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CTEPH: medical treatment

CTEPH: medical treatment options and guidelines

The first choice treatment for CTEPH is surgical, but medical treatment might also play 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

62.9% of patients were

considered operable. However, some patients (37%) 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

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

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

(Figure 1) 32 There is currently no evidence for the use

Rationale for using PAH-targeted treatment


PAH-targeted medical therapy might have a role in the treatment of select CTEPH patients (Figure 1). There are several reasons for considering PAH-targeted therapies in

CTEPH diagnosis Continue life-long anticoagulation Operability assessment by CTEPH team Operable Non-operable PEA

Second opinion Medical treatment

Persistent/ recurrent PH

Referral for lung transplant

Figure 1: Treatment options in CTEPH If the patient

of the new direct oral anticoagulants in this indication. Moreover, possible interactions with pulmonary arterial hypertension (PAH)-targeted therapy have not yet been investigated.

CTEPH. In response to increased flow and shear stress through distal vascular segments that are not obstructed by proximal thrombotic material, patients develop a small vessel arteriopathy or ‘distal CTEPH’. Histopathologically, the distal arteries in CTEPH patients present vascular changes similar to those in patients with PAH.5

to PAH, circulating endothelin-1 (ET-1) levels correlate with the haemodynamic and clinical severity of the disease in CTEPH patients.4,6


. Furthermore, similar

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