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ESC recommends DNA analysis in post mortems of young sudden death victims

London, UK – 29 Aug 2015: ESC Guidelines published today recommend DNA analysis as a fundamental component of post mortem assessment in young sudden death victims. Identification of a genetic cause helps to quickly diagnose and protect relatives.

Sudden Cardiac Death and Resuscitation
ICD and CRT
Acute Cardiac Care

 

EMBARGO : 29 August 2015 at 08:00 BST

The Guidelines are published online in European Heart Journal1 and on the ESC Website2 and are the European update to the 2006 European/American guidelines.3 They focus on preventing sudden cardiac death in patients with ventricular arrhythmias.

“For the first time these guidelines have incorporated the concept proposed by several consensus documents that DNA analysis should be a fundamental component of post mortem assessment in young sudden death victims,” said Professor Silvia Priori, Chairperson of the guidelines Task Force. “The molecular analysis helps to identify the presence of genetic diseases that can occur in a structurally normal heart and therefore cannot be identified during autopsy. Identification of a genetic cause as a substrate for a sudden death facilitates the early diagnosis of affected relatives and may help protect them through a personalised approach that spans from lifestyle modifications to the early use of therapies.”

Another innovation is the introduction of "emerging recommendations" which are not yet supported by evidence strong enough for a Class I recommendation but which are highly promising. One example is the Class IIa recommendation, level of evidence C, that supports the use of the old antiarrhythmic drug flecainide in addition to beta-blockers for patients with a diagnosis of catecholaminergic polymorphic ventricular tachycardia who experience recurrent syncope or polymorphic/bidirectional VT while on beta-blockers and have risks/contraindications for an implantable cardioverter defibrillator (ICD).

“This new indication for an old drug is particularly relevant, as it provides an additional therapeutic option for patients with an ‘orphan’ disease with a limited number of available treatments,” said Professor Priori.

With respect to new technologies, two Class IIb recommendations are introduced that cautiously inform the medical community about new treatment options that are still under investigation but could be used in select patients when alternatives are ineffective, unavailable or contraindicated. First, wearable cardioverter defibrillators may be considered for adult patients with poor left ventricular systolic function who are at risk of sudden arrhythmic death for a limited period and are not candidates for an ICD.4 Second, the subcutaneous ICD may be a useful alternative to a transvenous defibrillator when venous access is difficult, after the removal of a transvenous defibrillator due to infections, or in young patients with a long-term need for ICD therapy.

One of the most important recommendations is to identify patients with ischaemic heart disease who are at high risk of sudden cardiac death and may benefit from an ICD. An eight year follow-up of the MADIT II study found that patients with an ejection fraction below 30% and mild to moderate heart failure (NYHA class II and III) had a long-term improvement in survival with an ICD. ESC Guidelines published today recommend re-evaluation of left ventricular function at 6–12 weeks after myocardial infarction to assess the potential need for primary prevention ICD implantation.

“This recommendation is based on the fact that many patients with reduced ejection fraction early after a myocardial infarction will experience an improvement in their condition over time and therefore do not require the implantation of an ICD,” said Professor Carina Blomström-Lundqvist, co-Chairperson. “Reassessment of ejection fraction is critical to ensure that all efforts are made to avoid unnecessary ICDs while at the same time identifying patients whose ejection fractions remain low even after medical therapy and who need an ICD.”

Cardiac resynchronisation requires further study in a number of areas. “Its use in patients with atrial fibrillation has not been defined outside of observational datasets and there is a clear need for adequately powered randomised trials in this patient group,” said Professor Blomström-Lundqvist. “Furthermore, whether the duration or the ‘morphology’ of the QRS complex is the most important predictive factor for response to therapy remains a challenging issue requiring more extensive investigations.”

Professor Priori concluded: “The guidelines emphasise that early diagnosis of disorders that may predispose to sudden cardiac death can save lives. Screening in the general population may be expensive and not all experts support its use. But there is now a broad consensus that cascade screening should always be performed in families of affected individuals and in families with a history of sudden cardiac death in young people.”

ENDS

References

12015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. European Heart Journal. 2015. doi: 10.1093/eurheartj/ehv316
2ESC Guidelines on the ESC Website
3ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Europace. 2006 ;8:746–837. doi:10.1093/europace/eul108
4Examples of such patients are bridge to transplant, peripartum cardiomyopathy, active myocarditis and arrhythmias in the early post-myocardial infarction phase.

Guidelines at ESC Congress
The 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death will be featured in:
•    An ESC Guidelines 2015 overview session on August 30 at 08:30
•    A Meet the Guidelines Task Force session on August 30 at 15:40
•    A dedicated session on August 31 at 08:30

Notes to editor

SOURCES OF FUNDING: None.

DISCLOSURES: The disclosure forms of all experts involved in the development of these guidelines are available on the ESC website www.escardio.org/guidelines.

 

ESC Press Office
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About the European Society of Cardiology
The European Society of Cardiology (ESC) represents more than 90 000 cardiology professionals across Europe and worldwide. Its mission is to reduce the burden of cardiovascular disease in Europe.
 
About ESC Congress 2015
ESC Congress is the world’s largest and most influential cardiovascular event contributing to global awareness of the latest clinical trials and breakthrough discoveries. ESC Congress 2015 takes place 29 August to 2 September at ExCel London in London, UK. Access the scientific programme. More information is available from the ESC Press Office at press@escardio.org.

To access all the scientific resources from the sessions during the congress, visit ESC Congress 365.  

This press release accompanies both a presentation and an ESC press conference at the ESC Congress 2015. Edited by the ESC from material supplied by the investigators themselves, this press release does not necessarily reflect the opinion of the European Society of Cardiology. The content of the press release has been approved by the presenter.


 

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