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.

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. Patient's 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 Brugada's 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.

Repeat Procedures After Hybrid Thoracoscopic Ablation in the Setting of Longstanding Persistent Atrial Fibrillation: Electrophysiological Findings and 2-Year Clinical Outcome.

INTRODUCTION: In order to increase success rates of invasive treatment of persistent atrial fibrillation, the hybrid approach was developed, combining video-assisted thoracoscopic epicardial procedure with conventional endocardial catheter ablation. Currently, there are no reports of electrophysiological findings and clinical outcomes of repeat procedures after the hybrid approach. METHODS AND RESULTS: Out of 64 patients who were treated by hybrid ablation for persistent atrial fibrillation (AF), 14 underwent the repeat catheter ablation and were selected for this study. All 14 patients initially presented with longstanding persistent atrial fibrillation and markedly dilated atria. The hybrid procedure was performed in a single act and the mean time to redo procedure was 346 ± 227 days. In 57% of patients indication for redo procedure was regular atrial tachycardia, and the rest presented with recurrent atrial fibrillation. In 36% of patients, recovered conduction was found along the previous ablation lesions. Only 9% of pulmonary veins were reconnected (0.36 veins per patient) and 7% of box lesions were not complete. The overall success rate at 2 years follow-up after the repeat procedure, including second repeat procedure and patients taking antiarrhythmic drugs, was 64% (57% without drugs and further ablation). One case of moderate pulmonary vein stenosis was detected as a consequence of hybrid procedure. CONCLUSION: Hybrid atrial fibrillation ablation results in durable lesions and high rates of chronic pulmonary vein isolation even after long-term follow-up. Most of the repeat procedures after the hybrid approach are related to left atrial flutters that could be successfully treated by catheter ablation.

Serum S100B protein could help to detect cerebral complications associated with extracorporeal membrane oxygenation (ECMO).

BACKGROUND: To investigate if serum S100B protein levels could early detect cerebral complications under treatment extracorporeal membrane oxygenation (ECMO). METHODS: Serum S100B levels were measured over 5 days in 32 patients with cardiogenic and septic shock, including 15 patients who treated by ECMO and 17 who did not. Cerebral complications included hemorrhage, stroke, encephalopathy with myoclonus, and brain death. Delirium was identified by the positive Confusion Assessment Method in the ICU. RESULTS: S100B levels were elevated in 24/32 patients (75 %) at ICU admission. Five patients developed cerebral complications (2 hemorrhages with 1 brain death, 1 encephalopathy with myoclonus in the ECMO group and 2 strokes in the non-ECMO group). At day 5, S100B levels were higher in the 5 patients with cerebral complications than in the 27 without cerebral complications, regardless of ECMO (0.426 [0.421, 0.652] vs. 0.102 [0.085, 0.135] µg/L, p = 0.011). S100B levels were also more elevated in 3 patients with than in 12 without cerebral complications associated with ECMO (0.799 [0.325, 0.965] vs. 0.102 [0.09, 0.607] µg/L, p = 0.033). S100B levels were not associated with delirium after sedation withdrawal. CONCLUSIONS: Measurement serum S100B could be useful to detect cerebral complications in deeply sedated patients associated with ECMO but not for monitoring delirium after sedation withdrawal.

Complete epicardial resynchronization device implantation in a patient who underwent a replacement of mitral and tricuspid valve.

A 71-year-old woman with severe nonischemic dilated cardiomyopathy and low ejection fraction with severe mitral regurgitation and tricuspid regurgitation and pulmonary hypertension underwent multiple valve repairs and cardiac resynchronization therapy implantation with epicardial shock leads.

A chart review on surgical myocardial debridging in symptomatic patients: a safe procedure with good long-term clinical outcome and coronary computed tomographic angiography results.

OBJECTIVES: Myocardial bridging is mostly diagnosed as an incidental imaging finding but can result in severe vessel compression and significant clinical adverse complications. Since there is still an ongoing debate when to propose surgical unroofing, we studied a group of patients where this was performed as an isolated procedure. METHODS: In 16 patients (38.9 ± 15.7 years, 75% men) who had surgical unroofing for symptomatic isolated myocardial bridges of the left anterior descending artery, we retrospectively analysed symptomatology, medication, imaging modalities used, operative techniques, complications and long-term outcome. Computed tomographic fractional flow reserve was calculated to understand its potential value for decision-making. RESULTS: Most procedures were performed on-pump (75%, mean cardiopulmonary bypass 56.5 ± 27.9 min, mean aortic cross-clamping 36.4 ± 19.7 min). Three patients needed a left internal mammary artery bypass since the artery dived inside the ventricle. There were no major complications or deaths. The mean follow-up was 5.5 years. Although there was a dramatic improvement in symptoms, still 31% experienced atypical chest pain at various moments during follow-up. Postoperative radiological control was performed in 88%, showing no residual compression or recurrent myocardial bridge and patent bypass if performed. All postoperative computed tomographic flow calculations (7) showed a normalization of coronary flow. CONCLUSIONS: Surgical unroofing for symptomatic isolated myocardial bridging is a safe procedure. Patient selection remains difficult but introducing standard coronary computed tomographic angiography with flow calculations could be helpful in preoperative decision-making and during follow-up.

Endoscopic mitral and tricuspid valve surgery after previous cardiac surgery.

BACKGROUND: The purpose of this study was to evaluate the feasibility and effectiveness of a right video-assisted approach for atrioventricular valve disease after previous cardiac surgery. METHODS AND RESULTS: Between December 1st 1997 and May 1st 2006, 80 adults (mean age 65+/-12 years; 56% female) underwent reoperative surgery using a video-assisted approach without rib spreading. Previous cardiac operations included mitral valve (39%), CABG (29%), congenital (10%), and other (23%). For 25% of patients, this was at least their third cardiac operation. Mean time to redo surgery was 15+/-12 years. Femoral vessel cannulation and endoaortic clamping were routinely used. Mean preoperative Euroscore was 9.0+/-2.7 (5 to 20) and predicted mortality was 16.0+/-14.2% (4 to 86). Median preoperative NYHA class was II and mean follow-up was 25+/-22 months. Lung adhesions necessitated sternotomy in 4 cases and cannulation problems in another patient. Total operative mortality was 3.8% (n=3), O/E for mortality being 0.24. Procedures were mitral valve repair (45%; n=36), replacement (50%; n=40) and tricuspid valve replacement (5%; n=4). Additional procedures were performed in 44% (n=35). Mean aortic crossclamp and procedure time were 92+/-37 and 267+/-64 minutes. Mean postoperative blood loss was 815+/-1083 mL. Postoperative morbidity included 2 strokes (2.5%). Mean hospital stay was 10.7+/-6.7 days. Survival at 1 and 4 years was 93.6+/-2.8% and 85.6+/-6.4%. There was 1 late reoperation at 5 years. Median NYHA class at follow-up was II. When comparing, all but 1 patient (98.8%) preferred their minimally invasive approach when considering perioperative pain, postoperative rehabilitation, and final esthetic result. CONCLUSIONS: Video-assisted minimal access correction of atrioventricular valve disease after previous cardiac surgery is not only feasible but had lower than predicted mortality and strong patient satisfaction. It should therefore be used more frequently in today's practice.

Surgical treatment of Wolff-Parkinson-White syndrome: a timeless procedure?

Catheter-induced radiofrequency (RF) ablation has become the initial non-pharmacological treatment option for Wolff-Parkinson-White (WPW) syndrome. In this report, we present the successful surgical treatment of WPW syndrome in two patients in whom percutaneous ablation of the accessory pathway was not successful.

Long-term prognosis of drug-induced Brugada syndrome.

BACKGROUND: Patients with drug-induced Brugada syndrome (BS) are considered at a lower risk than those with a spontaneous type I pattern. Nevertheless, they can present arrhythmic events. OBJECTIVE: The purpose of this study was to investigate their clinical characteristics, long-term prognosis and risk factors. METHODS: A consecutive cohort of 343 patients with drug-induced BS was included and compared with 78 patients with a spontaneous type I pattern. RESULTS: The mean age was 40.7 ± 18.3 years. Sudden cardiac death (SCD) was the clinical presentation in 13 (3.8%) and syncope in 86 (25.1%); 244 (71.1%) were asymptomatic. Patients with drug-induced BS were less frequently men (180 (52.5%) vs 63 (80.8%); P < .01), were more frequently asymptomatic (244 (71.1%) vs 44 (56.4%); P < .01), and had less ventricular arrhythmias (VAs) induced during electrophysiology study (41 (13.2%) vs 31 (42.4%); P < .01). An implantable cardioverter-defibrillator was implanted in 128 patients (37.3%). During a median follow-up of 62.5 months (interquartile range 28.9-115.6 months), 34 patients presented arrhythmic events. The event rate was 1.1% person-year (vs 2.3% person-year in patients with a spontaneous type I pattern; P < .01). Presentation as SCD and inducible VAs were independent risk factors significantly associated with arrhythmic events (adjusted hazard ratio 22.0 and 3.5). Drug-induced BS was related to a better prognosis only in asymptomatic individuals. CONCLUSION: Drug-induced BS has a good prognosis if asymptomatic; however, SCD is possible. Clinical presentation as SCD and inducible VAs during electrophysiology study are independent risk factors for arrhythmic events. In asymptomatic patients, proband status and inducible VAs can help to identify patients at higher risk, but further evidence is needed.

Hybrid revascularization strategy: a pilot study on the association of robotically enhanced minimally invasive direct coronary artery bypass surgery and fractional-flow-reserve-guided percutaneous coronary intervention.

BACKGROUND: Robotically enhanced minimally invasive direct coronary artery bypass (RE-MIDCAB) graft of the left internal mammary artery to the left anterior descending coronary artery (LAD) and/or the first diagonal branch might be the least traumatic surgical revascularization approach available so far. When combined with fractional flow reserve (FFR)-guided percutaneous coronary intervention (PCI) in the non-LAD vessels, this "hybrid" strategy takes advantage of the survival benefit conferred by the internal mammary artery graft to the LAD while providing the patients with a truly minimally invasive, functionally complete revascularization. METHODS AND RESULTS: Twenty patients with multivessel disease were selected to undergo combined PCI and RE-MIDCAB because they had a lesion amenable to PCI in the right and/or the left circumflex coronary artery and a lesion in the LAD and/or the first diagonal branch that was considered less than ideal for PCI. PCI was actually performed only when FFR was <0.80 ("provisional PCI"). In 7 stenoses, FFR was >0.80 and the planned PCI was not performed. Surgery was performed before provisional PCI in 6 cases. An angiogram was obtained in all patients before discharge, and a complete clinical follow-up including a stress test was obtained in all patients after a mean of 12 months. There were no significant intraoperative complications, conversions to cardiopulmonary bypass, or reinterventions for bleeding. At early control angiogram, 2 moderate stenoses just proximal to anastomosis were observed, both with normal run-off. After 12 months there were no objective signs of ischemia at stress testing. After an average follow-up of 19+/-10 months there were no deaths, myocardial infarctions, or repeat revascularizations. CONCLUSIONS: A hybrid strategy combining FFR-guided PCI and RE-MIDCAB seems safe and provides selected patients with a functionally complete revascularization with minimal surgical trauma and excellent clinical outcomes.

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.

Clinical characterisation and long-term prognosis of women with Brugada syndrome.

OBJECTIVES: Brugada syndrome (BS) in women is considered an infrequent condition with a more favourable prognosis than in men. Nevertheless, arrhythmic events and sudden cardiac death (SCD) also occur in this population. Long-term follow-up data of this group are sparse. The purpose of the present study was to investigate the clinical characteristics and long-term prognosis of women with BS. METHODS: A consecutive cohort of 228 women presenting with spontaneous or drug-induced Brugada type I ECG at our institution were included and compared with 314 men with the same diagnosis. RESULTS: Mean age was 41.5±17.3 years. Clinical presentation was SCD in 6 (2.6%), syncope in 51 (22.4%) and the remaining 171 (75.0%) were asymptomatic. As compared with men, spontaneous type I ECG was less common (7.9% vs 23.2%, p<0.01) and less ventricular arrhythmias were induced during programmed electrical stimulation (5.5% vs 22.3%, p<0.01). An implantable cardioverter defibrillator (ICD) was implanted in 64 women (28.1%). During a mean follow-up of 73.2±56.2 months, seven patients developed arrhythmic events, constituting an event rate of 0.7% per year (as compared with 1.9% per year in men, p=0.02). Presentation as SCD or sinus node dysfunction (SND) was risk factor significantly associated with arrhythmic events (hazard risk (HR) 25.4 and 9.1). CONCLUSION: BS is common in women, representing 42% of patients in our database. Clinical presentation is less severe than men, with more asymptomatic status and less spontaneous type I ECG and prognosis is more favourable, with an event rate of 0.7% year. However, women with SCD or previous SND are at higher risk of arrhythmic events.

Long-Term Trends in Newly Diagnosed Brugada Syndrome: Implications for Risk Stratification.

BACKGROUND: A proband of Brugada syndrome (BrS) is the first patient diagnosed in a family. There are no data regarding this specific, high-risk population. OBJECTIVES: This study sought to investigate the Brugada probands diagnosed from 1986 through the next 28 years. METHODS: We included 447 probands belonging to families with a diagnostic type 1 electrocardiogram Brugada pattern. The database was divided into 2 periods: the first period identified patients who were part of the initial cohort that became the consensus document on BrS in 2002 (early group); the second period reflected patients first diagnosed from 2003 to January 2014 (latter group). RESULTS: There were 165 probands in the early group and 282 in the latter group. Aborted sudden death as the first manifestation of the disease occurred in 12.1% of the early group versus 4.6% of the latter group (p = 0.005). Inducibility during programmed electrical stimulation was achieved in 34.4% and 19.2% of patients, respectively (p < 0.001). A spontaneous type 1 electrocardiogram pattern at diagnosis was present in 50.3% early versus 26.2% latter patients (p = 0.0002). Early group patients had a higher probability of a recurrent arrhythmia during follow-up (19%) than those of the latter group (5%) (p = 0.007). The clinical suspicion and use of a sodium-channel blocker to unmask BrS has allowed earlier diagnoses in many patients. CONCLUSIONS: Since being first described, the presentation of BrS has changed. There has been a decrease in aborted sudden cardiac death as the first manifestation of the disease among patients who were more recently diagnosed. These variations in initial presentation have important clinical consequences. In this setting, the value of inducibility to stratify individuals with BrS has changed.

Mitral valve surgery can now routinely be performed endoscopically.

BACKGROUND: There is an increasing interest in minimally invasive cardiac surgery. METHODS AND RESULTS: Since February 1, 1997 till April 1, 2002, 306 patients underwent endoscopic mitral valve surgery (226 repair, MVP; 80 replacement, MVR). Predominant valve pathology was degenerative in MVP (83.6%) and rheumatic in MVR (65%). Mean age was 61.5+/-12.9 years. Median preoperative functional class (MVP+MVR) and mitral regurgitation (MVP) were II and 4+. Statistical analysis included Kaplan-Meier and Cox regression methods. Mean follow-up was 19.6+/-17.3 months and complete. The procedure was successfully performed in all but 6 patients. Hospital mortality included 3 patients (1%) and was technology related in one. Postoperative morbidity included aggressive re-exploration (8.5%), new onset atrial fibrillation (17.0%), and pacemaker implantation (2.3%). There were 1 early and 10 late reoperations, 5 of which were because of endocarditis. Freedom from mitral valve reoperation at 4 years was 91+/-3.5%. No risk factors for reoperation could be detected. Echocardiographic follow-up showed a median degree of mitral regurgitation (MVP) of 0 and a small paravalvular leak in four patients (MVR). Ninety-four percent of the patients reported no or mild postoperative pain and 99.3% felt they had an esthetically pleasing scar. Ninety-three percent would choose the same procedure again and 46.1% were back at work within 4 weeks. CONCLUSIONS: Endoscopic mitral valve surgery can be performed safely but definitely requires a learning curve. Good results and a high patient satisfaction are guaranteed. It is now our exclusive approach for isolated atrioventricular valve disease.

The effect of completeness of revascularization on event-free survival at one year in the ARTS trial.

OBJECTIVES: We sought to assess the relationship between completeness of revascularization and adverse events at one year in the ARTS (Arterial Revascularization Therapies Study) trial. BACKGROUND: There is uncertainty to what extent degree of completeness of revascularization, using up-to-date techniques, influences medium-term outcome. METHODS: After consensus between surgeon and cardiologist regarding the potential for equivalence in the completeness of revascularization, 1,205 patients with multivessel disease were randomly assigned to either bypass surgery or stent implantation. All baseline and procedural angiograms and surgical case-record forms were centrally assessed for completeness of revascularization. RESULTS: Of 1,205 patients randomized, 1,172 underwent the assigned treatment. Complete data for review were available in 1,143 patients (97.5%). Complete revascularization was achieved in 84.1% of the surgically treated patients and 70.5% of the angioplasty patients (p < 0.001). After one year, the stented angioplasty patients with incomplete revascularization showed a significantly lower event-free survival than stented patients with complete revascularization (i.e., freedom from death, myocardial infarction, cerebrovascular accident and repeat revascularization) (69.4% vs. 76.6%; p < 0.05). This difference was due to a higher incidence of subsequent bypass procedures (10.0% vs. 2.0%; p < 0.05). Conversely, at one year, bypass surgery patients with incomplete revascularization showed only a marginally lower event-free survival rate than those with complete revascularization (87.8% vs. 89.9%). CONCLUSIONS: Complete revascularization was more frequently accomplished by bypass surgery than by stent implantation. One year after bypass, there was no significant difference in event-free survival between surgically treated patients with complete revascularization and those with incomplete revascularization, but patients randomized to stenting with incomplete revascularization had a greater need for subsequent bypass surgery.

Surgical ablation of atrial fibrillation with off-pump, epicardial, high-intensity focused ultrasound: results of a multicenter trial.

BACKGROUND: A simplified alternative to the Cox maze procedure to treat atrial fibrillation with epicardial high-intensity focused ultrasound was evaluated clinically, and the initial clinical results were assessed at the 6-month follow-up visit. METHODS: From September 2002 through February 2004, 103 patients were prospectively enrolled in a multicenter study. Atrial fibrillation duration ranged from 6 to 240 months (mean, 44 months) and was permanent in 76 (74%) patients, paroxysmal in 22 (21%) patients, and persistent in 5 (5%) patients. All patients had concomitant operations, and ablation was performed epicardially on the beating heart before the concomitant procedure. The device automatically created a circumferential left atrial ablation around the pulmonary veins in an average of 10 minutes, and an additional mitral line was created epicardially in 35 (34%) patients with a handheld device by using the same technology. RESULTS: No complications or deaths were device or procedure related. There were 4 (3.8%) early deaths and 2 late extracardiac deaths. The 6-month follow-up was complete in all survivors. At the 6-month visit, freedom from atrial fibrillation was 85% in the entire study group (80% in patients with permanent atrial fibrillation, 88% in the 35 patients who had the additional mitral line, and 100% in patients with paroxysmal atrial fibrillation). A pacemaker was implanted in 8 patients. Only the duration and type of atrial fibrillation significantly increased the risk of recurrence. CONCLUSION: Epicardial, off-pump, beating-heart ablation with acoustic energy is safe and cures 80% of patients with permanent atrial fibrillation associated with long-standing structural heart disease.

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.

Prognostic value of programmed electrical stimulation in Brugada syndrome: 20 years experience.

BACKGROUND: The prognostic value of electrophysiological investigations in individuals with Brugada syndrome remains controversial. Different groups have published contradictory data. Long-term follow-up is needed to clarify this issue. METHODS AND RESULTS: Patients presenting with spontaneous or drug-induced Brugada type I ECG and in whom programmed electric stimulation was performed at our institution were considered eligible for this study. A total of 403 consecutive patients (235 males, 58.2%; mean age, 43.2±16.2 years) were included. Ventricular arrhythmias during programmed electric stimulation were induced in 73 (18.1%) patients. After a mean follow-up time of 74.3±57.3 months (median 57.3), 25 arrhythmic events occurred (16 in the inducible group and 9 in the noninducible). Ventricular arrhythmias inducibility presented a hazard ratio for events of 8.3 (95% confidence interval, 3.6-19.4), P<0.01. CONCLUSIONS: Programmed ventricular stimulation of the heart is a good predictor of outcome in individuals with Brugada syndrome. It might be of special value to guide further management when performed in asymptomatic individuals. The overall accuracy of the test makes it a suitable screening tool to reassure noninducible asymptomatic individuals.

Asymptomatic Brugada Syndrome: Clinical Characterization and Long-Term Prognosis.

BACKGROUND: Among Brugada syndrome patients, asymptomatic individuals are considered to be at the lowest risk. Nevertheless, arrhythmic events and sudden cardiac death are not negligible. Literature focused on this specific group of patients is sparse. The purpose of this study is to investigate the clinical characteristics, management, and long-term prognosis of asymptomatic Brugada syndrome patients. METHODS AND RESULTS: Patients presenting with spontaneous or drug-induced Brugada type I ECG and no symptoms at our institution were considered eligible. A total of 363 consecutive patients (200 men, 55.1%; mean age, 40.9±17.2 years; 41 [11.3%] with spontaneous type I ECG) were included. Electrophysiological study was performed in 321 (88.4%) patients, and ventricular arrhythmias were induced in 32 (10%) patients. An implantable cardioverter defibrillator was implanted in 61 (16.8%) patients. After a mean follow-up time of 73.2±58.9 months, 9 arrhythmic events occurred, accounting for an annual incidence rate of 0.5%. Event-free survival was 99.0% at 1 year, 96.2% at 5 years, and 95.4% at 10 and 15 years. Univariate analysis identified as risk factors: electrophysiological study inducibility (hazard ratio, 11.4; P<0.01), spontaneous type I (hazard ratio, 4.0; P=0.04), and previous sinus node dysfunction (hazard ratio, 8.0; 95% confidence interval, 1.0-63.9; P=0.05). At the multivariate analysis, only inducibility remained significant (hazard ratio, 9.1; P<0.01). CONCLUSIONS: Arrhythmic events in asymptomatic Brugada syndrome patients are not insignificant. Ventricular arrhythmia inducibility, spontaneous type I ECG, and presence of sinus node dysfunction might be considered as risk factors and used to drive long-term management.

Successful treatment of acute dissection of the donor aorta after orthotopic heart transplantation.

Acute aortic dissection is one of the rare aortic complications that occur after orthotopic heart transplantation. We report the second case of successful surgical treatment of aortic dissection confined to the donor aorta in a recipient of an orthotopic cardiac allograft. A 68-year-old patient was admitted with chest pain and shortness of breath 7 years after orthotopic heart transplantation. He previously had undergone twice coronary artery bypass grafting. Echocardiography revealed acute dissection of the donor aorta. The patient underwent urgent Bentall procedure with a prosthetic conduit. The post-operative course was uneventful. The heart donor was a 40-year-old man with known arterial hypertension and who had received long-term ergotamine tartrate therapy for migraine. This case demonstrates that heart-transplant recipients with arterial hypertension and donor-related risk factors are prone to aortic complications and require careful follow-up.