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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:

Risk Factors

PPH is more common in women aged 30-40 years. Other factors that may increase your risk of PPH include:

Symptoms

Initial symptoms of PPH may be minor. They will get progressively worse. PPH may cause:

Diagnosis

Your doctor will ask about your symptoms and medical history. A physical exam will be done. Diagnosis of PPH may be delayed. It is hard to detect until symptoms worsen.
A physical exam by your doctor may show:
Tests may include:
Imaging tests evaluate the lungs and surrounding structures. These may include:

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

Medication can improve blood flow, decrease the risk of blood clots, and improve the ability of the heart to pump blood. These may include:
  • Calcium channel blockers
  • Protacylins
  • Digoxin
  • Anticoagulants
  • Diuretics
  • Vasodilators
  • Endothelin receptor antagonists
  • Phosphodiesterase-5 inhibitors

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 or when other treatment methods fail.

Prevention

There are no current 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

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

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

References

Nuclear lung scan. Cedars-Sinai Medical Center website. Available at: http://www.cedars-sinai.edu/Patients/Programs-and-Services/Imaging-Center/For-Patients/Exams-by-Procedure/Nuclear-Medicine/Nuclear-Lung-Scan.aspx. Accessed September 25, 2014.

Primary pulmonary hypertension. American Lung Association website. Available at: http://www.lung.org/lung-disease/primary-pulmonary-hypertension. Accessed September 25, 2014.

Primary pulmonary hypertension in children. Cincinnati Children’s Hospital website. Available at: http://www.cincinnatichildrens.org/health/p/pulmonary-hypertension. Updated June 2014. Accessed September 25, 2014.

Pulmonary arterial hypertension (PAH). EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated June 30, 2014. Accessed September 25, 2014.

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

What is pulmonary hypertension? American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/HighBloodPressure/AboutHighBloodPressure/What-is-Pulmonary-Hypertension%5FUCM%5F301792%5FArticle.jsp. Updated August 12, 2014. Accessed September 25, 2014.

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

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