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Unilateral absence of a pulmonary artery

Other Names for this Disease
  • Congenital absence of the pulmonary artery
  • Isolated UAPA
  • Isolated unilateral absence of a pulmonary artery
  • Pulmonary artery, isolated unilateral absence of
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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.[1][2][3][4]

Last updated: 2/26/2014


  1. Mazurek B, Szydlowski L, Mazurek M, Kohut J, Glowacki J. Rare cardiovascular anomaly: congenital unilateral absence of the pulmonary artery. Arch Med Sci. 2013 Dec 30; 9(6):1162-4. Accessed 2/26/2014.
  2. El-Hattab AY, Calcaterra D, Parekh KR, Rossi NP, Davis JE, Turek JW. Semiautologous repair for congenital discontinuous right pulmonary artery. 2013 Dec; 96(6):2230-1. Accessed 2/26/2014.
  3. Ten Harkel AD, Blom NA, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest. 2002 Oct; 122(4):1471-7. Accessed 2/26/2014.
  4. Kruzliak P, Syamasundar RP, Novak M, Pechanova O, Kovacova G. Unilateral absence of pulmonary artery: pathophysiology, symptoms, diagnosis and current treatment. Arch Cardiovasc Dis. 2013 Aug-Sep; 106(8-9):448-54. Accessed 2/26/2014.
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