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Primary and Secondary Pulmonary Hypertension

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(608) 263-7203
(608) 263-5215
 
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Pulmonary hypertension (PH) is currently thought to be a disease of the blood vessels within the lung (the pulmonary arteries). In pulmonary hypertension, pressure in the pulmonary arteries becomes abnormally high. As the blood pressure increases, the arteries narrow, forcing the heart to work harder to pump blood to the lungs.
 
It is a rare but serious condition that, left untreated, can lead to:
  • Swelling of the legs
  • Blood clots
  • Difficulty breathing
  • Heart failure

UW Health's multidisciplinary pulmonary hypertension program combines the expertise of pulmonologists, cardiologists, radiologists, and surgeons. Using comprehensive evaluation, diagnosis, and treatment strategies, our team works together to provide effective and individualized care for patients with this complex disease.

 

Definition and Causes

 

In pulmonary hypertension (PH), pressure in the blood vessels that supply the lungs (the pulmonary arteries) becomes abnormally high. Normally, blood pressure in the lungs is one-sixth that of the rest of the body.

 

Pulmonary hypertension can develop without a known cause (primary pulmonary hypertension). It can also develop as a result of other diseases (secondary pulmonary hypertension). Secondary pulmonary hypertension is often associated with:

  • Emphysema and other forms of chronic obstructive pulmonary disease (COPD)
  • Collagen vascular diseases, such as scleroderma, CREST syndrome, and lupus
  • Congenital heart disorders, such as ventricular and atrial septal defects
  • Blood clots in the lungs and pulmonary arteries
  • HIV
  • Liver diseases
  • Diet drugs, such as fenfluramine and dexfenfluramine.

It is not known exactly how many people have pulmonary hypertension. According to the American Lung Association, there are an estimated 300 new cases of primary pulmonary hypertension each year. In 2000, there were 163,000 hospital discharges in which primary pulmonary hypertension was one of the diagnoses.

The disorder can affect anyone at any age; however, it is more common among women between the ages of 21 and 40.

 

Symptoms

 
Pulmonary hypertension can develop without a known cause (primary pulmonary hypertension), or can develop as a result of other heart or lung diseases.
 
As the disease progresses, the pulmonary arteries become thicker and narrower. The resulting increase in pressure forces the heart to work harder to pump blood to the lungs. This causes the heart muscle to enlarge and become weaker, which can lead to congestive heart failure.

Symptoms of pulmonary hypertension include:
  • Shortness of breath
  • Fatigue
  • Chest pain
  • Dizziness or fainting

Severe pulmonary hypertension can lead to heart failure. Recently, there have been major advances in treating primary pulmonary hypertension with oral medications. This opens new possibilities for improving symptoms and prolonging life for patients with the disorder. However, at this time, there is no known cure for pulmonary hypertension.

 

Diagnosis

 

Pulmonary hypertension can be difficult to diagnose, as its symptoms often resemble other heart and lung disorders. In fact, many people with PH do not know they have it until the disease has become more advanced.

To make an accurate diagnosis of pulmonary hypertension, and rule out other heart and lung diseases, our physicians may perform laboratory blood tests and/or any of the following tests:

The results of these tests will help our doctors determine whether you have pulmonary hypertension, what its cause may be, and what course of treatment is best for your individual needs.

 

Treatments

 

Although there is no cure for pulmonary hypertension, there are many treatments that can improve the quality of life for people with the disorder:

  • Pulmonary hypertension is first treated with medications. These medications, used either along or in combination with one another, may help:
    • Lower blood pressure
    • Thin the blood
    • Open up constricted blood vessels
    • Help the heart pump blood more effectively
    • And reduce fluid throughout the body
     
  • Some patients may also require supplemental oxygen to help them breathe
     
  • Patients with severe PH who do not respond to medical treatment may require a double-lung transplant. During a Lung Transplant, the diseased lungs are replaced with donor lungs.