Treatment of Chronic Thromboembolic Pulmonary Hypertension When Technically Inoperable

Clinical Scenario

This protocol addresses patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are technically inoperable — the fibrotic obstructions within the pulmonary arteries are located in segments not accessible by surgical intervention.

Because operative removal of the obstructing material is not feasible, management follows a non-surgical evidence-based pathway.

Treatment Approach

The cornerstone of therapy in technically inoperable CTEPH is lifelong anticoagulation, with agent selection guided by individual patient characteristics. Supervised exercise training is incorporated as a complementary measure.

The full protocol specifies the recommended anticoagulant class, the clinical factors that determine agent choice, and the complete sequence of management steps — view the structured regimen below.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1093/eurheartj/ehac237

Riociguat is recommended for symptomatic patients with inoperable CTEPH or persistent/recurrent PH after PEA
BPA is recommended in patients who are technically inoperable or have residual PH after PEA and distal obstructions amenable to BPA
Lifelong, therapeutic doses of anticoagulation are recommended in all patients with CTEPH
VKAs are recommended by experts, and are most widely used as background therapy for patients with CTEPH.
More recently, NOACs have more frequently been used as alternatives to VKAs, again, lacking evidence from RCTs.
In patients with CTEPH and antiphospholipid syndrome, anticoagulation with VKAs is recommended
General measures recommended for PAH also apply to CTEPH, including supervised exercise training, which is effective and safe in inoperable CTEPH patients, as well as early after PEA.

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