Sudden Cardiac Death in Hereditary Dilated Cardiomyopathy

Dilated cardiomyopathy (DCM) is characterized by the phenotype of a dilated left ventricle with systolic dysfunction. It is classified as hereditary when it is deemed of genetic origin; more than 50 genes are reported to be related to the condition. Symptoms include, among others, dyspnea, fatigue, arrhythmias, and syncope. Unfortunately, sudden cardiac death may be the first manifestation of the disease. Risk stratification regarding sudden death in hereditary DCM as well as preventive management poses a challenge due to the heterogeneity of the disease. The purpose of this chapter is to present the epidemiology, risk stratification, and preventive strategies of sudden cardiac death in hereditary DCM

An Overview of the Cardiomyopathies

Cardiomyopathies constitute a heterogeneous group of heart diseases. In fact, cardiomyopathies is a major cause of death either as end-stage heart failure or sudden cardiac death. Even though prognosis is, in many cases, poor there are several approaches to optimal disease management, which improves outcome and implies better quality of life including reduced risk of hospitalization. Differentiation of underlying etiology in individual cases of cardiomyopathies requires careful clinical evaluation. Echocardiography is the cornerstone in initial evaluation and follow-up but cardiac magnetic resonance provides additional value. ECG, biomarkers, detailed history taking and extracardiac features may provide clues to less common entities. While forty years ago cardiomyopathy was defined as heart muscle disease of unknown origin, the underlying pathophysiology has now been elucidated. Indeed, the last decades the genetic explanations have evolved. Advanced treatment with pacemakers, including cardiac resynchronization, implantable defibrillators, and mechanical devices in the most severe cases are nowadays available for many patients. The evidence-based pharmacological approach to heart failure provides multiple interaction of pathophysiological pathways and has improved outcome. In selected cases specific agents are indicated why differential diagnosis is crucial and the genetic link imply cascade screening. This chapter aims to present a comprehensive overview of the cardiomyopathies, categorized into: dilated-, hypertrophic-, restrictive-, arrhythmogenic and unclassified cardiomyopathy

Naxos Disease: Current Knowledge and Future Advances

Naxos disease is a genetic cardiocutaneous syndrome manifesting with a cardiomyopathy that belongs in the arrhythmogenic right ventricular cardiomyopathy (ARVC) spectrum and follows an autosomal recessive pattern. It manifests with wooly hair, keratosis of the extremities and right ventricular dysfunction. It is accompanied by risk of arrhythmias as well as sudden cardiac death (SCD), even at a young age. Furthermore, the disease often progresses to right ventricular heart failure, but can also affect the left ventricle. Patient management follows current guidelines on ARVC and principles for heart failure management. Bioengineering and research about pluripotent stem cells seem to have potential to improve future management of the disease. This chapter covers current knowledge on Naxos disease regarding clinical features, epidemiology, pathogenesis, guidelines on patient management and provides insights in research frontlines

The role of automated compression devices in out-of- and in- hospital cardiac arrest. Can we spare rescuers' hands?

Research regarding the use of mechanical compressions in the setting of a cardiac arrest, either outside of or inside the hospital environment has produced mixed results. The debate whether they can replace manual compressions still remains. The aim of this review is to present current literature contemplating the application of mechanical compressions in both settings, data comparing them to manual compressions as well as current guidelines regarding their implementation in everyday clinical use. Currently, their implementation in the resuscitation protocol seems to benefit the victims of an in-hospital cardiac arrest rather than the victims that sustain a cardiac arrest outside of the hospital

Radial Artery and Ulnar Artery Occlusions Following Coronary Procedures and the Impact of Anticoagulation: ARTEMIS (Radial and Ulnar ARTEry Occlusion Meta-AnalysIS) Systematic Review and Meta-Analysis

Background-Incidence of radial artery occclusions (RAO) and ulnar artery occclusions (UAO) in coronary procedures, factors predisposing to forearm arteries occlusion, and the benefit of anticoaggulation vary significantly in existing literature. We sought to determine the incidence of RAO/UAO and the impact of anticoagulation intensity. Methods and Results-Meta-analysis of 112 studies assessing RAO and/or UAO (N = 46 631) were included. Overall, there was no difference between crude RAO and UAO rates (5.2%; 95% confidence interval [CI], 4.4-6.0 versus 4.0%; 95% CI, 2.8-5.8; P= 0.171). The early occlusion rate (in-hospital or within 7 days after procedure) was higher than the late occlusion rate. The detection rate of occlusion was higher with vascular ultrasonography compared with clinical evaluation only. Low-dose heparin was associated with a significantly higher RAO rate compared with high-dose heparin (7.2%; 95% CI, 5.5-9.4 versus 4.3%; 95% CI, 3.5-5.3; Q = 8.81; P = 0.003). Early occlusions in low-dose heparin cohorts mounted at 8.0% (95% CI, 6.1-10.6). The RAO rate was higher after diagnostic angiographies compared with coronary interventions, presumably attributed to the higher intensity of anticoagulation in the latter group. Hemostatic techniques (patent versus nonpatent hemostasis), geography (US versus non-US cohorts) and sheath size did not impact on vessel patency. Conclusions-RAO and UAO occur with similar frequency and in the order of 7% to 8% when evaluated early by vascular ultrasonography following coronary procedures. More-intensive anticoagulation is protective. Late recanalization occurs in a substantial minority of patients