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.
The cause of PPH is unknown. Several factors may contribute to the development of the disease, including:
The following factors increase your chances of developing PPH:
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.
A physical exam by your doctor may show:
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:
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:
If breathing becomes difficult oxygen may be given. It may be given through a mask or tubes inserted into the nostrils.
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: 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_2760.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.
Last reviewed September 2013 by Michael J. Fucci, DO
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.