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CTEPH Treatment, Management Varies Widely Across Globe
A new report shows wide variability in terms of which patients with chronic thromboembolic pulmonary hypertension (CTEPH) are deemed eligible for pulmonary endarterectomy and balloon pulmonary angioplasty.
Recent medical advances have improved the treatment of chronic thromboembolic pulmonary hypertension (CTEPH), but a new report shows wide variability in which patients receive which therapies.
The study was published in the journal Pulmonary Circulation.1
New and existing treatments can significantly improve outcomes for patients with CTEPH, noted the study authors. However, they also said treatment decisions can depend on a number of factors, and many patients are deemed ineligible for some of the most effective therapies.
For example, pulmonary endarterectomy (PEA) can lead to long-term improvement in most patients, but the investigators said some patients refuse the procedure, and others are ineligible due to distal disease or an unfavorable risk profile. Additionally, about a quarter of patients require additional intervention even after PEA.
Treatments for chronic thromboembolic pulmonary hypertension depend on several factors, which include patient refusal or ineligibility because of distal diseaseImage Credit: Dzmitry - stock.Adobe.Com
Patients who cannot undergo PEA or who have symptoms following the procedure can benefit from balloon pulmonary angioplasty (BPA), they added. Several drugs have also been shown to lead to improvement in some patients with CTEPH, including the oral guanylate cyclase stimulator riociguat (Adempas; Bayer), and some physicians prescribe off-label use of therapies designed to treat pulmonary arterial hypertension, they said.
Yet, the study investigators said there remains a lack of consensus about the optimal therapeutic targets for patients, the ideal sequencing of therapies, and the role of medical therapy in the preoperative and interventional settings. In the absence of that consensus, they sought to better understand what is happening in real-world practice.
The authors used the CTEPH Global Cross-Sectional Scientific Survey (or CLARITY) survey, which included 110 closed- and open-ended questions and was available in 12 languages. It was distributed via 21 international, regional, and global scientific societies between September 2021 and May 2022. Recipients were asked to complete the survey if they were involved in the treatment of acute pulmonary embolism or CTEPH.
In all, 416 people responded to the survey. The new data are based on the 212 responses from clinicians who said they were involved in the treatment of CTEPH. Most respondents (approximately 71%) were from the Asia-Pacific region or Europe. Half were pulmonologists and 37% were cardiologists. Other respondents indicated they specialized in cardiothoracic surgery, internal medicine, vascular medicine, hematology, and radiology. A plurality of respondents (46%) said they had been working in their specialization between 15 and 29 years. Most said they were at centers that performed up to 50 PEA and/or BPA procedures per year.
The investigators found wide variation when they asked about the proportion of patients with CTEPH who were deemed eligible for PEA. In Europe, North America, and South America, more than half of respondents said that at least half of their patients in the previous year were deemed eligible for PEA. However, only 9% of respondents from the Asia-Pacific region said a majority of their patients were eligible.
In terms of carrying out the procedure, European and North American respondents said more than half of their patients with CTEPH underwent PEA, while most respondents from South America and the Asia-Pacific region said they performed the procedure on less than half of their patients with CTEPH.
When asked why patients eligible for PEA refused the procedure, the most common reasons were fear of adverse events, a preference for BPA, a lack of awareness about the treatment and outcomes, and concerns about the costs of the procedure and/or the hospital stay.
In terms of BPA, respondents from the Asia-Pacific region were more likely to report higher proportions of patients as eligible for the procedure. In Europe, North America, and South America, most respondents said a minority of patients were eligible for BPA.
Asked about the treatment goals for BPA, most respondents said quality of life, improved New York Heart Association functional class, symptom relief, and improved right ventricle function.
The study authors found that the use of medications such as riociguat was common, but also varied.
The investigators also said their findings suggest that right heart catheterization is underutilized. They cited a 2022 study showing that among 103 patients in the US who underwent PEA, only 5% were subsequently given right heart catheterization.2 The authors of that study said the findings imply that patients are not being referred for full diagnostic workups despite post-PEA symptoms of pulmonary hypertension.
In all, the present investigators said, the survey shows best practices for CTEPH therapy remain unclear.
"The observed heterogeneity in treatment approaches highlights the need to address access barriers to PEA and BPA, taking into consideration country-specific health care system factors, including the financing and organization of care," the investigators concluded. "In addition, the optimal sequence of multimodal therapies, taking into account the disease distribution, patient status, and respective treatment goals, warrants further research.
References
1. Skoro-Sajer N, Sheares K, Forfia P, et al. Treatment and management of chronic thromboembolic pulmonary hypertension (CTEPH): a global cross-sectional scientific survey (CLARITY). Pulm Circ. 2024;14(2):e12406. Doi:10.1002/pul2.12406
2. Butler O, Ju S, Hoernig S, Vogtländer K, Bansilal S, Heresi GA. Assessment for residual disease after pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension. ERJ Open Res. 2022;8(2):00572-2021. Doi:10.1183/23120541.00572-2021
Chronic Thromboembolic Pulmonary Hypertension
Content
Once CTEPH develops, the most effective cure is a surgical procedure known as pulmonary endarterectomy that reestablishes normal blood flow to the lungs.
This surgery can reverse pulmonary hypertension and reverse damage to the heart and other organs. With surgery, patients can expect excellent long-term survival approaching 90% at five years. Without surgery, patients have substantially reduced long-term survival and quality of life.
Are there non-surgical options for CTEPH?
Medications may be an option for patients with CTEPH to prevent further clot buildup and to relax the wall of the blood vessels.
In addition, patients may be candidates for a minimally invasive procedure called pulmonary balloon angioplasty.
How do you know if you have CTEPH?
CTEPH is a vastly underdiagnosed disease. But some of the symptoms include:
Any patient with a history of blood clot formations should consider seeing a specialist if they are having trouble performing daily activities due to less energy or shortness of breath.
Survivors of acute pulmonary embolism can develop long-term pulmonary hypertension. So any patient with a history of acute pulmonary embolism should undergo an annual echocardiogram to check the function of the heart.
The Pathway To A Medical Diagnosis
What you need to know to prepare for a conversation with your doctor, understand the diagnostic process, and manage your expectations.
Step 1: Seek answersEveryone has aches and pains now and then. Most of us know what it's like to feel extra-tired sometimes, too. These kinds of symptoms are usually due to an injury, or are caused by the flu virus or another temporary illness. But if you've had joint pain and extreme fatigue for more than a few weeks, or you've noticed unusual hair loss or a face rash—and especially if you have any risk factors for an autoimmune disease—it's time to stop waiting.
It's time to take the first step: to have a conversation with your doctor about the changes you've been experiencing, how your health is being affected, and whether these signs and symptoms could be due to the autoimmune disease lupus, or some other illness or condition.
Step 2: Learn how lupus is diagnosedIn order to give you answers about what your symptoms or risk factors may mean, your doctor will likely:
This information will allow the doctor to begin to rule out medical problems that don't fit your health picture. However, diagnosing lupus isn't easy. This is because:
So, while the information from your medical history and physical exam and lab tests may suggest lupus or another condition or disease, there can still be uncertainty. This is why your doctor will probably not be able to tell you, at your first visit, whether or not you have lupus.
Diagnosing a complex disease such as lupus will often take several months, and two or three doctor appointments, as you continue to track your symptoms, and your doctor continues to monitor your lab test results and your overall health.
If your doctor believes your signs and symptoms point to the possibility of lupus, he/she may recommend a referral to a specialist doctor who has training in the group of autoimmune diseases that includes lupus. This specialist is called a rheumatologist (ROOM-ah-TOHL-ah-JYST).
Learn more: Diagnosing Lupus: A Guide
Step 3: Prepare for your first appointmentBecause lupus is not a simple disease to recognize, it's a good idea to start by seeing a general medicine doctor—also known as Primary Care Provider, General Practitioner, or Family Physician.
There are several kinds of health care providers who work in the office of a primary care doctor. You may see a medical doctor (MD), a physician assistant (PA), or a nurse practitioner (NP). By asking you questions and listening to your answers, your provider will build a picture of your medical condition and immediate concerns. The clearer the picture, the sooner the provider will be able to give you an accurate diagnosis, or refer you to a specialist physician.
No matter which provider you see, it's helpful to remember that today's health care system works best when you look at your care as a team effort. You're also taking on a responsibility, because in any meeting between yourself and a provider, you are the team leader. And as the leader, you can make the job easier for other members of the team.
Here are seven ways you can help your health care providers better understand your health and your health care needs, and make decisions about a diagnosis.See more: Six keys to preparing for a doctor's appointment
Step 4: Follow upIt can be very hard to take in everything that's said at a doctor's appointment. Before the doctor leaves the room, ask for a brief summary of his/her assessment of your condition, and the action plan to address it, including follow-up. You can also request a printed summary to be mailed to you, including any instructions.
Your action plan may include some or all of these steps:
It's not easy to face the unknown. You may need all of your patience and courage while waiting for your doctor to share with you the results of his/her investigations. Stay busy with things you enjoy, get plenty of rest, and try not to worry. By seeking answers that explain sudden or long-term changes in your health you've taken a very important step along your journey to improved quality of life. It's a journey that affects everyone who cares about you and depends upon you!
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