Cardiomyopathies in children

1. Dr.Azad A Haleem AL.Brefkani
University Of Duhok
Faculty of Medical Science
School Of Medicine
Pediatrics Department
azad82d@gmail.com
2015

2. We are going to cover…
• 1. dilated cardiomyopathy (DCM)
• 2. hypertrophic cardiomyopathy (HCM)
• 3. restrictive cardiomyopathy (RCM)

3. ETIOLOGY
• A cardiomyopathy is an intrinsic disease of the
heart muscle and is not associated with other
forms of heart disease (are not the result of
congenital, acquired valvular, hypertensive,
coronary arterial and pericardial abnormalities)
• There are three types of cardiomyopathy based
on anatomic and functional features:
• (1) dilated,
• (2) hypertrophic,
• (3) restrictive .

4. Dilated cardiomyopathy
• are the most common form of cardiomyopathies.
• Is a disease of heart muscle characterised by
– dilation of the cardiac chambers
– Reduction in ventricular contractile function
• Etiology:
• They are often idiopathic,
• but may be due to infection (echovirus or coxsackie B virus)
or be
• post-infectious,
• familial,
• secondary to systemic disease, or
• secondary to cardiotoxic drugs.

5. Pathophysiology
• May be secondary to progression of any
process that affects myocardium
• Associated with atrial and ventricular
arrhythmia (probably responsible for the high
incidence of sudden death in the population)
• Results in Tricuspid and Mitral regurgitation
due to the effect of chamber dilatation on the
valvular apparatus.

7. Diagnosis
 Clinical presentation
– Symptomatic HF (syncope, dyspnea, volume overload)
– Some patients are asymptomatic
 Physical exam
– Variable degrees of cardiac enlargement
– Pulse pressure is narrow
– JVP raised
– 3rd or 4th heart sound are common
– Mitral/ tricuspid regurgitation may occur
 Diagnostic testing:
o CXR: cardiomegally.
o ECG shows nonspecific changes
• Sinus tachy/atrial fibrillation
• Ventricular arrhythmias
• atrial abnormality.
 Echocardiography :
• Confirm with echocardiography.
• 2D and Doppler Echo is helpful to differentiate from other CM

8. Treatment
• Therapeutic strategy include preload and
afterload reduction therapy.
• B-blocker should be used unless contra
indicated.
• Cardiac transplantation is the treatment of
choice.

9. Hypertrophic CM

10. Hypertrophic CM
• Hypertrophic cardiomyopathies are usually
familial with autosomal dominant inheritance,
but may occur sporadically.
• Most common cause of sudden death in young
athletes.
• Characterized by in appropriate and elaborate LV
hypertrophy with misalignment of the myocardial
fibres.
• Hypertrophy may be generalized or confined
largely to interventricular septum.

11. • Heart failure may develop because stiff non-
compliant ventricles impede systolic filling.
• Septal hypertrophy may cause dynamic LV
outflow obstruction.
• Mitral regurgitation occur due to abnormal
systolic anterior mitral valve leaflet.

13. Symptoms & Signs
• Hypertrophic cardiomyopathy is initially difficult to diagnose.
• Infants, but not older children, frequently present with signs of CHF.
• Older children may be asymptomatic, with sudden death as the initial
presentation.
• Symptoms
– Dyspnea, fatigue, chest pain, syncope or near-syncope, and palpitations may
be present.
– Dyspnea on effort
– Syncope on effort
• Signs
– Jerky pulse
– Palpable LV heave
– Double impulse at apex
– mid-systolic murmur –sign of LV outflow obstruction
– Pansystolic murmur

14. Diagnostic testing:
o CXR: cardiomegally.
o ECG : The ECG in infants and children with
hypertrophic cardiomyopathy is universally
abnormal, but changes are nonspecific. Primary
hypertrophic cardiomyopathy is associated with a
prolonged Q-T interval.
Echocardiography :
• Confirm with echocardiography.
• 2D and Doppler Echo is helpful to differentiate
from other CM

15. Treatment
• B-blocker and rate-limiting calcium antagonists
• No pharmacological treatment is known to
improve prognosis
• Arrhythmia is common and responds well to
amiodarone.
• Outflow tract obstruction can be improved by
surgery.
• Implantable cardiac defibrillation (ICD) for
patient with risk of sudden death.

16. Restrictive CM
• Restrictive cardiomyopathies are Rare condition.
• They may be idiopathic, but can be associated
with systemic disease.
• Ventricular filling is impaired because ventricles
are stiff.
• Lead to high atrial pressure with atrial
hypertrophy, dilatation and later atrial fibrillation.
• Presenting symptoms usually include dyspnea
exacerbated by a respiratory illness, syncope and
CHF.

18. Investigation
• Diagnosis is difficult
• Require complex Doppler echocardiography
• CT and MRI and endomyocardial biopsy may
be done.

19. Treatment
• Symptomatic
• Usually prognosis is poor
• Transplantation is the best treatment.

20. Anatomic and Functional Features of Cardiomyopathies
Dilated Hypertrophic Restrictive
Etiology Infectious Sporadic Infiltrative
Metabolic Inherited (autosomal
dominant)
Myocardial
hypertrophy
Toxic Myocardial fibrosis
Idiopathic Idiopathic
Hemodynamics Decreased systolic
function
Diastolic dysfunction
(impaired ventricular
filling)
Diastolic dysfunction
(impaired ventricular
filling)
Treatment Positive inotropes β-Blockers Diuretics
Diuretics Calcium channel
blockres
Anticoagulants
Afterload reduction Corticosteroids
β-Blockers Cardiac
transplantation
Antiarrhythmics
Anticoagulants
Cardiac
transplantation