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Improving usual care after sudden death in the young with focus on inherited cardiac diseases (the CAREFUL study): a
Christian van der Werf1, Anneke Hendrix2, Erwin Birnie3
1Department of Clinical and Experimental Cardiology, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands c.vanderwerf@amc.nl.
Insights
Sudden death (SD) in young individuals often stems from inherited cardiac diseases. Current autopsy and cardiogenetic evaluation rates are low, and interventions did not significantly improve this situation.
Area of Science:
- Cardiology
- Genetics
- Public Health
Background:
- Inherited cardiac diseases are a significant cause of sudden death (SD) in young individuals.
- Autopsy and cardiogenetic evaluation of relatives are crucial for identifying at-risk family members.
- Current practices for identifying inherited cardiac diseases after young SD are suboptimal.
Purpose of the Study:
- To investigate the usual care following sudden death (SD) in young individuals for identifying inherited cardiac disease.
- To assess the effectiveness of two interventions aimed at improving autopsy rates and cardiogenetic referrals for young SD victims and their families.
Main Methods:
- A community-based intervention study was conducted in the Netherlands involving young SD victims (ages 1-44).
- Interventions included a 24/7 telephone line and website (Amsterdam) and letters with educational meetings (Utrecht) for general practitioners and coroners.
- Two control regions monitored usual care; autopsy rates and cardiogenetic evaluations were compared across regions.
Main Results:
- Autopsy was performed in 43.3% of 390 SD cases. Cardiogenetic evaluation was indicated in 75.9% of cases, but only 8.4% of families attended a clinic.
- Intervention regions showed autopsy rates of 38.7% (Amsterdam) and 45.5% (Utrecht), compared to 49.0% in control regions.
- Cardiogenetics clinic attendance was 7.3% (Amsterdam), 9.9% (Utrecht), and 8.8% (control), indicating no substantial improvement.
Conclusions:
- The autopsy rate for young sudden death (SD) cases in the Netherlands is low.
- A very small proportion of families with young SD undergo cardiogenetic evaluation to identify inherited cardiac diseases.
- The implemented interventions did not lead to a significant improvement in autopsy rates or cardiogenetic evaluation for families affected by young SD.
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.
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