De Bukes Syndrome - Tetrology of Fallot with Absent Left Pulmonary Artery
Received Date: Jan 25, 2016 / Accepted Date: Feb 26, 2016 / Published Date: Jan 03, 1970
De Bucket syndrome - TOF with unilateral absence of a pulmonary artery (UAPA) is a rare condition with an estimated prevalence of 1 in 200,000 young adults. Most commonly, UAPA occurs in conjunction with cardiovascular abnormalities such as tetralogy of Fallot (TOF) coarctation of the aorta, VSD, subvalvular aortic stenosis, transposition of the great arteries (plus VSD or pulmonary stenosis), Taussig-Bing malformation and coarctation, congenitally corrected transposition and pulmonary stenosis, scimitar syndrome. Patients with isolated UAPA can remain asymptomatic into late adulthood but usually report symptoms such as dyspnea or chest pain or suffer from hemoptysis or recurrent infections. Diagnosis can be difficult due to the rarity of the condition and its nonspecific presentation. We present a case of a 5month old child who presented with TOF with Right pulmonary artery stenosis and absent left pulmonary artery, with typical findings on chest radiograph, angiographic features and treatment discussed.
Keywords: Tetrology of fallot; Right pulmonary artery; Left pulmonary artery; Balloon dilatation
Citation: Madhu KJ, Kumar S, Vijayalakshmi IB, Chitra Manjunath CN (2016) De Bukes Syndrome - Tetrology of Fallot with Absent Left Pulmonary Artery. Cardiovasc Ther 1: 105.
Copyright: © 2016 Madhu KJ, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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