Pulmonary-to-Systemic Arterial Shunt to Treat Children With Severe Pulmonary Hypertension.

Autor:	Grady RM; Department of Pediatrics, Washington University, St. Louis, Missouri, USA. Electronic address: rmgrady@wustl.edu., Canter MW; Department of Surgery, Washington University, St. Louis, Missouri, USA., Wan F; Department of Surgery, Washington University, St. Louis, Missouri, USA., Shmalts AA; Department of Pulmonary Hypertension, A.N. Bakoulev National Medical Research Center of Cardiovascular Surgery, Moscow, Russia., Coleman RD; Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA., Beghetti M; 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., Berger RMF; Center for Congenital Heart Disease, Department of Pediatric Cardiology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands., Del Cerro Marin MJ; Hospital Universitario Ramon y Cajal, Madrid, Spain., Fletcher SE; Department of Pediatrics, University of Nebraska, Creighton University at Children's Hospital and Medical Center, Omaha, Nebraska, USA., Hirsch R; Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio, USA., Humpl T; University of Bern, Bern, Switzerland., Ivy DD; Department of Pediatrics, University of Colorado, Denver, Colorado, USA., Kirkpatrick EC; Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA., Kulik TJ; Department of Pediatrics, Harvard University, Boston, Massachusetts, USA., Levy M; Congenital and Pediatric Cardiology Unit, Hospital Necker-Enfants Malades, APHP, Universitaire de Paris, Paris, France., Moledina S; Great Ormand Street Hospital for Children, London, England., Yung D; Department of Pediatrics, University of Washington, Seattle, Washington, USA., Eghtesady P; Department of Surgery, Washington University, St. Louis, Missouri, USA., Bonnet D; Congenital and Pediatric Cardiology Unit, Hospital Necker-Enfants Malades, APHP, Universitaire de Paris, Paris, France.
Jazyk:	angličtina
Zdroj:	Journal of the American College of Cardiology [J Am Coll Cardiol] 2021 Aug 03; Vol. 78 (5), pp. 468-477.
DOI:	10.1016/j.jacc.2021.05.039
Abstrakt:	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. Competing Interests: Funding Support and Author Disclosures Supported by the St. Louis Children’s Hospital Foundation. The authors have reported that they have no relationships relevant to the contents of this paper to disclose. (Copyright © 2021 American College of Cardiology Foundation. All rights reserved.)
Databáze:	MEDLINE
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