日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Background: Increasing number of automated external defibrillators (AED) has been installed in public institutions nationwide. Therefore, there is increasing interest in the prognosis of patients who survived after incidences of ventricular fibrillation (VF) and were resuscitated using AEDs. However, little information is available about VF survivors with congenital heart disease (CHD).

Methods: Six CHD patients (median age: 17.1 years), who were resuscitated using an AED and subsequently underwent implantable cardioverter defibrillator (ICD) implantation in our hospital between November 2006 and October 2012, were reviewed. Patient demographics and periprocedural data of the ICD were analyzed.

Results: Underlying diseases consisted of two patients with tetralogy of Fallot and one each of corrected transposition of the great arteries, subaortic stenosis, myocardial infarction post-surgery for congenital mitral regurgitation, and anomalous origin of the coronary artery. In five patients, repair of the cardiac abnormality was simultaneously performed with the ICD implantation. During a median follow-up period of 3.9 years, one patient received appropriate therapy (once) and three patients received inappropriate therapies (eight times in total).

Conclusions: Here we reported on our strategy and the clinical outcome of VF survivors with CHD who underwent ICD implantation at our hospital. Our treatment policy for ventricular tachycardia/VF includes aggressive intervention of the underlying cardiac abnormality and jeopardized hemodynamics using cardiac medications and surgery in addition to ICD implantation. As a result of this useful strategy, all patients are still alive without residual disability.