Highlights
- •Underdiagnosis of CTEPH is an important issue.
- •Diagnostic delay of CTEPH is currently longer than a year.
- •CTEPH is potentially curable by surgery, namely a pulmonary endarterectomy.
- •BPA and oral riociguat are alternative treatments in inoperable CTEPH patients.
Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is an uncommon and late complication
of pulmonary embolism resulting from misguided remodelling of residual pulmonary thromboembolic
material and small-vessel arteriopathy. CTEPH is the only form of pulmonary hypertension
(PH) potentially curable by pulmonary endarterectomy (PEA). Unfortunately, several
patients have either an unacceptable risk-benefit ratio for undergoing the surgical
intervention or develop persistent PH after PEA. Novel medical and endovascular therapies
can be considered for them. The soluble guanylate cyclase stimulator riociguat is
recommended for the treatment of patients with inoperable disease or with recurrent/persistent
PH after PEA. Other drugs developed for the treatment of other forms of PH, as prostanoids,
phosphodiesterase-5 inhibitors and endothelin receptor antagonists have been used
in the treatment of CTEPH, with limited benefit. Balloon pulmonary angioplasty is
a novel and promising technique and is progressively emerging from the pioneering
phase. Highly specialized training level and complex protocols of postoperative care
are mandatory to consolidate the technical success of the surgical and endovascular
intervention.
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Article info
Publication history
Published online: March 01, 2018
Accepted:
February 28,
2018
Received in revised form:
February 17,
2018
Received:
July 22,
2017
Identification
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