Pulmonary hypertension is high blood pressure that occurs specifically in the blood vessels of the lungs.
The condition often develops as a complication of some other medical condition, but in some cases, it has no apparent cause.
When you say pulmonary hypertension, there are actually five types," says pulmonary hypertension expert Samuel A. Allen, DO, director of the Pulmonary Hypertension Center at Beaumont Hospital in Troy, Mich.
Only one type known as group one, pulmonary arterial hypertension, or PAH has drug treatments approved by the Food and Drug Administration (FDA), Dr. Allen says.
Pulmonary hypertension is generally considered a rare or orphan disorder. Some experts believe that the number of people with this condition has been underestimated because it can be hard to diagnose pulmonary hypertension.
In addition, people dont always seek treatment when symptoms start. Allen says that by the time some people seek medical help, they have had symptoms for well over two years.
Over the past few decades, as diagnostic methods for pulmonary hypertension have improved and awareness has grown, healthcare providers have reported increasing numbers of cases of pulmonary hypertension.
Regardless of the underlying cause, pulmonary hypertension makes it harder for blood to move through the lungs. This forces the heart to work harder to overcome that resistance.
Ultimately, the heart enlarges and stiffens, becoming weaker and less able to pump blood throughout the body.
Symptoms of pulmonary hypertension include:
Estimating the incidence of the different types of pulmonary hypertension is difficult because they are associated with so many varied conditions.
However, the American Heart Association estimates that each year about 500 to 1,000 new cases of pulmonary arterial hypertension (PAH), which occur spontaneously and without an obvious underlying cause, are diagnosed.
Pulmonary hypertension appears most often among women 21 to 40 years old, but it can strike anyone at any age.
A Centers for Disease Control and Prevention (CDC) review of a decades worth of data shows that deaths from pulmonary arterial hypertension are increasing among men and women of all ages. Hospitalization for PAH has almost doubled for people over 85.
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You are at a higher risk for pulmonary hypertension if you:
Some cases of pulmonary hypertension might not be preventable, for example when the condition results from inherited risk, from unknown causes, or from conditions that you can't control, such as a congenital heart defect.
Allen stresses that it is possible to reduce the risk for developing pulmonary hypertension by avoiding activities that could contribute to developing it, such as the use of illegal drugs.
Another way to lower pulmonary hypertension risk is by treating the other health conditions associated with it, such as heart disease.
Pulmonary hypertension is progressive and its non-curable, Allen says. However, he adds, with the correct treatment, people living with pulmonary hypertension can improve their quality of life and slow the progression of the disease.
If you have symptoms, such as shortness of breath that occurs without strenuous activity, be proactive about seeking a diagnosis and getting treated by health professionals with expertise in managing the condition.
Pulmonary hypertension develops as a result of a wide variety of health conditions, ranging from inherited disorders to infections, to blood clots and heart disease.
Getting an accurate diagnosis is important because the pulmonary hypertension group determines treatment. The workup is extensive, Allen says.
Pulmonary hypertension groups based on the underlying cause, are:
Group 1: Called pulmonary arterial hypertension (PAH), it may be inherited; caused by prescription or non-prescription drugs, problems with the veins and blood vessels around the lungs, or other illnesses, such as HIV, congenital heart disease, or certain infections; or have no identifiable cause (idiopathic).
Group 2: Pulmonary hypertension in this group is caused by diseases that affect the left side of the heart, such as mitral valve disease or congestive heart failure.
Group 3: This type of pulmonary hypertension is caused by disordered breathing during sleep, known as sleep apnea. This type also is related to lung diseases, such as interstitial lung disease and chronic obstructive pulmonary disease, or COPD.
Group 4: Pulmonary hypertension in this group is caused by blood clots in the lungs or clotting disorders.
Group 5: This group includes pulmonary hypertension caused by all other disorders, including tumors, kidney disease, systemic disorders, blood disorders, and metabolic disorders.
Pulmonary hypertension which is elevated pressure in the vessels that carry blood to the lungs can be tricky to diagnose because its symptoms resemble those of so many other conditions.
The main symptom of pulmonary hypertension is shortness of breath when doing things that arent terribly strenuous.
Activities like household chores or climbing stairs become a problem, and some even have shortness of breath at rest.
Usually its a gradual thing that progresses over a period of months or years, says Myung H. Park, MD. Dr. Park is director of the Pulmonary Vascular Diseases Program at the University of Maryland School of Medicine in Baltimore.
People with pulmonary hypertension may also have chest pain. Sometimes this is severe enough that it mimics a heart attack, Dr. Park says.
Other common symptoms of pulmonary hypertension include:
These symptoms are often attributed to other things, which is why pulmonary hypertension often is misdiagnosed until its at a late stage, Park says.
The diagnostic process is extensive but important because there are five categories, called groups, of pulmonary hypertension. Your treatment options for pulmonary hypertension depend on the group you're in.
The groups, according to the NIH National Heart, Lung and Blood Institute, are:
If your doctor thinks you may have pulmonary hypertension, the doctor will take your medical history to find out about other health conditions that might be related to your symptoms. You'll also have a physical examination.
In addition, you'll likely be scheduled for a chest X-ray and other tests to evaluate your lung function and capacity.
You may also have an echocardiogram. This is a painless test in which a special probe, placed on the chest, directs sound waves into the chest cavity. Some of the waves bounce back when they encounter a solid structure, like the heart.
A computer uses this information to generate an echocardiogram, or picture of the heart. It also estimates the pressure in the pulmonary artery.
This is usually where pulmonary hypertension is first detected, Dr. Park says. Your doctor will discuss the results of the test with you and recommend further testing if it is necessary.
The only way to confirm a diagnosis of pulmonary hypertension is with a test called a right heart catheterization. It involves inserting a thin tube, or catheter, into a major vein in your neck or groin area.
The catheter is then threaded into the pulmonary artery, where the pulmonary arterial pressure is measured. Youll be sedated but remain awake for the procedure.
Until you perform this test, you cannot definitively say if someone has pulmonary hypertension or not, Park explains. Your doctor will discuss the results of this test with you, probably while you are recovering.
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Blood tests can determine if you have conditions often associated with pulmonary hypertension. These include connective tissue disease, HIV, liver diseases such as hepatitis C, or sickle cell anemia.
Results from blood tests such as these may take a few days to come back. Most centers will also have you walk for six minutes the distance you cover during that time reflects your stamina and heart function.
Because the symptoms mimic asthma, you may be tested for that as well. Asthma tests include chest X-rays and lung function tests.
In a thorough evaluation, you also may be asked to undergo a sleep study. This tests your breathing and oxygen levels during sleep to determine if you have sleep apnea.
People with sleep apnea stop breathing for dozens, sometimes hundreds, of brief episodes during the night. They snore and struggle for breath, but rarely wake up completely until the morning. They arise feeling sleepy or groggy, but without knowing why.
Sleep apnea can increase lung pressure, Park says. However, she adds, if thats the only medical condition you have, any associated pulmonary hypertension is likely to be mild.
Pulmonary hypertension is a disorder of high blood pressure in the arteries of the lungs.
This forces the heart to work harder to pump blood through the pulmonary blood vessels.
It's a progressive, potentially devastating condition without a cure.
However, treatment can greatly improve quality of life and prolong life expectancy.
Treatment of pulmonary hypertension depends on the specific type of pulmonary hypertension a person has.
Physicians also take into account underlying conditions that could be contributing to the disease.
Medical experts categorize pulmonary hypertension into five groups. Group 1, called pulmonary arterial hypertension or PAH, includes pulmonary hypertension caused by blood vessel problems in the lungs.
Group 1 also includes pulmonary hypertension with no known cause (idiopathic), and pulmonary hypertension caused by certain drugs or toxins.
The other four groups of pulmonary hypertension develop because of underlying conditions, such as blood clots, so proper treatment has to address those health problems.
Treatments for pulmonary hypertension may be given orally, by infusion, by inhalation, or subcutaneously (under the skin), some continuously.
Newer drugs used in the treatment of pulmonary hypertension, according to the Pulmonary Hypertension Association (PHA), include:
Vasodilators: These drugs widen and relax the blood vessels, allowing blood to travel through the lungs, become oxygenated, and carry that oxygen to the heart. In turn, the heart doesnt have to work as hard to pump blood through the rest of the body.
Specific vasodilators given for pulmonary hypertension belong to a group called synthetic prostacyclins. They mimic natural prostacyclin, a compound made in the body that works as a blood thinner as well as a vasodilator.
The PHA notes that people on epoprostenol (Flolan), which comes in a powder that must be liquefied, may live longer and have more exercise capacity and a greater sense of well-being than people who do not use this drug.
However, the drawbacks are considerable: Flolan must be taken as a continuous infusion directly into the heart, which requires having a catheter surgically implanted in the chest during a hospital stay and wearing a pump around the waist that delivers the drug to the catheter.
Blood levels of epoprostenol drop rapidly when the infusion cartridge runs out. This gives people only a three- to five-minute window of time to start a new one, or risk the return of symptoms, with possible life-threatening consequences.
The major side effects of this pulmonary hypertension treatment are related to the complex delivery system and include catheter infections, pump malfunction, and blood clotting. Possible side effects directly related to the drug include a rash, diarrhea, and joint pain.
Despite its disadvantages, epoprostenol has been called the single most important advance in the treatment of pulmonary hypertension.
Some people may be able to take a newer form of this medication, room-temperature-stable epoprostenol (Veletri), which is somewhat easier to use because it doesn't require refrigeration.
Treprostinil (Remodulin), another synthetic prostacyclin, can be given as a continuous infusion as well as subcutaneously, via a tube placed under the skin.
There's also an inhaled version of treprostinil (Tyvaso) and another inhaled synthetic prostacyclin, iloprost (Ventavist).
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Endothilin Receptor Antagonists: These drugs block the action of a chemical called endothelin, which normally causes the blood vessels to constrict.
Bosentan (Tracleer), which is taken orally, has been associated with an improvement in symptoms and increased walking time among people with pulmonary hypertension. However, it may also cause liver damage so liver enzymes must be checked with a monthly blood test.
With the newer medications ambrisentan (Letairis) and macitentan (Opsumit), liver enzymes still need to be checked but less frequently, according to the PHA.
Because of the possibility of birth defects, none of these drugs is appropriate for pregnant women.
Other serious side effects vary with each drug but may include sudden weight gain; swelling of the arms, hands, feet, or ankles; fast or irregular heartbeat; and fainting, dizziness, or blurred vision.
Flu-like symptoms can occur with ambrisentan and macitentan, and respiratory infections with bosentan.
Phosphodiesterase-5 Inhibitors: These medications support the lungs ability to make natural vasodilators and include sildenafil (Revatio) and tadalafil (Adcirca).
Taken orally three times a day, sildenafil can help people with pulmonary arterial hypertension walk farther, feel less short of breath, and delay the progression of their disease, says Brett Fenster, MD, a cardiologist and director of the Pulmonary Hypertension Center at National Jewish Hospital in Denver. Tadafil is taken just once a day.
Possible side effects of these drugs include headache, edema, flushing, stomach upset, diarrhea, and a stuffy nose.
Soluble Guanylate Cyclase Stimulators: Riociguat (Adempas), approved in 2013, supports the lungs ability to relax and widen blood vessels to make exercise possible and improve functioning.
According to the PHA, these medications may be used in conjunction with newer therapies:
Calcium Channel Blockers: These oral drugs help treat the vasoresponsive form of pulmonary hypertension. This happens when blood pressure in the lungs decreases because of certain inhaled or intravenous medications during a cardiac catheterization procedure, Dr. Fenster says.
Calcium channel blockers used for pulmonary hypertension include nifedipine (Nifedical, Procardia) and diltiazem (Cardizem, Cartia, Diltia). Side effects may include abnormally low blood pressure, water retention, and low blood oxygen levels.
Some people may be allergic to calcium channel blockers and experience swelling of the face and limbs or difficulty breathing.
Other possible side effects include headache and rash. Calcium channel blockers are prescribed less often today than in years past, with fewer than 10 percent of people with PAH taking them today.
Digoxin: (Digitek, Lanoxicaps, and Lanoxin) Digoxin slows and strengthens the heartbeat and relieves symptoms of heart failure, a common complication of pulmonary hypertension. Digoxin is taken orally as a tablet, capsule, or liquid.
Side effects of digoxin may include drowsiness or dizziness, vision changes, rash, and irregular heartbeat.
Diuretics: Also known as water pills, diuretics help the body get rid of excess fluid.
Diuretics are often prescribed for people with pulmonary hypertension. These drugs help people lose extra water, which makes it easier for the heart to work and relieves the fluid buildup that can cause the feet, ankles, legs, and face to swell.
Many types of diuretics are available; your doctor will choose the one thats right for you.
Blood Thinners: People with pulmonary hypertension have a higher-than-average chance of developing blood clots that may obstruct blood flow through the lungs, Fenster explains.
Blood-thinning medications prevent clots from forming or existing clots from enlarging. This class of medications includes warfarin (Coumadin), heparin, fondaparinux (Arixtra), argatroban (Acova), dabigatran (Pradaxa), apixaban (Eliquis), and rivaroxaban (Xarelto).
Blood-thinning medications can have significant side effects, such as prolonged bleeding from cuts or bruises and may cause internal bleeding.
If youre taking warfarin, tell your doctor if you start coughing up blood, if you experience nosebleeds or bleeding gums, if a cut does not stop bleeding within a reasonable period of time, if you see blood in your urine, or if you have black stools (a possible sign of blood in the stool).
Warfarin may also cause allergic reactions such as hives, rash, and itching.
No controlled studies have been done to compare blood thinners, according to a 2014 report in the Cochrane Database of Systematic Reviews.
However, there are differences between conventional drugs like warfarin, which require monitoring with monthly blood tests, and newer options, such as abigatran, apixaban, and rivaroxaban, which do not.
Other treatment strategies depend on the type of pulmonary hypertension you have, according to the National Heart, Lung, and Blood Institute. It notes that each of the five groups might require additional care:
Once you and your doctor find the right medication or combination of medications, you can get on the road to breathing and feeling better.
Atlanta resident Brittany Evans gave up a career she loved because of pulmonary hypertension, but she's found purpose as an advocate for those newly diagnosed with the disease.
About 10 years ago, at the age of 22, Evans was putting in 70-hour weeks as a pastry chef when she started feeling breathless and unusually tired.
Over the next 11 months, she visited an allergist and five other doctors, all of whom gave her different diagnoses, including the old standby Its all in your head.
By then, she says, I was so sick I could hardly speak without stopping to catch my breath.
Finally one of her doctors ordered a stress echocardiogram, which involves gathering ultrasound images of the heart before and after a six-minute walk on a treadmill.
But Evans never made it to the six-minute mark.
I passed out and turned blue three minutes in, she says.
Evans has the idiopathic form of pulmonary hypertension, in which doctors cannot identify a specific cause.
She was placed on Flolan (epoprostenol sodium), which she says immediately improved her breathing.
I didnt realize how sick I was until I could breathe again, she says.
However, Evans remained on the drug for only six months because of a host of complications.
She had nausea, aches and pains, and infections in the catheter feeding the drug to her heart.
Since then, she has tried many of the medications approved to treat pulmonary arterial hypertension (PAH).
I've been on a number of other therapies inhaled, subcutaneous, various pills," she says.
Of the 12 current approved drugs for pulmonary hypertension, "I've taken seven of them soon to be eight! She's also beaten the odds, living longer than her doctors first predicted.
In the year after Evans pulmonary hypertension diagnosis, she says, I was excited just to wake up every morning. I was never given any expectation that I'd live past two years, so I just focused on a day at a time.
With the approval of multiple new treatments and more in the pipeline, Evans says she feels more able to look toward the future.
Instead of just making it through the day, I worry about my long-term health, my emotional health, and, most importantly, being happy," she says. "Those weren't things that were even on my radar my first year in."
With so many more options available now, she says she advises others to be proactive about their treatment and ask not only about other medications that might be more effective but also about pulmonary rehabilitation and exercise.
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Pulmonary hypertension is a progressive, incurable disease, but Evans warns that its crucial to avoid succumbing to self-pity or despair.
The most important thing is to be optimistic and to have a strong-willed personality, she says.
Evans still has enough energy to attend the live music concerts she loves. She requires a handicapped sticker on her car and laments the outraged stares she sometimes gets from passers-by.
Its hard because you look perfectly healthy, she explains. People just have to take your word for how sick you are thats probably the hardest part of it.
Evans passion now revolves around building awareness and improving the patient experience.
I started with fundraising and awareness, holding two local benefit concerts and getting an article published in the local paper, she recalls.
Because she felt strongly that young adults like herself needed dedicated awareness programs, she helped create and served on the advisory board for Generation Hope, an online support group for patients in their twenties and thirties.
Evans also speaks publicly at conferences about her experience and mentors other patients online.
She has received one of 13 Tom Lantos Innovation in Community Service Award grants, which she's using to develop an information and resource handout to give to people newly diagnosed with pulmonary hypertension.
One of my biggest concerns has always been patients' diagnosis experiences and how we can make them less traumatic, she says.
Thanks to the Lantos grant, I was able to create small notepads where each sheet is printed with the Pulmonary Hypertension Association's most important online resources. My hope is that doctors and nurses will give these to new patients or patients who need a little extra support.
Evans says she hopes those pads will direct people to sources of support more quickly after diagnosis.
The notepads are available through the Pulmonary Hypertension Association's website.
Pulmonary hypertension affects the heart and lungs. The condition often has a profound impact on quality of life.
Whats more, some of the drugs used to treat pulmonary hypertension require special prescribing privileges, monitoring, equipment, and handling.
A primary care physician is often the first person you see for symptoms of pulmonary hypertension.
Most people will have symptoms such as shortness of breath, whether its with exertion or rest," says pulmonary hypertension expert Samuel A. Allen, DO, director of the pulmonary hypertension center at Beaumont Hospital in Troy, Mich.
"Others may have some palpitations, and a more common symptom would be leg swelling, Dr. Allen says.
Having such symptoms should lead to testing. If the diagnosis is pulmonary hypertension, you will be referred for more specialized care. At that point, you could be working with a team similar to Allens.
His includes a medical director, research director, clinical nurse coordinator, two pharmacists, two respiratory therapists, an office secretary, an office manager, a medical assistant, and a clinical psychologist.
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Medical professionals who may be involved in pulmonary hypertension care and treatment include:
Treatment for pulmonary hypertension is evolving rapidly, with an increasing number of options available to preserve lung function and improve quality of life.
From the patient's perspective, that makes it important to take charge of your care.
Seek out the most qualified medical team. Stay in touch with them about your symptoms, goals, and the newest developments in pulmonary hypertension research and treatment.
Source: http://www.everydayhealth.com
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