Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is one form of pulmonary hypertension (high blood pressure in the lungs).

In PAH, the pulmonary arteries become stiff and dysfunctional. They can thicken or narrow as the disease progresses.

The heart has to work harder to push blood through the stiff, narrow arteries. This causes the blood pressure in the lungs to rise.

Pulmonary Arterial Hypertension Types

The major types of pulmonary arterial hypertension (PAH) include:

• Idiopathic — developing without an apparent or known cause
• Heritable — common among family members due to a genetic mutation
• Drug and toxin-induced PAH

Pulmonary arterial hypertension (PAH) can also be associated with other conditions, such as:

• HIV
• Scleroderma/connective tissue diseases
• Schistosomiasis
• Pulmonary veno-occlusive disease
• Chronic high altitude exposure

Idiopathic Pulmonary Arterial Hypertension (IPAH)

• IPAH is a rare disease affecting less than 200,000 people in the United States.
• Women are twice as likely to generate the disease as compared to men.
• This is the most common disorder among the group of diseases within this category in the U.S.

IPAH symptoms, diagnosis, and treatments

Because symptoms of IPAH can be similar to more common diseases — such as asthma, COPD, or obesity — diagnosis is often delayed.

So, it is important for doctors to have a suspicion of PAH when they consider possible causes for shortness of breath.

Doctors at the UPMC Comprehensive Pulmonary Hypertension Program perform the following screenings and tests to diagnose IPAH:

• Echocardiogram
• Right heart catheterization

Medical treatments for IPAH can include:

• Oral medications
• Inhaled medications
• Subcutaneous and intravenous medications

Doctors choose the medication based on the severity of disease. Often, they will start patients on oral medication, and — if the disease worsens — prescribe additional medicines.

Close follow up with a PH specialist is essential to monitor progress of treatment.

In addition to medicine, doctors may:

• Prescribe a low-salt diet to minimize fluid retention.
• Advise monitored pulmonary rehabilitation.
• Refer people for a lung transplant evaluation in the event their disease is severe or progresses despite medicines.

Heritable Pulmonary Arterial Hypertension (HPAH)

• In few cases, PAH can run in families. HPAH is due to a genetic mutation passed from generation to generation.
• Approximately 20 percent of people with this gene mutation develop HPAH.
• Inheriting a genetic mutation from one’s parent does not mean that a person will automatically get PAH, however it does mean that the risk is higher than in the general population.

Drug and Toxin-Induced Pulmonary Arterial Hypertension

• PAH came to the forefront as an epidemic when a particular diet drug came on the market.
• The drug was removed from the market, but other drugs have been associated with PAH since then.

Definite risk factors for drug and toxin-induced PAH

• Aminorex
• Appetite suppressants
• Benfluorex
• Chemotherapeutic agents
• Dasatinib
• Dexfenfluramine
• Fenfluramine
• Toxic rapeseed oil

Likely risk factors for drug and toxin-induced PAH

• Amphetamine
• L-tryptophan
• Methamphetamine
• Selective serotonin reuptake inhibitors taken during pregnancy have been associated with persistent PAH of the newborn
• Stimulants

Possible risk factors for drug and toxin-induced PAH

• Chemotherapeutic agent
• Cocaine
• Phenylpropanolamine
• St. John’s Wort

Unlikely risk factors

• Cigarette smoking
• Estrogen
• Oral contraceptives

Make an Appointment for Pulmonary Arterial Hypertension Care Today

To schedule an appointment at the UPMC Comprehensive Pulmonary Hypertension Program, contact us at:

• 1-877-PH4-UPMC, toll-free
• PHprogram@upmc.edu

Learn More About PAH and Other Breathing Disorders

The links below open a new window.

From our Health Library:

• Pulmonary Hypertension