The validity of current implantable cardioverter-defibrillator guidelines in a real-world population of adults with congenital heart disease: A single-center experience.

Aims: Sudden cardiac death (SCD) is a major cause of mortality in adults with congenital heart disease (ACHD). The role of implantable cardioverter-defibrillator (ICDs) in preventing SCD has been established, however, robust, clinical evidence-based guidelines are lacking in ACHD. The aim of this study was to evaluate the ICD guidelines in ACHD patients.

The Role of Ablation in Prevention of Recurrent Implantable Cardioverter Defibrillator Shocks in Patients With Tetralogy of Fallot.

Background: Implantable cardioverter defibrillators (ICDs) are effective in preventing arrhythmic sudden cardiac death in patients with tetralogy of Fallot (TOF). Although ICD therapies for malignant ventricular arrhythmias can be life-saving, shocks could have deleterious consequences. Substrate-based ablation therapy has become the standard of care to prevent recurrent ICD shocks in patients with ischemic cardiomyopathy.

Impact of Catheter Ablation for Atrial Arrhythmias on Repeat Cardioversion in Adults With Congenital Heart Disease.

Background: Atrial tachyarrhythmias (AAs) are the main source of morbidity and mortality in adult congenital heart disease (ACHD). Direct-current cardioversion (DCCV) is an effective method to acutely terminate AAs, but many patients require repeated DCCV. Little is known about the impact of radiofrequency catheter ablation (RFCA) of AAs on the incidence of repeated DCCV in patients with ACHD.

Monitoring the patient with transposition of the great arteries: arterial switch versus atrial switch.

Sufficient time has passed that adult congenital heart disease (ACHD) specialists now frequently encounter survivors born with complete transposition of the great arteries and palliated with an atrial or arterial switch procedure. To ensure the ongoing health of these patients, it is of paramount importance that their surgeries are understood and that physicians are aware of and remain vigilant for potential late complications. Adult survivors should be assessed annually in a regional ACHD center.

Isovolumic acceleration at rest and during exercise in children normal values for the left ventricle and first noninvasive demonstration of exercise-induced force-frequency relationships.

Objectives: This study aimed to determine the normal variation of left ventricular (LV) isovolumic acceleration (IVA) in healthy children and to assess the feasibility of an entirely noninvasive method for demonstration of the LV force-frequency relationship (FFR).

Background: Pediatric cardiologists continue to seek noninvasive, load-independent indexes for the assessment of LV contractility and myocardial reserve.

Methods: Resting LV IVA was measured by echocardiogram in 236 healthy children and compared with their clinical characteristics.

Evaluation of mechanical dyssynchrony in children with idiopathic dilated cardiomyopathy and associated clinical outcomes.

We studied mechanical dyssynchrony and its association with clinical status in children with idiopathic dilated cardiomyopathy (IDC). The SD of QRS to peak systolic velocity interval by tissue Doppler was measured in 12 left ventricular segments, as a dyssynchrony index (DI), in each child with IDC during a 12-month period. Results were compared with a control cohort.