Pulmonary valve stenosis

Definition

Pulmonary valve stenosis is a congenital heart defect in which blood flow from the heart to the pulmonary artery is blocked.

Description

Pulmonary valve stenosis is an obstruction in the pulmonary valve, located between the right ventricle and the pulmonary artery. Normally, the pulmonary valve opens to let blood flow from the right ventricle to the lungs. When the pulmonary valve is malformed, it forces the right ventricle to pump harder to overcome the obstruction. In its most severe form, pulmonary valve stenosis can be life-threatening.

Patients with pulmonary valve stenosis are at increased risk for getting valve infections and must take antiobiotics to help prevent this before certain dental and surgical procedures. Pulmonary valve stenosis is also called pulmonary stenosis.

Causes & symptoms

Pulmonary valve stenosis is caused by a congenital malformation in which the pulmonary valve does not open properly. In most cases, scientists don't know why it occurs. In cases of mild or moderate stenosis, there are often no symptoms. With more severe obstruction, symptoms include a bluish skin tint and signs of heart failure.

Diagnosis

Diagnosis of pulmonary valve stenosis begins with the patient's medical history and a physical exam. Tests to confirm the diagnosis include chest x ray, echocardiogram, electrocardiogram, and catherization. An electrocardiograph shows the heart's activity. Electrodes covered with conducting jelly are placed on the patient. The electrodes send impulses that are traced on a recorder. Echocardiography uses sound waves to create an image of the heart's chambers and valves. The technician applies gel to a wand (transducer) and presses it against the patient's chest. The returning sound waves are converted into an image displayed on a monitor. Catherization is an invasive procedure used to diagnose, and in some cases treat, heart problems. A thin tube, called a catheter, is inserted into a blood vessel and threaded up into the heart, enabling physicians to see and sometimes correct the problems.

Treatment

Patients with mild to moderate pulmonary valve stenosis, and few or no symptoms, do not require treatment. In more severe cases, the blocked valve will be opened surgically, either through balloon valvuloplasty or surgical valvulotomy. For initial treatment, balloon valvuloplasty is the procedure of choice. This is a catherization procedure in which a special catheter containing a deflated balloon is inserted in a blood vessel and threaded up into the heart. The catheter is positioned in the narrowed heart valve and the balloon is inflated to stretch the valve open.

In some cases, surgical valvulotomy may be necessary. This is open heart surgery performed with a heart-lung machine. The valve is opened with an incision and in some cases, hypertrophied muscle in the right ventricle is removed. Rarely does the pulmonary valve need to be replaced.

Alternative treatment

Pulmonary valve stenosis can be life threatening and always requires a physician's care. In mild to moderate cases of pulmonary valve stenosis, general lifestyle changes, including dietary modifications, exercise, and stress reduction, can contribute to maintaining optimal wellness.

Prognosis

Patients with the most severe form of pulmonary valve stenosis may die in infancy. The prognosis for children with more severe stenosis who undergo balloon valvuloplasty or surgical valvulotomy is favorable. Patients with mild to moderate pulmonary stenosis can lead a normal life, but they require regular medical care.

Prevention

Pulmonary valve stenosis cannot be prevented.

Key Terms

Congenital

Present at birth.

Pulmonary

Relating to the opening leading from the right large chamber of the heart into the lung artery.

Stenosis

A narrowing or constriction, in this case of various heart valves. Stenosis reduces or cuts off the flow of blood.

Valve

Tissue between the heart's upper and lower chambers that controls blood flow.

Further Reading

For Your Information

Books

• DeBakey, Michael E., and Antonio M. Gotto, Jr. "Congenital Abnormalities of the Heart." In The New Living Heart. Holbrook, MA: Adams Media Corporation, 1997.
• Giuliani, Emilio R., et al., eds. "Pulmonary Stenosis with Intact Ventricular Septum." In Mayo Clinic Practice of Cardiology. 3rd ed. St. Louis: Mosby, 1996.
• Tierney, Lawrence M., Stephen J. McPhee, and Maxine A. Papadakis, eds. "Congenital Heart Disease." In Current Medical Diagnosis & Treatment. Stamford, CT: Appleton & Lange, 1997.

  Periodicals

• O'Laughlin, M.P. "Catherization Treatment of Stenosis and Hypoplasia of Pulmonary Arteries." Pediatric Cardiology 19 (1998): 48-56.
• Rome, J.J. "Balloon Pulmonary Valvuloplasty." Pediatric Cardiology 19 (1998): 18-24.

  Organizations

• American Heart Association. National Center. 7272 Greenville Avenue, Dallas, TX 75231-4596. (214) 373-6300. http://www.amhrt.org/.
• Congenital Heart Anomalies Support, Education & Resources, Inc. 2112 North Wilkins Road, Swanton, OH 43558. (419) 825-5575. http://www.csun.edu/~hfmthoo6/chaser/.
• Congenital Heart Disease Information and Resources. 1561 Clark Drive, Yardley, PA 19067. http://www.tchin.org/.
• Texas Heart Institute Heart Information Service. P.O. Box 20345, Houston, TX 77225-0345. (800) 292-2221. http://www.tmc.edu/thi/his.html.

Gale Encyclopedia of Medicine. Gale Research, 1999.