About Pulmonary Hypertension
What is Pulmonary Hypertension?
Pulmonary Hypertension is high blood pressure in the lungs. It is often difficult to diagnose, mostly because many PH symptoms are the same symptoms of more common diseases. The causes of PH also vary, which makes it a more complex condition to treat.
In some people, for various reasons, there are changes in the pulmonary arteries that can cause PH. These changes are responsible for compromising the arteries, and restricting the normal flow of blood from your heart to your lungs through the right ventricle. When the heart cannot properly pump blood through the arteries and into the lungs, the pressure in the arteries rises. This makes the heart work harder and thus strains and weakens the right ventricle. If left untreated, PH can cause heart failure.
Different types of PH
There are still many mysteries to solve when it comes to PH, which is why the classification for various types of this condition is subject to change as scientists learn more about it. Our Program has extensive experience in treating patients with all types of PH in all categories of severity.
Types of PH:
- Primary pulmonary hypertension (PPH)—also known as Pulmonary Arterial Hypertension, PAH, PPH, IPAH, and pulmonary vascular disease
- Secondary PH
- Thromboembolic hypertension
- PH associated with congenital heart disease, liver disease, and collagen vascular diseases
Common PH Symptoms
While there are several varieties of PH, the symptoms are generally the same for all of them. There are a couple of reasons why PH is not the first condition a physician may think of when a patient asks about the following symptom: 1) PH is a rare disease; 2) the most common PH symptoms are common symptoms for many other more common conditions.
If one or more of the following symptoms are familiar to you, please contact your physician to talk about them.
- Chest pain
- Chronic fatigue
- Swollen ankles and legs
- Dry cough
- Raynaud’s phenomenon—tendency to get chilly blue fingers
PH can affect people of any age, race or ethnic background. While PH is considered a rare disease, there are some risk factors that should trigger an investigation into the possibility that a patient does or does not have this condition. The sooner a patient is diagnosed and can start treatment, the sooner treatment—and relief—can begin.
- Gender—women are 2.5 times more likely than men to get PH
- Obstructive sleep apnea
- Chronically high cardiac output
- Collagen vascular diseases, like scleroderma, CREST syndrome, rheumatoid arthritis, systemic lupus erythematous
- Congenital heart disease
- Liver disease
- Family history of PH
There are several tests your doctor may use to determine if PH is causing your symptoms (and to rule out the possibility of other conditions). While many of these tests are helpful in developing a big picture of what is going on in your body, currently the only test that offers a definitive diagnosis for Pulmonary Hypertension is the right-heart catheterization.
Common tests for PH
- Electrocardiogram (EKG)
- Blood tests
- Chest x-rays
- Doppler Echocardiogram
- Transesophageal echocardiography
- CAT scan
- V/Q scan
- Pulmonary function test
- Carbon monoxide diffusing capacity test
- Exercise tolerance test
- Cardiopulmonary exercise testing
- Right-heart catheterization
- Left-heart catheterization
- Pulmonary angiogram
- Vasodilator study
Treatment for PH
There is no cure for PH. However, thanks to many advances in the treatment of PH over the past decade, many more effective options are available to patients than ever before. Due to the complex nature of this disease, every patient’s treatment protocol must be tailored and carefully monitored on a regular basis.
We are dedicated to patients with PH. Our physicians and staff are highly trained and skilled in determining which medical therapies will work best for which patients, and to adjust them as needed. We ask lots of questions and we expect our patients and their doctors to do the same. We are also very involved in multiple investigational trials for new treatments, which means that our patients have access to the latest and best options.
Our treatments include:
- Several FDA-approved and investigational protocols
- High-dose calcium channel blocker therapy
- Inhaled nitric oxide
- Continuous intravenous infusion of epoprostenol or Flolan
- Subcutaneous version of prostacyclin and Uniprost (investigational)
- Pulmonary angiography with the additional use of intravascular ultrasound
- Pulmonary thromboendarterectomy
- Pulmonary artery angioplasty