Pulmonic Stenosis

What is Pulmonic Stenosis ?

Pulmonic stenosis (PS) is a congenital (present from birth) condition that results in obstruction of flow from the right ventricle out to the pulmonary artery. The most common form of pulmonic stenosis is valvular pulmonic stenosis, and is the most amenable to correction. Valvular PS is a congenital defect whereby the pulmonic valve leaflets open incompletely in the contraction phase (systole) of the cardiac cycle. Supravalvular PS is when there is a ring or ridge of issue above the pulmonic valve causing obstruction of outflow while subvalvular PS refers to obstruction below the pulmonic valve. The rest of this discussion will focus on valvular PS but the pathophysiology is the same for all three types.

Dogs with PS have partial fusion of the valve leaflets that causes an acceleration of blood flow as it crosses the valve, forcing the right ventricle to pump harder to push blood through the valve. The abnormal blood flow is what causes the heart murmur that was ausculted by your veterinarian. Because the outflow tract is abnormally narrowed, the pressure the right ventricle must overcome in order to pump blood into the pulmonary artery is elevated. This change in pressure is termed the pressure gradient and the degree of elevation of the pressure gradient is how PS is classified. Like any muscle, the right ventricular muscle thickens and enlarges because it has to work harder to overcome the increase in pressure.

Diagnosis

How is Pulmonic Stenosis Diagnosed?

An echocardiogram is the diagnostic of choice because it enables your cardiologist to visualize the heart’s structure and function, and also to determine the severity and type of abnormality present. In normal dogs, the pressure gradient across the pulmonic valve is less than 20mmHg. In dogs with mild PS, the pressure gradient is between 20 and 50mmHg. Dogs with moderate PS have a pressure gradient between 50 and 80mmHg and dogs with severe PS have a pressure gradient greater than 80mmHg.

Not only is an echocardiogram used to make the diagnosis of PS and to classify its severity, but it is also important to evaluate for concurrent defects. Many dogs with PS also have a patent foramen ovale (abnormal connection between the right and leH atria) as well as a malformed tricuspid valve. These findings can significantly eff­ect prognosis and treatment options.

Treatment

Dogs with mild PS have a good prognosis and generally live a full life without treatment.

Dogs with moderate PS also have a good prognosis, however a small percentage may go on to develop muscle failure late in life. In dogs with moderate PS that have concurrent tricuspid valve dysplasia or a patent foramen ovale, therapy should be considered. Dogs with severe PS are at risk for sudden death, fainting, and exercise intolerance as well as eventual muscle failure.

Therapeutic options for valvular pulmonic stenosis include balloon valvuloplasty (BV) as well as surgical valvulotomy. BV is the preferred treatment as it is minimally invasive and o­ers a good outcome. It is performed by a cardiologist in a catheterization laboratory. Surgical valvulotomy is performed by a surgeon and entails a thoracotomy (incision into the thoracic cavity). Supra or sub valvular obstructions are less amenable to balloon valvuloplasty. Surgery is indicated in cases where the obstruction is severe. Few institutions are able to provide this option.

With therapy, the prognosis for severe PS is good. Some dogs require lifelong therapy of a beta blocker while others can be maintained on no medication. Lifelong monitoring with echocardiograms is warranted in all cases of moderate severe PS.

Frequently Asked Questions

What causes Pulmonic Stenosis?

It is usually a genetic condition that develops before birth. Certain breeds are more commonly affected.

What are the signs to watch for?

  • ♦Heart murmur (often found on routine exam)
  • ♦Exercise intolerance
  • ♦Weakness or fatigue
  • ♦Fainting (syncope)
  • ♦In severe cases, fluid buildup or difficulty breathing

How is it diagnosed?

Diagnosis may include:

  • Physical exam (heart murmur)
  • Echocardiogram (heart ultrasound – most important test)
  • Chest X-rays and ECG in some cases

How is it treated?

Treatment depends on severity and may include:

  • Balloon valvuloplasty (a minimally invasive procedure to widen the valve)
  • Medications to manage symptoms or heart function
  • Activity modification in some cases
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How Do I Schedule?

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