Abstract
IntroductionUnilateral absence of the pulmonary artery (UAPA) or pulmonary artery agenesis is a rare congenital disorder presenting with a wide spectrum of symptoms. The clinical presentation is variable and many patients can be asymptomatic for many years and even throughout their lives.Case presentationWe report the case of a 53-year-old African-American woman who was diagnosed with right pulmonary artery agenesis after presenting with uncontrolled asthma and recurrent bronchopulmonary infections.ConclusionIn an unexplained case of recurrent respiratory infections and shortness of breath, the possibility of a rare congenital anomaly like UAPA should be considered and an appropriate evaluation should be done.
Highlights
Unilateral absence of the pulmonary artery (UAPA) or pulmonary artery agenesis is a rare congenital disorder presenting with a wide spectrum of symptoms
Clinicians should be aware of the possibility of undiagnosed cases of UAPA presenting with recurrent respiratory infections
Confirmation of the diagnosis and anatomic details can be discerned by computed tomography (CT) scanning and Magnetic resonance imaging (MRI)
Summary
Unilateral absence of the pulmonary artery (UAPA) or pulmonary artery agenesis is a rare congenital disorder presenting with a wide spectrum of symptoms. Case presentation A 53-year-old African-American woman was referred to our pulmonary clinic because of uncontrolled asthma and frequent respiratory infections. Our patient reported frequent asthmatic attacks and symptoms requiring excessive use of rescue inhalers. She described symptoms consistent with gastroesophageal reflux. Her physical development was normal and there was no family history of congenital cardiovascular disease. Our patient was awake and alert, in no acute distress She was afebrile and her vital signs were stable. Her physical examination revealed decreased breath sounds with mild rhonchi in her right lower lung zone. FVC increased to 2.7 L (102% of predicted) which is consistent with a significant response
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