Absence of the pulmonary artery

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Absence of the pulmonary artery

Synonyms: Aplasia of pulmonary artery | UAPA | Unilateral Pulmonary Artery Absence | Unilateral pulmonary artery agenesis

A rare vascular anomaly characterized by congenital absence of the right or left pulmonary artery usually ending within 2 cm of its expected origin from the pulmonary trunk. Patients often also have other cardiovascular abnormalities and respective symptoms and are then typically diagnosed in infancy or childhood while isolated cases generally present with a mild clinical course and may go undiagnosed until adulthood. Presenting clinical features in isolated cases include hemoptysis exertional dyspnea and recurrent respiratory infections. The condition is typically accompanied by marked changes of lung tissue and may if unrecognized result in massive hemoptysis and pulmonary hypertension.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version December 2023

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Advocacy Organizations

Collaborative Research Advocacy for Vascular Anomalies Network (CaRAVAN)

As the research-based umbrella organization for all vascular anomalies, we are on a mission to develop a unified network of patients and their families, advocates, researchers, and clinicians who influence research from initial concept and design to patient care delivery

Project FAVA

Project FAVA is a 501(c)(3) non-profit patient advocacy group that promotes awareness of fibro-adipose vascular anomalies, educates patients, their families, and the global community, and provides helpful resources to those with FAVA. Project FAVA also advances research to move the needle towards more effective and less invasive treatment options for FAVA patients.

Clinical Trials

For a list of clinical trials in this disease area, please click here.