Systemic Consequences of Pulmonary Hypertension and Right-Sided Heart Failure | Circulation
Top 5 Most-Read PAH Articles Of 2024
The top articles about pulmonary arterial hypertension (PAH) in 2024 included FDA approvals, the effect of salt substitutes on hypertension, and how diagnosis time affects outcomes.
Outcomes based on diagnosis time, how salt substitute affects hypertension, and FDA approvals made up our 5 top articles of 2024 about pulmonary arterial hypertension (PAH).
Below are our top 5 most-read articles of this year. To view the entirely of our PAH coverage, visit our PAH page.
5. Researchers Emphasize Importance of Appropriate Kidney Transplant Management Based on PH Type
Considerations and management strategies for patients who are undergoing kidney transplant and also have pulmonary hypertension (PH) were outlined in a study published in February. These subtypes of PH included PAH and 4 groups that were secondary to other conditions, including heart disease, lung disease, and chronic thromboembolic disease. Disease severity, patient preferences, and safety profile should be considered for patients with PAH prior to their kidney transplant whereas the other groups should be treated through the use of the American Heart Association guidelines, oxygen therapy, or anticoagulation, depending on their condition.
Read the full article here.
4. FDA Approves Macitentan, Tadalafil Combination Tablet for Pulmonary Arterial Hypertension
The FDA approved the use of a single-tablet combination of macitentan (OPSUMIT) and tadalafil (Cialis) in March 2024 to be used for chronic treatment of PAH in those who are either on an endothelin receptor antagonist, phosphodiesterase 5 inhibitor, both, or are treatment-naïve. The results from the A DUE study was the basis of the approval, with patients using the combination therapy having greater reductions in pulmonary vascular resistance compared with those taking either macitentan or tadalafil monotherapy after 16 weeks.
Read the full article here.
3. Salt Substitute Reduces Incidence of Hypertension in Older Adults
Replacing regular salt with a salt substitute in older adults with a normal blood pressure was able to reduce the incidence of hypertension while not increasing the rate of hypotension. The study, which evaluated older adults in elderly care centers in China, found that those who used the salt substitute had an incidence of hypertension of 11.7 per 100 person-years after 2 years compared with 24.3 per 100 person-years in participants who used normal salt. Participants were 40% less likely to develop hypertension when using the salt substitute.
Read the full article here.
The top articles about PAH in 2024 centered on FDA approvals and hypertensionImage credit: tashatuvango - stock.Adobe.Com
2. FDA Approves Sotatercept, First-in-Class Treatment for Adults With PAH
Sotatercept (Winrevair; Merck), an activin signaling inhibitor, was approved in March 2024 for the treatment of patients with PAH. The approval was based on the STELLAR trial conducted in April 2023 which helped patients with PAH improve their 6-minute walking distance by a median of 34.4 m after 24 weeks compared with just 1.0 m in the placebo group. Sotatercept was also able to improve all secondary endpoints in patients, including World Health Organization functional class, time to death or clinical worsening, and change in pulmonary vascular resistance.
Read the full article here.
1. PAH Treatment Outcomes Similar Regardless of Diagnosis Time
A new study found that patients diagnosed with PAH recently had similar outcomes to those diagnosed more than 6 months prior. Both groups of patients had similar survival and hospitalization rates after receiving the combination of macitentan and tadalafil. Even though those newly diagnosed started receiving the combination therapy a median of less than 2 months after their diagnosis compared with a median of more than 3 years after diagnosis for those with a long-standing diagnosis, their survival rates (89% vs 93%) were similar after 1 year of receiving the combination therapy.
Read the full article here.
Understanding Altitude Hypertension And Its Effects
Not everyone will develop hypertension at high altitudes. Fitness level, genetics, rate of ascent, and hydration/nutrition can all matter.
You may be more likely to experience high altitude hypertension if you don't take time to acclimate, if you're dehydrated, and if you have pre-existing conditions. Even in the best physical health, the higher you go in elevation, the more likely you are to experience elevated blood pressure.
Why Early Diagnosis Of PAH Is So Important
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Each year, 500 to 1,000 people in the United States are diagnosed with pulmonary arterial hypertension (PAH). Because this progressive, chronic disorder is rare, it is often under-detected, causing delayed diagnosis with a potentially significant impact on people living with the disease.
To raise awareness and provide resources about PAH with a goal of speeding the path to diagnosis and understanding treatment options, the American Lung Association, with support from Merck, is sharing the following facts and insights:
What is PAH? Pulmonary arterial hypertension is a specific type of pulmonary hypertension caused when the tiny arteries in the lung become thickened and narrowed. This blocks blood flow through the lungs, raising the blood pressure in the lungs and causing the heart to work harder. In the early stages of PAH, it is possible a patient may not notice any symptoms at all. As the disease progresses, they will start to experience symptoms common to other lung diseases. The most common symptoms include:
Why is early diagnosis important? Left untreated, PAH will progress and is deadly. The path to diagnosis can be long, frustrating and difficult. In fact, the average time to accurate diagnosis is over two years. However, the sooner an individual is diagnosed, the sooner they can begin the correct treatment protocol to best manage their symptoms.
What barriers are there to treatment and diagnosis? Because symptoms are similar to other common lung diseases, it can often be hard to diagnose PAH. Individuals with rare diseases are more likely to have limited access to specialized care options, lack providers who understand their disease, and often face high treatment costs.
Who is most at risk? PAH is most common in women between ages 30-60 and disproportionately impacts Black and Hispanic women. Members of these groups are also more likely to experience worse health outcomes once diagnosed due to delayed diagnosis and barriers to care caused by systemic inequalities in the healthcare system.
How is PAH treated? Although there is no cure for PAH, there are medications and treatments that can slow the progression of the disease and improve a person's quality of life. Treatments include medication, supplemental oxygen, pulmonary rehabilitation or, in severe cases, a lung transplant. The treatments available for PAH specifically target and restore balance among one or more of three pathways in the lungs that contribute to disease progression: nitric oxide, endothelin, and prostacyclin.
Where can those living with PAH find support? Those living with PAH should work closely with their healthcare team to manage their disease. Support can be found by calling the American Lung Association HelpLine at 1-800-586-4872 and pressing 2. Callers can ask questions about their lung health and receive help finding a specialist, support or guidance for financial assistance.
Having PAH is life changing. However, being diagnosed as soon as possible can help you get on the right path to optimal disease management. For more information about PAH visit Lung.Org/pah.
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