Pulmonary vein stenosis with Down syndrome: a rare and frequently fatal cause of pulmonary hypertension in infants and children.

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 (38 mm Hg), 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.

journal_name

Congenit Heart Dis

journal_title

Congenital heart disease

authors

Gowda S,Bhat D,Feng Z,Chang CH,Ross RD

doi

10.1111/chd.12088

subject

Has Abstract

pub_date

2014-05-01 00:00:00

pages

E90-7

issue

3

eissn

1747-079X

issn

1747-0803

journal_volume

9

pub_type

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