Unilateral absence of a pulmonary artery

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Disease Overview

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.[7214][7215][7216][7217]


Synonyms

  • Isolated unilateral absence of a pulmonary artery
  • Isolated UAPA
  • Pulmonary artery, isolated unilateral absence of
  • Congenital absence of the pulmonary artery

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National Organization for Rare Disorders