Primary Pulmonary Hypertension (PPH) FAQ

What is primary pulmonary hypertension?

Primary pulmonary hypertension is a clinical syndrome where the blood flow that leaves the right side of the heart faces an increased resistance (pressure).

Normally blood flows from the right ventricle into the pulmonary arteries and then flows into smaller and smaller blood vessels until it reaches the capillaries (thin walled blood vessels near the small air chambers of the lungs or alveoli). There gas exchange occurs where oxygen is picked up and carbon dioxide is released. The blood vessels have muscles in their walls that can regulate their diameter and thereby allow more or less blood flow to enter the capillaries.

In primary pulmonary hypertension, the blood vessels of the lungs have an increased amount of muscle in the walls. This causes a higher resistance in the lungs. The right ventricle then has to work harder to pump blood out to the lungs. The right ventricle will enlarge and thicken in response to this extra work. With time, the extra work placed on the right side of the heart can cause it to fail.
 

What Causes Primary Pulmonary Hypertension?

Certain types of congenital heart disease can, if left unrepaired, lead to high pressure in the lungs. Primary lung problems such as cystic fibrosis or bronchopulmonary dysplasia (BPD) can lead to elevated resistance in the lungs.

A significant association exists between the use of the fen-phen diet drug and PPH. Fen-phen was taken off the market in the U.S. in 1997. Studies have shown that patients can develop the disease several years after having stopped taking diet drugs. Medical experts have testified that there is a potential latency of ten or more years between the last date on which a patient is exposed to diet drugs and the date at which the patient develops the first symptoms of what is ultimately diagnosed as PPH. If you have PPH and took fen-phen, please contact us.
 

Who gets primary pulmonary hypertension?

The incidence of primary pulmonary hypertension is 8 out of 100,000 people. Women between the ages of 20 and 40 are most at risk. There is often a family history of primary pulmonary hypertension or sudden death.

Primary pulmonary hypertension can occur at any age, especially in teenagers. Recently there has been an increase in patients with primary pulmonary hypertension in people who have taken fen-phen, a combination diet drug.
 

What are the signs and symptoms of Primary Pulmonary Hypertension?

The most common symptoms of primary pulmonary hypertension are related to difficulty with breathing. They include:

• Progressive shortness of breath (especially with activity)
• Hyperventilation (breathing harder and faster)
• Fatigue (tiring easily)
• Progressive weakness
• Fainting spells
• Lightheadedness or dizziness
• Coughing up blood
• Progressive cyanosis (blue tinge to lips hands and feet)

These symptoms may occur at presentation, or progress over a period of time. A patient with primary pulmonary hypertension must be monitored for these symptoms by routine visits to their cardiologist or pulmonologist. If any new symptoms develop, patients should notify their doctor immediately.

A physical examination of patients with primary pulmonary hypertension may show:

• Distension of veins in the neck
• Swelling of the legs and hands due to fluid retention
• Enlarged liver

All of these signs are related to the right ventricle working harder to pump blood into the high resistance of the lungs' blood vessels.
 

How is PPH Diagnosed?

A good history and physical exam can give the physician clues to the diagnosis.

An electrocardiogram should be performed which will show enlargement of the right side of the heart.

A chest X-ray may show enlargement of the size of the heart, and is often a good way to follow the patient's heart size over time.

An echocardiogram, or ultrasound of the heart, will be performed initially and can also be used to follow the progression of the disease. An echo can show enlargement of the right ventricle and can sometimes estimate the pressures of the right ventricle and pulmonary arteries.

The gold standard for making the diagnosis of primary pulmonary hypertension is a cardiac catheterization. This will actually measure the pressures on the right side of the heart and the resistance of the blood vessels in the lungs.

Various drugs may be used during a cardiac catheterization to determine if the blood vessels in the lungs will relax in response to them. This may lower the pressures in the lungs and can help guide therapy.
 

What are the treatment options for Primary Pulmonary Hypertension?

Unfortunately there is no cure for primary pulmonary hypertension. There are some treatments that have been found to relieve some of the symptoms and slow the progression of the disease:

• Oxygen can be used at home and often helps relieve some of the symptoms of difficult breathing. It is also known to relax the muscles in the arteries of the lungs, which will lower the pressures in the pulmonary arteries.
   
• Calcium channel blockers such as nifedipine can relax the muscles in blood vessel walls and thereby help with the function of the right side of the heart. Unfortunately it also tends to decrease the blood pressure in the entire cardiovascular system, which is sometimes not well tolerated.
   
• Diuretics such as Lasix are used to get rid of excess fluid in the body. These drugs must be used carefully, however, because if a patient gets too dehydrated, the poorly functioning right ventricle will not be able to pump out even a small amount of blood to the lungs and the patient will have severe problems with oxygenation.
   
• Prostacycline is an investigational drug being used in some centers. It has been shown to dilate the blood vessels of the lungs and decrease the resistance to blood flow. It is one of the more effective drugs available at this time. Unfortunately it can only be given as a continuous infusion via an intravenous line. For patients that have demonstrated a response to this drug by cardiac catheterization, it can be given at home through a permanent IV placed in a big vein.
   
• Inhaled nitric oxide is a relatively new drug that has been shown to relax the blood vessels in the lungs without having any effect on the blood vessels of the rest of the body. It is used in the inpatient setting and has recently been approved by the FDA for treatment for pulmonary hypertension. In rare cases it can be used as a home regimen, though it needs to be on continuously and is very bulky and difficult to manage.
   
• Sildenafil is a new investigational drug used in combination with inhaled Nirtric Oxide that further decreases pulmonary artery pressures and allows inhaled Nitric Oxide to be stopped without difficulty. Sildenafil is an oral medication that is used chronically, and ongoing studies are evaluating its efficacy in children with congenital heart disease.
   
• Bosentan is also a new investigational drug used to treat pulmonary hypertension both in the adult with primary palmary hypertension and the child with primary palmary hypertension related to congenital heart disease.
   

What is the prognosis for Primary Pulmonary Hypertension patients?

Unfortunately, the long-term prognosis is not good for patients with primary pulmonary hypertension. The pressures in the lungs even with treatment will get progressively worse and cause the right ventricle to fail.

As the right ventricle gets larger and larger, the patient can develop irregular heart rhythms which can lead to sudden death. Over time, the blood vessels in the lungs will get weak and be at risk for rupturing and causing massive bleeding in the lungs.

Patients will get progressively weaker and more easily fatigued, so that their quality of life will be affected.

The only true cure for this problem is a heart-lung transplant. As technology advances these are becoming more and more successful.

What should be done for patients who have been diagnosed with primary pulmonary hypertension?
It is important to see your doctor on a regular basis and report any changes in energy level, ability to exercise, or overall health.

Patients should restrict themselves from competitive sports and only participate in light activity if they're able to rest when tired.

If patients have been placed on therapy, it is important to use it regularly and not just when they feel bad.