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CTEPH: treatment

CTEPH: treatment options and guidelines

The first choice treatment for CTEPH is surgical, but medical treatment also plays a role in the treatment of select CTEPH patients

Catharina Belge MD PhD Dept of Respiratory Medicine, University Hospital Gasthuisberg, Leuven, Belgium

The first choice treatment for chronic thromboembolic pulmonary hypertension (CTEPH) is surgical, comprising the removal of intra-arterial obstructive material by pulmonary endarterectomy (PEA). PEA is the only potentially curative treatment for CTEPH. According to the international CTEPH registry,1

CTEPH diagnosis Continue life-long anticoagulation Operability assessment by CTEPH team Operable 62.9% of patients were considered

operable. However, some patients are inoperable owing to the occlusion of distal vessels or coexisting medical conditions, and some decline surgery. Furthermore, up to 16.7% have persistent pulmonary hypertension after surgery.2

As PEA remains the treatment of choice in operable CTEPH patients, all cases of CTEPH should be evaluated for operability. Given the complexity in assessing operability, it is crucial that all patients are referred to an experienced surgical centre for evaluation by an experienced CTEPH team.3 If the patient is considered inoperable, a second opinion by a more experienced CTEPH team is recommended. (Figure 1).


Once CTEPH is diagnosed, all patients should receive life-long anticoagulation therapy unless contra-indicated.3

Vitamin K

antagonists are usually adjusted to a target international normalised ratio (INR) between 2.0 and 3.0.4

CTEPH treatment Riociguat

34 Riociguat is the only approved therapy for PEA

Second opinion Medical treatment

Persistent/ recurrent PH

Referral for lung transplantation

Figure 1: Treatment options in CTEPH

the treatment of inoperable and persistent/ recurrent CTEPH (level of evidence IB in 2015 Guidelines). It belongs to a class of vasodilating drugs directly stimulating soluble guanylate cyclase (sGC).5,6

sGC is a

key signal-transduction enzyme activated by nitric oxide and it catalyses the conversion of guanosine-5’-triphosphate (GTP) into the second messenger cGMP.

Riociguat has a dual mode of action: it increases the sensitivity of sGC to endogenous bioavailable nitric oxide (NO) by stabilising the NO-sGC binding and stimulates sGC directly and independently of

NO. This is important because PH patients have a decreased level of NO, and with the progression of the disease, the NO levels decrease further. Oral administration of riociguat results in an increase of cGMP with antifibrotic (vascular remodelling) and vasodilating effects.

CHEST-1 (Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase–Stimulator Trial-1), including 261 patients of whom 27% had post-operative residual or persistent PH after PEA.7

Patients were randomised to receive either placebo or riociguat and

BPA Non-operable

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