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

Jun Muneuchi, Shinichiro Oda, Daisuke Shimizu

Research output: Contribution to journalArticlepeer-review

2 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1402-1405
Number of pages4
JournalCardiology in the Young
Volume27
Issue number7
DOIs
Publication statusPublished - Sep 1 2017
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

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