Systematic clinical testing, including drug provocation and advanced imaging, identified a specific underlying cause in 56% of patients with apparently unexplained cardiac arrest.
Observational (n=63)
Yes
Does systematic clinical testing identify the underlying cause in patients with unexplained cardiac arrest?
Systematic clinical testing, including drug provocation and advanced imaging, successfully identifies the underlying cause in over 50% of patients with apparently unexplained cardiac arrest, facilitating targeted genetic testing and family screening.
BACKGROUND: Cardiac arrest without evident cardiac disease may be caused by subclinical genetic conditions. Provocative testing to unmask a phenotype is often necessary to detect primary electrical disease, direct genetic testing, and perform family screening. METHODS AND RESULTS: Patients with apparently unexplained cardiac arrest and no evident cardiac disease (normal cardiac function on echocardiogram, no evidence of coronary artery disease, and a normal ECG) underwent systematic evaluation that included cardiac magnetic resonance imaging, signal-averaged ECG, exercise testing, drug challenge, and selective electrophysiological testing. Diagnostic criteria were based on accepted criteria or provocation of the characteristic clinical features for long-QT syndrome, catecholaminergic polymorphic ventricular tachycardia, Brugada syndrome, early repolarization, arrhythmogenic right ventricular cardiomyopathy, coronary spasm, and myocarditis. Sixty-three patients in 9 centers were enrolled (age 43.0+/-13.4 years, 29 women). A diagnosis was obtained in 35 patients (56%): Long-QT syndrome in 8, catecholaminergic polymorphic ventricular tachycardia in 8, arrhythmogenic right ventricular cardiomyopathy in 6, early repolarization in 5, coronary spasm in 4, Brugada syndrome in 3, and myocarditis in 1. Targeted genetic testing demonstrated evidence of causative mutations in 9 (47%) of 19 patients. Screening of 64 family members of these patients identified 15 affected individuals who were treated (24%). The remaining 28 patients (44%) were considered to have idiopathic ventricular fibrillation. CONCLUSIONS: Systematic clinical testing, including drug provocation and advanced imaging, results in unmasking of the cause of apparently unexplained cardiac arrest in >50% of patients. This approach assists in directing genetic testing to diagnose genetically mediated arrhythmia syndromes, which results in successful family screening.
Krahn et al. (Mon,) conducted a observational in Unexplained cardiac arrest (n=63). Systematic clinical testing (cardiac MRI, signal-averaged ECG, exercise testing, drug challenge, selective EP testing) was evaluated on Diagnosis of the underlying cause of cardiac arrest. Systematic clinical testing, including drug provocation and advanced imaging, identified a specific underlying cause in 56% of patients with apparently unexplained cardiac arrest.
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