What are the ESC (European Society of Cardiology) guidelines for the management of chronic thromboembolic pulmonary hypertension?

ESC Guidelines on Chronic Thromboembolic Pulmonary Hypertension

All patients with CTEPH require life-long anticoagulation and must be evaluated by a multidisciplinary expert team that includes an experienced pulmonary endarterectomy (PEA) surgeon to determine operability and treatment strategy. 1

Core Treatment Recommendations

Surgical Treatment (First-Line)

Surgical PEA performed in deep hypothermia circulatory arrest is the recommended treatment for patients with CTEPH (Class I, Level C). 1 This remains the treatment of choice as it offers the potential for cure and best long-term outcomes, with perioperative mortality <3% in expert centers. 2

Medical Therapy

Riociguat is recommended (Class I, Level B) for symptomatic patients who have: 1

• Persistent/recurrent CTEPH after surgical treatment, OR
• Inoperable CTEPH as determined by a CTEPH team including at least one experienced PEA surgeon

Off-label use of PAH-approved drugs may be considered (Class IIb, Level B) in symptomatic patients classified as having inoperable CTEPH by the expert team. 1

Interventional Treatment

Balloon pulmonary angioplasty (BPA) may be considered (Class IIb, Level C) in patients who are: 1

• Technically non-operable, OR
• Carry an unfavorable risk:benefit ratio for PEA

Recent data shows BPA has <1% mortality in expert centers and is increasingly used as part of multimodal treatment strategies. 3, 2

Essential Management Principles

Anticoagulation

Life-long anticoagulation is mandatory in all CTEPH patients (Class I, Level C). 1 While vitamin K antagonists have been the traditional standard, recent evidence suggests DOACs are equally effective and safe (no difference in VTE recurrence, bleeding, or mortality). 4

Multidisciplinary Team Evaluation

Every CTEPH patient must have operability assessed by a multidisciplinary team (Class I, Level C) that includes: 1

• At least one experienced PEA surgeon
• Cardiology and respiratory medicine physicians
• Radiologists with PH imaging expertise
• Access to interventional specialists

This is non-negotiable as surgical evaluation is the primary target, even if the patient ultimately receives other treatments.

Diagnostic Approach

Consider CTEPH in PE survivors with exercise dyspnea (Class IIa, Level C). 1 However, screening asymptomatic PE survivors is NOT recommended (Class III, Level C). 1

The diagnostic workup requires:

• Ventilation/perfusion or perfusion lung scan (Class I, Level C) 5
• Contrast CT pulmonary angiography (Class I, Level C) 5
• Right heart catheterization to confirm diagnosis 5, 6
• Pulmonary angiography should be considered (Class IIa, Level C) 5

Referral Center Requirements

Patients must be managed at specialized centers that: 1

• Follow ≥50 patients with PAH or CTEPH
• Receive ≥2 new CTEPH/PAH referrals monthly
• Have established networks with PEA surgery and lung transplantation services
• Provide multiprofessional team care including specialized nursing, psychological support, and 24/7 expertise

Treatment Algorithm

1. Confirm diagnosis via right heart catheterization showing mean PAP ≥25 mmHg, PAWP ≤15 mmHg, PVR >3 Wood units 5

2. Initiate life-long anticoagulation immediately 1

3. Refer to expert CTEPH center for multidisciplinary evaluation 1

4. Surgical assessment by experienced PEA surgeon determines:

   • Operable → Proceed with PEA surgery
   • Inoperable or persistent/recurrent post-PEA → Riociguat (first choice) or consider off-label PAH drugs
   • Technically non-operable or unfavorable surgical risk → Consider BPA

5. Multimodal approaches combining surgery, BPA, and medical therapy are increasingly common in carefully selected patients 2

Critical Pitfalls to Avoid

• Never make treatment decisions without surgical evaluation by an experienced PEA surgeon—patients deemed "inoperable" at non-expert centers may be surgical candidates at specialized centers
• Never delay anticoagulation while awaiting further evaluation
• Do not use medical therapy as first-line when surgery is feasible—this denies patients the only potentially curative option
• Do not rely on CT angiography alone for diagnosis—it can miss CTEPH; V/Q scanning is essential 5

The updated hemodynamic criteria (2022 ESC/ERS guidelines) now classify some patients previously labeled as CTEPD as having CTEPH, as these patients demonstrate impaired exercise physiology similar to more severe CTEPH. 7