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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

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
 

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.

 

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