Pulmonary hypertension (PH) can occur in people who have chronic lung diseases, such as COPD (chronic obstructive pulmonary disease) or idiopathic pulmonary fibrosis (IPF).
The cause of PH was long thought to be due to chronically low oxygen levels, but the exact cause is not known.
Because the nature of these diseases is complex, they can be difficult to treat.
COPD and pulmonary fibrosis are both chronic lung diseases that make it difficult for a person to breathe. However, these two diseases cause different kinds of damage to the lungs and have different causes.
History of smoking causes the majority of COPD cases. In COPD, also known as chronic bronchitis, the airways and sacs in the lungs become blocked making breathing difficult.
IPF is the build-up of scar tissue in the lungs making it difficult for patients to breath. In IPF, the cause is unknown.
Treating PH associated with chronic lung disease is challenging. Patients with lung disease do not necessarily respond the same way as patients without lung disease do.
It is important to weigh the risks and benefits of PH therapies in the setting of underlying lung disease.
Based on clinical experience and lack of evidence for great benefits in people with mild PH, the team at the UPMC Comprehensive Pulmonary Hypertension Program focuses on treating patients with severe PH.
People with severe PH are at highest risk of death and are most likely to be helped.
Given the complex nature of this diagnosis, we often recommend that patients:
In our program, we work closely with the Emphysema Clinical Research Center and Simmons Center for Interstitial Lung Disease, through which research studies often are available.
We are happy to evaluate patients for PH associated with chronic lung disease to explore available clinical trials and help form patient-specific treatment plans.
Our expertise in PH, COPD, pulmonary fibrosis, and lung transplantation allows our patients to explore all available treatment options for this complicated disease.
Pulmonary hypertension with COPD is classified into three categories:
Treatment options for PH with COPD depends on the severity of the disease. Treatment for mild PH with COPD includes lifestyle changes such as quitting smoking and exercising.
As pulmonary hypertension with COPD worsens, the treatment options change. When PH with COPD is classified as moderate or severe, treatment includes:
The lung scarring that is caused by IPF is permanent and cannot be reversed. Treatment options for PH with IPF work to slow the progression of the disease and improve the quality of life.
Pulmonary hypertension with idiopathic pulmonary fibrosis treatment options include:
UPMC Patient Education Materials on Breathing Disorders:
To make an appointment at the UPMC Comprehensive Pulmonary Hypertension Program, contact us at:
To schedule an appointment with the UPMC Comprehensive Pulmonary Hypertension Program team, call toll-free at 1-877-PH4-UPMC, or email PHprogram@upmc.edu.
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