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Improved heart transplant survival for children with congenital heart disease and heterotaxy syndrome in the current era: An analysis from the Pediatric Heart Transplant Society

Journal of Heart and Lung Transplantation Jul 26, 2021

Khan A, Pahl E, Koehl DA, et al. - As challenges exist with heterotaxy because of the complexity of heart disease, abnormal venous connections, and infection risks, researchers herein assessed heart transplant results for children with heterotaxy. They included all children suffering from congenital heart disease listed for transplant from 1993-2018. Of 4814 children listed, heterotaxy was present in 196 (4%). In waitlist outcomes of transplant, death, or removal, no differences were observed. Children with heterotaxy had worse post-transplant survival: 1-year survival 77.2% vs 85.1%, with and without heterotaxy, respectively. On stratification by era, an improvement in survival was evident over time. Findings revealed that in the recent transplant era, death risk linked with heterotaxy is mitigated. An improvement in waitlist outcomes for heterotaxy patients, who otherwise have a lower status at listing, may be achieved with early referral. In heterotaxy, lower freedom from both infection and severe rejection post-transplant was noted, which emphasizes the challenges of balancing immune suppression.

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