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Unilateral absence of a pulmonary artery


Other Names:
Isolated unilateral absence of a pulmonary artery; Isolated UAPA; Pulmonary artery, isolated unilateral absence of; Isolated unilateral absence of a pulmonary artery; Isolated UAPA; Pulmonary artery, isolated unilateral absence of; Congenital absence of the pulmonary artery See More

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
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
There is very limited data regarding long-term outcomes of treated infants with isolated UAPA. Most of the information available on this topic is individual case reports. These individual cases often report successful outcomes.[1][2][3][4] The estimated overall mortality rate across all age groups for people with UAPA and pulmonary hypertension is 7%. Infants with severe pulmonary hypertension tend to have a poorer outcome, than those whose symptoms develop later in life.[4]
Last updated: 2/26/2014

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  • My son was born with a heart defect. His right pulmonary artery isn't connected to his heart. His doctors want to do a surgery to reconnect it. Is this a very risky surgery? I can't find any information about this online. The doctors are telling me this isn't as bad as it could be, but I still am nervous. See answer


  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. http://www.ncbi.nlm.nih.gov/pubmed/24482666. 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. http://www.ncbi.nlm.nih.gov/pubmed/24296194. 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. http://www.ncbi.nlm.nih.gov/pubmed/12377882. 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. http://www.ncbi.nlm.nih.gov/pubmed/23938302. Accessed 2/26/2014.