A Clinical Diagnostic Test for Calcium Release Deficiency Syndrome.

Importance: Sudden death and cardiac arrest frequently occur without explanation, even after a thorough clinical evaluation. Calcium release deficiency syndrome (CRDS), a life-threatening genetic arrhythmia syndrome, is undetectable with standard testing and leads to unexplained cardiac arrest. Objective: To explore the cardiac repolarization response on an electrocardiogram after brief tachycardia and a pause as a clinical diagnostic test for CRDS. Design, Setting, and Participants: An international, multicenter, case-control study including individual cases of CRDS, 3 patient control groups (individuals with suspected supraventricular tachycardia; survivors of unexplained cardiac arrest [UCA]; and individuals with genotype-positive catecholaminergic polymorphic ventricular tachycardia [CPVT]), and genetic mouse models (CRDS, wild type, and CPVT were used to define the cellular mechanism) conducted at 10 centers in 7 countries. Patient tracings were recorded between June 2005 and December 2023, and the analyses were performed from April 2023 to December 2023. Intervention: Brief tachycardia and a subsequent pause (either spontaneous or mediated through cardiac pacing). Main Outcomes and Measures: Change in QT interval and change in T-wave amplitude (defined as the difference between their absolute values on the postpause sinus beat and the last beat prior to tachycardia). Results: Among 10 case patients with CRDS, 45 control patients with suspected supraventricular tachycardia, 10 control patients who experienced UCA, and 3 control patients with genotype-positive CPVT, the median change in T-wave amplitude on the postpause sinus beat (after brief ventricular tachycardia at ≥150 beats/min) was higher in patients with CRDS (P < .001). The smallest change in T-wave amplitude was 0.250 mV for a CRDS case patient compared with the largest change in T-wave amplitude of 0.160 mV for a control patient, indicating 100% discrimination. Although the median change in QT interval was longer in CRDS cases (P = .002), an overlap between the cases and controls was present. The genetic mouse models recapitulated the findings observed in humans and suggested the repolarization response was secondary to a pathologically large systolic release of calcium from the sarcoplasmic reticulum. Conclusions and Relevance: There is a unique repolarization response on an electrocardiogram after provocation with brief tachycardia and a subsequent pause in CRDS cases and mouse models, which is absent from the controls. If these findings are confirmed in larger studies, this easy to perform maneuver may serve as an effective clinical diagnostic test for CRDS and become an important part of the evaluation of cardiac arrest.

Pulmonary Vein Stenosis After Atrial Fibrillation Ablation: Insights From the ADVICE Trial.

BACKGROUND: Pulmonary vein (PV) stenosis is a complication of atrial fibrillation (AF) ablation. The incidence of PV stenosis after routine post-ablation imaging remains unclear and is limited to single-centre studies. Our objective was to determine the incidence and predictors of PV stenosis following circumferential radiofrequency ablation in the multicentre Adenosine Following Pulmonary Vein Isolation to Target Dormant Conduction Elimination (ADVICE) trial. METHODS: Patients with symptomatic AF underwent circumferential radiofrequency ablation in one of 13 trial centres. Computed tomographic (CTA) or magnetic resonance (MRA) angiography was performed before ablation and 90 days after ablation. Two blinded reviewers measured PV diameters and areas. PVs with stenosis were classified as severe (> 70%), moderate (50%-70%), or mild (< 50%). Predictors of PV stenosis were identified by means of multivariable logistic regression. RESULTS: A total of 197 patients (median age 59.5 years, 29.4% women) were included in this substudy. PV stenosis was identified in 41 patients (20.8%) and 47 (8.2%) of 573 ablated PVs. PV stenosis was classified as mild in 42 PVs (7.3%) and moderate in 5 PVs (0.9%). No PVs had severe stenosis. Both cross-sectional area and diameter yielded similar classifications for severity of PV stenosis. Diabetes was associated with a statistically significant increased risk of PV stenosis (OR 4.91, 95% CI 1.45-16.66). CONCLUSIONS: In the first systematic multicentre evaluation of post-ablation PV stenosis, no patient acquired severe PV stenosis. Although the results are encouraging for the safety of AF ablation, 20.8% of patients had mild or moderate PV stenosis, in which the long-term effects are unknown.

Harder Roads to Trek? Paradoxical Slowing of an Atrioventricular Reentrant Tachycardia With Contralateral Bundle Branch Block.

Slowing of the tachycardia with increase in cycle length and increase in VA time with ipsilateral bundle branch block is a characteristic feature of accessory pathway mediated AVRT. Contralateral bundle branch block has no effect on the tachycardia as it is not a part of the tachycardia circuit. We present an interesting phenomenon in which contralateral bundle branch block resulted in tachycardia slowing in a case of WPW syndrome.

Device-related infection among patients with pacemakers and implantable defibrillators: incidence, risk factors, and consequences.

BACKGROUND: Device-related infection is a major limitation of device therapy for cardiac arrhythmia. METHODS: The authors analyzed the incidence and risk factors for cardiac device infection (CDI) among consecutive patients implanted with pacemaker (PM) or implantable cardioverter defibrillator (ICD) (including cardiac resynchronization therapy devices) at a tertiary health center in Hamilton, Ontario, Canada. Most patients with device-related infections were identified by an internal infection control system that reports any positive wound and blood cultures following surgery, between 2005 and the present. A retrospective review of patient records was also performed for all patients who received an ICD or PM between July 1, 2003 and March 20, 2007. RESULTS: A total of 24 infections were identified among 2,417 patients having device surgery (1%). Fifteen of these infections (60%) were diagnosed within 90 days of the last surgical procedure. Univariate analysis showed that patients presenting with CDI were more likely to have had a device replacement, rather than a new implant, had more complex devices (dual/triple chamber vs single), and were more likely to have had a prior lead dislodgement. Multivariate analysis found device replacement (P = 0.02) and cardiac resynchronization therapy (CRT)/dual-chamber devices (P = 0.048) to be independent predictors of infection. One patient developed septic pulmonary emboli after having laser-assisted lead extraction. No patient died and 22 patients received a new device. CONCLUSION: CDI occurs in about 1% of cases in high volume facilities. Pulse generator replacement surgery and dual- or triple-chamber device implantation were associated with a significantly increased risk of infection.