full-text:
Sudden Death in the Young

Advances in Arrhythmia and Electrophysiology

Sudden Death in the Young

What Do We Know About It and How to Prevent?

1. Christian van der Werf, MD,
2. Irene M. van Langen, MD, PhD and
3. Arthur A.M. Wilde, MD, PhD

+ Author Affiliations

1. From the Heart Failure Research Center and the Department of Cardiology (C.v.d.W., A.A.M.W.), and the Department of Clinical Genetics (I.M.L.), Academic Medical Center, Amsterdam, The Netherlands.

1. Correspondence to Arthur A.M. Wilde, MD, PhD, Academic Medical Center, University of Amsterdam, Heart Failure Research Center, Department of Cardiology, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. E-mail a.a.wilde@amc.uva.nl

Key Words:

• death, sudden
• genetics
• prevention

Next Section

Introduction

Sudden death (SD) of children and adults ages 1 to 40 years has received increasing attention over the past decades. In this report, we provide an overview of this problem, consider several mass screening strategies to prevent these events, and critically appraise these strategies in light of the well known Wilson and Jungner criteria (Table 1).¹ Finally, we present an alternative approach: optimal assessment of first-degree relatives of newly diagnosed patients with an inherited cardiac disease and young SD victims to identify those relatives at risk of sudden cardiac death (SCD).

View this table:

• In this window
• In a new window

Table 1. Wilson and Jungner Criteria for Assessment of Screening

Previous SectionNext Section

The Problem

Definitions

SD or its synonyms are generally defined as natural, unexpected death within 1 hour of the onset of symptoms.² Four temporal elements should be considered in the use of this definition, that is, prodromes, onset of the terminal event, cardiac arrest, and biological death. The 1-hour definition refers to the period between onset of the terminal event, that is, acute changes in cardiovascular status, and cardiac arrest. Nonspecific prodromal symptoms, for example, chest pain, palpitations, or dyspnea, can be present during the days or weeks before a cardiac arrest.³ The biological legal death can occur days or weeks after the cardiac arrest, as patients can survive with irreversible brain damage and life support. In addition, when death occurs unwitnessed within 24 hours of being seen alive and functioning normally, this is also termed SD.² In this report, the 1-hour definition is applied unless otherwise indicated.

Depending on the underlying cause, SD can be divided into SCD, defined as SD from a cardiac cause,² and SD due to noncardiac causes, for example, intracranial hemorrhage, epilepsy, pulmonary embolism, or asthma.^4,5⇓ This subdivision is clinically relevant because cardiac causes are inherited in a significant proportion, whereas noncardiac causes usually are not. Death in absence of a diagnosis despite autopsy is generally termed sudden unexplained death (SUD) or autopsy-negative SUD. In countries where autopsy is not mandatory in the case of SD at a young age, cases in which no autopsy is performed are considered to be SUD.⁶

SD remaining unexplained after thorough postmortem investigation in infants under 1 year of age is termed sudden infant death syndrome (SIDS) and is considered a different entity in terms of etiology.⁷