A case of Williams syndrome with Wolff-Parkinson-White syndrome.

INTRODUCTION: Williams syndrome (WS) cases have been reported to have with 25-100 times greater increased risk of sudden cardiac death (SCD). SCD has been reported in cases without any evidence of structural cardiovascular anomalies. Wolff-Parkinson-White (WPW) syndrome is characterized by short PR interval and delta wave. Ventricular preexcitations can develop paroxysmal reentrant tachycardia through Kent bundle or less frequent atrial fibrillation and in some cases with accessory pathway effective refractory period (APERP) under 250 ms considered as risky and may lead to SCD. WS associated with WPW has not been reported before. CASE REPORT: An 11-year-old male who had been followed up with WS was referred to pediatric cardiology outpatient clinic with the complaint of palpitation. Electrocardiographic examination showed short PR interval and delta wave in the ECG consistent with WPW. He underwent electrophysiological study (EPS). Basic measurements were performed, and APERP was found at 280 ms cycle atrial pacing. RF energy was delivered using a 4 mm tip nonirrigated radiofrequency (RF) ablation catheter where the best ventriculoatrial (VA) signals were received and the AP was abolished within few seconds. DISCUSSION AND CONCLUSIONS: Although, WPW cases are usually asymptomatic or related to SVT, the risk of SCD should not be ignored. Thus, all patients with WPW deserve an EPS for assessing the AP conduction properties. Due to the increased risk of SCD in patients with WS compared to general population, in the presence of concomitant WPW, these patients should be evaluated with EPS even if they do not have symptoms.

Safety and efficacy of Amplatzer duct occluder II and konar-MF™ VSD occluder in the closure of perimembranous ventricular septal defects in children weighing less than 10†kg.

Introduction: Device closure of perimembranous ventricular septal defects (pmVSD) is a successful off-label treatment alternative. We aim to report and compare the outcomes of pmVSD closure in children weighing less than 10 kg using Amplatzer Duct Occluder II (ADOII) and Konar-MF VSD Occluder (MFO) devices. Methods: Retrospective clinical data review of 52 children with hemodynamically significant pmVSD, and sent for transcatheter closure using ADOII and MFO, between January 2018 and January 2023. Baseline, procedural, and follow-up data were compared according to the implanted device. Results: ADOII devices were implanted in 22 children with a median age of 11 months (IQR, 4.1-14.7) and weight of 7.4 kg (IQR, 2.7-9.7). MFO devices were implanted in 30 children with a median age of 11 months (IQR, 4.8-16.6) and weight of 8 kg (IQR, 4.1-9.6). ADOII were implanted (retrograde, 68.1%) in defects with a median left ventricular diameter of 4.6 mm (IQR, 3.8-5.7) and right ventricular diameter of 3.5 mm (IQR, 3.1-4.9) while MFO were implanted (antegrade, 63.3%) in defects with a median left ventricular diameter of 7 mm (IQR, 5.2-11.3) (p > 0.05) and right ventricular diameter of 5 mm (IQR, 2.0, 3.5-6.2) (p < 0.05). The procedural and fluoroscopy times were shorter with the MFO device (p < 0.05). On a median follow-up of 41.2 months (IQR, 19.7-49.3), valvular insufficiency was not observed. One 13-month-old child (6.3 kg) with ADOII developed a complete atrioventricular heart block (CAVB) six months postoperative and required pacemaker implantation. One 11-month-old child (5.9 kg) with MFO developed a CAVB 3 days postoperative and the device was removed. At 6 months post-procedure, only one child with MFO still experiences a minor residual shunt. There was one arterio-venous fistula that resolved spontaneously. Conclusion: Both the MFO and ADOII are effective closure devices in appropriately selected pmVSDs. CAVB can occur with both devices. The MFO is inherently advantageous for defects larger than 6 mm and subaortic rims smaller than 3 mm. In the literature, our series represents the first study comparing the mid-term outcomes of MFO and ADOII devices in children weighing less than 10 kg.