An infant presents to OPD with signs of heart failure. On examination, there is hypotonia; hepatomegaly, cardiomegaly. ECG shows tall QRS complex and short PR interval. Which enzyme is deficient in this condition?
Glucose 6 - phosphatase
Muscle phosphorylase
Liver phosphorylase
Acid maltase
Hypotonia, hepatomegaly, cardiomegalyare seen in Pompe disease.
Acid maltase or Alpha 1,4. Glucosidase is deficient in Pompe disease.