Family-based cardiac screening in relatives of victims of sudden arrhythmic death syndrome.

AIMS: Sudden arrhythmic death syndrome (SADS) occurs when a person suffers a sudden, unexpected death, with no cause found at postmortem examination. We aimed to describe the cardiac screening outcomes in a population of relatives of SADS victims METHODS AND RESULTS: Prospective and retrospective cohort study of consecutive families attending the Family Heart Screening clinic at the Mater Misericordiae Hospital in Dublin, Ireland, from January 2007 to September 2011. Family members of SADS victims underwent a standard screening protocol. Adjunct clinical and postmortem information was sought on the proband. Families who had an existing diagnosis, or where the proband had epilepsy, were excluded. Of 115 families identified, 73 were found to fit inclusion criteria and were retained for analysis, with data available on 262 relatives. Over half of the screened family members were female, and the mean age was 38.6 years (standard deviation 15.6). In 22 of 73 families (30%), and 36 of 262 family members (13.7%), a potentially inheritable cause of SADS was detected. Of the population screened, 32 patients (12.2%) were treated with medication, and 5 (1.9%) have received implantable cardiac defibrillators. Of the five families with long QT syndrome (LQTS) who had a pathogenic gene mutation identified, three carried two such mutations. CONCLUSION: In keeping with international estimates, 30% of families of SADS victims were found to have a potentially inherited cardiac disease. The most common positive finding was LQTS. Advances in postmortem standards and genetic studies may assist in achieving more diagnoses in these families.

The implantable defibrillator and return to operation of vehicles study.

AIMS: We sought to characterise driving habits of contemporary implantable cardioverter defibrillator (ICD) patients. METHODS AND RESULTS: We performed a multicentre prospective observational study of consecutive ICD recipients. Non-commercial drivers with a valid licence were eligible. Patient and ICD data were recorded. All patients completed an anonymous questionnaire regarding their driving habits. Among 275 patients, 25 (9.1%) stopped driving permanently after ICD implantation. During a mean follow-up of 26.5 ± 4.5 months, 25.3% of patients received an ICD shock (52.5% appropriate). The median time to first shock was 7.0 (2.5, 17.5) months and was not significantly different between primary and secondary ICD patients. However, shocks (36.5 vs. 21.3%, P = 0.027) and recurrent shock episodes (17.5 vs. 6.2%, P = 0.011) were more common in secondary ICD patients. Physician-recommended driving restrictions were not recalled by 37.9% and not followed by 23.0% of patients. Overall, the mean duration of driving abstinence was 2.2 ± 2.9 and 3.6 ± 5.3 months for primary and secondary patients, respectively. Notably, 36.5% of secondary patients drove within 1 month. Eight patients (3.3%) received a shock while driving, five of which resulted in road traffic accidents. The annual risk of a shock while driving was 1.5%. CONCLUSIONS: Patient driving behaviour following ICD implantation is variable, with over one-third not remembering and almost one-quarter not adhering to physician-directed driving restrictions. Over one-third of secondary ICD patients drive within 1 month despite physician recommendations. Further studies are required to establish the optimal duration of driving restriction in ICD recipients.

Multiple source surveillance incidence and aetiology of out-of-hospital sudden cardiac death in a rural population in the West of Ireland.

AIMS: There is a paucity of published data on prospectively identified rates of out-of-hospital sudden cardiac death (SCD). We sought to determine the incidence, survival and aetiology of out-of-hospital SCD in the West of Ireland for the year 2005. METHODS AND RESULTS: Data from emergency room resuscitation records were collected throughout the year from all hospitals in the West of Ireland and recorded according to pre-specified criteria. Hospital records of survivors were analysed. Simultaneously, autopsy reports from all pathology laboratories in the region were systematically reviewed and cases of SCD identified. Cardiac arrest associated with non-cardiac pathology was excluded. The population base was 414,277. There were 212 recorded cases of out-of-hospital SCD; 160 (75.5%) were male and the mean age was 63.3 years. The incidence rate was 51.2/100,000/year. The most common aetiology was coronary artery disease (161 cases; 75.9%). The majority of cases occurred in the home (152, 71.7%). Thirteen (6.1%) patients survived to admission of whom eight (3.8%) were alive at discharge. All survivors had ventricular fibrillation as the presenting rhythm. CONCLUSION: The burden of SCD in the West of Ireland is considerable. The vast majority of cases occur in the home. Survival rates in this rural population cohort remain low.