The approach to the cardiac arrest survivor Silvia G Priori MD PhD Molecular Cardiology, IRCCS Fondazione Salvatore Maugeri Pavia, Italy AND Leon Charney Division of Cardiology, Cardiovascular Genetics Program, Langone Medical Center, New York University School of Medicine, New York, USA
The topic of this presentation will be discussed in light of the recommendations provided by the ESC 2015 Guidelines for the prevention of SCD
Myerburg s Paradox
Myerburg s Paradox In the general population the incidence of Cardiac Arrest is low..however, a high percentage of SCD victims belong to this category!
Cardiac arrest in the structurally intact heart Sudden arrhythmic death in the absence of structural heart disease is an uncommon event. However, data from large series of patients resuscitated from cardiac arrest and necropsy data from victims of sudden death indicate that there is no evidence of structural heart disease in 5% of victims of sudden death. IVF is a diagnosis of exclusion. Therefore, it is of pivotal importance to define the conditions that have to be excluded as well as the most appropriate techniques for excluding them. Circulation. 1997; 95: 265-272
Diagnostic investigations required in a survivor of cardiac arrest If all the mandatory investigations are negative, the diagnosis of Idiopathic Ventricular Fibrillation can be established Survivors of Out-of-Hospital Cardiac Arrest With Apparently Normal Heart Need for Definition and Standardized Clinical Evaluation Consensus Statement of the Joint Steering Committees of the Unexplained Cardiac Arrest Registry of Europe* and of the Idiopathic Ventricular Fibrillation Registry of the United States Circulation. 1997; 95: 265-272
Minimal abnormalities compatible with the diagnosis of IVF Survivors of Out-of-Hospital Cardiac Arrest With Apparently Normal Heart Need for Definition and Standardized Clinical Evaluation Consensus Statement of the Joint Steering Committees of the Unexplained Cardiac Arrest Registry of Europe* and of the Idiopathic Ventricular Fibrillation Registry of the United States Circulation. 1997; 95: 265-272
A strong call for autopsy in SCD victims. This recommendation is established to the benefit of family members of young SCD victims.
Cardiac arrest with a structurally normal heart Survivors of cardiac arrest with a structurally normal heart have a Class I indication for the ICD Ablation of a triggering PVC is indicated with a Class I if performed by experienced operators
Cardiac arrest with a structural heart disease (1) Identification and correction of the reversible causes Optimal Medical Treatment for the underlying disease Reasonable expectation of survival with a good functional status at 1 year
Cardiac arrest with a structural heart disease (2) Secondary prevention of Cardiac Arrest: AVID;CASH;CIDS Connolly SJ, et al Meta-analysis of the implantable cardioverter defibrillator secondary prevention trials. AVID, CASH and CIDS studies. Antiarrhythmics vs Implantable Defibrillator study. Cardiac Arrest Study Hamburg. Canadian Implantable Defibrillator Study. Eur Heart J 2000;21:2071 2078.
Opitimizing management beyond the ICD: Ischemic substrate When acute ischemia precedes VF, there is evidence that coronary revascularization improves outcome. If VA are present, Amiodarone may be used to reduce symptoms BUT sodium channel blockers are contraindicated
CA Survivors with HF: ICD or CRT-D? In the absence of specific data for patients in secondary prevention of CA.the decision on implant of CRT-D should be based on the recommendations for primary prevention.
CA Survivors with HF and sustained VT: How to manage beyond the ICD? In the absence of specific data for HF patients in secondary prevention of CA.management of symptomatic sustained VT should be based on recommendation for HF patients: OPTIMIZATION OF HF THERAPY AMIODARONE/ ABLATION AMIODARONE/ ABLATION
Survivors of Cardiac Arrest with HCM CLASS I RECOMMENDATION FOR ICD IMPLANTATION IN HCM SURVIVORS OF CARDIAC ARREST ALSO EXTENDS TO THE PEDIATRIC POPULATION: SUSTAINED VT AND REPEATED ICD SHOCKS MAY BE MANAGED WITH AMIODARONE, BETA BLOCKERS OR CATHETER ABLATION.
Survivors of CA with ARVC The ability of sotalol, beta blockers and amiodarone to reduce symptomatic ventricular arrhythmias (PVCs, nsvt, Sust VT) is still debated. While consensus exists on their inability to prevent CA. In young patients, amiodarone presents the challenge of adverse events, so it may not be the first choice agent in young patients implanted with ICD and ablation might be preferable.
Cardiomyopathies of miscellaneous origin In cardiomyopathies with miscellaneous origin (Amyloid, Chagas, Restrictive, LV non Compaction..) there is a global recommendation to implant an ICD in patients with life-threatening arrhythmias and in survivors of ICD. This recommendation is not based on evidence, but on the lack of effective alternatives.
Survivors of CA with Long QT Syndrome According to the recommendation, LQTS survivors of a CA should get an ICD on top of beta blockers. Left cardiac sympathetic denervation may be an option for patients who refuse an ICD or when there is a contraindication to the ICD
Cumulative Survival (%) Survival in LQTS according to genotype 100 90 80 70 60 50 40 30 20 10 p=0.007 Genotype LQT1: LQT2: LQT3: LQT1 LQT2 LQT3 0 0 10 20 Age (years) 30 40 Priori SG et al NEJM 2003
Cardiac event free survival Cardiac Events on Beta Blockers in LQTS 1.0 0.9 0.8 0.7 0.6 LQT1 0.5 0.4 0.3 0.2 LQT3 LQT2 0.1 0.0 0 p<0.0001 5 10 Follow up (years) 15 20 Priori et al. JAMA 2004
EXCEPTION TO THE RECOMMENDATIONS?? In selected patients with LQTS surviving a cardiac arrest that occurred when the patient was not taking beta blockers, it may be reasonable to hold on ICD implantation and start beta blockers: Pediatric LQT1 patients are on top of such a list because of the high rate of side effects of the ICD in children and for the good response of LQT1 patients to beta blockers
How to manage a survivor of CA with diagnosis of Brugada Syndrome? How to manage an electrical storm/ repeated ICD shocks?
How to manage a survivor of CA with diagnosis of Catecholaminergic Polymorphic VT (CPVT)?
Event-free survival Why in CPVT do we not recommend waiting to implant an ICD if the cardiac arrest occurred without beta blocker therapy? 100 80 Age 10: 30% of pts with symptoms 60 40 Age 40: 79% of pts with symptoms 20 0 0 10 20 30 40
Event-free survival Why in CPVT do we not recommend waiting to implant an ICD if the cardiac arrest occurred without beta blocker therapy? 100 80 Age 10: 30% of pts with symptoms 60 40 SEVERE PROGNOSIS= Age 40: 79% of pts with symptoms MALIGNANT DISEASE 20 0 0 10 20 30 40
Cardiac event-free survival Why in CPVT do we not recommend waiting to implant an ICD if the cardiac arrest occurred without beta blocker therapy? 100 80 60 40 20 0 0 5 10 Time on therapy (years) N= 110 patients with -blockers Mean follow-up of 4 years 26% with recurrences N= 81 patients with, n= 21 without -blockers Mean follow-up of 8 years 27% with recurrences Priori et al Hayashi et al
Cardiac event-free survival Why in CPVT do we not recommend waiting to implant an ICD if the cardiac arrest occurred without beta blocker therapy? 100 80 60 40 20 0 0 5 10 Time on therapy (years) Poor response To beta blockers! N= 110 patients with -blockers Mean follow-up of 4 years 26% with recurrences N= 81 patients with, n= 21 without -blockers Mean follow-up of 8 years 27% with recurrences Priori et al Hayashi et al
Q&A Idiopathic Ventricular Fibrillation is a diagnosis established: 1. When the autopsy cannot be performed 2. When there is no clinical information on the clinical history of the patient 3. When ECG, Holter, Echocardiogram and Exercise stress test are negative 4. When a large set of tests to rule out the most common causes of SCD is performed and is negative
Q&A Which of the following is true? 1. Children surviving a cardiac arrest with long QT syndrome have a contraindication to the ICD 2. An ICD is indicated for survivors of cardiac arrest only when an ischemic substrate is documented 3. Most survivors of CA are candidates for cardiac resynchronization 4. The strategies to reduce ICD shocks in survivors of CA implanted with an ICD largely depend on the underlying disease
Conclusions (1) SCD occurs in association with a variety of conditions: the substrate underlying the arrhythmic event influences the management of survivors. SCD occurring in the absence of structural heart disease is called IVF: a definite set of investigations is required to exclude the presence of an underlying disease, thus leading to the diagnosis of IVF In victims of unexplained SCD, an autopsy with molecular autopsy is indicated
Conclusions (2) ICD in secondary prevention of CA is indicated in the vast majority of CA survivors, including children. Strategies for reduction of ICD shocks and prevention of electrical storms in CA survivors are tailored to the arrhythmogenic substrate.