A Rare Case of Isolated Unilateral Atresia of Pulmonary Artery in an Elderly Female

Abstract

Isolated unilateral agenesis/atresia of pulmonary artery (IUAPA) is a rare congenital disorder, an uncommon variant of unilateral agenesis of pulmonary artery (UAPA). Patients with IUAPA may remain asymptomatic and undiagnosed till late adulthood as they present with vague symptoms which may be overlooked. We report a case of IUAPA of right pulmonary artery in an elderly female who presented with complaints of productive cough and exertional dyspnea. Due to the formation of extensive collaterals, her lung parenchyma was preserved.

Isolated unilateral absence of pulmonary artery (IUAPA) in adults: A case series and review of literature

“…The prevalence rate is estimated to be approximately one in 200,000. 2 Most patients with UAPA are found to also have congenital heart defects, such as tetralogy of Fallot, atrial septal defects, truncus arteriosus and more. 1 …”

“…It was first described in 1868 by Frantzel O. Angeborener and was proven by Madoff et al in 1953 through angiography. The prevalence rate is estimated to be approximately one in 200,000 2 . Most patients with UAPA are found to also have congenital heart defects, such as tetralogy of Fallot, atrial septal defects, truncus arteriosus and more 1 …”

“…1 The prevalence rate is estimated to be approximately one in 200,000. 2 Most patients with UAPA are found to also have congenital heart defects, such as tetralogy of Fallot, atrial septal defects, truncus arteriosus and more. 1 A study conducted among 352 patients with UAPA in 2011 revealed that around 30% of patients with UAPA did not have cardiac anomalies.…”

Unilateral Choanal Atresia in a Child With Prolonged Nasal Congestion

“…Bilateral cases may be life-threatening, with symptoms of upper airway obstruction almost always prompting early detection in infants and neonates [ 4 ]. Alternatively, unilateral cases may go undetected for years, with more subtle cases being detected in adulthood [ 5 ]. Such cases can prove perplexing for physicians since a high index of suspicion is required to make the diagnosis.…”