Unilateral Absence of Main Branch of Pulmonary Artery

Background: Unilateral absence of a pulmonary artery (UAPA) is an uncommon anomaly with an estimated prevalence of ۱ in ۲۰۰,۰۰۰ young adults and a diverse clinical presentation. It may be diagnosed as an isolated anomaly or in combination with other congenital heart defects. The purpose of the present study was to investigate the clinical course and characteristics of forty patients with UAPA in whom the diagnosis was first established in childhood or adulthood.Material & Methods: From January ۲۰۰۲ through April ۲۰۲۱, ۴۰ patients with unilateral absence of one pulmonary artery were diagnosed at our institution. We retrospectively reviewed the medical records of these patients in detail and elicited information covered patients' demographics, associated anomalies, imaging studies and operation data.Results: All forty patients were between ۲ months and ۲۵ years old (mean age, ۶.۴۵ years) at first presentation, weighing ۴.۷۰-۵۹.۰۰ kg (mean weight, ۱۹.۱۷ kg) and twenty five patients were female (۶۲.۵%). Absent left pulmonary artery (LPA) was found in ۶۰% of cases (۲۴ patients) and ۴۰% had occult right pulmonary artery (RPA). Right-sided aortic arch was found in ۴ patients (۱۰%). Tetralogy of Fallot was diagnosed in ۱۶ patients while complex cardiac anomalies were found in ۱۳ cases.Conclusion: clinicians should consider the undiagnosed cases of UAPA especially in adults with suspicious symptoms such as unexplained hemoptysis. Applying different available imaging techniques for early diagnosis and treatment of isolated UAPA cases are important to prevent the devastating complications of pulmonary hypertension and massive hemoptysis in long term.

Keywords:

Unilateral absence of pulmonary artery , Symptoms , Surgical treatment