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:
PPH is more common in women aged 30-40 years. Other factors that may increase your risk of PPH include:
Initial symptoms of PPH may be minor. They will get progressively worse. PPH may cause:
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:
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 includes the following:
Medication can improve blood flow, decrease the risk of blood clots, and improve the ability of the heart to pump blood. These may include:
If breathing becomes difficult, oxygen therapy may be given. It may be given through a mask or tubes inserted into the nostrils.
National Heart, Lung, and Blood Institute
Pulmonary Hypertension Association
Heart and Stroke Foundation of Canada
The Lung Association
Explore pulmonary hypertension. National Heart, Lung, and Blood Institute website. Available at: https://www.nhlbi.nih.gov/health/health-topics/topics/pah. Updated August 2, 2011. Accessed September 14, 2017.
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 14, 2017.
Primary pulmonary hypertension in children. Cincinnati Children’s Hospital website. Available at: https://www.cincinnatichildrens.org/health/p/pulmonary-hypertension. Updated June 2014. Accessed September 14, 2017.
Pulmonary arterial hypertension (PAH). EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T115043/Pulmonary-arterial-hypertension-PAH. Updated June 14, 2017. Accessed September 14, 2017.
Pulmonary hypertension. American Lung Association website. Available at: http://www.lung.org/lung-health-and-diseases/lung-disease-lookup/pulmonary-hypertension. Accessed September 14, 2017.
Pulmonary hypertension—high blood pressure in the heart-to-lung system. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/HighBloodPressure/AboutHighBloodPressure/What-is-Pulmonary-Hypertension_UCM_301792_Article.jsp#.Wbr2xrKGNxA. Updated May 23, 2017. Accessed September 14, 2017.
Last reviewed September 2017 by EBSCO Medical Review Board Michael J. Fucci, DO, FACC
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.