Jens Czapla

Implantable Cardioverter-Defibrillator Therapy in Brugada Syndrome: A 20-Year Single-Center Experience

BACKGROUND Patients with Brugada syndrome and aborted sudden cardiac death or syncope have higher risks for ventricular arrhythmias (VAs) and should undergo implantable cardioverter-defibrillator (ICD) placement. Device-based management of asymptomatic patients is controversial. ICD therapy is associated with high rates of inappropriate shocks and device-related complications. OBJECTIVES The objective of this study was to investigate clinical features, management, and long-term follow-up of ICD therapy in patients with Brugada syndrome. METHODS Patients presenting with spontaneous or drug-induced Brugada type 1 electrocardiographic findings, who underwent ICD implantation and continuous follow-up at a single institution, were eligible for this study. RESULTS A total of 176 consecutive patients were included. During a mean follow-up period of 83.8 ± 57.3 months, spontaneous sustained VAs occurred in 30 patients (17%). Eight patients (4.5%) died. Appropriate ICD shocks occurred in 28 patients (15.9%), and 33 patients (18.7%) had inappropriate shocks. Electrical storm occurred in 4 subjects (2.3%). Twenty-eight patients (15.9%) experienced device-related complications. In multivariate Cox regression analysis, aborted sudden cardiac death and VA inducibility on electrophysiologic studies were independent predictors of appropriate shock occurrence. CONCLUSIONS ICD therapy was an effective strategy in Brugada syndrome, treating potentially lethal arrhythmias in 17% of patients during long-term follow-up. Appropriate shocks were significantly associated with the presence of aborted sudden cardiac death but also occurred in 13% of asymptomatic patients. Risk stratification by electrophysiologic study may identify asymptomatic patients at risk for arrhythmic events and could be helpful in investigating syncope not related to VAs. ICD placement is frequently associated with device-related complications, and rates of inappropriate shocks remain high regardless of careful device programming.

A score model to predict risk of events in patients with Brugada Syndrome

Aims Risk stratification in Brugada Syndrome (BS) remains challenging. Arrhythmic events can occur life-long and studies with long follow-ups are sparse. The aim of our study was to investigate long-term prognosis and risk stratification of BS patients. Methods and results A single centre consecutive cohort of 400 BS patients was included and analysed. Mean age was 41.1 years, 78 patients (19.5%) had a spontaneous type I electrocardiogram (ECG). Clinical presentation was aborted sudden cardiac death (SCD) in 20 patients (5.0%), syncope in 111 (27.8%) and asymptomatic in 269 (67.3%). Familial antecedents of SCD were found in 184 individuals (46.0%), in 31 (7.8%) occurred in first-degree relatives younger than 35 years. An implantable cardioverter defibrillator (ICD) was placed in 176 (44.0%). During a mean follow-up of 80.7 months, 34 arrhythmic events occurred (event rate: 1.4% year). Variables significantly associated to events were: presentation as aborted SCD (Hazard risk [HR] 20.0), syncope (HR 3.7), spontaneous type I (HR 2.7), male gender (HR 2.7), early SCD in first-degree relatives (HR 2.9), SND (HR 5.0), inducible VA (HR 4.7) and proband status (HR 2.1). A score including ECG pattern, early familial SCD antecedents, inducible electrophysiological study, presentation as syncope or as aborted SCD and SND had a predictive performance of 0.82. A score greater than 2 conferred a 5-year event probability of 9.2%. Conclusions BS patients remain at risk many years after diagnosis. Early SCD in first-degree relatives and SND are risk factors for arrhythmic events. A simple risk score might help in the stratification and management of BS patients.

Midterm clinical outcomes of concomitant thoracoscopic epicardial and transcatheter endocardial ablation for persistent and long-standing persistent atrial fibrillation: a single-centre experience.

Aims The purpose of this study was to analyse the efficacy and complication rates of the simultaneous hybrid procedure in a series of patients with persistent and long-standing persistent atrial fibrillation (AF) in a midterm follow-up. Methods and results Sixty-four consecutive patients (56 males, 59.7 ± 8.7 years) having undergone isolation of pulmonary veins (PVs) and posterior wall of left atrium (LA) by means of hybrid thoracoscopic ablation for symptomatic persistent (n = 21, 33%) and long-standing persistent AF (n = 43, 67%) were analysed. At a mean follow-up of 23.1 ± 14.1 months (median 21; range 6–57), the success rate without antiarrhythmic therapy was achieved in 67.2% of patients. Procedure-related complications were observed in 13 patients (20.3%) including 2 LA perforations (3.1%) requiring, respectively, conversion to sternotomy and small left-sided thoracotomy. The success rate did not significantly differ between persistent and long-standing persistent AF (respectively, 71.4 and 65.1%; P = 0.4). Patients with AF relapse during the blanking period were 4.60 times more likely to have AF recurrence after 3 months from the ablation procedure. Conclusion The hybrid procedure yields promising results in the setting of both persistent and long-standing persistent AF after midterm follow-up, at the expense of a non-negligible rate of adverse events. Our findings need to be confirmed by further larger and prospective studies.

Measuring resting energy expenditure during extracorporeal membrane oxygenation: preliminary clinical experience with a proposed theoretical model.

Extracorporeal membrane oxygenation (ECMO) is increasingly used in patients with severe respiratory failure. Indirect calorimetry (IC) is a safe and non‐invasive method for measuring resting energy expenditure (REE). No data exist on the use of IC in ECMO‐treated patients as oxygen uptake and carbon dioxide elimination are divided between mechanical ventilation and the artificial lung. We report our preliminary clinical experience with a theoretical model that derives REE from IC measurements obtained separately on the ventilator and on the artificial lung.

Long-Term Performance of the Riata/ST Implantable Cardioverter-Defibrillator Lead

Riata and Riata ST implantable cardioverter-defibrillator leads are prone to structural and electrical failure (EF). Our objective was to evaluate Riata/ST lead performance over a long-term follow-up. Of 184 patients having undergone Riata/ST and Riata ST Optim lead implantation from September 2003 to June 2008, 154 patients were evaluated for EF and radiographic conductor externalization (CE). Survival analysis for EF was performed for Riata/ST leads, both for failure-free lead survival and cumulative hazard. Subanalysis on 7Fr leads was performed to evaluate EF and CE rates both for different Riata ST lead management (monitoring vs proactive) and between Riata ST and Riata ST Optim leads. During a mean follow-up of 7 years, Riata/ST lead EF rate was 13% overall. Similar failure-free survival rate was noted for 7Fr as for 8Fr leads (log-rank, p = 0.63). Of all failed leads, 64% failed only after 5 years of follow-up. Compared with the absolute failure rate of 1.84% per device year, cumulative hazard analysis for leads surviving past 5 years revealed an estimated failure rate of 7% per year. No clinical or procedural predictors for EF were found. The subanalysis on 7Fr leads showed an excellent outcome both for a proactive lead management approach as for Optim leads. In conclusion, long-term survival of the Riata/ST lead is impaired with an accelerating EF risk over time. An initial exponential trend was followed by a linear lead failure pattern for leads surviving past 5 years, corresponding to an estimated 7% annual EF rate. These findings may have repercussions on the lead management strategy in patients currently surviving with a Riata/ST lead to prevent significant clinical events like inappropriate shocks or failed device interventions.

Unmasking a naked left atrial appendage closure device: A case of a silent embolic threat

Patients with atrial fibrillation (AF) have a higher risk for thromboembolic events, for which oral anticoagulation is the first choice of therapy. For patients in whom anticoagulation is contraindicated, new therapeutic approaches are needed. Recent studies have shown the noninferiority of percutaneous closure of the left atrial appendage (LAA) compared to oral anticoagulation. Therefore, these devices can be a potential alternative therapy for preventing thromboembolic complications in patients with AF. However, with intracardiac devices the amount of foreign material can cause thrombus formation. Here we describe a case of intraoperative discovery of a thrombus on an Amplatzer cardiac plug. The thrombus was not visible during peroperative transesophageal echocardiography.

Implantable cardioverter defibrillator therapy in young individuals: comparison of conventional and subcostal approaches-a single-centre experience.

Aim The aim of our study is to compare two approaches of implantable cardiac defibrillator (ICD) implantation, conventional (supra/subpectoral) and subcostal in young adults in terms of procedural complications and adverse events encountered during follow-up. Methods and results From January 2007 to December 2013, all patients under the age of 50 years who received an ICD in our centre were included in this study. Patients hospital records were analysed for procedural complications and adverse events during follow-up until December 2014. Data from device on first interrogation after implantation and on follow-up were also noted. A total of 106 patients of which 40.6% had Brugadas syndrome (65.1% male, age 33.6 ± 10.97 years) were included in analysis; 71 (61%) had ICD placed in (sub/supra) pectoral and 35 (33%) in subcostal position. Only seven patients received an epicardial lead system. During the follow-up period of 2.1 ± 1.8 years, 84.90% of the patients had no adverse events. Most of the complications, procedural and during follow-up, occur in conventionally placed, pectoral ICD. Lead follow-up data in both groups, conventional and subcostal, showed no difference in right ventricular (RV) shock impedance and R wave sensing, P-value = 0.56 and 0.77, respectively. Lead survival was 95 and 97%, respectively, in conventional and subcostal groups over a mean follow-up of 2.1 ± 1.8 years. Log-rank test for lead survival was not significant in terms of site of implantation. Conclusion To the best our knowledge, this is the first study demonstrating subcostal ICD placement in young adults and resulting in equivalent to better outcomes when compared with conventionally placed pectoral ICD. Subcostal ICD placement might be considered an alternative option in young adults as it results in better procedural outcomes and also comparable rate of adverse events during follow-up, but bigger studies with a larger number of patients are needed for a definitive conclusion.

Concomitant Brugada syndrome substrate ablation and epicardial abdominal cardioverter-defibrillator implantation in a child

Introduction Brugada syndrome (BrS) is a primary electrical disease of autosomal dominant inheritance, characterized by covedtype ST-segment elevation in the right precordial leads and increased risk of sudden cardiac death. Although the initial description included 3 children in a series of 8 patients, the prevalence of BrS in pediatric populations was extremely low (0.0098%) in subsequent studies compared to adults in the fourth or fifth decade of life (range 0.14%–0.7%). We report the case of a 3-year-old boy with highly symptomatic BrS, focusing on the feasibility and safety of combined epicardial substrate ablation of the right ventricular outflow tract (RVOT) and implantation of an epicardial implantable cardioverter-defibrillator (ICD).

Coronary artery bypass surgery in a patient with congenital absence of the left pericardium

Figure 1: Intra-operative view. Right pericardium (A). Congenital absence of the left pericardium (prevalence between 1 in 10 000 and 1 in 14 000) (B). Notice the rotation of the heart to the left pleural cavity. Figure 2: Intra-operative view. Right pericardium (A). Right ventricle (B). Left lung (C). Complete absence of the left pericardium. Aortovenous bypass (grafted onto two obtuse marginal artery branches and on the posterior descending artery) (D). Left internal thoracic artery (grafted on the LAD) (E).