The study demonstrated a significant pulmonary vascular resistance reduction of –24% (95% confidence interval: –32 to –16; p<0.0001). However, 6MWD in metres remained unchanged, with a treatment effect of 2m (95% confidence interval: –22 to 27; p=0.5449). Because of the lack of efficacy in both primary endpoints, bosentan is not approved for CTEPH.
Other treatment options Lung transplantation In select inoperable cases, or after PEA failure, lung transplantation could be considered.
Balloon pulmonary angioplasty In the context of a lack of highly experienced PEA centres, Japanese pulmonary hypertension specialists have developed an alternative technique: balloon pulmonary angioplasty (BPA). This technique could become a potential promising alternative for inoperable CTEPH or for persistent PH after PEA.21,22 This technique was initially proposed in 2001 by Feinstein and colleagues who published a series of 18 patients with non-operable CTEPH who had been subjected to balloon dilatation of the pulmonary arteries.23
Despite a significant
decrease in PAPm, 11 patients developed reperfusion pulmonary oedema, three required mechanical ventilation and one died from reperfusion injury and right ventricular failure after BPA. BPA has subsequently been refined further by Japanese groups by using smaller balloons, by cautiously limiting the number of balloon inflations per session to one or two pulmonary vascular segments, and by the use of intravascular imaging.21,23 The 2015 ESC/ERS guidelines include BPA in the updated treatment algorithm for CTEPH and recommend that interventional BPA may be considered in patients who are technically non-operable or carry an unfavourable risk:benefit ratio for PEA and for patients with persistent symptomatic PH after PEA.22
While BPA is
still not extensively used, it is rapidly gaining attention worldwide. As for PEA, BPA should only be performed in experienced and high-volume CTEPH centres.
Once CTEPH is diagnosed, all patients should receive life-long anticoagulation therapy unless contra-indicated. The standard and potentially curative
treatment for CTEPH is PEA, a surgical procedure in which the pulmonary blood vessels are cleared of obstructive material. Therefore all CTEPH patients should be evaluated for operability by an experienced CTEPH team.
If a patient is considered inoperable by an experienced CTEPH team, a second opinion is recommended. For inoperable patients, or patients with persistent PH after PEA, medical treatment is recommended. Several open-label and randomised controlled trials of three to six months duration have reported different degrees of efficacy with medical treatment. Riociguat is the first, and so far only, drug to demonstrate significant clinical efficacy in patients with inoperable CTEPH or persistent/recurrent CTEPH after pulmonary endarterectomy and has been approved by the US FDA and EMA.
BPA, a technique developed in Japan, could become a potential promising alternative.21
In select cases, lung transplantation could be considered. l
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