Transcript Cardiomyopathies

Nonrheumatic carditis in children.
Cardiomyopathy.
Prof. Pavlyshyn H.A.
Clinical manifestations
• Manifestations of myocarditis range from asymptomatic or nonspecific
illness to acute cardiogenic shock and sudden death.
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).
ECG – tachycardia, reduced QRS complex voltage,
ST-segment and T-wave abnormalities
Paroxismal
tachycardia
Premature ventricular
beats
Endocardial fibroelastosis – EFE
fetal endocarditis, endocardial fibrosis,
prenatal fibroelastosis
In primary EFE
– the left ventricular
chamber is dilated
In secondary EFE
- the ventricular cavity
is often contracted
In secondary EFE, severe congenital
heart disease of the left-side obstructive
type (AS, hypoplastic left heart syndrom,
severe CoA) is present.
Endocardial fibroelastosis – EFE
fetal endocarditis, endocardial fibrosis,
prenatal fibroelastosis
Clinical manifestations
• 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.
Endocardial fibroelastosis – EFE
Roentgenograms confirm
significant cardiac enlargement;
Note the enlargement of the heart,
without a distinctive contour and
clear lung fields
Endocardial fibroelastosis
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
Occurs through 3-4 mos after acute carditis or is diagnosed occasionally
• 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
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 THERAPY
Medicines 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
Cardiomyopathy
is 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
Idiopathic
Dilated
Cardiomyopathy
is characterized by
varying degrees of
dilatation of ventricles,
most prominently the
left.
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.
Dilated
Cardiomyopathy
• 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.
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 Cardiomyopathy
idiopathic 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)
Diagnosis
ECG
• 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
Echo-CG: LV hypertrophy – asymmetric, concentric, apical; systolic anterior motion of
the anterior leaflet of mitral valve
Doppler allow evaluation of mitral valve regurgitation
sept
Patient with
severe
asymmetric LV
hypertrophy;
Sept – septum
LVPW – left
ventricular posterior
wall
LV
LVPW
sept
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