Prevalence, timing of diagnosis and mortality of newborns with congenital heart defects: a population-based study.
OBJECTIVE
To assess the prevalence, timing of diagnosis and infant mortality of congenital heart defects (CHD) with population-based data and using a classification that allows regrouping of the International Paediatric and Congenital Cardiac Code into a manageable number of categories based on anatomic and clinical criteria (ACC-CHD).


METHODS
Population-based cohort study.


METHODS
Greater Paris.


METHODS
All cases (live births, terminations of pregnancy for foetal anomaly (TOPFA), foetal deaths) diagnosed prenatally, or up to 1 year of age in the birth cohorts, May 2005-April 2008, for women in Greater Paris (n=317 538 births). Diagnoses were confirmed in specialised centres and subsequently coded and classified into the categories of ACC-CHD by paediatric cardiologists in the study group.


RESULTS
The total number of CHD was 2867, including 2348 live births (82%), 466 TOPFA (16.2%) and 53 foetal deaths (1.8%). The total prevalence of CHD was 90 per 10 000. After exclusion of ventricular septal defects (VSD), 40% of 'isolated' CHD was diagnosed prenatally with about one half of the remaining diagnosed before 7 days of age. Nevertheless, one in five cases of these major CHD was diagnosed after the fourth week. Infant mortality of 'isolated' CHD-VSD excluded was 8.5% with 40% of deaths occurring after the fourth week of life. These outcomes varied substantially across categories of ACC-CHD.


CONCLUSIONS
Timing of diagnosis, TOPFA, risk and timing of infant mortality were highly variable across the categories of CHD in ACC-CHD, suggesting that it may be a useful measure of severity, and hence, predictor of outcomes of CHD.
Inserm, UMR S953, Recherche épidémiologique sur la santé périnatale et la santé des femmes et des enfants, UPMC, Université Paris-6, Maternité de Port-Royal, Paris, France. babak.khoshnood@inserm.fr
Babak
B
Khoshnood
Nathalie
N
Lelong
Lucile
L
Houyel
Anne-Claire
AC
Thieulin
Jean-Marie
JM
Jouannic
Suzel
S
Magnier
Anne-Lise
AL
Delezoide
Jean-François
JF
Magny
Caroline
C
Rambaud
Damien
D
Bonnet
François
F
Goffinet
D003430:Cross-Sectional Studies; D003937:Diagnosis, Differential; D042241:Early Diagnosis; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000453:epidemiology*; D006801:Humans; D007226:Infant Mortality / Q000639:trends; D007231:Infant, Newborn; D008297:Male; D010297:Paris / Q000453:epidemiology; D011159:Population Surveillance / Q000379:methods*; D015995:Prevalence; D011446:Prospective Studies; D013997:Time Factors