Unilateral Absence of Main Branch of Pulmonary Artery

Unilateral absence of a pulmonary artery (UAPA) is an uncommon anomaly with an estimated prevalence of 1 in 200,000 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.

From January 2002 through April 2021, 40 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.

All forty patients were between 2 months and 25 years old (mean age, 6.45 years) at first presentation, weighing 4.70-59.00 kg (mean weight, 19.17 kg) and twenty five patients were female (62.5%). Absent left pulmonary artery (LPA) was found in 60% of cases (24 patients) and 40% had occult right pulmonary artery (RPA). Right-sided aortic arch was found in 4 patients (10%). Tetralogy of Fallot was diagnosed in 16 patients while complex cardiac anomalies were found in 13 cases.

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.