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

0531: Long-term experience with heart transplantation in children and patients with congenital heart disease

This study assessed the long-term outcome of heart (HTx) and heart-lung transplantation (HLTx) in patients with congenital heart disease (CHD) and children with non-congenital cardiac or pulmonary disease.MethodsRetrospective single-centre analysis of long-term posttransplant outcome, with chart collection of clinical and paraclinical data.ResultsFrom 1984 to 2013, 111 first-HTx, 5 HLTx and 6 re-HTx were performed (62 males), in patients aged 11.7±8.2y: 96(79%) aged <18y. Cardiopathy included 61 cardiomyopathies (50.8%), 50 CHD (41.7%), 6 retransplants (5%). HLTx included 1 Eisenmenger, 1 PPHT, and 2 pulmonary diseases. Patients with cardiomyopathy were younger than CHD (8.7y vs 14.9y).Seventeen (14%) patients had circulatory mechanical support as bridge to transplant. Acute rejection occurred more frequently within the first year post-transplant or >5th year in non-compliant teenagers. Overall 33 patients died (27%), 3.5±4.6y postTx (1 day to 16.4y, med 1.5 months), due to early multivisceral failure in 6 (18%), pulmonary hypertension in 3 (9%), acute rejection in 7 (21%), graft coronary disease in 6 (18%), sepsis in 5 (15%) and miscellaneous in 6. Graft coronary disease occurred in 15(12.4%): 4 had re-HTx, 6 died and 5 are alive. Five lymphoma occurred, 4 months to 14y after HTx, cured in 4 (1died). Patient’s survival was 85% at 1y, 81% at 5y, 70% at 10y and 61% at 20y post-transplant. Graft survival rates were respectively 82%, 68% and 52% at 5y, 10y and 20y post-transplant. Survival did not differ with pretransplant disease, age, gender, pretransplant mechanical support. Mortality was higher in patients with coronary disease (40%) than those free from (25%).Conclusionlong-term prognosis after HTx and HLTx is favourable. Graft coronary disease is the main cause of failure, less frequent than in the adult non-CHD heart-transplanted population