Key result
Pulmonary-to-systemic arterial shunts yield ~58% 5-year transplant-free survival in pediatric severe pulmonary hypertension.
Why the study?
Earlier demonstrations of pulmonary-to-systemic arterial shunt placement as palliation for children with severe pulmonary hypertension were limited to relatively small studies, motivating an expanded analysis using an international registry.
What are the short- and long-term outcomes and risk factors for mortality or lung transplantation in children with severe pulmonary hypertension undergoing a pulmonary-to-systemic arterial shunt?
Cohort (n=110)
Yes
What are the short- and long-term outcomes and risk factors for mortality or lung transplantation in children with severe pulmonary hypertension undergoing a pulmonary-to-systemic arterial shunt?
Answers are grounded in this paper and cite their sources.
In the largest pediatric registry to date, pulmonary-to-systemic arterial shunting provided durable functional improvement, prostacyclin weaning, and 5-year transplant-free survival comparable to primary lung transplantation in stable children with severe PH, whereas critically ill children on ICU/ECMO support or with Group-3 PH had poor outcomes.
Background: The placement of a pulmonary-to-systemic arterial shunt in children with severe pulmonary hypertension (PH) has been demonstrated, in relatively small studies, to be an effective palliation for their disease. Objectives: To expand upon these earlier findings utilizing an international registry for children with PH who have undergone a shunt procedure. Methods: Retrospective data from 110 children with PH who underwent a shunt procedure collected from 13 institutions in Europe and the US. Results: Seventeen children died in-hospital post-procedure (15%). Of the 93 children successfully discharged home, 18 subsequently died or underwent lung transplantation (20%), mean follow-up 3.1 years (range: 25 days-17 years). The overall 1- and 5-year freedom from death or transplant were 77% and 58%, and 92% and 68% for those discharged home. Children discharged home had significantly improved World Health Organization (WHO) functional class (p<0.001), 6-minute walk distances (p=0.047) and lower brain natriuretic peptide levels (p<0.001). Post-procedure, 59% of children were weaned completely from their prostacyclin infusion (p<0.001). Pre-procedural risk factors for dying in-hospital post-procedure included ICU admission (Hazard ratio (HR) 3.2, p=0.02), mechanical ventilation (HR 8.3, p<0.001) and extracorporeal membrane oxygenation (HR 10.7, p<0.001). Conclusions: A pulmonary-to-systemic arterial shunt can provide a child with severe PH significant clinical improvement that is both durable and potentially free from continuous prostacyclin infusion. Five-year survival is comparable to children undergoing lung transplantation for PH. Children with severely decompensated disease requiring aggressive intensive care are not good candidates for the shunt procedure.
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Grady et al. (2021) conducted a cohort in Severe pulmonary hypertension (n=110). Pulmonary-to-systemic arterial shunt (surgical Potts shunt, conduit, PDA stenting, or transcatheter shunt) was evaluated on Overall 5-year freedom from death or lung transplantation. Pulmonary-to-systemic arterial shunt placement in children with severe PH yielded 58% 5-year freedom from death or transplant and allowed 59% to wean off prostacyclin.
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