CPC. 林世朋 李春銘 大夫 三 軍 總 醫 院 小 兒 部. Chief complaints. Heart murmur and moderate mitral regurgitation when he was at age one month Cough with sputum, runny nose, tachypnea and dyspnea when he was 7 months old. Present illness (1).
林世朋 李春銘 大夫
三 軍 總 醫 院 小 兒 部
1.Autosomal recessive(17q23), deficiency of lysosomal acid maltase
2.Infantile form : severe generalized myopathy and cardiomyopathy---cardiomegaly, hepatomegaly,diffusely hypotonic
---serum CK level is greatly elevated---muscle biopsy reveals a vacuolar myopathy
---EKG : prominent P waves; a short P-R interval; massive QRS voltage; signs of isolated left or biventricular hypertrophy; and interventricular conduction delay
---CxR : striking cardiomegaly with prominence of LV
---Echocardiogram : severe ventricular hypertrophy
3.Late childhood or adult form :---a much milder myopathy without cardiac or hepatic enlargement---myopathic weakness and hypotonia even in early infancy--- serum CK level is greatly elevated
4.Diagnostic---quantitative assay of acid maltase activity in muscle or liver biopsy
1.most common causative agents---coxsackievirus B and adenovirus
2.S/S depend on the patient’s age and the acute or chronic nature of the infection.
3.Neonate---fever, severe heart failure, respiratory distress, cyanosis, distant heart sound, a gallop rhythm, acidosis and shock.
4.evidence of viral hepatitis, aseptic meningitis and an associated rash may be present.
5.CxR---enlarged heart and pulmonary edemaEKG---sinus tachycardia, reduced QRS complex voltage, and ST segment and T-wave abnormality.
6.Older patient---a gradual onset of congestive heart failure or a sudden onset of ventricular arrhythmia.
7.ESR, CK & LDH may be elevated in acute or chronic myocarditis.
8.Echocardiogram---poor ventricular function and often a pericardial effusion, MR and absence of coronary artery or other congenital heart lesions.
9.Can be confirmed by endomyocardial biopsy.
1.Evidence of heart failure becomes apparent within the 1st few months of life, and it is often precipitated by respiratory infection.
2.Cardiac enlargement is moderate to massive.
3.Gallop rhythm is common (mitral insufficiency)
1.CxR : cardiomegaly
2.EKG : lead I & AVL---QR pattern followed by inverted T waves
V5 &V6---deep Q waves and exhibit elevated ST segments and inverted T waves.
3.Cardiac catheterization ---diagnostic
4.Aortography shows immediate opacification of the right coronary artery only.
Final diagnosis :
1.Anomalous origin of the left coronary
artery from the pulmonary artery
2. Myocarditis 3. Congenital mitral stenosis