the treatment of patients with bifascicular block (BfB) and syncope in the absence of structural heart disease (SHD) is not well defined. The objective of our study is to compare pacemaker empirical implantation with the use of electrophysiological studies (EPS). This is a prospective cohort study that included 77 patients with unexplained cardiogenic syncope and BFB without structural heart disease between 1997 and 2012. Two groups: 36 patients received empirical pacemakers (Group A) and 41 underwent EPS (Group B) to guide their treatment. The incidence of syncope recurrence and atrioventricular block was lower in group A. Mortality and complication rates were similar between both groups. Multivariate analysis demonstrated a higher number of events (combined endpoint) in group B. our study shows that treatment according to epS does not improve the results of a treatment strategy based on empirical pacemaker. Management of patients with syncope of unknown origin and bifascicular block (BBF) without significant structural heart disease remains a matter of debate. According to current European guidelines, an electrophysiology study (EPS) should be performed as initial approach and an event recorder or permanent pacemaker implanted based upon the results 1,2. However, this recommendation is not supported by large or prospective studies, and some authors postulate the potential benefit of a direct pacemaker implant giving the 40-50% reported incidence of atrioventricular (AV) block in the 2 years post-EPS 2-4. There is lack of prospective information regarding the clinical benefit of an empirical pacing approach compared to a treatment based on EPS results, in patients without structural heart disease. Our aim is to compare the clinical outcome of patients with syncope of unknown etiology and BFB without significant structural heart disease treated empirically with permanent pacemaker implantation compared to those whose treatment approach was based on the results of an EPS. Methods Patients and treatment algorithm. We designed a cohort study with prospective follow-up to include patients presenting with unexplained syncope and BFB defined as left bundle branch block, or right bundle branch block plus left anterior or posterior fascicular block. Patients signed a written informed consent. The study protocol conforms to the ethical guidelines of the 1975 Declaration of Helsinki as reflected in a priori approval by the institution's human research committee (The Clinical Research Ethics Committee of Granada (CEIC). Patients were excluded from the study in case of structural heart disease, defined as interventricular septal wall thickness >13 mm, left ventricular ejection fraction <50%, valvular heart disease ≥ grade 2, or ECG and cardiac imaging findings suggestive of arrhythmogenic cardiomyopathy 5 (Patients who met diagnostic criteria for arrhythmogenic dysplasia of the right ventricle, two major criteria, one major and two minor or four minor were excluded). Patients were also excluded if they fulfilled ECG criteria...

Introduction Cardiac channelopathies are a frequent cause of sudden cardiac death (SCD) and often manifest with convulsive syncope, leading to a misdiagnosis of epilepsy. We aim to evaluate the clinical impact of epilepsy misdiagnosis in a cohort of patients with cardiac channelopathies. Methods Fifty probands/families with a cardiac channelopathy were included. We retrospectively collected information from medical records to identify all patients who presented with convulsive syncope and were diagnosed with epilepsy after neurological evaluation. Clinical data and outcome were compared with those of patients without a previous epilepsy diagnosis. Results Eight patients had a previous diagnosis of epilepsy. At first episode, 3 of them presented a positive family history of SCD and 5 showed a pathological electrocardiogram; half presented with sudden cardiac arrest (SCA) and the rest with recurrent syncope despite treatment with 1 or more anti-epileptic drugs. Five patients had long QT syndrome, 2 had catecholaminergic polymorphic ventricular tachycardia, and 1 had Brugada syndrome. Epilepsy misdiagnosis was associated with an increased risk of SCA/SCD (OR 6.92, P = .04), a delay of 12 years (P = .047) in correct diagnosis, and a delay from first symptom to channelopathy diagnosis of 18.45 years (P < .0001). Conclusion Cardiac channelopathy patients can be misdiagnosed with epilepsy. This involves a delayed diagnosis, a delay from the first symptom to a correct diagnosis, and an increased risk of SCA/SCD.

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