Sudden cardiac death (SCD) accounts for 230,000 to 350,000 deaths per year in the United States. While many who suffer SCD possess underlying structural heart disease, inherited arrhythmia syndromes are also important contributors to SCD. In patients without structural heart disease, inherited arrhythmia syndromes are identified in more than 50% of the remaining patients. In this review, we will focus on the presentation and management of three major inherited syndromes that lead to SCD in patients without structural heart disease: long QT syndrome (LQTS), Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia (CPVT). All these syndromes can present in patients who are asymptomatic or, at the other extreme, with syncope and even SCD. LQTS syndrome and Brugada are the most common inherited arrhythmogenic syndromes, while CPVT is much rarer. Determining which patients need pharmacologic treatment and those who would benefit from more aggressive treatment such as sympathectomies and implantable defibrillators is not always clear.

Background/Objectives To study the mechanism of restoration of retinal photoreceptor ellipsoid zone (EZ), after intravitreal bevacizumab (IVB) therapy, in diabetic macular oedema (DMO). Subjects/Methods Forty-four consecutive patients aged 40-65 years having type 2 diabetes mellitus (DM) with DMO were prospectively recruited for IVB therapy. It comprised of three doses (1.25 mg in 0.05 ml) of IVB at monthly intervals. Patients with other ocular and systemic diseases affecting retinal vessels and earlier ophthalmological interventions were excluded. Visual acuity (logMAR VA) was recorded. Spectral domain optical coherence tomography (SD-OCT) was performed pre and post intervention. Central sub-foveal thickness (CST) and grades of disorganization of retinal inner layers (DRIL), external limiting membrane (ELM) and EZ were assessed. Data were statistically analysed on SPSS software. Clinical trials registry: CTRI/2019/03/018135. Results Mean logMAR VA decreased after IVB therapy from 1.78 ± 0.07 pre-intervention to 0.42 ± 0.05 post intervention (p < 0.001). Similarly, CST reduced from 354.23 ± 15.0 µm pre-intervention to 233.18 ± 7.88 µm post intervention (p < 0.001). Among qualitative variables, DRIL decreased from 93.2% pre-intervention to 13.6% post intervention. Likewise, global ELM disruption reduced from 81.8 to 9.1% and global EZ disruption reduced from 79.5 to 11.4%. ELM restoration preceded EZ restoration. Conclusion Anti-VEGF therapy restores the barrier effect of ELM. It causes ELM to restore first followed by EZ restoration in DMO.

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