Heart Diseases in Children

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    Acute rheumatic fever

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    Introduction

    Inflammatory diseases that involves heart, joint,CNS, skin and subcutaneous tissues

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    Epidemiology

    Most common cause of acquired heart disease inchildren

    Mainly in under developed countries

    First infection is usually btw 5-15 years old

    More common in girl > boy

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    Aetiology

    Streptococcus pyogenes (Group A hemolyticStreptococcus) pharyngeal infection

    This genetic susceptibility is inherited as an

    autosomal recessive gene

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    Pathogenesis

    Streptococci trigger autoimmune reaction

    inflammation

    - Not direct infection of the bacteria to the heart

    - Antibody produce by the infection showed to cross

    react with host tissue in the valvular tissue,myocardium, joint, subthalamic & caudate nuclei

    - Valvular damage in mitral and aortic, less in triscupid

    and pulmonary

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    Revised Jones criteria

    Major criteria- Sydenhams chorea (10%)

    - Subcutaneous nodules (rare)

    - Migratory polyarthritis or polyathralgia(80%), or

    aseptic monoarthritis

    - Carditis (50%)

    - Erythema marginatum (

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    Erythema marginatum

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    Minor criteria- Fever (temperature > 38 C)

    - Raised actue phase reactants, ESR > 30 mm/h or

    CRP > 30 mg/L

    - Prolonged PR interval

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    Recurrent attack of ARF

    - 2 major criteriaOr

    - 1 major criteria + 2 minor criteria

    Or

    - 3 minor criteria

    PLUS

    evidence of a preceding group A streptococcalinfection

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    ECG CXR

    Echo

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    Management

    Acute phase1. Bed rest

    2. Anti-streptococcal therapy

    IV C. Penicillin 50 000U/kg/dose 6H

    or oral Penicillin V 250 mg 6H (30kg) for 10 days

    oral Erythromycin for 10 days if allergic to penicillin.

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    3. Anti-inflammatory therapy

    Mild / no carditis:

    - oral Aspirin 80-100 mg/kg/day in 4 doses for 2-4

    weeks, taper over 4 weeks

    Pericarditis, or moderate to severe carditis:- oral Prednisolone 2 mg/kg/day in 2 divided doses for

    2 - 4 weeks,

    - taper with addition of aspirin as above.

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    4. Anti-failure medications - diuretics, ACE inhibitors, digoxin (to be used with

    caution).

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    Secondary Prophylaxis

    IM Benzathine Penicillin 0.6 mega units (30 kg) every 3 to 4 weeks

    oral Penicillin V 250 mg twice daily

    oral Erythromycin 250 mg twice daily if allergic to

    Penicillin

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    Duration of prophylaxis- until age 21 years or 5 years after last attack of ARF

    whichever was longer

    - lifelong for patients with carditis and valvular

    involvement.

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    Infective endocarditis

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    Introduction

    Microbacteria infection which cause exudative andproliferative inflammatory alteration of the

    endocardium or vascular endothelium

    An uncommon condition in children but has a high

    morbidity and mortality if untreated

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    Risk factors

    congenital heart disease repaired congenital heart defects

    congenital or acquired valvular heart diseases

    immunocompromised patients with indwelling central

    catheters

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    Aetiology

    1. Bacteria- Streptococcus viridans

    - Staphylococcus aerues

    - Community acquired enterococci

    - HACEK group

    Haemophilus spp

    Actinobacillus actinomycetemcomitan

    Cardiobacterium hominis Eikenella corrodens

    Kingella kingae

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    2. Fungus

    - Candida

    - Aspergillus- Histoplasma

    3. Others

    - Libman-Sacks endocarditis (SLE)

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    Clinical features

    Fever + new murmur is IE until proven otherwise

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    Symptoms

    Fever Lethargy

    Loss of appetite

    Loss of weight

    Arthralgia

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    Signs

    Peripheral stigmata- Splinter haemorrhage

    - Oslers node

    - Janeway lesion

    - Necrotic skin lesion

    - Clubbing (late)

    - pallor

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    Oslers node

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    Oslers node

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    Janeway lesion

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    Janeway lesion

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    Rothss spot on funduscopy

    Neurological signs from cerebral infaction

    New/changing murmur

    Splenomegaly

    Haematuria (microscopic)

    Arthritis

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    Modified Duke Criteria

    Major criteria

    1. blood culture positive:

    typical microorganisms from two separate blood

    cultures:

    - Viridans streptococci,- Streptococcus bovis,

    - HACEK group,

    - Staphylococcus aureus- community-acquired enterococci

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    2. evidence of endocardial involvement on

    echocardiogram

    - vegetation, abscess or dehiscence of prosthetic valve

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    Minor criteria

    1. Predisposing heart condition, prior heart surgery,

    indwelling catheter

    2. Fever, temperature > 38C

    3. Vascular phenomena:- major arterial emboli

    - septic pulmonary infarcts

    - mycotic aneurysm

    - intracranial hemorrhage,

    - conjunctival hemorrhages

    - Janeways lesions

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    4. immunologic phenomena:

    - glomerulonephritis- Oslers nodes

    - Roths spots

    - rheumatoid factor

    5. microbiological evidence:

    - positive blood culture not meeting major criterion

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    Diagnosis Definite IE

    Pathological criteria1. microorganisms by

    - Culture

    - histological examination of vegetation or intracardiacabscess specimen

    2. pathological lesions with active endocarditis

    Clinical criteria:

    2 major or1 major + 3 minor or

    5 minor

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    Possible IE

    - 1 major + 1 minor criteria

    or- 3 minor

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    Investigation

    Blood

    - FBC

    - ESR

    - CRP

    - BLOOD C&S

    - LFT

    - C3/C4

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    UFEME

    CXR

    ECG

    ECHO

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    Management

    Ensure 3 blood cultures taken before antibiotic

    therapy.

    Do not wait for echocardiography.

    Use empirical antibiotics,until culture results

    available

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    Guidelines on prophylaxis of IE

    Endocarditis prophylaxis recommended

    High-risk category

    - prosthetic cardiac valves

    - previous bacterial endocarditis

    - complex cyanotic congenital heart disease

    - surgical systemic pulmonary shunts

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    Moderate-risk category

    - other congenital cardiac malformations (other thanabove and below)

    - acquired valvar dysfunction (e.g rheumatic heart

    disease)

    - hypertrophic cardiomyopathy

    - mitral valve prolapse with regurgitation

    Endocarditis prophylaxis not

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    Endocarditis prophylaxis not

    recommended

    Negligible-risk category

    isolated secundum ASD

    repaired ASD & VSD

    patent ductus arteriosus (after 6 mths)

    mitral valve prolapse without regurgitation

    functional, or innocent heart murmurs

    previous Kawasaki disease without valvular

    dysfunction

    previous rheumatic fever without valvular dysfunction

    cardiac pacemakers and implanted defibrillators

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    Common procedures that require IE prophylaxis

    Oral, dental procedures

    - extractions, periodontal procedures

    - placement of orthodontic bands (but not brackets)

    - intraligamentary local anaesthetic injections

    - prophylactic cleaning of teeth

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    Respiratory procedures

    - tonsillectomy or adenoidectomy

    - surgical operations involving respiratory mucosa

    - rigid bronchoscopy

    - flexible bronchoscopy with biopsy

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    Gastrointestinal procedures

    - sclerotherapy for esophageal varices

    - oesophageal stricture dilatation

    - endoscopic retrograde cholangiography

    - biliary tract surgery

    - surgical operations involving intestinal mucosa

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    Genitourinary procedures

    - Cystoscopy

    - Urethral dilation

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    Standard general prophylaxis

    Oral Amoxicillin 50 mg/kg (max 2 Gm) one hour before

    procedure

    Or

    IV/IM Ampicillin 50 mg/kg (max 2 Gm)

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    Penicillin allergy

    Oral Clindamycin 20 mg/kg (max 600 mg)

    Or

    Oral Cephalexin 50 mg/kg (max 2 Gm)

    Or

    Oral Azithromycin/clarithromycin 50 mg/kg (max 500 mg)

    Or

    Oral Erythromycin 20 mg/kg (max 3 Gm)

    Or

    IV Clindamycin 20 mg/kg (max 600 mg)

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    Kawasaki disease

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    Introduction

    Also known as mucocutaneous lymph node

    syndrome

    Systemic febrile condition affecting children usually