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Extracorporeal Life Support in Multisystem Smooth Muscle Dysfunction Syndrome

Extracorporeal Life Support in Multisystem Smooth Muscle Dysfunction Syndrome We describe an infant with congenital mydriasis, patent ductus arteriosus (PDA), pulmonary hypertension, and cystic lung disease. She had all the major components of multisystemic smooth muscle dysfunction syndrome. Due to progressive respiratory deterioration, she required surgical PDA interruption, extracorporeal life support, and subsequent prolonged respiratory support. Genetic testing revealed ACTA2 R179H mutation and cystic lung disease on biopsy. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png World Journal for Pediatric and Congenital Heart Surgery SAGE

Extracorporeal Life Support in Multisystem Smooth Muscle Dysfunction Syndrome

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References (8)

Publisher
SAGE
Copyright
© The Author(s) 2016
ISSN
2150-1351
eISSN
2150-136X
DOI
10.1177/2150135116658457
pmid
27549731
Publisher site
See Article on Publisher Site

Abstract

We describe an infant with congenital mydriasis, patent ductus arteriosus (PDA), pulmonary hypertension, and cystic lung disease. She had all the major components of multisystemic smooth muscle dysfunction syndrome. Due to progressive respiratory deterioration, she required surgical PDA interruption, extracorporeal life support, and subsequent prolonged respiratory support. Genetic testing revealed ACTA2 R179H mutation and cystic lung disease on biopsy.

Journal

World Journal for Pediatric and Congenital Heart SurgerySAGE

Published: Nov 1, 2017

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