
M. LOPEZ-MESEGUER ET AL. 23
of the Pulmonary Hypertension Classification was made
for two reasons. First, CTEPH shares certain pathophysi-
ologic characteristics with PAH in terms of pulmonary
vasculature remodeling, which is known to occur even in
patients who show macroscopic resolution of the clot
[17]. Furthermore, some patients with CTEPH in whom
endarterectomy is not indicated respond to specific PAH
therapy, an alternative treatment for CTEPH that has
been tested in clinical trials [18,19]. Lastly, according to
our data, survival in CTEPH is similar to that in PAH.
The main limitations of this study are its retrospective
nature, a relatively small sample size, and the fact that it
was carried out in a single center. Furthermore, we can-
not know whether other variables related to treatment
response such as hemodynamic parameters or biomarkers
are also relevant to the prognosis. Validation of this
model of risk predictors in a larger sample would be ad-
visable. Future prospective studies particularly multicen-
ter studies, in which substantial patient groups can be
assembled, will be important to determine the value of
these results.
In conclusion, the prop ortional risks model u sed in this
study demonstrated the high degree of uncertainly re-
garding the prognosis of PAH and CTEPH at the start of
medical treatment, and showed that the data related to
treatment response are of value in estimating the progno-
sis of the disease.
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