A Young Woman with Hemoptysis as the main Manifestation of Isolated UAPA with Multiple Vascular Malformations.

Background: The unilateral absence of a pulmonary artery (UAPA) is a very rare congenital pulmonary vascular malformation. Since the first case was reported in 1868, the overall incidence of UAPA has been at a level of 1/300,000 - 1,200,000. Isolated UAPA without other cardiac anomalies is rare. Here we report a young woman who came to the clinic with hemoptysis as the main manifestation. Isolated UAPA with multiple vascular malformations was finally diagnosed by DSA angiography. The diagnosis and treatment experience and gains of UAPA are discussed in combination with the treatment process of this patient.

Methods: Appropriate laboratory tests, chest computed tomography (CT), Computed Tomography Pulmonary Angiogram (CTPA), and DSA angiography of the pulmonary arteries were used to explore the etiology.

Results: Chest CT showed that the bronchial tubes in the right lung segment and below were not obvious, and CTPA showed that the pulmonary arteries in the middle and lower lobes of the right lung were not visible, and the diagnosis of isolated unilateral pulmonary artery agenesis with multiple vascular malformations was clearly made by pulmonary artery DSA angiography.

Conclusions: In patients with unexplained hemoptysis, we should be alert to the possibility of vasculopathy, and angiographic techniques should be applied in a timely manner when plain CT fails to make a definitive diagnosis.