Pulmonary Hypertension Program
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Pulmonary Hypertension Program
Froedtert & The Medical College of Wisconsin are home to the largest academic Pulmonary Hypertension Program in the state. Our exceptional, board-certified physicians and other team members are dedicated to improving the quality of life for individuals affected by pulmonary hypertension in all of its forms.
What is Pulmonary Hypertension?
| Pulmonary Hypertension (PH) is high blood pressure in the blood vessels of the lungs. It occurs when the small blood vessels that go through the lungs become thicker, constrict or become plugged. This, in turn, leads to increased pressure in those vessels making the right side of the heart work harder to pump blood through the lungs. If the high pressure remains, the heart will become enlarged and weaker, pumping blood less efficiently into the lungs and throughout the body. Patients then develop progressive fatigue and shortness of breath, two typical symptoms of PH. |
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For more information about our program or to request an appointment, please contact us via our online form or call us at 414-805-3666 or 800-272-3666,
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PH has been viewed by many to be a chronic condition that is incurable. This has often led to a late diagnosis with patients being referred to PH specialists in advanced stages of the disease. Fortunately, recent treatment discoveries and continued research are giving specialists the tools to make patients feel better and live longer. This has encouraged primary physicians to look for this disease in earlier stages and to promptly refer patients to specialized centers.
Given the complexity of some of the drugs and how they are given, it is very important that patients are evaluated at a pulmonary hypertension center by physicians who can diagnose and treat PH with the full range of treatment options.
What are the symptoms of pulmonary hypertension? Early in the disease, symptoms may be nonspecific and can mimic symptoms of other medical conditions. It is also possible that patients may experience only limited symptoms.
Symptoms include:
- Progressive shortness of breath (also called dyspnea)
- Fatigue or tiredness in association to shortness of breath
- Chest pain or pressure that typically occurs during activity (angina)
- Dizzy spells that typically occur during activity or exercise
- Fainting
- Leg swelling (edema)
- Rapid pulse rate (tachycardia)
- Heart throbbing (palpitations)
Most causes of shortness of breath are usually benign and do not require an intensive diagnostic workup or specialized treatments. However, whenever a patient experiences progressive shortness of breath — typically worsening in a matter of weeks or months — that is not relieved by conventional treatments, other less common causes of shortness of breath should be investigated. In this case scenario, pulmonary hypertension is one of the causes that should be ruled out. Usually, a thorough workup by a pulmonary hypertension specialist is needed to confirm if a patient has pulmonary hypertension.
Based on the physician’s clinical suspicion, an echocardiogram of the heart may be ordered. This test obtains pictures of the heart and can estimate the blood pressure inside of the blood vessels that go through the lungs. The results of this test (and possibly a few others) may prompt a referral to a facility with experience in diagnosing and treating this disease. It is important to know that an echocardiogram is mainly used as a screening test when PH is suspected; it does not confirm the diagnosis (see heart catheterization).
What causes pulmonary hypertension?More than 20 causes of pulmonary hypertension have been established. PH can affect a person of any age, race and ethnic background. It is more common in women than in men.
Some of the most common causes of pulmonary hypertension include:
- Idiopathic pulmonary hypertension (also known as “primary” pulmonary hypertension), a type of PH that cannot be linked to other causes once an extensive workup is completed. It typically occurs in individuals who were otherwise healthy before PH developed.
- Familial pulmonary hypertension, a diagnosis given to patients with idiopathic (“primary”) PH who have siblings with the same diagnosis. About one in 20 patients with idiopathic PH will have one or more relatives with the disease.
- PH associated to collagen vascular disease, a group of diseases in which the patient’s immune system becomes “overactive” leading to inflammation in different organs of the body including the lungs (i.e., lung blood vessels). Some types of collagen vascular disease include lupus, scleroderma and rheumatoid arthritis
- PH associated to chronic lung disease. People who have chronically inflamed or “damaged” lungs from conditions such as emphysema or lung fibrosis (scarring) may be at a higher risk of developing PH.
- PH associated to sleep apnea and/or low oxygen levels. Sleep apnea is a condition in which there is narrowing or complete blockage of the windpipe (airway) during sleep. Patients with significant sleep apnea or who experience low oxygen levels at night carry a higher risk of developing PH.
- PH associated to chronic liver disease. The mechanisms that lead to PH are more complex here, but it is well known that a small percentage of patients with cirrhosis, regardless of its cause, will develop PH. Patients with cirrhosis who need of a liver transplant routinely undergo a diagnostic workup to exclude the presence of PH before a transplant is considered.
- PH associated to thromboembolic disease. Patients who have had blood clots in their veins (most commonly in leg veins) may develop PH after a number of those clots migrate to the lung blood vessels. A large percentage of patients in this category do not recall having had any symptoms that would have suggested the presence of blood clots in their veins.
- PH secondary to disease in the left side of the heart typically occurs in patients who have impaired contractility or impaired relaxation of the left heart muscle (also called diastolic dysfunction). Older patients and especially those with a history of coronary artery disease, systemic hypertension, diabetes and sleep apnea are at a higher risk of developing a “stiff” left heart muscle that leads to impaired relaxation and elevation in left heart pressures. Increased pressure in the left side of the heart will, in turn, back up into the lung blood vessels, leading to PH.
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