Cardiomyopathies Flashcards
Definition of dilated cardiomyopathy
is defined by
1) Increasing the size of the left ventricle provenby imaging method. (Echocardiography, MRI,
radioisotope ventriculography, CT) and
2) ventricular dysfunction in the absence of other causes of ventricular overload (valvular disease, hypertension).
It can coexist with impaired RV.
Arrhythmogenic Dysplasia of the
Right Ventricle
is a cardiomyopathy with typical
morphological findings - right ventricular free wall fatty-fibrous infiltrate.
Arrhythmogenic Dysplasia of the
Right Ventricle
Diagnostic criteria
◼ - ECG changes
(inverted-T repolarization abnormalities in precordials conduction disturbances, QRS prolongation)
◼ - histological modifications on endomyocardial biopsy or MRI,
◼ - History of ventricular arrhythmias (VT with LBBB aspect)
◼ -History sudden death
Arrhythmogenic Dysplasia of the
Right Ventricle
Epidemiology
The events occur in young, initially linked
with arrhythmia, following a period with
frequent episodes of clinically manifested
1) ventricular tachycardia with LBBB aspect with RV outflow tract origin and
2) the risk of sudden death.
Arrhythmogenic Dysplasia :
With the advent RV dilation reveals
phenomena of
1) right heart failure,
2) biventricular dilatation with subsequent congestive heart failure.
TREATMENT
Arrhythmogenic dysplasia
◼ Patients with arrhythmogenic right ventricular dysplasia requiring implantation of ICD
(intracardiac defibrillator) are at risk of
sudden death
◼ Patients with systolic dysfunction + cardiac insufficiency are benefiting from the treatment of conventional drugs (IEC, beta-blockers)
RESTRICTIVE CARDIOMYOPATY
- Rare
- characterized by restrictive
filling of the ventricles.
◼ This inability to relax and fill with blood to the ventricles lead to a “reserve” of blood in the atria, causing the lungs and body symptoms and signs of heart failure.
◼ It is rare - 2.5-5% of children diagnosed with cardiomyopathy
◼ The average age at diagnosis is 5-6 years.
RCM seems to affect girls
more often than boys.
◼ There is a family history of cardiomyopathy in about 30% of cases.
◼ In most cases, the disease is unknown (idiopathic), although a genetic
cause is suspected in most cases of pediatric RCM.
SIGNS AND SYMPTOMS
- Nonspecific onset, most frequently linked to lung damage.
- history of “repeated lung infections” or “asthma”.
◼ Clinical examination – HF signs: - hepatomegaly,
- ascites,
- peripheral edema;
- angina
◼ Systolic murmur, protodiastolic gallop
◼ In about 10% of cases, syncope - sudden death
Investigation findings:
Restrictive cardiomyopathy
◼ ECG: biatrial dilatation
◼ Echocardiography: marked dilatation of the atria, ventricles of normal dimensions.
◼ In advanced forms, the pressure in the pulmonary artery may be increased.
Cardiac Catheterization
◼ confirm diagnosis
◼ high diastolic pressures and varying degrees of increased pressure in the pulmonary artery in the absence of any structural heart disease.
◼ In rare cases, heart biopsy may be performed to search for possible causes of the disease (such as amyloidosis / sarcoidosis, which are common causes of RCM in adults, but rarely in children).
TREATMENT
◼ For those with HF:
- Diuretics
- β - Blockers
- afterload lowering drugs.
EXTREMELY RARE FORMS
◼ Mitochondrial cardiomyopathy
◼ LV Noncompaction
Mitochondrial cardiomyopathy
◼ early onset
◼ Extremely rare
◼ It can occur as a HCM and DCM
◼ Mechanism: mitochondrial abnormalities affecting cellular metabolism
◼ Frequently associated with other types of myopathies, neurologic, or the general
development disorder
◼ The cause is frequently genetic
LV NONCOMPACTION
◼ rare congenital cardiomyopathy that affects both children and adults
◼ also called spongiform cardiomyopathy
◼ a genetic cardiomyopathy
◼ Common symptoms: dyspneea, fatigue, edema, limited physical capacity and exercise intolerance
