Pompe's disease is a type II glycogen storage disorder resulting from deficiency of α-1,4 glucosidase. It is usually associated with dilated or hypertrophic cardiomyopathy. Association of apical hypertrophic cardiomyopathy is rarely seen. We present a case of a ten-month-old baby with clinical features of both apical hypertrophic cardiomyopathy and Pompe's disease.
Coarctoplasty with stenting is often an effective strategy in cases of recoarctation following surgical repair. The potential benefit of coarctoplasty in a patient with Eisenmenger's syndrome is unknown. We describe the case of a 21-year-old male who presented with claudication of lower limbs. He was known to have congenital heart disease, consisting of ventricular septal defect, patent ductus arteriosus (PDA), and coarctation of the aorta. Coarctation repair and PDA ligation had been done at two months of age. At the time of presentation for evaluation of claudication, echocardiography revealed severe coarctation and evidence of Eisenmenger's syndrome. This patient subsequently underwent balloon angioplasty and stenting of coarctation without any increase in cyanosis.
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