Biventricular repair with end-to-side aorta to pulmonary artery central shunt for ventricular septal defect, severe pulmonary stenosis with hypoplastic pulmonary artery

The case was 11-month-old girl diagnosed as tetralogy of Fallot with severe pulmonary valve stenosis and suffering from severe cyanosis. A catheter study demonstrated pulmonary artery (PA) was well arborized, but severely hypoplastic in spite of previous transcatheter pulmonary valve dilatation; PA index was calculated as 69 mm2/m2. A central end-to-side aorta to PA shunt was created. Cyanosis was well improved, but congestive heart failure occurred after 1 month from the operation. Subsequent catheter study demonstrated pulmonary artery growth, 166 mm2/m2 of PA index and major aortopulmonary collateral artery (MAPCA) coil embolization was performed. Patient underwent Rastelli type definitive repair 9 month after palliation. The central end-to-side aorta to PA shunt is reported as useful measure for promoting PA size increase and definitive repair achievement in patient with pulmonary atresia, ventricular septal defect and severely hypoplastic PA. Appropriate consideration of criteria, carefull follow up and treatments are necessary.