Pediatric Cardiomyopathy Quiz

Pediatric Cardiomyopathy: Causes, Types, and Management

• Cardiomyopathy refers to myocardial abnormalities affecting cardiac muscle contraction, being relatively rare in children.
• Etiological factors include familial/genetic causes, infection, deficiency states, metabolic abnormalities, and collagen vascular diseases.
• Most pediatric cardiomyopathies are considered primary/idiopathic, with unknown causes and no associated systemic disease.
• Known causes of secondary cardiomyopathy include anthracycline toxicity, hemochromatosis, Duchenne muscular dystrophy, Kawasaki disease, collagen diseases, and thyroid dysfunction.
• Cardiomyopathies can be classified into dilated, hypertrophic, and restrictive types based on structural and functional abnormalities.
• Dilated cardiomyopathy is the most common type in children, characterized by ventricular dilation, decreased contractility, and symptoms of heart failure (HF).
• Hypertrophic cardiomyopathy involves increased heart muscle mass without cavity enlargement, often familial, and presenting with anginal chest pain, dysrhythmias, and syncope.
• Sudden death risk is high in children with hypertrophic cardiomyopathy and unexplained syncope.
• Restrictive cardiomyopathy, rare in children, causes ventricular filling restriction due to endocardial or myocardial disease, with symptoms similar to HF.
• Therapeutic management aims to correct underlying causes when possible and manage HF and dysrhythmias with digoxin, diuretics, afterload reduction agents, and other specific treatments.
• Treatment approaches differ for each type of cardiomyopathy, with the use of beta blockers or calcium channel blockers for hypertrophic cardiomyopathy and AICD placement for high-risk patients.
• Nursing care involves providing support, symptom management, and psychological preparation for potential heart transplantation.

Pediatric Cardiomyopathy: Causes, Types, and Management

• Cardiomyopathy refers to myocardium abnormalities affecting the heart's ability to contract, with familial/genetic causes, infections, deficiency states, and metabolic abnormalities being possible etiological factors.
• Most pediatric cardiomyopathies are primary/idiopathic, with anthracycline toxicity, hemochromatosis, Duchenne muscular dystrophy, Kawasaki disease, collagen diseases, and thyroid dysfunction being known causes of secondary cardiomyopathy.
• Cardiomyopathies are clinically categorized into dilated, hypertrophic, and restrictive types, each with distinct structural and functional abnormalities.
• Dilated cardiomyopathy is the most common type in children, characterized by ventricular dilation, decreased contractility, and symptoms of heart failure (HF).
• Hypertrophic cardiomyopathy involves an increase in heart muscle mass without cavity enlargement, often presenting with anginal chest pain, dysrhythmias, and syncope.
• Children with known hypertrophic cardiomyopathy and unexplained syncope have a 60% cumulative risk of sudden death within 5 years of the syncope.
• Restrictive cardiomyopathy, rare in children, involves ventricular filling restriction due to endocardial or myocardial disease, with symptoms similar to HF.
• Therapeutic management aims to correct underlying causes when feasible, with treatment directed at managing HF and dysrhythmias, using medications such as digoxin, diuretics, and afterload reduction agents.
• Beta blockers and calcium channel blockers may be used for hypertrophic cardiomyopathy, while implantable defibrillators and anticoagulants may be considered for high-risk patients.
• Heart transplantation may be an option for patients with worsening symptoms despite maximum medical therapy.
• Nursing care for children with cardiomyopathy should address the poor prognosis, restricted activity, psychological preparation for transplantation, and informed consent for older children.
• Nurses play a crucial role in assessing the family's understanding of procedures and their long-term consequences, and children should be included in decisions regarding their activity and allowed to discuss their feelings.