Conversion of complex neonatal Ebstein’s anomaly into functional tricuspid or pulmonary atresia

摘要: Background: Ebstein’s anomaly, due to failure of delamination one or more leaflets the tricuspid valve (TV), is associated with varying degrees regurgitation (TR) and dysplasia right ventricle (RV). Although refinement valvuloplasty plication techniques have opened way a satisfactory outlook for majority older children adults, anomaly presenting at neonatal age, secondary ineffective forward flow into pulmonary systemic circulation, has reported mortality rate as high 75%. In order improve dismal outcome in we strived early univentricular palliation. Patients methods: Univentricular repair was performed five neonates (median age 5 days; range 2‐14 days) ductal dependent blood flow, severe TR, absence across valve, small left ventricular (LV) area right-to-left bowing septum LV loading indexed 10.5 cm 2 :m ). addition, two had moderate (PR), additional stenosis. all patients, combined atrium atrialized RV greater than that functional RV, atrium, 22.0 20.8 , respectively). The median preoperative oxygen tension 35 mmHg pH 7.28. Repair consisted TV closure pericardial patch (with coronary sinus draining RV) (n 3) or, presence PR, resection dysplastic division oversewing main artery 2), well excision atrial septum, redundant wall, construction an aortopulmonary shunt 5). Results: increased from 18.8 result effective ventricle. There no intraoperative late mortality. patients were extubated 7 days postoperatively. At discharge, 46 mmHg. 6, 7, 10, 12 16 weeks bidirectional cavopulmonary anastomosis been constructed. Conclusions: rational palliation consists surgical creation atresia PR stenosis, atresia. These procedures may decompression volume increase output improved clinical outcome. © 1998 Elsevier Science B.V. All rights reserved.