Congenital isolated unilateral agenesis of pulmonary arteries in adults: case series and review.

Abstract

Congenital, isolated unilateral agenesis of pulmonary arteries (UAPA) has a bimodal clinical presentation. It is either diagnosed in early infancy or during adulthood. Early diagnosis and timely surgical correction at infancy prevent the development of irreversible pulmonary hypertension and enable prolonged survival. Though surgical intervention is a conclusive method of treatment, its outcome depends upon the reversibility of pulmonary hypertension and the adequacy of the pulmonary artery dimension. Adults with UAPA can be asymptomatic (diagnosed incidentally) or symptomatic. They may present clinically with myriads of symptoms like exertional dyspnea, recurrent lung infection, hemoptysis, and atypical chest pain. For cases where definitive surgical treatment is not feasible, interventional and medical management should be carried out as part of palliative therapy. A retrospective analysis of 22 cases of UAPA in adults published in the literature from the years 2017-2020 are presented here, along with our institutional case.