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The purpose of this study was to analyze comprehensively the heart using modern and sensitive myocardial techniques in order to determine if structural or functional cardiac alterations are present in adult Pompe disease. Twelve patients with adult Pompe disease and a control group of 187 healthy subjects of similar age and gender were included. Structural and functional cardiac characteristics were analyzed by conventional and 2D speckle-tracking echocardiography. In addition, in a subgroup of adult Pompe patients, we analyzed the myocardial and musculoskeletal features by means of cardiac and whole-body muscle magnetic resonance imaging. Patients with Pompe disease had significant structural and functional musculoskeletal alterations such as atrophy with fatty replacement and weakness in trunk and extremities. In contrast, Pompe patients had similar structural and functional myocardial features to healthy subjects (LV strain -20.7 ± 1.9 vs. -21.3 ± 2.1%; RV strain -24.2 ± 5.3 vs. -24.8 ± 3.8%; LA strain 41.5 ± 10.3 vs. 44.8 ± 11.0%; P > 0.05; and no evidence of LV and RV hypertrophy or LA enlargement). In addition, there was no evidence of valvular cardiac alterations, electrocardiographic abnormalities, or myocardial fibrosis in Pompe patients. In the current study analyzing the heart with modern and sensitive myocardial techniques, we evidenced that functional and structural cardiac alterations are not present when Pompe disease begins in adulthood. Therefore, these findings suggest that adult Pompe disease should not be taken into consideration in the differential diagnostic of structural or functional cardiac disorders.

Aims: The aim of this multicentre study was to determine the normal range and the clinical relevance of the myocardial function of the left atrium (LA) analysed by 2D speckle-tracking echocardiography (2DSTE)., Methods and Results: We analysed 329 healthy adult subjects prospectively included in 10 centres and a validation group of 377 patients with left ventricular diastolic dysfunction (LVDD). LA myocardial function was analysed by LA strain rate peak during LA contraction (LA-SRa) and LA strain peak during LA relaxation (LA-Strain). The range of values of LA myocardial function in healthy subjects was LA-SRa -2.11 ± 0.61 s(-1) and LA-Strain 45.5 ± 11.4%, and the lowest expected values of these LA analyses (calculated as -1.96 SD from the mean of healthy subjects) were LA-SRa -0.91 s(-1) and LA-Strain 23.1%. Concerning the clinical relevance of these LA myocardial analyses, LA-SRa and LA-Strain detected subtle LA dysfunction in patients with LVDD, even though LA volumetric measurements were normal. In addition, in these patients we found that the functional class (dyspnoea-NYHA classification) was inversely related to both LA-Strain and LA-SRa., Conclusion: In the present multicentre study analysing a large cohort of healthy subjects and patients with LVDD, the normal range and the clinical relevance of the myocardial function of the LA using 2DSTE have been determined., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.)

Purpose: Pulmonary vein isolation (PVI) is widely established as a curative treatment option for atrial fibrillation (AF). A wide range of techniques to improve catheter manipulation and steerability has been developed over the past years. A new remote catheter system (RCS) has recently become available (Amigo Remote Catheter System, Catheter Robotics, Budd Lake, NJ, USA). Here, we present a dual-center study on the RCS for left atrial mapping and PVI in patients with paroxysmal AF compared to a control group undergoing conventional PVI., Methods: One hundred nineteen patients who underwent PVI for paroxysmal AF were studied. Forty patients underwent PVI with the use of the RCS. Seventy-nine patients, who underwent conventional PVI, served as control group. Procedural data were compared between the two groups., Results: PVI was achieved in all patients. In the RCS group compared to standard ablation group, there were no significant differences in procedure duration (159.1 ± 45.4 vs. 146 ± 30.1 min, p = 0.19), total energy delivery (78,146.3 ± 26,992.4 vs. 87,963.9 ± 79,202.1 Ws, p = 0.57), and total fluoroscopy time (21.2 ± 8.6 vs. 23.9 ± 5.4 min, p = 0.15). Operator fluoroscopy exposure was significantly reduced in the RCS group (13.4 ± 6.1 vs. 23.9 ± 5.4 min, p < 0.001)., Conclusions: These initial results suggest that left atrial mapping and PVI are feasible with the use of the Amigo RCS. Acute procedural efficacy is comparable to the standard approach. The use of the Amigo RCS leads to a significant reduction of operator fluoroscopy exposure.

Background: The aim of this multicenter study was to determine the normal ranges and the clinical relevance of multidirectional systolic parameters to evaluate global left ventricular (LV) systolic function., Methods: Three hundred twenty-three healthy adult subjects prospectively included at 10 centers and a cohort of 310 patients with hypertension were analyzed. Multidirectional global LV systolic function was analyzed using two-dimensional speckle-tracking echocardiography by means of two indices: longitudinal-circumferential systolic index (the average of longitudinal and circumferential global systolic strain) and global systolic index (the average of longitudinal, circumferential, and radial global systolic strain)., Results: The ranges of values of the multidirectional systolic parameters in healthy subjects were -21.22 ± 2.22% for longitudinal-circumferential systolic index and 29.71 ± 5.28% for global systolic index. In addition, the lowest expected values of these multidirectional indices were determined in this population (calculated as -1.96 SDs from the mean): -16.86% for longitudinal-circumferential systolic index and 19.36% for global systolic index. Concerning the clinical relevance of these measurements, these indices indicated the presence of subtle LV global systolic dysfunction in patients with hypertension, even though LV global longitudinal systolic strain and LV ejection fraction were normal. Moreover, in these patients, functional class (dyspnea [New York Heart Association classification]) was inversely related to both the longitudinal-circumferential index and the global systolic index., Conclusions: In the present multicenter study analyzing a large cohort of healthy subjects and patients with hypertension, the normal range and the clinical relevance of multidirectional systolic parameters to evaluate global LV systolic function have been determined., (Copyright © 2014 American Society of Echocardiography. Published by Mosby, Inc. All rights reserved.)

Background: Cardiac troponin I (cTnI) is highly specific for myocardial damage and for the diagnosis of acute coronary syndrome. We investigated cTnI utility and predictive value in patients with atrial fibrillation (AF) in the acute setting., Method: We studied 354 consecutive patients with the primary diagnosis of AF and clinical symptoms suggestive of myocardial ischemia presenting to our emergency department. cTnI was obtained on presentation. Patients with ST-segment elevation myocardial infarction were excluded. Coronary angiography was performed in 100 patients., Results: cTnI was elevated (>0.09 μg/L) in 51 of 354 (15%) patients. The mean cTnI in these patients was 0.37 μg/L (0.09-3.14). In 23 of 100 patients undergoing coronary angiography, cTnI was elevated. Only 6 of these 23 patients (26%) had significant stenosis. In 77 of 100 patients undergoing coronary angiography, cTnI was normal, revealing significant stenosis in 25 patients (33%). The positive predictive value of elevated cTnI for a coronary intervention was 26% and the negative predictive value was 68%. Using multivariate logistic regression, we found that heart rate on presentation, the presence of angina pectoris, left ventricular ejection fraction, serum creatinine, and hemoglobin independently predicted elevated cTnI level., Conclusion: These data are the first to show that AF in the acute setting is frequently associated with cTnI elevations. AF patients with high heart rate and/or angina pectoris often show false elevated cTnI levels. These findings are relevant for clinicians evaluating patients with acute AF and myocardial ischemia symptoms. Appropriate clinical guidelines must be established that also consider AF-related elevations in cTnI., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)