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Il Giornale Italiano di Radiologia Medica 2019 Marzo-Aprile;6(2):148-54

DOI: 10.23736/S2283-8376.19.00180-3

Copyright © 2019 EDIZIONI MINERVA MEDICA

language: English, Italian

Congenital unilateral absence of the pulmonary artery

Gianluca FICARRA 1, 2 , Aldo FISCHETTI 1, 2, Nicola ROMANO 1, 2, Valentina CLAUDIANI 2, Enrico F. MELANI 2, Gian Andrea ROLLANDI 2

1 Radiology Section, Department of Health Sciences DiSSal, IRCCS Policlinico San Martino, University of Genoa, Genoa, Italy; 2 Radiology Unit, E.O. Ospedali Galliera, Genoa, Italy


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Unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly which may present isolated or in association with other cardiovascular abnormalities (tetralogy of Fallot, atrial and ventricular septal defect, aortic coarctation, right sided aortic arch, truncus arteriosus and pulmonary valve atresia); a complete absence is caused by the involution of the proximal sixth aortic arch with failure in connection with the pulmonary trunk. Isolated UAPA is rare and patients who have no associated cardiac anomalies usually grow up without developing significant symptoms. When diagnosed in adulthood this condition is frequently incidentally detected during chest radiography or computed tomography scans performed for unrelated reasons, and therefore awareness of this condition is important also for general radiologists. Although there are different therapeutic options including surgery, endovascular embolization and conservative management there is actually no wide consensus on treatment; therapeutic choice should be tailored on patient’s needs on a case by case basis, considering symptoms, pulmonary function and patient’s anatomy. We herein report a case of a 32- year-old woman affected by this condition who sought medical care at our emergency department complaining fever, cough, wheezing and mild dyspnea. A review of current literature and insights regarding physiopathology, common clinical presentation, imaging features, possible complications and current treatment options are also presented.


KEY WORDS: Cardiac imaging techniques; Thorax; Pulmonary artery

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