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
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins
Genetic Factors Influencing the Onset, Severity or Outcome of Disease
Patient Factors Influencing the Onset, Severity or Outcome of Disease
Length of Life Associated with Pulmonary Venous Obstruction
Year of Publication: 2014
Age Focus: Pediatric
Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients). Literature Review and Analysis.
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No