Juvenile Systemic Lupus Erythematosus Presenting with Esophagitis and Severe Oral Mucositis.

We present a case of a previously healthy adolescent female who developed severe oral mucositis and acute esophagitis as her presenting symptoms of juvenile systemic lupus erythematosus. Mucositis involving the lips is infrequently reported in systemic lupus erythematosus, and to our knowledge, this is the first reported case of acute, non-infectious esophagitis as a presenting symptom in a pediatric systemic lupus erythematosus patient.

Arrhythmia after cone repair for Ebstein anomaly: The Mayo Clinic experience in 143 young patients.

BACKGROUND: The increased incidence of preoperative and postoperative arrhythmia in Ebstein anomaly (EA) prompted some clinicians to perform an electrophysiology study (EPS) in all patients prior to surgery for EA. The cone repair (CR) is the current surgical option of choice for most young patients with EA but the effect of the CR on arrhythmia is not well established. OBJECTIVES: To assess the burden of arrhythmia in young patients after CR and to assess the utility of selective preoperative EPS. MATERIALS AND METHODS: A retrospective review of all patients <21 years of age with EA who had a CR at Mayo Clinic from June 2007 to December 2015 was performed. Surveys were mailed and telephone calls were made to all individuals to assess antiarrhythmic medication use and EP/device procedures performed after CR. RESULTS: There were 143 patients; median age, 10 years (0.1-20.9 years). Thirty-five (24%) patients had a preoperative EPS of which 26 (18%) had a preoperative ablation. Indications for EPS were Wolff-Parkinson-White (WPW), documented arrhythmia, or suspected arrhythmia. Posthospital discharge data were available for 140 (98%) patients. Mean follow-up was 2.9 years (0.1-9.2 years). At follow-up, 7 (5%) patients were receiving antiarrhythmic medications. After CR, only 3 (2%) patients who did not have a preoperative EPS have required an ablation. CONCLUSIONS: The risk of arrhythmia after CR for EA in young patients is very low when a preoperative EPS is limited to those with WPW, known arrhythmia, or suspected arrhythmia. In smaller patients, it may be reasonable to defer the EPS.

Improving Results of Surgery for Ebstein Anomaly: Where Are We After 235 Cone Repairs?

BACKGROUND: Ebstein anomaly has heterogeneous anatomy and numerous operative techniques are described. Cone repair provides a near anatomic tricuspid valve repair. The purpose of this study was to examine our experience with cone repair. METHODS: Cone repair was performed in 235 consecutive patients with Ebstein anomaly, 134 children (57%) and 101 adults (43%), from June 2007 to October 2015. Median age was 15.6 years (range, 6 months to 73 years). Cone repair was the first operation in 192 patients (82%), the second in 41 (17%), and the third in 2 (1%). Previous tricuspid valve repair had been performed in 27 (12%). Echocardiograms were obtained preoperatively and at hospital dismissal for all patients and for a subgroup of patients at least 6 months after cone repair (n = 81). RESULTS: Leaflet augmentation was done in 67 patients (28%), Sebening stitch in 57 (24.2%), neochordae in 49 (21%), and annuloplasty band in 158 (67%). Bidirectional cavopulmonary shunt was performed in 46 patients (20%). There was 1 early death (0.4%). Early reoperation was required in 14 patients (5.9%); re-repair was possible in 7 (50%). The majority of early reoperations (11 of 14; 79%) occurred in the first third of the series. Mean follow-up was 3.5 ± 2.5 years. There was sustained reduction in tricuspid regurgitation (p < 0.0001), a progressive decline in right ventricle size (p < 0.0001), and late increase in right ventricle fractional area change after initial decline (p < 0.0001). Freedom from late reoperation was 97.9% at 6 years. CONCLUSIONS: Cone repair is safe, and the learning curve is significant. Sustained reduction in tricuspid regurgitation and favorable changes in the right ventricle at follow-up suggest that cone repair has an advantageous impact on right ventricular remodeling.