Acquired Heart Disease in Children

Dr Ahmad Rustam bin Mohd Zainudin

MD, MMed (UKM)

Heart Disease in Children

Acquired Congenital

Acyanotic Cyanotic

Common Acquired Heart Diseases in Children

• Kawasaki Disease

• Acute Rheumatic Fever & CRHD

• Infective Endocarditis

• Myocardial Disease

• Cardiovascular involvement with systemic diseases

KAWASAKI DISEASE

“Acute febrile mucocutaneous lymph node syndrome with multisystem vasculitis mainly affecting infants and small children less than 5 years of age”

Definition

Tomisaku Kawasaki

History of Kawasaki Disease

• 1961- 1st case observed by Dr. Kawasaki

• 1965 - CA thrombosis 1st recognised on autopsy

• 1967 - First Japanese report" Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children: clinical observations of 50 cases."

Jpn J Allergol.1967; 16 :178 –222

• 1974 - First English language report

Epidemiology

• Median age of affected children = 2.3 years• 80% of cases in children < 4 yrs, 5% of cases in children

> 10 yrs• Males:females = 1.5-1.7:1• Rate of recurrence in 1-3%• Positive family history in 2.1% (10-15 x higher)• 13% risk of occurrence in twins• Incidence: 219 per 100,000 in Japan (2008), 20 per 100,000

in US (2006), 32.5 per 100,000 in Singapore

• Seasonal variation– More cases in winter and spring but occurs

throughout the year

Etiology

• Idiopathic• Hypothesis:

– Infectious agent – Superantigenic bacterial toxin– Immunologic response triggered by one of several

microbial agents– Genetics

• ITPKC gene• FCGR2A• BLK (encoding B-lymphoid tyrosine kinase) &

CD40

Clinical Features

Clinical Course

Coronary artery aneurysm

Circulation 103(2):335-336. 2001.

Flow chart of morphological changes in coronary artery lesions in Kawasaki disease

Senzaki H Circulation 2008;118:2763-2772

Incomplete & Atypical KD

Incomplete KD• Present with < 4 of 5

diagnostic criteria• Compatible laboratory

findings• No other explanation

for the illness• More common in

children < 1 year of age

Atypical KD• Atypical presentation• eg: renal impairment,

hydrops GB

Newburger J. et al. AHA - Circulation Oct 26 , 2004

Treatment

Potentially Beneficial Treatments for Kawasaki Disease

Agents Potentiallly Beneficial Actions

Abciximab Modulation of vascular remodelling

Urinistatin Neutrophil elastase inhibition

Pentoxifylline, Infliximab

Anti TNF alpha

Cyclosporin A, Cyclophosphamide

Immunomodulation

Plasma exchange Removal of substances causing vasculitis

Follow Up

Acute Rheumatic Fever & Chronic Rheumatic Heart

Disease

Causes

• Acute rheumatic fever is an immunologic response that occurs as a delayed sequelae of group A streptococcal infection of the pharynx but not of the skin.

• The attack rate of acute rheumatic fever after streptococcal infection varies with the severity of the infection, ranging from 0.3 % to 3%.

• Predisposing factors include: – FH of rheumatic fever, – low socioeconomic status (poverty, poor hygiene,

medical deprivation), – age between 6 and 15 years (with a peak incidence at

8 years of age).

Pathology

• The inflammatory lesion can found in the heart, brain, joints, and skin

• Rheumatic carditis was considered to be pancarditis • In rheumatic myocarditis, myocardial contractility is rarely

impaired and the serum level of troponin is not elevated. • The valve leaflets are heavily involved with fibrinous

vegetations on the coapting surfaces, with annular dilatation and stretching of chordae tendineae

• Valvular damage most frequently and most severely involves the mitral valve, less commonly the aortic, and rarely the tricuspid and pulmonary valves

• Aschoff bodies in the myocardium are characteristic of rheumatic fever

Clinical manifestations

• Arthritis (70%) – Usually large joints – Migratory in nature

• Carditis (50%)

• Erythema marginatum (< 10%)

• Sydenham's chorea (St. Vitus' dance): 15% of cases

– more often in prepubertal girls

• Subcutaneous nodules (2-10%)

Diagnosis

Jones criteria for guidance in the diagnosis of acute rheumatic fever were initially proposed by T. Duckett Jones, MD, in 1944 Committees of the American Heart Association subsequently modified, revised, and edited these criteria.

Exceptions to Jones Criteria

• Chorea may occur as the only manifestation of rheumatic fever

• Indolent carditis may be the only manifestation in patients who come to medical attention months after the onset of rheumatic fever

• Both of these types of patients may have insufficient supporting historical, clinical, or laboratory findings to fulfill the Jones criteria.

Clinical Course

• Only carditis can cause permanent damage. Signs of mild carditis disappear rapidly in weeks, but those of severe carditis may last for 2 to 6 months

• Arthritis subsides within a few days to several weeks even without treatment, and does not cause permanent damage

• Chorea gradually subsides in 6 to 7 months or longer usually does not cause permanent neurologic sequelae

Atypical Arthritis

• Jones criteria have emphasized that arthritis = migratory polyarthritis and extremely painful in classical presentation.

• However, many arthritic symptoms of acute rheumatic fever are atypical, and monoarthritis is frequently observed

• The Australian and New Zealand guidelines have widened the spectrum of arthritis as a major criterion for the diagnosis of acute rheumatic fever

Management

• Eradication of streptococcus • Anti-inflammatory or suppressive therapy with salicylates or

steroids must not be started until a definite diagnosis is made

• Bed rest • Anti-inflammatory agents

– For mild - moderate carditis, aspirin alone is recommended in a dose of 90 to 100 mg/kg per day continued for 4 to 8 weeks, depending on the clinical response. After improvement, the therapy is withdrawn gradually over 4 to 6 weeks

– For arthritis, aspirin therapy is continued for 2 weeks and gradually withdrawn over the following 2 to 3 weeks

– Prednisone (2 mg/kg per day in four divided doses for 2 to 6 weeks) is indicated only in cases of severe carditis

• Treatment of CHF

Prevention

• Primary prevention

• Secondary prevention

• Tertiary prevention

Primary Prevention

• A 10-day course of penicillin therapy for streptococcal pharyngitis.

• However is not possible in all patients because: – 30% of the patients develop subclinical pharyngitis

and therefore do not seek medical treatment. – 30% of patients develop acute rheumatic fever

without symptoms of streptococcal pharyngitis.

Secondary Prevention

Duration of secondary prevention

Infective Endocarditis

High Risk Group• Majority of patients with congenital heart disease (CHD) • Post-operative hearts

– Cardiac surgery itself is an important risk factor for IE

– Highest risk in children who had repair or palliation of cyanotic CHD

– Incidence of IE in the first postoperative month is low for most defects and increases with time after surgery

– When prosthetic valves or conduits are used in surgical repairs, the risk for IE is high even in the immediate (first 2 weeks) postoperative period

– Corrective surgery for isolated VSD, secundum ASD or PDA with documentation of no residual leak, risk for IE is the same as for general population, 6 months after surgery

• Chronic rheumatic heart disease • Normal hearts with

– Central indwelling venous catheters

– Staphylococcus aureus bacteraemia

– Intravenous drug abuse

Organisms

• Most common organisms are gram-positive cocci - viridans group streptococci, staphylococci and enterococci

• Less commonly - HACEK group (Haemophilus parainfluenzae, H. aphrophilus, H. paraphrophilus, Actinobacillus actinomycetemcomitans, Cardiobacterium hominis, Eikenella species, and Kingella kingae)

• IE associated with indwelling central catheters, prosthetic valves or materials - Staphylococcus aureus, coagulase-negative staphylococci

• Newborn infants - Staphylococcus aureus, coagulase-negative staphylococci, candida species, group B streptococcus and streptococcus pneumoniae

Clinical Findings

Due to 4 underlying phenomena:

1. Bacteraemia (or fungaemia)

2. Valvulitis

3. Embolic phenomenon– major arterial emboli, septic pulmonary infarcts, mycotic

aneurysm, intracranial hemorrhage, conjunctival hemorrhages, splinter hemorrhages and Janeway lesions

4. Immunologic responses – glomerulonephritis, Osler nodes, Roth’s spots

Diagnosis

• High index of suspicion is important

• DUKE criteria

DUKE Criteria

DUKE Criteria

Diagnosis

Antimicrobial Therapy

• Prolonged course of intravenous therapy for at least 4 weeks duration or 3 weeks afebrile

• Consider 6 weeks antibiotics if: – Prosthetic valve IE – Highly virulent organisms – Relative antibiotic resistance of organism

• Dual or more antibiotics for synergistic effect

• Outpatient antibiotic therapy can be considered in uncomplicated cases on a case by-case basis, after the initial hospital treatment

Prevention of IE Guidelines (AHA 2007)

Myocardial Disease

Myocardial Disease

• Cardiomyopathy

• Viral Myocarditis

• Anthracycline Cardiomyopathy

Cardiomyopathy

Clinical features Hypertrophicc Dilated Restrictive

Cause Inherited, AD (50%), Sporadic (new mutation)

Toxic, metabolic, infection, alcohol, doxorubicin,etc

Myocardial fibrosis, hypertrophy, infiltration (amyloid, haemochromatosis)

Haemodynamic dysfunction

Diastolic dysfunction Systolic dysfunction Diastolic dysfunction

Echo Thickened LV, supernormal LV contractility

Biventricular dilatationReduced LVEF

Biatrial enlargement

Doppler Reduced relaxation patternLVOT obstruction

Reduced relaxation pattern

Restrictive pattern

Treatment Beta blockerCalcium antagonist

VasodilatorDigitalis + diureticsBeta blockerAnticoagulantsAnti arrhythmicCardiac transplantation

DiureticsAnticoagulantsCorticosteroidPPM for heart blockCardiac transplantation

Viral Myocarditis

• "Inflammation of the myocardium with myocellular necrosis"• Etiology:

– Viruses (most common): Enterovirus 71, Coxsackie B, Adenovirus, Parvovirus B19, CMV, Echovirus

– Others: Mycoplasma, typhoid fever, diphteria toxins• Clinical features:

– Sudden onset of illness with anorexia, vomiting, lethargy, and, occasionally, circulatory shock

– Signs of CHF include poor heart contractility, tachycardia, gallop rhythm, tachypnea, and rarely cyanosis.

– Arrhythmia- Heart Block / VT• Treatment: Supportive (ICU, mechanical ventilator, inotropic support,

ECMO)• Prognosis:

– 1/3 recover– 1/3 recovered with residual myocardial dysfunction– 1/3 died

Anthracycline Cardiomyopathy

• Dose-related cardiotoxicity (eg: Doxorubicin, Daunorubicin)

• 2% to 5% of patients who have received a cumulative dose of 400 to 500 mg/m2

• Up to 50% of patients who have received more than 1000 mg/m2

• Dilated LV, decreased contractility, elevated filling pressures of the LV, and reduced cardiac output

Thank You