Right pulmonary artery agenesis presenting with uncontrolled asthma in an adult: a case report

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.

Unilateral acute respiratory distress syndrome with contralateral pulmonary artery agenesis: A rare scenario

“…UPAA has no specific symptoms, and as many as 30% individuals may remain asymptomatic, making its diagnosis difficult. [ 7 ] It is due to a defect in the connection of the pulmonary trunk with the sixth aortic arch. [ 6 ] Pulmonary angiography is the gold standard for diagnosing UPAA.…”

Pulmonary Artery Agenesis in Young Adult - Two Case Reports

“…The angiography remains the method of choice for the diagnosis of pulmonary artery agenesis. But is reserved for cases where embolization for massive hemoptysis is indicated or for pre-operatory evaluation of patients undergoing surgical revascularization [4,7]. Treatment strategies of PAA depend very much on the clinical framework.…”

“…Treatment strategies of PAA depend very much on the clinical framework. Patients with hemoptysis, recurrent pulmonary infections are candidates for surgical treatment by pneumonectomy or lobectomy [4,7]. Embolization of bronchial systemic arteries is an alternative for patients with massive hemoptysis not eligible for surgery [4].…”

“…Patients with hemoptysis, recurrent pulmonary infections are candidates for surgical treatment by pneumonectomy or lobectomy [4,7]. Embolization of bronchial systemic arteries is an alternative for patients with massive hemoptysis not eligible for surgery [4]. If pulmonary hypertension is present the options are revascularization of arterial missing branch or, if not feasible, treatment with specific medical therapy [1] as in the first case we reported.…”

“…Mortality rate is about 7%, but both the presence of pulmonary hypertension and pulmonary bleeding phenomena can adversely affect the long-term prognosis [1,2]. In most cases, this condition is diagnosed in childhood, but some patients may remain mildly symptomatic or completely asymptomatic for many years and the diagnosis be quite incidental [4]. The most common clinical features include exertional dyspnoea, recurrent pulmonary…”