Definition

Pulmonary valve stenosis is a condition in which a deformity on or near the pulmonary valve slows blood flow.  The pulmonary valve helps direct the blood flow from the heart to the lungs and stenosis (or narrowing), occurs when the valves are unable to open wide enough. As a result, blood flow to the lungs is diminished.

In most cases, pulmonary valve stenosis is a complication of another illness; however, it is more frequent before birth as a congenital heart defect.

Treatment options for  pulmonary stenosis typically involve surgery. Most individuals who undergo surgery can expect positive end results with no further complications or recurrences.

 

Symptoms

Pulmonary valve stenosis signs and symptoms vary depending on an individual’s overall health and the extent of obstruction within the body.

Pulmonary valve stenosis signs and symptoms may include:

  • Chest pain
  • Tiredness
  • Fainting
  • Shortness of breath
  • Heart murmur
  • Bluish color of the skin (cyanosis)
  • Sudden death

 

Causes

In most cases, pulmonary valve stenosis is present at birth. If the pulmonary valve doesn't develop properly during fetal development, it can restrict blood flow and the infant may end up developing heart abnormities.  

Sometimes other medical conditions can cause pulmonary valve stenosis, such as:

  • Carcinoid syndrome: A set of symptoms caused by carcinoid tumors, which grow  out of the cells that are part of the endocrine system. They release a chemical called serotonin, which can cause symptoms of a carcinoid tumor (flushing of the skin, diarrhea, stomach cramps).
  • Rheumatic fever:  A disease that can result from complications of improper treatment of strep throat or scarlet fever.

 

Risk factors

Certain conditions that can increase the risk of developing pulmonary valve stenosis later in life include the following:

  • Carcinoid syndrome
  • Rheumatic fever
  • Noonan's syndrome

 

Complications

Complications that can arise from severe cases of pulmonary stenosis may include:

  • Infectious endocarditis: Bacterial infections in the inner lining of the heart
  • Right ventricular hypertrophy: A form of ventricular hypertrophy that affects the right ventricle, causing it to pump blood harder and direct it toward the pulmonary artery. This pressure in turn causes the muscular wall of the ventricle to harden and the chamber within the ventricle to enlarge. Eventually, the heart becomes stiff and may weaken.
  • Arrhythmia: irregular heart rhythm (beating of the heart is either too fast or too slow).
  • Heart failure:  A condition in which the heart doesn’t pump blood as well as it should.

 

Treatment

Depending on the degree of obstruction, treatment options for severe pulmonary stenosis typically involve either balloon valvuloplasty or open-heart surgery.

  • Balloon valvuloplasty: A procedure that involves widening of a heart valve that is narrowed. By insertion of a small tube through a vein in the leg to the heart, an uninflated balloon is placed through the opening of the narrowed pulmonary valve. By inflating the balloon, the narrowed valve widens and blood flow is increased. Possible side effects may include bleeding, blood clots or infection.
  • Open-heart surgery: A surgical procedure that involves repairing the  pulmonary artery or valve or replacing the original valve with an artificial one. Possible side effects may include bleeding, blood clots or infection.