Chronic Pulmonary Thromboembolism: Primary Treatment
For patients with chronic thromboembolic pulmonary hypertension (CTEPH), pulmonary thromboendarterectomy is the primary treatment of choice when the disease is surgically accessible, combined with lifelong anticoagulation. 1
Treatment Algorithm
Step 1: Initiate Lifelong Anticoagulation Immediately
- Extended anticoagulation is mandatory for all CTEPH patients and should never be stopped 1
- Warfarin (vitamin K antagonist) targeting INR 2.0-3.0 (target 2.5) remains the standard anticoagulation approach 1
- This reduces the risk of recurrent pulmonary embolism and is recommended before, during, and after any surgical evaluation 1
Important caveat: While direct oral anticoagulants (DOACs) like rivaroxaban are FDA-approved for acute PE treatment 2, their use in CTEPH specifically lacks robust evidence. The 2012 ACCP guidelines were prepared before adequate DOAC safety data were available and gave weak recommendations favoring warfarin and LMWH over DOACs 1. Small observational studies suggest DOACs may be safe in CTEPH 3, but warfarin remains the evidence-based choice until larger trials are completed 4.
Step 2: Refer to Experienced Surgical Center for Evaluation
- All CTEPH patients should be referred to an experienced pulmonary thromboendarterectomy center as soon as possible, even if symptoms are mild 1
- Pulmonary thromboendarterectomy is recommended for selected patients with CTEPH, particularly those with central (proximal) disease 1
- This is a Grade 2C recommendation, reflecting the specialized nature of the procedure but its potential for cure 1
Critical distinction: The determination of "operability" should only be made by experienced surgical teams at specialized centers—not by referring physicians 1. Factors that appear prohibitive (suprasystemic pulmonary pressures, severe RV dysfunction, high PVR >1400 dyne·s·cm⁻⁵) are NOT absolute contraindications at experienced centers 1.
Step 3: Surgical vs. Medical Management Decision
Surgical candidates (central disease):
- Pulmonary thromboendarterectomy offers potential cure with near-normalization of pulmonary hemodynamics 1
- Perioperative survival is 97.7%, with 5-year survival of 75-92.3% 1
- Patients experience marked improvement: 93% achieve NYHA class I or II post-operatively (compared to 95% in class III or IV pre-operatively) 1
- Continue lifelong anticoagulation post-surgery 1
Inoperable candidates (distal disease or significant microvascular component):
- Continue lifelong anticoagulation as primary therapy 1
- Consider pulmonary hypertension-specific medications (though these are approved for PAH, not specifically CTEPH) 5
- Lung transplantation may be considered in select cases 5
Step 4: Long-term Monitoring
- Routine re-evaluation at 3-6 months after acute PE to identify CTEPH development 1
- Refer symptomatic patients with mismatched perfusion defects on V/Q scan beyond 3 months to a pulmonary hypertension/CTEPH expert center 1
- Use echocardiography, natriuretic peptides, and cardiopulmonary exercise testing to guide referral decisions 1
Common Pitfalls to Avoid
Do not deem patients "inoperable" without expert surgical evaluation 1—severe hemodynamic abnormalities alone should not exclude surgical consideration
Do not routinely use inferior vena cava filters in CTEPH patients 1—they are only indicated for absolute contraindications to anticoagulation or recurrent PE despite adequate anticoagulation
Do not switch to DOACs without understanding the limited evidence base 1, 3, 4—warfarin remains the gold standard with the most robust data
Do not delay referral to specialized centers 1—early evaluation is critical even in minimally symptomatic patients, as surgical outcomes are better with earlier intervention