The patients in this group were characterized by an intermediate
age, time between diagnosis and referral to our centre, increase
of PVR among the groups, and by a predominance of post-tricuspid
shunts (72.7%) similar to that in the Eisenmenger Syndrome
patients. The reasons for the initiation and progression of PAH
in these subjects include a delayed correction of the defects especially
if the pulmonary vascular disease had already developed. In
our series, the defects were corrected at a relatively late age
(median age at correction was 11.0 years), and the presence of
PAH was detected after a median of 16.9 years from cardiac
defect correction. The 44 patients of this group underwent
surgery in multiple centres and it is not retrospectively possible
to identify the operability criteria used in each individual centre
at the time the correction was performed. In addition, in the majority
of patients, the pre-operative right heart catheterization was
either not performed (operability based on echocardiographic
parameters) or not available, and the pre-operative values of pulmonary
arterial pressure and PVR were unknown
The patients in this group were characterized by an intermediateage, time between diagnosis and referral to our centre, increaseof PVR among the groups, and by a predominance of post-tricuspidshunts (72.7%) similar to that in the Eisenmenger Syndromepatients. The reasons for the initiation and progression of PAHin these subjects include a delayed correction of the defects especiallyif the pulmonary vascular disease had already developed. Inour series, the defects were corrected at a relatively late age(median age at correction was 11.0 years), and the presence ofPAH was detected after a median of 16.9 years from cardiacdefect correction. The 44 patients of this group underwentsurgery in multiple centres and it is not retrospectively possibleto identify the operability criteria used in each individual centreat the time the correction was performed. In addition, in the majorityof patients, the pre-operative right heart catheterization waseither not performed (operability based on echocardiographicparameters) or not available, and the pre-operative values of pulmonaryarterial pressure and PVR were unknown
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