Nonrheumatic carditis in children.Cardiomyopathy.

Prof. Pavlyshyn H.A.

• Manifestations of myocarditis range from asymptomatic or nonspecific illness to acute cardiogenic shock and sudden death.

Clinical manifestations

• Infants and young children more often have a fulminant presentation with fever, respiratory distress, tachycardia, hypotension, gallop rhythm and cardiac murmur. Associated findings may include a rash or signs of hepatitis, aseptic meningitis.

• Patients with acute or chronic myocarditis may present with chest discomfort, fever, palpitations, easy fatigability, or syncope. Hepatic enlargement, peripheral edema, and pulmonary findings such as wheezes or rales may be present in patients with decompensated congestive heart failure.

Clinical manifestations

• Arrhythmias may be the first clinical manifestation;• Cyanosis, skin discolouration in blue or grey tones; • Swelling in the face, feet or legs; poor circulation, showing as cold hands and feet;• .

Severe heart failure - distant heart sounds, weak pulses, tachycardia out of proportion to the fever, mitral insufficiency caused by dilatation of the valve annulus, gallop rhythm, respiratory distress, acidosis, shock.In the most fulminate form, death may occur within 1-7 days of the onset of symptoms

DIAGNOSIS• ESR, heart enzymes (creatine

phosphokinase, lactate dehydrogenase), BNP (brain natriuretic peptide), cardiac troponin may be elevated;

• PCR of serum samples and endomyocardial biopsy have shown viral genome and identifying inflammatory cell infiltrates or myocyte damage;

• X-ray chest – enlarged heart, pulmonary

edema;• Echo-CG – poor ventricular function,

often pericardial effusion, mitral valve regurgitation.

• Cardiac catheterization and endomyocardial biopsy – can detect other causes of cardiomyopathy (mitochondrial defects, storage disease).

Paroxismal tachycardia

Premature ventricular beats

ECG – tachycardia, reduced QRS complex voltage, ST-segment and T-wave abnormalities

Endocardial fibroelastosis – EFEfetal endocarditis, endocardial fibrosis,

prenatal fibroelastosis

In primary EFE – the left ventricular chamber is dilated

In secondary EFE - the ventricular cavity

is often contractedIn secondary EFE, severe congenital heart disease of the left-side obstructive type (AS, hypoplastic left heart syndrom, severe CoA) is present.

Endocardial fibroelastosis – EFEfetal endocarditis, endocardial fibrosis,

prenatal fibroelastosis

• Dyspnea, cough, anorexia, hepatomegaly, edema, failure to thrive, recurrent pulmonary infections.

• X-ray chest – cardiac enlargement• ECG – signs of LA, LV hypertrophy

with strain• Echo-CG – dilated, poorly functioning

LV. • MRI – may delineate the fibrotic

endomyocardial surface.

• Infants in whom valvular lesions or associated congenital cardiovascular defects are predominant die in the 1st mo of life.

Clinical manifestations

Endocardial fibroelastosis

Roentgenograms confirm significant cardiac enlargement;

Note the enlargement of the heart, without a distinctive contour and clear lung fields

Endocardial fibroelastosis – EFE

ACUTE CARDITIS• Pale skin, weakness, headache, decline

of appetite, fever, shortness of breath;• for elder children - cardialgia,

heartbeating;• Chest pain due to coexisting

pericarditis

• Enlarged heart, tachycardia, weakness of heart tones, soft systolic murmur on the apex, decreased arterial pressure

• ECG: decrease of waves voltage, conduction impairments, arrhythmias

• Echo-CG: myocardium is diffuse thick, dilatation of chambers, reduced myocardial contractility, possible presence of liquid in pericardium

• Increased levels of LDG1, LDG2, CPK (creatine phosphokinase), BNP (brain natriuretic peptide)

SUBACUTE CARDITIS

• Unpleasant feelings, interruptions of the heart, palpitation, periodic cardialgias

• Moderate cardiomegaly mainly due to the LV

• Weakness of heart tones, arrhythmias, functional systolic murmur, moderate signs of HF (heart failure)

• ECG: arrhythmias, conduction impairments, signs of hypoxia of myocardium

• Echo-CG: signs of CF, hypokinesia of the left ventricle

Occurs through 3-4 mos after acute carditis or is diagnosed occasionally

CHRONIC CARDITIS

• More frequently is diagnosed as primary-chronic with the signs of chronic left heart failure;

• Possibly occurs after acute or subacute carditis lasting more than 12 – 18 months;

• Signs of HF, cardiomegaly, tachy- bradycardia, weakeness of cardiac tones, cardiac hump;

• Physical retardation, encephalopathy, anaemia; • ECG: arrhythmias, hypertrophy of the LV • Echo-CG: dilatation of the LV,- decreased retractive function ; - decreased of ejection fraction ;- reduce myocardial contractility.

Congestive Heart Failure

• Decreasing of cardiac output; • Systolic dysfunction – decreased myocardial

contractility;• Diastolic dysfunction – insufficient expansion

for ventricular volume; • Problems are accentuated by increased

metabolic and oxygen demand, developed severe heart failure

TREATMENT OF CARDITIS • REGIME: duration of the bed rest is determined according

to the degree of cardiomegaly and cardiac insufficiency, on the average 2 – 6 weeks with gradual expansion;

DIET: Table №10, uses of salt and liquid according to the degree of cardiac failure: excluding piquant products, replacing them on products with potassium and vitamines;

TREATMENT OF CARDITIS

Acute carditis:• Antiviral and antibacterial therapy - duration of the antibacterial therapy should be not less than 3-4 weeks (Penicillin - for elimination the chronic infectious foci present in most patients with carditis).

• glucocorticoids (prednisolon 0,5-1,5 mg/kg) 2-4 weeks with gradual decreasing• NSAID - nonsteroid anti-inflammatory drugs (Aspirin 100 mg/kg,

Ibuprophen 10-15mg/kg, Voltaren 2-3 mg/kg, Indometacin 2-3mg/kg Misulid 5-10

mg/kg, Mephenamin acid 50 mg/kg, Amizon 50 mg/kg) - 4 weeks with gradual decreasing of dose during 2-3 weeks;

End-stage EFE with signs of HF

despite maximal medical treatment, is an indication for heart transplantation

TREATMENT OF CARDITIS

•Subacute and chronic:

Chinoline derivatives (Delagil 5 mg/kg, Planquenil 8 mg/kg) 4-6 mos, decrease to 1/2 dose, give by ys

NSAID, Antibiotic therapy is used only at bacterial carditis with high activity

TREATMENT OF CARDITIS

SYMPTOMATIC THERAPYMedicines for improvement the function of myocardium:• Digitalis (Strophanthin 0,012 mg/kg, Digoxin 0,03 - 0,05 mg/kg) at cardiac insufficiency•Diuretics (Lasix 1- 3 mg/kg, Spironolactone-Veroshpiron 1-3 mg/kg, Hypothiazid 2-5 mg/kg);•ACE inhibitors - peripheral vasodilatations - Captopryl 0,5-1 mg/kg,•Anticoagulants (Heparin of 100 U/kg), antiaggregants - Curantil 2,5-3mg/kg,• B2-blocking agents (Metoprolol, Obzidan 1.0 – 2.0 mg/kg)•Phosphaden, Panangin, Riboxin, Mildronat, Cardonat 1 - 1,5 month

Cardiomyopathyis based on the predominant structural and

functional abnormalities

• Dilated Cardiomyopathy –

primarily systolic dysfunction

• Hypertrophic Cardiomopathy – primarily diastolic dysfunction

• Restrictive Cardiomyopathy - primarily diastolic, but often combined systolic dysfunction

is characterized by varying degrees of

dilatation of ventricles, most prominently the

left.

Idiopathic Dilated

Cardiomyopathy

LV dilatation and systolic dysfunction pathology:

• increased heart size and weight,

• ventricular dilatation, normal wall thickness,

• heart dysfunction out of portion to fibrosis

Dilated Cardiomyopathy

• The cause - a genetic basis or are the sequelae of viral myocarditis.

• In 20-50 % of cases, the disease is recognized as familial.

• Autosomal dominant

inheritance is most commonly encountered and mutations in several cardiac structural or metabolic genes have been identified.

• Failure of the LV causes an increase in end-diastolic volume, which results in increase in LA, pulmonary venous and pulmonary capillary pressure.

Mitral valve regurgitation may result from papillary muscle dysfunction or severe dilatation of the valve annulus.

Dilated Cardiomyopathy

Clinical Manifestations• The skin is cool and pale;

• The arterial pulse is decreased, tachycardia, palpitation is present.

• Jugular venous pressure is increased, hepatomegaly, edema are common;

• The heart is enlarged, holosystolic murmurs of mitral and tricuspid insufficiency may be present;

• Blood pressure may be low, pulse pressure narrow;

• A gallop rhythm is audible.

Hypertrophic Cardiomyopathyidiopathic hypertrophic subaortic stenosis,

idiopathic obstructive cardiomyopathy

The septum is thickened out of proportion to the left ventricular wall, which may also be thickened.

Hypertrophic Cardiomyopathy

• Hypertrophy of ventricular septum (95%)

• Disarray of myofibrils (100%)

• Volume reduction of ventricles (90%)

• Endocardial thickening of LV (75%)

• Mitral valve leaflet thickening (75%)

• Dilated atria (100%)

• Abnormal intramural coronaries (50%)

Clinical Manifestation• Asymptomatic, Echo-CG finding• Symptomatic

– Weakness, fatigue, dyspnea on effort (because of an inability to significantly increase cardiac output with exercise) in 90%

– Palpitations, chest pain, angina pectoris (due to myocardial ischemia) in 75%

– Dizziness, pre-syncope, syncope

risk of SCD in children and adolescents

Clinical Manifestation• The pulse can be brisk – early systolic ejection of blood

from ventricle;• A prominent LV lift, double apical impulse (peaked)

because ejection is interrupted by septal obstruction;• The 1st and 2nd heart sounds – normal;• Systolic ejection click • The systolic murmur is ejection in type and of medium

intensity – is heard maximally at the LSB and apex;• Murmur may increase shortly after exercise is

discontinued (Valsalva’s maneuver)

DiagnosisECG• LV hypertrophy, ST-segment depression and T-wave inversion.• Signs of the WPW (Wolff-Parkinson-White)-syndrome, • Rhythm disturbances are defined by Holter monitoring.

X-ray chest • Mild cardiomegaly with prominence of the left ventricle;

Diagnosis

septsept

LV

LVPW

Echo-CG: LV hypertrophy – asymmetric, concentric, apical; systolic anterior motion of the anterior leaflet of mitral valve

Doppler allow evaluation of mitral valve regurgitation

Patient with severe asymmetric LV hypertrophy;

Sept – septumLVPW – left ventricular posterior wall

Management

• Competitive sports and strenuous physical activity should be prohibited because most sudden deaths occur during or immediately after vigorous physical exertion, especially in adolescents and young adults.

• Digitalis or aggressive diuresis is contraindicated in most patients because of the potential to increase LV outflow obstruction

• beta-adrenergic blocking agents (propanolol, atenolol)) and calcium channel blocking agents (verapamil, nifedipine) may be useful in diminishing ventricular outflow tract obstruction, modifying ventricular hypertrophy, and improving ventricular

DCM v.s. HCM

Restrictive Cardiomyopathies

• Hallmark: abnormal diastolic function

• Rigid ventricular wall with impaired ventricular filling

• Bear some functional resemblance to constrictive pericarditis