Search Encyclopedia:    
List of Topics Print This Page
 

Absent pulmonary valve


Alternative Names:

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

Treatment:

Infants who have breathing symptoms typically need immediate surgery. Infants without severe symptoms typically have surgery within the first 3 to 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).

Outlook (Prognosis):

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

In many cases, surgery can treat the condition and relieve symptoms.

Possible Complications:
  • Brain infection (abscess)
  • Lung collapse (atelectasis)
  • Pneumonia
  • Right-sided heart failure
  • Stroke
When to Contact a Medical Professional:

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.

Prevention:

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

References:

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.

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.

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.

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


Review Date: 2/17/2014
Reviewed By: Kurt R. Schumacher, MD, Pediatric Cardiology, University of Michigan Congenital Heart Center, Ann Arbor, MI. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2002 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

   View History
  Absent pulmonary valve

Related Links
Request an Appointment Online or call
1-800-789-PENN (7366)
   
   

 

About UPHS   Contact Us   Site Map   Privacy Statement   Legal Disclaimer   Terms of Use

The University of Pennsylvania Health System, Philadelphia, PA 1-800-789-PENN © 2014, The Trustees of the University of Pennsylvania