SEKILAS INFO

CARDIOMYOPATHY
Definition: disease/abnormalities of the muscle fibers
• Primary the muscle cells are abnormal (usually due to a
gene mutation).
• Secondary healthy heart muscle cells that are adversely
affected by other conditions (low blood flow to the heart, low
blood oxygen, high blood pressure and infections)
Cardiomyopathies;
• DILATED CARDIOMYOPATHY
• HYPERTROPHIC CARDIOMYOPATHY
• RESTRICTIVE CARDIOMYOPATHY
DILATED CARDIOMYOPATHY

• The heart is enlarged (dilated) and the pumping chambers contract poorly
(usually left side worse than right).
• The most common form of cardiomyopathy  55–60% of all childhood
cardiomyopathies.
• Idiopathic, viral infection, alcohol, nutritional marasmus
• Can be familial (genetic)
SIGNS AND SYMPTOMS
• Along a spectrum of no symptoms, subtle symptoms or, in the more
severe cases, congestive heart failure (CHF)
• Infants and young children  viral upper respiratory tract
infection/recurrent “pneumonia”
• Older children and adolescents  decreased exercise capacity or easy
fatigability.
• Symptoms due to heart rhythm problems  palpitations, syncope, seizure,
sudden cardiac arrest
 TREATMENT Heart failure:
• No therapies that can “cure” Diuretics
Inotropic Agents
• Treatments  Afterload Reducing Agents
Beta-blockers
improve symptoms and decrease risk in
Pacemakers
children depends on the clinical condition Implantable
Cardioverter
Defibrillator
Surgical
Heart Transplantation
HYPERTROPHIC CARDIOMYOPATHY

• Definition abnormal growth of muscle fibers in the heart.

• The thick heart muscle is stiff  difficult for the heart to relax
and for blood to fill the heart chambers.
• The second most common form of heart muscle disease 35–
40% of cardiomyopathies in children
• The Pediatric Cardiomyopathy Registry  5/1.000.000 children
• Most often diagnosed during infancy or adolescence
• Usually genetically transmitted
 SIGNS AND SYMPTOMS

• Symptoms in children: dyspnea, angina, presyncope, syncope, exercise

intolerance or palpitations/arrhythmias
• Symptoms in infants more difficult to detect (difficulty breathing,
poor growth, excessive sweating or crying and agitation during feeding.
• Severe  symptoms of heart failure
TREATMENT

• No therapies that can “cure”

• Depends on the clinical condition
• Medications  beta-blocking and calcium channel-blocking
• Implantable Cardioverter Defibrillator
• Surgery
• Heart Transplantation
 RESTRICTIVE CARDIOMYOPATHY

• Characterized by restrictive filling of the ventricles  the

contractile function and wall thicknesses usually normal, but the
relaxation or filling phase of the heart is very abnormal.
• Idiopathic genetic cause is suspected in most cases.
SIGNS AND SYMPTOMS

• The most common symptoms lung related (repeated lung

infections” or “asthma.”
• chest x-ray heart enlargement
• Examination  ascites, hepatomegaly and edema with
additional cardiac signs or symptoms.
CAUSES

• Scientific evidence suggesting individual genetic “mutations”

TREATMENT

• No therapies that can “cure

• Medical Therapies: Diuretics, Beta-blockers,
• Heart transplantation is the only effective surgery
THANK YOU