New Therapies Offer Hope for Patients with Pulmonary Arterial Hypertension

    Two decades ago, patients suffering from pulmonary arterial hypertension (PAH) had no medical treatment options and death was frequently imminent. Today, there is an arsenal of FDA-approved and experimental medications for the different types of the progressively severe condition.

    “While there is still no cure, we can offer significant improvement in patients’ symptoms and life expectancy if we treat them early with these new medications,” says David Ross, MD, medical director of the UCLA Lung and Heart-Lung Transplant Program.

    Unfortunately, says Dr. Ross, the earliest signs of PAH, are often mistaken for other conditions, such as asthma or a lack of physical conditioning. Patients who fall into a higher-risk category for potential development of PAH should have symptoms evaluated immediately. Higher-risk groups may include individuals with chronic liver disease, HIV, collagen-vascular diseases such as scleroderma and systemic lupus erythematosus or mixed connective-tissue disease.

    Since the mid-1990s, eight drugs have been approved for use in patients with PAH, bringing the median survival up to more than five years. The medications, which are designed to improve the blood flow through the abnormal vessels in the lungs, work in a variety of ways and come in oral, inhaled, intravenous and subcutaneous forms. Several promising experimental treatments are also available, increasing the likelihood that with proper treatment, patients with PAH can return to a satisfactory quality of life or survive with a “bridge” to lung transplantation. “We now have a broad spectrum of therapies, and the investigational drugs will further complement our options for effective threapy,” says Dr. Ross.

    For patients who have PAH that is so severe that it requires hospitalization, the parenteral (intravenous or subcutaneous infusion) therapies can often enable specialists to take patients from the brink of death to being able to leave the hospital and thrive at home. “These therapies allow us to attack the disease in an
    acute fashion with titratable medications — drugs that can be increased and decreased in real time — to optimize a particular patient’s response,” explains Rajan Saggar, MD, a pulmonary and critical-care physician and director of the medical intensive care unit at Ronald Reagan UCLA Medical Center.

    Parenteral therapies are increasingly being used outside the hospital. “Some patients with this condition are extremely ill with progressive pulmonary vascular disease but may not require hospitalization,” Dr. Saggar says. “We no longer wait for a hospitalization to consider initiation of more aggressive drug regimens.” Similarly, patients with the forms of pulmonary hypertension known to have the worst survival rates are being treated more aggressively in the outpatient setting. These include patients with liver disease, parenchymal lung disease and connective-tissue disease.

    What Is Pulmonary Arterial Hypertension?

    Pulmonary arterial hypertension is high blood pressure in the arteries leading from the heart to the lungs. Pulmonary arterial hypertension is a different condition than ordinary high blood pressure (hypertension).

    Symptoms Include:

    • Shortness of breath (usually starts slowly and gets worse over time)
    • Chest pain
    • Fatigue
    • Lethargy
    • Passing out suddenly
    • Swelling of the legs (edema)

    Causes Include:

    • Congestive heart failure
    • Venous thromboembolic disease (blood clots in the lungs)
    • HIV infection
    • Illegal drug use (cocaine, methamphetamine)
    • Cirrhosis of the liver
    • Appetite-suppressant medications (fenfluramine, dexfenfluramine, diethylpropion)
    • Autoimmune diseases (lupus, scleroderma and rheumatoid arthritis)
    • Heart shunts (abnormal blood flow between heart chambers)
    • Chronic lung disease (emphysema, chronic bronchitis and pulmonary fibrosis)
    • Obstructive sleep apnea

    When no cause can be identified after testing, the condition is called idiopathic pulmonary arterial hypertension. This condition was formerly known as primary pulmonary hypertension.

    illustration by Mark Summers
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