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Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease characterized by fibrofatty replacement of the myocardium and ventricular arrhythmias. Biventricular involvement in ARVC may lead to heart failure. This study aimed to investigate the role of plasma biomarkers soluble (s)ST2, Galectin-3 (Gal-3) and GDF-15 in predicting biventricular involvement and adverse outcomes in ARVC.Methods and Results: ARVC patients from 2 independent cohorts, were studied. The Bejing (Chinese) cohort (n = 108) was the discovery cohort, whereas the Zurich (Swiss) cohort (n = 47) served as validation. All patients had a definite ARVC diagnosis at time of blood withdrawal. Biomarkers were independently correlated with NT-proBNP and left ventricular (LV)-function. ARVC patients with LV involvement had higher levels of sST2 and GDF-15 as compared to controls and patients with isolated right ventricle (RV) involvement. sST2 and GDF-15 were significantly correlated with late gadolinium enhancement in CMR and with adverse heart failure outcomes. Gal-3 was elevated in ARVC patients with and without LV involvement. The combined use of the three biomarkers (sST2, GDF-15 and NT-proBNP) showed the best performance in predicting LV involvement in both cohorts. Plasma drawn from the coronary arteries and coronary sinus indicated a transmyocardial elevation of sST2, but no transmyocardial gradient of GDF-15. After heart transplantation, both sST2 and GDF-15 returned to near-normal levels.Conclusion: Our study showed that sST2 and GDF-15 may predict biventricular involvement in ARVC. The combined use of sST2, GDF-15 and NT-proBNP showed the best prediction of biventricular involvement in ARVC. [ABSTRACT FROM AUTHOR]

Background: The clinical presentation of cardiac sarcoidosis (CS) may resemble that of arrhythmogenic right ventricular cardiomyopathy (ARVC).Objective: The purpose of this study was to identify clinical variables to better discriminate between patients with genetically determined ARVC and those with CS fulfilling definite 2010 ARVC Task Force Criteria (TFC).Methods: In this multicenter study, 10 patients with CS fulfilling definite 2010 ARVC TFC were age and gender matched with 10 genetically proven ARVC patients. A cardiac 18F-fluorodeoxyglucose positron emission tomographic (18F-FDG PET) scan was required for patients to be included in the study.Results: The 2010 ARVC TFC did not reliably differentiate between the 2 diseases. CS patients presented with longer PR intervals, advanced atrioventricular block (AVB), and longer QRS duration (P <.001 and P = .009, respectively), whereas T-wave inversions (TWIs) in the peripheral leads were more common in ARVC patients (P = .009). CS patients presented with more extensive left ventricular involvement and lower left ventricular ejection fraction (LVEF), whereas ARVC patients had a larger right ventricular outflow tract (RVOT) (P = .044). PET scan positivity was only present in CS patients (90% vs 0%).Conclusion: The 2010 ARVC TFC do not reliably differentiate between CS patients fulfilling 2010 ARVC TFC and those with hereditary ARVC. Prolonged PR interval, advanced AVB, longer QRS duration, right ventricular apical involvement, reduced LVEF, and positive 18F-FDG PET scan should raise the suspicion of CS, whereas larger RVOT dimensions, subtricuspid involvement and peripheral TWI favor a diagnosis of hereditary ARVC. [ABSTRACT FROM AUTHOR]

Background: Disease progression and ventricular arrhythmias (VAs) in arrhythmogenic right ventricular cardiomyopathy (ARVC) are correlated with physical exercise, and clinical detraining and avoidance of competitive sport practice are suggested for ARVC patients. An algorithm assessing primary arrhythmic risk in ARVC patients was recently developed by Cadrin-Tourigny et al. Data regarding its transferability to athletes are lacking.Objective: The purpose of this study was to assess the reliability of the Cadrin-Tourigny risk prediction algorithm in a cohort of athletes with ARVC and to describe the impact of clinical detraining on disease progression.Methods: All athletes undergoing clinical detraining after ARVC diagnosis at our institution were enrolled. Baseline and follow-up clinical characteristics and data on VA events occurring during follow-up were collected. The Cadrin-Tourigny algorithm was used to calculate the a priori predicted VA risk, which was compared with the observed outcomes.Results: Twenty-five athletes (age 36.1 ± 14.0 years; 80% male) with definite ARVC who were undergoing clinical detraining were enrolled. Over median (interquartile range) follow-up of 5.3 (3.2-6.6) years, a reduction in premature ventricular complex (PVC) burden (P = .001) was assessed, and 10 VA events (40%) were recorded. The a priori algorithm-predicted risk seemed to fit with the observed cohort arrhythmic risk [mean observed-predicted risk difference over 5 years -0.85% (interquartile range -4.8% to +3.1%); P = .85]. At 1-year follow-up, 11 patients (44%) had an improved stress ECG response, and no significant changes in right ventricular ejection fraction were observed.Conclusion: Clinical detraining is associated with PVC burden reduction in athletes with ARVC. The novel risk prediction algorithm does not seem to require any correction for its application to ARVC athletes. [ABSTRACT FROM AUTHOR]

Aim: The impact of clinical characteristics for predicting patterns of ventricular involvement in arrhythmogenic right ventricular cardiomyopathy (ARVC) are not well defined. The aims of this study were to characterize different patterns of ventricular involvement in patients with ARVC and to stratify them based on clinical characteristics exercise and underlying genetic mutations.Methods: Sixty-four patients with definite ARVC from the Swiss ARVC Registry were enrolled. Right and left ventricular functions were assessed at baseline and most recent follow-up. All patients received genetic testing. Serum high-sensitivity cardiac Troponin T (hs-cTNT) and N-terminal of pro-brain natriuretic peptide (NT-proBNP) were determined at baseline.Results: Thirty-five patients (55%) had isolated right ventricular (RV) involvement, 12 patients (19%) had biventricular (BiV) involvement at baseline and 17 patients (26%) had no left ventricular (LV) involvement at baseline, but revealed new onset LV involvement at mean follow-up of 7.5 years. Patients with BiV involvement at baseline harbored significantly more desmoplakin and multiple mutations and patients with new-onset LV involvement at follow-up frequently showed non-desmosomal mutations. Patients engaging in competitive sports more often showed LV involvement during follow-up. Baseline hs-cTNT and NT-proBNP levels were higher in patients developing BiV involvement.Conclusion: Multiple mutations are more common in ARVC patients with BiV involvement. Competitive exercise is associated with disease progression resulting in BiV involvement. Hs-cTNT and NT-proBNP are elevated in patients with BiV involvement and may help to identify ARVC patients at risk for developing BiV disease. [ABSTRACT FROM AUTHOR]

Background: The comparative efficacy of antiarrhythmic drug (AAD) therapy vs ventricular tachycardia (VT) ablation in arrhythmogenic right ventricular cardiomyopathy (ARVC) is unknown.Objective: We compared outcomes of AAD and/or β-blocker (BB) therapy with those of VT ablation (with AAD/BB) in patients with ARVC who had recurrent VT.Methods: In a multicenter retrospective study, 110 patients with ARVC (mean age 38 ± 17 years; 91[83%] men) with a minimum of 3 VT episodes were included; 77 (70%) were initially treated with AAD/BB and 32 (29%) underwent ablation. Subsequently, 43 of the 77 patients treated with AAD/BB alone also underwent ablation. Overall, 75 patients underwent ablation.Results: When comparing initial AAD/BB therapy (n = 77) and VT ablation (n = 32) after ≥3 VT episodes, a single ablation procedure rendered 35% of patients free of VT at 3 years compared with 28% of AAD/BB-only-treated patients (P = .46). Of the 77 AAD/BB-only-treated patients, 43 subsequently underwent ablation. For all 75 patients who underwent ablation, 56% were VT-free at 3 years after the last ablation procedure. Epicardial ablation was used in 40/75 (53%) and was associated with lower VT recurrence after the last ablation procedure (endocardial/epicardial vs endocardial-only; 71% vs 47% 3-year VT-free survival; P = .05). Importantly, there was no difference in survival free of death or transplantation between the ablation- and AAD/BB-only-treated patients (P = .61).Conclusion: In patients with ARVC and a high VT burden, mortality and transplantation-free survival are not significantly different between drug- and ablation-treated patients. These patients have a high risk of recurrent VT despite drug therapy. Combined endocardial/epicardial ablation is associated with reduced VT recurrence as compared with endocardial-only ablation. [ABSTRACT FROM AUTHOR]

Background: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is mainly an autosomal dominant disease characterized by fibrofatty infiltration of the right ventricle, leading to ventricular arrhythmias. Mutations in desmosomal proteins can be identified in about half of the patients. The pathogenic mechanisms leading to disease expression remain unclear.Objective: The purpose of this study was to investigate myocardial expression profiles of candidate molecules involved in the pathogenesis of ARVC/D.Methods: Myocardial messenger RNA (mRNA) expression of 62 junctional molecules, 5 cardiac ion channel molecules, 8 structural molecules, 4 apoptotic molecules, and 6 adipogenic molecules was studied. The averaged expression of candidate mRNAs was compared between ARVC/D samples (n = 10), nonfamilial dilated cardiomyopathy (DCM) samples (n = 10), and healthy control samples (n = 8). Immunohistochemistry and quantitative protein expression analysis were performed. Genetic analysis using next generation sequencing was performed in all patients with ARVC/D.Results: Following mRNA levels were significantly increased in patients with ARVC/D compared to those with DCM and healthy controls: phospholamban (P ≤ .001 vs DCM; P ≤ .001 vs controls), healthy tumor protein 53 apoptosis effector (P = .001 vs DCM; P ≤ .001 vs controls), and carnitine palmitoyltransferase 1β (P ≤ .001 vs DCM; P = 0.008 vs controls). Plakophillin-2 (PKP-2) mRNA was downregulated in patients with ARVC/D with PKP-2 mutations compared with patients with ARVC/D without PKP-2 mutations (P = .04). Immunohistochemistry revealed significantly increased protein expression of phospholamban, tumor protein 53 apoptosis effector, and carnitine palmitoyltransferase 1β in patients with ARVC/D and decreased PKP-2 expression in patients with ARVC/D carrying a PKP-2 mutation.Conclusion: Changes in the expression profiles of sarcolemmal calcium channel regulation, apoptosis, and adipogenesis suggest that these molecular pathways may play a critical role in the pathogenesis of ARVC/D, independent of the underlying genetic mutations. [ABSTRACT FROM AUTHOR]

Background: Magnetic resonance imaging (MRI) of pacemaker patients is contraindicated due to documented potential risks to the patient from hazardous interactions between the MRI and pacemaker system. Objective: The purpose of this prospective, randomized, controlled, worldwide clinical trial was to evaluate the safety and effectiveness of a pacemaker system designed for safe use in MRI for any bradycardia indicated patient. Methods: Patients (n = 464) were randomized to undergo an MRI scan between 9 and 12 weeks postimplant (MRI group, n = 258) or not to undergo MRI (control group, n = 206) after successful implantation of the specially designed dual-chamber pacemaker and leads. Patients were monitored for arrhythmias, symptoms, and pacemaker system function during 14 nonclinically indicated relevant brain and lumbar MRI sequences. Sequences were performed at 1.5 T and included scans with high radiofrequency power deposition and/or high gradient dB/dt exposure. Clinical evaluation of the pacemaker system function occurred immediately before and after MRI, 1 week and 1 month post-MRI, and at corresponding times for the control group. Primary endpoints for safety analyzed the MRI procedure complication-free rate and for effectiveness compared capture and sensing performance between MRI and control groups. Results: No MRI-related complications occurred during or after MRI, including sustained ventricular arrhythmias, pacemaker inhibition or output failures, electrical resets, or other pacemaker malfunctions. Pacing capture threshold and sensed electrogram amplitude changes were minimal and similar between study groups. Conclusion: This trial documented the ability of this pacemaker system to be exposed in a controlled fashion to MRI in a 1.5 T scanner without adverse impact on patient outcomes or pacemaker system function. [ABSTRACT FROM AUTHOR]

Background: Patients with left ventricular noncompaction (LVNC) have an increased risk for life-threatening ventricular arrhythmias. The benefit from implantable cardioverter-defibrillators (ICD) in these patients has been investigated only in small series. Therefore, the aim of the present study was to analyze the clinical outcome of a larger population of patients with LVNC who were treated with an ICD. Methods: Thirty patients (mean age 48 ± 14) with LVNC who underwent ICD implantation for secondary (n = 12) or primary (n = 18) prevention were included in the study. The mean follow-up period was 40 ± 34 months. Results: During follow-up, 11 patients (37%) presented with appropriate ICD therapies: three with antitachycardia pacing, four with ICD shocks, and four with both antitachycardia pacing and ICD shocks. Of these 11 patients, five received the ICD for secondary prevention and six for primary prevention. In six patients, in whom a biventricular ICD was implanted, functional New York Heart Association (NYHA) class improved from 2.5 ± 0.5 to 1.6 ± 0.8. Conclusions: In the present study, with the largest cohort of LVNC patients with ICD to date, we demonstrate that such therapy is effective in these patients with an indication for secondary or primary prevention of sudden cardiac death. However, no clinical predictors for appropriate ICD therapy could have been elaborated in these patients. Cardiac resynchronization therapy improves functional NYHA class in patients with LVNC and may hence be considered in patients with a left ventricular ejection fraction ≤35% and signs of ventricular dyssynchrony. [ABSTRACT FROM AUTHOR]

Background: Magnetic fields may interfere with the function of cardiac pacemakers and implantable cardioverter-defibrillators (ICDs). Neodymium-iron-boron (NdFeB) magnets, which are small in size but produce strong magnetic fields, have become widely available in recent years. Therefore, NdFeB magnets may be associated with an emerging risk of device interference.Objective: We conducted a clinical study to evaluate the potential of small NdFeB magnets to interfere with cardiac pacemakers and ICDs.Methods: The effect of four NdFeB magnets (two spherical magnets 8 and 10 mm in diameter, a necklace made of 45 spherical magnets, and a magnetic name tag) was tested in forty-one ambulatory patients with a pacemaker and 29 patients with an ICD. The maximum distance at which the magnetic switch of a device was influenced was observed.Results: Magnetic interference was observed in all patients. The maximum distance resulting in device interference was 3 cm. No significant differences were found with respect to device manufacturer and device types.Conclusion: Small NdFeB magnets may cause interference with cardiac pacemakers and ICDs. Patients should be cautioned about the interference risk associated with NdFeB magnets during daily life. [ABSTRACT FROM AUTHOR]

An aortic aneurysm is defined as a balloon-shaped bulging of all three histologic components of the aortic vessel walls (intima, media and adventitia). This dilation results from vessel weakening owing to remodeling, i.e. due to cystic degeneration of the Tunica media (Marfan), progression of atherosclerosis or presence of a bicuspid aortic valve. The growth rate of the aortic diameter varies from patient to patient and may progress until the aneurysm ultimately ruptures. The role of hemodynamics, i.e. blood flow patterns, and shear stresses that are supposed to intensify during aneurysm growth are not yet fully understood, but thought to play a key role in the enlargement process. The aim of this study is to characterize the aortic blood flow in a silicone model of a pathological aorta with ascending aneurysm, to analyze the differences in the blood flow pattern compared to a healthy aortic model, and to single out possible blood flow characteristics measurable using phase contrast magnetic resonance imaging (MRI) that could serve as indicators for aneurysm severity. MRI simulations were performed under physiological, pulsatile flow conditions using data obtained from optical three dimensional particle tracking measurements. In comparison to the healthy geometry, elevated turbulence intensity and pressure loss are measured in the diseased aorta, which we propose as a complimentary indicator for assessing the aneurysmal severity. Our results shed a light on the interplay between the blood flow dynamics and their contribution to the pathophysiology and possible role for future risk assessment of ascending aortic aneurysms. [ABSTRACT FROM AUTHOR]

• First magnetic resonance image-guided cardiac radioablation (MRgRA) of sustained ventricular tachycardia. • CIED MR safety and distortion artefact evaluation performed by pretreatment in silico testing. • Successful real-time tracking and beam-gating despite interference by CIED distortion artefacts. • MRgRA may have positive implications for the target volume definition and dose distribution. To report the feasibility of magnetic resonance imaging-guided cardiac single fraction radioablation (MRgRA) in a patient with dilated cardiomyopathy and recurrent sustained ventricular tachycardia (VT) leading to electrical storms (ES). A workflow to perform Stereotactic Arrhythmia Radioablation (STAR) on a hybrid MR-Linac with real-time tracking and beam-gating was established. Challenges of the MRgRA approach included: (a) the safety of a non-MR compatible cardiac implantable electronic device (CIED) in the MR-Linac field, (b) artefacts caused by the CIED and (c) respiratory motion management with cine-tracking of the moving heart. The specific absorption rate and slew rate of the MR-Linac were within the specifications of a MR-conditional CIED. Phantom measurements showed CIED distortion artefacts of less than 1.5 mm. During MR simulation, tracking could be established on the upper liver to avoid interference with the moving heart and CIED extinction artefacts. Areas of anatomical scarring and critical substrate were identified using invasive three-dimensional electroanatomical mapping of the clinical VT during electrophysiological studies and cardiac MR imaging/computed tomography to build a volumetric target. A 71-year-old male patient with non-ischemic dilated cardiomyopathy and recurrent therapy-refractory sustained VT with repetitive implantable cardioverter-defibrillator (ICD) shocks was treated with a single fraction of 25 Gy @85% isodose, cine-tracking time was 46 min, beam-on time 24 min. 24 h post intervention the patient developed an aggravation of the clinical VT and prolonged ES. VT ceased following high-dose dexamethasone administration after 48 h. After this point, the patient remained without any episodes of sustained ventricular tachyarrhythmia requiring ICD interventions until the last follow-up at three months. Real-time tracking and beam-gating were successfully applied in this first MRgRA to treat sustained VT. [ABSTRACT FROM AUTHOR]

The value of electrocardiographic findings predicting adverse outcome in patients with arrhythmogenic right ventricular dysplasia (ARVD) is not well known. We hypothesized that ventricular depolarization and repolarization abnormalities on the 12-lead surface electrocardiogram (ECG) predict adverse outcome in patients with ARVD. ECGs of 111 patients screened for the 2010 ARVD Task Force Criteria from 3 Swiss tertiary care centers were digitized and analyzed with a digital caliper by 2 independent observers blinded to the outcome. ECGs were compared in 2 patient groups: (1) patients with major adverse cardiovascular events (MACE: a composite of cardiac death, heart transplantation, survived sudden cardiac death, ventricular fibrillation, sustained ventricular tachycardia, or arrhythmic syncope) and (2) all remaining patients. A total of 51 patients (46%) experienced MACE during a follow-up period with median of 4.6 years (interquartile range 1.8 to 10.0). Kaplan-Meier analysis revealed reduced times to MACE for patients with repolarization abnormalities according to Task Force Criteria (p [ 0.009), a precordial QRS amplitude ratio (...PQRS mV V1 to V3/...PQRS mV V1 to V6) of £0.48 (p [ 0.019), and QRS fragmentation (p [ 0.045). In multivariable Cox regression, a precordial QRS amplitude ratio of £0.48 (hazard ratio [HR] 2.92, 95% confidence interval [CI] 1.39 to 6.15, p [ 0.005), inferior leads T-wave inversions (HR 2.44, 95% CI 1.15 to 5.18, p [ 0.020), and QRS fragmentation (HR 2.65, 95% CI 1.1 to 6.34, p [ 0.029) remained as independent predictors of MACE. In conclusion, in this multicenter, observational, long-term study, electrocardiographic findings were useful for risk stratification in patients with ARVD, with repolarization criteria, inferior leads TWI, a precordial QRS amplitude ratio of £0.48, and QRS fragmentation constituting valuable variables to predict adverse outcome. [ABSTRACT FROM AUTHOR]

The role of the electrophysiologic (EP) study for risk stratification in patients with arrhythmogenic right ventricular cardiomyopathy is controversial. We investigated the role of inducible sustained monomorphic ventricular tachycardia (SMVT) for the prediction of an adverse outcome (AO), defined as the occurrence of cardiac death, heart transplantation, sudden cardiac death, ventricular fibrillation, ventricular tachycardia with hemodynamic compromise or syncope. Of 62 patients who fulfilled the 2010 Arrhythmogenic Right Ventricular Cardiomyopathy Task Force criteria and underwent an EP study, 30 (48%) experienced an adverse outcome during a median follow-up of 9.8 years. SMVT was inducible in 34 patients (55%), 22 (65%) of whom had an adverse outcome. In contrast, in 28 patients without inducible SMVT, 8 (29%) had an adverse outcome. Kaplan-Meier analysis showed an event-free survival benefit for patients without inducible SMVT (logrank p = 0.008) with a cumulative survival free of an adverse outcome of 72% (95% confidence interval [CI] 56% to 92%) in the group without inducible SMVT compared to 26% (95% CI 14% to 50%) in the other group after 10 years. The inducibility of SMVT during the EP study (hazard ratio [HR] 2.99, 95% CI 1.23 to 7.27), nonadherence (HR 2.74, 95% CI 1.3 to 5.77), and heart failure New York Heart Association functional class II and III (HR 2.25, 95% CI 1.04 to 4.87) were associated with an adverse outcome on univariate Cox regression analysis. The inducibility of SMVT (HR 2.52, 95% CI 1.03 to 6.16, p = 0.043) and nonadherence (HR 2.34, 95% CI 1.1 to 4.99, p = 0.028) remained as significant predictors on multivariate analysis. This long-term observational data suggest that SMVT inducibility during EP study might predict an adverse outcome in patients with arrhythmogenic right ventricular cardiomyopathy, advocating a role for EP study in risk stratification. [ABSTRACT FROM AUTHOR]

Abstract: Background: Duty-cycled (DC) radiofrequency ablation (RFA) for atrial fibrillation (AF) has been introduced, however, data on large patient series and comparison to conventional RFA are scarce. Methods: Between 2006 and 2008DC RFA was performed in 209 consecutive patients (143 (68%) paroxysmal and 66 (32%) persistent AF). As controls served 211 patients, 155 (73%) with paroxysmal and 56 (27%) with persistent AF (p =0.3). In DC RFA, the pulmonary veins (PV) were isolated followed by ablation at the septum and left atrium, if AF persisted. Conventional PV isolation was followed by anatomical lines at the roof and mitral isthmus. Results: Freedom of paroxysmal AF was demonstrated after 1.08DC RFA procedures per patient in 82% and after 1.19 conventional procedures in 87% after 8.5±6.5months (ns). In persistent AF, success rates were 79% after 1.35DC RFA procedures and 80% after 1.34 conventional procedures after 11.5±8.5months (ns). The subgroup analysis of 119 patients with follow-up ≥12months (17.5 [14.1–23.6] months) showed similar results. Left atrial flutter occurred in 3% and 8% after paroxysmal AF ablation (p <0.05) and in 12% and 23% after persistent AF ablation (p =0.1). Multivariate predictors for success in both groups were age, left atrial size, presence of persistent vs. paroxysmal AF and previous pacemaker implantation, but not the ablation technique used. Non-fatal complications were seen in 2.8% with no differences between the groups. Conclusion: Outcome in DC RFA is similar to conventional RFA with a final success rate exceeding 80% in both paroxysmal and persistent AF in the absence of fatal complications. [Copyright &y& Elsevier]

Implantable cardioverter–defibrillator (ICD) therapy decreases arrhythmic and all-cause mortality in patients at high risk of sudden death. However, its clinical benefit in elderly patients is uncertain. The aim of this study was to assess the long-term efficacy of ICD treatment in elderly patients and to identify markers of successful ICD therapy and risk factors of mortality. We performed multivariate analysis of a prospective long-term database from 2 tertiary care centers including 936 consecutive patients with an ICD. Predictors of ICD therapy and risk factors for mortality were assessed in patients ≥75 years old at ICD implantation compared to younger patients. Mean follow-up time was 43 ±40 months. Rates of ICD therapy were similar in the 2 age groups. No significant predictors of ICD therapy could be identified in older patients. Median estimated survival was 132 months in patients <75 years and 81 months in those ≥75 years old (p = 0.006). Decreased ejection fraction (hazard ratio 1.62 per 10% decrease, p = 0.03) and impaired renal function (hazard ratio 1.57 per 10 ml/kg/m2 decrease in estimated glomerular filtration rate, p = 0.02) were independent risk factors of mortality in patients ≥75 years old. However, mortality of older patients was similar to that of the age-matched general population irrespective of delivery of ICD therapy. In conclusion, ICD therapy is effective for treatment of life-threatening arrhythmias in all age groups. However, prevention of sudden cardiac death may have limited impact on overall mortality in older patients. Despite a similar rate of appropriate ICD therapies, risk of death is increased 1.6-fold in ICD recipients ≥75 years old compared to younger patients. Patients with decreased ejection fraction and impaired renal function are at highest risk. [Copyright &y& Elsevier]

Left ventricular hypertrophy is 1 of the most frequent cardiac manifestations associated with an unfavorable prognosis. However, many different causes of left ventricular hypertrophy exist. The aim of the present study was to assess the diagnostic value of common electrocardiographic (ECG) parameters to differentiate Fabry disease (FD), amyloidosis, and nonobstructive hypertrophic cardiomyopathy (HC) from hypertensive heart disease (HHD) and aortic stenosis (AS). In 94 patients with newly diagnosed FD (n = 17), HHD (n = 20), amyloidosis (n = 17), AS (n = 20), and HC (n = 20), common ECG parameters were analyzed and tested for their diagnostic value. A stepwise approach including the Sokolow–Lyon index, corrected QT duration, and PQ interval minus P-wave duration in lead II to overcome P-wave abnormalities was applied. A corrected QT duration <440 ms in combination with a PQ interval minus P-wave duration in lead II <40 ms was 100% sensitive and 99% specific for the diagnosis of FD, whereas a corrected QT duration >440 ms and a Sokolow–Lyon index ≤1.5 mV were found to have a sensitivity and specificity of 85% and 100%, respectively, for the diagnosis of amyloidosis and differentiation from HC, AS, and HHD. Moreover, a novel index ([PQ interval minus P-wave duration in lead II multiplied by corrected QT duration]/Sokolow–Lyon index) proved to be highly diagnostic for the differentiation of amyloidosis (area under the curve 0.92) and FD (area under the curve 0.91) by receiver operator characteristic analysis. In conclusion, a combined analysis of PQ interval minus P-wave duration in lead II, corrected QT duration, and Sokolow–Lyon index proved highly sensitive and specific in the differentiation of FD, amyloidosis, and HC compared to HHD and AS. Analysis of these easy-to-assess ECG parameters may be of substantial help in the diagnostic workup of these 5 conditions. [ABSTRACT FROM AUTHOR]

Congenital left ventricular aneurysms and diverticula (LVA/Ds) are rare cardiac malformations that can be detected using echocardiography or other imaging techniques. Some of these patients present with ventricular arrhythmias. This study investigated clinical characteristics of patients with congenital LVA/D presenting with arrhythmic manifestations. Over the previous 20 years 250 patients were diagnosed to have congenital LVA/D at our institution. Diagnosis was made using echocardiography after exclusion of coronary artery disease, local cardiac inflammatory processes, traumatic causes, or cardiomyopathies. At initial presentation 32 of the 250 patients (13%, average age 45 years, range 25 to 65, 21 men and 11 women) exhibited arrhythmias. At least 2 LVA/Ds were present in 6 of these patients. LVA/Ds were localized at the posterobasal, apical, anteroseptal, and anterolateral walls in 12, 11, 4, and 5 patients, respectively. The most common complaints at presentation were syncope or presyncope in 18 patients and palpitations in 11 patients. One patient had survived sudden cardiac death. Long-term electrocardiographic recordings showed ventricular tachycardia (VT) or ventricular fibrillation in 17 patients (53%). Twelve patients underwent electrophysiologic testing. Nine patients had inducible ventricular tachyarrhythmia, whereas induced tachycardia was similar to that during spontaneous arrhythmia in 7 patients. In conclusion, patients with congenital LVA/Ds who present with arrhythmic manifestations commonly have VT. Electrophysiologic testing can reproduce clinical VT in most of these patients. [Copyright &y& Elsevier]

Fabry disease (FD) is an X-chromosomal inherited lysosomal storage disease resulting in intracellular storage of globotriaosylceramide. Cardiac involvement is most frequently manifested as left ventricular hypertrophy. However, patients with FD may also have from various conduction abnormalities particularly affecting atrioventricular (AV) conduction. The present study was designed to analyze primarily AV conduction abnormalities on baseline electrocardiograms of patients with FD and to investigate the correlation with echocardiographic findings. Electrocardiograms at rest of 207 patients with FD were compared to echocardiograms. PQ-interval shortening and first-degree AV block could be found in only 29 cases (14%) and 3 cases (1.4%), respectively. No echocardiographic differences could be found in patients with and without PQ-interval shortening, including left ventricular hypertrophy, atrial size, and diastolic parameters. Furthermore, no correlation of the PQ interval with any echocardiographic parameters was detected. There was no difference between men and women in baseline clinical and electrocardiographic parameters. In conclusion, shortening of the PQ interval was not a common electrocardiographic finding in patients newly diagnosed with FD. Furthermore, no correlation with typical echocardiographic findings or disease stage in FD at baseline could be found. [Copyright &y& Elsevier]

Isolated ventricular noncompaction (IVNC) is a primary cardiomyopathy characterized by a specific morphologic pattern. Patients with IVNC can develop various arrhythmic complications such as life-threatening ventricular arrhythmias, as well as heart failure or systemic embolic events. The present study was designed to comprehensively analyze the electrocardiographic (ECG) pattern at the initial diagnosis in patients with IVNC and to investigate their correlation with the clinical features and echocardiographic findings. Electrocardiograms from the initial diagnosis of IVNC were available for 78 patients from March 1995 to November 2008. The most common findings were intraventricular conduction delay (especially left bundle branch block), voltage signs of left ventricular (LV) hypertrophy, and repolarization abnormalities. An entirely normal electrocardiogram was present in 10 subjects (13%). However, no ECG findings or patterns specific for IVNC were found. A striking overlap was observed between the presence of intraventricular conduction delay (left bundle branch block, in particular), atrial conduction delay (PR interval prolongation or atrioventricular block), and prolongation of the QTc and reduced systolic LV function and LV/left atrial dilation. Moreover, patients with ECG voltage signs of LV hypertrophy more often presented with, or had a history of, systemic embolic events. In conclusion, our results have provided a comprehensive analysis of ECG findings of patients newly diagnosed with IVNC. Although intraventricular conduction delay, repolarization abnormalities, and LV hypertrophy are frequently present, no ECG patterns specific for IVNC at the first presentation with the disease were found. Whether these findings have prognostic implications needs to be investigated in long-term controlled studies. [Copyright &y& Elsevier]

Natriuretic peptides have been shown to be high in patients with myocardial ischemia. We sought to create a diagnostic score using clinical data, stress testing, and B-type natriuretic peptide (BNP) levels to improve noninvasive prediction of coronary artery disease (CAD). Patients with stable angina pectoris and normal systolic left ventricular function were eligible for this prospective cohort study. Patients with arrhythmias, valvular heart disease, impaired left ventricular function, or renal dysfunction were excluded. All patients underwent clinical evaluation, bicycle stress testing, BNP testing, and coronary angiography. Then a diagnostic risk score was derived that combined cardiovascular risk factors, results of exercise testing, and BNP measurements and added 1 point for the presence of each of these variables. Seventy-one patients (52 years of age, range 31 to 61; 46 men) were included in the study. Prevalence of CAD, defined by 50% narrowing of ≥1 coronary artery on coronary angiography, was 45%. For 0 point in the risk score system, the negative predictive value was 93% with a negative likelihood ratio of 0.1 (95% confidence interval [CI] 0.02 to 0.38); for a score of 3 points, the positive predictive value was 93% with a positive likelihood ratio of 15.9 (95% CI 2.19 to 114.7). Serum BNP level >50 ng/L at rest was the best single diagnostic parameter, with 66% sensitivity and 97% specificity, and a positive likelihood ratio of 25.6 (95% CI 3.64 to 180) and a negative likelihood ratio of 0.35 (95% CI 0.22 to 0.57). In conclusion, a diagnostic score combining exercise testing, clinical data, and serum BNP values at rest can distinguish patients with CAD from those without CAD with high accuracy. [Copyright &y& Elsevier]