A Pediatric Case of Refractory Torsades de Pointes in Autoimmune Hypothyroidism.

Hypothyroidism can have a significant impact on cardiac contractility, vascular resistance, blood pressure, and cardiac rhythm. Ventricular arrhythmias induced by hypothyroidism are infrequently reported, especially in pediatric cases. A 15-year-old girl with autoimmune hypothyroidism experienced pulseless ventricular arrhythmias on 2 separate occasions because of nonadherence to levothyroxine medication. Subsequent investigations revealed an SCN5A mutation associated with Brugada syndrome. A loop recorder captured polymorphic ventricular tachycardia (PMVT), specifically Torsades de Pointes during her second event. Both arrhythmias were addressed only after stabilizing her thyroid hormone levels with replacement therapy. Although rare, patients with uncontrolled hypothyroidism may present with ventricular arrhythmias, particularly PMVT. The cornerstone of treatment for hypothyroidism-induced ventricular arrhythmia is thyroid replacement therapy. The identification of an SCN5A mutation unmasked by overt hypothyroidism emphasizes the need for a comprehensive cardiac evaluation in patients with hypothyroidism being assessed for PMVT.

Pediatric Tandem Therapeutic Apheresis: A Multidisciplinary Approach.

The epidemiology, safety, and efficacy of pediatric multiple tandem extracorporeal therapies are not well understood. We conducted a retrospective chart review of therapeutic apheresis (TA) from January 1, 2012 to October 31, 2015. We collected procedural/clinical demographics, American Society for Apheresis (ASFA) indication, complications, and mortality. One hundred eighty tandem TA procedures were performed in 53 patients. Median age was 9 years (range: 2 months to 21 years) with a median weight of 28 kg (range: 6-170.3 kg) with nine patients weighing < 10 kg. Forty-five percent of patients were in tandem with continuous veno-venous hemofiltration (CVVH), 21% cardiopulmonary bypass (CPB), 4% extracorporeal membrane oxygenation (ECMO), and 11% had multiple extracorporeal therapies (CVVH and ECMO). Common indications were solid organ transplant (50% cardiac, 13% renal) and sepsis-induced thrombocytopenia-associated multiple organ failure (26%). Equipment (4%) and patient (4%) complications occurred, with rare failure (1%) and no procedure-related mortality. Tandem procedures are used in critically ill pediatric patients with higher morbidity and mortality (21%) than typical TA patients. The high percentage of patients outside of category I or II (83%) underscores the emerging nature of tandem extracorporeal therapies and need for further investigation.

Epidemiology of therapeutic apheresis with a multidisciplinary approach at a high volume pediatric center.

INTRODUCTION: Therapeutic apheresis (TA) is used inconsistently in pediatric populations. We seek to define our multidisciplinary institutional practice. METHODS: We conducted a retrospective chart review of patients receiving TA from January 1, 2012 through October 31, 2015. Data collected included demographics, American Society of Apheresis (ASFA) indication, complications, and mortality. RESULTS: Over 46 months, 1198 TA procedures were conducted on 289 patients ranging in age from 5 months to 21 years with weights ranging from 4.76 to 170.3 kg (16 procedures in patients <10 kg). The procedures were 86% therapeutic plasma exchange, 10% red blood cell exchange, 4% extracorporeal photopheresis, and 5 leukocytapheresis procedures. TA was initiated in different clinical environments: 41% outpatient, 37% intensive care, 15% general inpatient, and 7% operating room. The ASFA category (6th edition) indications for the 1198 procedures included: 44% category I, 25% category II, 23% category III, a single category IV procedure, and the remainder (8%) uncategorized by ASFA. The rate of procedure failure and procedure-related mortality are 1 and 0%, respectively. Case mortality rate was 4%. CONCLUSION: At a large volume pediatric hospital, TA is commonly used and can be performed safely in a variety of settings by a multidisciplinary team. This demographic review catalogs the number and type of procedures performed as a second-line therapy or on the basis of limited evidence. Additional collaborative investigation is needed to evaluate unique implications of TA in pediatrics to maximize efficacy while preserving safety.