Abstract
Introduction: Current guidelines describe 4 groups of PH-CHD: A Eisenmengers; B Large defect with left-right shunt; C Small defect; D Previously closed defect. There are sparse data comparing these groups.
Methods: Data for all 198 PH-CHD pts in the ASPIRE (Assessing the Spectrum of Patients Identified at a REferral centre) registry were reviewed.
Results: Although group B had milder haemodynamics and a lower proportion in functional class (FC) III or IV compared with groups C and D (Table) there was no significant difference in survival (Figure). In group A, Age, FC, DLCO and incremental shuttle walking distance (ISWD) predicted survival at univariate, and ISWD at multivariate analysis, while survival in the 23% with trisomy-21 was not different to those without trisomy-21.
Conclusions: Despite differing characteristics, survival of PH-CHD pts referred to specialist centres does not significantly differ between the 4 groups. Further details of medical and interventional therapies will be presented.
- © 2013 ERS