Pulmonary Vein stenosis with Down syndrome: A rare and frequently fatal cause of pulmonary hypertension in infants and children

Srinath Gowda, Deepti Bhat, Zhuang Feng, Chung Ho Chang, Robert D. Ross

Research output: Contribution to journalArticlepeer-review

20 Scopus citations

Abstract

Down syndrome (DS) patients are prone to pulmonary hypertension (PHTN) due to various cardiopulmonary causes. However, the association of DS with pulmonary vein stenosis (PVS) is not adequately described. We illustrate three cases from our center and an additional 13 cases from an extensive review of the literature of DS patients with PHTN and PVS. In DS patients PVS is rare, they were diagnosed at a young age (<7.2 months), had high mean pulmonary artery pressures (38mmHg), and had rapid progression of the stenosis with an increased mortality (91%) in patients with two or more vein involvement. In DS patients, PVS may be missed by echocardiography; thus, any DS patients with persistent PHTN should undergo cardiac catheterization to assess hemodynamics and to evaluate all four pulmonary veins.

Original languageEnglish
Pages (from-to)E90-E97
JournalCongenital Heart Disease
Volume9
Issue number3
DOIs
StatePublished - 2014

Keywords

  • Catheterization
  • Down syndrome
  • Pulmonary hypertension
  • Pulmonary vein stenosis

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