Acquired Heart Disease in Children Acute CA

8/13/2019 Acquired Heart Disease in Children Acute CA

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Acquired Heart Disease

in Children

Jill Narron, M.D.

Clinical Assistant Professor of Pediatrics

Boonshoft School of MedicineWright State University


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Acute Rheumatic Fever

Most common cause of acquired heart disease inchildren worldwide

Estimated that ARF and RHD affect nearly 20 millionpeople in developing countries worldwide

Leading cause of CV death in first 5 decades of life Localized outbreaks in US in mid-1980s

Caused by untreated Group A -hemolyticstreptococcal pharyngitis in susceptible host

Occurs ~ 3 weeks after asymptomatic latency period

History of preceding sore throatmay not be evident


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Epidemiology

Usually seen in school age children (age5-15 years) and rare before age 5 in U.S.

Also seen in at-riskadults

Military recruits Parents of school-age children in crowded

housing

Male=Female in incidence

Peak occurrence in spring in U.S. All socioeconomic groups affected


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Pathogenesis: Current Theory

Group A

streptococcus

Susceptible

Host

Immune

Reaction

Tissue/Organ

Inflammation

ACUTE RHEUMATIC FEVER


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The Jones Criteria

Major Minor

Migratory polyarthritis

Carditis Sydenhams chorea

Subcutaneous

nodules

E. marginatum

Elevated ESR

Increased PR interval Fever

Arthralgia

Prior history of ARF

Plus.Evidence of a previous strep infection!


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Cardiac Pathology

Myocarditis Poor function

Aschoff body

Pancarditis

Addition of pericarditis Pericarditis RARE without endocarditis or

myocarditis


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More Major Manifestations

Erythema marginatum Subcutaneous nodules

From:

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Acute Rheumatic FeverLaboratory Findings

Elevated CRP or ESR

+ Throat Culture

Elevated WBC

HematuriaElevated ASO, or

Anti-DNase B, or anti-

Streptokinase, or anti-

hyaluronidase

90%

33%

80%

6%95%


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Antibiotic RegimensPrimary Prevent ion

Preferred agents:

-Benzathine penicillin G (IM) x 1 dose

-Penicillin V (PO) x 10 days

-Amoxicillin (PO) x 10 days

In PCN-allergic patients:

-Narrow spectrum cephalosporin (PO) x 10days

-Clindamycin (PO) x 10 days

-Azithromycin (PO) x 5 days-Clarithromycin (PO) x 10 days


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Treatment of Carditis

Mild

-ASA 90-100 mg/kg/day x 4-8 weeks-Taper based on clinical status, ESR, CRP

Moderate (Cardiomegaly, Pericarditis)-Bedrest-Prednisone 1 mg/kg/day x 7 10 days- ASA before steroids are withdrawn

Severe (Congestive heart failure)

-Bedrest-Prednisone 4 6 weeks (dontforget to taper!!)-ASA before steroids are withdrawn-Digoxin, diuretics, afterload reducing agents, etc.


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Antibiotic Regimens

Length of prophylaxis depends on presence ofcarditis with initial episode & residual valve disease

-NO carditis: at least 5 years or until age 21

-WITH carditis but no residual valve disease: atleast 10 years or until age 21-WITH residual valve disease: at least 10 yearsand at least until age 40, sometimes lifelong

Endocarditis (SBE) prophylaxis is required forpatients s/p valve replacement using a different

antibiotic from that prescribed for RF prophylaxis


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Infective Endocarditis

Defined as microbial infection ofendothelial surface of heart

Native or prosthetic valves most frequentlyinvolved

Septal defects also involved along withintravascular foreign devices (patches,surgical shunts, IV catheters)

Previously occurred with underlyingrheumatic heart disease


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Pathogenesis

Pre-existing congenital or acquired lesionUSUAL

Indwelling catheters also possible

Vegetations usually occur where there ispressure gradient causing turbulent bloodflow

Damage to endothelium -> formation ofnonbacterial thrombotic endocarditis

(NBTE) -> occurrence of transientbacteremia -> adherence of bacteria toNTBE -> proliferation of bacteria withinvegetation


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Clinical Findings

Fever Nonspecific symptoms

(HA, myalgia, arthralgia, malaise) Murmur (new or changing) Heart failure Petechiae Embolic phenomena Splenomegaly Neurologic findings Osler nodes, Janeway lesions, Roth spots,

Splinter hemorrhages

+++++++

++++++++++++

+

Neonates may have few specific symptoms


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Clinical Manifestations

Splinter

hemorrhage

Osler nodes

Conjunctival

petechiae

Janeway

lesions

From: NEJM 345(10), 739:2001


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Therapy

Bactericidal IV antibiotics (typically 4-6

weeks; sometimes longer with prosthetic

valves)

Surgical intervention more likely with: Significant embolic events

Persistent infection

Progressive cardiac failure

Prosthetic valve

Fungal endocarditis poor prognosis


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Indicated Procedures

Dental work involving manipulation ofgingival tissue or periapical region of teethor perforation of oral mucosa

Procedures on respiratory tract involvingperforation of mucosa

Procedures on infected skin, skinstructures, or musculoskeletal tissue

NO LONGER recommended for GI or GUprocedures


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Prevention (SBE Prophylaxis)An t ib io t ic Regimens

Typically Amoxicillin 50 mg/kg (max dose 2

grams) one hour prior to procedure

See AHA Card for complete details


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Epidemiology

Highest rate in Asian population

Age: mean 18-24 months; ~75% < 5 yo

Male/female ratio=1.5:1

More common in winter and early spring

No etiologic agent identified to date


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Making the Diagnosis

Classic- Fever of 5 daysduration and at

least 4 of 5 principal criteria

Fever and fewer than 4 other criteria are

diagnostic when coronary artery abnormality

identified

In presence of 4clinical criteria, AHA now

states that diagnosis can be made on day 4 of

fever

Exclusion of other diagnoses


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Principal CriteriaConjunctivitis & inflamed oral mucosae


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Principal CriteriaStrawberry tongue


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Principal CriteriaRash


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Principal CriteriaPalm erythema & swelling


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Principal CriteriaPeriungual desquamation


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Principal CriteriaCervical lymphadenopathy


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Associated Cardiac


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Associated Cardiac

Manifestations Echo findings

Coronary aneurysms

Pericardial effusion

Mitral insufficiency

Ventricular dysfunction

A i d N di


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Associated Non-cardiac

Manifestations

GI abdominal pain, diarrhea, vomiting,hepatic enlargement with jaundice,gallbladder hydrops (~15%)

GU urethritis CNS irritability, transient sensorineural

hearing loss

M-S arthralgia, arthritis (~40%)


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Associated Lab Findings

Leukocytosis with left shift

Anemia

Elevated ESR, C- reactive protein

Thrombocytosis (500,000 to >1 million) during

subacute phase Elevated transaminases (~40%) & GGT

(67%)

Hypoalbuminemia

Sterile pyuria (~33%) Pleocytosis of CSF (~50%)

Higher Risk for


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Higher Risk for

Coronary Complications

Male gender

< 1 yo or > 8 yo

Prolonged &/or recurrent fever Other CV involvement (myocarditis,

effusion, arrhythmia)

Hypoalbuminemia, anemia

Thrombocytopenia

No or delayed therapy


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Treatment

Aimed at reduction of inflammation and

prevention of thrombosis by inhibiting

platelet aggregation

IV -globulin at 2 grams/kg/dose

+

Aspirin therapy at 80-100 mg/kg/day

within 10 days of onset

Treatment


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Treatment(Refractory Cases)

Retreatment with IVIG 2 grams/kg/dose

indicated for persistent or recurrent

fever 36 hours after 1st dose

completed

Steroids are controversial but may help

in cases refractory to 2 doses of IVIG


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Treatment

Reduce aspirin to 3-5 mg/kg/day(antiplatelet dose) once afebrile for 48-72 hours or at day 14 of illness

Discontinue aspirin 6-8 weeks afteronset of illness if no coronaryabnormalities identified

Long-term aspirin therapy is indicated if

coronary abnormalities present at 6-8weeks


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Acute Pericarditis

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Acute Pericarditis
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Acute PericarditisSymptoms

Precordial chest pain In up to 80% in children

Worse with coughing, breathing, or motion

Most comfortable in upright position

Fever

Tachycardia out of proportion to degree offever

Respiratory distress- uncommon unlesstamponade or pneumonitis also present

Acute Pericarditis


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Acute PericarditisSigns of Effusion

Friction rub Grating, scratching sound

Best heard during inspiration with patient

leaning forward

Ewarts sign

Subscapular dullness to percussion

Represents compression of left lung by

enlarged heart

Acute Pericarditis


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Acute PericarditisSigns of Tamponade

Low cardiac output

Elevated CVP

Pulsus paradoxus: > 10 mmHg fall in SBP

with inspiration Muffled or diminished heart sounds

JVD increasing with inspiration

(Kussmauls sign)

Hepatomegaly, peripheral edema

Acute Pericarditis


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Acute PericarditisClinical Findings

ECG PR depression

ST elevation

Low voltages in presence of large effusion

Chest x-ray Cardiomegaly if effusion present

Water bottle heartenlarged triangular heartwith smoothed-out cardiac borders with

massive effusion

Acute Pericarditis


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Acute PericarditisEtiologies

Infectious Rheumatic/collagen vascular

Drug therapy

Cardiac surgery- post-pericardiotomy

Renal failure Idiopathic up to 30% of cases (presumed

to be viral)

Infectious Pericarditis


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Infectious PericarditisBacterial

Life-threatening Mortality rates 25-75%

May be primary or secondary todissemination from another site (lung, brain,bone, joint)

Staph aureus 50-80% of cases

Also see H flu, Strep pneumo, and others

Treatment DRAINAGE

Antibiotics for 3-4 weeks



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Infectious PericarditisViral

Occurs more often in children Preceding respiratory or GI illness in 40-75% of

cases

Coxsackie, Echovirus, Adenovirus, Influenza, EBV,Mumps, VZV, HIV

Ill-appearing but not as toxic as in bacterialdisease

Friction rub in up to 80% of patients

Treatment Drainage if tamponade +/- pericardial drain

Bedrest, salicylates or NSAIDs, rarely steroids



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Infectious PericarditisTuberculous

More common in underdevelopedcountries

Onset may be insidious (weight loss, night

sweats, dyspnea, and chest pain)

Mantoux test positive (place anergy panel

in immunocompromised)

Combination therapy required due to drug

resistance

Non Infectious Pericarditis


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Non-Infectious Pericarditis

Rheumatic/Collagen VascularAcute rheumatic fever usually with pancarditis

JRAsymptomatic in 10%

SLE -symptomatic in 25%

Treatment: anti-inflammatory agents

Drug-induced

Hydralazine, isoniazid, procainamide

Cause lupus-like syndrome

Treatment: Stop drug; anti-inflammatory agents

Non Infectious Pericarditis


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Non-Infectious Pericarditis

Post-pericardiotomy syndrome Occurs in up to 30% following cardiac surgery

Symptoms: fever, chest pain, irritability,decreased appetite

Treat with aspirin 50-75 mg/kg/day for 4-6 weeks

Steroids (2 mg/kg/day) effective but increase riskof immunosuppresion

Uremic Sign of end-stage renal disease

Dialysis resolves most effusions

Anti-inflammatory agents aid with chest pain andfever but do not resolve effusions


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Quick Quiz!


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Quick Quiz!

Which valve is most commonly affected in

acute rheumatic fever?


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Quick Quiz!


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Quick Quiz!

What is the most common organism in

infective endocarditis?

Quick Quiz!


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Quick Quiz!

What is the most common organism in

infective endocarditis?

lpha-hemo lyt ic Strep

Quick Quiz!


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Quick Quiz!

What is the most common cause of acquired

heart disease in U.S. children?

Quick Quiz!


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Quick Quiz!

What is the most common cause of acquired

heart disease in U.S. children?

Kawasaki Disease

Bonus Quest ion !


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Bonus Quest ion !

Where did Dr. T. Duckett Jones graduate

from medical school?

Bonus Quest ion !


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Bonus Quest ion !

Where did Dr. T. Duckett Jones graduate

from medical school?

THE University o f Virg inia!

Acquired Heart Disease in Children Acute CA

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