Management of Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
All patients with CTEPH require lifelong anticoagulation, and selected patients with accessible central disease should undergo pulmonary thromboendarterectomy (PEA) at an experienced center, as this is the only potentially curative treatment. 1
Mandatory Anticoagulation
Extended anticoagulation is recommended indefinitely for all patients with CTEPH (Grade 1B recommendation), as this prevents recurrent venous thromboembolism and potentially slows disease progression. 1
Vitamin K antagonists (VKA) remain the preferred anticoagulant, with target INR maintained at 2.5 (range 2.0-3.0) regardless of treatment duration. 1
Direct oral anticoagulants (DOACs) show promise but have concerning data: while associated with lower mortality (RR 0.54), they carry a significantly higher risk of recurrent pulmonary embolism (RR 3.80) compared to VKA. 2 Given this increased PE recurrence risk, VKA should be preferred over DOACs in CTEPH until more robust evidence emerges.
Reassess anticoagulation tolerance, adherence, hepatic and renal function, and bleeding risk at regular intervals. 1
Surgical Treatment: Pulmonary Endarterectomy
PEA surgery is the cornerstone and treatment of choice for CTEPH patients with accessible central disease who are under the care of an experienced thromboendarterectomy team (Grade 2C recommendation). 1, 3
PEA should be performed at specialized centers performing a minimum of 50 PEA surgeries per year. 4
This procedure can be curative and provides the best long-term outcomes when disease is surgically accessible and patients lack significant comorbidities. 3
Patients with persistent pulmonary hypertension after PEA should receive medical therapy and be considered for balloon pulmonary angioplasty (BPA) or re-do endarterectomy. 3
Interventional Treatment: Balloon Pulmonary Angioplasty
BPA should be considered for patients with disease burden that is surgically inaccessible or when surgical risk is prohibitive. 3, 5
BPA is performed at specialized centers conducting a minimum of 100 BPA interventions per year. 4
This procedure is safe and effective in select patients, though evidence comes primarily from small observational studies with follow-up limited to 1 year. 5
Medical Therapy
Pulmonary hypertension-targeted medical therapy should be initiated in patients who are not surgical candidates or who have persistent pulmonary hypertension after PEA. 3, 4
Medical therapy alone is appropriate for patients with inaccessible disease or those who fail to justify the risk of major surgery. 3
The three treatment modalities (PEA, BPA, and medical therapy) are not mutually exclusive and are increasingly combined in multimodal treatment concepts. 4
Diagnostic Confirmation and Referral
CTEPH is diagnosed by abnormal lung perfusion despite 3 months of oral anticoagulation, with persistent pulmonary artery thromboembolic/fibrotic material on cross-sectional imaging and pulmonary hypertension confirmed on right heart catheterization. 3
Symptomatic patients with mismatched perfusion defects on V/Q scan beyond 3 months after acute PE must be referred to a pulmonary hypertension/CTEPH expert center, taking into account echocardiography, natriuretic peptide, and/or cardiopulmonary exercise testing results. 1
Early referral to specialized CTEPH centers is critical, as treatment decisions require expertise in determining whether PEA, BPA, or medical therapy is most beneficial. 5, 4
Post-Treatment Follow-Up
All CTEPH patients require long-term follow-up, preferably at specialized CTEPH centers. 4
Routine clinical evaluation should occur at 3-6 months after acute PE to screen for CTEPH development. 1
An integrated care model is essential to ensure optimal transition from hospital to ambulatory care. 1
Critical Pitfalls to Avoid
Never discontinue anticoagulation in CTEPH patients—the prognosis without treatment is bleak, but effective therapies exist. 3
Do not delay referral to a CTEPH expert center once the diagnosis is suspected, as treatment decisions are complex and require specialized expertise. 1
Do not assume all CTEPH patients are surgical candidates—treatment strategy must be determined by a multidisciplinary team at an experienced center. 3
Avoid using DOACs as first-line anticoagulation given the higher recurrent PE risk; VKA remains the gold standard. 2