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.

Keywords: Tetrology of fallot; Right pulmonary artery; Left pulmonary artery; Balloon dilatation