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. At present, only one type known pulmonary hypertension, group one pulmonary arterial hypertension (PAH) has drug treatments pproved by the Food and Drug Administration (FDA). Newer drugs used in the treatment of pulmonary hypertension, according to the Pulmonary Hypertension Association (PHA), include:
VasodilatorsEndothilin receptor antagonistsPhosphodiesterase-5 inhibitorsSoluble guanylate cyclase stimulators
Drugs in these classes have been approved by the FDA for the treatment of PAH, but they are often used off-label for other forms of pulmonary hypertension. Vasodilators 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 doesn’t 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: epoprostenol 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 epoprostenol are related to the complex delivery system and include:
Catheter infectionsPump malfunctionBlood clotting
Possible side effects directly related to the drug include:
RashDiarrheaJoint 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). RELATED: Your Pulmonary Hypertension Health Team Endothilin receptor antagonists 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 gainSwelling of the arms, hands, feet, or anklesFast or irregular heartbeatFainting, dizziness, or blurred vision
Flu-like symptoms can occur with ambrisentan and macitentan, and respiratory infections with bosentan. Phosphodiesterase-5 inhibitors support the lung’s ability to make natural vasodilators. Examples of drugs in this class include:
Sildenafil (Revatio, Viagra)Tadalafil (Adcirca, Cialis)
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:
HeadacheEdemaFlushingStomach upsetDiarrheaStuffy nose
In October 2015, the Food and Drug Administration (FDA) approved the two-drug combination of ambrisentan and tadalafil to treat PAH. Riociguat (Adempas), approved in 2013, is a soluble guanylate cyclase stimulators that supports the lung’s ability to relax and widen blood vessels to make exercise possible and improve functioning.
Pulmonary Hypertension Conventional Therapies
According to the PHA, these medications may be used in conjunction with newer therapies:
Calcium channel blockersDigoxin (Digitek, Lanoxicaps, and Lanoxin)Diuretics (water pills)Blood thinners
As with newer therapiess, drugs in these classes have been approved by the FDA for the treatment of PAH, and they are used off-label for the treatment of other forms of pulmonary hypertension. Calcium channel blockers help treat the vasoresponsive form of pulmonary hypertension. In vasoresponsive pulmonary hypertension, 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)Diltiazem (Cardizem, Cartia, Diltia)
Side effects may include:
Abnormally low blood pressureWater retentionLow 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, according to Dr. Fenster. 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 dizzinessVision changesRashIrregular heartbeat
Diuretics help the body get rid of excess fluid and are often prescribed for people with pulmonary hypertension. Diuretics 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 that’s right for you. People with pulmonary hypertension have a higher-than-average chance of developing blood clots that may obstruct blood flow through the lungs, Dr. Fenster explains. Blood-thinning medications prevent clots from forming or existing clots from enlarging. This class of medications includes:
Warfarin (Coumadin)HeparinFondaparinux (Arixtra)Argatroban (Acova)Dabigatran (Pradaxa)Apixaban (Eliquis)Rivaroxaban (Xarelto)
Blood-thinning medications can have significant side effects, such as prolonged bleeding from cuts or bruises and may cause internal bleeding. If you’re taking warfarin, tell your doctor if you:
Cough up bloodExperience nosebleeds or bleeding gumsHave a cut that does not stop bleeding within a reasonable period of timeSee blood in your urineHave black stools (a possible sign of blood in the stool)
Warfarin may also cause allergic reactions such as hives, rash, and itching. Conventional drugs like warfarin require monitoring with monthly blood tests. Newer options, such as abigatran, apixaban, and rivaroxaban, do not.
Additional Treatment Options
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:
Group 1. Besides prescribing medications, your doctor may recommend a surgical procedure, such as atrial septostomy, lung transplant, or heart-lung transplant.Group 2. Pulmonary hypertension in this group is related to problems affecting the left side of the heart, including heart failure, and requires lifestyle changes, medication, or surgery.Group 3. Lung disease contributes to pulmonary hypertension in this group. Your doctor might recommend oxygen therapy as part of your treatment.Group 4. Pulmonary hypertension in this group is caused by clots or clotting disorders. Blood-thinning medication and surgery may be necessary to manage clots or the risk of clotting.Group 5. Pulmonary hypertension in this group can result from a number of health problems. Treating those underlying conditions will be part of pulmonary hypertension treatment.
Once you and your doctor find the right medication or combination of medications, you can get on the road to breathing and feeling better.
