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Genetic and Rare Diseases Information Center (GARD)

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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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How might unilateral absence of the pulmonary artery be treated?

Treatment options for unilateral absence of the pulmonary artery (UAPA), include observation (symptom-free, low risk cases), surgery to improve blood flow between the heart and lung (shunting or re-connecting existing arteries to the lung), surgery and/or medications to address lung complications, and embolization (blocking off) of dangerous blood vessels. Surgical approach is individually tailored based upon the person’s condition and anatomy. Early treatment (in infancy) can protect against late complications and improve lung development.[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.