R. Mark Grady, Matthew W. Canter, Fei Wan, Anton A. Shmalts, Ryan D. Coleman, Maurice Beghetti, Rolf M. F. Berger, Maria J. del Cerro Marin, MD, Scott E. Fletcher, Russel Hirsch, Tilman Humpl, D. Dunbar Ivy, Edward C. Kirkpatrick, Thomas J. Kulik, Marilyne Levy, Shahin Moledina, Delphine Yung, Pirooz Eghtesady, Damien Bonnet
Washington University, A.N. Bakoulev National Medical Research Center of Cardiovascular Surgery, Baylor College of Medicine, University of Geneva and Centre Universitaire Romand de Cardiologie et Chirurgie Cardiaque Pédiatrique, University of Groningen, Groningen, Universitario Ramon y Cajal, University of Nebraska, University of Cincinnati, University of Bern, University of Colorado, Medical College of Wisconsin, Harvard University. Hospital Necker-Enfants Malades and Universitaire de Paris. Great Ormand Street Hospital for Children. University of Washington and Seattle Children’s Hospital.
United States, Russia, Switzerland, Netherlands, Spain, France, Germany and United Kingdom
Journal of the American College of Cardiology
J Am Coll Cardiol 2021; 78: 468-477
DOI: 10.1016/j.jacc.2021.05.039
Abstract
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.
Category
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
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Free PDF File or Full Text Article Available Through PubMed or DOI: Yes