Srinath Gowda, Deepti Bhat, Zhuang Feng, Chung-Ho Chang, Robert D. Ross
The Children’s Hospital of Michigan and Detroit Medical Center.
United States
Congenital Heart Disease
Congenit Heart Dis 2014; 9: E90-E97
DOI: 10.1111/chd.12088
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.
Category
Class II. Pulmonary Hypertension Associated with Pulmonary Vein Stenosis
Genetic Factors Associated with Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
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