in adulthood
(cardiac defect).
 EPIDEMIOLOGY
• RF and RHD is the most common cause of heart
disease in 5-30 age groups throughout the world.

• It accounts for 12-65% of hospital admissions related to

CVD in developing countries.
IN
INDIA
• RHD is prevalent in range of 5-7/1000 in 5-15 age
groups.

• About 1 million cases of RHD

• RHD constitutes 20-30% hospital admissions due to

CVD.

• Streptococcal infections common in children living in

under –privileged conditions and RF accounts for 1- 3%
of the cases.
 RISK FACTOR

• Age: 6-15 years

• Untreated streptococcal infection

• Familial predisposition

• Over crowding

• Poverty ,Poor hygiene

• Lack of access to medical care

 CAUSES
• Everyday oral activities. Activities such as brushing your
teeth or chewing food can allow bacteria to enter your
bloodstream
• An infection or other medical condition. From an
infected area, such as a skin sore. Gum disease, a
sexually transmitted infection.
• Weakened immune system
• Certain dental procedures. Some dental procedures
that can cut your gums may allow bacteria to enter your
bloodstream.
 PATHOGEN
• S. Pyogenes also known as Group A Streptococcus (GAS)
is the causative agent in Group A Streptococcal infections
including Streptococcal pharyngitis, acute rheumatic
fever , scatlet fever, and acute
glomerulonephritis.
 PATHOPHYSIOLOGY
• Based on currently based evidence, RF is caused by
group A streptococcal (GAS) pharyngeal infection.
• Postulated that series of preceding streptococcal
infection is needed to prime the immune system prior
to final infection that directly causes the disease.

• Group A strep pharyngeal infection

precedes clinical manifestations of
ARF by 2 - 6 weeks.
 PATHOPHYSIOLOGY
• Body produce antibodies against streptococci .

• These antibodies cross react with human tissues

because of the antigenic similarity between
streptococcal components and human connective
tissues (molecular mimicry) [there is
certain amino acid sequence that is similar btw GAS and
human tissue]

• Immunologically mediated inflammation & damage

(autoimmune) to human tissues which have antigenic
similarity with streptococcal components- like heart, joint,
 PATHOLOGIC LESIONS
● Fibrinoid degeneration of connective
tissue, inflammatory edema, inflammatory cell
infiltration & proliferation of specific cells
resulting in formation of A shcoff nodules,
resulting in-
-Pancarditis in the heart
-Arthritis in the joints
-Ashcoff nodules in the
subcutaneous tissue
-Basal gangliar lesions resulting in chorea
PATHOPHYSIOLOG
Y
CLINICAL
FEATURES
STREPTOCOCCUS SORE
THROAT
• Tender lymph nodes
• Strawberry tounge
• Excoriated nares( crusted lesions) in infants
• Tonsillar exudates in older children
• Abdominal pain
• More common in winter/ rainy season.
 Clinical features of RF
• Streptococcal sore throat with
fever
• Recurrence of fever with
manifestation of acute rheumatic
fever
• Shortness of breath
 FEATURES
Following upper airway infection with GAS
Silent period of 2 - 6 weeks
Sudden onset of fever, pallor, malaise, fatigue.

Commonly GAS streptococcal infection is subclinical;

such cases confirmed using streptococcal antibody
testing .
• There is no definitive test.

• Diagnosis of ARF relies on presence of combination of

typical clinical features together with evidence of the
precipitating GAS infection .

• This uncertainty led Dr.T.Duckett Jones in 1944 to

develop a set of criteria Jones Criteria to aid
diagnosis.

• Now Diagnosis based on MODIFIED JONES

CRITERIA .
 Major criteria: oint
• Migratory polyarthritis of large joint
• Usually >5 joints affected and mainly large
joints Knees, ankles, wrists, elbows, shoulders
• Redness, warmth, swelling, pain , movement
limitation
• Quick to appear, lasts 3- 7 days, subside and appear in
other joint.
• Respond to salicylates and NSAIDS
• Commoner in older patients.
• Small joints and cervical spine less commonly
 Major criteria: Carditis
• Early manifestation 90% (within 2 weeks of onset)
• Pancarditis
▫ Pericarditis : precordial pain, friction rub, ST and T
changes
▫ Endocarditis: pansystolic murmur of MR w/wo AR
murmur
▫ Others : cardiac enlargement, soft S1, S3 gallop,
congestive cardiac failure,
Carditis is the only manifestation of rheumatic fever that
leaves a sequelae & permanent damage to the organ
Carditis
ECG: Prolonged PR interval
 Major criteria: odules
• Late manifestation 3- 20% ( 6 weeks after onset of RF)
• Non- tender subcutaneous nodules on bony
prominence
• Small, painless, mobile hard lumps beneath
skin.
• Most common along -
extensor surfaces of joint-Knees, elbows,
wrists
• Also: on bony prominences, tendons, dorsi of feet,
oicnciput or cervical spine.
Nodules -Firm, non-tender, isolated or
in clusters
 Major criteria:
rythema marginatum
• Early manifestation <3%
• Erythematous, serpiginous, non-pruritic, macular
lesions with pale centre.
• Pink macules - Clear centrally ,serpiginous spreading
edge. (has slightly elevated red margins)
• More on trunks & limbs, Almost never on face.
• Worsens with application of heat.
• Often associated with chronic carditis.
 Major criteria :
ydenham’s Chorea
• Neurological manifestation of rheumatic fever
• Late manifestation 10-15% ( 3 months after onset)
• Semi- purposeful , jerky movements  deranged
speech, muscular incoordination, facial grimacing
▫ Exacerbated by stress and disappears with sleep
• More common in females
• Clinical maneuvers to elicit chorea:
▫ Demostrate milkmaid’s grip
▫ Handwriting examination
• Self- limiting ( 2-6 weeks)
Chorea - demonstration
Milkmaid’s grip

Spooning and pronation of the hands when the patient’s arms

are extended

Pronator sign: Inability to hold the hand above the head with
palms facing each other it results in pronation of forearm and
palm face outwards
Wormian darting movements of the tongue upon protrusion (Jack
in the Box)

Examination of handwriting to evaluate the motor movements

Hung up Reflux: On eliciting Knee jerk: knee extends and the leg
and foot hangs up in the mid air due to chorea.
 MINOR CRITERIAS
• FEVER

• Present at onset of acute illness

• High grade fever >39ºC
• Lasts for about 12 weeks, tends to
recur.
• Alternates with normal temperature.
• Weakness ,malaise , weight loss ,
anorexia
 MINOR CRITERIAS
Arthralgia
• Pain in one or more joints
• Without the presence of arthritis
• Epistaxis
• Abdominal pain
 LAB DIAGNOSIS
• High ESR
• Anemia, leucocytosis
• Elevated C-reactive protien
• Elevated ASO or other streptococcal antibody titer
• Anti-DNase B test
• Throat culture-GABH streptococci
• ECG: prolonged PR interval
 OTHER FEATURES
• Aschoff bodies are nodules found in the hearts of
individuals with rheumatic fever. They result from
inflammation in the heart muscle .

Aschoff cells
 Some Important Terminology
A new episode of rheumatic fever following
01 Recurrence another GABHS infection, occurring after
8 weeks following stopping treatment

Manifestations of rheumatic fever

02 Rebound occurring within 4-6 weeks of
stopping treatment or while tapering

ARF drugs
Worsening of rheumatic fever while
03 Relapse
Course under treatment and often with
carditis

04
Subclinical When clinical exam is normal but
echocardiogram is abnormal.
carditis (30%)

Indolent Persistent features of CHF, murmur and

05 cardiomegaly. No or very few features of
carditis.
carditis
 DIAGNOSIS OF RF
JONES CRITERIA (REVISED)
Jones Criteria (Revised) for Guidance in the Diagnosis of
Rheumatic Fever – 2015 revision

Acute RF occur in > 2/100 000

01 school age children.

Moderate
Rheumatic heart disease is diagnosed in
/ High risk > 1/1000 patients at any age during one year

population

Acute RF occur in ≤ 2/100 000 Low Risk

02
school age children. Population

Rheumatic heart disease is diagnosed in

≤ 1/1000 patients at any age during one year
Jones Criteria (Revised) for Guidance in the Diagnosis of Rheumatic
Fever – 2015 revision
Major Criteria
Low Risk Population High Risk Population
Carditis (clinical or subclinical) Carditis (clinical or subclinical)
Arthritis – only polyarthritis Arthritis – monoarthritis or polyarthritis
Chorea Polyarthralgia
Erythema marginatum Chorea
Subcutaneous nodules Erythema marginatum
Subcutaneous nodules
Minor Criteria
Low Risk Population High Risk Population
Polyarthralgia Monoarthralgia
Hyperpyrexia (>38.50C) Hyperpyrexia (>380C)
ESR > 60 mm/h and/or CRP > 3.0 mg/dl ESR > 30 mm/h and/or CRP > 3.0 mg/dl
Prolonged PR interval (age related) Prolonged PR interval (age related)
Jones Criteria (Revised) for Diagnosis of
Rheumatic Fever 2015 Revision
Diagnosis of a first attack Diagnosis
of recurrent acute
• 2 major manifestations,
• 2 major manifestations, OR
OR • 1 major and 2 minor,
• 1 major and 2 minor manifestations, PLUS
PLUS • Evidence of recent GAS infection

• Evidence of recent GAS infection. • 3 minor manifestations (in

the Moderate/High-Risk
population),
PLUS

Supporting evidence of antecedent group A streptococcal infection:

• Positive throat culture or rapid streptococcal antigen test
• Elevated or increasing streptococcal antibody titer (>250 U/dL)

(Essential Criteria)
Exceptions to Jones
criteria

1) Chorea

2) Indolent carditis with insidious onset

3)Previous h/o acute rheumatic fever in

particularly high-risk populations or RHD
Major manifestations Carditis

Polyarthritis

Chorea

Erythema marginatum

Subcutaneous nodules

Minor manifestations Clinical: fever, polyarthralgia

Laboratory: elevated erythrocyte

sedimentation rate or leukocyte counte

Electrocardiogram: prolonged P-R interval

Supporting evidence of a preceding Elevated or rising anti-streptolysin O or

streptococcal infection within the last 45 other streptococcal antibody, or
days
A positive throat culture, or

Rapid antigen test for group A

streptococcus, or

Recent scarlet fevere

 Differential Diagnosis
ARTHRITIS CARDITIS CHOREA
Juvenile idiopathic arthritis Viral myocarditis Huntington chorea
Reactive arthritis (e.g., Viral pericarditis
Shigella, Salmonella, Wilson disease
Yersinia)
Serum sickness Infective endocarditis Cerebral palsy
Sickle cell disease Kawasaki disease Tic disorder
Systemic lupus erythematosus Mitral valve prolapse Hyperactivity
Innocent murmurs
Lyme disease (Borrelia burgdorferi)

Pyogenic arthritis Congenital heart disease

Post-streptococcal reactive arthritis
Treatment of RF - Principles
1. Bed rest

2. Eradication of Streptococci

3. Anti inflammatory therapy

4. Treatment of CCF

5. Treatment of Chorea

6. Prevention of Recurrences

7. Surgical : Acute and Chronic

Treatment - GENERAL MEASURES
• Mild : Questionable cardiomegaly.
• Moderate : Definite but mild cardiomegaly.
• Severe : Marked cardiomegaly or heart
failure.

Measures ARTHRITS MILD CARDITIS MODERATE SEVERE

ONLY CARDITIS CARDITIS

BEDREST 1-2 Weeks 3-4 Weeks 4-6 Weeks As long as CHF

present
INDOOR 1-2 Weeks 3-4 Weeks 4-6 Weeks 2-3 months
AMBUALTION
Anti-streptococcal treatment
• A single injection of benzathine penicillin is given
when the diagnosis of rheumatic fever is made.
– 1.2 million unit (>27 kg) after sensitivity test
0.6 million units(< 27 kg)
• Penicillin V
– 250 mg four times a day for 10 days) is an alternative;
• To those with penicillin allergy. (Oral)
– Erythromycin (20 mg/kg/dose max 500 mg for 10
days OR
– Azithromycin 12.5 mg/kg once daily for 5 days
 Treatment - Arthritis
Anti-inflammatory treatment
Aspirin Regimen I
Starting dose: 100 mg/kg/d for 2-3 weeks
Tapering dose: once symptoms resolver, 60-70
mg/kg/s Total 12 weeks
Regimen II
50-60 mg/kg/d for total of 12 weeks

Neproxen 10-20 mg/kg/d for 12 weeks

(if Aspirins intolerance
detected, or no response
to aspirin in 4 days)

• Switch over to steroids if no response within 4 days of treatment.

• R/o inflammatory myeloproliferative disorders
 Treatment - Carditis
• Carditis alone – Aspirin only
• Carditis with cardiomegaly – Aspirin only
• Patients with carditis and more than minimal cardiomegaly
and/or congestive heart failure should receive corticosteroids .

Steroids:
• Predinisalone: 2-2.5 mg/kg/d in 4 divided doses for 2 weeks (till
ESR normalizes) and by half the dose for 2-3 wk and then tapering
of the dose by 5 mg/24 hr every 2-3 days.
• When prednisone is being tapered, aspirin should be started at 50 • Decongestive measures
-75mg/kg/day in 4 divided doses for 6 wk to prevent rebound of • Diuretics, digitalis
inflammation.- (complete total 12 weeks)
• Methyl prednisolone IV: If no response to oral steroid therapy.
• Bed rest
30 • Low sodium diet
mg/kg/d for 3 days
 Treatment - Chorea

• Anti inflammatory agents not needed

• Phenobarbitone (16-32 mg every 6-8 hr PO) – drug of
choice
• Haloperidol: 0.01 - 0.03 mg/kg/d in 2 divided doses
orally
• Chlorpromazine: 0.5 mg/kg Q4 or 6H Oral
• Sodium valproate: 15mg/kg/d • Treatment should be
• Carbamazapine: 7-20 mg/kg/d continued for 2-4 weeks after
• Steroids – in some cases clinical improvement .
Management of Complications
• Heart Failure
– Reduce physical activity
– Monitor fluid balance
– Treat anemia with iron/pRBC
– Drugs
• Digoxine 10 mcg/kg total digitalization dose and 7.5 mcg/kg
maintenance dose
• Diruretics: Frusemide 0.5 – 2 mg /kg/d
• Captopril: 0.25 mg/kg/d
– Surgery
• For sever MR due to chordal rupture leading to refractory
CHF
 Prevention of RF

Primordial Preventing Strep throat - ? Vaccine – not avb

Primary Treating Strep throat infection

Secondary Preventing Rheumatic
recurrence by
chemoprophylaxis
Tertiary Treating RHD
 Primary Prophylaxis

• Vulnerable children from 5 – 15 yrs with pharyngitis

• ORAL
– Penicillin V 250-500 mg BID for 10 days
– Amoxicillin 50 mg/kg OD for 10 days
– Azithromycin 12 mg/kg/d OD for 5 days
– First gen Cephalosporin – 10 days
– Azithromycin 12 mg/kg/d OD – 5 days
• PARENTARAL
– < 27 Kg single dose IM Benzathine Penicillin 600,000 U
– > 27 kg single dose IM Benzathine Penicillin 1200,000 U
AHA Recommendation
 Secondary Prevention of Rheumatic Fever
(Prevention of Recurrent Attacks)

Agent Dose Mode

Benzathine penicillin G 1 200 000 U >27 AND 600,000 Intramuscular

U < 27kg every 4 weeks*
or
Penicillin V 250 mg twice daily Oral

For individuals allergic to penicillin and sulfadiazine

Erythromycin 250 mg twice daily Oral

Recommendations of American Heart

 Duration of Secondary Rheumatic Fever
Prophylaxis
Category Duration
Rheumatic fever without carditis At least 5 y or until age 21y,(whichever
is longer)
Rheumatic fever with carditis and At least 10 y since last residual
heart disease (persistent valvular episode and at least until age 40 y
disease*) sometimes lifelong prophylaxis

RF with carditis disease but no residual Rheumatic fever 10 y or well into

heart disease (no valvular disease*) adulthood (whichever is longer )

More severe valvular disease Lifelong

Post- valve surgery cases
*Clinical or echocardiographic
evidence.

Recommendations ofofAmerican
Recommendations Heart
Am erican Heart Association
Association
 PROGNOSIS
• Rheumatic fever can recur whenever the individual
experience new GABH streptococcal infection, If not on
prophylactic medicines.

• Good prognosis for older age group & if no carditis

during the initial attack

• Bad prognosis for younger children & those with

carditis with valvar lesions
 RHEUMATICHEART
DISEASE
• Rheumatic heart disease is an immunologic disease
characterized by valvular damage or dysfunction followed
by one or more episodes of rheumatic fever caused by
pharyngeal infection with GAB hemolytic streptococci.

• Rheumatic Heart Disease is the permanent heart valve

damage resulting from one or more attacks of ARF.

• It is thought that 40-60% of patients with ARF will go on to

developing RHD.

• Sadly, RHD can go undetected with the result that

patients present with debilitating heart failure.
 RHEUMATIC HEART DISEASE
• RHD is the result of damage to the heart valves which
occur after repeated episodes of ARF

• The order of frequency of involvement depends on the

hemodynamic stress placed on the various chambers

• Thus the order is mitral> aortic > tricuspid> pulmonic

65-70% 20-25% 10% Rarely

• Valve incompetence is more common than stenosis

 The extent of the damage
depends on the heart area that
the disease strikes
 PATHOPHYSIOLOG
Y
Causative agent
Group A Beta-hemolytic streptococci

Untreated strep throat

Rheumatic fever

All layers of the heart and the mitral valve become inflammed

Vegetation forms

Valvular Regurgitation
and stenosis

Heart Failure
 THE DIFFERENCE

Normal valve is RHD: Thick, fibrous

Transparent, scarred stenotic &
avascular, thin fixed (MS/MR)
flexible membrane. with Blood Vessels.
High pulse
rate

mitral or aortic regurgitation-

Murmur
endocardium involved

Cardiomegaly myocardium
involvement
Pericardial
friction rub Pericarditis

Prolonged Myocardial inflammation

PR interval affecting electrical
conduction
Cardiac
failure
VALVE INVOLVEMENT IN RHD
 VALVE CHANGES
• Leaflet thickeninig

• Commissural fusion

• Shortening and thickening of chordae

• Orifice is narrowed
 VALVE INVOLVEMENT IN RHD
• MITRAL Valve is affected in 60 – 70% of cases
🞄 Mitral regurgitation most commonly found in
children and adolescent.
🞄 Mitral stenosis represent longer term chronic
disease, commonly in adults.
🞄 Most common complication in mitral stenosis is
atrial fibrillation.
MITRAL
STENOSIS
 VALVE INVOLVEMENT IN RHD
• AORTIC Valve next most commonly affected
🞄 Generally associated with diseases of the mitral
valve.
🞄 Aortic stenosis is one of the most common and
most serious valve disease problems in elderly
population.

• Tricuspid and pulmonary valves re much less

commonly affected
🞄 Usually affected in very severs RHD when all
valves are affected.
 COMPLICATION
S failure
• Congestive heart

• Infective endocarditis

• Arrhythmias mainly atrial fibrillation

• Embolic episodes

• cardiomegaly
 TREATMENT for VALVULAR
HEART DISEASE

MEDICAL- digoxin, diuretics, antibiotic prophylaxis,

control arrythmias.

SURGICAL- closed mitral commisurotomy, percutaneous

transluminal ballon valvuloplasty,

 OTHERS –Ross procedure, bentalls procedure

 Balloon MITRAL
Valvuloplast COMMISSUROTOMY
y
• Elongated leaflets
▫ Leaflet plication
▫ Leaflet resection
• Holes in the leaflets
▫ Pericardial patch repair
• Short leaflets
▫ Most often repaired by chordoplasty
• Repair of the chordae tendinae
• Mostly used for mitral valve
• Gore-Tex can be used to create chordae tendinae.
ROSS PROCEDURE
• Performed when valvuloplasty is not suitable
• Approached through a median sternotomy or mitral
valve (at times) – right thoracotomy incision
• Two types of prosthetic valves :-
▫ Mechanical valves
▫ Tissue(biologic) valves
▫ Caged ball valve (Starr-Edwards)
▫ Tilting disc valve (Medtrionic-Hall)
▫ Bileaflet valve(St. Jude Medical)
▫ Trileaflet valve

Caged ball valve Tilting disc valve Bileaflet valve

PREVENTION
 PREVENTION

SECONDARY-
Secondary prevention
is directed at
PRIMARY-10 days preventing acute
course of penicillin GABHS pharyngitis in
therapy; patients at substantial
risk of recurrent
acute rheumatic fever
KEY MESSAGE