Pulmonary Valve Atresia

In pulmonary valve disease, the pulmonary valve may not be closing properly (regurgitation), may be narrowed (stenosis), or may be missing (atresia). Pulmonary atresia is a type of congenital heart defect (present at birth) that's normally diagnosed soon after birth. In pulmonary atresia, the valve that lets blood out of the heart to go to your or your baby's lungs (pulmonary valve) doesn't form correctly. Instead of opening and closing to allow blood to travel from the heart to the lungs, a solid sheet of tissue forms. So blood can't travel by its normal route to pick up oxygen from the lungs. If not addressed immediately, life-threatening consequences can occur.

Causes of Pulmonary Valve Atresia

Pulmonary valve atresia is typically caused by a heart defect present at birth (congenital). Other causes include:

  • A parent who has a congenital heart defect
  • A mother who is obese before getting pregnant
  • Smoking before or during pregnancy
  • A mother who has poorly controlled diabetes
  • Use of some types of medications during pregnancy, such as certain acne drugs and blood pressure medications

Symptoms of Pulmonary Valve Atresia

If your baby is born with pulmonary atresia, symptoms will be noticeable soon after birth. Your baby's signs and symptoms may include:

  • A bluish discoloration of the lips and skin caused by low oxygen (cyanosis)
  • Fast breathing or shortness of breath
  • Easily tiring or being fatigued
  • Feeding problems

Diagnosis of Pulmonary Valve Atresia

To diagnose this condition, your doctor will perform a variety of tests, including but not limited to:

Diagnostic tests and procedures

Treatment of Pulmonary Valve Atresia

Your baby will need urgent medical attention once pulmonary atresia symptoms develop. The choice of surgeries or procedures will depend on the severity of your child's condition. Some treatment options include:

Medications

  • An intravenous drug called prostaglandin will prevent closure of the natural connection (ductus arteriosus) between the pulmonary artery and the aorta.

Medical and surgical procedures (depends on type of pulmonary atresia)



Newark Beth Israel Medical Center
201 Lyons Avenue at Osborne Terrace
Newark, NJ 07112
(973) 926-7000
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Children's Hospital of New Jersey at Newark Beth Israel Medical Center
201 Lyons Avenue at Osborne Terrace
Newark, NJ 07112
(973) 926-7000
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Pulmonary valve atresia Treatment & Care

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