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

. Author manuscript; available in PMC: 2021 Jun 11.

Published in final edited form as: Heart Rhythm. 2020 Oct 19;18(1):e1–e50. doi: 10.1016/j.hrthm.2020.10.010

Recommendations for investigation of SCA survivors: provocative testing

COR	LOE	Recommendations	References
1	B-NR	1. Exercise testing is recommended in all undiagnosed SCA survivors to induce arrhythmias that may support the diagnoses of arrhythmogenic cardiomyopathy and CPVT and to evaluate dynamic depolarization or repolarization features that may support the diagnoses of Brugada syndrome, arrhythmogenic cardiomyopathy, and long QT syndrome.	^{216,220,263–265,270,271,302,303}
2a	B-NR	2. Lying to standing ECGs can be useful in SCA survivors for the diagnosis of long QT syndrome, but must be interpreted with caution in children.	^266–270
2b	B-NR	3. Epinephrine challenge may be considered for the diagnosis of long QT syndrome and CPVT, in those unable to exercise.	^{273–275,277,304}
1	B-NR	4. Sodium channel blocker challenge with standard and high precordial ECG leads is recommended for the diagnosis of Brugada syndrome in undiagnosed SCA survivors with suggestive clinical characteristics, including a type 2 or 3 Brugada ECG pattern.	^{233,273–275,277,286}
2a	B-NR	5. Sodium channel blocker challenge with standard and high precordial ECG leads can be useful for the diagnosis of Brugada syndrome in SCA survivors where no other disorder has been identified.	^{233,279–282}
2b	B-NR	6. Ergonovine, acetylcholine, or hyperventilation testing when performed in experienced centers may be considered for the diagnosis of coronary vasospasm as the cause of SCA in a survivor where no other disorder has been identified.	^216,294,295
2b	C-LD	7. Adenosine challenge may be useful for the unmasking of ventricular pre-excitation and therefore the diagnosis of rapidly conducted atrial arrhythmia as the likely cause of SCA in a survivor where no other disorder has been identified.	²⁹⁷
2a	C-LD	8. An electrophysiological study can be considered if bundle branch re-entrant ventricular tachycardia, pre-excited atrial fibrillation, or supraventricular tachycardia are suspected in an SCA survivor.	^298,299
2b	C-LD	9. Electroanatomic right ventricular voltage mapping may be considered for detection of subclinical arrhythmogenic cardiomyopathy in an SCA survivor where no other disorder has been identified.	³⁰⁰
2b	C-LD	10. An electrophysiological study may be considered in an SCA survivor where no other disorder has been identified to evaluate potential underlying substrate.	³⁰¹