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Aims: Pulmonary artery pulsatility index (PAPi) is an indicator of right ventricular (RV) function and an independent predictor of right ventricular failure (RVF) following left ventricular assist device (LVAD) implantation. Administration of vasodilator challenge during right heart catheterization (RHC) could reduce RV workload allowing a better assessment of its functional reserve., Methods and Results: Patients undergoing LVAD implantation at our Institution between May 2013 and August 2021 were enrolled. Only patients who had undergone RHC and vasodilator challenge with sodium nitroprusside were analyzed. We collected all available clinical, instrumental, and haemodynamic parameters, at baseline and after nitroprusside infusion and evaluated potential associations with post-LVAD RVF. Of the 54 patients analyzed, 19 (35%) developed RVF after LVAD implantation. Fractional area change (FAC) (OR: 0.647, CI: 0.481-0.871; P = 0.004), pulmonary artery systolic pressure (PASP) (OR: 0.856, CI: 0.761-0.964; P = 0.010), and post-sodium nitroprusside (NTP) PAPi (OR: 0.218, CI: 0.073-0.653; P = 0.006) were independent predictors of post-LVAD RVF. The model combining FAC, PASP, and post-NTP PAPi demonstrated a predictive accuracy of 90.7%. Addition of post-NTP PAPi significantly increased the predictive accuracy of the European Registry for Patients with Mechanical Circulatory Support right-sided heart failure risk score [79.4 vs. 70.4%; area under the curve (AUC): 0.841 vs. 0.724, P = 0.022] and the CRITT score (79.6% vs. 74%; AUC: 0.861 vs. 0.767 P = 0.033)., Conclusion: Post-NTP PAPi has observed to be an independent predictor of RVF following LVAD implantation. Dynamic assessment of PAPi using a vasodilator challenge may represent a method of testing RV functional reserve in candidates for LVAD implantation. Larger and prospective studies are needed to confirm this hypothesis., Competing Interests: Conflict of interest: None declared., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Echocardiography is the most common diagnostic tool to screen for Fabry cardiomyopathy as it is fast, non-invasive, low-cost, widely available, easily applicable and reproducible. Echocardiography is the first-line investigation, being useful in all the stages of the disease: (1) in gene-positive patients, to unveil signs of early cardiac involvement and allowing timely treatment; (2) in patients with overt cardiomyopathy to estimate the severity of cardiac involvement, the possible related complications, and the effect of treatment. Recently, advanced echocardiographic techniques, such as speckle tracking analysis, are offering new insights in the assessment of Fabry disease patients and in the differential diagnosis of cardiomyopathies with hypertrophic phenotype. The aim of this review is to provide a comprehensive overview on the cardiac structural and functional abnormalities described in Fabry disease by means of echocardiography., Competing Interests: Francesca Graziani: Honoraria for presentations, board meetings and travel support from Amicus Therapeutics, Sanofi-Genzyme and Takeda. Antonia Camporeale: Honoraria for presentations and board meetings from Amicus Therapeutics, Sanofi-Genzyme and Takeda. Research grant from Amicus Therapeutics. Maurizio Pieroni: advisory board honoraria from Amicus Therapeutics and Sanofi Genzyme; he has received speaker honoraria from Amicus Therapeutics, Sanofi Genzyme, and Takeda. Rosa Lillo: Honoraria for board meetings and travel support from Amicus Therapeutics, Sanofi-Genzyme and Takeda. Maurizio Pieroni is serving as one of the Editorial Board members/Guest editors of this journal. We declare that Maurizio Pieroni had no involvement in the peer review of this article and has no access to information regarding its peer review. Full responsibility for the editorial process for this article was delegated to Jerome L. Fleg. Other authors have no conflicts of interest., (Copyright: © 2022 The Author(s). Published by IMR Press.)

In patients with Fabry disease (FD), cardiovascular involvement is the main cause of death and reduction of quality of life. Left ventricular hypertrophy mimicking hypertrophic cardiomyopathy is the main feature of FD cardiac involvement although glycolipid storage occurs in all cardiac cellular types. Accumulation of lysosomal globotriasylceramide represents the main mechanism of cardiac damage in early stages, but secondary pathways of cellular and tissue damage, triggered by lysosomal storage, and including altered energy production, inflammation and cell death, contribute to cardiac damage and disease progression. These mechanisms appear prominent in more advanced stages, hampering and reducing the efficacy of FD-specific treatments. Therefore, additional cardiovascular therapies are important to manage cardiovascular symptoms and reduce cardiovascular events. Although new therapies targeting lysosomal storage are in development, a better definition and comprehension of the complex pathophysiology of cardiac damage in FD, may lead to identify new therapeutic targets beyond storage and new therapeutic strategies., Competing Interests: Maurizio Pieroni: advisory board honoraria from Amicus Therapeutics and Sanofi Genzyme; he has received speaker honoraria from Amicus Therapeutics, Sanofi Genzyme, and Takeda. Francesca Graziani: Honoraria for presentations, board meetings and travel support from Amicus Therapeutics, Sanofi-Genzyme and Takeda. Antonia Camporeale: Honoraria for presentations and board meetings from Amicus Therapeutics, Sanofi-Genzyme and Takeda. Research grant from Amicus Therapeutics. Rosa Lillo: Honoraria for board meetings and travel support from Amicus Therapeutics, Sanofi-Genzyme and Takeda. Maurizio Pieroni is serving as one of the Editorial Board members/Guest editors of this journal. We declare that Maurizio Pieroni had no involvement in the peer review of this article and has no access to information regarding its peer review. Full responsibility for the editorial process for this article was delegated to Sophie Mavrogeni. Other authors have no conflicts of interest., (Copyright: © 2022 The Author(s). Published by IMR Press.)

In the past few years, the wide application of cardiac magnetic resonance (CMR) significantly changed the approach to the study of cardiac involvement in Fabry Disease (FD). The possibility to perform non-invasive tissue characterization, including new sequences such as T1/T2 mapping, offered a powerful tool for differential diagnosis with other forms of left ventricular hypertrophy. In patients with confirmed diagnosis of FD, CMR is the most sensitive non-invasive technique for early detection of cardiac involvement and it provides new insight into the evolution of cardiac damage, including gender-specific features. Finally, CMR multiparametric detection of subtle changes in cardiac morphology, function and tissue composition is potentially useful for monitoring the efficacy of specific treatment over time. This paper aims to provide a comprehensive review of current knowledge regarding the application of CMR in FD cardiac involvement and its clinical implication., Competing Interests: AC: Honoraria for presentations and board meetings from Amicus Therapeutics, Sanofi-Genzyme and Shire. Research grant from Amicus Therapeutics. MP: speaker and advisory board honoraria, and travel support from Sanofi-Genzyme, Amicus Therapeutics and Shire. AD, GP, LT, SP, MC: The authors declare no conflict of interest. FG: travel sponsor from Genzyme, Shire and Amicus, honoraria speaker for Takeda-Shire and Genzyme. ML: Honoraria for presentations from Shire. MP is serving as one of the Editorial Board members/Guest editors of this journal. We declare that MP had no involvement in the peer review of this article and has no access to information regarding its peer review. Full responsibility for the editorial process for this article was delegated to Grigorios Korosoglou and Sophie Mavrogeni., (Copyright: © 2022 The Author(s). Published by IMR Press.)

Purpose of Review: Histologic evidence of myocardial inflammatory infiltrate not secondary to an ischemic injury is required by current diagnostic criteria to reach a definite diagnosis of myocarditis. Endomyocardial biopsy (EMB) is therefore often indicated for the diagnosis of myocarditis, although it may lack sufficient sensitivity considering the limited possibility of myocardial sampling. Improving the diagnostic yield and utility of EMB is of high priority in the fields of heart failure cardiology and myocarditis in particular. The aim of the present review is to highlight indications, strengths, and shortcomings of current EMB techniques, and discuss innovations currently being tested in ongoing clinical studies, especially in the setting of acute myocarditis and chronic inflammatory cardiomyopathy., Recent Findings: EMB provides unique diagnostic elements and prognostic information which can effectively guide the treatment of myocarditis. Issues affecting the diagnostic performance in the setting of acute myocarditis and chronic inflammatory cardiomyopathies will be discussed in this review in the light of recent expert consensus documents on the management of these conditions and on indication to EMB. Recent innovations using electroanatomic mapping (EAM)-guided EMB and fluoroscopic-guided EMB during temporary mechanical circulatory support have improved the utility of the procedure. EMB remains an important diagnostic test whose results need to be interpreted in the context of (1) clinical pre-test probability, (2) timing of sampling, (3) quality of sampling (4) site of sampling, (5) histologic type of myocarditis, and (6) analytic methods that are applied. Herein we will review these caveats as well as perspectives and innovations related to the use of this diagnostic tool., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objectives: We sought to determine whether, in a real word context of patients with Acute Myocardial Infarction (AMI), multivessel disease (MVD) and cardiogenic shock (CS), the successful treatment with primary percutaneous coronary intervention (p-PCI) of only culprit lesions (OC-PCI) is associated with better long-term mortality rates than multivessel PCI (MV-PCI) of all significant lesions., Methods: From our registry of all consecutive patients admitted for AMI between January 1995 and December 2016 we selected those presenting with CS and MVD successfully treated with p-PCI, and compared those who underwent OC-PCI against MV-PCI, either during the p-PCI (MV-pPCI) or by staged revascularization (Staged-PCI) during hospitalization. The primary endpoint was 2-year all-cause death., Results: Among 4210 patients with AMI, 406 (9.6%) presented CS (Killip class IV). A total of 292 patients had MVD. Of them, 252 (86.3%) were successfully treated with p-PCI, 159 patients with OC-PCI and 93 with MV-PCI, either in the same (n = 29) or staged procedure (n = 64). At 2-year follow-up the overall mortality was 47.6%, lower in MV-PCI group (37.6% vs 53.5% in OC-PCI, p = 0.019). Diabetes (HR = 1.50, 1.01-2.22), three vessel disease (HR = 1.49, 1.02-2.17) and basal left ventricular ejection fraction <15% (HR = 3.39, 2.41-6.27) were independent predictors of mortality, while MV-PCI was the only variable associated with improved survival (HR = 0.54, 0.36-0.81)., Conclusions: In this real world registry of AMI patients with MVD presenting CS, MV-PCI was associated with better long-term survival., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Despite numerous studies assessing the natural history of patients with hypertrophic cardiomyopathy (HCM), there is lack of data regarding the burden of hospitalization. Aim of this study was to describe prevalence, causes and predictors of cardiovascular hospitalization in patients with HCM., Methods: We retrospectively included 253 patients with HCM undergoing first evaluation at our center. Enrolment criteria included cardiac magnetic resonance imaging (CMRI) at baseline and > 1-year follow-up. All hospital admissions were recorded during follow-up and adjudicated as acute vs elective and cardiovascular (CV) vs non-cardiovascular (non-CV)., Results: During 6.4 ± 4.0 years there were 187 hospitalizations in 92 patients (36%, at a rate of 5.7%/year). Most were CV-related (158/187,84.5%; 4.8%/year) while non-CV admissions were 29/187 (15.5%, 0.88%/year). There was a slight predominance of elective (n = 96, 58%, 2.8%/year) vs acute (n = 62, 41.8%, 2.0%/year) CV hospitalizations. Independent predictors of CV hospitalization were baseline symptoms (NYHA class II vs I: HR 2.06; 95% CI 1.24-3.43, NYHA III-IV vs I: HR 3.05; 95% CI 1.40-6.65, p = .004), indexed left atrial (LA) volume (HR 1.03; 95% CI 1.01-1.04, p < .001), and lower indexed right ventricular end-diastolic volume iRVEDV) at cardiac magnetic resonance (HR 0.99; 95% CI 0.97-0.99, p = .03)., Conclusions: In little over 6 years, CV hospitalization was required in over one-in-three of our HCM patients, often unplanned and due to acute disease-related complications. Symptomatic status, larger LA volume and reduced iRVEDV at baseline were independently associated with CV admissions. Strategies aimed at preventing hospitalizations are an important target to reduce the burden of disease in HCM patients., Competing Interests: Declaration of Competing Interest The authors report no relationships that could be construed as a conflict of interest., (Copyright © 2020 Elsevier B.V. All rights reserved.)

The management of ventricular arrhythmias (VA) in the presence of an apparently normal heart represents a major clinical challenge and a main field of clinical research. In the past years, new imaging techniques and the spreading of new generation genetic testing have improved our knowledge of the pathogenesis of apparently idiopathic VA. However, in the absence of specific recommendations, the type and the number of noninvasive and invasive studies necessary to rule out a possible underlying cause of VA or sudden cardiac death remain extremely variable. Therefore, in many patients the underlying cardiac disease is not recognized, and a possible specific therapeutic approach cannot be initiated. Endomyocardial biopsy (EMB) can provide a significant contribution to the identification of myocardial disorders causing VA but has never been definitively included in the routine diagnostic work-up of these patients due to the possible sampling error particularly in disorders with a focal or patchy distribution. Three-dimensional electroanatomic mapping (EAM) may guide EMB allowing to draw myocardial samples from abnormal voltage, areas of the ventricular wall, thus reducing sampling error and increasing the sensitivity of EMB. The systematic association of EAM with EMB represents a crucial approach to characterize the pathological substrate of electroanatomic abnormalities and VA and to further clarify the arrhythmogenic mechanisms of acquired and also inherited arrhythmic disorders., (© 2020 Wiley Periodicals LLC.)

Introduction: It is not clear if differences exist about treating left main bifurcation (LMB) and non-left main bifurcation (non-LMB) lesions by means of percutaneous coronary intervention (PCI)., Methods: We prospectively analyzed all consecutive patients treated at our center for bifurcation lesions from January 1, 2011 to December 31, 2015, including acute myocardial infarction (MI) and cardiogenic shock, and compared the angiographic and clinical outcomes of patients with LMB and non-LMB lesions treated with PCI and second-generation drug-eluting stent (2G-DES) implantation. The primary endpoint was the major adverse cardiac event (MACE) composite, including MI, clinically indicated target-vessel revascularization (TVR), and cardiac death (CD) at 2-year follow-up. We also compared the angiographic patency of the vessel, which was a composite of the restenosis-reocclusion (RR) rate., Results: Out of 1081 patients (1368 bifurcations), a total of 320 patients had LMB (29%). Overall, procedural success was 98.4%. Clinical follow-up rate was 100%. Angiographic follow-up rate was 83.7%. No differences were seen regarding the primary endpoint of all MACE (17.8% in LMB vs 18.0% in non-LMB; P>.99), MI rate (4.3% in LMB vs 2.9% in non-LMB; P=.20), and CD (8.7% in LMB vs 5.8% in non-LMB; P=.08). The overall RR rate was 11.8%, with 5% RR rate in the LMB group (16/320 lesions) and 9.7% RR rate in the non-LMB group (102/1048 lesions); P<.01. The LMB group had a better TVR rate (5.0% vs 9.4% in the non-LMB group; P=.01)., Conclusion: PCI with 2G-DES for LMB has better target-vessel patency and TVR rates when compared with non-LMB lesions, without clinical differences in terms of 2-year clinical outcomes.

The present study evaluated short- and long-term efficacy and tolerability of augmentative vagus nerve stimulation (VNS) in a group of patients with treatment-resistant depression (N = 6). A statistically significant improvement in the Hamilton Depression Rating Scale (HDRS21) and Montgomery-Asberg Depression Rating Scale after 3 months (P = 0.039 and P = 0.05, respectively) was found in comparison with baseline (VNS implant). After 12 months, a statistically significant improvement was observed in the Hamilton Depression Rating Scale (HDRS21), Montgomery-Asberg Depression Rating Scale, and Clinical Global Impression (P = 0.01, P = 0.005, and P = 0.001, respectively). Patients showed an overall favorable tolerability. Present data support VNS short- and long-term efficacy and tolerability in a small group of patients with treatment-resistant depression. Further controlled investigation is necessary to confirm the present open findings.

Background: : The efficacy of repetitive transcranial magnetic stimulation (rTMS) has been poorly investigated in the long-term. The present follow-up study was aimed to assess the long-term efficacy and the discontinuation effects of rTMS in a sample of depressed bipolar patients., Methods: : After the completion of an acute trial with augmentative, low-frequency, navigated rTMS, 11 drug-resistant depressed bipolar subjects (Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition [Text Revision] criteria) entered a naturalistic follow-up with monthly evaluations through the Hamilton Depression Rating Scale and the Young Mania Rating Scale., Results: : After 1 year of follow-up, results showed that the achievement of remission after acute rTMS was predictive of maintenance of response at 1 year. On the other hand, the absence of acute rTMS response predicted the absence of subsequent response in the long-term., Conclusions: : This first report on the long-term discontinuation effects after acute rTMS suggests that immediate remission is predictive of sustained benefit after 1 year. Larger controlled studies are needed to confirm present preliminary findings.

Objective: The study evaluates the association between subjective well-being and psychopathology in bipolar inpatients at the time of hospitalization and during a follow-up period., Method: One hundred twenty consecutive inpatients with a diagnosis of bipolar affective disorder were studied on admission (T0), at discharge (T1) and every 6 weeks for 18 weeks after hospitalization. The Young's Mania Rating Scale (YMRS) and the Hamilton Rating Scale for Depression (HAM-D) were used to determine affective symptoms, while subjective well-being was assessed by subjective well-being under neuroleptic (SWN). Associations between SWN and HAM-D or YMRS scores and between their changes were analyzed across the different time points by using Pearson correlation coefficients. Linear regression models were constructed using SWN as the dependent variable and demographic and clinical characteristics as possible predictors., Results: At baseline, depression explained 24% and mania explained an additional 16% of baseline SWN variance. Changes in SWN and HAM-D total score displayed an inverse correlation during hospitalization and follow-up. End point severity of depression was associated with the end point SWN total score explaining additional 26% of SWN total score variance, whereas severity of mania was inversely associated with SWN total score., Conclusion: Data of this study provide further support for the need to consider the subjective well-being as a personal variable associated to psychopathological state in bipolar patients. However, results seem to be in line with authors who suggest to use other subjective quality of life scales in acute mania., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objectives: The efficacy of transcranial magnetic stimulation (TMS) has been poorly investigated in bipolar depression. The present study aimed to assess the efficacy of low-frequency repetitive TMS (rTMS) of the right dorsolateral prefrontal cortex (DLPFC) combined with brain navigation in a sample of bipolar depressed subjects., Methods: Eleven subjects with bipolar I or bipolar II disorder and major depressive episode who did not respond to previous pharmacological treatment were treated with three weeks of open-label rTMS at 1 Hz, 110% of motor threshold, 300 stimuli/day., Results: All subjects completed the trial showing a statistically significant improvement on the 21-item Hamilton Depression Rating Scale (HAM-D), Montgomery-Asberg Depression Rating Scale, and Clinical Global Impression severity of illness scale (ANOVAs with repeated measures: F = 22.36, p < 0.0001; F = 12.66, p < 0.0001; and F = 10.41, p < 0.0001, respectively). In addition, stimulation response, defined as an endpoint HAM-D score reduction of > or =50% compared to baseline, was achieved by 6 out of 11 subjects, 4 of whom were considered remitters (HAM-D endpoint score < or = 8). Partial response (endpoint HAM-D score reduction between 25% and 50%) was achieved by 3/11 patients. No manic/hypomanic activation was detected during the treatment according to Young Mania Rating Scale scores (ANOVAs with repeated measures: F = 0.62, p = 0.61). Side effects were slight and were limited to the first days of treatment., Conclusions: Augmentative low-frequency rTMS of the right DLPFC combined with brain navigation was effective and well tolerated in a small sample of drug-resistant bipolar depressive patients, even though the lack of a sham controlled group limits confidence in the results.