As discussed, an increase in mortality rate in paediatric CHD
patients with post-operative PAH compared with paediatric Eisenmenger
Syndrome patients has already been reported.14 However,
in this series, PAH was likely to be a relatively early complication
after cardiac defect correction (average age of children was 6.9
years) compared with our patient population in which PAH was
detected very late after correction (Table 1). In the Euro Heart
Survey on adult CHD, a better survival of corrected and uncorrected
CHD patients with PAH compared with Eisenmenger Syndrome
patients was reported.21 However, in this study, the
definition of PAH was questionable (systolic pulmonary arterial
pressure ≥40 mmHg or qualitatively defined as ‘abnormal’)