Challenging indication of cardioverter defibrillator implantation after sudden cardiac arrest in the very young: a case series of catecholaminergic polymorphic ventricular tachycardia secondary to de novo calmodulin p.Asn98Ser.

BACKGROUND: Calmodulinopathy is an emerging group of primary electrical disease with various, severe, and early onset phenotype. Sudden cardiac arrest (SCA)/death can be the first symptom and current medical management seems insufficient to prevent recurrences. Implantable cardioverter-defibrillator (ICD) in the young is challenging and can be harmful. CASE SUMMARY: We report the management of two very young boys (aged 3.5 and 5.5 years old) who survived an SCA due to calmodulin mutation responsible of a catecholaminergic polymorphic ventricular tachycardia phenotype. In both case, SCA had an adrenergic trigger. Despite SCA, ICD implantation was denied by the parents. After thorough discussion with the family, the patients were managed with solely betablocker treatment and loop recorder implantation. At last follow-up of 30 and 23 months, respectively, there were no recurrence of any cardiac event. DISCUSSION: The benefits of ICD implantation at a very young age must be weighed against the risk complication. In the youngest, whom recreative activities are under constant supervision, the decision, jointly made with the parents, could be to postpone ICD.

Cavotricuspid isthmus is constantly a zone of slow conduction: Data from ultra-high-resolution mapping.

BACKGROUND: Whether cavotricuspid isthmus (CTI) is a region of conduction slowing during typical flutter has been discussed with conflicting results in the literature. We aimed to evaluate conduction velocity (CV) along the different portions of the typical flutter circuit with a recently proposed method by means of ultra-high-resolution (UHR) mapping. METHODS: Consecutive patients referred for typical atrial flutter (AFL) ablation underwent UHR mapping (Rhythmia, Boston Scientific). CVs were measured in the CTI as well as laterally and septally, respectively, from its lateral and septal borders. RESULTS: A total of 33 patients (mean age: 65 ± 13 years; right atrial volume: 134 ± 57 mL) were mapped either during ongoing counterclockwise (n = 25), or clockwise (n = 3) AFL (mean cycle length: 264 ± 38 ms), or during coronary sinus pacing at 400 ms (n = 1), 500 ms (n = 1), or 600 ms (n = 3). A total of 13 671 ± 7264 electrograms were acquired in 14 ± 9 min. CTI CV was significantly lower (0.56  ± 0.18 m/s) in comparison with the lateral CV (1.31 ± 0.29 m/s; P < .0001) and the septal border CV of the CTI (1.29 ± 0.31 m/s; P < .0001). CONCLUSION: UHR mapping confirmed that CTI CV was systematically twice lower than atrial conduction velocities outside the CTI.

Scar identification, quantification, and characterization in complex atrial tachycardia: a path to targeted ablation?

Successful catheter ablation of scar-related atrial tachycardia depends on correct identification of the critical isthmus. Often, this is a represented by a small bundle of viable conducting tissue within a low-voltage area. It's identification depends on the magnitude of the signal/noise ratio. Ultra-high density mapping, multipolar catheters with small (eventually unidirectional) and closely-spaced electrodes improves low-voltage electrogram detection. Background noise limitation is also of major importance for improving the signal/noise ratio. Electrophysiological properties of the critical isthmus and the characteristics of the local bipolar electrograms have been recently demonstrated as hallmarks of successful ablation sites in the setting of scar-related atrial tachycardia.