CTEPH

2 min read Updated 2026-03-24
respirology cardiology pulmonary-hypertension thromboembolism cteph
Contents
CTEPH

Chronic thromboembolic pulmonary hypertension (group 4 PH) results from organised thrombus obstructing the pulmonary arteries, with secondary distal vasculopathy. It is the only potentially curable form of pulmonary hypertension — pulmonary endarterectomy (PEA) is curative in operable patients. ~25% of patients have no history of prior DVT/PE. V/Q scan is the key screening test (sensitivity 96–97%). All patients require lifelong anticoagulation.

diagnosis

  • V/Q scan is mandatory in all PH workups (sensitivity 96–97% for CTEPH; CTPA may miss chronic disease)

  • ~25% of CTEPH patients have no history of prior DVT/PE

  • Lifelong therapeutic anticoagulation for all patients (ESC/ERS 2022, Class I, Level C)

  • VKAs (warfarin) remain expert-preferred — best studied, guideline-aligned

  • VKAs mandatory if antiphospholipid syndrome (~10% of CTEPH) — DOACs inferior in APS

  • DOACs increasingly used with likely comparable efficacy and lower bleeding, but no CTEPH-specific RCT data yet and concern for higher acute thrombi at surgery

  • Individualise: VKAs if APS or pre-PEA; DOACs reasonable in stable patients without APS

do not rely on CTPA alone

CT pulmonary angiography has lower sensitivity than V/Q scanning for chronic thromboembolic disease. A normal CTPA does not exclude CTEPH — V/Q scan is the screening test of choice.

treatment hierarchy

1. pulmonary endarterectomy (PEA)

Only curative therapy. All patients should be evaluated for operability at an expert centre. Perioperative mortality < 5% at experienced centres. Refer early — delay allows secondary distal vasculopathy to develop, making surgery less effective.

  • Operable: proximal thrombi (main, lobar, segmental arteries) accessible to surgery
  • Inoperable (~1/3 of patients): distal disease, PVR–thrombus mismatch, or prohibitive comorbidities
  • Do not routinely use PAH-specific therapy as bridge to surgery (may delay referral without mortality benefit). Exception: haemodynamic stabilisation in RV failure pre-PEA.

2. balloon pulmonary angioplasty (BPA)

For inoperable disease or residual PH post-PEA.

  • Multiple sessions required (median 5)
  • Contemporary complication rates: 7–12% overall (reperfusion pulmonary oedema, PA perforation); severe complications < 2% at experienced centres
  • 5-year survival ~90–95% at experienced centres (international registry 2025: 3-year overall survival > 94%)

3. riociguat

For inoperable CTEPH or persistent PH post-PEA (CHEST-1 (2013)). Often combined with BPA — RACE (2022) compared BPA vs riociguat in inoperable CTEPH; BPA showed greater haemodynamic improvement (PVR reduction) than riociguat alone.

4. lung transplantation

Last resort for patients refractory to PEA, BPA, and medical therapy.

key trials summary

TrialYearNDesignKey finding
CHEST-12013261RCTRiociguat improved 6MWD by 46m in inoperable CTEPH
RACE2022105RCTBPA superior to riociguat for PVR reduction in inoperable CTEPH

Key references

All sources (9)