Acquired Heart Disease -pediatric Cardio A

INFECTIVE ENDOCARDITIS ACQUIRED HEART DISEASE Maria Dolores B. Victor, M.D. Department of Child Health FEU Hospital

• Acute and Subacute, non bacterial • Significant cause of morbidity and mortality among children and adolescents

EPIDEMIOLOGY

ETIOLOGY Streptococcus viridans

• Most often a complication of congenital or RHD

in pediatric patients

Staph. Endo: No Heart Disease

• Can occur in children without heart disease

S. viridans: After dental proc. Grp D. Enterococci: Lower bowel or G-U manipulation Pseudomonas: IV drug users Fungal organisms: After open heart surgery

• Rare in infancy

Prevalence: 0.5 – 1 per 1000 hospital admission • Pathogenesis: a) Two factors are important: 1. presence of structural abnormalities of the heart 2. bacteremia •

Staphylococcus aureus

leading causative agents

b. Predispose to endocarditis -all CHDs except ASD secundum -rheumatic heart disease -prosthetic heart valves -MVP with mitral regurgitation -HOCM -drug addicts

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CLINICAL MANIFESTATIONS  1. 2. 3. 4. 5. 6. 7. 8. 9.

Symptoms: Fever Chills Fatigue Loss of appetite Pallor Chest and abdominal pain Arthralgia Weight loss CNS manifestations – stroke, seizures, headache

CLINICAL MANIFESTATIONS  Signs: 5. Embolic phenomena a. b. c. d. e.

Pulmonary emboli Seizure Hemiparesis Hematuria Roth spot ( retinal hemorrhage )

6. Clubbing of fingers 7. Heart failure and arrhythmia

CLINICAL MANIFESTATIONS  1. 2. 3. 4.

Signs: Fever ( 38 to 39 C in 80 – 90 % ) New heart murmur in 100 % Splenomegaly Skin manifestations from emboli in 50 % a. b. c. d.

Petecchiae Osler’s nodes- tender nodes at ends of fingers Janeway lesions- hemorrhagic areas at palms and soles Splinter hemorrhages- linear lesions berneath the nails

LABORATORY FINDINGS • Blood culture 90% of case (+) in 1st two blood cultures • Culture other secretions • Echocardiography: -valvular vegetations -valve dysfunction -embolic complications

DIAGNOSIS • High index of suspicion • Blood culture

PROGNOSIS • Fatal in the pre-antibiotic era • 20-25% mortality • 50-60%: serious morbidity 2º HF • Pulmonary and systemic embolism

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TREATMENT

PREVENTION

1.Treatment is started with penicilin or Oxacillin plus Gentamycin 2.Final selection of antibiotics is based on the organism isolated and antibiotic sensitivity 3.Duration of treatment: 4-6 weeks 4.Operative intervention

• Antimicrobial prophylaxis prior to various procedures (dental, respiratory, G-1

KAWASAKI DISEASE

Prophylactic regimens

A.For dental,oral,respiratory tract or esophageal procedures

• Acute febrile condition primarily affecting

Situation

Agent

Regimen

Standard general prophylaxis

Amoxicillin

C:50mg/kg PO 1hr before procedure A:2 g PO 1hr before procedure

Unable to take oral medications

Ampicillin

and g-U tracts)

C:50mg/kg IM or IV within 30 min before procedure A:2 g IM or IV within 30 min before procedure

children • Association with vasculitis of coronary blood vessels • Constellation of other systemic complaints

ETIOLOGY

EPIDEMIOLOGY • 1st reported in Japanese children

• UNKNOWN

• Leading cause of acquired heart disease in

• Bacterial toxins similar to staphylococcus toxins of TSS

US and recognized worldwide • Peak age incidence : 1 – 2 years

- 50 % of cases occur before the age of 2 years - ~80 % occur before the age of 5 years - seldom beyond the age of 8 years

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Fever

CHARACTERISTIC CLINICAL SIGNS A.

Fever for at least 5 days PLUS

B. Presence of four of the following: 1. Bilateral nonpurulent conjunctival injection 2. Changes in the mucosa of oropharynx: infected pharynx, dry fissured lips, strawberry tongue 3. Changes of the peripheral extremities -edema/ erythema of hands on feet -desquamation 4. Rash-truncal, polymorphus, nonvesicular 5. Cervical lympahdenopathy

Conjunctival injection • Discrete vascular injection of bulbar conjunctivae • Ocular discharge and corneal ulceration do not occur

• More than 5 days • Generally high, abrupt, and sustained with peak temperatures exceeding 40 C • Persists for an average of 12 days • May not respond to antipyretics • Usually resolves in 1-2 days after treatment with intravenous gamma globulin

Changes in the mouth • Erythematous, fissuring, peeling, and bleeding of lips • Diffuse erythema of the oropharynx • Strawberry tongue

Changes in the peripheral extremities • In the acute phase- firm induration of hands and feet • Diffuse and deep erythema of hands and soles • Desquamation of fingertips and toenails on the second week • Transverse grooves across fingernails 2-3 months after onset ( Beau’s lines )

Erythematous rash • Usually truncal, polymorphous, nonvesicular and without crusts • Maybe morbilliform, maculopapular or scarlatiniform • May resemble erythema multiforme

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Enlarged lymph node • Usually unilateral and cervical • Occurs in more than half of the patients • The node usually measures more than 1.5 cms • Usually non-fluctuant, nontender or only slightly tender

CHARACTERISTICS CLINICAL SIGNS C. Illness not explained by other known disease process *Incomplete or Atypical Kawasaki: more common in patients <1 yr of age Cardiac involvement: 10-40%: Coronary Vasculitis/ aneurysms

Incomplete ( Atypical ) Kawasaki - patients who do not fulfill the criteria for Kawasaki disease

LABORATORY FINDINGS 1. Leukocytosis-

typical during acute stage ; 50 % of patients have WBC count > 15,000

- more common in young infants

2. Anemia 3. Elevated ESR and CRP – universal in

- should be considered in all children with unexplained fever for >5 days associated with 2 or 3 of the principal features of KD

4. Abnormal plasma lipids 5. Hypoalbuminemia 6. Hyponatremia

KD, returning to normal by 6 to 10 wks after onset of illness

LABORATORY

PROGNOSIS

7. Thrombocytosis-

a characteristic feature of the later phase of illness; platelet counts ranging from 500,000 to > 1 million ; rarely present in the 1st week of illness, usually appears in the 2nd week, and peaks in the 3rd week with gradual return to normal by 4 to 8 weeks after onset

• Complete recovery in those without coronary vasculitis • 1-2% die with cardiac complications:

*A low platelet count at illness presentation is a risk factor for coronary aneurysm

8. Sterile pyuria 9. Elevated serum transaminases

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HF, arrhythmia, MI

TREATMENT • IVIG during acute febrile disease : 2 gm/k single dose over 10-12 hr infusion given within 10 days from onset of fever

• Measles and varicella immunizations should be deferred for 11 months after a child receives high dose IVIG

• Salicylate : 80 – 100 mg/kg in 4 divided doses • Thrombolytic therapy

• Unoperated CHD

DISEASES OF THE

• Long-standing volume/ pressure load • Chronic hypoxia

MYOCARDIUM

• Others

Cardiomyopathy

CAUSES:

• Is a disease of the heart muscle itself, not associated with congenital, valvular or coronary heart disease 1.Primary or idiopathic 2.Secondary • The classification is based on the anatomic and functional features of the heart

• CARDIOMYOPATHY: Specific Gene Defects Prior Viral Myocarditis Prevalence in newborn period: 10 per 100,000 livebirths All children: 36/100,000 (dilated cardiomyopathy) 2/100,000 (hypertrophic & restrictive cardiomyopathy)

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1. Dilated Cardiomyopathy

1. Dilated Cardiomyopathy

• Pathology and pathophysiology: 1. Varying degrees of myocyte hypertrophy and fibrosis 2. Weakening of systolic contraction associated with dilatation of all four chambers. Although heart weight is increased, the ventricular walls are not thickened

• Clinical manifestations 1. Onset is insidious. History of fatigue, weakness and symptoms of left-sided heart failure. 2. History of prior viral infection occasionally is elicited. 3. Signs of CHF; tachycardia, crackles, weak peripheral pulses, distended neck veins, hepatomegaly. Apical impulse is displaced.

1. Dilated Cardiomyopathy

1. Dilated Cardiomyopathy

• Diagnosis 1. ECG: sinus tachycardia, combination of atrial enlargement, varying degrees of left and right ventricular hypertrophy, nonspecific T-wave abnormalities 2. CXR: cardiomegaly, with or without signs of pulmonary edema 3. Echo: dilatation of heart and poor contractility

• Management 1. Aggressive treatment of CHF – digoxin, diuretics, vasodilators 2. Critically ill children: intubation, IV inotropes 3. Antiarrhythmia 4. Cardiac transplant

1. Dilated Cardiomyopathy • Prognosis: progressive deterioration is the rule rather than the exception. About 2/3 of patients die from intractable heart failure within 4 years after the onset of symptoms of CHF

2. Hypertrophic Cardiomyopathy • •

Familial disorder of the heart muscle 30 – 60 % transmitted as autosomal dominant trait

• Pathology and pathophysiology: 1. Most characteristic abnormality is the hypertrophied LV, with ventricular cavity usually small or normal in size

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2. Hypertrophic Cardiomyopathy

2. Hypertrophic Cardiomyopathy

• Clinical manifestations 1. Many children are asymptomatic 2. Family history is positive for the disease in 30 – 60 % 3. Easy fatigability, dyspnea, palpitation, or anginal pain 4. Sudden death may occur with an incidence of 4 – 6 % a year 5. Arterial pulse is characteristically brisk, prominent LV lift 6. A soft systolic murmur of MR is often present

• Diagnosis 1. ECG : abnormal in majority of patients; LVH with or without ST depression, abnormally deep Q waves 2. CXR : mild cardiomegaly with prominence of LV 3. Echo : LVH predominantly affecting the interventricular septum, concentric hypertrophy of LV, left ventricular outflow obstruction

2. Hypertrophic Cardiomyopathy

3. Restrictive Cardiomyopathy

• 1. 2. 3.

Treatment No standard treatment Moderate restriction of physical activities Digitalis and aggressive diuresis is contraindicated 4. B-blocking agents ( Propranolol) and calcium channel blocking agents ( Verapamil ) have been use with success 5. Pacemakers 6. Surgery : ventricular septal myotomy

3. Restrictive Cardiomyopathy • Clinical manifestations 1. History of exercise intolerance, weakness, chest pain 2. In its full-blown form: dyspnea, edema, ascites, hepatomegaly, increase venous pressure , pulmonary congestion

• Extremely rare form of cardiomyopathy • Maybe idiopathic, or associated with systemic diseases, malignancies, or radiation therapy • Characterized by markedly dilated atria and generally normal ventricular dimensions. Diastolic filling is impaired, resulting from excessively stiff ventricular walls. • Contractile function of the heart is normal

3. Restrictive Cardiomyopathy • Diagnosis 1. ECG : prominent P waves, atrial fibrillation or supraventricular tachycardia 2. CXR : mild to moderate cardiomegaly, pulmonary congestion, effusion 3. Echo : diagnostic; markedly enlarged atria and small to normal sized ventricles. Systolic function is normal. Atrial thrombus maybe present

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3. Restrictive Cardiomyopathy • 1. 2. 3.

Treatment Supportive Diuretics to relieve congestive symptoms Calcium channel blockers to increase diastolic compliance 4. Antiarrhythmia/ pacemaker 5. Anticoagulation 6. Heart transplant •

CLASSIFICATION OF CARDIOMYOPATHIES ACCORDING TO STRUCTURAL AND FUNCTIONAL ABNORMALITIES

PATHOPHYSIOLOG Y

DILATED

HYPERTROPHIC

Massive cardiomegaly⇒ extensive dilatation of ventricle

Massive ventricular hypertrophy with principal involvement of ventricular septum, LV & RV⇒ ↑resistance to LV filling ⇒ LVOTO in 25%⇒ MR

Prognosis : poor

RESTRICTIVE

Poor ventricular compliance is the major abnormality⇒ simulate constrictive pericarditis

CLASSIFICATION OF CARDIOMYOPATHIES ACCORDING TO STRUCTURAL AND FUNCTIONAL ABNORMALITIES

CLASSIFICATION OF CARDIOMYOPATHIES ACCORDING TO STRUCTURAL AND FUNCTIONAL ABNORMALITIES

DILATED CLINICAL

DILATED

All age group affected with

ON

insidious onset



Many are

RESTRICTIVE 

Dyspnea, edema,

asymptomatic;

ascites,

50%- heart murmur

hepatomegaly,

irritability, anorexia,

or affected family

↑venous pressure,

autosomal dominant

abdominal pain,

member

pulmonary

pattern

cough, dyspnea on

HYPERTROPHIC

EPIDEMIOLOGY



MANIFESTATI

HYPERTROPHIC

RESTRICTIVE

Inherited in an



Greater tendency for RVOTO 

LV wall diffusely





congestion

Weakness, fatigue,

Heart: mildly

exertion⇒ heart

DOE, palpitations,

failure

angina pectoris,

enlarged

Holosystolic

dizziness, syncope

nonspecific

Brisk pulse, systolic

murmurs

murmurs of MR

thickened





and TR

murmur at left

Cardiomegaly

sternal edge and



apex

CLASSIFICATION OF CARDIOMYOPATHIES ACCORDING TO STRUCTURAL AND FUNCTIONAL ABNORMALITIES LABORATORY DIAGNOSIS EKG

DILATED 

Bi-atrial enlargement

HYPERTROPHIC 

LVH, nonspecific Twave abnormalities Chest Roentgenogram

Echocardiogram

Cardiac Catheterization





Cardiomegaly, pulmonary congestion, pleural effusion Dilated LA, LV, poor contractility, pulmonary hypertension



LVH with or without ST segment depression and Twave inversion

CLASSIFICATION OF CARDIOMYOPATHIES ACCORDING TO STRUCTURAL AND FUNCTIONAL ABNORMALITIES

RESTRICTIVE 

Mild cardiomegaly with LV prominence

Prominent P waves ST segment depression

DILATED PROGNOSIS

Progressively downhill course,

T wave inversion

survival beyond

Mild to moderate cardiomegaly

one year is poor

HYPERTROPHIC Unpredictable, esp. in asymptomatic patient Progress to chronic heart failure Risk for sudden





LVH affecting intravent, septum, SAM of AMVL, LVOTO

•

Biatrial enlargement, normal ventricular chambers

Assess patient’s candidacy for surgery

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death caused by arrhythmia

RESTRICTIVE Generally poor

Rheumatic Fever

CLASSIFICATION OF CARDIOMYOPATHIES ACCORDING TO STRUCTURAL AND FUNCTIONAL ABNORMALITIES DILATED TREATMENT









HEART FAILURE treatment Anti-arrhythmic drugs

HYPERTROPHIC 



Systemic anticoagulation Heart transplantation

NO STANDARDIZED THERAPY Discourage competitive sports and strenuous physical activity

RESTRICTIVE  



 







Beta blocker, calcium channel blockers

• •

Heart failure relief Calcium channel blockers Anti-arrhythmic drugs Anti-coagulation Cardiac transplantation

•

Attack rate is 3 % with PR of 1/1000 Secondary to Group A B-hemolytic streptococcus Pathology and manifestations: found in different organ systems

a. b. c. d.

Heart Brain Joints Skin

•

Others organs may be affected-no obvious manifestations

Pacemaker for arrhythmias Surgery

Rheumatic Fever •

• • -

Characteristics of Strep Throat

Valvular damage is one of the most serious complications of this disease, with a mitral valve predilection, followed by aortic valve, TV, PV Aschoff bodies are the characteristic findings on histopath Symptoms are seen after 3-5 weeks , preceding strep throat infection Average latent period: 3 weeks after infection Chorea has a longer latent period of 3-6 months

Signs and symptoms which are characteristics of RF maybe classified under Jones criteria

Clinical Characteristics Age

Strep throat

Non-strep throat

5 – 15 years

All ages

Onset Initial symptom

sudden gradual Sore throat with pain on Mild sore throat swallowing

Appearance of throat

Redness, hyperemia Edema. Exudates, enlargement of tonsils with exudates

Redness of pharnyx

Fever

High

Not so high

Other signs

Tender anterior cervical LN,scabby erosions on nostrils. Clinical picture of scarlet fever

Cough, hoarseness,watery nasal discharge, conjunctivitis

Jones Criteria • 1. 2. 3. 4. 5.

Major Carditis Polyarthritis Chorea Erythema marginatum Subcutaneous nodules

Jones Criteria

•

Minor

1. -

Clinical Fever Arthralgia

2. -

Laboratory Increase ESR/ CRP Prolong PR interval

Plus : supporting evidence of antecedent group A strep infection 1. Positive throat culture or rapid streptococcal antigen test 2. Elevated ASO titer

•

Two major criteria or 1 major plus 2 minor criteria - Plus evidence of a preceding strep infection indicate a high probability of RF, EXCEPT in 3 cases: 1. Chorea 2. Insidious or late-onset carditis 3. Rheumatic recurrence

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Arthritis

Carditis • Most serious , occurring in 40 – 50 % • Varies: organic heart murmur, heart enlargement, tacchycardia, heart failure • Indicated by apical systolic murmur, apical middiastolic murmur , basal diastolic murmur, friction rub , gallop rhythm • May present as chest pain, palpitations, dyspnea • Involves all layers of the heart

• Most common , 75 % ; least specific • affects mostly of large joints ( knees, ankles, elbows, wrist ) • Rarely involves small joints • Asymmetric and polymigratory • Disappears spontaneously w/o leaving joint deformity • Very good response with salicylates (aspirin)

Chorea

Subcutaneous Nodules

• St. Vitus’ dance • A triad of involuntary movements, muscular weakness, and emotional disturbance • More common in females • Characteristic involuntary movements usually begins in the face and affects the arms more than the legs • Disappears during sleep • Long latent period of 3 – 6 months

• Small, non-tender pea-sized nodules that appear over the extensor surface of the joints • Uncommonly seen, last only for 1 to 2 weeks • When present, is often in association with carditis

Management

Erythema Marginatum • Rare, seen in less than 10 % • Occurs only in patients with carditis • A macular, non-pruritic, pink rash with pale center • Affects the trunk and proximal limbs, do not involve the face • Application of heat may accentuate the appearance of lesions

• • •

Rest Anti inflammatory drugs Aspirin Prednisone Anti-decongestive- inotropes, diuretics, afterloaders • Primary prevention • Secondary prevention

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Primary prevention- treatment of streptococcal pharygitis to prevent RF

Advantages: 1. Allows eradication of disease 2. Duration of treatment is brief 3. Prevents severe and irreversible heart damage ( RHD ) from initial attack of RF

• • •

Secondary Prevention- administration of longacting penicillin for prolonged period of time to patients who have developed RF to prevent recurrence of attacks • Benzathine benzyl penicillin 1.2 M units deep IM every 21 to 28 days • Penicillin V 250 mg twice daily

Benzathine Penicillin G 1.2 M units deep IM single dose Penicillin V ( Phenoxymethyl Penicillin) 250 mg 3x daily for 10 days Erythromycin estolate 20 – 40 mg/kg/day 2 to 4 times daily for 10 days

• Erythromycin 250 mg daily

Duration of Secondary Prophylaxis

Anti-inflammatory Therapy In RF • Arthralgia or mild arthritis, no carditis – analgesics only

• RF without carditis -- 5 years or until age 21 years, whichever is longer

• Moderate or severe arthritis; no carditis, or carditis without heart failure -- Aspirin 90 – 100 mg/kg/day for 2 weeks, longer if necessary

• RF with carditis but no residual heart disease – 10 years or well into adulthood whichever is longer

• Carditis with failure; with or w/o joint manifestations Prednisone 40 – 60 mg/day, after 2 – 3 wks slow withdrawal to be completed in 3 more weeks - Aspirin to overlap for 4 – 6 weeks after discontinuation of Prednisone

• RF with carditis and residual heart disease – at least 10 years since last episode and at least 40 years old, sometimes lifelong prophylaxis

The End  “ Med school provides perhaps the best substantiation for Charles Darwin's Theory of Natural Selection. For here, we see in its cruelest form the survival of the fittest. Not the smartest, as one should expect. But the fittest to cope with inhuman pressures, the demands made not only on the brain but on the psyche... “

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