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

Abstract

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.

Keywords: Absence unilatérale de l’artère pulmonaire; CT; Cardiopathie congénitale; Computed tomography; Congenital heart disease; MRA; MRI; Magnetic resonance angiography; Magnetic resonance imaging; PA; PHT; Pathophysiologie; Pathophysiology; Pulmonary artery; Pulmonary hypertension; Symptoms; Symptômes; Treatment; UAPA; Unilateral absence of pulmonary artery.