I Can’t Breathe! Understanding Pulmonary Hypertension: PPH, PAH or IPAH

By Cheryl Kaye Tardif

What is Pulmonary Hypertension?

Pulmonary hypertension is a rare, serious and incurable lung disorder that affects how blood flows from the lungs to the heart. It is categorized in two ways: primary, meaning there is no obvious cause; and secondary, in which a cause is known, such as bronchitis or emphysema. Primary pulmonary hypertension (PPH), also referred to as pulmonary arterial hypertension (PAH) and more recently, idiopathic pulmonary arterial hypertension (IPAH), causes increased blood pressure in the pulmonary artery, a blood vessel that carries oxygen-poor blood from the right ventricle of the heart to the lungs.

Increased blood pressure can have serious results. The muscles within the walls of the arteries may tighten, causing the arteries to constrict. The walls of the pulmonary arteries may thicken. Scar tissue may form, causing the arteries to become increasingly narrow. Tiny blood clots may form within the smaller arteries, causing blockages. In more serious cases, when the right ventricle no longer functions properly, progressive heart failure occurs, leading most often to death.

Symptoms:

Symptoms of PPH frequently occur over a period of time, making the condition difficult to diagnose. Although most of the symptoms relate to breathing issues, such as shortness of breath and hyperventilation, other symptoms may include:

• Extreme fatigue
• Dizziness or fainting
• Weakness of the body
• Racing pulse
• Chest pain
• Swelling of legs and hands
• Coughing up blood
• Bluish discoloration of lips and skin (cyanosis)

Diagnosing PPH:

PPH is regularly misdiagnosed in routine medical examinations since its symptoms can be confused with other more common conditions. These conditions must be ruled out first, along with secondary pulmonary hypertension disorders. Unfortunately this means that PPH is usually diagnosed after the appearance of many of the symptoms, and by that time the disorder is likely to have progressed to a more serious stage.

Tests to diagnose PPH include:

• X-ray of the chest
• Electrocardiogram
• Echocardiogram
• Cardiac catheterization
• Blood tests
• MRI
• Pulmonary function tests
• Connective tissue serology
• Perfusion lung scans

In most cases, the cause of primary pulmonary hypertension is unknown, yet could be attributed to genetic or familial predisposition, immune system disease or drug/chemical exposure. A number of drugs, such as cocaine, amphetamines and the diet drug Fen Phen (taken off the market in September 1997) have been linked to causing PPH.

Treatment:

PPH requires proper medical diagnosis, treatment and follow-up. Most treatment regimes require drugs that help lower blood pressure, or affect the blood, blood vessels, lungs and/or heart. In recent studies, Viagra (Sildenafil) has been found to improve the condition of PPH. It is awaiting approval for use as a treatment for PPH.

Other drug therapies may include:
• Anticoagulants
• Calcium channel blockers
• Diuretics
• Endothelin receptor antagonists
• Prostacyclin analogues

Since every patient responds differently to drug combinations, amounts and types of drugs must be carefully monitored and often changed. For patients who do not respond to drug therapy, the other alternatives are heart-lung or lung transplantation. However, transplantation can lead to complications that could result in death.

Statistics:

The Montefiore Medical Center states that the first recorded case of primary pulmonary hypertension occurred in 1891. Each year in the United States, an estimated 500 to 1,000 new cases are diagnosed, most of them women between the ages of 20 and 40. However, both genders and any age can develop PPH. According to the American Lung Association, there were 3,065 deaths attributed to PPH in 2000. It can also be a genetic disorder―familial primary pulmonary hypertension―passed on by a parent. If a parent is known to have PPH, genetic counseling is recommended. According to Ronald J Oudiz, MD, “IPAH is responsible for approximately 125-150 deaths per year”.

Life expectancy:

Before 1990, the diagnosis of PPH was virtually a death sentence, with little hope of prolonging life. Since then, with the advances in new treatments and drug therapies, patients with PPH may have an increased life expectancy. Untreated, the survival rate is about 68% at one year, 48% at three years, and 34 % at five years, according to the University of North Carolina at Chapel Hill Pulmonary & Critical Care Medicine. With treatment, life expectancy can increase on average 3-5 years.

Follow-Up Treatments:

As with any disease, a diagnosis of pulmonary hypertension requires counseling for all involved―the patient and family. Having all the facts and knowing what treatment options are available and what lifestyle changes to make are vital to the continued health of the patient. For more information, please visit the National Heart Lung and Blood Institute or any of the other resources for heart and lung care.

Cheryl Kaye Tardif has worked in the past as a journalist and book editor. Currently, she is the author of three novels: The River, Divine Intervention and the bestselling novel Whale Song, a novel that includes a character with PPH. Cheryl spent over a month researching PPH and this article is the result of her findings. Besides PPH, Whale Song deals with many controversial and emotional issues, including the assisted death of a loved one.

For more information on Cheryl and Whale Song, please visit http://www.whalesongbook.com.