Cardiomyopathies

Cardiomyopathies in children

1. Dr.Azad A Haleem AL.Brefkani University Of Duhok Faculty of Medical Science School Of Medicine Pediatrics Department [email protected] 2015 Cardiomyopathies in children

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.

6. 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 3 rd or 4 th heart sound are common Mitral/ tricuspid regurgitation may occur Diagnostic testing: CXR : cardiomegally . 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

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

8. Hypertrophic CM

9. 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.

10. 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.

11. 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

12. Diagnostic testing: CXR : cardiomegally . 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

13. 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.

14. 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.

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

16. Treatment Symptomatic Usually prognosis is poor Transplantation is the best treatment.

17. 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