Pulmonary Hypertension (PH)

Overview
Diagnosis
Treatment

Pulmonary Hypertension Overview

Pulmonary hypertension (PH) is a disease that causes elevated blood pressure in the blood vessels of the lungs, leading to difficulty breathing and possible failure of the right side of the heart (right-ventricula r dysfunction). In many cases, early detection of high blood pressure can help prevent full-blown hypertension.

PH is defined as a mean pulmonary arterial pressure in the lungs that is greater than 25 millimeters of mercury (mmHg). Normal blood pressure in the lungs is around 15 mmHg.

PH may be idiopathic (of unknown cause), hereditary, or associated with underlying autoimmune (rheumatologic), cardiac, hepatic (liver), or pulmonary diseases.

A comprehensive pulmonary hypertension program

While the evaluation of affected patients is complex, the UPMC Comprehensive Pulmonary Hypertension Program, one of the largest in the country, offers people with PH access to state-of-the-art diagnostics, therapeutics, and opportunities to participate in clinical research.

Our specialists in cardiology, pulmonary and critical care medicine, and transplant medicine work together to ensure that the full range of medical and surgical options is available for each patient.

This unique, integrated approach to care benefits patients from the time of their initial evaluation, which can normally take place on a single day and in one location.

Additional Resources

Learn more about the Division of Pulmonary, Allergy, and Critical Care Medicine.

Diagnosing Pulmonary Hypertension (PH)

At your evaluation, your UPMC doctor will ask about your symptoms and medical history.

Pulmonary hypertension symptoms

Frequent symptoms include shortness of breath and reduced exercise capacity.
Some people have very general complaints, such as persistent fatigue, weight gain, and abdominal bloating.
In later stages, abdominal fluid and swelling in the lower limbs may develop, indicating the presence of heart failure.

Testing for pulmonary hypertension

At UPMC, our specialists care for every patient suspected of having PH with a careful diagnostic work-up to determine the diagnosis, clarify the severity, and identify the potential cause.

Some tests may include:

A cardiac catheterization
An electrocardiogram
An echocardiogram
A chest x-ray and/or CT scan
Pulmonary function testing
Blood tests

Because evaluation of PH can be complicated, you may see either a heart or a lung specialist first, along with another specialist, on the same day. We take this approach to review these tests together and arrive at the right diagnosis so we can develop a proper treatment strategy.

Pulmonary Hypertension (PH) Treatments

The UPMC Comprehensive Pulmonary Hypertension Program offers a full spectrum of treatments, as well as new and better medical therapies, ranging from oral agents to inhaled, subcutaneous (under the skin), and intravenous (vein) therapies.

Our level of expertise in managing the most complex cases means we can:

Facilitate diagnostic testing
Participate in disease treatment
Foster long-term management and co-management of patients with their doctors at home

Lung and heart-lung transplantation

For patients who are not responding to medical therapy, UPMC specialists are there every step of the way with surgical options, such as lung and heart-lung transplantation.

A unique feature of our program is the Heart/Lung Pulmonary Hypertension Clinic, which offers effective, high-level evaluation and treatment of people with PH and intrinsic lung disease.

Should patients develop right-ventricular dysfunction, as can occur in the late stages of disease, combined heart-lung transplantation may be the best option. The clinic provides patients ready access to the UPMC Lung and Heart/Lung Transplantation programs, but early referral for future transplantation is recommended.

Striving to improve pulmonary hypertension treatments

UPMC Comprehensive Pulmonary Hypertension Program specialists are constantly investigating new ways to enhance our program with promising therapies that may help individualize future treatments for people with PH.

The program also is researching new technologies to evaluate right-heart function, and new strategies to identify and eliminate factors which delay successful treatment in patients at risk for PH, such as: