PH

Here is a little info on PH.... All this is taken straight from the PHA website
                                                          http://www.phassociation.org

Pulmonary hypertension (PH) is a general term used to describe high blood pressure in the lungs from any cause.

Pulmonary arterial hypertension (PAH) is high blood pressure in the pulmonary arteries from diseases that affect the small vessels of the lung, causing them to narrow and impede blood flow.

Major Types of Pulmonary Arterial Hypertension

In idiopathic pulmonary arterial hypertension, the cause of the PAH is unknown.

Heritable PAH, formerly known as familial or genetic PAH, is a form of PAH that can be inherited. Heritable PAH is relatively uncommon. Of the small percentage of people who do carry the PH gene, only a small number of carriers will develop the disease. 

In associated pulmonary arterial hypertension, the PAH is associated with another disease or condition. PAH is commonly associated with connective tissue diseases (such as scleroderma, CREST syndrome and lupus), congenital heart disease, chronic liver disease, HIV, drugs and toxins, and more.

SYMPTOMS
The symptoms for all types of pulmonary hypertension may be similar, but the severity of the symptoms usually correlates with the progression of the disease.

• Breathlessness on exertion
• Chest pain (also called angina pectoris)
• Dizziness upon standing, climbing stairs, straightening up from a bent position, or even while just sitting
• Fainting (also called syncope)
• Loss of energy
• Swollen ankles and legs (also called edema)
• Depression
• Dry cough
• Raynaud’s Phenomenon (chalky white and/or dusky blue fingers that may be painful and can sometimes be provoked by the cold)

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

If a doctor can see no other obvious cause for these symptoms, pulmonary hypertension should be considered and diagnostic tests should be performed.

Preliminary Tests

Blood Tests
Blood tests check the oxygen levels in the blood, they observe liver and kidney function, and they identify whether the patient has collagen vascular disease, thyroid problems, signs of infection or HIV antibodies. One test, the brain natriuretic peptide, helps to assess the strain on the heart and may also be used to monitor response to treatment.

 

Chest X-Rays

Chest X-rays can reveal an enlarged right ventricle or pulmonary arteries. Chest X-rays can also show signs of emphysema or interstitial fibrosis (scarring) of the lungs.

 

Electrocardiogram (ECG)

Electrodes are attached to the patient's skin and a recording is made of the electrical impulses of the heart. An ECG cannot diagnose PH by itself. If your doctor performs an ECG, he or she will also perform one or more additional procedures to identify PH.

 

Doppler Echocardiogram

In this procedure, electrodes are placed on the patient’s skin and a sonogram of the heart is taken. This painless procedure is often used to make a preliminary diagnosis by estimating the pressures in the right heart and examination of the heart function. Other heart conditions that produce symptoms similar to PH may be diagnosed. In addition, an echocardiogram may be used to monitor a patient’s condition.

 

Pulmonary Function Tests

These tests measure how much air your lungs can hold, how much air moves in and out of them and the lungs’ ability to exchange oxygen. These tests may be performed to potentially identify its cause.

 

Exercise Tolerance Test (Six-Minute Walk Test)

During this test, a patient will be asked to perform an exercise, most commonly a six-minute walk. The purpose is to identity the patient’s exercise tolerance level.

 

Nuclear Scan (a.k.a. Ventilation/Perfusion Scan or V/Q Scan)

This diagnostic tool tests for blood clots in the lungs by producing a picture of lung air and blood flow to the lungs. A small dose of radioactive material is breathed in and another small dose is injected via a blood vessel into the lungs. The doctor will review the images that are produced to evaluate the health of the lungs.

 

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 with PH.

 

Vasodilator Study (a.k.a. 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, but it also has prognostic value. 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



Although there is currently no cure for pulmonary hypertension, there are treatment options available and more are on the horizon. Treatments include conventional medical therapies and oral, inhaled, intravenous (into the vein) and subcutaneous (into the skin) options. Depending on the severity of PH, heart or lung transplant may also be an option.