Log in to MyACC
Worldwide CTEPH Registry Long-Term Outcomes
Quick Takes
- The current CTEPH registry aimed to evaluate the effect of the current therapeutic options for this condition, pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA), and medical therapy, in a worldwide context.
- Reduced mortality was associated with undergoing PEA and BPA.
- There was no survival difference in patients receiving vitamin K antagonists (VKAs) and non–VKA oral anticoagulants (NOACs).
Study Questions:
What are the effects of the current therapeutic options for chronic thromboembolic pulmonary hypertension (CTEPH) in a worldwide context?
Methods:
The European Chronic Thromboembolic Pulmonary Hypertension registry (CTEPH) prospectively collected data on consecutive CTEPH patients from 34 PH centers worldwide in 20 countries. Patients were required to be enrolled within 12 months of diagnosis and to be naïve to mechanical treatment (no pulmonary endarterectomy [PEA] or balloon pulmonary angioplasty [BPA] allowed before registry enrollment). They were enrolled between February 2015 and September 2016, and the registry was closed in September 2019.
This registry aimed to: 1) evaluate survival with current therapeutic approaches in a worldwide context, for PEA versus BPA versus no PEA/BPA, and for vitamin K antagonists (VKAs) versus non–VKA oral anticoagulants (NOACs); and 2) identify predictors of outcome in all patients and in patients undergoing PEA or BPA.
Results:
A total of 1,009 consecutive patients with CTEPH were included in this registry: 697 in Europe, 114 in Japan, and 44 in the United States. Their median age at diagnosis was 63 years, and 48% were male. A history of venous thromboembolism (VTE) was reported in 76% of patients. Median pulmonary artery pressure (PAP) was 44 mm Hg, and median pulmonary vascular resistance (PVR) was 643 dynes/sec·cm-5 (8 WU). Among these patients, 60% underwent PEA, 18% underwent BPA, and 22% received no mechanical treatment. Among the patients who did not undergo a PEA, 56% were technically inoperable, 11% declined surgery, 14% had comorbidities precluding surgery, and 19% were considered operable but were not operated. Most patients undergoing BPA (63%) received PH drugs before the procedure compared with 25% of the patients undergoing PEA. The anticoagulation of choice was a VKA only in 54% of patients, and NOAC only in 24% of patients (14% of patients had an undefined anticoagulation regimen).
In the PEA group, 14% of patients had additional cardiac procedures: coronary artery bypass grafting (6%), patent foramen ovale or atrial septal defect closure (5%), tricuspid valve surgery (2%), aortic/mitral valve surgery (2%), redo sternotomy (1%), or other interventions (2%). The 185 BPA patients underwent a total of 908 BPA sessions (median 5) within 192 days. The 16 patients with BPA after PEA had a total of 57 BPA sessions (median 2.5). At the right heart catheterization performed >3 months after the intervention, PVR similarly decreased by 57% and 59% for PEA and BPA, respectively. Persisting PH, defined as mean PAP >25 mm Hg, was observed in approximately 50% of patients undergoing PEA or BPA.
The survival rates of the overall cohort were 95%, 92%, and 89% at 1, 2, and 3 years, respectively. Survival rates at 3 years were 94% for PEA, 92% for BPA, and 71% for patients not receiving PEA or BPA. There was no significant difference in survival between patients on VKA or NOAC or “switchers.”
Conclusions:
This second international CTEPH registry reveals important improvement in patient survival since the introduction of BPA and an approved drug for PH. The type of anticoagulation regimen did not influence survival.
Perspective:
The European CTEPH registry, which was conducted between 2007 and 2012, reported the impact of PEA on long-term outcomes in patients with CTEPH, showing a significantly worse survival in those patients who did not undergo PEA. Since then, additional therapeutic options have become available for the management of this condition, including BPA and medical therapy—mostly in the form of riociguat—but their long-term impact in this patient population is a topic of debate.
The worldwide CTEPH registry is very timely and informative on how outcomes are affected by the currently available therapeutic options. Patients who undergo PEA have the best survival, followed closely by those who undergo BPA. Patients who cannot undergo either PEA or BPA have a dramatically lower long-term survival. These findings underscore the importance of mechanical revascularization. PEA remains the treatment of choice for patients with CTEPH who have surgical accessible disease. The long-term favorable outcomes associated with BPA reinforce this therapeutic option as an alternative for patients who do not have surgically accessible disease. It is important to mention that the outcomes presented in this manuscript likely represent a selected group of expert and experienced centers and highlights the importance of a multidisciplinary CTEPH approach to the treatment of this condition.
Much debate has occurred around the question: What is the anticoagulation of choice in patients with CTEPH? The findings presented by Delcroix et al. support the concept that either VKAs or NOACs are appropriate choices for anticoagulation in this population.
Clinical Topics: Anticoagulation Management, Diabetes and Cardiometabolic Disease, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Vascular Medicine, Pulmonary Hypertension, Interventions and Vascular Medicine, Hypertension
Keywords: Angioplasty, Balloon, Anticoagulants, Endarterectomy, Hypertension, Pulmonary
< Back to Listings
