Description

3 yo female, 14.8 lbs, 26 in, 0.3m^2 with history of DTGA, PA with discontinuous PAs, large perimembranous VSD, additional swiss-cheese type VSD defect, RAA s/p left modified BT shunt (LMBTS) and bilateral PA plasty. Left sided BTS. Mildly hypoplastic RPA measuring 6.0 mm x 7.8 mm near its origin. Long segment of severe LPA stenosis: The LPA is mildly hypoplastic distally with a long segment of proximal stenosis. The origin of the LPA has a diameter of 5.4 mm x 4.6 mm, then a 7.8 mm segment of narrowing occurs with a measured diameter of 1.8 mm x 2.3 mm before increasing to a measured diameter of 4.1 x 5.4 mm. Qp:Qs of 1:1. The anticipated Rastelli pathway is visualized without obstruction from VSD or interference with Tricuspid valve. Learning points Model clearly demonstrates the Rastelli pathway but heavily trabeculated apex of LV and RV 3-dimensionally conveyed swiss-cheese VSD which was not appreciated in 2D imaging.