Post-Hypoxic Myoclonus Status following Out-of-Hospital Cardiac Arrest-Does It still Predict a Poor Outcome? A Retrospective Study.

In patients with out-of-hospital cardiac arrest (OHCA), the initial prehospital treatment and transfer of patients directly to intervention clinics-bypassing smaller hospitals-have improved outcomes in recent years. Despite the improved treatment strategies, some patients develop myoclonic status following OHCA, and this phenomenon is usually considered an indicator of poor outcome. With this study, we wanted to challenge this perception. The regional prehospital database in Odense in the Region of Southern Denmark was searched for patients with OHCA from the period of 2011-2016. All 900 patients presenting with a diagnosis of OHCA were included in the study. Patients surviving to the hospital and presenting with myoclonic status were followed for up to one year. Only 2 out of 38 patients with myoclonic status and status epilepticus verified by an EEG survived more than one year. Eleven out of 36 patients with myoclonic status but without status epilepticus survived for more than one year. We found no evidence that myoclonic status is an unmistakable sign of poor outcome when not associated with EEG-verified status epilepticus. The conclusion for clinicians involved in post-resuscitation care is that myoclonic status is uncomfortable to witness but does not necessarily indicate that further treatment is futile.

The Danish in-hospital cardiac arrest registry (DANARREST).

Aim of database: The aim of DANARREST is to collect data on processes of care and outcomes for patients with in-hospital cardiac arrest in Denmark, and thereby facilitate and monitor quality and quality improvement initiatives. Study population: In-hospital cardiac arrest patients with a clinical indication for cardiopulmonary resuscitation in Denmark. Main variables: DANARREST includes a number of descriptive variables as well as seven quality of care indicators; four related to processes of care and three related to clinical outcomes. The four process measures are related to whether the cardiac arrest was witnessed, whether the cardiac arrest was ECG-monitored, the timing of cardiopulmonary resuscitation, and the timing of the first rhythm analysis. The three outcomes measures include return of spontaneous circulation, 30-day survival, and 1-year survival. Database status: DANARREST started in 2013, and the coverage has increased steadily since. As of 2017, 95% of relevant hospitals are reporting data with an estimated coverage rate of approximately 80%. Conclusion: DANARREST is a relatively new national registry of in-hospital cardiac arrests in Denmark, with a high coverage rate. The registry provides an opportunity to monitor and improve quality of care for patients with in-hospital cardiac arrest.

Mutations in Danish patients with long QT syndrome and the identification of a large founder family with p.F29L in KCNH2.

BACKGROUND: Long QT syndrome (LQTS) is a cardiac ion channelopathy which presents clinically with palpitations, syncope or sudden death. More than 700 LQTS-causing mutations have been identified in 13 genes, all of which encode proteins involved in the execution of the cardiac action potential. The most frequently affected genes, covering > 90% of cases, are KCNQ1, KCNH2 and SCN5A. METHODS: We describe 64 different mutations in 70 unrelated Danish families using a routine five-gene screen, comprising KCNQ1, KCNH2 and SCN5A as well as KCNE1 and KCNE2. RESULTS: Twenty-two mutations were found in KCNQ1, 28 in KCNH2, 9 in SCN5A, 3 in KCNE1 and 2 in KCNE2. Twenty-six of these have only been described in the Danish population and 18 are novel. One double heterozygote (1.4% of families) was found. A founder mutation, p.F29L in KCNH2, was identified in 5 "unrelated" families. Disease association, in 31.2% of cases, was based on the type of mutation identified (nonsense, insertion/deletion, frameshift or splice-site). Functional data was available for 22.7% of the missense mutations. None of the mutations were found in 364 Danish alleles and only three, all functionally characterised, were recorded in the Exome Variation Server, albeit at a frequency of < 1:1000. CONCLUSION: The genetic etiology of LQTS in Denmark is similar to that found in other populations. A large founder family with p.F29L in KCNH2 was identified. In 48.4% of the mutations disease causation was based on mutation type or functional analysis.