title: 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.
authors: Pediatric Cardiology, The Children's Hospital of Michigan, Detroit Medical Center, Detroit, Mich, USA.
authors: Srinath
authors: S
authors: Gowda
authors: Deepti
authors: D
authors: Bhat
authors: Zhuang
authors: Z
authors: Feng
authors: Chung-Ho
authors: CH
authors: Chang
authors: Robert D
authors: RD
authors: Ross
mesh_terms: D000367:Age Factors; D000800:Angioplasty, Balloon; D000959:Antihypertensive Agents / Q000627:therapeutic use; D062186:Arterial Pressure; D003251:Constriction, Pathologic; D018450:Disease Progression; D004314:Down Syndrome / Q000150:complications* / Q000175:diagnosis; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D007223:Infant; D008297:Male; D011651:Pulmonary Artery / Q000503:physiopathology; D011652:Pulmonary Circulation; D011667:Pulmonary Veins / Q000503:physiopathology; D011668:Pulmonary Veno-Occlusive Disease / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures
chemical_list: D000959:Antihypertensive Agents
keywords: Catheterization; Down Syndrome; Pulmonary Hypertension; Pulmonary Vein Stenosis