Abstract 15809: Surgical Correction of a Simple Heart Anomaly Improves Survival After Hospital Discharge in Infants With Full Trisomy 18

IntroductionCardiac surgery has started to be recognized as an effective part of a comprehensive care strategy in infants with full trisomy 18 (FT18), but there are still difficulties in determining whether to perform open-heart surgery for anatomical correction.HypothesisThe hypothesis of this retrospective study was that an active surgical approach to correct a simple heart anomaly can influence outcome in infants with FT18.MethodsAfter providing individual counseling to parents and using a program-level decision-making process, 21 consecutive infants with FT18 (5 males) underwent open-heart anatomical correction between November 2009 and May 2018.ResultsDiagnosis was confirmed at birth (birth weightmedian 1.6 kg, range 0.6-2.5 kg) for 20 cases of ventricular septal defect (VSD) and 1 of Tetralogy of Fallot (TOF). Eighteen palliative surgeries (16 pulmonary artery banding (PAB) + PDA ligation, 1 PAB only, 1 PDA ligation only) were performed prior to anatomical correction (stage interval; mean 12.6±8.4 months). Ten patients underwent tracheostomy for trachea- or broncho-malacia and 9 had severe lung atelectasis combined with emphysema. All 21 patients underwent definitive surgeries (17 VSD closure + PA reconstruction, 2 VSD closure + RV muscle resection, 1 VSD closure only, and 1 TOF repair) at a mean age of 16.0±10.7 months and a mean body weight of 5.6±2.0 kg. Three cases required perioperative pulmonary vasodilator therapy and ECMO was used in 2 cases for severe hypoxia (successfully weaned off). There were 2 in-hospital deaths due to severe sepsis. Nineteen patients were discharged from hospital. Hepatoblastoma occurred in five cases, but all were in complete remission after standard care. One patient died due to severe pneumonia and sepsis 37.6 months after definitive repair. Consequently, the cumulative Kaplan-Meier median survival after hospital discharge was 93.8 months (95% CI80.6 to 106.9 months), with a median follow up period of 28.6 months (range0.9-100.9 months).ConclusionsAnatomical correction by open-heart surgery achieved acceptable survival after hospital discharge due to management and treatment of specific perioperative events in infants with FT18.