278 A CASE OF ACCIDENTAL DIAGNOSIS OF ALCAPA SYNDROME IN ASYMPTOMATIC CHILD

Abstract A 7-year-old (y.o.) boy was referred to our center for a specialist evaluation due alteration of the ventricular repolarization in anterior and lateral lead on electrocardiogram at medical physical activity. The child totally asymptomatic, with normal growth unremarkable past history. examination revealed only S4 minimal apical systolic murmur. Transthoracic echocardiogram demonstrated mildly dilated left ventricle (LV) ejection fraction. Strain pattern basal-medium segments wall reduced. Right coronary artery (RCA) appeared dilatated (LCA) showed an inverse flow Doppler evaluation. Findings were suspicious ALCAPA diagnosis (Anomalous Left Coronary Artery Arising from Pulmonary Artery); rare congenital heart disease that, if not treated, leads death 90% cases during first year life. Adult type (adolescence-60 y.o) is even more can cause failure dilatative cardiomyopathy, chronic cardiac ischemia and, 80-90% cases, sudden death. We performed angiography that confirmed anomalous origin LCA pulmonary artery. RCA dominant, enlarged supplied important collateral LCA. indicated confirmation, but also tree demonstration, especially adult ALCAPA, because presence anomalies or “adaptation” peripheral circulation. All these features are positive prognostic factors enable survival beyond infancy. ostium stenosis bronchial vessels common weren't seen this case. In surgery usually time diagnosis, so button transfer Valsalva sinus reconstruction autologous pericardial patch performed. This preferred surgical technique patients as it most anatomic correction. Since before hospitalization therapy betablocker introduced after aspirin started too. Post-surgery transthoracic highlighted moderate effusion without haemodynamic impact pleural effusion, anti-inflammatory ibuprofen, cortisone then colchicine started. After discharge under-titrated until suspension based gradual reduction effusion. Patient always free symptoms good performance status. Bland–White–Garland syndrome uncommon disease. adequate circulation between allows survive infancy (adult type). described case early asymptomatic thanks careful basal non-competitive sport practice.