Pulmonary Atresia

Pulmonary atresia is a rare birth defect of the pulmonary valve. Sometimes the pulmonary valve is missing completely; other times the valve is blocked.

Normally, the pulmonary valve acts like a door that allows blood to flow from the right ventricle through the pulmonary artery to the lungs to pick up oxygen.

The pulmonary valve is not formed in babies with pulmonary atresia, so there is no way for blood to go from the right ventricle to the pulmonary artery. Consequently, the central pulmonary artery or each of the right and left branches may be small and unusually arranged.

Babies with pulmonary atresia may get some blood to their lungs by a different way, such as a patent ductus arteriosus (PDA). Doctors may give medicine to keep the PDA open until a baby with pulmonary atresia goes to surgery. In addition, pulmonary atresia may occur with or without a ventricular septal defect (VSD). If a baby does not have a VSD, the condition is called pulmonary atresia with intact ventricular septum (PA/IVS). Babies with PA/IVS have a small right ventricle and may have coronary artery abnormalities.

Pulmonary atresia makes babies look blue (cyanotic) because not enough blood flow to the lungs.

The specialists at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine — the leading providers of pediatric heart care in Louisville and Southern Indiana — can help your child with pulmonary atresia.

The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute have the skills and experience to provide a precise diagnosis and develop a customized treatment plan for you and your child.

Norton Children’s has a network of outreach diagnostic and treatment services throughout Kentucky and Southern Indiana.

Pulmonary Atresia Symptoms

Most babies with pulmonary atresia show symptoms during the first few hours of life. However, in some babies, symptoms do not show up until a few days after birth.

Symptoms may include:

  • Bluish skin tone (cyanosis)
  • Clammy skin
  • Fast breathing
  • Poor feeding
  • Tiring easily while feeding
  • Working hard to breathe

Pulmonary atresia happens while the heart is forming, during the first eight weeks of fetal development. Some congenital heart defects have a genetic link, or family history. But, most of the time, this defect has no clear cause.

Diagnosing Pulmonary Atresia

The specialists at Norton Children’s Heart Institute can diagnose pulmonary atresia before the baby is born using a fetal echocardiogram (fetal echo). If your family has a history of congenital heart disease or a routine prenatal ultrasound shows a potential congenital heart condition, your doctor may have you get a fetal echo and refer you to a pediatric cardiologist.

The Norton Children’s Heart Institute has fetal cardiology specialists who perform fetal echos at six sites in Kentucky in the cities of: Ashland, Lexington, Louisville, Owensboro and Paducah.

After birth, if pulmonary atresia is suspected, your baby will have an exam and additional tests, which may include:

  • Chest X-ray: This shows pictures of the heart and lungs, and can show heart issues, extra blood flow or fluid in the lungs due to defects.
  • Echocardiogram (echo): This test uses sound waves (ultrasound) to produce images of the heart and blood vessels’ structures on a screen. It can show structure of the heart and ductus arteriosus and also the function of the heart. Norton Children’s Heart Institute has 28 tele-echo locations throughout Kentucky and Southern Indiana.
  • Electrocardiogram (ECG or EKG): This test checks the heart’s electrical activity to show damage or irregular rhythms.
  • Heart catheterization: This invasive procedure studies the structure, function, and provides direct pressure measurements of the heart.

Pulmonary Atresia Treatment

Initial treatment will focus on keeping the ductus arteriosus open with a medication called prostaglandin to keep blood flowing to the lungs. If the baby does not have a ventricular septal defect or a large atrial communication, a balloon atrial septostomy will be emergently performed. This will allow blood on the right side of the heart to get to the left side of the heart to be pumped out to the body.

Surgery will be needed to create a way to get blood flow to the lungs. Different options include surgically reconstructing the right ventricular outflow tract. This can occur by opening a connection from the right ventricle to the pulmonary artery with a valved tube called a conduit or patching the right ventricular outflow tract. Another option is a Blalock-Taussig shunt (BT shunt), a small tube that connect the pulmonary artery and the subclavian artery.

Subsequent surgeries will depend on a number of factors, including right ventricle size and other defects present. The specialists at Norton Children’s Heart Institute will look at all these factors and help you choose the best treatment option available for your infant.

Why Choose Norton Children’s Heart Institute

The Adult Congenital Heart Association has recognized Norton Children's Heart Institute for its expertise treating adult congenital heart disease.

  • Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
  • The American Board of Thoracic Surgery has certified our cardiothoracic surgeons in congenital heart surgery.
  • The Adult Congenital Heart Association has accredited Norton Children’s Heart Institute’s Adult Congenital Heart Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
  • More than 5,000 children a year visit Norton Children’s Heart Institute for advanced heart care.
  • Norton Children’s Heart Institute has offices across Kentucky and Southern Indiana to bring quality pediatric heart care closer to home.
  • The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) at Norton Children’s Hospital is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.
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