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Hypertrophic cardiomyopathy (HCM) is defined by the presence of increased left ventricular (LV) wall thickness that is not explained by abnormal loading conditions without a priori assumptions about etiology or myocardial pathology even in neonate, children and adults. The diagnosis of HCM is mainly the detection of increased LV wall thickness by any imaging modality. Due to its diverse etiology, the disease phenotype includes chest pain, palpitation, syncope and heart failure. In most patients, there is a life-long process of progressive and adverse cardiac remodeling, characterized by myocardial fibrosis and wall thinning. However, there was no report about cardiac remodeling of progressive wall thinning in children with HCMP. A 5 year-old boy was evaluated due to familiar HCMP, and finally confirmed with HCMP by echocardiography. His father was diagnosed as asymmetric HCMP and treated with septal alcohol ablation 3 years ago. His family referred for genetic counselling. His symptom was vague because he was very young. Initial electrocardiographic finding was normal sinus rhythm with left ventricular hypertrophy. Ambulatory electrocardiography (ECG) showed isolated premature ventricular contraction. Initial echocardiographic findings revealed asymmetric septal hypertrophy with diffuse hypertrophied LV myocardium, systolic anterior motion of anterior leaflet of mitral valve without LV outflow tract obstruction (LVOTO) and only mild dynamic obstruction on mid-septum of LV. His cardiovascular magnetic resonance imaging (CMR) showed similar to echocardiographic findings and no late gadolinium enhancement (LGE). He started to take medication with beta blocker, and later addon verapamil. After 3 years later, his ambulatory ECG showed non-sustained ventricular tachycardia of 4 episodes. Follow-up echocardiographic findings revealed progressive septal wall thickness from 13mm to 19 mm, marked LVOTO with peak pressure gradient of 128 mmHg and profound dynamic obstruction on mid-septum of LV. Follow-up CMR imaging showed additional progressive apical wall thinning without suspicious or definite LGE on any other myocardium. Urgently he undertook the implantation of implantable cardioverter defibrillatorsfor prevention of sudden cardiac death. Now he is listing and waiting for heart transplantation. Cardiac remodeling is progressively life-long process in most HCMP, but in this case with pediatric HCMP, even though he took medical treatment its progression was rapid cardiac remodeling.

114 A case of progressive wall thinning of apical myocardium in 5 year- old boy with hypertrophic cardiomyopathy