Pediatric Cardiomyopathy
Cardiomyopathy is a disease that weakens and eventually enlarges the heart muscle. Since the heart is weak, it is unable to pump enough blood or rather it makes it harder for the heart to pump blood and deliver it to the rest of the body.
Classifications
- Dilated – most common type of heart muscle disease in children
- Dilated Cardiomyopathy refers to a congestive cardiac failure that is secondary to dilatation and systolic dysfunction of the ventricles, in the absence of any congenital, valvular, or coronary artery disease or any systemic disease known to cause myocardial dysfunction. It is difficult to identify the cause for it is generally idiopathic in most cases.
- Hypertrophic – involves abnormal growth or thickening of the heart muscle, particularly affecting the muscle of the heart’s main pumping chamber
- Restrictive – the heart muscle becomes rigid and less elastic; this does not allow the heart to properly expand and be filled with blood in between heartbeats
Pathophysiology
- Injury to any part of the myocardial cell causes a number of cell deaths. If significant cell loss occurs, the myocardium will fail to generate enough contractile force to produce adequate and necessary cardiac output. This results in the activation of compensatory mechanisms such as renin-angiotensin-aldosterone system.
- Compensatory mechanisms help to maintain cardiac output in the initial phase. However, if not treated and intervened, myocardial damage progresses. Persistent and excessive activation of the compensatory mechanisms can be detrimental to cardiac function, leading to evident congestive heart failure.
Etiology
- Nutritional deficiency (carnitine or selenium)
- Viral infection (myocarditis) or HIV
- Collagen vascular disease (SLE)
- Postpartum
Signs and Symptoms
- Signs of CHF – tachycardia, tachypnea, dyspnea, crackles, hepatomegaly
- Decreased activity tolerance
- Easy fatigability, sweating
- Ventricular arrythmias
- Poor weight gain, poor feeding
- Chest pain
- Syncope
- Nausea, abdominal tenderness
Diagnostic Tests
- Auscultation: systolic regurgitant murmur, S2 is normal or narrowly split, prominent S3 gallop
- ECG: tachycardia, abnormal ST segments, arrythmias
- Chest X-ray: cardiomegaly, pulmonary congestion
- Echocardiogram: poor ventricular systolic function, dilated heart chambers, AV valve insufficiency
Medical Management
- General Measures:
- Identify and treat underlying cause.
- Maximize caloric intake: fortify formula, supplememtal OGT/NGT feedings
- Supplemental oxygen
- Activity restriction, especially in strenuous and competitive sports.
- Treatment:
- Diuretics: Furosemide, Spironolactone
- Inotropics: Digoxin
- Afterload reduction: Captopril, Enalapril
- Anticoagulants: Warfarin
- Antiarrythmics
- Use of cardioverter-defibrillator
- Cardiac transplant
Nursing Management
- Maximizing cardiac output.
- Providing maximal nutritional support.
- Family education and health maintenance.
- Monitor for hypotension, tachycardia, increasing tachypnea.
- Administer oxygen as needed.
- Monitor response to diuretics and intake and output.
- Restrict activity level
- Encourage small, frequent meals. Provide foods the child likes.
- Provide high calorie supplements (eg. milkshake)
- Promoting effective coping and control within the family.
- Explain the disease process, management, and prognosis to parents and older patients, including the warning signs and symptoms that need referral to the physician.
- Compliance to medications.
- Emphasize the importance of avoiding strenuous activities and sports.
- Teach family members and caregivers hoe to do infant and child CPR.
Byron Webb Romero, RN, MSN
Finished BSN at Lyceum of the Philippines University, and Master of Science in Nursing Major in Adult Health Nursing at the University of the East Ramon Magsaysay Memorial Medical Center. Currently working at Manila Doctors College of Nursing as a Team Leader for Level I and II, Lecturer for Professional Nursing Subjects, and also a Clinical Instructor.
