Diagnostic evaluation of unexplained ventricular tachyarrhythmias in
younger adults
Abstract
Background: The diagnostic work-up for cardiac arrest
from ventricular tachyarrhythmias occurring in younger adults and
structurally normal hearts is variable and often incomplete.
Methods: We reviewed records for all recipients of a
secondary prevention implantable cardiac defibrillator (ICD) younger
than 60 years at a single quaternary referral hospital from 2010-2021.
Patients were included if they had unexplained ventricular arrhythmias
(UVA) and absence of structural heart disease on echocardiogram, normal
coronary assessment and no clear diagnostic features on ECG. We
specifically evaluated the adoption rate of five modalities of
‘second-line’ cardiac investigations: cardiac magnetic resonance imaging
(CMR), exercise ECG, flecainide challenge, electrophysiology study (EPS)
and genetic testing. We also evaluated patterns of anti-arrhythmic drug
therapy and device-detected arrhythmias and compared them with secondary
prevention ICD recipients with a clear aetiology found on initial
assessment. Results: 102 recipients of a secondary
prevention ICD under the age of 60 were analysed. 39 patients (38.2%)
were identified with UVA and were compared with the remaining 63
patients with VA of clear aetiology (61.8%). UVA patients were younger
(35.6 ± 13.0 years vs 46.0 ± 8.6 years, p<0.001) and were more
often female (48.7% vs 28.6%, p=0.04). CMR was performed in 32
patients with UVA (82.1%), whereas flecainide challenge, stress ECG,
genetic testing and EPS were only performed in a minority of patients.
Overall, the use of a second-line investigation suggested an aetiology
in 17 patients with UVA (43.5%). Compared to patients with VA of clear
aetiology, UVA patients had lower rates of antiarrhythmic drug
prescription (64.1% vs 88.9%, p=0.003) and had a higher rate of
device-delivered tachy-therapies (30.8% vs 14.3%, p=0.045).
Conclusion: In this real-world analysis of patients with
UVA, the diagnostic work-up is often incomplete. While CMR was
increasingly utilized at our institution, investigations for
channelopathies and genetic causes appear to be underutilized.
Implementation of a systematic protocol for work-up of these patients
requires further study.