R Mark Grady1, Matthew W Canter2, Fei Wan2, Anton A Shmalts3, Ryan D Coleman4, Maurice Beghetti5, Rolf M F Berger6, Maria J Del Cerro Marin7, Scott E Fletcher8, Russel Hirsch9, Tilman Humpl10, D Dunbar Ivy11, Edward C Kirkpatrick12, Thomas J Kulik13, Marilyne Levy14, Shahin Moledina15, Delphine Yung16, Pirooz Eghtesady2, Damien Bonnet14. 1. Department of Pediatrics, Washington University, St. Louis, Missouri, USA. Electronic address: rmgrady@wustl.edu. 2. Department of Surgery, Washington University, St. Louis, Missouri, USA. 3. Department of Pulmonary Hypertension, A.N. Bakoulev National Medical Research Center of Cardiovascular Surgery, Moscow, Russia. 4. Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA. 5. Pediatric Cardiology Unit, Faculty of Medicine, University of Geneva and Centre Universitaire Romand de Cardiologie et Chirurgie Cardiaque Pédiatrique, University Hospitals of Geneva and Lausanne, Geneva and Lausanne, Switzerland. 6. Center for Congenital Heart Disease, Department of Pediatric Cardiology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands. 7. Hospital Universitario Ramon y Cajal, Madrid, Spain. 8. Department of Pediatrics, University of Nebraska, Creighton University at Children's Hospital and Medical Center, Omaha, Nebraska, USA. 9. Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio, USA. 10. University of Bern, Bern, Switzerland. 11. Department of Pediatrics, University of Colorado, Denver, Colorado, USA. 12. Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA. 13. Department of Pediatrics, Harvard University, Boston, Massachusetts, USA. 14. Congenital and Pediatric Cardiology Unit, Hospital Necker-Enfants Malades, APHP, Universitaire de Paris, Paris, France. 15. Great Ormand Street Hospital for Children, London, England. 16. Department of Pediatrics, University of Washington, Seattle, Washington, USA.
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.
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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