Pediatric transplantation · 2025
Pediatric Marfan Syndrome and Heart Transplantation: Insights From the PHIS Database.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Philip Stephanie S, Martinez Hugo R, Mikulski Matthew F, Well Andrew, Jani Sagar, Glass Lauren, Fraser Charles D, Castleberry Chesney
Аннотация
BACKGROUND: Marfan syndrome (MFS) is a connective tissue disorder associated with significant cardiovascular complications, including heart failure. Orthotopic heart transplantation (OHT) is considered controversial in this population due to concerns about post-transplant aortic complications, particularly in children.
METHODS: We conducted a retrospective review of the Pediatric Health Information System (PHIS) database to identify patients under 18 years of age with a diagnosis of MFS who underwent OHT between 2004 and 2024. Patients were propensity matched (3:1) to non-MFS OHT recipients. Outcomes included graft failure, aortic events, and re-transplant-free survival.
RESULTS: Ten pediatric MFS patients were identified among 5493 OHT recipients. The median age at OHT was 12.5 years. Over a median follow-up of 3.73 years, no in-hospital mortalities or repeat transplants were observed; two patients experienced rejection, and one developed aortic root dilation without requiring intervention. Propensity-matched analysis showed no significant differences in rejection, aortic events, or transplant-free survival between MFS and non-MFS cohorts.
CONCLUSION: Though rare, pediatric MFS patients undergoing OHT demonstrated excellent short-term outcomes with no MFS-related surgical complications. These findings challenge current exclusionary practices and support further research into long-term outcomes.
METHODS: We conducted a retrospective review of the Pediatric Health Information System (PHIS) database to identify patients under 18 years of age with a diagnosis of MFS who underwent OHT between 2004 and 2024. Patients were propensity matched (3:1) to non-MFS OHT recipients. Outcomes included graft failure, aortic events, and re-transplant-free survival.
RESULTS: Ten pediatric MFS patients were identified among 5493 OHT recipients. The median age at OHT was 12.5 years. Over a median follow-up of 3.73 years, no in-hospital mortalities or repeat transplants were observed; two patients experienced rejection, and one developed aortic root dilation without requiring intervention. Propensity-matched analysis showed no significant differences in rejection, aortic events, or transplant-free survival between MFS and non-MFS cohorts.
CONCLUSION: Though rare, pediatric MFS patients undergoing OHT demonstrated excellent short-term outcomes with no MFS-related surgical complications. These findings challenge current exclusionary practices and support further research into long-term outcomes.