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Published on: February 11, 2017
Pulmonary-to-Systemic Arterial Shunt to Treat Children With Severe Pulmonary Hypertension
R Mark Grady1, Matthew W Canter2, Fei Wan2
1Department of Pediatrics, Washington University, St. Louis, Missouri, USA.
Insights
Pulmonary-to-systemic arterial shunts offer significant clinical improvement for children with severe pulmonary hypertension (PH). This procedure provides durable palliation, with survival rates comparable to lung transplantation, but is not suitable for critically ill patients.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Pulmonary Hypertension Management
Background:
- Severe pulmonary hypertension (PH) in children poses significant challenges.
- Pulmonary-to-systemic arterial shunts have shown promise as a palliative treatment in smaller studies.
Purpose of the Study:
- To evaluate the effectiveness and outcomes of pulmonary-to-systemic arterial shunts in a larger, international cohort of pediatric PH patients.
- To identify factors influencing in-hospital mortality and long-term survival post-shunt procedure.
Main Methods:
- Retrospective analysis of data from 110 children with PH who underwent a shunt procedure.
- Data collected from 13 institutions across Europe and the United States.
- Follow-up data analyzed for survival, functional status, and need for prostacyclin therapy.
Main Results:
- Overall 1- and 5-year survival rates were 77% and 58%, respectively.
- Children discharged home showed significant improvements in functional class, 6-minute walk distance, and reduced brain natriuretic peptide levels.
- 59% of patients were successfully weaned from prostacyclin infusion post-procedure.
Conclusions:
- Pulmonary-to-systemic arterial shunts provide durable clinical improvement and palliation for pediatric PH.
- Survival rates are comparable to lung transplantation, offering a viable alternative.
- Critically ill children requiring intensive care are not ideal candidates for this procedure.
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:
The aim of this study was to expand upon these earlier findings using an international registry for children with PH who have undergone a shunt procedure.
Methods:
Retrospective data were obtained from 110 children with PH who underwent a shunt procedure collected from 13 institutions in Europe and the United States.
Results:
Seventeen children died in-hospital postprocedure (15%). Of the 93 children successfully discharged home, 18 subsequently died or underwent lung transplantation (20%); the mean follow-up was 3.1 years (range: 25 days to 17 years). The overall 1- and 5-year freedom from death or transplant rates were 77% and 58%, respectively, and 92% and 68% for those discharged home, respectively. Children discharged home had significantly improved World Health Organization functional class (P < 0.001), 6-minute walk distances (P = 0.047) and lower brain natriuretic peptide levels (P < 0.001). Postprocedure, 59% of children were weaned completely from their prostacyclin infusion (P < 0.001). Preprocedural risk factors for dying in-hospital postprocedure included intensive care unit 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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