DRUGS & CONDITIONS

WHAT IS PULMONARY ARTERIAL HYPERTENSION?

Topics:

First, a few words of reassurance. It's natural to feel confused and anxious after being diagnosed with pulmonary arterial hypertension. The condition is not exactly a household word, and it is a serious illness. Pulmonary hypertension often requires treatment by specialists at large medical centers with specific expertise. Being diagnosed with the condition may also require you to make significant changes in your life.

But keep in mind that many people with pulmonary arterial hypertension can manage the disease and live their lives quite normally. New advances in the understanding of this condition and how to treat it have changed the outlook dramatically in recent years.

What's the difference between hypertension and pulmonary hypertension?

"Pulmonary" is from the Latin root "pulmo," or "the lung." "Hypertension" means high blood pressure. Pulmonary hypertension is a condition caused by abnormally high blood pressure in the arteries that supply the lungs with blood.

Most people are familiar with blood pressure as measured with a cuff that goes around the upper arm. This simple test charts the pressure in blood vessels in the body's extremities. If elevated, this is referred to simply as "hypertension."

Pulmonary hypertension, in contrast, is abnormally high blood pressure in just one part of the body: the vessels that supply blood to the lungs. Normally, the pressure in these vessels is much lower than that in the rest of the body. Typical blood pressure measured with a cuff around your arm is 120/80 mm Hg. Normal pulmonary artery blood pressure is only about 25/15 mm Hg.

What is pulmonary arterial hypertension, and how does it affect the body?

The World Health Organization divides pulmonary hypertension into five groups, based on causes of the disease. Group 1 is pulmonary arterial hypertension, or PAH; until recently, it was known as primary pulmonary hypertension. Groups 2-5 are collectively known as secondary arterial hypertension.

Like other forms of pulmonary hypertension, PAH occurs when blood pressure in the pulmonary arteries is dangerously high – more than 15 mm Hg at rest and more than 30 mm Hg during physical activity. Pulmonary arterial hypertension includes cases of PAH with no known cause, PAH that’s inherited, and PAH that’s caused by conditions such as HIV infection, certain liver and thyroid diseases, congenital heart disease (heart disease that’s present at birth), and the use of certain diet medications or street drugs such as cocaine.

Regardless of the cause, all types of pulmonary hypertension can take a harmful course without treatment. When pulmonary blood pressure becomes abnormally high, it can seriously damage the heart and lungs. Pulmonary arterial hypertension often occurs when blood vessels become narrowed or blood flow through these vessels is hindered. The pressure of blood moving through the constricted passage increases, just the way water pressure increases if you hold your thumb over the end of a garden hose. Elevated blood pressure can increase the risk of injury to blood vessels. It also puts an extra strain on the heart, which has to work harder to pump blood through narrowed or partially blocked vessels.

In the case of pulmonary hypertension, the right ventricle (the muscle of the right side of the heart that pumps blood to the lungs) has to work harder. The added strain causes the muscle to enlarge and become less efficient.

The extra pressure within the vessels also puts strain on their inner walls, causing them to become stiffer and thicker. Some vessels may gradually become completely blocked. Other complications, such as low oxygen levels, can also ensue. In some people the body begins to produce more red blood cells to make up for lowered levels of oxygen in the blood. This causes blood to become thicker and stickier, further increasing the load on the heart and raising the risk of blood clots.

New insights, new treatments

Pulmonary arterial hypertension is a challenging illness. But there is much that doctors can do to control the problem and treat the symptoms. Newly developed drugs can lower pulmonary blood pressure and reduce the strain on heart muscles. This can allow people with pulmonary hypertension to lead longer and more active lives. Thanks to these advances, the death rate from pulmonary hypertension has dropped significantly in the last few years. Understanding all you can about pulmonary hypertension is an important part of a comprehensive treatment plan.

-- Peter Jaret is a contributing editor for Health magazine and a winner of the American Medical Association's award for medical reporting. His work has appeared in National Geographic, Newsweek, Hippocrates, and many other national magazines. He is also the author of In Self-Defense (Harcourt Brace Jovanovich), Active Living Every Day, and Heart Healthy for Life.

References

Pulmonary hypertension. Types of pulmonary hypertension. National Heart Blood and Lung Institute. August 2009.

Budev, M.M. et al. Diagnosis and evaluation of pulmonary hypertension. Cleveland Clinic Journal of Medicine, April 2003, pp S9-S27

Nauser, T.D., MD, and Stites, S.W., MD. Diagnosis and treatment of pulmonary hypertension. American Family Physician, May 1, 2001, pp 1789-1798

Primary Pulmonary Hypertension. National Institutes of Health, National Heart, Lung, and Blood Institute, Division of Lung Diseases

Advances in Pulmonary Hypertension. Journal of the Pulmonary Hypertension Association, Autumn 2002

Nauser, T.D., MD, and Stites, S.W., MD. Pulmonary Hypertension: New Perspectives. Medscape, www.medscape.com

Nauser, T.D., and Stites, S.W., MD. Pulmonary Hypertension: new perspectives. Congestive Heart Failure, 2003. May-June; 9 (3):155-62.

Farber, H.W. Pulmonary Hypertension. The Merck Manual, Second Home Edition.

Pulmonary Hypertension. The American Heart Association, www.americanheart.org

Pulmonary Hypertension Association, www.phassociation.org

The Rush Heart Institute Center for Pulmonary Heart Disease, www.rush.edu

National Heart, Lung, and Blood Institute. What Is Pulmonary Arterial Hypertension? August 2006. http://www.nhlbi.nih.gov/health/dci/Diseases/pah/pah_what.html


Reviewed by Trenton D. Nauser, MD, FACP, FCCP, who practices pulmonary and critical care medicine at the Veterans Administration Medical Center in Kansas City, Missouri. He also serves as an assistant professor of medicine at the University of Kansas Medical Center, Kansas City, Kansas.

Last updated August 18, 2009

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

WHO'S AT RISK FOR PAH?

Topics:

Because pulmonary hypertension is a complex condition that can result from a wide variety of factors, it shows up in many different kinds of patients. Pulmonary hypertension -- high blood pressure in the arteries supplying blood to the lungs -- has been diagnosed in newborns and in older people, in men and women, and in people with other serious health problems as well as those who have no other diseases.

Pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) can strike at any age, although it occurs most often among people 20 to 40 years old, according to the National Institutes of Health. The disease – also known as primary pulmonary hypertension -- affects people of all racial and ethnic origins equally. During childhood, it affects both males and females equally. After puberty, PAH strikes women almost twice as often as men. Why that gender difference exists is a mystery.

The cause of some cases of pulmonary arterial hypertension is unknown, but researchers do know that the condition can run in families. About 6 percent of patients diagnosed with pulmonary arterial hypertension are found to have at least one other family member with the disease. Because of this inherited risk, experts may recommend that all first-order relatives of a patient (siblings, children, and parents) be screened for the disease. The recommended test is an echocardiogram, which is relatively simple and painless. A device that resembles a microphone is placed against the chest. Using sonic waves, the device creates a moving picture of the heart. By studying the image, doctors can estimate pulmonary artery blood pressure. They can also detect changes characteristic of pulmonary hypertension, such as an enlarged right ventricle in the heart.

Appetite suppressants -- most recently, fenfluramine and dexfenfluramine -- have also been definitely associated with the onset of the disease, as has the use of street drugs such as cocaine. Congenital heart disease – heart disease that shows up at birth – can affect the way blood flows through the chambers of the heart, causing elevated pulmonary blood pressure. This is usually diagnosed in childhood, but may go undetected until later in life. Other possible causes of PAH include HIV infection and certain liver or thyroid diseases.

Experts have speculated that people who develop the disease have blood vessels that are abnormally sensitive to certain factors, including cocaine, HIV/AIDS, and a form of human herpes, HHV-8 (one that targets blood vessels and is unrelated to the herpes that causes cold viruses). Researchers at the University of Colorado Health Sciences Center in Denver, for example, reported that some patients with pulmonary hypertension were infected with HHV-8.

Secondary pulmonary hypertension

Because it is the result of other medical conditions, including heart and lung diseases that often take decades to develop, secondary pulmonary hypertension (SPH) tends to occur later in life than primary pulmonary hypertension. A wide range of underlying conditions can lead to SPH, including chronic obstructive pulmonary disease, sleep disorders such as sleep apnea, sickle cell disease, diseases that cause lung scarring, mitral valve disease, blood clots, collagen vascular diseases such as scleroderma or rheumatoid arthritis, and tumors that press on the pulmonary blood vessels.

-- Peter Jaret is a contributing editor for Health magazine and a winner of the American Medical Association's award for medical reporting. His work has appeared in National Geographic, Newsweek, Hippocrates, and many other national magazines. He is also the author of In Self-Defense (Harcourt Brace Jovanovich), Active Living Every Day, and Heart Healthy for Life.

References

Pulmonary hypertension. Types of pulmonary hypertension. National Heart Lung and Blood Institute, Nationals Institutes of Health. August 2009.

Cool, C.D. et al. Expression of human herpesvirus 8 in primary pulmonary hypertension. New England Journal of Medicine, Sep 2003, pp 107-109.

Budev, M.M. et al. Diagnosis and evaluation of pulmonary hypertension. Cleveland Clinic Journal of Medicine, April 2003, pp S9-S27.

Nauser, T.D., MD, and Stites, S.W., MD. Diagnosis and treatment of pulmonary hypertension. American Family Physician, May 1, 2001, pp 1789-1798.

Primary Pulmonary Hypertension. National Institutes of Health, National Heart, Lung, and Blood Institute, Division of Lung Diseases.

Advances in Pulmonary Hypertension. Journal of the Pulmonary Hypertension Association, Autumn 2002.

Nauser, T.D., MD, and Stites, S.W., MD. Pulmonary Hypertension: New Perspectives. Medscape, www.medscape.com

Farber, H.W. Pulmonary Hypertension. The Merck Manual, Second Home Edition.

Pulmonary Hypertension. The American Heart Association, www.americanheart.org

Pulmonary Hypertension Association, www.phassociation.org

The Rush Heart Institute Center for Pulmonary Heart Disease, www.rush.edu

Mayo Clinic. Pulmonary Hypertension: Causes. February 2006. http://www.mayoclinic.com/health/pulmonary-hypertension/DS00430/DSECTION=3

Pulmonary hypertension associated with sickle cell disease: pathophysiology and rationale for treatment. Benza RL. Lung. 186(4):247-54. July-Aug 2008.


Reviewed by Trenton D. Nauser, MD, FACP, FCCP, who practices pulmonary and critical care medicine at the Veterans Administration Medical Center in Kansas City, Missouri. He also serves as an assistant professor of medicine at the University of Kansas Medical Center, Kansas City, Kansas.

Last updated August 18, 2009

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

WHAT CAUSES PAH?

Topics:

Pulmonary hypertension, or PH, is abnormally high pressure in the pulmonary arteries. These arteries carry blood from your heart to your lungs to gather oxygen.

The World Health Organization divides pulmonary hypertension into five groups. Group 1 is primary arterial hypertension, or PAH. It's a rare disease; only 1,000 new cases are diagnosed in the United States each year. Groups 2-5 are sometimes called secondary pulmonary hypertension.

What causes pulmonary arterial hypertension?

Researchers do not know what causes some cases of pulmonary arterial hypertension. One reason it remains a mystery is that it affects so few people. Its rarity makes it difficult for scientists to learn more about it. In the last four decades, two puzzling outbreaks of pulmonary hypertension led medical sleuths to a surprising suspect: appetite suppressants. Between 1967 and 1973, the number of recorded cases of pulmonary hypertension jumped tenfold in Europe. Medical detectives traced the outbreak to a weight-loss drug called aminorex fumarate, which was introduced in 1965 in Europe. When the drug was withdrawn, the number of cases fell. More recently, health experts in the United States detected another spike in cases of primary pulmonary hypertension. The epidemic was traced to two new prescription weight-loss drugs, fenfluramine and dexfenfluramine. Both have since been withdrawn from the market.

Some experts speculate that people who develop the disease have blood vessels that are abnormally sensitive to certain factors. Cocaine use, the HIV virus, and the diet drugs noted above are among the suspected factors, but there are no doubt others. In some people, these factors may make the lining of the pulmonary arteries particularly susceptible to damage. However, most people with these factors don't go on to develop pulmonary hypertension.

Why some people are at increased risk of PAH is largely unknown. Women are at significantly higher risk than men, but why women are more susceptible remains a puzzle.

The genetic link to this mystifying illness is better known. Scientists have learned, for example, that pulmonary arterial hypertension sometimes runs in families. Researchers have identified the gene involved, which is called BMPR2. (There may be other genes involved that aren’t yet identified.) Because of the family connection, doctors often recommend that other close relatives of the family be tested when one person has been diagnosed with PAH. But inherited PAH is exceedingly rare. Fewer than 100 families in the United States have been found in which the disease has occurred in at least two first-degree relative (parent, child, sibling). The likelihood of a first-degree relative being affected when only one person in the family is known to have pulmonary arterial hypertension is estimated to be 0.6 to 1.2 percent, according to a WHO-sponsored world symposium on the disease.

Research into the genetic factors linked to PAH has begun to offer clues about what may cause the disease. Mutations of the BMPR2 gene interrupt normal signals to cells in blood vessel linings, causing them to multiply abnormally. In turn, the inner walls and the smooth muscles of pulmonary arteries thicken, researchers speculate. Scarring, or fibrosis, can also occur.

Other causes of PAH include congenital heart disease (heart disease present at birth), HIV infection, liver and thyroid disease, and the use of certain street drugs such as cocaine.

Pulmonary arterial hypertension of unknown origin (also known as idiopathic PAH, or IPAH) is diagnosed when doctors are unable to identify other medical conditions that might be causing the dangerously high blood pressure in pulmonary arteries. As a result, it remains a difficult disease to treat. Physicians can try to alleviate the symptoms and ease the strain on the heart and lungs by lowering the pulmonary blood pressure.

What causes secondary pulmonary hypertension?

Secondary pulmonary hypertension is far more common than PPH. Because it frequently goes undiagnosed, researchers don't know precisely how many people suffer from secondary pulmonary hypertension. Most cases are only recognized when the symptoms become serious enough for people to seek medical attention.

Causes of secondary pulmonary hypertension can include autoimmune disorders such as lupus or scleroderma, conditions that affect the left side of the heart, COPD, emphysema, pulmonary fibrosis, sarcoidosis, blood clots in pulmonary arteries, sickle cell disease, sleep disorders such as sleep apnea, tumors that press on the pulmonary blood vessels, and other conditions. In some cases, doctors can treat the underlying problem and reduce blood pressure in the pulmonary arteries. In others, they treat the symptoms of elevated pulmonary pressure.

-- Peter Jaret is a contributing editor for Health magazine and a winner of the American Medical Association's award for medical reporting. His work has appeared in National Geographic, Newsweek, Hippocrates, and many other national magazines. He is also the author of In Self-Defense (Harcourt Brace Jovanovich), Active Living Every Day, and Heart Healthy for Life.

References

Pulmonary hypertension. Types of pulmonary hypertension. National Heart Lung and Blood Institute. August 2009.

Budev, M. M. et al. Diagnosis and evaluation of pulmonary hypertension. Cleveland Clinic Journal of Medicine, April 2003, pp S9-S27.

Nauser, T.D., MD, and Stites, S.W., MD. Diagnosis and treatment of pulmonary hypertension. American Family Physician, May 1, 2001, pp 1789-1798.

Primary Pulmonary Hypertension. National Institutes of Health, National Heart, Lung, and Blood Institute, Division of Lung Diseases.

Advances in Pulmonary Hypertension. Journal of the Pulmonary Hypertension Association, Autumn 2002.

Nauser, T.D., MD, and Stites, S.W., MD. Pulmonary Hypertension: New Perspectives. Medscape, www.medscape.com

Farber, H.W. Pulmonary Hypertension. The Merck Manual, Second Home Edition.

Pulmonary Hypertension. The American Heart Association, www.americanheart.org

Pulmonary Hypertension Association, www.phassociation.org

The Rush Heart Institute Center for Pulmonary Heart Disease, www.rush.edu

Dramer, M.S. and D.A. LaneA. Aminorex, dexfenfluramine, and primary pulmonary hypertension. Journal of Clinical Epidemiology, April 1998. 51 (4): 361-4.

Vivero, L.E., et al. A close look at fenfluramine and dexfenfluramine. Journal of Emergency Medicine. March-April 1998. 16 (2): 197-205

Rich S et al. Primary pulmonary hypertension: A national prospective study. Annals of Internal Medicine. 1987: 107:216-223.

Rich S et al. Executive summary from the World Symposium on PPH, co-sponsored by the World Health Organization, 1998, Evian, France.

American Heart Association. Your High Blood Pressure Questions Answered: Pulmonary Hypertension. April 22, 2008.


Reviewed by Trenton D. Nauser, MD, FACP, FCCP, who practices pulmonary and critical care medicine at the Veterans Administration Medical Center in Kansas City, Missouri. He also serves as an assistant professor of medicine at the University of Kansas Medical Center, Kansas City, Kansas.

Last updated August 18, 2009

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

HOW IS PULMONARY ARTERIAL HYPERTENSION DIAGNOSED?

Topics:

Diagnosing pulmonary arterial hypertension (PAH) can be difficult. PAH includes pulmonary hypertension that’s inherited or of unknown origin; it also includes cases of pulmonary hypertension linked to congenital heart disease, HIV, the uses of certain diet or street drugs (such as cocaine), and thyroid disorders. Like other types of pulmonary hypertension, it’s marked by a mean pulmonary arterial pressure greater than 25 mmHg at rest and greater than 30 mmHg during exercise.

Early symptoms, such as shortness of breath or fatigue, resemble those of many other conditions. Even a routine medical examination may not reveal the first stages of the disease, which is marked by abnormally high blood pressure in the arteries that supply the lungs with blood. As a result, PAH is frequently advanced by the time it is detected.

When doctors suspect pulmonary arterial hypertension, they begin by taking a careful medical history. Doctors typically ask about symptoms like fatigue, shortness of breath, chest pain, or fainting spells. They also discuss previous medical conditions, drug use, and family history of disease. A physical exam done in a doctor's office can also provide important clues. Doctors inspect the jugular vein in the neck for increased pressure, for instance. There are also telltale heart sounds doctors can detect through a stethoscope that suggest pulmonary hypertension. They may note enlargement of the liver or swelling of the legs or feet, all of which suggest advanced PAH.

Typically, since the symptoms and physical signs of pulmonary arterial hypertension are not specific to any disease, physicians would generally order an ECG (electrocardiogram) and chest X-ray for further evaluation of the findings. If the chest X-ray or ECG doesn't turn up another unrelated disorder (such as heart attack, a heart rhythm problem, or pneumonia), and the clinician continued to suspect pulmonary hypertension, he or she would next order an echocardiogram. The "echo," as it is called, is the most useful screening test pulmonary hypertension. If the "echo" showed evidence of PH, they would then try to determine the underlying cause of the disease with further testing.

First steps

Here are the first steps in the orderly diagnosis of pulmonary arterial hypertension and other types of PH:

When doctors identify pulmonary hypertension on the "Echo," they first try to discover if there are any underlying causes. If so, it is much easier to treat. Doctors diagnose pulmonary arterial hypertension when tests show that blood pressure in the pulmonary artery is abnormally high but there is no evidence of other conditions that might explain it.

A wide range of tests are available to help experts further evaluate different types of pulmonary hypertension. Each provides specific information. Doctors frequently do several tests in order to get a full picture of a patient's condition. The most commonly performed procedures include:

New tests under investigation

In addition to these commonly used tests, researchers are investigating other approaches that could help doctors diagnose and monitor the disease. One promising technique may require only a simple blood test. Researchers at the University of Colorado School of Medicine's Division of Pulmonary Sciences and Critical Care Medicine reported that patients with pulmonary arterial hypertension have abnormally high levels of endothelial cells in their blood. That finding makes sense: Endothelial cells are the cells that line the inner walls of arteries. These are the cells that begin to proliferate abnormally with pulmonary hypertension, causing the stiffening of arteries. Some of these abnormal cells break loose and circulate in the blood. Researchers hope they may eventually be able to follow the progress of the disease more precisely by measuring the level of these cells in the blood.

In addition, the discovery of a gene linked to pulmonary arterial hypertension represents a big step in understanding what causes the disease. However, even if a child inherits the abnormal gene, the likelihood of developing familial pulmonary hypertension is only about 20 percent. Because of this, and because medical screening recommendations remain the same for those with the gene, at this stage most experts advise against genetic tests to identify the gene. Researchers still have much to learn about how the presence of this gene interacts with other factors to cause the disease.

If you have been diagnosed with pulmonary hypertension, your doctor is likely to recommend that some of the more common tests be performed from time to time. Tests such as the echocardiogram or 6-minute walk tests (the distance you can walk in six minutes) are used to monitor your condition and the progress of the disease. If you have any questions about how a test is done or why it has been prescribed, ask your doctor.

-- Peter Jaret is a contributing editor for Health magazine and a winner of the American Medical Association's award for medical reporting. His work has appeared in National Geographic, Newsweek, Hippocrates, and many other national magazines. He is also the author of In Self-Defense (Harcourt Brace Jovanovich), Active Living Every Day, and Heart Healthy for Life.

References:

Budev, M. M. et al. Diagnosis and evaluation of pulmonary hypertension. Cleveland Clinic Journal of Medicine, April 2003, pp S9-S27

Nauser, Trenton D., MD, and Stites, Steven W., MD. Diagnosis and treatment of pulmonary hypertension. American Family Physician, May 1, 2001, pp 1789-1798

Primary Pulmonary Hypertension. National Institutes of Health, National Heart, Lung, and Blood Institute, Division of Lung Diseases

Advances in Pulmonary Hypertension. Journal of the Pulmonary Hypertension Association, Autumn 2002

Nauser, T.D., MD, and Stites, S.W., MD. Pulmonary Hypertension: New Perspectives. Medscape, www.medscape.com

Farber, H.W. Pulmonary Hypertension. The Merck Manual, Second Home Edition.

Pulmonary Hypertension. The American Heart Association, www.americanheart.org

Pulmonary Hypertension Association, www.phassociation.org

The Rush Heart Institute Center for Pulmonary Heart Disease, www.rush.edu


Reviewed by Trenton D. Nauser, MD, FACP, FCCP, who practices pulmonary and critical care medicine at the Veterans Administration Medical Center in Kansas City, Missouri. He also serves as an assistant professor of medicine at the University of Kansas Medical Center, Kansas City, Kansas.

Last updated November 25, 2008

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

WHAT CAN YOU EXPECT?


One of the hardest aspects of living with pulmonary arterial hypertension is dealing with uncertainty. Doctors have gained many important insights into this condition and its treatment. Still, it is difficult for physicians to predict what the future course of the disease will be in an individual patient. In some people, the condition remains mild and poses little danger. In others, pulmonary hypertension is life-threatening. Some people respond well to medications like calcium channel blockers; others need more complex treatments.

The prognosis for secondary pulmonary hypertension has an especially broad range, because it can be caused by such a wide variety of underlying conditions. If doctors are able to treat the condition that is producing pulmonary hypertension, then the right ventricle may recover. The heart can become stronger and the symptoms of pulmonary hypertension can be relieved.

If the underlying cause is chronic blood clots in the pulmonary arteries, for instance, treatments can effectively remove these obstructions and prevent future clots. In such a case, the disease may have no effect on life expectancy. If the underlying cause is a collagen vascular disease like scleroderma, however, there is less doctors can do, and the prognosis is worse.

Like other forms of the illness, pulmonary arterial hypertension is a progressive, often fast-moving disease. This means it’s crucial to get treatment, as symptoms will worsen over time. In general, the best prognosis is in people with mild symptoms, good heart function, and pulmonary arteries that will relax in response to various drugs. After taking a full medical history, performing a physical exam, and running the appropriate tests, your doctor will be able to give you the best idea of what you can expect. Your doctor is likely to prescribe procedures like the 6-minute walk test or echocardiograms from time to time to monitor your condition and the effectiveness of drugs you take. Results from tests will also give you a good sense of what you can expect later.

Pulmonary hypertension is a serious, sometimes life-threatening condition. Still, it is important to remember that the prognosis for people with pulmonary hypertension has improved dramatically in the past decade, thanks to new drugs that reduce blood pressure in the lungs. Prospects are likely to improve even more as new drugs under investigation become available.

Many clinical trials are under way to test combinations of existing drugs and new therapies. A tremendous range of research is also being conducted on ways to treat the underlying conditions that can lead to pulmonary hypertension. Advances in these areas could lead to new treatments and perhaps even cures. Farther down the road, gene therapies may become available. For more information on clinical trials, click here.

-- Peter Jaret is a contributing editor for Health magazine and a winner of the American Medical Association's Award for medical reporting. He is the author of In Self-Defense (Harcourt Brace Jovanovich), Active Living Every Day, and Heart Healthy for Life.

References:

Pulmonary Arterial Hypertension. National Institutes of Health, National Heart, Lung, and Blood Institute, Division of Lung Diseases

Pulmonary Hypertension. The American Heart Association, www.americanheart.org

Pulmonary Hypertension Association, http://www.phassociation.org

The Rush Heart Institute Center for Pulmonary Heart Disease, http://www.rush.edu

The Mayo Clinic, www.mayoclinic.com


Reviewed by Trenton D. Nauser, MD, FACP, FCCP, who practices pulmonary and critical care medicine at the Veterans Administration Medical Center in Kansas City, Missouri. He also serves as an assistant professor of medicine at the University of Kansas Medical Center, Kansas City, Kansas.

Last updated August 21, 2009

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

PULMONARY ARTERIAL HYPERTENSION IN INFANTS AND CHILDREN

Topics:

Pulmonary hypertension typically shows up in middle age. Secondary pulmonary hypertension associated with diseases like scleroderma is also usually diagnosed in adults. But pulmonary hypertension can also occur in infants and children.

There are a variety of causes. In infants, a condition called persistent pulmonary hypertension of the newborn is found in about one per 1,000 births. In such cases, a baby's circulatory and respiratory systems fail to make the normal transition at birth. In the womb, the baby's lungs aren't needed to get oxygen; the placenta is the fetal organ for gas exchange via the umbilical cord. In utero, a fetus's pulmonary artery sends blood back to the heart and bypasses the lungs. At birth, the pulmonary artery normally begins pumping blood into the lungs so the baby can begin breathing. When this switch-over fails to happen, the result can be persistent pulmonary hypertension of the newborn, or PPHN. This condition is associated with other respiratory diagnoses like pneumonia or meconium in the majority of infants.

Pulmonary hypertension can occur in infants and young children for other reasons as well. A variety of congenital defects in the structure of the heart or its valves can cause elevated blood pressure in the pulmonary arteries, for instance. In rare cases, infants and young children develop pulmonary arterial hypertension of unknown origin, the form of the disease that is not linked to any underlying disease or defect. As with the adult-onset version, researchers don't fully understand what causes this type of pulmonary arterial hypertension in children. An inherited gene has been linked to some cases of PAH. Researchers suspect that patients with the condition have cells in the lining of their artery walls that are unusually sensitive to certain factors.

Recent evidence suggests that the use of selective serotonin-reuptake inhibitors (SSRIs) late in the third trimester of pregnancy may be a risk factor for pulmonary hypertension in newborns. A small study published in the New England Journal of Medicine in 2006 found that pulmonary hypertension was six times more common in babies whose mothers took an SSRI antidepressant after the 20th week of pregnancy. This study prompted the FDA to issue a warning about the use of SSRIs in the last half of pregnancy. However, the FDA advises all women taking anti-depressant medications to talk with their doctor before stopping their medication.

Whatever the cause, pulmonary hypertension in children poses special challenges. In the young, the disease often progresses more rapidly than in middle-aged patients. And while many of the drugs that are proving to be effective in controlling symptoms in adults also work well in children, few controlled studies have been done.

How do doctors diagnose pulmonary hypertension in infants and children?

The symptoms of abnormal pulmonary blood pressure in infants and children are similar to those in adults. They include:

A physical examination by a doctor may turn up other signs of pulmonary hypertension. These include unusual heartbeat sounds sometimes associated with the disease, subtle swelling in the extremities, or an enlarged liver.

Many of the same tests employed to diagnose pulmonary hypertension in adults are used with infants and children when doctors strongly suspect pulmonary hypertension. These include chest X-ray, EKG, arterial blood gas, echocardiograms, and cardiac catheterization. Doctors often perform tests to determine if the cause of PH in children is sleep apnea, a condition caused when breathing is interrupted during sleep.

How is pulmonary hypertension treated in infants and children?

Infants suffering from persistent pulmonary hypertension of the newborn (PPHN) are placed in a neonatal intensive care unit so that they can be monitored and supplied with supplemental oxygen that widens the pulmonary arteries. In some cases, the problem resolves on its own. Other drugs that widen the pulmonary artery, such as nitric oxide, have been shown to be helpful in newborns. In serious cases, surgery may be necessary. Serious treatment is available for several of the associated lung diseases.

Children diagnosed with pulmonary hypertension are treated with many of the same drugs used to treat adults. These include calcium channel blockers, prostacyclins, endothelin-1 inhibitors, and blood-thinning agents. Children with PH sometimes require supplemental oxygen either during the day or while they sleep. Bosentan, a dual endothelin-receptor antagonist, has been found to be safe and helpful in stabilizing the disease in infants and young children with PAH linked to congenital heart disease.

Pulmonary hypertension and your child

Children with any type of pulmonary hypertension must be followed closely by medical specialists. Because the condition is uncommon, that may require travel to a large medical center that specializes in the disease.

Along with treatment for the condition itself, it is important to keep children as healthy as possible to avoid unnecessary strain on the heart and lungs. Doctors recommend an annual influenza vaccination, as well as preventive treatment with palivizumab, which helps protect against respiratory syncytial virus (RSV), a virus hazardous to certain high-risk infants and young children. All respiratory illnesses should be treated quickly and completely. Fevers should also be reduced because they can weaken children and make them more susceptible to breathing difficulties. Depending on the severity of the illness, some doctors recommend keeping supplemental oxygen equipment at home for use in an emergency.

Many but not all children with pulmonary hypertension have to limit their physical activity. A safe level of activity is usually determined by graded exercise testing, which monitors breathing and oxygen consumption. If you have a child with PH, talk to your doctor about the kinds of physical activity that are safe and appropriate.

-- Peter Jaret is a contributing editor for Health magazine and a winner of the American Medical Association's award for medical reporting. His work has appeared in National Geographic, Newsweek, Hippocrates, and many other national magazines. He is also the author of In Self-Defense (Harcourt Brace Jovanovich), Active Living Every Day, and Heart Healthy for Life.

References:

Gilbert, N. et al. Kardiologie. Initial experience with bosentan (Tracleer) as treatment for pulmonary arterial hypertension (PAH) due to congenital heart disease in infants and young children. September 3, 2005.

Haworth, S.G. Pulmonary Hypertension in Children: New Insights Offer Opportunity to Reverse the Disease Process. Advances in Pulmonary Hypertension, phassociation.org

Berger, S., Director of the Heart Center, Children's Hospital of Wisconsin. Primary Pulmonary Hypertension, emedicine.com.

Finer, N.N. et al. Nitric oxide for respiratory failure in infants born at or near term. Cochrane Database Syst Review, 2001.

Maxwell, A.J. et al. Pediatric Primary Pulmonary Hypertension. Current Treatment Options in Cardiovascular Medicine, Oct 2001, 371-383.

Primary Pulmonary Hypertension. National Institutes of Health, National Heart, Lung, and Blood Institute, Division of Lung Diseases

Walsh-Sukys, M, et al. Primary Pulmonary Hypertension of the Newborn in the Era Before Nitric Oxide: Practice Variation and Outcome. Pediatrics, Jan. 2000; 105(1): 14-20.

Chambers, CD, et al. Selective Serotonin Reuptake Inhibitors and Risk of Persistent Pulmonary Hypertension of the Newborn. New England Journal of Medicine. Feb 9, 2006; 354(6): 579-87.

Food and Drug Administration. SSRIs and Treatment Challenges in Depression. July 19, 2006.


Reviewed by Trenton D. Nauser, MD, FACP, FCCP, who practices pulmonary and critical care medicine at the Veterans Administration Medical Center in Kansas City, Missouri. He also serves as an assistant professor of medicine at the University of Kansas Medical Center, Kansas City, Kansas.

Last updated August 20, 2009

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

LIVING WITH PULMONARY ARTERIAL HYPERTENSION

Topics:

Learning that you have pulmonary arterial hypertension (PAH) can be frightening. Like most people, you will probably need a little time to adjust to the news that you have been diagnosed with a serious medical condition. Along with being worried, you may find yourself feeling angry or depressed. That's perfectly natural.

But keep this in mind: Many people with PAH discover that they are able to live active lives, working, raising children, and enjoying life. They don't look sick or even feel sick. As long as they pace their physical activities, they may not feel the symptoms of pulmonary hypertension at all. Even if the condition does cause symptoms that limit what you can do, there are ways to adjust your life so you can go on enjoying your family, friends, hobbies, and other activities.

Following a few simple lifestyle recommendations will help you stay as healthy as you can and control the symptoms of pulmonary hypertension. Some of the suggestions are good advice whether or not you have pulmonary arterial hypertension. Other recommendations are specific to people with it. Here's what to consider:

Watch your diet

Eat a healthy diet with moderate portions. Abundant evidence shows that what you eat plays a big role in cardiovascular health. Also, talk to your doctor about whether you need to restrict salt and fluids in your diet, and whether you need to regulate foods that contain vitamin K. These foods can affect how well blood-thinning medicines work. Following the familiar guidelines for a heart-healthy diet are especially important, given that your heart is under strain.

Make fresh vegetables and fruit, along with whole-grain breads and cereals, the centerpiece of your menu. Help yourself to lean chicken and fish, and go easy on fatty meats. Limit saturated fats by drinking skim or low-fat milk and using vegetable oils instead of butter whenever you can. Avoid hydrogenated oils, also called trans fats. These artificially made fats are found most often in processed foods, including many snack items. Trans fats, like saturated fats, have been shown to increase levels of bad cholesterol and eventually clog coronary arteries.

Watch your weight

If you are overweight, your doctor may recommend that you try to lose weight. The reason: Excess weight puts an extra strain on the heart. Losing weight will also make you feel better and give you more energy. For advice on the healthiest way to lose weight, talk to your doctor.

If you smoke, quit

Smoking poses a severe danger to the heart and lungs, especially for people suffering from conditions like pulmonary arterial hypertension. If you smoke, the best thing you can do is quit. That's not easy, of course, but many people have successfully kicked the habit. Nicotine patches, gum, and other aids can make it easier. Support groups are available in many communities. You'll find useful resources at these Web sites:

http://surgeongeneral.gov/tobacco

http://www.lungusa.org/stop-smoking/

http://www.quitnet.com

For more information about quitting smoking, talk to your doctor.

Avoid secondhand smoke

Being exposed to secondhand smoke has been linked to a variety of health problems, including lung cancer and heart attacks. There is no direct evidence that secondhand smoke worsens symptoms of PAH. Still, experts agree that it's wise to avoid exposure as much as possible.

If you live with someone who smokes, ask them to quit. If they won't quit, ask that he or she smoke outside or in a separate space, such as the garage or back porch. Avoid places frequented by smokers. Choose non-smoking restaurants or the non-smoking sections. On days when air quality is poor, it's also a good idea to remain inside as much as possible.

Stay active

People with pulmonary hypertension often worry that exercise could be dangerous. It's true that overly strenuous exercise can sometimes bring on the symptoms of PAH, such as shortness of breath or dizziness. Exercise that involves lifting anything over 25 pounds can increase blood pressure in the pulmonary arteries, putting unwanted stress on the heart and lungs. Experts advise against heavy weight-lifting exercises for that reason.

At the same time, remaining as physically active as possible is essential to staying healthy when you have pulmonary hypertension.

The appropriate level of activity depends on you: your age, your overall health, and what you enjoy doing. Moderately strenuous activities such as walking are good for your heart and arteries and may have other health benefits, including relieving stress. Even people with advanced PAH are encouraged to walk, using portable oxygen. If walking is too tiring, you can stay invigorated with everyday activities. Even taking a spin in a wheelchair or on a motorized scooter is better than sitting or lying down. If you're not sure how active you should be, talk to your doctor.

Find time to rest

Get plenty of rest and find opportunities to relax. Getting enough rest is the best way to deal with fatigue, which is a common complaint among people with PAH. Most adults need approximately eight hours of sleep each night: Sleep, like diet and exercise, is important for our bodies to function their best. Taking time out to relax will also help you deal with the inevitable stresses of dealing with a serious medical condition.

Stress, especially if it feels out of control, can be hard on your heart. People with pulmonary arterial hypertension often say they feel better after doing simple relaxation exercises, such as closing their eyes and focusing on their breathing. A positive mental attitude can also help you feel better and may even keep you healthier. Of course, it is always important to plan time every day to do something you enjoy or to spend time with people you care for. For more tips on how to fight stress, click here.

Avoid becoming pregnant

Pregnancy can create a dangerous strain on the heart and lungs in women with pulmonary hypertension, endangering both mother and baby. Some of the medications used to treat pulmonary hypertension can also be harmful to the fetus. Doctors advise women of childbearing age with PH not to become pregnant.

Unfortunately, hormonal contraceptives, like birth control pills and patches, increase the risk of blood clots and thus also pose a risk for women with pulmonary hypertension. For that reason, doctors recommend using alternative forms of birth control. Many women with advanced pulmonary hypertension opt for sterilization because of the extreme risks to them if they become pregnant. If you want to have children, you may want to consider adoption.

Avoid living at high altitudes

Because the air is thinner at high altitudes, the heart and lungs have to work harder to get enough oxygen. This can aggravate the symptoms of pulmonary hypertension. Over time, living at high altitudes has the potential to speed the progress of the disease. If you live in a city like Denver or another location at high altitude, your doctor may recommend that you consider moving to a lower elevation, if possible. Many patients with respiratory difficulties find that they breathe more easily and have more energy at low elevations.

Not everyone is affected the same way by residing at elevation, however. Many doctors recommend that patients with PH who live in areas of high elevation spend a week or two at sea level. If they notice that they have an easier time breathing or feel less fatigued, then it's worth considering a move.

Check with your doctor before flying

Flying can pose a hazard for people with PAH, because even in the pressurized cabins of planes the air contains less oxygen than air on the ground. People with advanced PAH may need to travel with supplemental oxygen. Before getting on a plane, talk with your doctor. If he or she thinks you should travel with supplemental oxygen, your doctor will provide the airline with information about your medical condition and the need for oxygen.

The airline's medical team will also evaluate your medical record. Passengers are not allowed to use their own oxygen tanks and equipment. The airline will supply the appropriate equipment. Remember: Airlines have limited seats available for people who need in-flight oxygen. An airline can also refuse to fly passengers whom they deem unfit to travel for health reasons. Finally, make sure that you arrange to have oxygen cylinders and equipment available when you arrive at the airport. The airline is only responsible for providing the equipment on the flight. Oxygen distributors are found in most cities.

Physical exertion during the flight can increase the risk of worsening symptoms of pulmonary arterial hypertension. Avoid drinking excess fluid before the flight in order to prevent trips to the lavatory. But remember to stretch your legs at your seat in order to prevent blood clots from forming in your legs.

Be your own watchdog

Take good care of yourself. If you develop symptoms of respiratory tract infections, see your doctor immediately. Antibiotic therapy for lung infections is especially important for people with pulmonary arterial hypertension. It's also important to get a pneumococcal pneumonia vaccine and yearly flu vaccinations to prevent these respiratory illnesses, which can be very serious in pulmonary hypertension patients.

-- Peter Jaret is a contributing editor for Health magazine and a winner of the American Medical Association's award for medical reporting. His work has appeared in National Geographic, Newsweek, Hippocrates, and many other national magazines. He is also the author of In Self-Defense (Harcourt Brace Jovanovich), Active Living Every Day, and Heart Healthy for Life.

References:

Primary Pulmonary Hypertension. National Institutes of Health, National Heart, Lung, and Blood Institute, Division of Lung Diseases

Nauser, TD and Stites, SW. Pulmonary Hypertension: New Perspectives. Medscape, www.medscape.com

Farber, H.W. Pulmonary Hypertension. The Merck Manual, Second Home Edition.

Pulmonary Hypertension. The American Heart Association, http://www.americanheart.org

Pulmonary Hypertension Association, http://www.phassociation.org

The Rush Heart Institute Center for Pulmonary Heart Disease, http://www.rush.edu

The Mayo Clinic, http://www.mayoclinic.com

National Heart, Lung, and Blood Institute. Living with Pulmonary Hypertension. http://www.nhlbi.nih.gov/health/dci/Diseases/pah/pah_living.html

Mayo Clinic. Pulmonary Hypertension: Lifestyle and Home Remedies. February 2008. http://www.mayoclinic.com/health/pulmonary-hypertension/DS00430/DSECTION


Reviewed by Trenton D. Nauser, MD, FACP, FCCP, who practices pulmonary and critical care medicine at the Veterans Administration Medical Center in Kansas City, Missouri. He also serves as an assistant professor of medicine at the University of Kansas Medical Center, Kansas City, Kansas.

Last updated October 29, 2009

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

PROFILE OF A SURVIVOR

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You have to admire someone who can study for the bar exam without leaving her bedroom. While most aspiring lawyers haunt law libraries, Tishisa Braziel (pronounced "Brazil," like the country) would listen to lectures on CD and read books in her bed. She wanted to spend more time out of the house, but her sick lungs and heart just wouldn't let her. "The whole time I was doing it, I couldn't get Descartes out of my mind," she says. "He was famous for working in bed."

Braziel, a resident of Richmond, Virginia, has pulmonary arterial hypertension, a rare and potentially fatal disease that strains her heart and hampers her breathing. For a while, it also kept her from looking more than two weeks into the future. She feels much better these days --well enough to get out of bed for long stretches -- and, thanks to successful treatments, she now plans as far ahead as any other 33-year-old. She recently passed the bar exam, and now she hopes to become a tax lawyer for the IRS, retire with a government pension, and travel the world. Most of all, she plans on staying alive.

There was a time when people with pulmonary arterial hypertension didn't have much to look forward to. The disease occurs when the main artery in the lungs begins to narrow, forcing the heart to work extra hard to keep blood flowing. The blood pressure in the artery rises, and both the lungs and heart became gradually weaker. Without treatment -- and sometimes even with treatment -- the condition eventually leads to heart failure and death.

Out of formation

Nobody knows what causes pulmonary arterial hypertension, until recently known as "primary pulmonary hypertension". In fact, the term "primary" is doctor code for "something that happens for no apparent reason." Braziel can't say for sure why she got this one-in-a-million disease, but she has her suspicions. She spent the early 1990s in the army, and she was stationed in Turkey during Operation Provide Comfort, a mission that supported Kurdish refugees. She suspects she may have been exposed to something during her service that set her disease in motion.

Without a doubt, her symptoms began to emerge while she was still in uniform. After serving in Turkey, she was stationed at a base in Germany. The daily exercise drills should have kept her in top shape, but she felt like she was sliding downhill. One day, she fell out of formation during a run and promptly passed out. Her sergeant sent her to sick call, but doctors couldn't find anything wrong with her. It would become a recurring theme.

After four years in the army, Braziel returned to civilian life at Mary Baldwin College in Staunton, Virginia. Nobody was asking her to run in formation and shout cadences, and it was easy for her to forget about her physical troubles. She got out of breath walking up the hills on campus, but she told herself she was just out of shape. Things got better when she started law school, mainly because the campus at the University of Virginia is relatively flat.

Elusive diagnosis

The relief was only temporary. Braziel took a year off from school, and by the time she returned, her symptoms were impossible to ignore. "I couldn't walk 10 feet without doubling over for air," she says. "I thought to myself, 'I'm only 28-years-old. Nothing should make me feel like this.' " She went to the Student Health Center, where she was diagnosed with asthma. When asthma medications didn't clear up her symptoms, the doctor strongly implied that her problem was mainly in her head. She kept coping with her "asthma," but life got increasingly difficult. One day, she had to climb a flight of 20 stairs at the university library. She got extremely dizzy at the top and probably would have fallen backwards if someone hadn't grabbed hold of her arm.

It was time to get serious. Braziel promptly scheduled an appointment with a pulmonologist at the University of Virginia. The specialist at first suspected chronic bronchitis or emphysema -- relatively common diseases that block air flow in the lungs -- and the first series of tests showed nothing out of the ordinary.

Perplexed, the pulmonologist decided to put Braziel through one more simple test. He placed an oxygen sensor on her finger and asked her to take about 15 steps down the hall. As Braziel walked, her oxygen saturation level plunged to 56, the type of number one would expect to see in a person who was near drowning. "They told me everything was fine, but they started going crazy," Braziel says. "They put me in a wheelchair and wouldn't let me take another step."

After a battery of more tests, the pulmonologist finally made the proper diagnosis. When he shared the news with Braziel in her hospital room, he gave no hint of the gravity of the situation. "He was so calm I thought pulmonary hypertension must be something like asthma," Braziel recalls. She got the real story when a friend from law school gathered a huge binder of information on the disease and brought it to her.

Short afternoon naps

Several medications can relieve pulmonary arterial hypertension, but no single approach works for every patient. Finding the right treatment usually requires persistent trial-and-error. Braziel's pulmonologist wanted to put her on Flolan (prostacyclin), a drug that is delivered to the heart through a catheter in the chest. Patients must carry a Walkman-sized pump with them at all times. The drug is generally very effective and many patients quickly get used to the pump and the catheter, but Braziel decided to get a second opinion.

After a two-month wait, an expert at Columbia University was finally able to take her case. As part of a series of tests, the pulmonologist gave Braziel calcium channel blockers -- a common type of oral blood pressure medicine -- while monitoring the pressure in her lungs with a computer. As soon as the drugs hit her system, the pressure dropped immediately. Braziel later learned that only 10 to 20 percent of people with PAH respond to calcium channel blockers. She would be one of the lucky ones to have a cheap, easy-to-take, relatively effective treatment for her disease. "I've been totally blessed," she says.

Braziel feels much better than she has in years. She used to be attached to an oxygen tank 24 hours a day; now she only needs extra oxygen when she has a cold or flu. She used to be practically confined to her bed; now she can go to the grocery store and do many chores around the house When she lands her job as a tax lawyer, she expects to be able to keep normal office hours, although she may need short afternoon naps.

As grateful as she feels, her life is far from easy. She takes 10 pills a day, including calcium channel blockers, diuretics (drugs that reduce the water retention caused by calcium channel blockers), and potassium supplements (to replace the nutrients flushed out by the diuretics.) The medications keep her disease in check, but they also make her tired and dizzy. The Veterans Administration pays for a large portion of her medical bills, but she still faces a lifetime of high healthcare costs. And unless treatments improve dramatically, she knows she'll never be as physically active as she'd like.

For Braziel -- and for many other women with her disease -- the biggest disappointment is that she can never have a biological child. Pregnancy would put too much strain on her already weakened heart, and she would almost certainly die before a baby could be delivered. "When they told me that, I literally started shaking," she says. She had always planned on having kids, but for now she's envisioning a family of one. "I'll have to be enough," she says. If that's the way it works out, it will be a remarkable family: one Army veteran, one tax lawyer, and, most of all, one survivor.

-- Chris Woolston, MS, is a health and medical writer with a master's degree in biology. He is a contributing editor at Consumer Health Interactive and was a staff writer at Hippocrates, a magazine for physicians. His reporting on occupational health for CHI earned him an award from the Northern California chapter of the Society of Professional Journalists.

References:

Pulmonary Arterial Hypertension. National Institutes of Health, National Heart, Lung, and Blood Institute, Division of Lung Diseases


Reviewed by Trenton D. Nauser, MD, FACP, FCCP, who practices pulmonary and critical care medicine at the Veterans Administration Medical Center in Kansas City, Missouri. He also serves as an assistant professor of medicine at the University of Kansas Medical Center, Kansas City, Kansas.

Last updated August 20, 2009

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

SURGERY AND PAH

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Can surgery be used to treat pulmonary arterial hypertension?

In some cases of pulmonary arterial hypertension and other forms of the disease, surgery can be useful and even curative.

Surgery is occasionally used to treat the underlying condition that is causing some types of pulmonary arterial hypertension. For instance, an operation to correct certain heart abnormalities, such as congenital heart defects, can eliminate the problem that is producing elevated pulmonary blood pressure.

Surgery to remove blood clots

Some types of secondary pulmonary hypertension also benefit from surgery. If the cause of pulmonary hypertension is blood clots that impair blood flow to the lungs, surgeons can sometimes operate to remove the clots. The operation, called a thromboendarterectomy, is a complicated and risky one; it is used only for patients with severe symptoms. To evaluate whether the operation is appropriate, doctors perform a right heart catheterization and a pulmonary angiogram. These tests allow physicians to gauge the extent of blood clots and to assess whether they can be removed with surgery.

Thromboendarterectomy is an open-heart procedure that is done routinely only at a few specialized centers. Surgeons open the chest through the breast bone. The patient is placed on a heart-and-lung by-pass pump. Opening the main pulmonary artery and its first branches, surgeons remove old blood clots and damaged artery linings. Recovery requires several days or more in an intensive care unit. The operation has a death rate between 5 percent and 10 percent. Blood clots must be prevented lifelong after the operation.

According to the Mayo Clinic, the operation may provide a cure for the particular type of pulmonary hypertension caused by abnormal particles in the blood that fail to dissolve, sticking to the blood vessel wall and obstructing normal blood flow instead; removing them can not only reverse right heart failure, but restore normal pulmonary artery pressures.

Liver transplant for cirrhosis can also be helpful for porto-pulmonary hypertension.

Heart-lung transplants

When all other treatment options have failed, and the lungs have been badly damaged by the disease, lung or heart-and-lung transplants are sometimes performed. A single lung transplant is a common method used in cases of pulmonary arterial hypertension. Some transplant centers prefer to replace both lungs partly because they believe double transplants cause fewer complications (a recent Johns Hopkins study found that patients with various diseases who received a double lung transplant lived twice as long on the average as people who received a single lung.) Combined heart and lung transplants are done less often, because of their increased risk.

Single lung transplants are usually reserved for patients under 65 who are otherwise in good health and double lung transplants for those under 60. (Heart-lung transplants, because of the greater risk they pose, are usually reserved for patients under 55.) However, as the population ages and transplant success rates improve, these recommended age limits may increase. Doctors carefully screen candidates for transplant surgery in order to ensure that the patients who undergo such operations are those who stand the best chance of surviving surgery and benefiting from it.

The survival rate for transplant surgery has been improving dramatically since the operations were first pioneered in the 1980s. (The latest figures can be obtained at http://www.optn.org). One reason lung transplant surgery has proved successful is the surprising ability of the right ventricle to heal itself after pulmonary blood pressure is normalized. Studies have shown that both its structure and function improve after transplantation.

Still, organ transplants of any kind carry serious risks. The most troublesome complication is rejection. This occurs when the immune system reacts to foreign "fingerprints" on the cells of the transplanted organ and attacks them. Untreated, immune rejection mechanisms can destroy a transplanted organ within days. To prevent rejection, doctors prescribe drugs that suppress the immune system. Anti-rejection drugs are usually given by injection during the first several weeks, and in pill form later. Unfortunately, these drugs can make patients more vulnerable to infections. Over time, recipients can usually reduce the dosage of anti-rejection drugs. However, transplant patients must continue to take the drugs for the rest of their lives.

-- Peter Jaret is a contributing editor for Health magazine and a winner of the American Medical Association's award for medical reporting. His work has appeared in National Geographic, Newsweek, Hippocrates, and many other national magazines. He is also the author of In Self-Defense (Harcourt Brace Jovanovich), Active Living Every Day, and Heart Healthy for Life.

References:

Bennett, L.E. et al. Worldwide thoracic organ transplantation: a report from the UNOS/ISHLT international registry for thoracic organ transplantation. Clinical Transplantation, 2001, pp. 25-40.

Budev, M.M. et al. Diagnosis and evaluation of pulmonary hypertension. Cleveland Clinic Journal of Medicine, April 2003, pp S9-S27.

Nauser, T.D., MD, and Stites, S.W., MD.. Diagnosis and treatment of pulmonary hypertension. American Family Physician, May 1, 2001, pp 1789-1798.

Primary Pulmonary Hypertension. National Institutes of Health, National Heart, Lung, and Blood Institute, Division of Lung Diseases.

Nauser, T.D., MD, and Stites, S.W., MD. Pulmonary Hypertension: New Perspectives. Medscape, www.medscape.com.

Farber, H.W. Pulmonary Hypertension. The Merck Manual, Second Home Edition.

Pulmonary Hypertension. The American Heart Association, http://www.americanheart.org.

Pulmonary Hypertension Association, http://www.phassociation.org, August 2009.

The Rush Heart Institute Center for Pulmonary Heart Disease, http://www.rush.edu.

US Department of Health and Human Services. 2007 Organ Procurement and Transplantation Network/Scientific Registry of Transplant Recipients Annual Report: Transplant Data 1997 - 2006. May 1, 2007.


Reviewed by Trenton D. Nauser, MD, FACP, FCCP, who practices pulmonary and critical care medicine at the Veterans Administration Medical Center in Kansas City, Missouri. He also serves as an assistant professor of medicine at the University of Kansas Medical Center, Kansas City, Kansas.

Last updated August 20, 2009

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

NEW ADVANCES IN PAH RESEARCH

Topics:

Remarkable progress has been made in understanding and treating pulmonary hypertension over the past decade. It’s worth noting that until the late 1970s, doctors had almost nothing they could offer patients with pulmonary arterial hypertension (PAH) and other forms of the disease. The prognosis for most patients was poor. Then calcium channel-blocking drugs were shown to improve pulmonary blood flow and ease the symptoms of pulmonary hypertension in some patients, offering them the first treatment option.

In 1995 the Food and Drug Administration approved an entirely new class of drugs, called prostacyclin analogues. These medications, based on substances produced by the body, lessen symptoms, slow the deterioration caused by pulmonary hypertension, and improve prognosis. In 2002 another new class of drugs, called endothelin-1 inhibitors, became available. Now new versions of old drugs, which are easier to take and have fewer side effects, are improving the quality of life for patients with the disease. Meanwhile, other drugs, including sildenafil -- more popularly known as Viagra -- have been approved to treat the condition and are showing great promise.

These new treatments are the result of important advances in basic scientific research. For instance, endothelin-1 inhibitors were developed after scientists discovered a substance, released by cells that line the walls of arteries, that causes arteries to constrict. By creating drugs that block receptors for this substance, pharmaceutical scientists then found a way to keep vessels open and blood flowing.

New research initiatives under way promise even more advances, improving the quality of life and slowing the progress of PAH and other types of pulmonary hypertension. Some of the leading areas of research today include the following:

Assessing the benefits of combined treatments

Now that doctors have several options for treating pulmonary hypertension, clinicians are investigating the benefits of combining different therapeutic approaches. In a report published in 2002 in the Annals of Internal Medicine, for instance, researchers showed that a combination of sildenafil and iloprost, an inhaled form of prostacyclin, lessened the symptoms of pulmonary hypertension more effectively than either drug alone. More recent studies have confirmed these findings. In other research, clinicians are experimenting with combinations of prostacyclin analogues and endothelin-1 inhibitors. Over the next few years, controlled trials should provide doctors with important new information about how to best use existing drugs, either alone or together. For example, researchers are studying the effects of combining standard treatment with drugs commonly used to treat other diseases - such as the anti-cholesterol drug simavastatin and the antidepressant escitalopram. There’s an urgent need for more information about how available drugs affect infants and children with pulmonary hypertension.

Making today's effective drugs easier to take

Many of the effective drugs for pulmonary hypertension are difficult to take and have serious side effects. Epoprostenol, for instance, has to be delivered continuously via an I.V. Another prostacyclin analog, trepostinil, recently approved by the Food and Drug Administration, has to be administered under the skin using a small pump. But now researchers are studying new forms of PH treatment that can be taken in pill form or with inhalers. One clinical trial, for example, is studying an oral form of prostacyclin. This would be an important breakthrough. Ongoing research will continue to make it easier for patients to get the benefits of drugs more easily.

Developing new drugs

Intensive efforts are under way to identify brand-new drugs to stop the progress of pulmonary arterial hypertension and other forms of the disease so that patients can live longer, more active lives. Basic scientific research is likely to lead the way. Not long ago, researchers spotted an enzyme called Rho-kinase, which plays a role in the constriction of blood vessels. A drug that inhibits this enzyme has been developed and is being tested. Japanese scientists at the 13th annual Symposium on Atherosclerosis reported that the drug, called fasudil, was effective in reducing resistance to blood flow in the pulmonary arteries in a small group of patients. Larger studies are continuing.

Meanwhile, in what could prove to be a major breakthrough in basic research, scientists reported the discovery of certain key chemical characteristics in many patients with pulmonary hypertension. The researchers found unusually high levels of a protein produced by smooth muscle cells in pulmonary vessels, called angiopoietin-1. At the same time, they noted very low levels of markers for another protein, called BMPR1A. These results suggest for the first time a common molecular basis to PH. The findings also point out directions for future drug development.

Researchers are also working to better understand the genetics of the disease. They have discovered that people with a BMPR2 gene mutation are predisposed to pulmonary hypertension. Eventually, gene therapies may be able to correct genetic abnormalities that sometimes underlie the condition. Scientists are also studying the role of serotonin in the development of pulmonary hypertension and the potential of serotonin inhibitors to help treat the disease.

Improving surgical options and developing new treatments

For patients whose pulmonary hypertension is caused by small blood clots in the pulmonary artery, an operation called a thromboendarterectomy can offer dramatic benefits. The surgery involves removing these stubborn clots and opening up constricted blood vessels. As surgeons gain more experience performing this complicated and risky procedure, they are improving its success rate dramatically. For example, a technique that involves cooling a patient’s body during surgery appears to improve outcome. Other recent findings suggest that the use of inhaled iloprost, a form of prostacyclin, after surgery may help reduce arterial blood pressure and improve the success of thromboendarterectomies.

A procedure known as balloon atrial septostomy is also promising. In treating primary pulmonary hypertension, this procedure is used to reduce the work load on the right heart by creating a hold between the upper chambers of the left and right heart. This creates a path of lesser resistance for blood flow and allows the right heart to function better.

For selected patients with advanced pulmonary hypertension who don’t benefit from medications, lung or heart-lung transplants represent a last resort. The success rate for these complicated operations has improved dramatically over the past decade as surgeons have gained experience. One of the biggest challenges remains the threat of rejection.

Today, researchers are investigating ways to monitor organ rejection that won’t require invasive biopsies. Improved anti-rejection drugs with fewer long-term side effects are also being tested. Another challenge is developing a more effective and efficient system for getting donated organs to patients in need. At the moment, many patients may wait as long as two years for donor lungs to become available. Hospitals nationwide are working to develop a better system for identifying donors and matching donated organs to patients who need them. The use of the Internet has made it easier to manage the vast amount of information required. Transplant surgeons and patients awaiting organ transplants now have access to expanded data via the national Organ Procurement and Transplantation Network’s Web site at http://www.optn.org.

On the cutting edge of new treatments in development are stem cell gene therapies. In one small study reported in the Journal of Clinical Investigation, researchers discovered that a protein found primarily in cancer cells could also be found in the arteries of patients and rats with pulmonary hypertension. This protein, called survivin, prevents the body from restricting cell growth. In the study, researchers mutated the protein to negate this effect then used a virus to deliver the mutated gene to rats. The rats showed a reversal of pulmonary hypertension and a prolonged survival rate of 25 percent.

Other researchers are examining the regenerative qualities of bone-marrow cells. These cells -- endothelial-like progenitor cells or ELPCs -- graft into vascular areas that are scarred or injured and have been shown to improve the survival and reduce pulmonary hypertension in rats. Now scientists are looking into whether this will work in humans.

The explosion in the number of available therapies for PAH over the past 15 years has been impressive, according to David Langleben of McGill University in Montreal. In an article for the PAH Association, he notes that patients and caregivers now have access to treatments from three broad classes of drugs: prostanoids, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors (PDE-5). However, it’s unclear whether the therapies actually attack or reverse the disease, he says, which is why scientists are paying close attention to three novel therapies undergoing clinical trials. One involves using cicletanine and ricioguat to help restore normal signaling within the affected cells. Another uses anticancer drugs to help cells repair and maintain healthy pulmonary blood vessels, and a third uses a cell-based gene therapy to reseed the lining of the affected blood vessels with healthier cells.

On many fronts, research progress is offering new hope to patients diagnosed with pulmonary hypertension. The rapid pace of research makes it more important than ever to stay informed. Fortunately, a variety of Internet information sources is also making it easier than ever to follow the latest research results.

-- Peter Jaret is a contributing editor for Health magazine and a winner of the American Medical Association's award for medical reporting. His work has appeared in National Geographic, Newsweek, Hippocrates, and many other national magazines. He is also the author of In Self-Defense (Harcourt Brace Jovanovich), Active Living Every Day, and Heart Healthy for Life.

References:

Langleben, David. Near-Term Novel Therapies for PAH. Advances in Pulmonary Hypertension, Spring 2009. Vol.8, No.1.

Du, L. et al. Signaling molecules in nonfamilial pulmonary hypertension. New England Journal of Medicine, Feb 6, 2003, pp 500-509.

Ghofrani, H.A. Sildenafil (Viagra) may help improve control of pulmonary hypertension. Annals of Internal Medicine, Apr 2, 2002, pp 515-522.

Advances in Pulmonary Hypertension. Journal of the Pulmonary Hypertension Association, Winter 2003, Spring 2002, Summer 2002, Autumn 2002.

ClinicalTrials.gov, the National Institutes of Health.

Optn.org, the Organ Procurement and Transplantation Network.

Kramm, T. et al. Inhaled iloprost in patients with chronic thromboembolic pulmonary hypertension: effects before and after pulmonary thromboendarterectomy. Annals of Thoracic Surgery, Sep 2003, pp 711-718.

Nauser, Trenton D., MD, and Stites, Steven W., MD. Diagnosis and treatment of pulmonary hypertension. American Family Physician, May 1, 2001, pp 1789-1798.

McMurtry, MS et al. Gene therapy targeting survivin selectively induces pulmonary vascular apoptosis and reverses pulmonary arterial hypertension. Journal of Clinical Investigation. 115:1479-1491. March 2005. http://www.jci.org/cgi/content/abstract/115/6/1479.

National Heart, Blood and Lung Institute. Adult Stem Cells, Lung Biology and Lung Disease. May 2006. http://www.nhlbi.nih.gov/meetings/workshops/sc-wksp.htm

Cleveland Clinic. Lung Transplant Process -- Finding an Organ Donor. http://www.clevelandclinic.org/thoracic/Airway/Transplant_Process.htm

Safdar, Z. Phase 2 and 3 Clinical Trials in Pulmonary Arterial Hypertension. Advances in Pulmonary Hypertension. 2008; vol. 17 (1).

Humbert, M. Update in Pulmonary Arterial Hypertension 2007. American Journal of Respiratory and Critical Care Medicine. 2008; 177: 574-79.

McLaughlin, W, et al. Randomized Study of Adding Inhaled Iloprost to Existing Bosentan in Pulmonary Arterial Hypertension. American Journal of Respiratory and Critical Care Medicine. December 1, 2006; 174(11): 1257-63.


Reviewed by Trenton D. Nauser, MD, FACP, FCCP, who practices pulmonary and critical care medicine at the Veterans Administration Medical Center in Kansas City, Missouri. He also serves as an assistant professor of medicine at the University of Kansas Medical Center, Kansas City, Kansas.

Last updated August 19, 2009

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

CLINICAL TRIALS AND PAH

Topics:

Researchers have made dramatic progress in diagnosing and treating pulmonary hypertension in all its forms, including PAH. Ongoing research continues to provide fresh insights into the processes that lead to pulmonary hypertension and new ways to treat it.

But much work remains to be done. Many drugs are effective for only a small proportion of patients. The disease still poses a life-threatening danger for many people. For that reason, a wide variety of clinical trials are under way or actively recruiting volunteers -- investigations designed to learn more about the disease process and to test promising therapies. Researchers are conducting clinical trials not only for pulmonary hypertension but for the many disease conditions that can lead to it, from autoimmune disorders to chronic lung conditions. Several are looking at pulmonary hypertension in people with sickle cell disease, for instance. Others are studying genetic factors in pulmonary arterial hypertension.

What are clinical trials?

Clinical trials are the means by which new drugs and treatments are tested to determine whether they work. They are the engine that drives progress in medicine. In addition, for patients who have run out of other options, clinical trials offer a last chance at a potentially effective therapy. In some cases they provide access to research treatments before they are made widely available.

It's important to remember that clinical trials often involve unproven therapies. There is no guarantee that the treatments under investigation will work. And there is always a chance that they may cause serious side effects. Still, by participating in clinical trials, patients help advance the science of medicine.

If you or someone you know is interested in participating in a clinical trial, there are some important things to remember.

Clinical trials have specific guidelines about participation. Not everyone can qualify to be part of every clinical trial. To make sure clinical trials are safe and provide reliable scientific information, researchers carefully choose who can -- and who can't -- take part. Clinical trials have "inclusion criteria" (the requirements for participating) and "exclusion criteria" (factors that disallow people from taking part). These are usually spelled out in descriptions of clinical trials made available to the public.

Participants must be informed and give their consent. Doctors, nurses, and other health professionals involved in running a study are required to explain the details of the investigation to prospective participants. They provide an "informed consent" document with the details of the study explained in writing. Participants are asked to sign the document. Additional information is sometimes provided during the study, as it becomes available.

Can you withdraw from a clinical trial?

Participants are always free to withdraw from a study. Even after signing an informed consent document and joining a clinical trial, volunteers can withdraw at any time. Withdrawing can jeopardize research, however, so it's important to learn everything you can before agreeing to join a trial.

Not everyone in a study always receives the active drug or treatment. Some studies test an active drug against a placebo. A placebo is an inert substance such as a sugar pill: It's used in clinical trials to compare the effect of a given treatment against no treatment. In blinded studies, neither participants nor researchers are allowed to know who is getting the real drug and who is being given the placebo. (A sealed code is used to keep track for later analysis.) Blinded studies are essential to eliminate bias in interpreting the results.

Typically, if a drug appears to be working effectively, researchers will decide to "unblind" the study and offer the new treatment to everyone in the study. Not all studies involve a placebo, however. Some test the safety of a new drug. Others are designed to better understand the process of a disease.

What kind of steps do clinical trials involve?

Clinical studies are conducted in four phases. Phase I trials test a new drug or treatment in a small group of people to make sure it's safe, to determine a safe dosage range, and to look at side effects. Phase II trials usually include more people and study whether the drug is safe and whether it's effective.

Phase III trials involve still more participants. These tests are designed to confirm a treatment's effectiveness, monitor its side effects, and sometimes compare it to commonly used treatments. In Phase IV trials, drugs that have already been approved are studied for more information about their benefits, uses, or potential risks.

Investigational drugs are sometimes offered outside of clinical trials. The FDA allows manufacturers of investigational drugs to offer them to people who might benefit but who don't qualify for clinical trials. This "expanded access," as it's called, is usually offered to people with a serious or life-threatening illness. The drug must already have been shown to be safe and effective in well-controlled studies.

How do you find a clinical trial?

For more information about clinical trials, talk to your doctor or check out these comprehensive sites:

National Institutes of Health
http://www.ClinicalTrials.gov

This site provides detailed information about clinical trials and has a search feature that locates ongoing clinical trials.

CenterWatch
http://www.CenterWatch.com

This site includes a listing of clinical trials organized by state.

-- Peter Jaret is a contributing editor for Health magazine and a winner of the American Medical Association's award for medical reporting. His work has appeared in National Geographic, Newsweek, Hippocrates, and many other national magazines. He is also the author of In Self-Defense (Harcourt Brace Jovanovich), Active Living Every Day, and Heart Healthy for Life.

References:

National Institutes of Health. An Introduction to Clinical Trials. http://www.clinicaltrials.gov.


Reviewed by Trenton D. Nauser, MD, FACP, FCCP, who practices pulmonary and critical care medicine at the Veterans Administration Medical Center in Kansas City, Missouri. He also serves as an assistant professor of medicine at the University of Kansas Medical Center, Kansas City, Kansas.

Last updated August 21, 2009

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

STRESS AND PAH

Topics:

Being diagnosed with any serious medical condition is stressful. Learning you have pulmonary arterial hypertension (PAH) can be especially upsetting. The symptoms of fatigue and shortness of breath may get in the way of living your normal life, and it is natural to worry about serious complications occurring if the disease progresses.

Emotional stress can lessen the quality of life. That's reason enough to find every way you can to let off pressure. Can stress also exacerbate PAH?

It's well known that emotional stresses can affect overall blood pressure. In fact, chronic stress is a risk factor for common hypertension -- the condition that doctors measure by putting a cuff around the upper arm and inflating it. Emotional stress is now known to alter the balance of a variety of brain chemicals, including cortisol and adrenaline. These changes can also impair the ability of blood vessels to widen or constrict as needed.

The link between mental stress and pulmonary arterial hypertension, or high blood pressure in the pulmonary arteries, isn't as clear as it is with general high blood pressure. But there is some evidence that being under stress may affect the level of strain on the heart. In research published in the journal Clinical Physiology, Swiss researchers investigated whether mental stress increases pulmonary artery pressure. The experimenters administered a standardized 10-minute mental stress test to seven patients. While taking the test, the pressure in their pulmonary arteries increased significantly.

Good stress, bad stress

Not all stress is negative, of course. Some of the pressures in life push us to do our best. The worst stresses are those we feel we can't control. The pressure of a deadline at work doesn't have to be overly stressful, as long as you have the time and resources you need to meet it, for instance. Negative or harmful stress occurs if you're given a deadline but not the time or means to complete it. When you continually find yourself in stressful situations that are entirely out of your control, the pressures of life can turn into harmful chronic stress.

When you're diagnosed with a serious medical problem like pulmonary arterial hypertension, you may feel as if there's nothing you can do. But there are plenty of ways you can protect your health. There's also plenty you can do to ease the stresses of dealing with PAH. That's important to remember: The last thing you want to do is add to stress by worrying that tension at home or at work is harming your heart.

Know the danger signs

Immediate physical reactions to stress can include a racing pulse, sweating, and a fluttering feeling in your chest. Blood pressure climbs. Adrenaline, the biochemical that readies the body for fight or flight, surges into the bloodstream. Other typical signs of stress include the following:

There is no objective test for stress. If you feel as if the pressures you face in dealing with pulmonary arterial hypertension and other stresses in your life are a problem, it's time to do something about it.

What works best? There is no single antidote. Most people try a few approaches before finding the ones that have the best effect for them. The good news is that you won't have to turn your life upside down to tame stress, says psychologist Frederic Luskin, PhD, a researcher at the Stanford University Center for Research in Disease Prevention. "A few simple techniques, things you can do anywhere and that don't have to take more than a few minutes, can stop the stress response before it goes out of control," Luskin points out.

Here are eight strategies for soothing the savage beast of stress:

1. Clear your head

At least once every day, find a quiet corner and take five or 10 minutes to sit quietly and do nothing. Sitting quietly slows heart rate and reduces blood pressure, countering two of the most obvious effects of stress. A quiet break can also increase your sense of control over events. At the University of Massachusetts Center for Mindfulness in Medicine, patients are encouraged to sit and become mindful of how they feel and the sounds around them. Taking a quiet break like this can help you get past fatigue, which is a common symptom of pulmonary arterial hypertension.

2. Focus on the positive

Shifting the focus of your thoughts from things that worry you to things that bring you happiness can change your mood for the better -- and ease stress. Psychologists call the technique "positive emotion refocusing." Thinking pleasant, calming thoughts can counteract the physiological changes that occur during stress -- you can actually slow your heart rate, for example, and lower your blood pressure.

3. Take a deep breath

Deep-breathing exercises have been part of meditation techniques for centuries -- with good reason. Concentrating on the act of breathing in and breathing out almost invariably calms mind and body. Some people get even more benefit from repeating a mantra-like word or phrase each time they breathe in. Another technique: Imagine each inhaled breath filling your body with soothing light. Imagine each exhaled breath blowing away tension and stress.

4. Have a laugh

Laughter really is the best medicine, according to studies at Loma Linda University in California. Researchers there have shown that laughter lowers levels of the stress hormones cortisol and epinephrine. A good belly laugh also boosts immunity -- and the physiological effects can last up to 24 hours. Amazingly, the Loma Linda team has found that just looking forward to something funny helps. Telling volunteers that they would participate in an experiment that involved watching a funny video lowered their stress levels and created a more positive mood.

5. Do what you love

Having a health problem like PAH sometimes forces you to do things you don't necessarily want to do. The best antidote: Make time in your day to do at least one thing you really love to do -- listen to music, play music, dance, garden, play with the kids or the dog, paint, read, whatever. Listening to music may be especially soothing. At Monash University in Victoria, Australia, two groups of students were told to prepare an oral presentation. Some worked in silence. Others listened to the gentle strains of Pachelbel's Canon in D major. Blood pressure and heart rate was more likely to climb among the silent workers. Those who listened to music reported feeling much less stress. Another study, this one at the Medical College of Ohio in Toledo, found that patients who listen to music while undergoing uncomfortable medical procedures require less sedation.

6. Take a walk

Physical activity can help ease stress. If you have pulmonary hypertension, however, it's important to choose safe activities. Experts warn against lifting anything that weighs more than 25 pounds or that causes you to strain. That kind of exertion can increase blood pressure in the pulmonary arteries and bring on symptoms such as dizziness or shortness of breath. Walking is an especially good choice, since it puts minimum strain on the body. You can do it almost anywhere. And walking has proven benefits. In an investigation at the Stanford University School of Medicine, researchers looked at people who were taking care of relatives with Alzheimer's. Volunteers who began walking for 30 to 40 minutes four times a week reported feeling less distressed. Their sleep improved, and tests showed that their blood pressure was more likely to hold steady when they were under pressure.

7. Change what you can

If you notice yourself getting stressed out again and again in the same situations or because of the same problem, think about what you can change. Overwhelmed by chores at home? Work out a chore-sharing plan with other members of the household. Does your blood pressure climb every time you find yourself searching for your car keys or glasses? Decide on one place to keep them and get into the habit of putting them there. Having trouble with your boss at work? Consider sitting down to talk about the situation and offer constructive ways to make things better.

8. Accept the rest

Of course some of life's frustrations and worries can't be eliminated. If you or someone close to you has been diagnosed with pulmonary arterial hypertension, this reality is one of the things you have to live with. It's important to recognize what you can't change and move on. The process is very much like forgiving someone who has hurt you, according to psychologist Luskin. Accepting what you can't change allows you to let go of hurt and anger and to focus on more constructive thoughts.

-- Peter Jaret is a contributing editor for Health magazine and a winner of the American Medical Association's award for medical reporting. His work has appeared in National Geographic, Newsweek, Hippocrates, and many other national magazines. He is also the author of In Self-Defense (Harcourt Brace Jovanovich), Active Living Every Day, and Heart Healthy for Life.

References:

Schachinger, H. et al. Mental stress increases right heart afterload in severe pulmonary hypertension. Clinical Physiology, Nov 2000, pp 483-7.

Lindvall, K. Stress-induced changes in blood pressure and left ventricular function in mild hypertension. Clinical Cardiology, Feb 1991, pp 125-32.

Benson, H. The Relaxation Response. Harper Collins.

King, A.C. et al. Effects of moderate-intensity exercise on psychological, behavioral, and emotional responses to family caregiving: a randomized controlled trial. Journal of Gerontology, Jan 2002, pp M26-36.

Smolen, D. et al., The effect of self-selected music during colonsocopy on anxiety, heart rate, and blood pressure. Applied Nursing Research, Aug 2002, pp 126-36.

Knight, W.E. et al. Relaxing music prevents stress-induced increases in subjective anxiety, systolic blood pressure, and heart rate in healthy males and females. Journal of Music Therapy, Winter 2001, pp 254-72.

Berk, L.S. et al. Modulation of neuroimmune parameters during the eustress of humor-associated mirthful laughter. Alternative Therapies in Health and Medicine, Mar 2001, pp 62-76.

Berk et al. Neuroendocrine and stress hormone changes during mirthful laughter. American Journal of Medical Science, Dec 1989, pp 390-396.


Reviewed by Trenton D. Nauser, MD, FACP, FCCP, who practices pulmonary and critical care medicine at the Veterans Administration Medical Center in Kansas City, Missouri. He also serves as an assistant professor of medicine at the University of Kansas Medical Center, Kansas City, Kansas.

Last updated March 30, 2009

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

TELLING YOUR FAMILY

Topics:

Unless they happened to catch a re-run episode of ER, most people have never heard of pulmonary arterial hypertension (PAH). Those who did see the episode probably have a one-sided, unrealistic view of the disease -- while real-life patients often lead long and essentially normal lives, the young character found that she had little time left. If you've been diagnosed with the disease, you'll have to become an educator as well as a patient.

Getting support

Helping friends and family members understand pulmonary hypertension is the key to getting support, according to Pat Paton, a 67-year-old resident of Indiantown, Florida, who has lived with PAH since 1987. People need to understand your condition, your physical limitations, and, most of all, your prognosis. The best person to explain your situation to them clearly is you. If the people around you try searching for information on their own, their search can easily leave them more confused than ever. "There's a lot of bad information out there," says Paton, a founder of the Pulmonary Hypertension Association, where she now works as a help-line staffer. "We hear from people who found things on the Internet telling them that they only have six months left to live."

Pulmonary hypertension can cause many misunderstandings. For one thing, patients usually don't look sick. In fact, they appear so healthy that they often get glares when they use handicapped parking spaces. Friends and family members should be prepared to stand up for you, and they must understand that you can't lift heavy objects or overexert yourself, no matter how well you look, Paton says.

Work misconceptions

Another common misperception about pulmonary arterial hypertension and other forms of PH is that it makes it impossible for a person to work. For this reason, people with the disease may want to think about who really needs to know about it, Paton says. Family members, close friends, and doctors, yes. Employers, maybe not. As long as a job isn't strenuous, there's no reason why a person with pulmonary arterial hypertension couldn't do it as well as anyone else.

Patients who need to spread the word about the disease have several excellent resources to turn to. The Pulmonary Hypertension Association offers many informative brochures and fact sheets on its Web site, http://www.phassociation.org . Anyone who wants more information -- friends and family members included -- can also call the PHA help line at 800/748-7274. The person at the other end of the line -- often Paton herself -- can field all sorts of questions about the disease.

The people around you may not know much about pulmonary hypertension, but they'll be eager to learn. With your guidance, they can understand the disease and help you get on with your life.

-- Chris Woolston, MS, is a health and medical writer with a master's degree in biology. He is a contributing editor at Consumer Health Interactive and was a staff writer at Hippocrates, a magazine for physicians. His reporting on occupational health earned him an award from the Northern California chapter of the Society of Professional Journalists.

References:

Interview with Pat Paton, PPH patient and a founder and help-line staffer with the Pulmonary Hypertension Association.

Pulmonary Hypertension Association. Pulmonary Hypertension: Helpful information for patients and families. 2002.


Reviewed by Trenton D. Nauser, MD, FACP, FCCP, who practices pulmonary and critical care medicine at the Veterans Administration Medical Center in Kansas City, Missouri. He also serves as an assistant professor of medicine at the University of Kansas Medical Center, Kansas City, Kansas.

Last updated August 20, 2009

Copyright © 2003 Consumer Health Interactive

DRUGS & CONDITIONS

PAH RESOURCES

Topics:

People with pulmonary hypertension (PH) often feel isolated and alone. Their friends and family members will know almost nothing about their disease, and even their family doctor will probably have more questions than answers. So where can patients go for advice and support? In this highly connected world, help isn't far away. Here are three organizations that provide valuable services for people with pulmonary hypertension.

Pulmonary Hypertension Association (PHA)

http://www.phassociation.org

Toll-free help line (days and evenings): 800/748-7274

Founded more than a decade ago by three women with pulmonary hypertension, the PHA could be a model for support organizations everywhere. Patients staff the help line; if you want to find someone who knows what you're going through, you won't have to look far. "I received about 25 calls yesterday, and 10 people ended the call by saying, 'Thank you, you really saved my life,'" says Pat Paton, one of the founders of PHA. "When I was diagnosed in 1987, it took me two years to find another patient to talk to."

The Web site offers detailed information on the disease, message boards, a free newsletter, a calendar of related events, and news updates. Visitors can also order educational material, including the PHA's well-received "Pulmonary Hypertension: A Patient's Survival Guide."

American Lung Association

http://www.lungusa.org

Toll-free number: 800/LUNG-USA (800/586-4872)

If you need help finding a qualified physician or just want some basic tips for keeping your lungs healthy, the American Lung Association can be a valuable resource.

American Heart Association

http://www.americanheart.org

Toll-free phone: 800/AHA-USA-1 (800/242-8721)

Like the American Lung Association, the American Heart Association is a large organization that supports patients with many diseases, including primary pulmonary hypertension. If you need to find a cardiologist or want more information about your heart and how to protect it, visit the AHA's website or give them a call.

National Heart, Lung, and Blood Institute

http://www.nhlbi.nih.gov

Telephone: 301/592-8573

Part of the National Institutes of Health, the NHLBI has an extensive consumer-friendly section on pulmonary hypertension, which covers its symptoms, diagnosis, and treatment; it also includes a helpful glossary. A bonus is that you can search for clinical trials across the United States.

National Organization for Rare Disorders (NORD)

http://www.rarediseases.org

Toll-free phone: 800/999-6673

This site covers 1,150 rare diseases, including both primary and secondary hypertension and related disorders. Importantly, this advocacy group’s effort also includes breaking news and contact details about current investigational therapies for both conditions. You can also speak to an RN or a genetic counselor at 203-744-0100, or ask a question about caring for someone with a rare disease at RN@raredisease.org


Last updated August 21, 2009

Copyright © 2003 Consumer Health Interactive