0534: Antenatal echocardiographic parameters to predict postnatal outcome of neonates with Ebstein anomaly

Ebstein tricuspide valve anomaly is a rare CHD with uncertain postnatal prognosis. Criteria to predict outcome are still a matter of debate. The aim of this study was to determine antenatal echocardiographic predictive parameters.MethodsRetrospective multicentric analysis of fetus with diagnosis of Ebstein anomaly. Echocardiographic measurements of ventricles, atria, great vessels and tricuspid regurgitation were collected. Comparisons were made between group I (poor outcome= death occurred in utero or within the first 3 months of life) and group II (favourable outcome: postnatal survival >3 months).Results16 fetuses were included in the study: 10 in group I (62.5%: 2 TOP, 2 fetal deaths, 6 postnatal deaths) and 6 in group II (37.5%). Mean gestationnal age at diagnosis was 29weeks (22 to 38). The mean number of echocardiographic records per patient was 2 (1 to 6). LV to RV ratio, tricuspid valve regurgitation grade and retrograde or anterograde ductal flow did not differ between the 2 groups. Significative differences were found between groups I and II regarding the presence of pulmonary flow (none or mild RV to PA flow: 8 of 9 cases died= 89%), AO to PA ratio (75% death if > 97°p vs 25% if 3-97°p), RA diameter (77.3% death if > 97°p vs 0%), PA diameter (100% death if < 3°p) and pericardial effusion (80% death vs 0%). Only 1 case had arrhythmia and died.ConclusionThis small sample size study showed that the absence of RV to PA flow and/ or pulmonary valve opening, increased AO to PA ratio, RA and decreased PA diameter and the presence of pericardial effusion might represent prognosis factors in fetus with Ebstein anomaly. These results should be confirmed by large scale prospective study

Marfan Sartan: a randomized, double-blind, placebo-controlled trial

International audienceAims - To evaluate the benefit of adding Losartan to baseline therapy in patients with Marfan syndrome (MFS). Methods and results - A double-blind, randomized, multi-centre, placebo-controlled, add on trial comparing Losartan (50 mg when 10 years old, and receiving standard therapy. 303 patients, mean age 29.9 years old, were randomized. The two groups were similar at baseline, 86% receiving β-blocker therapy. The median follow-up was 3.5 years. The evolution of aortic diameter at the level of the sinuses of Valsalva was not modified by the adjunction of Losartan, with a mean increase in aortic diameter at the level of the sinuses of Valsalva of 0.44 mm/year (s.e. = 0.07) (-0.043 z/year, s.e. = 0.04) in patients receiving Losartan and 0.51 mm/year (s.e. = 0.06) (-0.01 z/year, s.e. = 0.03) in those receiving placebo (P = 0.36 for the comparison on slopes in millimeter per year and P = 0.69 for the comparison on slopes on z-scores). Patients receiving Losartan had a slight but significant decrease in systolic and diastolic blood pressure throughout the study (5 mmHg). During the study period, aortic surgery was performed in 28 patients (15 Losartan, 13 placebo), death occurred in 3 patients [0 Losartan, 3 placebo, sudden death (1) suicide (1) oesophagus cancer (1)]. Conclusion - Losartan was able to decrease blood pressure in patients with MFS but not to limit aortic dilatation during a 3-year period in patients >10 years old. β-Blocker therapy alone should therefore remain the standard first line therapy in these patients