Risk of cardioembolic ischemic events and relation to atrial fibrillation/flutter in patients with arrhythmogenic cardiomyopathy during a long-term follow-up.

BACKGROUND: Arrhythmogenic cardiomyopathy (ACM) is an inherited heart disease characterized by fibro-fatty replacement of myocardium. Limited data is available concerning cardioembolic stroke. This study sought to determine the occurrence of cardioembolic ischemic events (CIEs) in ACM patients and to identify clinical and imaging predictors of CIEs. METHODS: Every consecutive ACM patient was enrolled. ECG, Holter monitoring or implantable cardiac devices were used to detect atrial arrhythmias (AAs). CIEs were defined according to TOAST classification. RESULTS: In our cohort of 111 patients, CIEs were observed in eleven (10%) over a 12.9-year median follow-up, with an incidence of 7.9 event/1000 patient-year (HR 4.12 compared to general population). Mean age at the event was 42 ± 9 years. Female sex (p = 0.03), T-wave inversion (p = 0.03), RVOT dilatation (p = 0.006) and lower LVEF (p = 0.006) were associated with CIEs. Among patients with AAs (23/111, 20.7%), 5 (21.7%) experienced CIEs. CHA2DS2-VASc did not prove useful to define patients at higher risk of CIEs (p = 0.098). 60% of stroke suffering patients had a pre-event score between 0 and 1 (if female). CONCLUSIONS: In ACM patients, CIEs are much more common than in general population and present a high burden at younger age. AAs relate to less than half of these events. In AAs patients, CHA2DS2-VASc is not useful to stratify those requiring oral anticoagulation. As a hypothesis-generating study, our research proposes the role of atrial myopathy, irrespective of AAs, as a pivotal factor in thrombogenesis risk, pointing out a definite unmet need in ACM therapeutic algorithm.

Phenotypic expression of ARVC: How 12 lead ECG can predict left or right ventricle involvement. A familiar case series and a review of literature.

AIMS: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart-muscle disease primarily affecting the right ventricle (RV) and potentially causing sudden death in young people. The natural history of the disease is firstly characterized by a concealed form progressing over a biventricular involvement. Three different cases coming from the same family are presented together with a review of the literature. METHODS AND RESULTS: Multi-parameter analysis including imaging and electrocardiographic analysis is presented since the first medical referral with follow-up ranging from 11 to 38years. Case 1 presented a typical RV involvement in agreement with the ECG pattern. Case 2 presented a prevalent left ventricular involvement leading from the beginning to a pattern of dilated cardiomyopathy in agreement with his ECG evolution over the years. On the other side, Case 3 came to observation with a typical RV involvement (similar to Case 1) but with ECG evolution of typical left ventricle involvement (similar to Case 2). The genetic analysis showed a mutation in desmoglein-2 (DSG2) gene: p. Arg49His. Comparison between size and localization of ventricular dyskinesia at cardiovascular imaging and the surface 12 lead electrocardiography are proposed. CONCLUSIONS: ARVC may lead to an extreme phenotypic variability in clinical manifestations even within patients coming from the same family in which ARVC is caused by the same genetic mutation. ECG progression over time reflects disease evolution and in particular cases may anticipate wall motion abnormalities by years.

Arrhythmogenic right ventricular cardiomyopathy: ECG progression over time and correlation with long-term follow-up.

AIMS: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease primarily affecting the right ventricle and potentially causing sudden death in young people. Our aims are to analyse the progression over time of electrocardiographic (ECG) findings and to investigate their prognostic impact. METHODS: Sixty-eight patients (69% men; age 31 ±â€Š19 years) with ARVC diagnosis were followed up for a mean of 17 ±â€Š8 years. Follow-up included baseline ECG, 24-h Holter ECG, signal-averaged ECG, stress test, echocardiography, cardiac magnetic resonance and electrophysiologic study. RESULTS: During follow-up 12 (18%) patients died: three of sudden cardiac death (SCD), four of end-stage heart failure and five of noncardiac causes. Aborted SCD occurred in 7 (10%) patients, syncope in 31 (46%), sustained ventricular tachycardia in 43 (63%), heart failure in 18 (26%), atrial fibrillation in 16 (24%) and 3 (4%) patients underwent heart transplant. Twenty-four (35%) patients had implantable cardiac defibrillator (15 and 5 of them received both appropriate and inappropriate interventions, respectively and 7 experienced device-related complications). Of the ECG parameters registered at the enrolment, left anterior fascicular block (P = 0.001), QRS duration in lead 1 (P < 0.001), Epsilon wave (P < 0.001), T wave inversion in V4-V5-V6 (P = 0.012, P = 0.001 and P = 0.006) and low QRS voltages (P = 0.001) progressed over time. At multivariate analysis Epsilon wave (odds ratio 20.9, confidence interval 95% 1.8-239.8, P = 0.015) was the only predictor of the composite endpoint of SCD, heart failure-related death or heart transplant. CONCLUSION: Apart from playing a pivotal role in ARVC diagnosis, a simple ECG feature such as Epsilon wave is a marker of poor prognosis.

Short QT Syndrome: a familial cause of sudden death.

BACKGROUND: A prolonged QT interval is associated with a risk for life-threatening events. However, little is known about prognostic implications of the reverse-a short QT interval. Several members of 2 different families were referred for syncope, palpitations, and resuscitated cardiac arrest in the presence of a positive family history for sudden cardiac death. Autopsy did not reveal any structural heart disease. All patients had a constantly and uniformly short QT interval at ECG. METHODS AND RESULTS: Six patients from both families were submitted to extensive noninvasive and invasive work-up, including serial resting ECGs, echocardiogram, cardiac MRI, exercise testing, Holter ECG, and signal-averaged ECG. Four of 6 patients underwent electrophysiological evaluation including programmed ventricular stimulation. In all subjects, a structural heart disease was excluded. At baseline ECG, all patients exhibited a QT interval

Prevalence of type 1 brugada electrocardiographic pattern evaluated by twelve-lead twenty-four-hour holter monitoring.

Patients with drug-induced type 1 Brugada electrocardiograms (BrECGs) are considered to have good prognosis. Spontaneous type 1 is, instead, considered a risk factor; however, it is probably underestimated because of the BrECG fluctuations. The aim of this study was to analyze, in a large population of patients with Br, the real prevalence of type 1 BrECG using 12-lead 24-hour Holter monitoring (12L-Holter) and its correlation with the time of the day. We recorded 303 12L-Holter in 251 patients. Seventy-five (30%) patients exhibited spontaneous type 1 BrECG at 12-lead ECG (group 1) and 176 (70%) had only drug-induced type 1 (group 2). Type 1 BrECG was defined as "persistent" (>85% of the recording), "intermittent" (<85%), or "absent." In group 1, 12% showed persistent type 1 at 12L-Holter, 57% intermittent type 1%, and 31% never had type 1; in group 2, none had persistent type 1, 20% had intermittent type 1%, and 80% never showed type 1. To evaluate the circadian fluctuations of BrECG, 4 periods in the day were considered. Type 1 BrECG was more frequent between 12-noon and 6 p.m. (52%, p <0.001). In conclusion, in patients with drug-induced type 1, spontaneous type 1 BrECG can be detected more frequently with 12L-Holter than with conventional follow-up with periodic ECGs and this has important implications in the risk stratification. 12L-Holter recording might avoid 20% of the pharmacological challenges with sodium channel blockers, which are not without risks, and should thus be considered as the first screening test, particularly in children or in presence of borderline diagnostic basal ECG.

Percutaneous catheter cryothermal ablation of atrioventricular nodal reentrant tachycardia: efficacy and safety of a new ablation technique.

BACKGROUND: Radiofrequency catheter ablation is nowadays a widely used technique for the treatment of arrhythmias; however, due to the possible complications such as atrioventricular block when radiofrequency is delivered in the septal area, this type of energy is not optimal. In contrast, cryoenergy has several positive features; first of all, it allows for the creation of reversible lesions and hence to test the effects of the ablation while the lesion is still forming thus reducing the number of ineffective and useless lesions. In addition, it also allows for the evaluation of the acute effects on the structures adjacent to the ablation site. The aim of the present study was to analyze the effectiveness and safety of catheter cryoablation in the treatment of atrioventricular nodal reentrant tachycardia (AVNRT). METHODS: Thirty-two patients presenting with AVNRT underwent catheter cryoablation using a 7F catheter. When the optimal parameters were recorded, "ice mapping" at -30 degrees C was performed for 80 s to validate the ablation site by means of a reversible lesion. If the expected result was achieved, the cryoablation was carried out lowering the temperature to -75 degrees C for 4 min thus creating a permanent lesion. RESULTS: Slow pathway ablation guided by a slow pathway potential was successfully performed in 31 out of the 32 patients with a mean of 2.6 +/- 1.0 cryoapplications. No complications occurred in any patients. Transient AH prolongation was observed in 2 patients in a midseptal site during the ice mapping phase of AVNRT ablation. CONCLUSIONS: Cryoablation is a safe and effective technique for AVNRT ablation. It may be useful particularly when the ablation must be performed close to the atrioventricular node or His bundle, due to the possibility of validating the site of ablation by means of ice mapping that creates only a reversible lesion.

Atrial fibrillation in a large population with Brugada electrocardiographic pattern: prevalence, management, and correlation with prognosis.

BACKGROUND: A high prevalence of atrial fibrillation/atrial flutter (AF/AFl) has been reported in small series of Brugada patients, with discordant data. OBJECTIVE: The purpose of this study was to analyze, in a large population of Brugada patients, the prevalence of AF/AFl, its correlation with prognosis, and the efficacy of hydroquinidine (HQ) treatment. METHODS: Among 560 patients with Brugada type 1 ECG (BrECG), 48 (9%) had AF/AFl. Three groups were considered: 23 patients with BrECG pattern recognized before AF/AFl (group 1); 25 patients first diagnosed with AF/AFl in whom Class IC antiarrhythmic drugs administered for cardioversion/prophylaxis unmasked BrECG (group 2); and 512 patients without AF/AFl (group 3). Recurrence of AF/AFl and occurrence of ventricular arrhythmias were evaluated at follow-up. RESULTS: Mean age was 47 ± 15 years, 59 ± 11 years, and 44 ± 14 years in groups 1, 2, and 3, respectively. Seven subjects (32%) in group 1 had syncope/aborted sudden death, 1 (4%) in group 2, and 122 (24%) in group 3. Ventricular arrhythmia occurred in three patients in group 1, none in group 2, and 10 in group 3 at median follow-up of 51, 68, and 41 months, respectively. Nine patients in group 1 and nine in group 2 received HQ for AF/AFl prophylaxis; on therapy, none had AF/AFl recurrence. CONCLUSION: Prevalence of AF/AFl in Brugada patients is higher than in the general population of the same age. Patients in group 1 are younger than those in group 2 and have a worse prognosis compared to both groups 2 and 3. HQ therapy has proved useful and safe in patients with AF/AFl and BrECG.

Safety and efficacy of cryoablation of accessory pathways adjacent to the normal conduction system.

UNLABELLED: Cryoablation of Septal Accessory Pathways. INTRODUCTION: Catheter ablation has become a routine treatment for patients with Wolff-Parkinson-White syndrome because of its low risk and high efficacy; however, radiofrequency ablation in the septum close to the AV node or His bundle still carries a definite risk for AV block. Cryoenergy catheter ablation has recently become available. This technique has specific features, such as the ability to create reversible loss of function to predict the effects of ablation (ice mapping) and the adherence of the catheter tip to the endocardium with freezing, which avoids the risk for dislodgment. Both of these characteristics may minimize the risk of complications. The aim of this study was to analyze the effectiveness and safety of catheter cryoablation in 20 patients with para-Hisian or midseptal accessory pathways (AP). METHODS AND RESULTS: Eleven patients with para-Hisian and 9 patients with midseptal AP underwent catheter cryoablation. Ice mapping at -30 degrees C was performed to ascertain the disappearance of AP conduction and the absence of impairment of AV nodal conduction. If the expected result was obtained, cryoablation was performed by lowering the temperature to -75 degrees C for 4 minutes in order to create a permanent lesion. Cryoablation was successful in all patients using a mean of 1.2 +/- 0.4 applications. Recurrences occurred in 4 patients (20%) who underwent a second successful cryoablation session. No complications were observed. CONCLUSION: Cryoablation appears to be a safe and effective technique for ablation of APs close to the AV node or His bundle because of the ability to predict the acute effects of ablation with ice mapping before creation of an irreversible lesion.