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Introduction
Congenital heart defects (CHD) are the most prevalent group of major congenital anomalies. 1-4 Despite considerable progress in medical and surgical management of newborns with CHD, 5-7 there is considerable mortality and morbidity associated with severe forms of CHD, which comprise the first cause of mortality by congenital anomalies. 1 8-11 Moreover, survivors may have considerable short-term morbidity and long-term adverse neuro-developmental outcomes. 12 13
There is substantial literature on various biological, clinical and epidemiological aspects of CHD. However, by far, most studies are based on data from a few specialised centres, and population-based studies remain relatively rare. 1 5 7 14 15 This is the case for questions related to the prevalence, timing of diagnosis and, particularly, health outcomes of infants with CHD. The paucity of population-based data, in turn, limits the generalisability of available data for evaluation of the mortality, morbidity and long-term health outcomes of newborns with CHD.
CHD represent a wide spectrum of heterogeneous anomalies that show considerable variability in their prevalence, anatomy, developmental origin, severity, modalities of clinical and surgical management, short- and longer-term mortality, morbidity and neuro-developmental outcomes. Given this heterogeneity, coding and classification of CHD becomes a major and challenging question. Currently, the most widely used coding and classification for CHD is the 10th version of the International Classification of Disease (ICD10). However, it is increasingly recognised, particularly by paediatric cardiologists and cardiac surgeons, that ICD10 has important shortcomings for characterising CHD. 16 17 In order to address these shortcomings, a comprehensive coding system, the International Paediatric and Congenital Cardiac Code (IPCCC) has been devised. 16-19 The IPCCC has many advantages but has never been used in a population-based study, most likely due to its complexity requiring highly specialised coding. Moreover, given the number of codes in the IPCCC (the long list of IPCCC includes more than 10 000 individual codes), its use in most clinical and epidemiological studies requires regrouping of individual anomalies. A newly proposed classification, the Anatomic and Clinical Classification of CHD (ACC-CHD) accomplishes this regrouping based on anatomic and clinical criteria. 20 However, the extent to which the categories of ACC-CHD categories may be associated with medical management and outcomes of infants with CHD has not been evaluated.
Our objective was to assess...