Pulmonary atresia

  • Definition
    • Pulmonary atresia is a form of heart disease in which the pulmonary valve does not form properly. It is present from birth (congenital heart disease). The pulmonary valve is an opening on the right side of the heart that regulates blood flow from the right ventricle (right side pumping chamber) to the lungs.

      In pulmonary atresia, a solid sheet of tissue forms where the valve opening should be, and the valve stays closed. Because of this defect, blood from the right side of the heart cannot go to the lungs to pick up oxygen.

  • Alternative Names
    • Pulmonary atresia - intact ventricular septum; PA/IVS; Congenital heart disease - pulmonary atresia; Cyanotic heart disease - pulmonary atresia; Valve - disorder pulmonary atresia

  • Causes
    • As with most congenital heart diseases, there is no known cause of pulmonary atresia. The condition is linked with another type of congenital heart defect called a patent ductus arteriosus (PDA).

      Pulmonary atresia may occur with or without a ventricular septal defect (VSD).

      • If the person does not have a VSD, the condition is called pulmonary atresia with intact ventricular septum (PA/IVS).
      • If the person has both problems, the condition is called pulmonary atresia with VSD. This is an extreme form of tetralogy of Fallot.

      Although both conditions are called pulmonary atresia, they are actually different defects. This article discusses pulmonary atresia without a VSD.

      Persons with pulmonary atresia with intact ventricular septum may also have a poorly developed tricuspid valve. They may also have an underdeveloped right ventricle and abnormal blood vessels feeding the heart.

  • Symptoms
    • Symptoms usually occur in the first few hours of life, although it may take up to a few days.

      Symptoms may include:

      • Bluish colored skin (cyanosis)
      • Fast breathing
      • Fatigue
      • Poor eating habits (babies may get tired while nursing or sweat during feedings)
      • Shortness of breath
  • Exams and Tests
  • Treatment
    • A medicine called prostaglandin E1 is usually used to help the blood move (circulate) into the lungs. This medicine keeps a blood vessel open between the pulmonary artery and aorta. The vessel is called a PDA.

      Multiple treatments are possible, but depend on the extent of the heart abnormalities that accompany the pulmonary valve defect. Potential treatments include:

      • A thin, flexible tube (heart catheterization) to repair the problem
      • Open heart surgery to repair or replace the valve, or to place a tube between the right ventricle and the pulmonary (lung) arteries
      • Reconstructing the heart as a single ventricle (1 pumping chamber instead of 2)
      • Heart transplant
  • Outlook (Prognosis)
    • Most cases can be helped with surgery. How well a baby does depends on:

      • Size and connections of the pulmonary artery (the artery that takes blood to the lungs)
      • How well the heart is beating
      • How well the other heart valves are formed or how much they are leaking

      Outcome varies because of the different forms of this defect. A baby may need only a single procedure or could need 3 or more surgeries and have only a single working ventricle.

  • Possible Complications
    • Complications may include:

      • Delayed growth and development
      • Seizures
      • Stroke
      • Infectious endocarditis
      • Heart failure
      • Death
  • When to Contact a Medical Professional
    • Call your provider if the baby has:

      • Problems breathing
      • Skin or nails that appear blue (cyanosis)
  • Prevention
    • There is no known way to prevent this condition.

      All pregnant women should get routine prenatal care. Many congenital defects can be found on routine ultrasound exams.

      If the defect is found before birth, medical specialists (such as a pediatric cardiologist, cardiothoracic surgeon, and neonatologist) can be present at the birth, and ready to help as needed. This preparation can mean the difference between life and death for some babies.

  • References
    • Fraser CD, Carberry KE. Congenital heart disease. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 19th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 59.

      Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In: Mann DL, Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 10th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 62.