Absent pulmonary valve syndrome; Congenital absence of the pulmonary valve; Pulmonary valve agenesis

Symptoms can vary depending on which other defects the infant has, but may include:

• Blue coloring to the skin (cyanosis)
• Coughing
• Failure to thrive
• Poor appetite
• Rapid breathing
• Respiratory failure
• Wheezing

Infants who have breathing symptoms should have surgery as quickly as possible. Even infants without symptoms should have surgery within the first 3-6 months of life.

Depending on the type of other heart defects the infant has, surgery may involve:

• Closing the hole in the wall between the left and right ventricles of the heart (ventricular septal defect)
• Closing a blood vessel that connects the aorta of the heart to the pulmonary artery (ductus arteriosis)
• Enlarging the flow from the right ventricle to the lungs

Types of surgery for absent pulmonary valve include:

• Moving the pulmonary artery to the front of the aorta and away from the airways
• Rebuilding the artery wall in the lungs to reduce pressure on the airways (reduction pulmonary arterioplasty)
• Rebuilding the windpipe and breathing tubes to the lungs
• Replacing the abnormal pulmonary valve with one taken from human or animal tissue

Infants with severe breathing symptoms may need to get oxygen or be put on a breathing machine (ventilator).

Without surgery, most infants who have severe lung complications will die.

Surgery can treat the condition and relieve symptoms in many cases.

• Brain infection (abscess)
• Lung collapse (atelectasis)
• Pneumonia
• Right-sided heart failure
• Stroke

Call your health care provider if your infant has symptoms of absent pulmonary valve. If you have a family history of heart defects, talk to your doctor before or during pregnancy.

Although there is no way to prevent this condition, families may be evaluated to determine their risk of congenital defects.

Park, MK. Park: Pediatric Cardiology for Practitioners. 5th ed. Philadelphia, Pa: Mosby; 2008.

Nölke L, Azakie A, Anagnostopoulos PV, Alphonso N, Karl TR. The Lecompte maneuver for relief of airway compression in absent pulmonary valve syndrome. Ann Thorac Surg, 2006;81:1802-1807.

Brown JW, Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW. Surgical treatment of absent pulmonary valve syndrome associated with bronchial obstruction. Ann Thoracic Surg, 2006;82:2221-2226.

Bernstein D. Cyanotic congenital heart lesions: Lesions associated with decreased pulmonary blood flow. In: Kliegman RM, Behrman Re, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 424.