Venoarterial Extracorporeal Membrane Oxygenation and Implantable Cardioverter-Defibrillator Implantation in a Hemodynamically Unstable Infant with Ventricular Tachycardia from Multiple Cardiac Rhabdomyomas

Tuberous sclerosis complex (TSC) is a neurocutaneous disorder characterized by benign tissue hamartomas in multiple organ systems, including cardiac rhabdomyomas. Though prevalent in TSC, cardiac tumors are rare in children, occurring in about 0.03%–0.17%. Rhabdomyomas are the most common, accounting for 45%. When present, they are multiple and in the ventricular myocardium. Frequently, they regress and surveillance is all that is required until spontaneous regression. Intervention is necessary when life-threatening obstruction or hemodynamically significant refractory arrhythmias occur. This case highlights the course of a 6-month-old infant with TSC and cardiac rhabdomyomas who presented in refractory ventricular tachycardia (VT) with decompensation and cardiac arrest necessitating venoarterial extracorporeal membrane oxygenation (VA-ECMO), complex antiarrhythmic therapy, and ultimately implantable cardioverter-defibrillator (ICD) implantation

Updated international tuberous sclerosis complex diagnostic criteria and surveillance and management recommendations

Background Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disease affecting multiple body systems with wide variability in presentation. In 2013, Pediatric Neurology published articles outlining updated diagnostic criteria and recommendations for surveillance and management of disease manifestations. Advances in knowledge and approvals of new therapies necessitated a revision of those criteria and recommendations. Methods Chairs and working group cochairs from the 2012 International TSC Consensus Group were invited to meet face-to-face over two days at the 2018 World TSC Conference on July 25 and 26 in Dallas, TX, USA. Before the meeting, working group cochairs worked with group members via e-mail and telephone to (1) review TSC literature since the 2013 publication, (2) confirm or amend prior recommendations, and (3) provide new recommendations as required. Results Only two changes were made to clinical diagnostic criteria reported in 2013: “multiple cortical tubers and/or radial migration lines” replaced the more general term “cortical dysplasias,” and sclerotic bone lesions were reinstated as a minor criterion. Genetic diagnostic criteria were reaffirmed, including highlighting recent findings that some individuals with TSC are genetically mosaic for variants in TSC1 or TSC2. Changes to surveillance and management criteria largely reflected increased emphasis on early screening for electroencephalographic abnormalities, enhanced surveillance and management of TSC-associated neuropsychiatric disorders, and new medication approvals. Conclusions Updated TSC diagnostic criteria and surveillance and management recommendations presented here should provide an improved framework for optimal care of those living with TSC and their families

Atrioventricular accessory pathways in 89 dogs: Clinical features and outcome after radiofrequency catheter ablation.

BackgroundAtrioventricular accessory pathways (APs) in dogs have been reported rarely. Data regarding clinical presentation and long-term outcome after radiofrequency catheter ablation (RFCA) are limited.Hypothesis/objectivesTo study clinical features, electrophysiologic characteristics, and outcome of RFCA in dogs with APs.AnimalsEighty-nine dogs presented consecutively for RFCA of APs.MethodsCase series.ResultsLabrador retrievers (47.2% of dogs) and male dogs (67.4% of dogs) were most commonly affected. Labrador retrievers were more likely to be male than non-Labrador breeds (P = .043). Clinical signs were nonspecific and most commonly included lethargy and gastrointestinal signs. Concealed APs were more prevalent in Labrador retrievers than other breeds (P = .001). Right-sided APs (91.7%) predominated over left-sided (8.3%). Tachycardia-induced cardiomyopathy (TICM) occurred in 46.1% of dogs, with complete resolution or substantial improvement noted on one-month postablation echocardiograms. Radiofrequency catheter ablation successfully eliminated AP conduction long term in 98.8% of dogs in which it was performed. Complications occurred in 5/89 dogs. Recurrence in 3 dogs was eliminated long term with a second procedure.Clinical importance/conclusionsAccessory pathways are challenging to recognize in dogs because of nonspecific clinical signs, frequency of concealed APs that show no evidence of their presence during sinus rhythm, and intermittent occurrence of tachyarrhythmias resulting from APs. Tachycardia-induced cardiomyopathy commonly occurs with AP-mediated tachycardias and should be considered in any dog presenting with a dilated cardiomyopathic phenotype because of its good long-term prognosis with rhythm control. Radiofrequency catheter ablation is a highly effective method for eliminating AP conduction and providing long-term resolution