Every Day

January - April 2008 Issue

Many Conditions May Cause Pulmonary Hyptertension

Francisco Javier Soto, MD, MS

Medical College of Wisconsin Pulmonologist
Named one of the “Best Doctors in America®” 2007 by Best Doctors, Inc.

Pulmonary hypertension (PH) — high blood pressure in the lungs — is a serious chronic illness. Up until a decade ago, most patients who had severe PH faced only two possible outcomes: a lung transplant or death. Today, a diagnosis of PH doesn’t necessarily mean a person can’t lead an active life or live for many years. Dr. Francisco Soto discusses the causes of pulmonary hypertension and newer treatment options available.

Q. What is pulmonary hypertension?

Pulmonary hypertension (PH) is abnormally high blood pressure in the lungs. It occurs when the lung vessels become narrowed and cannot carry enough blood. This, in turn, increases the blood pressure in the vessels and makes the heart work harder to pump blood. Without treatment to open the narrowed vessels, the heart will ultimately fail.

Q. Who is at risk for PH?

PH is currently divided into several groups. The most important and serious one, pulmonary arterial hypertension (PAH), can occur secondary to other causes. This group includes patients with idiopathic PH (of unknown origin), formerly called “primary PH.” In this case, PH occurs without an underlying illness and affects about six in one million people.

Other causes of PAH include collagen vascular disease such as scleroderma, lupus or rheumatoid arthritis. Patients with scleroderma — a chronic condition that causes inflammation and thickening of the skin and other organs — have a 30 percent to 60 percent chance of developing PH at some point in their lives. People with chronic liver disease have about a one in 200 chance of developing PAH. HIV infection also increases the risk and about one in 100 people with HIV disease will get PAH, regardless of their HIV disease status. Patients born with a congenital heart defect also have a higher risk of developing PAH.

People at risk for other PH causes (less severe than the PAH category) include those with advanced lung disease, chronic left-heart disease, severe sleep apnea, blood clots in the lungs, and a family history of PH or conditions that may cause PH.

Q. What are the signs and symptoms of PH?

Symptoms include progressive shortness of breath with activities of daily living that progresses to breathlessness even with minimal activity, fatigue, chest pain, dizzy spells, fainting and leg swelling. The severity of PH symptoms varies widely among patients.

Q. How is PH diagnosed?

A diagnosis usually begins when a physician suspects a patient has PH based on progressive shortness of breath that cannot be explained by other medical conditions. Lack of response to therapies used to treat common causes of shortness of breath such as asthma or deconditioning (lack of exercise) may also raise a red flag.

PH can be difficult to diagnose because the initial symptoms can be non-specific and may be confused with other conditions. Therefore, it is often diagnosed late in many cases after it has progressed to an advanced stage. Fortunately, in recent years an increased public and physician awareness of this disease has led to a much earlier diagnosis in many of the cases we see.

If the suspicion for PH is present, the patient’s physician will typically order an echocardiogram to test the blood pressure in the lungs. This is a heart test that uses sound waves to create a picture of the heart and its chambers and evaluate the blood pressure inside the heart and the lungs.

If the blood pressure is high, the patient, hopefully, will be referred to a physician who specializes in this disease. Froedtert & the Medical College of Wisconsin have the largest academic PH program in Wisconsin. Our valued referring physicians request evaluation for patients from places as far away as northern Wisconsin, the Upper Peninsula of Michigan and Illinois.

When we evaluate patients with suspected PH, in addition to confirming if PH is present, we also try to identify the disease that may be causing it. This requires additional evaluation such as testing for blood clots in the lungs, assessing lung capacity with a breathing test or testing for sleep apnea. Most patients will ultimately undergo a cardiac catheterization to directly measure the blood pressure in the heart and lungs. This information is used to determine the best possible treatment.

Q. What treatments are available for pulmonary hypertension?

While PH is typically a chronic condition for which there is no cure, new treatments and continued research are helping patients feel better and live longer. The medication choice depends on the severity of the disease, the level of damage caused and the blood pressure measurements obtained during the heart catheterization.

For patients in the PAH group — the more severe type of PH — many new treatments have become available in the last 10 years that greatly improve long-term survival rates and quality of life.

In general, three types of medication may be prescribed for patients with more severe PH — oral medications, inhaled medications and infusion therapies in which the drug is continuously pumped into a large vein or underneath the abdominal skin via a tiny plastic needle. An external small pump is worn by the patient 24 hours a day.

Other treatments and medications that may be also used include oxygen, anticoagulants (blood thinners), diuretics (water pills) and blood pressure medications.

Because of the many possible causes of PH and the need to address each cause individually, patients may also need treatment for the underlying disease causing PH. We may consult with other specialists here, such as cardiologists, gastroenterologists and rheumatologists, and refer patients to them when appropriate.

Q. What are the short- and long-term outcomes for people who have pulmonary hypertension?

The survival rate varies with the underlying disease. For example, only half of the patients with severe idiopathic (primary) PH (“primary PH”) will be alive after five years of diagnosis if they do not receive treatment. About 40 percent of patients with scleroderma will be alive after three years without treatment. For people with HIV who get PH, only 20 percent will be alive after three years without PH therapy.

When PH therapy is started, we expect that over 80 percent of patients with PAH will survive longer than five years. This number will hopefully continue to increase as newer therapies become available.

Today, many patients who receive treatment are able to enjoy a good quality of life several years after a PH diagnosis. However, since most PH medications are relatively new, we don’t yet have long-term information to guarantee this. The experience accumulated during the last few years gives us hope that PH can be effectively managed over a person’s life for a large number of patients.

Although most patients do well with medication, some may need lung transplantation, provided they are eligible for it.

Q. Can I minimize my risk for getting pulmonary hypertension?

If you experience shortness of breath, especially if it is progressive, don’t minimize this by saying, “I’m just getting old,” or “I’m out of shape.” Encourage your doctor to look into the cause of your shortness of breath and check for serious underlying reasons. As a cause of progressive breathlessness, PH should be in your physician’s radar, especially in patients with medical conditions such as scleroderma, left-heart disease, history of blood clots or other high-risk condition. Physicians should explore the symptoms and consider a referral to a PH specialist whenever suspicion is high or if an echocardiogram supports this diagnosis. The sooner PH is detected, the better the response to treatment and its impact on long-term prognosis.

Source: Every Day

Date: Jan - April 2008 Issue