Implantable Devices in Genetic Heart Disease: Disease-Specific Device Selection and Programming.

Abstract

Diagnosis and risk stratification of rare genetic heart diseases remains clinically challenging. In many cases, there are few data and insufficient numbers to support randomized controlled trials. While implantable cardioverter defibrillator (ICD) use is vital to protect higher-risk individuals from life-threatening ventricular arrhythmias, low-risk individuals also require protection from unnecessary ICDs and their associated complications. Once an ICD has been implanted, appropriate device programming is essential to ensure maximal protection while balancing the risks of inappropriate therapy.