Chronic Thromboembolic Pulmonary Hypertension
ICD-10 I27.2 · ICD-11 BB01.3

Treatment of CTEPH with Operable Fibrotic Pulmonary Artery Obstructions

This protocol addresses chronic thromboembolic pulmonary hypertension (CTEPH) in patients where fibrotic, organised obstructions are present within the pulmonary arteries and the anatomy is confirmed to be surgically accessible — the operable form of CTEPH.

Clinical Scenario

Fibrotic thromboembolic material obstructs the pulmonary arteries, and the lesions are located in segments accessible by surgery. Surgical pulmonary endarterectomy (PEA) is the treatment of choice for patients with CTEPH and such accessible pulmonary artery lesions.

Management Approach (partial overview)

Lifelong anticoagulation forms a central component of management in all patients with CTEPH. The choice of anticoagulant agent is guided by individual patient factors. Additional supportive measures are incorporated alongside the primary intervention. Full agent selection, sequencing, and the complete regimen are available in the structured protocol →

Instant Access to Structured Evidence-Based Regimens

References

  1. PEA is recommended as the treatment of choice for patients with CTEPH and fibrotic obstructions within pulmonary arteries accessible by surgery.
  2. Surgical PEA is the treatment of choice for patients with accessible PA lesions.
  3. Lifelong, therapeutic doses of anticoagulation are recommended in all patients with CTEPH.
  4. VKAs are recommended by experts, and are most widely used as background therapy for patients with CTEPH.
  5. 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.

DOI: 10.1093/eurheartj/ehac237

View source ↗