Abstract
Due to its diverse population composition and the paucity of medical resources, the Antilles-Guyane M3 C pediatric cardiology center based in Martinique, provides regular medical support to French Guiana (other overseas territory) using a collaborative dedicated network. To date, prevalence, efficacy of prenatal screening, as well as outcomes of complex congenital heart disease (cCHD), remain totally unknown in this part of the world. A prospective population-based study of all cCHD cases identified from January 2012 to December 2016 including live births, terminations of pregnancy for fetal anomalies (TOPFA), fetal deaths identified either prenatally or up to 1 year of age in the birth cohorts. We identified a total of 71 (47 fetal, 24 post natal) cCHD cases over 33796 births (32,975 live births). The total prevalence of cCHD was 21 for 10,000 births while live birth prevalence was 16.98 per 10000. Non-chromosomal isolated cCHD (39 fetal, 17 post natal, TOPFA in 38.4%) occurred in 12.43 per 10,000 live births. Fetal studies allowed an overall detection rate of 66% of all cCHD and 100% of univentricular hearts. The prevalence (7.98 per 10,000) of functionally univentricular hearts (27 cases including 19 hypoplastic left heart syndrome: TOPFA rate 51.8%) exceeded the expected average observed in other European or American populations. Finally, the overall one- year mortality of live births infants with cCHD was found significant (51.5%), mostly related to compassionate care policy, geographical location and other major considerations (anatomical findings, prematurity, birth weight, non-cardiac problems, social issues). Despite very low medical resources, the cCHD detection program in French Guiana appears effective. Complex congenital heart diseases, especially univentricular hearts, are common and deserve strict medical attention. High prevalence might suggest for additional unknown risk factors.
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