Gerry B.

Acosta, MD, FPPS, FPCC

Pediatric Cardiology
 An inflammatory process mediated by an
immunologic reaction initiated by
Streptococcal Infection occurring in certain
susceptible individuals who have a
predisposition to this disease

 Itmay affect many parts of the body : the

heart, the joints, the brain , and the skin
Group A streptotococcus Susceptible Host
Positive for
Rheumatogenic strains:
HLA DR 4,2,1,3,7
Serotypes M1,3,5,6,18
Dw10 , DRw53
mucoid
And/or Allotype D8/17
Tissue / Organ Immune reaction

Inflammation of:
Cross-reactive antibody and/or cell
Heart Vascular
mediated immunity
Joints Connective tissue

Brain RHEUMATIC FEVER

 Strep Viral
Pharyngitis
Pharyngitis

Season cool, rainy month varies

Onset 5-15 y/o all ages

Initial Sx often abrupt more

gradual

Appetite poor retained

Cough, Colds absent present

Pharyngeal beefy, red,

homogenously
appearance granular ; boggy
ulcers
+ / - exudates minute
petechiae,
vesicles
 Children and young adults of school age
level, especially from 5-18 years of age

 Living in crowded conditions, in unsanitary

conditions , malnutrition
 There is no single clinical
manifestation or specific
diagnostic laboratory test
that unequivocably
establishes the diagnosis of
Rheumatic Fever
 Major Minor Manifestation Supporting Evidence
Manifestation Clinical Laboratory of Streptococcal
Infection

CARDITIS Arthralgia Acute phase UPDATE

reactants :
POLYARTHRITIS Fever Elevated Positive Throat culture or
Erythrocyte Rapid Strep Antigen Test
sedimentation rate
(ESR )
CHOREA Elevated or rising Strep
(+) C reactive Antibody titer of at least two
protein (CRP) fold from baseline
ERYTHEMA
MARGINATUM
Prolonged PR
interval
SUBCUTANEOUS
NODULE
 Polyarthritis
 Most common manifestation (75%)
 Latent period 1-3 mos.
 Affects large joints: ankle, knees, elbow, wrist
 Swelling heat, redness, joint pain, tenderness &
limitation of motion
 Asymmetric, Polymigratory
 Responds dramatically to ASA (48-72 hrs)
 Subsides 1-2 weeks with NO sequelae
 Low incidence of carditis and chorea
 Poststreptococcal arthritic condition does not fullfill Jones Criteria
 Clinical Features:
 Additive rather than migratory arthritis that responds

poorly to salicylates and non steroidal agents

 Persistence for mean of 2 months

 Elevated acute phase reactants

 Laboratory ( usually serologic) evidence of recent Group A

streptococcal infection
 6% develop mitral valve disease

 Antistreptococcal prophylaxis be administered for 1 year and then

discontinued if there is no evidence of cardiac involvement

Clinical Practice Guidelines: The evaluation and management of RF/RHD suspect

 Carditis
 Most serious manifestation & cause permanent
damage
 In 40-50% of patients
 Latent period of 1-3 mos.
Mild:
▪ Palpitation, shortness of breath, chest pain, murmur on
auscultation
Moderate to Severe:
▪ Difficulty of breathing, edema of the legs, presence of
murmur on auscultation, friction rub on auscultation, heart
enlargement on examination or in chest X-ray
 Severe carditis may last for 2-6 mos
 Erythema Marginatum

Occur in < 10% of pts.

Macular, non-pruritic, pink skin rash, with
pale center, serpiginous margin
Trunk & inner proximal portions of the
extremities and do not involve the face
Evanescent disappearing in exposure to colds
 Erythema Marginatum
 Subcutaneous Nodule
 In 2- 10% of cases
 Hard, painless non-pruritic, freely movable swelling &
0.2-2 cm in diameter
 Found symmetrically on the extensor surfaces of large
& small joints, scalp or along the spine
 Delayed appearance & lasts for weeks
 Significant association with carditis & with higher
mortality rate
 Chorea
 More common in prepubertal girls (8-12 yrs)
 Affects the basal ganglia and caudate nuclei
 Latent period 1-6 mos.
 Clinical manifestation appear > 3 months after
 No neurological sequelae
 Normal ASO titer & Acute phase reactants
 TRIAD: involuntary movement, muscular
weakness, emotional disturbance
 Arthritis
 Fever
 Anti Streptolysin O (ASO)
 Significant: >300 todd (children) ; >250 todd (adults)
 Elevated two weeks after Strep infection, peaks 2-4 weeks,
decrease in another 2 weeks
 ESR
 Normal or low in CHF
 Maybe elevated for 6 wks – 3 mos. if ARF is untreated
 CRP
 Not influenced by anemia or heart failure
 Reflects rheumatic activity more precisely than ESR
 Prolonged PR interval
 Major Minor Manifestation Supporting Evidence
Manifestation Clinical Laboratory of Streptococcal
Infection

CARDITIS Arthralgia Acute phase UPDATE

reactants :
POLYARTHRITIS Fever Elevated Positive Throat culture or
Erythrocyte Rapid Strep Antigen Test
sedimentation rate
(ESR )
CHOREA Elevated or rising Strep
(+) C reactive Antibody titer of at least two
protein (CRP) fold from baseline
ERYTHEMA
MARGINATUM
Prolonged PR
interval
SUBCUTANEOUS
NODULE
* The presence of two major criteria, or of one major and two minor criteria, indicates a high
probability of acute rheumatic fever, if supported by evidence of Group A streptococcal infection
Diagnostic Criteria Criteria
Primary episode of RF Two major or one major and two minor
manifestations plus evidence of a
preceding group A steptococcal infection

Recurrent attack of RF in a patient Two major or one major and two minor
without established RHD manifestations plus evidence of a
preceding group A steptococcal infection

Recurrent attack of RF in a patient Two minor manifestations plus evidence of

with established RHD preceding group A steptococcal infection

Rheumatic Chorea Other major manifestation or evidence of

Insidious onset rheumatic carditis group A steptococcal infection not
required

Chronic valve lesions of RHD Do not require any other criteria to be

(patients presenting for the first time with diagnosed as having rheumatic heart
mixed pure mitral stenosis or mitral valve disease disease
and/ or aortic valve disease)
 Group A Beta
Hemolytic Streptococcus
Primary
Prophylaxis

Susceptible Person
Secondary
Prophylaxis

ACUTE RHEUMATIC
FEVER

NO CARDITIS CARDITIS

NO RHD RHD
 AGENT DOSE MODE DURATION EVIDENCE

Benzathine 600,000 U for px < 27 kg IM Once II A

Penicillin G ( 60 lbs.)
1,200,000 U for px >27 kg.

Penicillin V Children: 250 mg 2-3 x daily Oral 10 days IIA

(Phenoxymethyl Adolescents and adults : 500mg 2-3
Penicillin) times daily

Amoxicillin 25-50mg/kg/day in three doses Oral 10 days IIA

First Generation Varies with agent Oral 10 days IIA

Cephalosporins

Erythromycin 40 mg/kg/d Oral 10 days IIA

Ethylsuccinate 2- 4 times daily ( max 1g /d)

Azithromycin 500 mg on first day Oral 5 days IIA

250 mg/d for the next 4 d
Clinical Practice Guidelines: The evaluation and management of RF/RHD suspect
 Agent Dose Mode Evidence
Benzathine 1,200,000 U every 4 Intramuscular IIA
Penicillin G wks (every 3 wks for
high-risk* pxs such
as those with
residual carditis)
Penicillin V 250 mg twice daily Oral IIA

For individuals
allergic
to penicillin and
sulfadiazine
250 mg twice daily Oral IIA
Erythromycin

Clinical Practice Guidelines: The evaluation and management of RF/RHD suspect

 Category Duration
After valve surgery Lifelong

More severe valvular disease Lifelong

Rheumatic fever with carditis 10 years after the last attack or

(mild mitral regurgitation or at least until 25 years of age
healed carditis) (whichever is longer)
Rheumatic Fever without proven 5 years after the last attack or
Carditis until age 21 of age (whichever is
longer)

World Heart Federation Diagnosis and Management of ARF/RHD,2007

 Clinical Severity
Arthralgia or mild arthritis;
no carditis
Moderate or severe arthritis;
no carditis, or carditis with or
without cardiomegaly, but
without failure
Carditis with failure; with or
without joint manifestation
Treatment
Analgesics only
Aspirin 90 – 100 mg/kg/day for 2
weeks; longer if necessary at 60-70
mg/kg/day
Prednisone, 40-60 mg/day; after
2-3 weeks slow withdrawal to be
completed in 3 more weeks
Aspirin to overlap for 4-6 weeks after
discontinuation of Prednisone
 Risk of serious reaction is reduced in children
under 12 yrs. old
 Duration of prophylaxis does not appear to
increase the risk of an allergic reaction
 WHO
▪ Allergic reaction: 3.2%
▪ Anaphylaxis : 0.2%
 Warm cold syringe to room temperature between hands
 Apply gentle pressure for 10 seconds with the finger or
thumb before injection
 Ensure that skin swabbed with alcohol is dry before
injecting
 Deliver the injection slowly ( preferably at least 2 or 3
minutes)
 Use distraction to focus attention away from the
injection
 Encourage movement ( e.g. walking) following injection
 Add 1 ml 1% Lidocaine to syringe following preparation
( if available)
 Degree of crowding in the family
 A family history of RF/RHD
 Socioeconomic and educational status of the
individuals
 Risk of streptococcal infection in the area
 Whether the patients is willing to receive
injections
 Occupation or place of employment of the patient
 Age : ARF recurrence is less common after age 25 and rare after
age 40
 Severity of RHD: additional ARF illness could be life
threatening for people with moderate RHD and following
valve surgery
 Carditis during initial ARF: Early heart damage increases the
risk of further damage with recurrent ARF
 Length of time since last ARF: ARF is less common more than 5
years since last episode
 Compliance : Regular prophylaxis in the first few years after
the initial ARF may provide greater protection from
recurrences than irregular prophylaxis for many years
 Disease progression: Evidence of worsening RHD at any stage
may require extended prophylaxis
 Prompt and proper treatment of Strep throat
 Proper nutrition
 Environmental and personal hygiene
 Avoidance of overcrowding
 Consult a doctor if with sorethroat

 If with RF, comply with monthly Benzathine

Penicillin injection to prevent RHD
 It is the result of the inflammation and
scarring of the heart valves caused by the
rheumatic fever
 Result as complications of rheumatic fever
Fibrosis of Heart Valve

Impaired Hemodynamic

Congestive Heart
Failure
Heart valves are scarred due to healing process following ARF
RHD is more likely to develop following ARF if
• The initial episode of ARF was severe
• The heart was affected with ARF
• ARF occurred at a young age
• There has been recurrent ARF

50% of people with RHD do not remember

having ARF
Symptoms of RHD may not show for many years
 A murmur but no symptoms usually suggests mild-moderate
disease
 Symptoms usually suggest moderate-severe disease
Symptoms depend upon the type and severity of the valve lesion,
and may include
 Breathlessness with exertion or when lying down flat
 Waking at night feeling breathless
 Feeling tired
 General weakness
 Peripheral oedema
 Mitral valve is affected in over 90% of cases of RHD
 Mitral regurgitation most commonly found in children & adolescents
 Mitral stenosis represents longer term chronic disease, commonly in
adults
 Most common complication of mitral stenosis is atrial fibrillation

 Aortic valve next most commonly affected

 Generally associated with disease of the mitral valve.
 Tends to develop as a long term complication of aortic regurgitation

 Tricuspid and pulmonary valves are much less commonly

affected
 Usually affected in very severe RHD when all valves are affected
 Electrocardiogram (ECG)
 Determine sinus rhythm
 Detect ventricular failure
 Chest X-ray (CXR)
 Determine size and placement of heart
 Detect cardiac failure (pulmonary congestion)
 Echocardiography
 Detect heart valve damage
 Estimate severity of disease
 Useful to compare results with later follow-up
 Secondary prophylaxis
Secondary prophylaxis
Secondary prophylaxis
Function of secondary prophylaxis with established RHD
 Prevent Group A Streptococcal infections
 Prevent the repeated development of ARF
 Prevent the development of RHD
 Reduce the severity of RHD
 Help reduce the risk of death from severe RHD.
 Pitfalls in Diagnosis and Management of Rheumatic Fever
Number
MYTH PEARL
1. Elevated ASOT = rheumatic fever Not in 95% of patients
2. Single ASOT is usually sufficient A two fold rise is more meaningful

3. Negative ASOT excludes acute rheumatic Not in certain circumstances. Anti D Nase B
fever adds value to the screen. Late onset chorea
may not have positive titers. 15% of patients
may have negative ASOT.
4. Arthralgia and fever with high ASOT = At least one major criteria is needed for the
ARF diagnosis of ARF
5. ASOT is not positive in other diseases Positive in 30% of children with SOJIA
6. Arthritis for more than 12 weeks needs Arthritis for more than 8-12 weeks virtually
Penadur excludes ARF
7. Neck, Back and small joints of the hands These joints are very infrequently involved in
are involved commonly in ARF ARF
8. Carditis occurs in all patients 50% of patients don't develop carditis

9. Pericarditis in isolation occurs in ARF It always occurs as a part of pancarditis

10. Steroids are needed for treatment of There is no indication for steroids for the
arthritis treatment of arthritis
11. Echocardiography does not add value to If available and affordable it adds detail to
patient assessment examination and diagnosis.
12. Steroids alter the long term cardiac They don't impact on long term cardiac
outlook disease.