alexa De Bukes Syndrome - Tetrology of Fallot with Absent Left Pulmonary Artery

Cardiovascular Therapy: Open Access
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Case Report

De Bukes Syndrome - Tetrology of Fallot with Absent Left Pulmonary Artery

Madhu KJ*, Sunil kumar, Vijayalakshmi IB, Chitra and Manjunath CN

Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, India

*Corresponding Author:
Madhu KJ
Department of Cardiology
Sri Jayadeva Institute of Cardiovascular Sciences and Research, India
Tel: +9902783016
E-mail: [email protected]

Received date: January 25, 2016 Accepted date: February 26, 2016 Published date: March 03, 2016

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.

 

Abstract

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.

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