Emergent Transcatheter Aortic Valve Replacement (TAVR) Performed on Patient With DiGeorge Syndrome

Emergent Transcatheter Aortic Valve Replacement (TAVR) is a strategy that has been used for management of severely decompensated patients who are unlikely to tolerate an open procedure (Kolte et al., 2018). Recently, in the context of degenerated valve bioprosthesis, valve-in-valve (ViV) transcatheter aortic valve replacement has become an acceptable management strategy (Kalra et al., 2019 [2]). Here, we present this rare case of a 25-year-old, post-partum female with DiGeorge Syndrome, who presented with severe bioprosthetic valve stenosis leading to heart failure. She initially had received a biologic valve in order to have children; however, following delivery of her child, she developed valve failure that was severe enough to preclude her from receiving a surgical aortic valve replacement. ViV TAVR was performed emergently to improve heart failure and bridge the time to definitive treatment, when she would be able to safely receive a mechanical valve. After valve placement, echocardiogram showed no evidence of aortic regurgitation or paravalvular leak with a mean gradient of 2 mmHg, and she was ultimately discharged. Our patient was recovering well at her two-month follow up appointment. This case highlights the need for further research in the use of ViV TAVR in younger patient populations in emergent situations.