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Left ventricular (LV) hypertrophy is the main feature of cardiac involvement in Anderson-Fabry disease (FD), but the right ventricle (RV) is also frequently affected. Previous studies failed to demonstrate an independent association between conventional parameters of RV performance and outcomes in FD. Nevertheless, if RV free wall strain (RV-FWS), assessed by 2D speckle tracking analysis, may provide a better prognostication is currently unknown. We retrospectively evaluated the association between RV-FWS and the occurrence of cardiovascular events in a cohort of 56 patients with FD. The study endpoint comprises cardiovascular mortality, severe heart failure symptoms, new-onset atrial fibrillation and major arrhythmias requiring device implantation. Reduced RV-FWS, defined by values lower than 23%, was found in 25 (45%) patients. During a median follow-up of 47 months, 16 (29%) patients met the study endpoint. A ROC-curve analysis confirmed the threshold of reduced RV-FWS (<23%) as the best cut-off for predicting cardiovascular events, but with a lower power compared to left-sided parameters. On univariable Cox regression analysis, RV-FWS, expressed as continuous variable, was significantly associated with the study endpoint (HR: 0.795, 95% CI: 0.710–0.889, p < 0.001). However, RV-FWS did not retain a significant association with outcomes, after adjustment for LV global longitudinal strain or indexed left atrial volume (p = 0.340 and p = 0.289 respectively). RV-FWS was not independently associated with the occurrence of cardiovascular events in FD, confirming previous observations that prognosis is mainly driven by the severity of LV cardiomyopathy. • Impaired RV-FWS is associated with a higher risk of cardiac events in Fabry disease. • However this association was not significant after adjustment for left-sided parameters. • The severity of LV cardiomyopathy is the main prognostic determinant in Fabry disease. [ABSTRACT FROM AUTHOR]

To identify the association between comorbidities and left atrial (LA) and right atrial (RA) function in patients with paroxysmal atrial fibrillation (AF). This is a cross-sectional study. Speckle-tracking echocardiography was performed in 344 patients with paroxysmal AF at baseline, and available in 298 patients after 1-year follow-up. The number of comorbidities (hypertension, diabetes mellitus, coronary artery disease, body mass index > 25 kg/m2, age > 65 years, moderate to severe mitral valve regurgitation and kidney dysfunction (estimated glomerular filtration rate < 60 ml/min/1.73 m2)) was determined and the association with atrial strain was tested. Mean age of the patients was 58 (SD 12) years and 137 patients were women (40%). Patients with a higher number of comorbidities had larger LA volumes (p for trend <0.001), and had a decrease in all strain phases from the LA and RA, except for the RA contraction phase (p for trend 0.47). A higher number of comorbidities was associated with LA reservoir and conduit strain decrease independently of LA volume (p < 0.001, p < 0.001 respectively). Patients with 1–2 comorbidities, but not patients with 3 or more comorbidities, showed a further progression of impaired LA and RA function in almost all atrial strain phases at 14 [13–17] months follow-up. In patients with paroxysmal AF, individual and combined comorbidities are related to lower LA and RA strain. In patients with few comorbidities, impairment in atrial function progresses during one year of follow-up. Whether comorbidity management prevents or reverses decrease in atrial function warrants further study. • Individual and combined comorbidities in paroxysmal AF patients are associated with lower strain of both atria, independent of atrial volumes. • In paroxysmal AF patients with few comorbidities, impairment in atrial function progresses during one year of follow-up. • Patients with paroxysmal AF patients with 3 or more comorbidities had less or no further progressive impairment of LA and RA function. [ABSTRACT FROM AUTHOR]

Recent evidence suggests that an implantable cardioverter defibrillator (ICD) in non-ischemic cardiomyopathy (NICM) may not offer mortality benefit. We aimed to investigate if etiology of heart failure and strain echocardiography can improve risk stratification of life threatening ventricular arrhythmia (VA) in heart failure patients. This prospective multi-center follow-up study consecutively included NICM and ischemic cardiomyopathy (ICM) patients with left ventricular ejection fraction (LVEF) <40%. We assessed LVEF, global longitudinal strain (GLS) and mechanical dispersion (MD) by echocardiography. Ventricular arrhythmia was defined as sustained ventricular tachycardia, sudden cardiac death or appropriate shock from an ICD. We included 290 patients (67 ± 13 years old, 74% males, 207(71%) ICM). During 22 ± 12 months follow up, VA occurred in 32(11%) patients. MD and GLS were both markers of VA in patients with ICM and NICM, whereas LVEF was not (p = 0.14). MD independently predicted VA (HR: 1.19; 95% CI 1.08–1.32, p = 0.001), with excellent arrhythmia free survival in patients with MD <70 ms (Log rank p < 0.001). Patients with NICM and MD <70 ms had the lowest VA incidence with an event rate of 3%/year, while patients with ICM and MD >70 ms had highest VA incidence with an event rate of 16%/year. Patients with NICM and normal MD had low arrhythmic event rate, comparable to the general population. Patients with ICM and MD >70 ms had the highest risk of VA. Combining heart failure etiology and strain echocardiography may classify heart failure patients in low, intermediate and high risk of VA and thereby aid ICD decision strategies. • Risk stratification by strain echocardiography may add important information when considering a primary prophylactic ICD. • Non-ischemic dilated cardiomyopathy with low mechanical dispersion had an arrhythmic rate close to the general population. • Ejection fraction < 40% and mechanical dispersion >70 ms indicates monitoring for arrhythmias irrespective of etiology. [ABSTRACT FROM AUTHOR]

Left atrial appendage (LAA) dysfunction is associated with increased risk of thromboembolic events. However, little is known about LAA mechanical dispersion (MD) would provide additional information toward thromboembolism over the CHA2DS2-VASc score. The aim of this study was to determine the association of LAA mechanics as assessed by speckle-tracking imaging with thromboembolic events in patients with nonvalvular atrial fbrillation (AF). A total of 116 consecutive patients with AF referred for transesophageal echocardiography (TEE) were prospectively enrolled. Of these, 17(14.7%) patients had prior embolic events. Using speckle-tracking echocardiography (STE), we measured the LAA strain in each of 24 segments in mid-esophageal TEE views obtained at 0°, 45°, 90° and 135°. LAA MD was defined as the standard deviation (SD) of time to peak positive strain corrected by the R-R interval. Patients with embolism had lower LAA global longitudinal strain (GLS) (8.56 ± 2.62% vs 11.37 ± 5.54%, p = 0.002) and higher LAA MD (16.90 ± 6.67% vs 12.10 ± 3.94%; P = 0.010) than those without embolism. LAA MD >13.1% differentiated patients with embolism from controls, with an area under the curve (AUC) of 0.709(p = 0.004). LAA MD was independently associated with the presence of thromboembolism in multivariate analysis (odds ratio, 1.24; 95% confidence interval, 1.08–1.42; P = 0.002). The model based on CHA 2 DS 2 -VASc score for discrimination of patients with embolism was significantly improved by adding LAA MD (P < 0.01). LAA MD obtained from strain echocardiography was significantly associated with a prior history of embolic events and had incremental diagnostic value over CHA2DS2VASc score, suggesting that LAA MD may be useful in refining thromboembolic risk stratification in patients with AF. • Patients with embolism had higher LAA mechanical dispersion (MD) than those without embolism. • LAA MD was independently associated with the presence of thromboembolism in multivariate analysis. • LAA MD significantly improved the model based on CHA2DS2-VASc score for discrimination of patients with embolism. [ABSTRACT FROM AUTHOR]

Postoperative atrial fibrillation (PoAF) is a common complication after cardiac surgery. A pre-existing atrial substrate appears to be important in postoperative development of dysrhythmia, but its preoperative estimation is challenging. We tested the hypothesis that a combination of clinical predictors, noninvasive surrogate markers for atrial fibrosis defining abnormal left atrial (LA) mechanics, and biomarkers of collagen turnover is superior to clinical predictors alone in identifying patients at-risk for PoAF. In patients without prior AF undergoing coronary artery bypass grafting, concentrations of biomarkers reflecting collagen synthesis and degradation, extracellular matrix, and regulatory microRNA-29s were determined in serum from preoperative blood samples and correlated to atrial fibrosis extent, alteration in atrial deformation properties determined by 3D speckle-tracking echocardiography, and AF development. Of 90 patients without prior AF, 34 who developed PoAF were older than non-PoAF patients (72.04 ± 10.7 y; P = 0.043) with no significant difference in baseline comorbidities, LA size, or ventricular function. Global (P = 0.007) and regional longitudinal LA strain and ejection fraction (P = 0.01) were reduced in PoAF vs. non-PoAF patients. Preoperative amino-terminal-procollagen-III-peptide (PIIINP) (103.1 ± 39.7 vs. 35.1 ± 19.3; P = 0.041) and carboxy-terminal-procollagen-I-peptide levels were elevated in PoAF vs. non-PoAF patients with a reduction in miR-29 levels and correlated with atrial fibrosis extent. Combining age as the only significant clinical predictor with PIIINP and miR-29a provided a model that identified PoAF patients with higher predictive accuracy. In patients without a previous history of AF, using age and biomarkers of collagen synthesis and regulation, a noninvasive tool was developed to identify those at risk for new-onset PoAF. • Postoperative AF is a common problem in patients post cardiac surgery. • Clinical predictors used to distinguish patients at risk of PoAF have limitations. • Available biomarkers do not fully reflect the substrate responsible for PoAF. • We developed a model better able to find CABG patients at risk of PoAF. • Further studies are warranted to validate the utility of this noninvasive approach. [ABSTRACT FROM AUTHOR]

Fabry disease (FD) and transthyretin cardiac amyloidosis (TTR CA) are cardiomyopathies with hypertrophic phenotype that share several features, including left atrial (LA) enlargement and dysfunction, but direct comparative data are lacking. Aim of the present study was to perform a comparative analysis of LA remodelling between the two diseases. In this prospective study, a total of 114 patients (31 FD and 83 TTR CA) were included; all of them had left ventricular hypertrophy (LVH), defined as left ventricular (LV) wall thickness ≥ 12 mm. Despite similar degree of LVH, patients with TTR CA showed worse LV systolic and diastolic function. LA maximal volume index was not significantly different between the two groups (p = 0.084), while patients with TTR CA showed larger LA minimal volume index (p = 0.001). Moreover, all phases of LA mechanics were more impaired in the TTR CA group vs FD (reservoir: 6.9[4.2–15.5] vs 19.0[15.5–29.5], p < 0.001). After excluding patients with atrial fibrillation (AF), these differences remained clearly significant. In multivariable regression analyses, LA reservoir strain showed an independent correlation with TTR CA, controlling for demographic characteristics, AF and LV systolic and diastolic performance (p ≤ 0.001), whereas LV global longitudinal strain did not. Finally, among echocardiographic parameters, LA function demonstrated the highest accuracy in discriminating the two diseases. TTR CA is characterized by a more advanced LA structural and functional remodelling in comparison to patients with FD and similar degree of LVH. The association between TTR CA and LA dysfunction remains consistent after adjustment for potential confounders. [Display omitted] • LA remodelling was compared between patients with FD and TTR CA. • The two study groups had a similar degree of LV hypertrophy. • TTR CA patients showed larger LAVmin, whereas there were no differences in LAVImax. • TTR CA patients were characterized by worse LA functional parameters. • This association was confirmed after adjustment for potential confounders. [ABSTRACT FROM AUTHOR]

Abstract Background Although exercise-induced cardiac hypertrophy has been intensively investigated, its development and regression dynamics have not been comprehensively described. In the current study, we aimed to characterize the effects of regular exercise training and detraining on left ventricular (LV) morphology and function. Methods Rats were divided into exercised (n = 12) and control (n = 12) groups. Exercised rats swam 200 min/day for 12 weeks. After completion of the training protocol, rats remained sedentary for 8 weeks (detraining period). Echocardiographic follow-up was performed regularly to obtain LV long- and short-axis recordings for speckle-tracking echocardiography analysis. Global longitudinal and circumferential strain and systolic strain rate were measured. LV pressure-volume analysis was performed using additional groups of rats to obtain haemodynamic data. Results Echocardiographic examinations showed the development of LV hypertrophy in the exercised group. These differences disappeared during the detraining period. Strain and strain rate values were all increased after the training period, whereas supernormal values rapidly reversed to the control level after training cessation. Load-independent haemodynamic indices, e.g., preload recruitable stroke work, confirmed the exercise-induced systolic improvement and complete regression after detraining. Conclusions and translational aspect Our results provide the first comprehensive data to describe the development and regression dynamics of morphological and functional aspects of physiological hypertrophy in detail. Speckle-tracking echocardiography has been proven to be feasible to follow-up changes induced by exercise training and detraining and might provide an early possibility to differentiate between physiological and pathological conditions. Highlights • Description of development and regression dynamics of exercise-induced hypertrophy • Training and detraining-induced alterations by speckle-tracking echocardiography • Strain and strain rate parameters to follow-up changes induced by exercise training • Recognition of overlapping pathological conditions with athlete's heart [ABSTRACT FROM AUTHOR]

Abstract Background and aim In autosomal dominant polycystic kidney disease (ADPKD) cardiac abnormalities have been observed before the onset of hypertension or renal dysfunction. We sought to characterize, in ADPKD patients, left ventricular (LV) function and its changes after somatostatin-analogue octreotide-LAR treatment. Methods In a 1:1:1 cross-sectional study, we evaluated LV function by speckle-tracking echocardiography in 34 ADPKD patients from one ALADIN-trial center and in 34 age- and gender-matched healthy controls and 34 equally-matched renal controls with non-cystic chronic kidney disease. Changes in LV function were compared in the 16 and 18 ADPKD patients originally randomized to 3 year-treatment with octreotide-LAR or placebo, respectively. Results LV twist and untwisting rates were lower in ADPKD patients that in healthy or renal controls (6.1 ± 2.6° vs. 11.1 ± 2.1° and 10.2 ± 3.7°; −49.5 ± 18.1°/s vs. −79.8 ± 12.2°/s and −84.3 ± 25.9°/s, respectively, all p < 0.001). The correlation between LV mass or diastolic BP and untwisting rate was positive in ADPKD patients (r = 0.38, p = 0.025 and r = 0.44, p = 0.011, respectively), not significant in healthy controls and negative in renal controls (r = −0.38; p = 0.023 and r = −0.40, p = 0.012, respectively. LV untwisting rate improved from −49.9 ± 18.6°/s to −70.3 ± 27.5°/s with octreotide-LAR, but did not change with placebo (p = 0.027 for treatment effect). At adjusted linear regression analysis, octreotide-LAR therapy emerged as the only independent predictor of untwisting rate improvement at final visit [beta coefficient −0.504 (95% CI −46.905–−6.367), p = 0.014]. Conclusions In ADPKD patients LV function is early impaired. Somatostatin-analogue therapy might help in preventing or ameliorating LV dysfunction in this population. Clinical Trial Registration http://www.clinicaltrials.gov , NCT0030928. Highlights • In ADPKD patients LV function is impaired trough disease-specific mechanisms. • Treatment with octreotide-LAR, a somatostatin analogue, is able to preserve LV function in these patients. • Indications of somatostatin-analogue therapy might be broadened to cardiovascular risk amelioration. [ABSTRACT FROM AUTHOR]

Background The clinical work-up of patients presenting with chest pain is a diagnostic challenge. We investigated the diagnostic performance of global (GLS) and territorial (TLS) longitudinal strain to predict coronary artery disease (CAD) in patients presenting with suspected non-ST-segment elevation acute coronary syndrome (NSTE-ACS) but apparent normal global and regional systolic function. Methods 150 consecutive suspected NSTE-ACS patients were initially screened for inclusion ; 58 patients with normal LVEF (≥ 55%) and WMSI (= 1) were prospectively enrolled. Speckle-tracking echocardiography was performed on admission and all the patients underwent coronary angiography. CAD was defined as the presence of stenosis of > 50%. Results CAD was present in 33 patients (57%). LVEF was 60.7 ± 4.6% in group 1 (CAD) and 61.1 ± 5.0% in group 2 (no CAD). Global longitudinal strain (GLS) was altered in group 1 (− 16.7 ± 3.4%) as compared to group 2 (− 22.4 ± 2.9%, p < 0.001). ROC curve analysis showed a high diagnostic value of GLS for the prediction of CAD (AUC = 0.92 [0.84–1.00], p = 0.0001). TLS was able to discriminate between coronary stenosis in the LAD, LCX or RCA. Conclusions Longitudinal 2D strain has a good diagnostic value and can efficiently localize the culprit lesion in patients presenting with NSTE-ACS but apparent normal global and regional systolic function. [ABSTRACT FROM AUTHOR]

Aims Little is known about the adaptation of the right ventricle (RV) to endurance exercise in children. The aim of this study was to assess the effects of 5 months of intensive training on RV morphology and function in preadolescent endurance athletes. Methods Ninety-four children were evaluated in this study. Fifty-seven male competitive swimmers (aged 10.8 ± 0.2 years) were evaluated before (baseline) and after 5 months of the training (peak-training), and compared to 37 age- and sex-matched non-athlete children evaluated at baseline and after 5 months of natural growth. All subjects were asymptomatic, with negative family history for cardiomyopathies. Results At baseline no differences were found between athletes and controls for indexed RV outflow tract (RVOT) (18.5 ± 2.7 vs. 16.8 ± 5.0 mm/m 2 , p = 0.18) and RV basal end-diastolic diameter (EDD) (24.9 ± 4.1 vs. 23.6 ± 3.0 mm/m 2 , p = 0.15). After 5 months, indexed RVOT and RV basal EDD significantly increased in athletes (20.2 ± 2.9 mm/m 2 and 25.4 ± 3.3 mm/m 2 , p < 0.0001 vs. baseline) while no differences were observed in controls ( p = 0.84 and p = 0.25). Despite the increase in RV size, RV function remained normal in athletes, with no changes in RV fractional area change ( p = 0.97), s′ value ( p = 0.22), and RV longitudinal strain ( p = 0.28). Conclusions Endurance training influences the growing heart of male preadolescent athletes with an addictive increase in RV dimensions, with a preserved RV function. Therefore, in children engaged in endurance sports the increase in RV size associated with normal RV function represents a physiological expression of the athlete's heart and should not be misinterpreted as an expression of incipient RV cardiomyopathy. [ABSTRACT FROM AUTHOR]

Background Radiofrequency catheter ablation (RFCA) is a potentially curative treatment for atrial fibrillation (AF), however, whether or not additional left atrial (LA) linear ablation for recurrent AF adversely affects LA remodeling is unknown. Methods Thirty-eight patients experiencing AF recurrence after the 1st circumferential pulmonary vein isolation (CPVI) underwent a repeat RFCA, including 20 and 18 patients receiving a repeat CPVI (group I) or CPVI plus LA linear ablation (group II), respectively. 2-D echocardiography was performed during sinus rhythm within 24 h, at 1-m and 6-m after RFCA. Longitudinal strains and strain rate were measured with speckle-tracking echocardiography. The standard deviation of contraction duration was defined as LA mechanical dispersion. Results One and two patients experienced AF recurrence after the 2nd RFCA in group I and II, respectively (P = NS). The 1st CPVI with AF recurrence did not reduce LA size significantly in two groups. After a repeat CPVI, LA diameter but not LA maximal and minimal volume was significantly reduced in group I; additional LA linear ablation significantly decreased LA diameter, maximal and minimal volume in group II. However, there was no significant difference in LA emptying function, global and segmental LA strain and strain rate among the baseline, 1-m and 6-m follow-up in two groups. RFCA did not significantly increase LA mechanical dispersion regardless of the AF ablation strategies. Conclusions In patients with recurrent AF, a successful repeat CPVI with or without additional LA linear ablation reduced LA size without significant deleterious effects on LA function and mechanical dispersion. [ABSTRACT FROM AUTHOR]

Aims Previous studies investigated the exercise-induced adaptation of left (LA) and right atrium (RA) in adults, but little is known about respective changes in the growing heart of children. We aimed to longitudinally investigate the effects of endurance training on biatrial remodelling in preadolescent athletes. Methods and results Ninety-four children (57 endurance athletes, 37 sedentary controls; mean age 10.8 ± 0.2 and 10.2 ± 0.2 years, respectively) were evaluated at baseline and after 5 months by ECG and by two-dimensional, three-dimensional (3D) and speckle-tracking echocardiography. Athletes were trained at least 10 h/week. The resting heart rate was lower in athletes ( p = 0.046) and decreased further after training ( p < 0.0001). Neither athletes nor controls had ECG evidence for LA or RA enlargement. At baseline, indexed LA volumes did not differ between groups ( p = 0.14) but indexed RA dimensions were larger in athletes ( p = 0.007). After 5 months, indexed LA volumes increased in athletes but not in controls ( p < 0.0001, p = 0.29; respectively) while indexed RA volumes increased in both groups (p < 0.0001, p = 0.018; respectively). At the same time, slight differences in biatrial reservoir and contractile function were found either in athletes, as demonstrated by speckle-tracking echocardiography, but 3D-derived LA and RA ejection fraction remained stable in both groups. Conclusion Endurance training influences the growing heart of preadolescent athletes with an additive increase in biatrial size, suggesting that morphological adaptations can occur also in the early phases of the sports career. Training-induced remodelling was associated with a preserved biatrial function, supporting the hypothesis of a physiological remodelling. [ABSTRACT FROM AUTHOR]