CAUSES

​

The cause of primary pulmonary hypertension (PPH) is unknown.  At this point the disease is believed to be caused by inherited genes that make the pulmonary vasculature more sensitive and reactive to different factors.

 

 

SYMPTOMS

​

Symptoms may include:

• Fatigue

• Shortness of breath, especially with exertion

• Dry cough

• Dizziness

• Fainting

• Swelling in extremities

• Bluish lips and skin

• Chest pain (angina)

• Palpitations

 

In advanced stages, you may:

• Have symptoms at rest

• Have extreme fatigue

​

 

DIAGNOSIS

​

The early signs can mimic many diseases.  PPH may be diagnosed when other diseases are ruled out. Tests may include:

• Chest X-ray:  This test takes a picture of the heart and lungs.  Pulmonary vascular congestion on the x-ray may lead the physician to suspect PPH.

• Electrocardiogram (ECG):  This test records the strength and timing of the electrical activity of the heart.  It may also pick-up damage to the right side of the heart if PPH has been present for a long time.

• Echocardiogram (echo):  This test uses sound waves to check the heart's chambers and valves.  The echo sound waves create a picture on a screen as an ultrasound transducer is passed over the skin over the heart.  The echo can show if there are any physical abnormalities of the heart and it's valves in addition to checking if the proper amount of fluid is being moved through it during the cardiac cycle.

• Pulmonary function tests (PFTs):  This test can be done in the office.  This evaluates the function of the lungs.  Someone with a more advanced PPH may show an impairment of gas exchange during a PFT.

• Perfusion lung scan:  A type of nuclear radiology test in which a small amount of a radioactive substance is used to help evaluate blood flow within the lungs. 

• Right sided heart cath:   With this procedure, X-rays are taken after a dye (contrast agent) is injected into the pulmonary artery to look for any narrowing, blockages, or other changes. This is the only test that directly measures the pressure inside the pulmonary arteries.

• Blood tests:  These can be used to check the oxygen and carbon dioxide levels in the blood.  Other blood tests can evaluate liver and kidney function, and also the strain on the heart.

 

​

TREATMENT

​

There is no cure for primary pulmonary hypertension (PPH). Treatment is geared toward managing symptoms.

• Anticoagulants:  These reduce the strain on the heart by reducing the viscosity (thickness) of the blood.

• Diuretics:  These pills increase urination.  This reduces the fluid volume in the body therefor reducing the preload on the right side of the heart.

• Calcium channel blocking/vasodilator drugs:  These cause the heart to pump with less force, thus preventing it from over-working.

• Oxygen therapy:  Oxygen  therapy may be utilized if you have a low SPO2 (oxygen saturation).

• Lung or heart-lung transplant:  This is a consideration for people with end-stage PPH.

​