This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
Unilateral absence of the pulmonary
artery (UAPA) is a heart defect that is present from birth. The pulmonary artery
takes blood from the heart to the lungs. In the absence of a pulmonary
artery, other blood vessels compensate by supplying blood to the
lungs. Pressure can build inside these vessels and lead to heart and lung
complications. UAPA can be left sided or
right sided. It most often occurs alone, but can occur with other heart
anomalies and birth defects. Signs and symptoms include repeat lung
infections, lower exercise tolerance, shortness of breath, chest pain, and
fluid in the lungs. Risk for these symptoms increase with age.
Complications of UAPA include, hemoptysis (coughing up blood), respiratory hemorrhage,
and high blood pressure in the arteries to the lung (pulmonary
hypertension). Serious complications may be triggered by stress on
the body, such as pregnancy and altitude sickness.
For more information, visit GARD.