Pulmonary hypertension (PH) occurs when the pressure in the blood vessels leading from the heart to the lungs is abnormally high. When PH strikes, it forces the heart to work harder. If left untreated, PH can lead to heart failure.

“PH is not curable and was once considered rapidly fatal. But today, many patients enjoy longer, healthier lives thanks to the availability of medical therapies,” S. Clark Berngard, MD, of Boulder Valley Pulmonology told a crowd of more than 100 people during a free health lecture held in Lafayette, CO.

“However, proper treatment is essential and early, accurate diagnosis is key.”

Signs Can Be Mistaken for Asthma, COPD or a Heart Condition
According to Dr. Berngard, early in the disease the symptoms are subtle. “Many people have PH for a long time before they realize something is wrong,” he said.

But once symptoms of PH develop, they can look like other conditions, causing them to be confused with other conditions such as asthma, COPD or a heart problem.

Dr. Berngard said the signs and symptoms of PH include:

  • Shortness of breath
  • Fatigue
  • Decreased exercise tolerance
  • Leg swelling
  • Lightheadedness
  • Abnormal heart sounds
  • Low oxygen levels

Determining the Exact Cause is Key
“There are numerous causes for PH, which can be familial or acquired as a result of other medical conditions such as heart disease, lung disease or connective tissue disease. In many cases of PH, the cause is unknown,” Dr. Berngard said. Pinpointing the underlying cause of PH is essential for the best treatment and prognosis.

PH is classified by the World Health Organization according to its causes or associated underlying conditions:

  • Group 1: Pulmonary arterial hypertension (PAH)
    PH caused by "PAH refers to a specific type of pulmonary hypertension in which a disease process directly causes the narrowing and obstruction of the arteries in the lungs, resulting in increased pressure,” Dr. Berngard explained. “PAH can be a consequence of autoimmune diseases, drug use, HIV, congenital heart disease and genetic disorders. But many times the exact cause of PAH is unknown or idiopathic.”
  • Group 2: Pulmonary venous hypertension
    PH caused by left-sided heart diseases such as congestive heart failure, diastolic dysfunction or failure and valvular heart disease.
  • Group 3: Pulmonary hypertension due to chronic lung disease
    PH caused by lung problems such as chronic obstructive pulmonary disease (COPD), asthma, chronic hypoxemia, sleep apnea, interstitial lung disease and chronic exposure to high altitudes.
  • Group 4: Chronic thromboembolic pulmonary hypertension (CTEPH)
    PH caused by pulmonary embolism (a blood clot that travels to a lung), which develops into scar tissue and blocks normal blood flow.
  • Group 5: Unclear, multifactorial mechanisms
    PH with miscellaneous causes that don’t fit into the other four classes.

How is PH Diagnosed?
Doctors may order several tests to diagnose PH and determine its cause. According to Dr. Berngard, tests for PH may include:

  • Echocardiogram - This test uses an ultrasound transducer to create sound waves that produce an image for uncovering any damage or enlargement of the heart.
  • Lung function testing and overnight oximetry - These are diagnostic tests that measure the ability to move air into and out of your lungs. You might also be sent home with a device that clips on your finger (oximeter) to wear while sleeping. The oximeter records the oxygen levels in your blood and pulse rate during hours of sleep, helping to detect sleep apnea.
  • Ventilation perfusion scan – This is a type of nuclear radiology test that assesses the function of the lungs. A small amount of a radioactive substance is used to help find changes in the arteries leading to the lungs and blood flow within the lungs.
  • Right heart catheterization, the gold standard for diagnosing PH - A long, thin and flexible tube, called a catheter, is passed through a vein and into the heart. It allows your doctor to directly measure the pressures in the main pulmonary arteries and heart’s right ventricle and to assess how well blood is flowing through the heart.

Treatment for PH
“Given there are so many causes for PH, treatments can vary dramatically. That’s why it’s
important to accurately diagnose and determine the type of pulmonary hypertension in order to correctly treat the condition, minimize side effects and improve quality of life.” Dr. Berngard summarized. “Luckily, there are many available treatment options and more on the horizon.”

Talk to your doctor if you’re having shortness of breath, fatigue, lightheadedness or swelling in your legs.

To make an appointment with S. Clark Berngard, MD, or if you have further questions about pulmonary hypertension, call 303-835-9260.

View PowerPoint slides from Dr. Berngard’s lecture on pulmonary hypertension.