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Rapidly progressive pulmonary veno-occlusive disease in an infant with Down syndrome

Published online by Cambridge University Press:  27 March 2017

Jun Muneuchi ,
Shinichiro Oda  and
Daisuke Shimizu
Jun Muneuchi*
Affiliation:
Department of Pediatrics, Japan Community Healthcare Organization Kyushu Hospital, Kitakyushu, Japan
Shinichiro Oda
Affiliation:
Department of Cardiovascular Surgery, Japan Community Healthcare Organization Kyushu Hospital, Kitakyushu, Japan
Daisuke Shimizu
Affiliation:
Department of Pediatrics, Japan Community Healthcare Organization Kyushu Hospital, Kitakyushu, Japan
*
Correspondence to: J. Muneuchi, MD, Department of Pediatrics, Japan Community Healthcare Organization Kyushu Hospital, 1-8-1, Kishinoura, Yahatanishi-ku, Fukuoka 806-8501, Japan. Tel: +81 93 641 5111; Fax: +81 93 642 1868; E-mail: jmun@msn.com
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Abstract

A 4-month-old girl with Down syndrome showed unexpected deterioration of pulmonary hypertension. Despite aggressive pulmonary vasodilation therapy, the patient died at 5 months of age. Lung autopsy showed that the pulmonary veins were obliterated by intimal fibrous thickening, and the media of the veins was arterialised with an increase in elastic fibres. Pulmonary veno-occlusive disease should be considered in the management of individuals with Down syndrome.

Keywords

Pulmonary veno-occlusive diseasepulmonary arterial hypertensionCHDDown syndromechromosomal anomaly
Type
Brief Report
Information
Cardiology in the Young , Volume 27 , Issue 7 , September 2017 , pp. 1402 - 1405
Copyright
© Cambridge University Press 2017 

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