0346: Neonatal arterial switch operation: the sooner the better!

ObjectiveTo evaluate the impact of preoperative management on in-hospital post-operative outcomes after an arterial switch operation (ASO) in newborns with transposition of the great arteries (TGA)MethodsIn this retrospective monocentric study, we included all newborns with TGA±ventricular septal defect who underwent an ASO between 2008 and 2014. Collected data included demographics, clinical and anatomic characteristics, and preoperative management (Rashkind, prostaglandin infusion, mechanical ventilation, age at surgery). Univariate and multivariate analyses were performed to study the impact of preoperative characteristics on time to extubation, a surrogate marker of postoperative morbidity.ResultsAmong the 59 included newborns, 47 (79%) had an antenatal diagnosis, 31 (52%) received prostaglandins (median duration: 96 h; range 0-192h), and 38 (65%) underwent a Rashkind procedure. Mean age at surgery was 5±2 days. The mortality rate was 5% (n=3). Younger age at surgery significantly increased the probability to be extubated within 2 days after surgery, both in univariate analysis (Odd ratio 0.64; confidence interval 0.44-0.92) (p=0.01) and after adjustment for preoperative characteristics and management (OR 0.61; CI 0.39-0.95) (p=0.03). The presence of coronary anomalies tended to increase time to extubation, whereas Rashkind procedure and prostaglandin treatment had no impact.ConclusionOur study shows that younger age at surgery is associated with lower postoperative morbidity in newborns undergoing an ASO. These findings suggest that early surgery is preferable to delay with palliative management such as Rashkind and/or Prostaglandins

Health Outcome of 215 Mothers of Children with Autoimmune Congenital Heart Block: Analysis of the French Neonatal Lupus Syndrome Registry.

International audienceObjective Transplacental passage of maternal anti-SSA and anti-SSB antibodies, potentially associated with maternal autoimmune diseases, can cause neonatal lupus syndrome. Given the paucity of data in this setting, we report short- and long-term outcomes of mothers of offspring with congenital heart block (CHB).Methods This retrospective study included anti-SSA/SSB antibody–positive mothers of fetuses with high-degree CHB and focused on their health status before pregnancy, at CHB diagnosis, and thereafter.Results We analyzed 215 women with at least 1 pregnancy with CHB. Prior to this diagnosis, only 52 (24%) mothers had been diagnosed with an autoimmune disease, mainly systemic lupus erythematosus (SLE; n = 26, 12%) and Sjögren syndrome (SS; n = 16, 7%). Six more were diagnosed with an autoimmune disease during the index pregnancy. Of the 157 mothers (73%) with no such diagnosis at childbirth, 77 (49%) developed one after a median follow-up of 11 years (range: 21 days to 54 years). By the end of follow-up, 135 women (63%) had an autoimmune disease diagnosis, mainly SLE (n = 54, 25%) and SS (n = 72, 33%). Three patients with SLE had renal involvement, and only 6 (3%) had required an immunosuppressive drug at any point. The symptoms best predicting autoimmune disease development were arthralgia and myalgia (P < 0.001), dry syndrome (P = 0.01), and parotid swelling (P = 0.05).Conclusion One-quarter of the patients had an autoimmune disease diagnosis at the time of the fetal CHB diagnosis. Nearly half of those without an initial diagnosis progressed during follow-up, most without severe manifestations. Severe diseases such as lupus nephritis were rarely seen, and immunosuppressive drugs were rarely required

Familial Recurrence Patterns in Congenitally Corrected Transposition of the Great Arteries: An International Study

International audienc