Congenital heart defects in La Réunion Island: a 6-year survey within a EUROCAT-affiliated congenital anomalies registry.

OBJECTIVES: This study compares the prevalence and perinatal mortality of congenital heart defects on La Réunion with European (EUROCAT) standards. METHODS AND RESULTS: Data were extracted from a EUROCAT-affiliated congenital malformations registry, covering 88,025 births during the period 2002-2007, on the whole island territory. A total of 512 congenital heart defects were registered, including 424 live births, 18 foetal deaths from 16 weeks of gestation, and 70 terminations of pregnancy. The total prevalence of congenital heart defects was 5.8 per 1000 births and live birth prevalence was 4.8 per 1000. The total prevalence of non-chromosomal congenital heart defects was 5.1 per 1000 births, of which 3% were perinatal deaths, 33.3% prenatally diagnosed, and 11.6% termination of pregnancy. Severe non-chromosomal congenital heart defects - excluding ventricular septal defects, atrial septal defects, and pulmonary valve stenosis - occurred in 2.1 per 1000 births, of which 10.3% were perinatal deaths, 59.1% prenatally diagnosed, and 24.3% termination of pregnancy. Of the severe congenital heart defects, the rates of single ventricle (0.20‰), Ebstein anomaly (0.11‰), common arterial trunk (0.25‰), and atrioventricular septal defect (0.62‰) exceeded averages found in Europe, although coarctation of the aorta was infrequent. Conversely, rates of ventricular septal defects, atrial septal defects, and pulmonary valve stenosis were inferior to European standards. Slightly less than half of the congenital heart defects of chromosomal origin were associated with Down syndrome. CONCLUSION: In La Réunion, the total prevalence of congenital heart defects is far inferior to that found in Europe. The difference can be attributable to lower prevalences of mild congenital heart defects.

[Early maternal-fetal transmission of the Chikungunya virus]. / Transmission materno-foetale précoce du virus Chikungunya.

INTRODUCTION: Since the onset of the Chikungunya outbreak in Reunion Island, vertical maternal-fetal transmission of the virus has been observed in newborns, but no such transmission has been demonstrated early during pregnancy. We report here the first three cases of maternal-fetal transmission of the Chikungunya virus (CHIKV) before 16 weeks' gestational age. CASES: Maternal infections occurred at terms of 12 weeks and 4 days, 15 weeks and 5 days, and 15 weeks and were confirmed by positive findings for specific anti-CHIKV IgM. Fetal deaths were subsequently observed, and at that point, CHIKV RT-PCR was negative for all three maternal blood samples. Amniocentesis preceded rupture of membranes in all three cases. RT-PCR showed viral genome in the amniotic fluid of the three fetuses, in the placentas of two, and in the brains of two. Autopsy found no malformations, and all other bacterial and viral test results were negative. DISCUSSION: These findings demonstrate early maternal-fetal transmission of CHIKV, which is suspected to be directly linked to the fetal deaths. This vertical transmission, probably abortifacient, should be considered in the light of human and animal responses to other arboviruses.

Case report: a prenatal case of Jarcho-Levin syndrome diagnosed during the first trimester of pregnancy.

The Jarcho-Levin syndrome is a specific form of spondylocostal/spondylothoracic dysostosis. There have been various classifications of this syndrome. We present the case of a severe prenatal Jarcho-Levin syndrome, diagnosed by ultrasound examination during the first trimester of pregnancy in a family with no previous medical history of an affected child. X-ray exploration, high-resolution spiral computed tomography and autopsy confirmed the diagnosis.