Background—: We previously showed a survival benefit of the implantable cardioverter defibrillator (ICD) in males with arrhythmogenic right ventricular cardiomyopathy caused by a p.S358L mutation in TMEM43 . We present long-term data (median follow-up 8.5years) after ICD for primary (PP) and secondary prophylaxis in males and females, determine whether ICD discharges for ventricular tachycardia/ventricular fibrillation were equivalent to an aborted death, and assess relevant clinical predictors. Methods and Results—: We studied 24 multiplex families segregating an autosomal dominant p.S358L mutation in TMEM43 . We compared survival in 148 mutation carriers with an ICD to 148 controls matched for age, sex, disease status, and family. Of 80 male mutation carriers with ICDs (median age at implantation 31 years), 61 (76%) were for PP; of 68 females (median age at implantation 43 years), 66 (97%) were for PP. In males, irrespective of indication, survival was better in the ICD groups compared with control groups (relative risk 9.3 [95% confidence interval 3.3–26] for PP and 9.7 [95% confidence interval 3.2–29.6] for secondary prophylaxis). For PP females, the relative risk was 3.6 (95% confidence interval 1.3–9.5). ICD discharge-free survival for ventricular tachycardia/ventricular fibrillation ≥240 beats per minute was equivalent to the control survival rate. Ectopy (≥1000 premature ventricular complexes/24 hours) was the only independent clinical predictor of ICD discharge in males, and no predictor was identified in females. Conclusions—: ICD therapy is indicated for PP in postpubertal males and in females ≥30 years with the p.S358L TMEM43 mutation. ICD termination of rapid ventricular tachycardia/ventricular fibrillation can reasonably be considered an aborted death.
Ovarian cancer (OC) is Canada’s third most common gynecological cancer, with an estimated 3000 new cases and 1950 deaths projected in 2022. No effective screening has been found to identify OC, especially the most common subtype, high-grade serous carcinoma (HGSC), at an earlier, curable stage. In patients with hereditary predispositions such as BRCA mutations, the rates of HGSC are significantly elevated, leading to the use of risk-reducing salpingo-oophorectomy as the key preventative intervention. Although surgery has been shown to prevent HGSC in high-risk women, the associated premature menopause has adverse long-term sequelae and mortality due to non-cancer causes. The fact that 75% of HGSCs are sporadic means that most women diagnosed with HGSC will not have had the option to avail of either screening or prevention. Recent research suggests that the fimbrial distal fallopian tube is the most likely origin of HGSC. This has led to the development of a prevention plan for the general population: opportunistic salpingectomy, the removal of both fallopian tubes. This article aims to compile and review the studies evaluating the effect of opportunistic salpingectomy on surgical-related complications, ovarian reserve, cost, and OC incidence when performed along with hysterectomy or instead of tubal ligation in the general population.
Accidental death is the leading manner of pediatric death worldwide. In Canada, this phenomenon has been explored in-depth in select provinces however published assessments of accidental pediatric death in the province of Newfoundland & Labrador (NL) remain limited. A retrospective cohort study of cases of accidental death in children and youth aged 0- to 21- years that occurred between January 1, 2004 and December 31, 2013 and referred to the Office of the Chief Medical Examiner (OCME) of NL was carried out. Comparison was made between accidental death in NL and the provinces of New Brunswick (NB) and Manitoba (MB) matching ages and years of occurrence. A total of 139 cases occurred with a mean age of 15.8 (+/-4.9) years. Males accounted for more cases than females (p < 0.001). Leading cause of death was mechanical trauma due to a motor vehicle collision (MVC) (52%). Among all causes of death statistical significance was present (p < 0.001). Within NL regional differences were present with the highest incidence of all accidental death occurring in the Labrador Grenfell Health region (p = 0.002). When comparing NL to MB, rates of accidents were comparable. More differences were seen when comparing to NB regarding specific causes of death, and sexes. Accident incidence differed significantly between NL provincial regions with rural areas manifesting more accidents per capita than urban areas. NL data was similar to MB and lower than NB in overall accidental death, cause specific death and among sexes.