2020 APHRS/HRS expert consensus statement on the investigation of decedents with sudden unexplained death and patients with sudden cardiac arrest, and of their families

Abstract

This international multidisciplinary document intends to provide clinicians with evidence-based practical patient-centered recommendations for evaluating patients and decedents with (aborted) sudden cardiac arrest and their families. The document includes a framework for the investigation of the family allowing steps to be taken, should an inherited condition be found, to minimize further events in affected relatives. Integral to the process is counseling of the patients and families, not only because of the emotionally charged subject, but because finding (or not finding) the cause of the arrest may influence management of family members. The formation of multidisciplinary teams is essential to provide a complete service to the patients and their families, and the varied expertise of the writing committee was formulated to reflect this need. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by Class of Recommendation and Level of Evidence. The recommendations were opened for public comment and reviewed by the relevant scientific and clinical document committees of the Asia Pacific Heart Rhythm Society (APHRS) and the Heart Rhythm Society (HRS); the document underwent external review and endorsement by the partner and collaborating societies. While the recommendations are for optimal care, it is recognized that not all resources will be available to all clinicians. Nevertheless, this document articulates the evaluation that the clinician should aspire to provide for patients with sudden cardiac arrest, decedents with sudden unexplained death, and their families.

Keywords: Brugada syndrome; Cardiac arrest; Cardiac genetics; Catecholaminergic polymorphic ventricular tachycardia; Defibrillator; Expert consensus statement; Genetic counseling; Guidelines; Long QT syndrome; Postmortem; Resuscitation; Sudden arrhythmic death syndrome; Sudden cardiac death; Sudden unexplained death; Ventricular arrhythmia.

Figure 1

Distribution of causes of death among autopsied cases of sudden cardiac death (n = 753) according to age in persons aged 1–49 years in Denmark (J.T.-H., unpublished data). SADS = sudden arrhythmic death syndrome. *Coronary artery disease, especially in young persons, may be due to inherited disease (eg, familial hypercholesterolemia).

Figure 2

Cumulative overall survival rates, by participating emergency medical services agency—Cardiac Arrest Registry to Enhance Survival (CARES), United States, October 1, 2005–December 31, 2010. Agencies sorted by total number of out-of-hospital cardiac arrest events in CARES (from low to high; range: 18–5,434).

Figure 3

Cumulative Utstein survival rates (patients alive when arriving to hospital) by participating emergency medical services agency—Cardiac Arrest Registry to Enhance Survival (CARES), United States, October 1, 2005–December 31, 2010. Agencies sorted by total number of out-of-hospital cardiac arrest events in CARES (from low to high). †Utstein survival refers to survival to hospital discharge of persons whose cardiac arrest events were witnessed by a bystander and had an initial rhythm of ventricular fibrillation or pulseless ventricular tachycardia (range: 0–598).

Figure 4

Bystander treatments of patients with out-of-hospital cardiac arrest before emergency medical services arrival among neighborhoods by percentage of black residents. Reprinted with permission from the American Medical Association. AED = automated external defibrillator; CPR = cardiopulmonary resuscitation.

Figure 5

Bystander-witnessed arrest, bystander cardiopulmonary resuscitation (CPR), shockable heart rhythm as first recorded rhythm, and survival on arrival at the hospital, Denmark, 2001–2010. Reprinted with permission from the American Medical Association.

Figure 6

Examples of complimentary bystander cardiopulmonary resuscitation (CPR) programs. Reprinted with permission from Elsevier.

Figure 7

Referral flow for cardiac genetic investigation of sudden cardiac death (SCD) or resuscitated sudden cardiac arrest (SCA). MDT = multidisciplinary team.

Figure 8

Participants in a cardiac genetic service. “Pathologists” includes forensic pathologists. Modified with permission from Elsevier.

Figure 9

Psychological care following a sudden cardiac arrest (SCA) or a sudden unexplained death (SUD) where a genetic cause is suspected. Colors correspond to the Class of Recommendation in Table 1.

Figure 10

Investigation of sudden unexplained death: personal and family history. Colors correspond to the Class of Recommendation in Table 1. SCD = sudden cardiac death.

Figure 11

Investigation of sudden unexplained death: the postmortem examination and imaging. Colors correspond to the Class of Recommendation in Table 1.

Figure 12

Clinical and genetic evaluation after sudden death where a phenotype is known. Colors correspond to the Class of Recommendation in Table 1. *See Section 8.2.

Figure 13

Investigation of sudden death: genetic evaluation where the phenotype is unknown. Colors correspond to the Class of Recommendation in Table 1. SCD = sudden cardiac death.

Figure 14

Investigation of sudden death. Colors correspond to the Class of Recommendation in Table 1. AED = automated external defibrillator; CIED = cardiovascular implantable electronic device; ECG = electrocardiogram; SCD = sudden cardiac death.

Figure 15

Investigation of sudden cardiac arrest (SCA) survivors: genetic evaluation. Colors correspond to the Class of Recommendation in Table 1.

Figure 16

Investigation of sudden cardiac arrest survivors. Colors correspond to the Class of Recommendation in Table 1. AED = automated external defibrillator; AF = atrial fibrillation; BBR-VT = bundle branch re-entry ventricular tachycardia; CIED = cardiovascular implantable electronic device; CMR = cardiac magnetic resonance imaging; CPVT = catecholaminergic polymorphic ventricular tachycardia; ECG = electrocardiogram; EP = electrophysiological; LQTS = long QT syndrome; SCA = sudden cardiac arrest; SVT = supraventricular tachycardia.

Figure 17

Investigation of the family affected by sudden cardiac arrest and/or sudden unexplained death when cause is identified or not identified. Colors correspond to the Class of Recommendation in Table 1. CMR = cardiac magnetic resonance imaging; ECG = electrocardiogram; SCA = sudden cardiac arrest; SCD = sudden cardiac death; SUD = sudden unexplained death.