There are few reports of cone reconstruction for neonatal Epstein's anomaly. We report 14-day-old girl with symptomatic Ebstein's anomaly with prenatal diagnosis prenatally and who was born by caesarean section. Continuous infusion of prostaglandin was started immediately and she was transferred to our institution. The cardiothoracic ratio (CTR) was 74% on chest roentgenograph. The echocardiograph showed severe tricuspid valve regurgitation (TR) and functional pulmonary atresia. Despite aggressive medical therapy, congestive heart failure had advanced and early surgical intervention was required. The anterior leaflet was severely plastered to free the wall, like the Carpentier type C. After cone reconstruction, the intraoperative echocardiography showed mild to moderate TR, antegrade flow to the pulmonary arteries and good function of both ventricles. The patient was extubated on the 6th postoperative day and stayed in the intensive care unit for another 2 days. The CTR was 58%, and the echocardiography showed a well-functioning RV with mild to moderate TR and a right to left transatrial shunt flow with an arterial saturation level of over 90%. Cone reconstruction might even be effective in such border line cases of biventricular repair with dysplastic anterior leaflet of tricuspid valve.

Background/Aims: The Lyon Consensus defined parameters based on upper endoscopy and 24-hour combined multichannel intraluminal impedancepH (MII-pH), that conclusively establish the presence of gastroesophageal reflux disease (GERD). However, the true role of upper endoscopy and MII-pH to evaluate patients with extraesophageal symptoms (EES) has not been well established. Hypopharyngeal MII (HMII), which directly measures laryngopharyngeal reflux (LPR) events, has been utilized to evaluate patients with EES suggestive of LPR. Methods: This was a retrospective study involving patients with EES for > 12 weeks despite proton pump inhibitor therapy, and had no endoscopic confirmatory evidence for GERD and negative MII-pH. All patients were subsequently referred for further evaluation of EES with “unknown” etiology and underwent laryngoscopy and HMII. Based on HMII, abnormal proximal exposure (APE) was defined as LPR ≥ 1/day and/or full column reflux (reflux 2 cm distal to the upper esophageal sphincter) > 4/day. Patients with APE were offered antireflux surgery (ARS) and the outcome of ARS was objectively assessed using Reflux Symptom Index. Results: Of 21 patients with EES which was thought to be GERD-unrelated based on endoscopy and MII-pH, 17 patients (81%) had APE. Eight patients with APE who had undergone ARS had significant symptomatic improvement in the Reflux Symptom Index score (19.6 ± 4.9 pre-ARS to 5.8 ± 1.4 post-ARS, P = 0.008). Conclusions A conventional diagnostic approach using endoscopy and MII-pH may not be sufficient to evaluate patients with EES suggestive of LPR. HMII is essential to evaluate patients with EES, and APE could be a reliable indicator for successful treatment outcomes.