Primary Pulmonary Hypertension

Primary Pulmonary Hypertension
(PPH, Unexplained Pulmonary Hypertension, Idiopathic Pulmonary Hypertension, Pulmonary Arterial Hypertension, Sporadic Primary Pulmonary Hypertension, Familial Primary Pulmonary Hypertension)
Pronounced: PRY-mair-ee PUL-mo-nair-ee hi-per-TEN-shun
Definition
Primary pulmonary hypertension (PPH) is a rare disease that causes high blood pressure in the blood vessels of the lungs. A person with PPH has extra muscle in the walls of those blood vessels. That extra muscle makes it more difficult for blood to flow through them. So, the right side of the heart, which pumps blood to the lungs, has to work harder. This additional strain can eventually lead to heart failure.
PPH is a serious condition that requires care from your doctor.
Heart and Lungs

Causes
The cause of PPH is unknown, but several factors may contribute to the development of the disease, including:
• Immune system disease
• Exposure to certain drugs or chemicals
• Genetic defects
Risk Factors
A risk factor is something that increases your chances of getting a disease or condition. The following factors increase your chances of developing PPH:
• Female between the ages of 20 and 40
• Family history of PPH
• Use of appetite suppressants – diet drugs
• Cocaine use
Symptoms
Initial symptoms of PPH may be minor, and will get progressively worse. If you experience any of these symptoms do not assume it is due to PPH. These symptoms may be caused by other, less serious health conditions. If you experience any one of them, see your physician.
• Shortness of breath, especially when you’re active
• Hyperventilation – abnormally rapid, deep breathing
• Fatigue – tiredness
• Progressive weakness
• Fainting spells
• Light-headedness during exercise
• Dizziness
• Coughing up blood
• Cyanosis – a bluish tint to the lips and skin
• Swelling of the legs and hands
• Chest pain
A physical exam by your doctor may show:
• Swelling of the veins in your neck
• Enlarged liver
• A heart murmur – an abnormal sound in the heart
Diagnosis
Diagnosis of PPH may be delayed because it is hard to detect until symptoms worsen. Evaluation should take place at a medical center with expertise in pulmonary hypertension.
Your doctor will ask about your symptoms and medical history, and perform a physical exam. Tests may include the following:
• Electrocardiogram (ECG, EKG) – a test that records the heart’s activity by measuring electrical currents through the heart muscle.
• Chest x-ray – a test that uses radiation to take a picture of structures inside the body. A chest x-ray can show if the heart is enlarged.
• CT scan of the chest – a type of x-ray that uses a computer to make pictures of structures inside the chest.
• Echocardiogram – a test that uses high-frequency sound waves (ultrasound) to examine the size, shape and motion of the heart.
• Pulmonary function tests – non-invasive tests, like blowing into a tube, that measure how well your lungs are working.
• Cardiac catheterization – a tube-like instrument inserted into the heart through a vein or artery (usually in the arm or leg) to detect problems with the heart and its blood supply.
• Pulmonary arteriogram – a type of x-ray that takes pictures of the blood vessels in the lungs, to detect blood clots and other blockages.
• Nuclear lung scan – a special camera takes pictures of the lungs, to look for blockages in the blood vessels.
Treatment
There is no cure for PPH. Treatment is used to help alleviate and control the symptoms. Talk with your doctor about the best treatment plan for you. Treatment options include the following:
Medication
Calcium channel-blocking drugs – to relax the muscles in the blood vessels in the lungs
Anticoagulants – to decrease the chance of blood clots in the lungs
Diuretics – to reduce the amount of fluid in the body and in turn reduce strain on the heart
Vasodilators – to help reduce blood pressure in the blood vessels in the lungs
Supplemental oxygen
Delivered through a mask or tubes inserted into the nostrils, if breathing becomes difficult
Lung transplant or heart-lung transplant
Defective lungs and/or heart are replaced with donor organs. This option is used only in severe cases of PPH.
Prevention
There are no guidelines for preventing PPH because the cause is not known.