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Idiopathic Pulmonary Arterial Hypertension

(PPH; Unexplained Pulmonary Hypertension; Idiopathic Pulmonary Hypertension; Pulmonary Arterial Hypertension; Sporadic Primary Pulmonary Hypertension; Familial Primary Pulmonary Hypertension; Primary Pulmonary Hypertension)

Definition

Primary pulmonary hypertension (PPH) is a rare disease. It is high blood pressure in the blood vessels of the lungs.
A person with PPH has extra muscle in the walls of these blood vessels. That extra muscle makes it more difficult for blood to flow through them. As a result, the right side of the heart has to work harder to push blood to the lungs. This additional strain can eventually lead to heart failure.
PPH is a serious condition. It requires care from your doctor.
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Causes

The cause of PPH is unknown. Several factors may contribute to the development of the disease, including:
  • Immune system disease
  • Exposure to certain drugs or chemicals
  • Genetic defects

Risk Factors

The following factors increase your chances of developing PPH:
  • Female between the ages of 30 and 40
  • Liver cirrhosis
  • Portal hypertension
  • HIV infection
  • Family history of PPH
  • Use of appetite suppressants—diet drugs (eg, fenfluramine)
  • Cocaine use

Symptoms

Initial symptoms of PPH may be minor. They 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 doctor.
  • Shortness of breath, especially when you are active or at rest
  • Hyperventilation—abnormally rapid, deep breathing
  • Fatigue—tiredness
  • Progressive weakness
  • Fainting spells
  • Dizziness
  • Coughing up blood
  • Cyanosis—a bluish tint to the lips and skin
  • Swelling of the legs and hands
  • Chest pain
  • Lack of appetite
  • Cold hands and feet
  • Low blood pressure
A physical exam by your doctor may show:
  • Swelling of the veins in your neck
  • Enlarged liver and swollen abdomen
  • A heart murmur —an abnormal sound in the heart

Diagnosis

Diagnosis of PPH may be delayed. It is hard to detect until symptoms worsen. Evaluation should take place at a medical center with expertise in PPH.
Your doctor will ask about your symptoms and medical history. A physical exam will be done. Tests may include the following:
  • Blood tests—liver function, coagulation tests, test for HIV, autoantibody
  • Blood gases to determine the oxygen status
  • Electrocardiogram (ECG, EKG) —a test that records the heart’s electrical activity
  • Chest x-ray —a test that uses radiation to take a picture of structures inside the body and 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 —to detect problems with the heart and its blood supply
  • Pulmonary arteriogram—a type of x-ray 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
  • Six minute walk to determine the amount of shortness of breath, an indirect measure of the severity of PHH

Treatment

There is no cure for PPH. Treatment is used to help alleviate and control the symptoms. Talk with your doctor about the best plan for you. Treatment options include the following:

Medication

  • Calcium channel-blocking drugs ( Nifedipine , diltiazem , and amlodipine )—to relax the muscles in the blood vessels in the lungs
  • Protacylin drugs ( epoprostenol , treprostinil ) and synthetic prostacylins ( iloprost , cisaprost)—to relax the muscles in the blood vessels in the lungs
  • Digoxin —to improve the ability of the heart to pump blood
  • 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
  • Endothelin receptor antagonists (Bosentan)—to relax muscles in blood vessels of lungs
  • Phosphodiesterase-5 inhibitors ( sildenafil )—to relax muscles in blood vessels of lungs

Supplemental Oxygen

If breathing becomes difficult oxygen may be given. It may be given through a mask or tubes inserted into the nostrils.

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.

RESOURCES

PPH Cure Foundation http://www.pphcure.org/

Pulmonary Hypertension Association http://www.phassociation.org/

CANADIAN RESOURCES

Heart and Stroke Foundation of Canada http://www.heartandstroke.com/

The Canadian Lung Association http://www.lung.ca/

References

American Heart Association. Primary or unexplained pulmonary hypertension. American Heart Association website. Available at: http://www.americanheart.org/presenter.jhtml?identifier=4752 . Accessed November 10, 2010.

American Lung Association. Primary pulmonary hypertension (PPH) fact sheet. American Lung Association website. Available at: http://www.lung.org/lung-disease/primary-pulmonary-hypertension/ . Accessed September 12, 2005.

Cedars-Sinai Medical Center. Imaging–nuclear lung scan. Cedars-Sinai Medical Center website. Available at: http://www.csmc.edu/pf%5F2760.html. Accessed September 21, 2005.

Cincinnati Children’s Hospital Medical Center. Primary pulmonary hypertension. Cincinnati Children’s Hospital Medical Center website. Available at: http://www.cincinnatichildrens.org/health/p/pulmonary-hypertension/ . Accessed November 10, 2010.

DynaMed Editorial Team. Primary pulmonary hypertension. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php. Updated September 29, 2010. Accessed November 10, 2010.

Rich S. the current treatment of pulmonary arterial hypertension: time to redefine success. Chest . 2006;130:1198-1202.

Zamanian RT, Haddad F, Doyle RL, Weinacker AB. Management strategies for patients with pulmonary hypertension in the intensive care unit. Crit Care Med . 2007;35:2037-2050.

Revision Information

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.

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