Most patients with long QT syndrome or catecholaminergic polymorphic ventricular tachycardia do not need an implantable cardioverter defibrillator when treated with optimal medical therapy and left cardiac sympathetic denervation.
Does an implantable cardioverter defibrillator improve survival compared to optimal medical therapy in patients with long QT syndrome or catecholaminergic polymorphic ventricular tachycardia?
Optimal medical therapy and left cardiac sympathetic denervation may provide sufficient protection against mortality in LQTS and CPVT, challenging the routine use of ICDs.
Few medical decisions have a greater impact on the life of patients affected by either long QT syndrome (LQTS) or catecholaminergic polymorphic ventricular tachycardia (CPVT) as the one to recommend an implantable cardioverter defibrillator (ICD) or not. If the decision is correct, a life might be saved or a litany of ICD-related complications might be avoided. If it is wrong, well you can fill in the blanks. Many physicians take the fastest and simplest road by recommending an ICD which represents 'double protection': for the patients and for themselves. Our opinion is that this requires a very careful consideration about the pros and cons and should be taken neither lightly nor quickly. Moreover, there is another side to this moon, which also requires more thought than usually assumed. As to LQTS and CPVT, the current data strongly indicate that when high risk patients are treated with either combination drug therapy comprising beta blockers and mexiletine (LQTS) or beta blockers and flecainide (CPVT) or triple therapy with left cardiac sympathetic denervation as the treatment intensifier, mortality is close to zero and most patients have no life-threatening events. Thus, we stand by our view that most patients still do not need and should not receive an ICD. However, sometimes patients are desperately worried for the fear of dying suddenly and ask for an ICD despite being at low risk. Their request should be listened to, respected, and considered with empathy because immortality cannot be guaranteed. We believe that both expertise and compassion are quintessential to practicing the science and art of medicine.
Schwartz et al. (Thu,) conducted a editorial in Long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT). Implantable cardioverter defibrillator (ICD) vs. Medical therapy and left cardiac sympathetic denervation (LCSD) was evaluated. Most patients with long QT syndrome or catecholaminergic polymorphic ventricular tachycardia do not need an implantable cardioverter defibrillator when treated with optimal medical therapy and left cardiac sympathetic denervation.