Dilated Cardiomyopathy
Treating Pulmonary Arterial Hypertension
Pulmonary arterial hypertension (PAH) is one of five types of pulmonary hypertension. It happens when the blood vessels in your lungs narrow and scar so much that it's hard for blood to move through them. This can trigger high blood pressure in the lungs.
PAH can be hard to diagnose. That's because the symptoms – like shortness of breath – are common to several other medical conditions. There's no cure. But there are FDA-approved treatments. Here's what you need to know.
How Is Pulmonary Arterial Hypertension Treated?What caused your PAH, the symptoms, and how advanced your condition is all impact what treatment might work best for you. It may take time to figure out, but your doctor is there to help.
Options include:
Prescription drugs called vasodilators specifically treat PAH. These may be taken by mouth or given through an IVor subcutaneously, meaning under the skin. Some you inhale, or breathe in, from a nebulizer.
In some cases, you may need more than one type of drug to get your condition under control. You might also need other therapies like extra oxygen along with diet and lifestyle changes to help you feel better.
Intravenous (IV) treatments
Epoprostenol is a vasodilator that relaxes your blood vessels so it's easier for blood to move through them. There are two brands: Flolan and Veletri.
Flolan. In 1995, this was the first drug the FDA approved to treat PAH. It's given as a continuous injection through a vein for short-term use. A doctor can also surgically place a special catheter for long-term use. One study found that it helped make exercise easier and boosted survival rates.
Flolan has to be mixed every day. You must also:
Your catheter also needs to be checked often for signs of possible infection.
Side effects may include:
Selexipag IV (Uptravi). It can help slow the progression of PAH and lower your risk of being hospitalized. It's used only in patients who temporarily can't take oral medication.
Treprostinil (Remodulin). This is another IV formula of the same class of drugs as Flolan and Veletri. It's also stable at room temperature and doesn't have to be mixed daily. It can also be given under the skin.
Veletri. It's a newer version of epoprostenol approved by the FDA in 2010. It doesn't have to be mixed daily, and you can keep it at room temperature. Your doctor may suggest Veletri if you have severe PAH and other treatments haven't worked.
Side effects for Veletri and Remodulin are similar to those of Flolan. Catheters used for IVs should be checked regularly to avoid infections.
Nebulizer treatments
Iloprost (Ventavis). You breathe it in through a nebulizer about six to nine times every day. If you have asthma or other conditions that can make it hard to breathe, this may worsen those symptoms.
Treprostinil (Tyvaso). Your doctor may recommend this drug if you have moderate to severe PAH symptoms. You'll likely take it about four times a day. Research suggests it can help you walk longer distances.
Side effects of iloprost and treprostinil are similar to those of IV-based PAH drugs. But you may have less flushing or diarrhea with iloprost. Treprostinil may cause a sore throat and cough when you first start to use it.
Oral treatments
Ambrisentan (Letairis). This is a once-a-day medication, but unlike bosentan, it doesn't affect your liver.
Bosentan (Tracleer). This medication is taken two times a day. The drug could affect your liver function and hemoglobin levels. It's important to get your blood count and liver function tested regularly when you're taking bosentan.
Neither bosentan nor ambrisentan is recommended for people who are pregnant. Experts recommend two separate birth control options. Monthly pregnancy tests are required to continue using these drugs.
Sildenafil (Revatio). Small studies suggest this drug can improve blood flow in your lungs. The recommended dose is a 20-milligram pill taken three times a day, but it can be increased if you have a more severe form of PAH.
Side effects of sildenafil include:
Tadalafil (Adcirca). This is a once-a-day pill prescribed if you have moderate to severe PAH.
Treprostinil (Orenitram) is the oral formulation of Remodulin and can substitute for the IV medications after you have stabilized.
Other Ways to Treat Pulmonary Arterial HypertensionThese include:
Calcium channel blockers. This class of drugs, in select patients, can help lower the blood pressure in your lungs.
Blood thinners. Your risk of forming blood clots is higher if you have PAH. Blood thinners, like warfarin (Coumadin), are given to most people with PAH to cut down on clots and improve overall survival. But warfarin interacts with several foods and drugs. Your doctor can tell you what to avoid.
Diuretic. Sometimes called water pills, diuretics can help your body get rid of extra salt and water. This happens to most people with PAH. Your doctor will usually recommend one pill a day. But if you have a severe form of PAH, they may up the dosage.
Digoxin. This is an older medication sometimes prescribed to people with severe PAH. It's designed to help your heart pump blood better.
Oxygen therapy
When you have moderate to severe PAH, it can make it hard to breathe properly and affect your day-to-day life. The low levels of oxygen can make the blood vessels constrict further, which can make your PAH worse.
Getting a little extra oxygen can help you not only breathe better but also be able to get through daily tasks and routines. It can also boost your mood and sleep. While some may need extra oxygen every now and then, others may need it continuously.
Oxygen therapy comes in three types:
Before you try oxygen therapy, ask your doctor how much you need and how often you may need it. They can also guide you toward the type that works best for your lifestyle.
Surgery
If you have severe PAH and medications aren't working, your doctor may recommend a lung transplant. Lung transplant surgery is challenging and can come with serious complications. These include:
Your health care team can help you decide if it's right for you.
Things You Can Do to Manage Pulmonary Arterial HypertensionThere are some lifestyle changes you can try to help ease your symptoms.
You can:
Try to exercise and stay as active as you can. But don't lift heavy weight or do intense workouts. Talk to your doctor first if you're not sure what's safe.
PAH Treatment Outcomes Similar Regardless Of Diagnosis Time
The study findings underscore the importance of early initiation of macitentan and tadalafil among patients who have pulmonary arterial hypertension (PAH), and represent a shift in understanding of prognosis based on diagnosis timing.
Patients recently diagnosed with pulmonary arterial hypertension (PAH) have similar outcomes as those diagnosed more than 6 months ago, suggest new study findings published in Advances in Therapy.1
The study, which found that survival and hospitalization rates were comparable between the 2 groups of patients receiving macitentan and tadalafil (M+T), underscore the importance of early initiation of M+T and represent a shift in understanding of prognosis based on diagnosis timing.
"Historically, patients with PAH who are within 6 months of diagnosis (incident) have had a poorer prognosis in terms of survival and disease progression compared to patients who are further out from diagnosis (prevalent), an observation that is often attributed to the fact that prevalent populations are prone to survivor bias," explained the researchers. "However, analyses from the more recent US-based [Pulmonary Hypertension Association Registry] have shown no difference in all-cause mortality between incident and prevalent patients. Although the reasons for this shift are presumably multifactorial, more contemporary registries likely reflect advances made in the management of PAH, including greater and earlier use of combination therapy."
In this analysis, survival and hospitalization rates were comparable between newly diagnosed patients and those with a longstanding diagnosis.Image Credit: © Designpics - stock.Adobe.Com
The new findings from 2 cohorts: the prospective multicenter OPUS (NCT00125034) and retrospective PrPHeUS (NCT03197688) studies. Of the 1300 patients, 453 were newly diagnosed (≤ 6 month), 272 were newly diagnosed and received M+T as initial treatment, and 837 had longstanding diagnoses (> 6 months).
Results were similar across outcomes, despite newly diagnosed patients having more severe disease than those with a longstanding diagnosis, especially those who received M+T as initial treatment. These patients had worse World Health Organization Functional Class disease, 6-minute walking distances, and natriuretic peptide levels.
Newly diagnosed patients began taking M+T a median of less than 2 months after receiving their diagnosis, while patients with a long-standing diagnosis received the combined therapy a median of more than 3 years after diagnosis. At the time of analysis, patients were receiving M+T for an average of 12 to 14 months, and treatment exposure was similar between the groups. The treatment combination gained FDA approval in March, becoming the first and only once-daily, single-tablet combination for patients with PAH.2
By 1 year after the combination was initiated in patients, outcomes were similar: the survival rates were 91% (95% CI, 87.7%-93.7%) of newly diagnosed patients, 89% (95% CI, 83.2%-92.2%) of newly diagnosed patients receiving M+T as first-line treatment, and 93% (95% CI, 90.6%-94.6%) of longstanding patients estimated to be alive at 12 months.
The proportion of patients who remained free of hospitalization at 12 months were 59% (95% CI, 54.1%-64.4%) for newly diagnosed patients, 56% (95% C%, 49.1%-62.9%) for newly diagnosed patients receiving M+T as first-line treatment, and 62% (95% CI, 58.5%-65.9%) for long-standing patients.
"…in this analysis, the all-cause hospitalization rates were similar between the cohorts and high overall. Although the specific reasons for hospitalization are not available, hospital admission in general is most often related to PAH, and is one of the most common clinical worsening events in PAH trials," wrote the researchers. "While macitentan treatment has been shown to reduce all-cause hospitalizations in clinical trials, these observations highlight the need for continued risk assessment and pro- active treatment escalation, in order to further optimize outcomes."
Safety findings across the groups were also similar, and the observed safety profiles were consistent with those seen previously. Approximately half of each subgroup discontinued treatment throughout the study period, with the reasoning for discontinuation also being comparable between groups.
References
1. Chin KM, Channick R, Kim NH, et al. Macitentan and tadalafil combination therapy in incident and prevalent pulmonary arterial hypertension: real-world evidence from the OPUS/OrPHeUS studies. Adv Ther. Published online September 24, 2024. Doi:10.1007/s12325-024-02964-0
2. McNulty R. FDA approves macitentan, tadalafil combination tablet for pulmonary arterial hypertension. AJMC®. March 24, 2024. Accessed October 2, 2024. Https://www.Ajmc.Com/view/fda-approves-macitentan-tadalafil-combination-tablet-for-pulmonary-arterial-hypertension
Exercising When You Have Pulmonary Hypertension
Because pulmonary arterial hypertension (PAH) often causes shortness of breath, swelling, and fatigue, exercise may seem like the last thing you should do. But the right kind -- with your doctor's OK -- can help symptoms and boost quality of life.
How Exercise HelpsAlmost any way you slice it, exercise is good medicine.
"We call sitting the new smoking," says Eugene Chung, MD, chair of the American College of Cardiology Sports and Exercise Cardiology Leadership Council and professor of internal medicine at the University of Michigan. "If you live a predominantly sedentary lifestyle, the biggest bang for your buck, health-wise, is to get up and start moving around more often."
Regular exercise helps the heart and body work smarter, not harder. It keeps your blood vessels in good shape and eases inflammation. All these have a positive impact on PAH.
Here's how: PAH stresses the right ventricle of your heart. This stress starts a cascade of changes in your body. One of those is a bump in adrenaline, the hormone that triggers your "fight or flight" response.
"Exercise has been shown in multiple studies to help with decreasing inflammation and train the heart to respond to the adrenaline surge," says Chung.
This improves your peak exercise heart rate. That's how many beats per minute your heart can safely pump when exercising. It also lowers your blood pressure, which helps keep the cascade of changes from happening in the first place.
In short, exercise helps break the cycle of worsening PAH symptoms.
Best Ways to Move"Any exercise program should be started in consultation with your doctors, and you should be followed regularly," says Chung.
Your doctor understands your limitations and can tell you what's OK. Ideally, you'll exercise under the supervision of a cardiopulmonary rehabilitation program. As for the type of exercise that's best, Chung says they focus on aerobic activity. That's the kind that gets your heart pumping, not isometric moves that make you hold your muscle in a contracted position or high resistance exercise like weightlifting.
To get your blood pumping, you can try:
Walking. Get your steps in on a treadmill or by taking a brisk stroll around your neighborhood. Aim for 1-hour sessions three times a week.
Swimming. Working out in water tones your muscles without straining your joints. You can do water aerobics or simply swim laps.
Cycling. Elliptical bikes and recumbent bikes are a safe way to cycle without risking a fall.
Other types of exercise include:
Yoga. Although there isn't much research about the direct benefits of yoga on PAH, its slow and mindful stretching reduces stress and lowers inflammation in your body.
Light resistance training. You can keep your muscles flexible and strong by using light weights (soup cans can work well) or just body weight. A rehabilitation specialist can teach you moves such as chair squats, wall pushups, calf raises, bicep curls, and more.
It's important not to lift heavy weights because it can worsen symptoms.
"If you were to push it and do more high-intensity weightlifting, there's a chance that, depending on the cause of pulmonary hypertension, you could put increased strain on the right side of the heart," says Chung.
You're more likely to hold your breath as you lift as well, which raises the pressure in your chest cavity.
What to Watch ForLike with any exercise routine, be on the lookout for signs you've done too much. Keep these safety tips in mind:
Tell your doctor about any concerning side effects, like swelling or more shortness of breath than usual.
Comments
Post a Comment