WHAT IS PULMONARY HYPERTENSION?

Pulmonary Hypertension (PH) is a rare, complex and chronic lung disease, which currently has no cure. PH, or high blood pressure in the lungs, makes it difficult for a patient to receive adequate oxygen and forces the heart to work harder, often resulting in heart failure and death.  It is often confused with asthma or COPD.

PH patients experience symptoms such as shortness of breath, dizziness and fatigue. While there’s currently no cure, there are 14 FDA-approved therapies available to help patients live better lives. Without treatment, mean survivability is only 2.8 years.

This is worse than most cancers. 


PH SYMPTOMS

The symptoms for all types of PH may be similar, and symptoms are usually more severe as the disease progresses. Symptoms of PH may include:

  • Fatigue which may be due to poorly oxygenated blood
  • Shortness of breath.  In early phase this may occur when you are exercising but later will progress and become apparent when you are resting
  • You may experience pain or perhaps pressure in the chest area
  • Fast heart beat or skipped beats (palpitations or arrhythmias)
  • You may feel faint or dizzy which is known as syncope
  • Your lips or skin may turn blue, this is referred to as cyanosis (lack of oxygen in the blood)
  •  Swelling in your legs and ankles/feet also known as edema
  • Internal lung bleeding and coughing up blood (hemoptysis)

In advanced stages of pulmonary hypertension, minimal activity may produce some or all of these symptoms. Patients in advanced stages may experience irregular hearbeat, a racing pulse, passing out and difficulty breathing at rest.

Sometimes these symptoms mean you have another condition, but sometimes, these symptoms mean you have pulmonary hypertension.


Who gets pulmonary hypertension?

PH affects people of all ages, races and ethnic backgrounds. Although anyone can get PH, there are risk factors that make some people more susceptible.

  • Family history: PH can have a genetic component
  • Gender: Idiopathic PAH and familial PAH are at least two-and-a-half times more common in women than in men.
  • Pregnancy
  • Altitude
  • Associated diseases, like congenital heart disease, lung disease, liver disease, schistosomiasis, and connective tissue disorders
  • Drugs and toxins, like methamphetamines and the diet drug "fen phen"

DIAGNOSIS

Pulmonary hypertension (PH) can be difficult to diagnose in a routine medical exam because the most common symptoms of PH are also associated with many other conditions. If your doctor suspects that you have PH, he or she will want to review your medical and family history, perform a physical exam and perform one or more diagnostic tests.


Preliminary Tests

To determine if you have PH, your medical team will schedule specialized tests. If your medical team suspects PH as a result of one or more of the following tests, they will go on to schedule a right-heart catheterization, which is required to confirm diagnosis.

  • Blood tests
  • Chest X-rays
  • Electrocardiogram (ECG)
  • Echocardiogram
  • Pulmonary function tests
  • Exercise Tolerance Test (Six-Minute Walk Test)
  • Nuclear Scan (Ventilation/Perfusion Scan or V/Q Scan)

The Gold Standard for PH Diagnosis

Right-Heart Catheterization
If the results of initial tests point to PH, your doctor will schedule a right-heart catheterization (commonly referred to as a “right heart cath”). Right-heart catheterization is one of the most accurate and useful tests to get a definitive diagnosis for pulmonary hypertension. This is the only test that directly measures the pressure inside the pulmonary arteries, and it should be done in all patients at least once to confirm a patient's diagnosis of PH. During the test, doctors insert a catheter (a thin rubber tube) through a large vein in the patient's groin or neck. They then pass the catheter up into the patient's heart to measure the blood pressure in the right side of the heart and lungs.

Vasodilator Study (Acute Vasodilator Challenge)
This test is used for patients who have already been diagnosed with pulmonary hypertension to determine how much their pulmonary blood vessels can relax over a brief period of time. Its main purpose is to screen for patients who might respond favorably to calcium channel blockers, a form of medication. The test can also help determine the patient's prognosis. With a right heart catheter in place, the patient is given drugs that relax the pulmonary arteries. The test drug is given to the patient in higher and higher doses, pausing at each dose to see how the patient reacts. Once a significant response occurs or the side effects become intolerable, the test is considered complete. 


PROGNOSIS

Once a person is diagnosed with Pulmonary Hypertension, it may take a considerable amount of time to determine the best therapy and course of action.  Since there are a number of causes for PH, the therapies can be complex and require repeated follow-up care.  Patients may have to have their therapy changed if and when it becomes ineffective.  Unfortunately, at this time, there is no cure for PH, however, early diagnosis makes it easier to control and ensures a better quality of life.

It was once thought that PH was a disease that primarily affected women of childbearing age, but it is now known that it can affect many ages and races.  However, older women account for the majority of cases and decedents with this condition.  Depending on when a person is diagnosed and the particular type of PH they have, the mortality rate can be as high as 90 percent within 3 to 5 years.


5 different classes of PH:

Arterial PH
Venous PH
Hypoxic PH
Thromboembolic PH
Miscellaneous PH