Ventricular arrhythmias and sudden cardiac death in adults with congenital heart disease

Abstract

Remarkable gains in survival have led to an unprecedented number of adults with congenital heart disease. Arrhythmias collectively comprise the most common complication encountered. Recognising the unique issues and challenges involved in managing arrhythmias in adults with congenital heart disease and the consequential decisions surrounding sudden death prevention, expert societies have proposed evidence-based recommendations. On the whole, acute ventricular arrhythmias are managed according to general cardiology guidelines, while taking into consideration congenital heart disease-specific issues, such as positioning of patches or paddles according to location of the heart. Implantable cardioverter-defibrillators (ICDs) are indicated for secondary prevention in patients with sustained ventricular tachycardia or resuscitated cardiac arrest in the absence of a reversible cause. Pharmacological therapy and catheter ablation can be effective in reducing recurrent ICD shocks. Risk-benefit assessment for primary prevention ICDs is a major challenge. Although a clearer picture has emerged of the high-risk patient with tetralogy of Fallot, ICD indications for those with systemic right ventricles or univentricular hearts remain contentious. Challenges to ICD implantation include obstructed veins, conduits and baffles, atrioventricular valve disease and intracardiac shunts. In selected patients, customised systems with epicardial and/or subcutaneous coils may represent a viable solution. Alternatively, the subcutaneous ICD is an attractive option for patients in whom transvenous access is not feasible or desirable and in whom bradycardia and antitachycardia pacing features are not essential. Continued advances in risk stratification and device technologies carry the potential to further improve efficacy and safety outcomes in this growing population of patients.

Rebuilding a broken heart: lessons from developmental and regenerative biology

“…Cardiac arrhythmias are the leading cause of sudden death (Khairy, 2016;Priori et al, 2016;Saffitz and Corradi, 2016), and many talks focused on the molecular mechanisms underlying such arrhythmias. Examining a post-injury paradigm that occurs in many human tissues, Catherine Lipovsky (from Stacey Rentschler's lab, Washington University in St Louis, USA) found that transient activation of Notch1 in the adult mouse myocardium leads to sinus bradycardia (low heart rate) and slow atrial conduction velocity, suggesting a direct role for Notch activation in long-term maintenance of the atrial electrical phenotype.…”

Risk markers for excess mortality in adults with congenital heart disease: does one size fit all?

“…For more accurate identification of risk markers, one solution to dealing with effect modification is to perform lesion-specific analyses (20). This was addressed in part by Oliver et al's online supplement, which presents survival curves separately for individual types of CHD (3).…”

About Baby Devran: Preventable Maternal Deaths and Ethical Responsibility

“…Pediatrist ya da pediatrik kardiyologların hastalarını erişkinlik döneminde erişkin kardiyoloji ya da kalp damar cerrahisi anabilim dallarına yönlendirmeleri ve bu konuda gerekli bilgilendirmeyi yapmaları, tıbbi ve etik sorumlulukları arasındadır (5). Konjenital ya da edinsel kalp hastalığı olan kadının, hastalığın olası komplikasyonlarından korunması ikincil korumadır (6).…”