Munson Health
Idiopathic Pulmonary Arterial Hypertension

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by de la Rocha K

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



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


PPH Cure Foundation

Pulmonary Hypertension Association



Heart and Stroke Foundation of Canada

The Canadian Lung Association



American Heart Association. Primary or unexplained pulmonary hypertension. American Heart Association website. Available at: . Accessed November 10, 2010.

American Lung Association. Primary pulmonary hypertension (PPH) fact sheet. American Lung Association website. Available at: . Accessed September 12, 2005.

Cedars-Sinai Medical Center. Imaging–nuclear lung scan. Cedars-Sinai Medical Center website. Available at: Accessed September 21, 2005.

Cincinnati Children’s Hospital Medical Center. Primary pulmonary hypertension. Cincinnati Children’s Hospital Medical Center website. Available at: . Accessed November 10, 2010.

DynaMed Editorial Team. Primary pulmonary hypertension. EBSCO DynaMed website. Available at: . 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.


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