Treatment of Technically Inoperable Chronic Thromboembolic Pulmonary Hypertension

In a subset of patients with chronic thromboembolic pulmonary hypertension (CTEPH), fibrotic obstructions within the pulmonary arteries are located in segments not surgically accessible, making pulmonary endarterectomy technically infeasible. This distinct presentation requires a dedicated medical approach.

Clinical Scenario

CTEPH in which fibrotic obstructions within the pulmonary arteries are not accessible by surgery. Pulmonary endarterectomy cannot be performed, and non-surgical strategies must be evaluated to address elevated pulmonary vascular resistance and impaired functional status.

Treatment Approach (Overview)

Medical therapy is the cornerstone of management for technically inoperable CTEPH. A Class I–recommended targeted agent exists for this population, and the selection among available medical options is guided by the patient's functional class and haemodynamic profile. The relationship between medical therapy and certain interventional approaches also shapes the overall management sequence.

The complete regimen — including agent selection, functional-class thresholds, sequencing with interventional options, and the role of combination strategies — is detailed in the structured protocol.

Treatment Goals

The primary clinical objectives are achieving a good functional class (WHO-FC I–II) and normalization or near-normalization of haemodynamics at rest, assessed by right heart catheterization 3–6 months after the procedure.

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.
Treprostinil s.c. may be considered in patients in WHO-FC III-IV who have inoperable CTEPH or persistent/recurrent PH after PEA.
Off-label use of drugs approved for PAH may be considered in symptomatic patients who have inoperable CTEPH.
In patients with inoperable CTEPH, a combination of sGC stimulator/PDE5i, ERA, or parenteral prostacyclin analogues may be considered.
In patients with CTEPH who are candidates for BPA, medical therapy should be considered prior to the intervention.
Most experts accept achieving a good functional class (WHO-FC I-II) and/or normalization or near normalization of haemodynamics at rest, obtained at RHC 3-6 months post-procedure (PEA or last BPA), and improvement in quality of life.

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