Christopher M. Janson

Predictors of marriage and divorce in adult survivors of childhood cancers: a report from the Childhood Cancer Survivor Study.

Background/Objective: Adult survivors of childhood cancer can have altered social functioning. We sought to identify factors that predict marriage and divorce outcomes in this growing population. Methods: This was a retrospective cohort study of 8,928 ≥5-year adult survivors of childhood malignancy and 2,879 random sibling controls participating in the Childhood Cancer Survivor Study. Marital status, current health, psychological status, and neurocognitive functioning were determined from surveys and validated instruments. Results: Survivors were more likely to be never-married than siblings [relative risk (RR), 1.21; 95% confidence interval (95% CI), 1.15-1.26] and the U.S. population (RR, 1.25; 95% CI, 1.21-1.29), after adjusting for age, gender, and race. Patients with central nervous system tumors were at greatest risk of not marrying (RR, 1.50; 95% CI, 1.41-1.59). Married survivors divorced at frequencies similar to controls. In multivariable regression analysis, nonmarriage was most associated with cranial radiation (RR, 1.15; 95% CI, 1.02-1.31 for >2,400 centigray). In analysis of neurobehavioral functioning, nonmarriage was associated with worse task efficiency (RR, 1.27; 95% CI, 1.20-1.35), but not with emotional distress, or problems with emotional regulation, memory, or organization. Physical conditions predictive of nonmarriage included short stature (RR, 1.27; 95% CI, 1.20-1.34) and poor physical function (RR, 1.08; 95% CI, 1.00-1.18). Structural equation modeling suggested that cranial radiation influenced marriage status through short stature, cognitive problems, and poor physical function. Conclusions: Childhood cancer survivors married at lower frequencies compared with peers. Patients with central nervous system tumors, cranial radiation, impaired processing efficiency, and short stature were more likely to never marry. Divorce patterns in survivors were similar to peers. (Cancer Epidemiol Biomarkers Prev 2009;18(10):2626–35)

Implantable cardioverter-defibrillator lead failure in children and young adults: a matter of lead diameter or lead design?

OBJECTIVES This study aimed to investigate the impact of lead diameter and design on implantable cardioverter-defibrillator (ICD) lead survival in children and young adults. BACKGROUND Recent reports have called attention to high rates of lead failure in adults with small-diameter ICD leads, but data in the pediatric population is limited. METHODS We reviewed lead performance in consecutive subjects ≤30 years with transvenous right ventricular ICD leads implanted at our center between January 1995 and October 2011. Lead failure was defined as fracture, perforation, or sensing failure necessitating revision. RESULTS A total of 120 ICD leads were implanted in 101 patients at a mean age of 15.5 ± 4.9 years. There were 47 small-diameter (≤8-F) and 73 standard-diameter (>8-F) leads. During a median follow-up of 28.7 months (interquartile range: 14.4 to 59.2 months), there were 25 lead failures (21% prevalence), with an incidence of 5.6%/year (95% confidence interval: 3.4 to 7.8). Sprint Fidelis (SF) (Medtronic, Inc., Minneapolis, Minnesota) leads had lower 3-year (69% vs. 92%, p < 0.01) and 5-year (44% vs. 86%, p < 0.01) survival probabilities than standard-diameter leads. In multivariate Cox regression, SF design conferred the greatest hazard ratio for lead failure (hazard ratio: 4.42, 95% confidence interval: 1.73 to 11.29, p < 0.01). Age and linear growth were not significantly associated with lead failure. CONCLUSIONS In this single-center pediatric study that evaluated lead diameter, lead design, and patient factors, the SF design conferred the highest risk of lead failure, suggesting that design rather than diameter is the critical issue in ICD lead performance.

Combined inhibition of Na+ and Ca2+ channels: A novel paradigm for the treatment of incessant ventricular arrhythmias in Andersen-Tawil syndrome

Introduction Andersen-Tawil syndrome type 1 (ATS1), also referred to as long QT syndrome type 7, is a disorder of ventricular repolarization caused by mutations in the KCNJ2 gene. The cardiac phenotype is characterized by bidirectional ventricular tachycardia (VT), polymorphic VT, and multifocal premature ventricular contractions, with or without corrected QT prolongation. In addition, patients manifest facial dysmorphisms and periodic skeletal muscle paralysis. Treatment of ventricular arrhythmia in ATS1 can be difficult, and patients with a high burden of ventricular ectopy can develop tachycardia-induced cardiomyopathy. In this case report, we describe a novel approach to the treatment of refractory ventricular arrhythmia in an adolescent patient with ATS1.

The “hidden” concealed left-sided accessory pathway: An uncommon cause of SVT in young people

Concealed left‐sided accessory pathways (CLAP) are a cause of supraventricular tachycardia (SVT) in the young. Most are mapped with right ventricular (RV) apical/outflow pacing. Rarely, alternative means of mapping are required. We review our experience from three pediatric electrophysiology (EP) centers with a rare form of “hidden” CLAP.

Supraventricular Tachycardia in Adult Congenital Heart Disease: Mechanisms, Diagnosis, and Clinical Aspects

Supraventricular arrhythmias represent a major source of morbidity in adults with congenital heart disease (ACHD). Anatomic variants and post-operative changes contribute to a unique electrophysiologic milieu ripe for the development of supraventricular tachycardia. Intra-atrial reentrant tachycardia is the most prevalent mechanism. Atrioventricular reciprocating tachycardia is common in lesions associated with accessory pathways. Abnormal anatomy complicates the management of atrioventricular nodal reentrant tachycardia. Tachycardia mediated by twin atrioventricular nodes is rare. Focal tachycardias are considerations in the ACHD population. Each of these tachycardia mechanisms is reviewed, focusing on the inherent diagnostic and therapeutic challenges.

A multicenter review of ablation in the aortic cusps in young people

Ablation within the aortic cusp is safe and effective in adults. There are little data on aortic cusp ablation in the pediatric literature. We investigated the safety and efficacy of aortic cusp ablation in young patients.

Compound heterozygous CASQ2 mutations and long‐term course of catecholaminergic polymorphic ventricular tachycardia

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a potentially lethal inherited cardiac disorder characterized by episodic ventricular tachycardia during adrenergic stimulation. It is associated with significant morbidity and mortality. Knowledge of the underlying genetic cause, pathogenesis, and the natural history of the disease remains incomplete. Approximately 50% of CPVT cases are caused by dominant mutations in the cardiac ryanodine receptor (RYR2) gene, <5% of cases are accounted for by recessive mutations in cardiac calsequestrin (CASQ2) or Triadin (TRDN).