Unilateral absence of pulmonary artery: Pathophysiology, symptoms, diagnosis and current treatment

Summary Unilateral absence of pulmonary artery (UAPA) is a rare malformation that can present as an isolated lesion or may be associated with other congenital heart defects. UAPA is often associated with other congenital cardiovascular anomalies, such as tetralogy of Fallot, atrial septal defect, co...

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Bibliographic Details
Published in:Archives of cardiovascular diseases 2013-08, Vol.106 (8), p.448-454
Main Authors: Kruzliak, Peter, Syamasundar, Rao P, Novak, Miroslav, Pechanova, Olga, Kovacova, Gabriela
Format: Article
Language:English
Subjects:
Absence unilatérale de l’artère pulmonaire
Cardiopathie congénitale
Cardiovascular
Congenital heart disease
Diagnostic Imaging - methods
Heart Defects, Congenital - complications
Heart Defects, Congenital - diagnosis
Heart Defects, Congenital - physiopathology
Heart Defects, Congenital - therapy
Humans
Internal Medicine
Pathophysiologie
Pathophysiology
Physical Examination
Pneumonectomy
Predictive Value of Tests
Pulmonary Artery - abnormalities
Pulmonary Artery - physiopathology
Pulmonary Artery - surgery
Symptoms
Symptômes
Treatment
Treatment Outcome
Unilateral absence of pulmonary artery
Vascular Surgical Procedures
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Summary:Summary Unilateral absence of pulmonary artery (UAPA) is a rare malformation that can present as an isolated lesion or may be associated with other congenital heart defects. UAPA is often associated with other congenital cardiovascular anomalies, such as tetralogy of Fallot, atrial septal defect, coarctation of aorta, right aortic arch, truncus arteriosus and pulmonary atresia. Diagnosis of UAPA is very difficult and is based on taking a complete medical history, physical examination and imaging examinations. Clinical symptoms include exercise intolerance, haemoptysis and recurrent respiratory infections. Adult patients with UAPA are often asymptomatic. There is no consensus regarding the treatment for UAPA. The therapeutic approach should be based on symptoms of the patient, pulmonary artery anatomy and associated aortopulmonary collaterals. Treatment options for these patients include partial or total pneumonectomy, closure of selected collateral arteries not solely responsible for pulmonary blood flow or a primary versus staged pulmonary artery anastomosis. This review summarizes pathophysiology, symptomatology and current diagnosis and treatment of this disease.
ISSN:1875-2136
1875-2128
DOI:10.1016/j.acvd.2013.05.004