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Absent pulmonary valve - Overview

Alternative Names

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

Definition of Absent pulmonary valve:

Absent pulmonary valve is a rare defect in which the pulmonary valve that carries oxygen-poor blood from the heart to the lungs is either missing or poorly formed. This condition is present at birth (congenital).

Causes, incidence, and risk factors:

Absent pulmonary valve occurs when the pulmonary valve doesn� ' t develop properly while the baby is in the mother� ' s womb. It often occurs as part of a rare condition called tetralogy of Fallot.

The pulmonary valve is either absent or poorly formed and the ring that holds the valve in place (annulus) is usually small. This leads to a blockage in the flow of blood across the valve, and valve leakage.

There is also usually a hole between the left and right ventricles of the heart (ventricular septal defect). One of the most important parts of this syndrome is very enlarged (dilated) branch pulmonary arteries, which press on the tubes that bring air to the lungs (bronchi).

Other heart defects that can occur with absent pulmonary valve include:

Abnormal tricuspid valve
Atrial septal defect
Double outlet right ventricle
Ductus arteriosis
Endocardial cushion defect
Marfans syndrome
Tricuspid atresia

Heart problems that occur with absent pulmonary valve may be due to defects of the genes (chromosomes).

Absent pulmonary valve

Reviewed last on: 3/7/2008
Mark A Fogel, MD, FACC, FAAP,Associate Professor of Pediatrics and Radiology, Director of Cardiac MR, The Children's Hospital of PhiladelphiaDivision of Cardiology, Philadelphia, PA.

References

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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