Primary Pulmonary Hypertension (PPH, Unexplained Pulmonary Hypertension, Idiopathic Pulmonary Hypertension, Pulmonary Arterial H

Shortness of breath, especially when youre active or at restHyperventilation abnormally rapid, deep breathingFatigue tirednessProgressive weaknessFainting spellsDizzinessCoughing up bloodCyanosisa bluish tint to the lips and skinSwelling of the legs and handsChest painLack of appetiteCold hands and feetLow blood pressure A physical exam by your doctor may show: Swelling of the veins in your neckEnlarged liver and swollen abdomen A heart murmur an abnormal sound in the heart DiagnosisDiagnosis of PPH may be delayed because it is hard to detect until symptoms worsen. Evaluation should take place at a medical center with expertise in pulmonary hypertension. Your doctor will ask about your symptoms and medical history, and perform a physical exam. Tests may include the following: Blood testsliver function, coagulation tests, test for HIV, autoantibodyPulmonary function testsBlood gases to determine the oxygen statusElectrocardiogram (ECG, EKG) a test that records the hearts activity by measuring electrical currents through the heart muscle. Chest x-ray a test that uses radiation to take a picture of structures inside the body. A chest x-ray can show if the heart is enlarged. CT scan of the chesta 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 testsnon-invasive tests, like blowing into a tube, that measure how well your lungs are working.Cardiac catheterization a tube-like instrument inserted into the heart through a vein or artery (usually in the arm or leg) to detect problems with the heart and its blood supply. Pulmonary arteriograma type of x-ray that takes pictures of the blood vessels in the lungs, to detect blood clots and other blockages.Nuclear lung scana 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 TreatmentThere is no cure for PPH. Treatment is used to help alleviate and control the symptoms. Talk with your doctor about the best treatment plan for you. Treatment options include the following:

MedicationCalcium channel-blocking drugsto relax the muscles in the blood vessels in the lungsProtacylin drugs (epoprostenol, treprostinil, lioprost)to relax the muscles in the blood vessels in the lungsDigoxinto improve the ability of the heart to pump bloodAnticoagulantsto decrease the chance of blood clots in the lungsDiureticsto reduce the amount of fluid in the body and in turn reduce strain on the heartVasodilatorsto help reduce blood pressure in the blood vessels in the lungs Supplemental OxygenDelivered through a mask or tubes inserted into the nostrils, if breathing becomes difficult. Lung Transplant or Heart-Lung TransplantDefective lungs and/or heart are replaced with donor organs. This option is used only in severe cases of PPH. PreventionThere are no guidelines for preventing PPH because the cause is not known. RESOURCES: American Heart Associationhttp://www.americanheart.org American Lung Associationhttp://www.lungusa.org PPH Cure Foundationhttp://www.pphcure.org Pulmonary Hypertension Associationhttp://www.phassociation.org CANADIAN RESOURCES: Heart and Stroke Foundation of Canadahttp://ww2.heartandstroke.ca The Canadian Lung Associationhttp://www.lung.ca References: Imagingnuclear lung scan. Cedars-Sinai Medical Center website. Available at: http://www.csmc.edu/pf_2760.html . Accessed September 21, 2005.

Primary or unexplained pulmonary hypertension. American Heart Association website. Available at: http://www.americanheart.org/presenter.jhtml?identifier=4752 . Accessed September 12, 2005. Primary pulmonary hypertension. Cincinnati Childrens Hospital Medical Center website. Available at: http://www.cincinnatichildrens.org/health/heart-encyclopedia/disease/pph.htm . Accessed September 12, 2005. Primary pulmonary hypertension. US National Library of Medicine and the US National Institutes of Health Medline Plus website. Available at: http://www.nlm.nih.gov/medlineplus/ency/article/000112.htm . Accessed September 12, 2005. Primary pulmonary hypertension (PPH) fact sheet. American Lung Association website. Available at: http://www.lungusa.org/site/pp.asp?c=dvLUK9O0E&b=35699 . Accessed September 12, 2005. Rich S: the current treatment of pulmonary arterial hypertension: time to redefine success. Chest . 2006;130:1198-202. Zamanian RT, Haddad F, Doyle RL, Weinacker AB: Management strategies for patients with pulmonary hypertension in the intensive care unit. Crit Care Med . 2007;35:2037-50. Last reviewed January 2008 by David Juan, MDPlease 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.