Cardiomyopathy in neonates and children

Posted by e-Medical PPT
Some cardiomyopathies are treatable
Cardiomyopathy may presents as recurrent wheeze

Classification of Cardiomyopathy
Dilated
Chronic
Acute Viral myocarditis (Inflammatory Cardiomyopathy)
Hypertrophic
Restrictive

Dilated Cardiomyopathy
Insidious onset, may be acute in up to 25% of patients, exacerbated by a complicating LRTI
Cough, poor feeding, irritability, and shortness of breath are usually the initial presenting symptoms.
Pallor, sweating, easy fatigability, failure to gain weight, and decreased urine output may be present.
Wheezing may be an important clinical sign, suggesting congestive heart failure (CHF) manifestation in infants.
Chest pain, palpitations, orthopnea, hemoptysis, frothy sputum, sudden death, abdominal pain, syncope, and neurologic deficit are other modes of presentation (20%).
Cardiomegaly detected incidentally on a chest radiograph or an arrhythmia detected incidentally on an ECG may be the basis for initial cardiac referral.
Approximately 50% of patients with dilated cardiomyopathy (DCM) have a history of preceding viral illness. A detailed family history for familial cardiomyopathy is revealing in up to 25% of cases.

In established disease, features of CCF are dominant.
Major cardiac findings include cardiomegaly, quiet precordium, tachycardia, gallop rhythm (S3 and/or S4), accentuated P-2, and murmurs of mitral and tricuspid regurgitation. Murmurs may be inconspicuous initially when presenting in acute heart failure.
Infants often present with predominantly respiratory signs and, in the absence of a precordial heave or prominent murmur, the underlying cardiac disease may remain undiagnosed until cardiomegaly is detected on chest radiograph.

ECG
Presence of Q waves and inversion of T waves in leads I, II, aVL, and V4 through V6 (anterolateral infarction pattern): ALCAPA
Significant arrhythmia: Arrythmia causing DCM
Low Voltage complexes: Pericardial effusion

ECHO
Dilated left ventricle (>95th percentile) with global hypokinesia (fractional shortening<25%, ejection fraction <;50%), and no demonstrable structural heart disease: DCM Left ventricular posterior wall hypokinesia with hyper-echoic papillary muscles, retrograde continuous flow into proximal pulmonary artery: ALCAPA Significant pericardial effusion with satisfactory left ventricular ejection fraction: Pericardial effusion...

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