Get answers to your Pediatric Surgery questions.
Absent pulmonary valve syndrome; Congenital absence of the pulmonary valve; Pulmonary valve agenesis
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 heart defects the infant has, surgery may involve:
Types of surgery for absent pulmonary valve include:
Infants with severe breathing symptoms may need to get oxygen or be put on a breathing machine.
Without surgery, most infants who have severe lung complications will die.
Surgery can treat the condition and relieve symptoms in many cases.
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.
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.
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