Abstract Ref Number = APCP768
DOUBLE OUTLET LEFT VENTRICLE WITH ATRIOVENTRICULAR SEPTAL DEFECT, PATENT DUCTUS ARTERIOSUS, TRICUSPID ATRESIA AND RIGHT VENTRICLE HYPOPLASIA
Inayah Djabir,Aizah Lawang,Ema Alasiry
Departemen Ilmu Kesehatan Anak Fakultas Kedokteran Universitas Hasanuddin
Background : Double outlet left ventricle (DOLV) is an extremely rare congenital cardiac anomaly in which both great arteries (aorta and pulmonary trunk) arise from the anatomical left ventricle. It occurs in approximately 0,5-1 % of all congenital heart disease. DOLV is associated with high mortality, generally due to heart failure.
Case Presentation Summary : A 3 month old baby girl admitted to hospital due to dyspnea and cyanosis especially during feeding. It was noticed since born and got worse since 2 weeks before admitted to hospital. Physical examination revealed cyanosis, subcostal chest indrawal, and pansystolic murmur grade 3/6 with punctum maximum on left sternal border. Chest X-ray showed cardiomegaly with right to left shunt. Electrocardiography showed left axis deviation. Echocardiography showed Double outlet left ventricle, atrial septal defect, ventricle septal defect, patent ductus arteriousus, tricuspid atresia and right ventricle hypoplasia. She was treated with symptomatic treatment and surgical procedure.
Learning Points/Discussion : • Double outlet left ventricle is quite rare case of congenital cardiac anomaly in children • The clinical manifestations depend largely on the type of the associated cardiac defects • With surgery, the five-year survival rate is estimated at 70-75% • Most patients will continue to present with residual cardiac anomalies, such as aortic or mitral valve regurgitation, arrhythmias or hypertension.
Keywords: Double outlet left ventricle (DOLV) great arteries congenital heart disease congenital cardiac anomaly