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 Developed in partnership with and endorsed by the Asia Pacific Heart Rhythm Society (APHRS) and the Heart Rhythm Society (HRS). Developed in collaboration with and endorsed by the Association for European Cardiovascular Pathology (AECVP), the European Society of Human Genetics (ESHG), the Latin American Heart Rhythm Society (LAHRS), the National Society of Genetic Counselors (NSGC), and the Pediatric and Congenital Electrophysiology Society (PACES). Developed in collaboration with the European Heart Rhythm Association (EHRA). For copies of this document, please contact the Elsevier Inc. Reprint Department

Global approaches to postmortem genetic testing after sudden cardiac death in the young: A survey among healthcare professionals

“…These services require strong leadership and adequate resourcing. The model of the specialized multidisciplinary investigative service as described by recent international guidelines is the gold standard for management of families following a young SCD, and efforts to ensure these are accessible equitably around the world are needed (7).…”

“…This likely perpetuates inequities whereby families with resources can navigate and find appropriate care, while others do not. Systematic processes, as well as adequate resources, are needed to ensure all families who experience a SCD can receive the recommended standard of care, ideally including autopsy, postmortem genetic testing – including genetic counselling - and clinical monitoring of surviving relatives at a multidisciplinary cardiogenetic clinic (7).…”

“…In cases where no structural abnormalities are identified at postmortem evaluation and an underlying inherited arrhythmia syndrome is suspected, genetic testing as part of a multidisciplinary investigation of sudden unexpected death, and this practice is supported by international guideline documents (6)(7)(8)(9). Postmortem genetic testing may identify an underlying inherited cardiac disease in 15-40% of cases (3,(10)(11)(12).…”

Uncovering “bipolar blindness” with high-density orthogonal mapping at the scar-related critical isthmus in repaired congenitally corrected transposition of the great arteries

“…Scar-mediated atrial and ventricular arrhythmias are leading indications for hospitalization and risk factors for sudden cardiac death in the postoperative congenital heart population. 1 Successful transcatheter arrhythmia ablation would, in turn, decrease hospitalizations, improve patient quality of life, and drive down healthcare costs. Arrhythmia mapping and ablation in these patients, however, is plagued by lowvoltage substrates, multiple circuits, and tachycardia recurrence.…”

Investigating sudden cardiac death – A new paradigm

“…The 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, has the quality and strength to enhance the conditions for sudden cardiac arrest survivors and their relatives as well as for relatives of sudden unexplained death victims. 2 …”