Hearts, Volume 1, Issue 3 (December 2020) – 5 articles

Inherited cardiac arrhythmias contribute substantially to sudden cardiac death in the young. The underlying pathophysiology remains incompletely understood because of the lack of representative study models and the labour-intensive nature of electrophysiological patch clamp experiments. Whereas patch clamp is still considered the gold standard for investigating electrical properties in a cell, optical mapping of voltage and calcium transients has paved the way for high-throughput studies. Moreover, the development of human-induced pluripotent stem-cell-derived cardiomyocytes (hiPSC-CMs) has enabled the study of patient specific cell lines capturing the full genomic background. Nevertheless, hiPSC-CMs do not fully address the complex interactions between various cell types in the heart. Studies using in vivo models, are therefore necessary. Given the analogies between the human and zebrafish cardiovascular system, zebrafish has emerged as a cost-efficient model for arrhythmogenic diseases. In this review, we describe how hiPSC-CM and zebrafish are employed as models to study primary electrical disorders. We provide an overview of the contemporary electrophysiological phenotyping tools and discuss in more depth the different strategies available for optical mapping. We consider the current advantages and disadvantages of both hiPSC-CM and zebrafish as a model and optical mapping as phenotyping tool and propose strategies for further improvement. Overall, the combination of experimental readouts at cellular (hiPSC-CM) and whole organ (zebrafish) level can raise our understanding of the complexity of inherited cardiac arrhythmia disorders to the next level. Full article

Cardiovascular imaging techniques, including echocardiography, nuclear cardiology, multi-slice computed tomography, and cardiac magnetic resonance, have wide applications in cardiac resynchronization therapy (CRT). Our aim was to provide an update of cardiovascular imaging applications before, during, and after implantation of a CRT device. Before CRT implantation, cardiovascular imaging techniques may integrate current clinical and electrocardiographic selection criteria in the identification of patients who may most likely benefit from CRT. Assessment of myocardial viability by ultrasound, nuclear cardiology, or cardiac magnetic resonance may guide optimal left ventricular (LV) lead positioning and help to predict LV function improvement by CRT. During implantation, echocardiographic techniques may guide in the identification of the best site of LV pacing. After CRT implantation, cardiovascular imaging plays an important role in the assessment of CRT response, which can be defined according to LV reverse remodeling, function and dyssynchrony indices. Furthermore, imaging techniques may be used for CRT programming optimization during follow-up, especially in patients who turn out to be non-responders. However, in the clinical settings, the use of proposed functional indices for different imaging techniques is still debated, due to their suboptimal feasibility and reproducibility. Moreover, identifying CRT responders before implantation and turning non-responders into responders at follow-up remain challenging issues. Full article

Aortic dissection (AD) causes more deaths each year in the United Kingdom than road traffic collisions. Yet the incidence of AD is not known. The management of acute type B AD (TBAD) is changing, with the greater use of thoracic aortic stent grafts (TEVAR) in treatment and fewer open surgical procedures performed. The study’s aim is to review the worldwide, English language published, literature on acute TBAD incidence and treatment, to report on its strengths and limitations, and better understand changes in incidence over time and between countries. Thirty-one studies were identified that focus on the epidemiology and treatment of TBAD. Eight of these studies report the incidence of acute TBAD as between of 0.5–6.3 per 100,000 person years. Hospital admissions for aortic dissection are reported to be increasing in six studies and stable in one study. The proportion of patients with TBAD operated on varies between studies (range 13% to 76%). Studies identify patient age (median 51–77 years), gender (range 48%–81% male) and prevalence of cardio-vascular risk factors, specifically hypertension, in the populations studied as independent factors influencing aortic dissection incidence. Treatment of acute TBAD remains largely conservative with analgesia, hypertension control and serial cross-sectional imaging (range 24%–87% TBAD medically treated). The use of TEVAR to treat acute AD is increasing worldwide (range 13%–76% TBAD treated with TEVAR). The incidence of TBAD is under-reported due to out of hospital deaths, variable clinical presentation (miss-diagnosis) and coding errors. Importantly for research, the single International Classification of Diseases (ICD) code for aortic dissection, I17.0, does not distinguish between acute, chronic, type A or type B dissection types. Similarly, the OPCS Classification of Interventions and Procedures version 4 (OPCS-4) codes for TEVAR, L27.4 and L28.4, do not distinguish between acute and chronic AD presentation, unlike the codes for open thoracic aortic replacement. Standardised reporting of aortic dissection type, and the urgency of both the initial presentation (acute or chronic) and treatment (emergency, urgent or planned) in future studies would allow more meaningful comparisons between populations. Full article