Improving usual care after sudden death in the young with focus on inherited cardiac diseases (the CAREFUL study): a community-based intervention study.

AIMS: Inherited cardiac diseases play an important role in sudden death (SD) in the young. Autopsy and cardiogenetic evaluation of relatives of young SD victims identifies relatives at risk. We studied the usual care after SD in the young aimed at identifying inherited cardiac disease, and assessed the efficacy of two interventions to improve this usual care. METHODS AND RESULTS: We conducted a community-based intervention study to increase autopsy rates of young SD victims aged 1-44 years and referral of their relatives to cardiogenetic clinics. In the Amsterdam study region, a 24/7 central telephone number and a website were available to inform general practitioners and coroners. In the Utrecht study region, they were informed by a letter and educational meetings. In two control regions usual care was monitored. Autopsy was performed in 169 of 390 registered SD cases (43.3%). Cardiogenetic evaluation of relatives was indicated in 296 of 390 cases (75.9%), but only 25 of 296 families (8.4%) attended a cardiogenetics clinic. Autopsy rates were 38.7% in the Amsterdam study region, 45.5% in the Utrecht study region, and 49.0% in the control regions. The proportion of families evaluated at cardiogenetics clinics in the Amsterdam study region, the Utrecht study region, and the control regions was 7.3, 9.9, and 8.8%, respectively. CONCLUSIONS: The autopsy rate in young SD cases in the Netherlands is low and few families undergo cardiogenetic evaluation to detect inherited cardiac diseases. Two different interventions did not improve this suboptimal situation substantially.

Regional management of worsening heart failure: rationale and design of the CHAIN-HF registry.

AIMS: Heart failure (HF) is a progressive disease in which periods of clinical stability are interrupted by episodes of clinical deterioration known as worsening heart failure (WHF). Patients who develop WHF are at high risk of subsequent death, rehospitalization, and excessive healthcare costs. As such, WHF could be seen as a separate disease stage and precursor of advanced HF. Whether WHF has a substantial health, societal, and economic impact evidence regarding its multifactorial nature and the specific barriers in treatment, including advanced HF therapies, remains scarce. The CHAIN-HF registry aims to describe the incidence, characteristics, current treatment, and outcomes of WHF. Additionally, it will promote structured regional collaboration and educate on increasing awareness for WHF and describe the implementation of guideline directed medical therapy and utilization of advanced HF therapies in a collaborative network. METHODS AND RESULTS: The CHAIN-HF registry is a prospective, observational, and multicentre study from the collaborating hospitals (Rijnmond HF Network) in the Rotterdam area. Unselected and consecutive patients (irrespective of ejection fraction) with a WHF event will be included. Comprehensive data including demographics, co-morbidities, treatment, and in-hospital and post-discharge outcomes will be collected. Notably, data on socio-economic status, treatment decisions, and referral for advanced HF therapies will be included. CONCLUSIONS: CHAIN-HF will be the first prospective, dedicated WHF registry in a collaborative network of hospitals that will provide robust real-world evidence on the incidence, characteristics, and outcomes of WHF. Moreover, it will provide information on of the value of regional collaboration to improve awareness and outcomes of WHF.

Cardiogenetic screening of first-degree relatives after sudden cardiac death in the young: a population-based approach.

AIMS: To investigate the yield of cardiogenetic screening of relatives of young sudden cardiac death (SCD) and sudden unexplained death (SUD) victims in a population-based setting. METHODS AND RESULTS: A population-based study was carried out between 2000 and 2006. Records of the hospital, death declaration certificates, and resuscitation records were reviewed for SCD and SUD cases (1-40 years). Information on autopsy results and cardiogenetic screening of the victims' first-degree relatives was collected. Relatives were invited for additional cardiogenetic screening when this had not yet been performed. The search led to 16 cases of SCD/SUD and 4 cases of aborted SCD/SUD. Causes of SCD/SUD were myocardial infarction (n = 3), arrhythmogenic right ventricular cardiomyopathy (ARVC) (n = 2), long-QT syndrome (n = 1), hypertrophic cardiomyopathy (n = 2), left ventricular hypertrophy due to aortic stenosis (n = 1), and unknown cause of death (n = 7). Causes of aborted SCD/SUD were myocardial infarction (n = 2), idiopatic ventricular fibrillation (n = 1), and the Brugada syndrome (n = 1). The cardiogenetic screening of 37 relatives of 12 victims led to a diagnosis of Brugada syndrome in 3 relatives and the suspicion of ARVC in 2 relatives. The yield of screening of these relatives was 14% (95% confidence interval: 3-25%). CONCLUSION: In the usual care, relatives of (aborted) SCD and SUD victims are often not referred for cardiogenetic screening. Screening is often not performed according to a systematic approach, and the detection rate of inherited diseases in relatives of (aborted) SCD and SUD victims in a population-based setting, although substantial, is lower than expected based on previous studies.

Sudden death in persons younger than 40 years of age: incidence and causes.

BACKGROUND: The problem of sudden death in the young is currently debated and measures for prevention are being evaluated worldwide. Information on the incidence and causes of sudden (cardiac) death in the young (1-40 years) is essential for the development of these preventive strategies. METHODS: Incidence estimates and causes of death were determined using death certificate data of out-of-hospital sudden deaths recorded by Statistics Netherlands from 1996 to 2006. To define sudden death, International Classification of Diseases codes were selected based on a systematic review of the literature assessing the most common causes of sudden death in the young. RESULTS: The incidence of sudden death was 2.07 (2.06-2.07 per 100 000 person-years). The incidence was higher for men (2.86 per 100 000 person-years) than for women (1.24 per 100 000 person-years) and increased by age. The majority of sudden deaths was of cardiac origin. Sudden cardiac death incidence was 1.62 (1.61-1.62 per 100 000 person-years). In 9% the cause of death remained unexplained. CONCLUSION: The incidence of sudden death in the young is 2.07 per 100 000 person-years. Treatable cardiac causes (such as coronary atherosclerosis and inherited cardiac diseases) are often underlying for the sudden death. This information is helpful in the development of preventive strategies.

Sudden death victims <45 years: Agreement between cause of death established by the forensic physician and autopsy results.

PURPOSE: The goal of this study was to ascertain accordance between cause of death established by the forensic physician and autopsy results in young sudden death victims in the Netherlands. METHODS: Sudden death victims aged 1-45 years examined by forensic physicians operating in the participating regions which also underwent an autopsy between January 2006 and December 2011 were included (n = 70). Cause of death established by the forensic physician based on the external medicolegal examination was compared with autopsy findings using the ICD10-classification. RESULTS: Autopsy findings revealed that the majority of sudden death victims have died from a cardiac disease (n = 51, 73%). Most of the presumed heart disease related cases were confirmed by autopsy (n = 13, 87%). On the contrary, a large number of deaths caused by circulatory diseases were not recognised by the forensic physician (n = 38, 75%). In most of these cases, the forensic physician was forced to report an undetermined cause due to the lack of a solid explanation for death. Cause of death reported by the forensic physician appeared to be in agreement with the autopsy results in 12 cases (17%). CONCLUSIONS: Cause of death determination in young sudden death victims is a difficult task for forensic physicians due to the limited tools available during the medicolegal examination. An effort should be made to standardize extensive post-mortem investigation after sudden death in the young. Autopsy can provide valuable information regarding the cause of death, which is of great importance in view of the identification of inheritable diseases among decedents and their families.