Ways of treating CTEPH can vary widely across globe, study finds
Look into responses of experts worldwide who answered CLARITY survey
How chronic thromboembolic pulmonary hypertension (CTEPH) is managed across regions globally varies widely despite treatment advances, a survey study found, highlighting the need for more research, better guidelines, and ongoing education for healthcare providers.
The study, “Treatment and management of chronic thromboembolic pulmonary hypertension (CTEPH): A global cross-sectional scientific survey (CLARITY),” was published in Pulmonary Circulation by an international team of researchers.
CTEPH is a rare form of pulmonary hypertension that occurs when blood clots block the pulmonary arteries in the lungs, resulting in abnormally high blood pressure in those arteries. This makes the heart work harder to pump blood, which can cause heart failure over time.
Recent advances in treatment have improved how CTEPH is managed, leading to better outcomes for patients whose prognosis without treatment would be poor. However, “global insight on clinical practices remains limited,” the researchers wrote.
212 CTEPH clinicians answered the 2021-22 worldwide survey
Scientists at centers worldwide collected information on how CTEPH is treated and managed through the global CLARITY survey, which was sent to hospital-based medical specialists from September 2021 to May 2022. The goal was to understand current clinical practices and identify areas where more support is needed.
A total of 212 specialists responded to the 110-question survey, which was available in 12 languages. Most respondents were from the Asia-Pacific region (39%) or Europe (32%), with 14% from countries in both North and South America, followed by 2% from countries in the Middle East and Africa. Most clinicians, 80%, had five to 29 years of working experience.
Most also came from centers that perform up to 50 pulmonary endarterectomy (PEA) and/or balloon pulmonary angioplasty (BPA) procedures each year. PEA is a surgery to remove blood clots from the pulmonary arteries, whereas BPA uses a balloon to open narrowed pulmonary arteries.
Survey answers showed a lot of variation in how many patients are considered suitable for these procedures, and how many actually receive them. This finding suggests differences in how doctors at different centers treat CTEPH and in the resources available to them.
For example, across the Asia-Pacific region and South America, fewer patients underwent PEA, with the most common reason for refusing the surgery being fear of complications. PEAs were more frequently given to eligible patients in Europe and North America.
According to the specialists, some eligible patients do not undergo PEA because of few specialized centers and doctors with expertise in performing them. Financial issues, such as the cost to patients and reimbursement policies, also limit access to the surgery.
Most specialists schedule a follow-up within three months of a patient’s surgery, with up to about half following patients across their lifetime. Some patients experience ongoing or returning pulmonary hypertension despite the surgery, but few undergo a second PEA, the survey showed.
The proportion of patients considered eligible to undergo BPA was higher in the Asia-Pacific region, and centers performing more than 50 procedures each year had a higher proportion of eligible patients compared to those with fewer procedures.
Most specialists considered BPA for inoperable patients with blood clots in areas far from the body’s trunk, persistent or recurrent pulmonary hypertension after PEA, an unfavorable risk/benefit ratio, or a poor response to medication.
A follow-up appointment, again, typically took place within three months of BPA, with about two-thirds of specialists reporting to follow patients lifelong. Barriers to BPA included few specialized centers and a lack of expertise, insufficient data on long-term benefits, and financial issues. These barriers were less common in Europe, where 43% of doctors reported no such limitations, than in the Asia-Pacific region, where 18 doctors (22%) also saw no such limits but most (14 of the 18) were based in Japan.
Medications to prevent blood clots in common use globally
Differences were found in how doctors combine treatments and in how they follow up with patients after treatment. Some centers use a combination of surgery and medications, while others focus on just one type of treatment.
Medications to treat pulmonary arterial hypertension often were used before surgery and for CTEPH patients who could not have surgery. Vitamin K antagonists and direct oral anticoagulants — both given to prevent blood clots — were commonly used (86% and 82%, respectively), but reasons for their use differed.
Follow-up care practices also varied, highlighting the lack of a standard approach in caring for patients under treatment. Differences in CTEPH treatment approaches likely are due to the differing experience of specialists at given medical centers and regional healthcare barriers, the team suggested.
As scientists in an earlier analysis of the CLARITY survey concluded, greater efforts in advancing research, developing clinical guidelines, and providing ongoing education for healthcare providers are needed to ensure consistent care for people with CTEPH worldwide.
“Our findings highlight the importance of additional clinical and cohort [group] studies, comprehensive clinical guidelines, and continued education to optimize patient care from treatment decision-making to patient follow-up,” the researchers in this study also concluded.
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