Idiopathic pulmonary artery dilatation

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Idiopathic pulmonary artery dilatation

Idiopathic pulmonary artery dilatation is a rare developmental defect during embryogenesis characterized by the dilatation of the main pulmonary artery with or without dilatation of the right and left pulmonary artery branches and not attributed to any other cardiac pulmonary and/or arterial wall disease. It may present with exertional dyspnea fatigue cough hemoptysis palpitation and chest pain but may also be asymptomatic. In serious cases trachea constriction due to postural changes may lead to attacks of cyanosis with severe dyspnea. Sudden cardiac death has been reported in some cases.

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: // Data version September 2023.

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Idiopathic pulmonary artery dilatation?

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Advocacy Organizations

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.

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

Clinical Trials

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