![]() UPAA and RAA are both postulated secondary to inaccruate embryological development of primitive aortic arches. Our patient has another accompanying rare great vessel anomaly which is unusual for this subtype. While congenital heart diseases and vascular anomalies are associated with type 1, cardiovasculary disorders are rarely seen together with type 2. Type I is the most common encountered subgroup. Type I, with mirrorimage branching of the major arteries type II, with an aberrant subclavian artery, concomitant Kommerell diverticulum may be present and type III, with isolation of the subclavian artery (where the subclavian artery is connected to the pulmonary artery through the ductus arteriosus. RAA is classified into three main groups based on anatomical structure. UPAA is relatively uncommon on the left side. UPAA is an infrequent condition which occurs in approximately 1 in 200,000 people. Chest X-ray demonstrated shift of the mediastinal structures to the left with volume loss of the left lung, decreased left pulmonary vascularization and hyperlucent right lung ( Figure 1).įigure 2: Axial chest CT image (a) with 3D reconstruction image (b) and coronal reformat image (c) with 3D reconstruction image (d) showed absence of the left pulmonary artery and branches (yellow arrow) with right sided aortic arch, also accompanying Kommerell's diverticulum (white arrow), and shift of the mediastinal structures to the left. The rest physical examination and laboratory tests were normal. His physical examination revealed reduced pulmonary sounds on the left hemithorax. The patient was a non-smoker with no history of exposure to noxious environmental or occupational factors. Case ReportĪ 24-year-old male patient was admitted to our hospital due to exertional dyspnea. Most common symptoms experienced include recurrent pulmonary infections, hemoptysis, and shortness of breath upon exertion. UAPA is believed to result from the involution of the proximal sixth aortic arch, thereby forgoing expected embryological fusion with the pulmonary trunk. This explains why the more prevalent right UAPA occurs with a normal left-sided arch. The affected pulmonary artery is most commonly opposite to the aortic arch. Īgenesis of the right pulmonary artery has a greater incidence than agenesis of the left. Physicians should consider the possibility of undiagnosed UPAA in adults. A number of additional Computed tomographytechniques are available to aid the diagnosis. UPAA can remain asymptomatic and diagnosis in adult age is possible, usually after an abnormal chest radiograph. Unilateral absence of pulmonary artery (UAPA) is a rare congenital malformation that may develop in isolation or in association with congenital cardiovascular anomalies. Unilateral Pulmonary Artery Agenesis (UAPA) Right-sided aortic arch Kommerell’s diverticulum Computer Tomography (CT) Introduction ![]()
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |