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Abstract Background Takotsubo cardiomyopathy is an acute cardiac syndrome characterized by transient LV regional wall motion abnormalities (with peculiar apical ballooning appearance), chest pain or dyspnea, ST-segment elevation and minor elevations of cardiac enzyme levels Case presentation A 68-year-old woman was admitted to the Emergency Department because of sudden onset chest pain occurred while transferring her daughter, who had earlier suffered a major seizure, to the hospital. The EKG showed sinus tachycardia with ST-segment elevation in leads V2–V3 and ST-segment depression in leads V5–V6, she was, thus, referred for emergency coronary angiography. A pre-procedural transthoracic echocardiogram revealed regional systolic dysfunction of the LV walls with hypokinesis of the mid-apical segments and hyperkinesis of the basal segments. Coronary angiography showed patent epicardial coronary arteries; LV angiography demonstrated the characteristic morphology of apical ballooning with hyperkinesis of the basal segments and hypokinesis of the mid-apical segments. The post-procedural course was uneventful; on day 5 after admission the echocardiogram revealed full recovery of apical and mid-ventricular regional wall-motion abnormalities. Conclusion Takotsubo cardiomyopathy is a relatively rare, unique entity that has only recently been widely appreciated. Acute stress has been indicated as a common trigger for the transient LV apical ballooning syndrome, especially in postmenopausal women. The present report is a typical example of stress-induced takotsubo cardiomyopathy in a Caucasian Italian postmenopausal woman.

Background and Purpose: Atrial fibrillation (AF) has a high prevalence in the population and it is responsible for up to the 25% of the strokes in elderly people. The aim of our study was to assess the correlations of left atrial (LA) functional parameter, global peak atrial longitudinal strain (PALS), derived from speckle tracking echocardiography (STE), with transesophageal echocardiography (TEE) findings in patients with persistent AF undergoing TEE before electrical cardioversion or ablation procedures. Methods: 79 patients (58 males, 21 females) with persistent AF waiting for cardioversion were included in the study. The patients underwent conventional two-dimensional (2D) and 2D speckle tracking echocardiogram. PALS were measured in all subjects. Patients were divided into two groups according to the presence of reduced LA appendage (LAA) emptying velocity (<25 cm/s) and/or thrombus in the LAA at TEE examination. Results: Patients with reduced LAA emptying velocity and/or thrombus at TEE examination showed a significantly higher LA volume and increased E/E’ ratio. 4-chamber, 2-chamber and global PALS were significantly lower in patients with reduced LAA emptying velocity and/ or thrombus (6.8 ± 2.0% vs. 27.5 ± 5.4%, P < 0.0001; 8.6 ± 3.5% vs. 29.4 ± 7.1%, P < 0.0001; 7.9 ± 3.2% vs. 28.5 ± 6.1%, P <0.0001, respectively). Among all variables analyzed, global PALS demonstrated the highest diagnostic accuracy (AUC of 0.92) and, with a cut-off value less than 8.1%, good sensitivity and specificity of 87% and 94%, respectively, to predict LAA thrombus and/or reduced LAA emptying velocity. Conclusion: Among all parameters derived from transthoracic echocardiography, global PALS was the best predictor of LAA thrombus and/or reduced LAA emptying in patients with persistent non-valvular AF undergoing TEE before electrical cardioversion or ablation procedures. [ABSTRACT FROM AUTHOR]

Rett syndrome (RTT) is a devastating neurodevelopmental disorder with a 300-fold increased risk rate for sudden cardiac death. A subclinical myocardial biventricular dysfunction has been recently reported in RTT by our group and found to be associated with an enhanced oxidative stress (OS) status. Here, we tested the effects of the naturally occurring antioxidants ω-3 polyunsaturated fatty acids (ω-3 PUFAs) on echocardiographic parameters and systemic OS markers in a population of RTT patients with the typical clinical form. A total of 66 RTT girls were evaluated, half of whom being treated for 12 months with a dietary supplementation of ω-3 PUFAs at high dosage (docosahexaenoic acid ∼71.9 ± 13.9mg/kg b.w./day plus eicosapentaenoic acid ∼115.5 ± 22.4mg/kg b.w./day) versus the remaining half untreated population. Echocardiographic systolic longitudinal parameters of both ventricles, but not biventricular diastolic measures, improved following ω-3 PUFAs supplementation, with a parallel decrease in the OS markers levels. No significant changes in the examined echocardiographic parameters nor in the OS markers were detectable in the untreated RTT population. Our data indicate that ω-3 PUFAs are able to improve the biventricular myocardial systolic function in RTT and that this functional gain is partially mediated through a regulation of the redox balance. [ABSTRACT FROM AUTHOR]

Background Methods Results Conclusion Biventricular pacing (BIV) is the gold standard for cardiac resynchronization therapy (CRT). Thirty percent of patients do not respond to CRT. Conduction system pacing (CSP) represents a viable alternative. Interventricular conduction delay (IVCD), as electrical desynchrony marker, is a CRT response predictor. The aim of this study was to determine the incidence of CRT responders by selecting the best approach between BIV and CPS based on intraoperative IVCD measurement in patients with HFrEF and LBBB.Ninety‐six patients were randomly assigned in a 1:1 ratio to either a standard BIV group(control group, CG) or a group where the CRT approach was determined based on IVCD evaluation(study group, SG). If the right ventricular sensed electrogram (RVs)–left ventricular sensed electrogram (LVs) interval was ≥100 ms, the lead was left in its original position; otherwise, the LV lead was removed, and CSP was performed instead. Clinical, EKG, and echocardiographic features have been assessed pre‐ and 6 months post‐implant. Echocardiographic and clinical responder were evaluated.Thirty‐seven percent of patients in the SG underwent CSP, as the operative algorithm. The incidence of CRT responders was significantly higher in the SG (echocardiographic criterion: 92.5% vs. 69.8%, p:.009; clinical criterion 87.5% vs. 62.8%, p:.014). The SG showed a significantly greater difference in EF between pre‐ and post‐implant as well as reduced end‐diastolic and systolic volumes. Univariate and multivariate regression analysis indicated that enrollment in the SG was the only factor associated with CRT response.Intraoperative assessment of IVCD could help determine the optimal CRT approach between BIV and CSP, leading to a significant improvement in the rate of CRT responders. [ABSTRACT FROM AUTHOR]

Electrical storm (ES) is defined as three or more episodes of sustained ventricular tachycardia (VT) or fibrillation (VF) within 24 h, or an incessant VT/VF lasting more than 12 h. It usually occurs in implantable cardioverter-defibrillator (ICD) recipients, and three or more device interventions are typically used for the diagnosis. ES incidence is particularly high in case of ICD implanted in secondary prevention (10-30%), with recurrences occurring in up to 80% of patients. A comprehensive evaluation of triggers, predictive factors of high-risk patients and an appropriate management of the acute/subacute and chronic phases are pivotal to reduce mortality and recurrences. Medical therapy with antiarrhythmic and anesthetic drugs, with appropriate device reprogramming and neuroaxial modulation if needed, are used to cool down the ES, which should ultimately be treated with ablation therapy or, less often, with an alternative treatment, such as denervation or stereotactic radiosurgery. An optimization of the clinical pathway in a network modeling is crucial to achieve the best treatment, eventually addressing patients to centers with VT ablation programs, and identifying the most challenging procedures and the most critical patients that should be treated only in high-volume tertiary centers. In this paper, we present a proposal of healthcare network modeling for ES treatment in a regional setting.

We describe the case of a patient with a mitral bioprosthetic valve undergoing percutaneous closure with fluoroscopy and echocardiographic guidance of a rare left sinus of Valsalva‒left atrial fistula. The original aspect of this case is that the fistula was located at the level of the left sinus of Valsalva next to the mitral valve bioprosthesis with subsequent risk of prosthesis damage and/or occlusion of the left coronary artery at its origin during the procedure.

Background: Levosimendan improves clinical and hemodynamic parameters exerting an anti-inflammatory and antiapoptotic effect in decompensated heart failure. The aim of this study was to evaluate the effects of levosimendan on LV torsion, plasma levels of NT-proBNP and on the balance between pro-inflammatory (TNF-α, IL-6) and anti-inflammatory cytokines (IL-10)., Methods: We enrolled 24 patients (age 62 ± 7 years) with acute HF, NYHA class IV and severe LV dysfunction. All patients underwent transthoracic echocardiography using two-dimensional speckle tracking analysis to detect LV twist angle (LVTA), at baseline and 1 week after treatment with levosimendan infusion. Biochemical parameters (pro-BNP, IL-6, IL-10, TNF-α) were determined by enzyme-linked immunosorbent (ELISA)., Results: After one week, we observed an improvement in LV function especially in LVTA (4.15 ± 2.54 vs 2.9 ± 2.1 p < 0.01), in LV ejection fraction (27.3 ± 8.04 vs 21.6 ± 6.8 p = 0.03) and also a significant reduction in BNP levels (1844 ± 560 vs 4713 ± 1050, p = 0.03). The multiple linear regression analysis showed a significant relation between a reduction of TNF α/IL-10 ratio (Δ > 20%) and BNP (Δ > 40%), LVEF (Δ > 10%) and LVTA (Δ > 20%) (O.R. 1.77, 95% C.I. 1.11-2.83; O.R. 1.49, 95% C.I. 1.08-2.67; O.R. 1.66, 95% C.I. 1.10-2.74, respectively, confirmed p, all < 0.01 by Hosmer-Lemeshov confirmation and the formal test for interaction)., Conclusions: Levosimendan exerts a hemodynamic effect by improving EF and LV torsion in patients with acute HF in association with a positive effect on the balance between pro and anti-inflammatory cytokines.

Aims: Rett syndrome (RTT) is a rare neurodevelopmental disorder frequently linked to methyl-CpG-binding protein 2 (MeCP2) gene mutations. RTT is associated with a 300-fold increased risk of sudden cardiac death. Rhythm abnormalities and cardiac dysautonomia do not to fully account for cardiac mortality. Conversely, heart function in RTT has not been explored to date. Recent data indicate a previously unrecognized role of MeCP2 in cardiomyocytes development. Besides, increased oxidative stress markers (OS) have been found in RTT. We hypothesized that (i) RTT patients present a subclinical biventricular dysfunction and (ii) the myocardial dysfunction correlate with OS., Methods and Results: We evaluated typical (n = 72) and atypical (n = 20) RTT female and healthy controls (n = 92). Main outcome measurements were (i) echocardiographic biventricular systo-diastolic parameters; (ii) correlation between echocardiographic measures and OS levels, i.e. plasma and intra-erythrocyte non-protein-bound iron (NPBI) and plasma F2-Isoprostanes (F2-IsoPs). A significant reduction in longitudinal biventricular function (tricuspid annular plane systolic excursion, mitral annular plane systolic excursion, S' of lateral and septal mitral annulus, S' of tricuspidal annulus) was evidenced in RTT patients vs. controls. No significant changes in the LV ejection fraction were found. Peak-early filling parameters (E, E' of lateral mitral annulus, E' of tricuspidal annulus) and right ventricular systolic pressure were reduced. A-wave, E/A, and E/E' were normal. OS markers were increased, but only F2-IsoPs correlated to LV systolic dysfunction., Conclusion: These data indicate a previously unrecognized subclinical systo-diastolic biventricular myocardial dysfunction in typical and atypical RTT patients. A reduced preload is evidenced. The biventricular dysfunction is partially related to OS damage.

Papillary muscle rupture is an infrequent but often fatal mechanical complication of acute myocardial infarction (AMI). We report the case of an AMI complicated by the development of an abrupt cardiogenic shock due to the rupture of the head of the postero-medial papillary muscle with echocardiographic demonstration of severe mitral regurgitation due to flail posterior mitral valve leaflet. After initial stabilization with medical therapy and diagnostic coronary angiography, the patient was referred for urgent cardiac surgery and successfully underwent mitral valve replacement with implantation of a bioprosthesis. This case confirms the importance of transthoracic echocardiography in diagnosing mechanical acute complications during an AMI and in the decision making of patients with sudden onset of hemodynamic compromise. Transthoracic echocardiography should be immediately carried out in all patients in whom a mechanical complication during an AMI is suspected.

Syncope and pre-syncope represent common symptoms in patients with hypertrophic cardiomyopathy. The clinical scenario of recurrent and frequent syncopal episodes is one of the most challenging in the management of patients with hypertrophic cardiomyopathy. Syncope in hypertrophic cardiomyopathy patients usually recognizes two underlying mechanisms: arrhythmias or primary hemodynamic mechanism. The concomitant presence of myocardial ischemia is one of the potential triggers in the context of syncope due to hemodynamic mechanism. Myocardial ischemia is not generally related to epicardial coronary artery atherosclerotic disease but rather to the presence of partially obliterated intramural coronary arteries or narrowed vessels. We report the rare case of a patient with hypertrophic cardiomyopathy presenting with recurrent syncopal episodes due to myocardial ischemia secondary to severe stenosis of the left anterior descending coronary artery.