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Background Despite the use of optimal medical therapy, HFrEF remains a leading cause of morbidity, mortality and health care costs. The introduction of angiotensin receptor/neprilysin inhibitors (ARNIs) had a revolutionary impact on the treatment of patients with heart failure and reduced left ventricular ejection fraction (HFrEF). Methods The aim of the study was to monitor over time the perceived quality of life, the physical performance, the trend of BNP and NT-ProBNP and the NYHA functional class in patients with HFrEF during treatment with sacubitril/valsartan. We enrolled 37 patients (63 ± 10 years old, 76% men) who underwent a total of one year follow-up. All patients underwent clinical evaluation, 6MWT, blood analysis (in particular NT-pro-BNP and BNP, renal function test); Kansas City Cardiomyopathy Questionnaire (KCCQ) and the NYHA functional class assessment were also performed, at the beginning of the study and after 3, 6 and 12 months of therapy. Results We observed at each follow-up a significant improvement of KCCQ score, 6MWT, NTProBNP, BNP and of NYHA class. However, analyzing the Δ % of variation of each single parameter, the improvement was not uniform in time. We also observed that only 37% of patients tolerated the full recommended dose of sacubitril/valsartan (97/103 mg b.i.d.); of the remaining, 40% tolerated the intermediate dose (49/51 mg b.i.d.) and 23% the minimum (24/26 md b.i.d.). Conclusions Sacubitril/valsartan therapy improves significantly quality of life, physical effort resistance, BNP and NT-ProBNP and NYHA functional class in patients with HFrEF. Although not all the patients tolerated the maximum recommended dose, the beneficial effects were significant even at lower doses.

Stage A and B heart failure (HF) include asymptomatic patients without and with structural cardiac disorder, respectively. Asymptomatic left ventricular (LV) dysfunction represents an early stage of HF that should be recognized to prevent overt HF development. Echocardiography plays a pivotal role in assessment of cardiac structure and function and represents the ideal imaging technique for screening in the general population, thanks to its availability, feasibility, and low cost. Traditional echocardiography, with LV systolic and diastolic function and cardiac remodeling assessment, is usually performed. Development of new technologies may offer additional information and insights in detection of early LV dysfunction.

Aims Bicuspid aortic valve (BAV) may be complicated by aortic aneurysms and dissection. This study aimed to evaluate the prognostic efficacy of markers from cardiac imaging, as well as genetic and new biomarkers, to early predict aortic complications. Methods and results We re-evaluated after a mean time of 48 ± 11 months 47 BAV patients who had undergone previous echocardiography for evaluation of aortic stiffness and 2D aortic longitudinal strain (LS) (by speckle-tracking analysis), and who had given a blood sample for the assessment of a single-nucleotide polymorphism of elastin gene (ELN rs2 071307) and quantification of elastin soluble fragments (ESF). Surgical treatment of aortic aneurysm/dissection was the primary endpoint, and an aortic dimension increase (of one or more aortic segments) ≥1 mm/year was the secondary endpoint. Nine patients underwent surgical treatment of ascending aorta (AA) aneurysms. Out of the 38 patients who did not need surgical intervention, 16 showed an increase of aortic root and/or AA dimension ≥1 mm/year. At multivariate Cox regression analysis, an impaired AA LS was an independent predictor of aortic surgery [P = 0.04; hazard ratio (HR) 0.961; 95% confidence interval (CI) 0.924–0.984] and aortic dilatation (P = 0.007; HR 0.960; 95% CI 0.932–0.989). An increased quantity of ESF was correlated (P = 0.015) with the primary endpoint at univariate Cox regression analysis but it did not keep statistical significance at multivariate analysis. Conclusion In BAV patients, impairment of elastic properties of the AA, as assessed by 2D LS, is an effective predictor of aortic complications.

Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with a high risk of sudden cardiac death. Three different prediction models for the indication of implanted cardioverter defibrillator (ICD) are now available: the 5 year ARVC risk score, the International Task Force Consensus (ITFC) criteria, and the Heart Rhythm Society (HRS) criteria. We compared these three prediction models in a validation cohort of patients with definite ARVC. Methods and results In a cohort of 140 patients with definite ARVC, the 5 year ARVC risk score and the ITFC and HRS criteria were compared for the prediction of a major combined endpoint of sudden cardiac death, appropriate ICD intervention, resuscitated cardiac arrest, and sustained ventricular tachycardia. During the follow‐up, 65 major events occurred. The 5 year ARVC risk score with a threshold >10%, derived from the maximally selected rank statistic, predicted 62 (95%) events [odds ratio (OR) 9.1, 95% confidence interval (CI) 2.6–32, P = 0.0006], the ITFC criteria 53 (81%, OR 4.8, 95% CI 2.2–10.3, P = 0.0001), and the HRS criteria 29 (45%, OR 4.2, 95% CI 1.9–9.3, P = 0.0003). At the analysis of decision curve for ICD implantation, a 5 year ARVC risk score >10% showed a greater net benefit than the ITFC and HRS criteria over a wide range of threshold probability of events. Finally, at multivariate analysis, the 5 year ARVC risk score >10% was the only independent predictor of major events. Conclusions The 5 year score with a threshold of >10% was more effective for predicting events than the ITFC and HRS criteria.

Cardiac amyloidosis (CA) is cardiomyopathy with a hypertrophic phenotype characterised by diffuse deposition of anomalous fibrillar proteins in the extracellular matrix.To evaluate the prevalence and diagnostic value of extra left ventricle echocardiographic findings in patients with left ventricular (LV) hypertrophic phenotype and amyloid deposition.A group of 146 patients with LV thickness ≥15 mm were enrolled: 70 patients who received a definite diagnosis of sarcomeric hypertrophic cardiomyopathy (HCM group) and 76 patients with transthyretin cardiac amyloidosis (CA group). Echocardiographic analysis of crista terminalis (CriT), atrio-ventricular plane (AVP), mitro-aortic lamina (MAL), anterior ascending aortic wall, interatrial septum (IAS), Eustachian valve (EusV) and coumadin ridge (CouR) was performed in all patients, and these structures were compared among the two groups.CA group showed significantly higher dimensions of CriT, IAS, CouR, AVP, MAL and IAS compared to the HCM group. The logistic analysis showed that LV EF, LV septal thickness, CriT presence, CriT area, MAL and IAS were all predictors of CA in univariate analyses. The stepwise multivariate analysis showed independent predictors of CA: CriT area, MAL and LVEF. According to areas under the receiver operating characteristic curves the best cut-off values to determine CA were identified (IAS9 mm, MAL7 mm, CriT9 mmevidence of extra left ventricle sites of amyloid deposition is a frequent finding in CA. In the context of hypertrophic phenocopies, an increased thickness of IAS, and/or CT and/or MAL should suggest a diagnosis of transthyretin CA.

BackgroundCardiovascular magnetic resonance permits assessment of irreversible myocardial fibrosis and contractile function in patients with previous myocardial infarction. We aimed to assess the prognostic value of myocardial fibrotic tissue with preserved/restored contractile activity.MethodsIn 730 consecutive myocardial infarction patients (64 ± 11 years), we quantified left ventricular (LV) end-diastolic (EDV) and end-systolic (ESV) volumes, ejection fraction (EF), regional wall motion (WM) (1 normal, 2 hypokinetic, 3 akinetic, 4 dyskinetic), and WM score index (WMSI), and measured the transmural (1–50 and 51–100) and global extent of the infarct scar by late gadolinium enhancement (LGE). Contractile fibrotic (CT-F) segments were identified as those showing WM-1 and WM-2 with LGE ≤ or ≥ 50%.ResultsDuring follow-up (median 2.5, range 1–4.7 years), cardiac events (cardiac death or appropriate implantable defibrillator shocks) occurred in 123 patients (17%). At univariate analysis, age, LVEDV, LVESV, LVEF, WMSI, extent of LGE, segments with transmural extent > 50%, and CT-F segments were associated with cardiac events. At multivariate analysis, age > 65 years, LVEF 1.7, and dilated LVEDV independently predicted cardiac events, while CT-F tissue was the only independent predictor of better outcome. After adjustment for LVEF ConclusionsIn addition to CMR imaging parameters associated with adverse outcome (severe LV dysfunction, poor WM, and dilated EDV), the presence of fibrotic myocardium showing contractile activity in patients with previous myocardial infarction yields a beneficial effect on patient survival.

We sought to recognize the blood flow velocity (BFV) through the left anterior descending (LAD) coronary artery and its small intramyocardial (IM) branches by transthoracic Doppler-echocardiography in patients with aortic stenosis (AS). Sixty-two patients, aged 74.0 ± 9.6 years, 37 women, with preserved left ventricular (LV) function, apparently free of active ischemic disease, were enrolled and classified into 3 groups according to the mean gradient (MG) across the aortic valve: 13 patients (21%) entered the group A (MG ≤ 20 mmHg), 29 (48%) group B (MG 21-40 mmHg) and 20 (31%) group C (MG 40 mmHg). Peak and mean coronary BFVs were demonstrated to gradually increase according to AV gradient, especially through the IM arteries. Peak IM-BFV was 58.9 cm/s (95% CI 46.4-71.4) in group A, 73.2 cm/s (95% CI 64.8-81.6) in group B, and 96.4 cm/s (95% CI 86.3-106.5) in group C (p 0.001), whereas peak LAD-BFV was 38.1 cm/s (95% CI 32.8-43.3), 44.4 cm/s (95% CI 40.9-47.9) and 47.3 cm/s (95% CI 43.1-52.5), respectively (p = 0.03). Also, 34 patients complaining with unspecific symptoms showed much higher IM-BFV than those who were not. High values were also recognized in patients with LV ejection fraction/velocity ratio (EFVR) ≤ 0.90 (IM-BFV 91 ± 26 cm/s vs. 72 ± 24 cm/s in those with EFVR 0.90, p = 0.001). In conclusion, AS patients in the present study showed gradually higher coronary BFVs according to AS gradient, especially through the IM vessels, and both peak and mean velocities were discriminating specific patient subsets. Pathophysiological mechanisms and potential clinical implications are discussed.

Hereditary transthyretin amyloidosis (hATTR) is a life-threatening multisystemic disease with sensory-motor peripheral neuropathy, cardiomyopathy and dysautonomia. Although the six-minute walk test (6MWT) is one of the most popular clinical tests to assess functional exercise capacity in cardiopulmonary and neuromuscular diseases, little is known about 6MWT in evaluating hATTR patients. A prospective single-center pilot study was performed in twenty hATTR patients, comparing 6MWT with widely used outcome measures. After 18 months, fourteen patients were re-evaluated. 6MWT performance was highly related with familial amyloidotic polyneuropathy stage and polyneuropathy disability score, and with CMT examination score, neuropathy impairment score-lower limbs and Kumamoto score. There was no correlation with compound autonomic dysfunction test, modified body mass index and numerous indices of heart dysfunction. After 18 months, familial amyloidotic polyneuropathy stage and polyneuropathy disability score systems were not able to reveal any significant change, whereas all other outcome measures significantly worsened. Among the outcome measures monitoring the neuropathic disturbances, neuropathy impairment score-lower limbs showed the highest responsiveness to change (adjusted effect size: 0.79), followed by CMT examination score (0.67), Kumamoto scale (0.65), 6MWT (0.62). 10MWT showed a very small value (0.21). Compound autonomic dysfunction test had a large value (0.91) whereas modified body mass index a small/moderate value (0.49). 6MWT is a simple and sensitive tool to monitor neuropathic involvement but not cardiac dysfunction in hATTR course.

Background: Over the past decade, three new drugs have been approved for the treatment of hereditary amyloid transthyretin (ATTRv) polyneuropathy. The aim of this work was to analyze whether current therapies prolong survival for patients affected by ATTRv amyloidosis. Methods: The study was conducted retrospectively, analyzing the medical records of 105 patients with genetic diagnoses of familial amyloidotic polyneuropathy followed at the two referral centers for the disease in Sicily, Italy. Of these, 71 received disease-modifying therapy, while 34 received only symptomatic treatment or no therapy. Results: The most used treatment in our patient cohort was tafamidis, followed by liver transplantation, patisiran, inotersen, and diflunisal. The median survival was significantly longer for treated vs. untreated patients (12 years vs. 8 years). In the 71 patients who received disease-modifying treatment, the presence of cardiac involvement, weight loss, or autonomic dysfunction at diagnosis was not related to survival. Conversely, patients diagnosed in the early stage of the disease (PND 1) had significantly longer survival than those diagnosed in the late stage (PND 2–4).

Background We aimed to determine predictors and the additive prognostic role of moderate to severe (MS) ischemic mitral regurgitation (MR) in myocardial infarction (MI). Methods Four hundred twenty-two patients with previous MI underwent cardiac magnetic resonance (CMR) imaging for the assessment of left ventricular (LV) ejection fraction (EF), end-diastolic (EDV) and end-systolic volume (ESV), sphericity index, wall motion score index (WMSI), and late gadolinium enhancement (LGE). Echocardiography was performed to assess MR. Results Thirty-eight had from moderate to severe MR (MS-MR group) and 384 did not (No MS-MR group). The S-MR group had higher LV volumes, sphericity index, WMSI, and LGE extent, and lower LVEF. At univariate logistic regression analysis, dilated volumes, SI >0.43, dyskinesia of inferolateral wall, papillary muscle (PM)-LGE, and LGE extent >16% were associated with MS-MR. At multivariate analysis, only SI (OR=5.7) and PM-LGE (OR=3) were independently associated with MS-MR. Considering only patients without LV dilatation, only dyskinesia in the inferolateral wall was a predictor of MS-MR (OR 34.8). Thirty cardiac events (cardiac death, appropriate implantable cardioverter-defibrillator firing, and resuscitated cardiac arrest) occurred during a median follow-up of 1,276 days. After adjusting the prognostic variables at univariate analysis by age (>65 years) and selecting those that were significant (EDV > 95 ml/m2, ESV >53 ml/m2, EF 1.65, LGE >12%, S-MR), only WMSI >1.65 and MS-MR remained an independent predictor of cardiac events. Conclusions Increased WMSI and PM-LGE in the overall population and inferolateral dyskinesia in patients without ESV dilatation are predictors of MS-MR; MS-MR and elevated WMSI have independent negative prognostic value.

Myocardial infarction (MI) is a dramatic event often caused by atherosclerotic plaque erosion or rupture and subsequent thrombotic occlusion of a coronary vessel. The low supply of oxygen and nutrients in the infarcted area may result in cardiomyocytes necrosis, replacement of intact myocardium with non-contractile fibrous tissue and left ventricular (LV) function impairment if blood flow is not quickly restored. In this review, we summarized the possible correlation between adenosine system, purinergic system and Wnt/β-catenin pathway and their role in the pathogenesis of cardiac damage following MI. In this context, several pathways are involved and, in particular, the adenosine receptors system shows different interactions between its members and purinergic receptors: their modulation might be effective not only for a normal functional recovery but also for the treatment of heart diseases, thus avoiding fibrosis, reducing infarcted area and limiting scaring. Similarly, it has been shown that Wnt/β catenin pathway is activated following myocardial injury and its unbalanced activation might promote cardiac fibrosis and, consequently, LV systolic function impairment. In this regard, the therapeutic benefits of Wnt inhibitors use were highlighted, thus demonstrating that Wnt/β-catenin pathway might be considered as a therapeutic target to prevent adverse LV remodeling and heart failure following MI.

Background Amyloid transthyretin (ATTR) amyloidosis is caused by the systemic deposition of transthyretin molecules, either normal (wild‐type ATTR, ATTRwt) or mutated (variant ATTR, ATTRv). ATTR amyloidosis is a disease with a severe impact on patients’ quality of life (QoL). Nonetheless, limited attention has been paid to QoL so far, and no specific tools for QoL assessment in ATTR amyloidosis currently exist. QoL can be evaluated through patient‐reported outcome measures (PROMs), which are completed by patients, or through scales, which are compiled by clinicians. The scales investigate QoL either directly or indirectly, i.e., by assessing the degree of functional impairment and limitations imposed by the disease. Design Search for the measures of QoL evaluated in phase 2 and phase 3 clinical trials on ATTR amyloidosis. Results Clinical trials on ATTR amyloidosis have used measures of general health status, such as the Short Form 36 Health Survey (SF‐36), or tools developed in other disease settings such as the Kansas City Cardiomyopathy Questionnaire (KCCQ) or adaptations of other scales such as the modified Neuropathy Impairment Score +7 (mNIS+7). Conclusions Scales or PROMs for ATTR amyloidosis would be useful to better characterize newly diagnosed patients and to assess disease progression and response to treatment. The ongoing ITALY (Impact of Transthyretin Amyloidosis on Life qualitY) study aims to develop and validate 2 PROMs encompassing the whole phenotypic spectrum of ATTRwt and ATTRv amyloidosis, that might be helpful for patient management and may serve as surrogate endpoints for clinical trials.

Intracardiac thrombosis is reported to occur frequently in cardiac amyloidosis (CA). However, data regarding arterial thrombo-embolic events (AEs) in CA are limited. We aimed at assessing prevalence, clinical characteristics and predictors of AEs in a large multicentric CA cohort. Four-hundred-six consecutive CA patients (134 AL, 73 ATTRm and 199 ATTRwt) from 5 Italian referral centres were retrospectively evaluated and followed-up for a median time of 19 months. Thirty-one patients (7.6%) suffered from an AE, of whom 10 (32.2%) were in sinus rhythm and had no history of AF. There were no significant differences in terms of age, gender and type of CA between patients with or without AEs. Fourteen (7.6%) of 185 patients on anticoagulation had an AE despite therapy. Anticoagulation therapy did not appear to fully protect from the risk of events (HR 1.23, 95%CI 0.52–2.92, p = .64). The only predictor of AEs, in particular among CA patients in sinus rhythm, was a CHA2DS2-VASC score ≥ 3 (HR 2.84, 95%CI 1.02–7.92, p = .05 in overall population; HR 10.13, 95%CI 1.12–91.19, p = .04 in patients in sinus rhythm). In our large, multicentric, real-world cohort, prevalence and incidence rate of AEs was high. A consistent proportion of events occurred despite anticoagulation therapy or in patients in sinus rhythm. A higher CHA2DS2-VASc score might identify patients at risk of AEs also among those in sinus rhythm.

Aims The use of beta‐blocker therapy in cardiac amyloidosis (CA) is debated. We aimed at describing patterns of beta‐blocker prescription through a nationwide survey. Methods and results From 11 referral centres, we retrospectively collected data of CA patients with a first evaluation after 2016 (n = 642). Clinical characteristics at first and last evaluation were collected, with a focus on medical therapy. For patients in whom beta‐blocker therapy was started, stopped, or continued between first and last evaluation, the main reason for beta‐blocker management was requested. Median age of study population was 77 years; 81% were men. Arterial hypertension was found in 58% of patients, atrial fibrillation (AF) in 57%, and coronary artery disease in 16%. Left ventricular ejection fraction was preserved in 62% of cases, and 74% of patients had advanced diastolic dysfunction. Out of the 250 CA patients on beta‐blockers at last evaluation, 215 (33%) were already taking this therapy at first evaluation, while 35 (5%) were started it, in both cases primarily because of high‐rate AF. One‐hundred‐nineteen patients (19%) who were on beta‐blocker at first evaluation had this therapy withdrawn, mainly because of intolerance in the presence of heart failure with advanced diastolic dysfunction. The remaining 273 patients (43%) had never received beta‐blocker therapy. Beta‐blockers usage was similar between CA aetiologies. Patients taking vs. not taking beta‐blockers differed only for a greater prevalence of arterial hypertension, coronary artery disease, AF, and non‐restrictive filling pattern (P

Background: Our study aimed to evaluate right ventricular (RV) morphology and strain (S) in the early stage of familial transthyretin (TTR) cardiac amyloidosis (CA). Methods and Results: Thirty-seven patients with transthyretin mutation underwent 99mTc-3,3-diphosphono-1,2 propanodicarboxylic acid (99mTc-DPD) scans and/or cardiac magnetic resonance (CMR) to identify TTR CA. Each patient underwent echocardiography to quantify RV dimensions, tricuspid annular plane systolic excursion (TAPSE), systolic pulmonary artery pressure (sPAP), longitudinal (L) strain of the RV free wall, left ventricular (LV) septal thickness (ST), ejection fraction, E/E', LV global (G) L, radial (R), and circumferential (C) S. 99mTc-DPD and CMR revealed the accumulation in 22 of 37 patients (CA group) and no accumulation in 15 patients (no-CA group). Left ventricular (LV) septal thickness (ST) was higher (P < 0.0001) while LV ejection fraction and E/E' were lower (P < 0.05) in the CA group than the no-CA group. LV-global longitudinal strain (LS) was lower (P < 0.0001) in the CA-group than the no CA-group, whereas LV-global circumferential strain and LV-global radial strain were similar. The CA group showed higher values of RV dimensions (P < 0.05) and sPAP (0.02) and a lower (P = 0.002) TAPSE. Globally, RV-LS was lower (P = 0.005) in the CA group than the no-CA group. Basal and mid segments of the RV free wall showed a lower LS in the CA group than the no-CA group (P < 0.01), while apical S was similar between groups. Conclusions: RV deformation, particularly in basal and mid segments, is early impaired in CA.

Left ventricular intramyocardial fat (LV-IMF) is often found in patients with previous irreversible myocardial damage and may be detected by cardiac magnetic resonance (CMR). No data are currently available about the prevalence of LV-IMF in patients with previous myocarditis. Our aim was to assess the prevalence of LV-IMF in patients with previous myocarditis by repeating after3 years a follow-up CMR examination and to evaluate its clinical and prognostic role. Patients with clinical suspected myocarditis who underwent CMR within the first week from the onset of their symptoms and underwent repeated CMR were enrolled. LV-IMF was detected as areas of left ventricular intramyocardial "India ink" black boundary with or without a hyperintense core. Overall, in 235 patients with a definitive diagnosis of acute myocarditis, CMR was repeated after a median of 4 (3 to 6) years from symptom onset. LV-IMF positive patients (n = 35, 15%) presented greater ventricular volumes and more frequently a mid-wall late gadolinium enhancement than those without LV-IMF (both p0.05). Patients presenting major cardiac events (sudden cardiac deaths, resuscitated cardiac arrest, and appropriate implantable cardioverter-defibrillator-firing) at follow-up had a greater prevalence of LV-IMF than those without (55% vs 11%, p0.001). Patients with LV-IMF had a higher incidence myocarditis relapse (27% vs 9%, p = 0.003) and a greater risk of major cardiac events (p0.0001) than those without. At logistic regression analysis, LV-IMF was an independent predictor of major cardiac events. In conclusion, LV-IMF is not an uncommon finding in patients with previous myocarditis and is associated with worse ventricular remodeling and prognosis.

We sought to evaluate the prognostic role of cardiac magnetic resonance (CMR) in patients with definite, borderline and possible diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) as defined by the International Task Force (TF) in 2010. CMR was performed in 175 patients: 52 with definite, 50 with borderline and 73 possible ARVC. Abnormal-CMR was defined as the presence of ≥1 CMR abnormalities (including abnormalities of right ventricular and left ventricular wall motion, fat infiltration, late gadolinium enhancement, dilation and dysfunction of either ventricles). During the follow-up time 35 patients had hard cardiac events (sudden cardiac death, appropriate implantable cardioverter defibrillator shock and resuscitated cardiac arrest), and 34 of them occurred in patients with abnormal-CMR (negative predictive value = 96.9%). At the multivariate Cox-regression analysis LV involvement at CMR (fat infiltration and/or late gadolinium enhancement), and episode of nonsustained ventricular tachycardia (NSVT) were independent predictors of cardiac events in both the whole population (LV involvement: HR 3.69, 95% CI 1.57-8.65, p = 0.0002; NSVT: HR 5.8, 95% CI 2.82-11.9, p < 0.0001), and in the group of patients with definite ARVC (LV involvement: HR 3.03, 95% CI 1.15 to 8.02, p = 0.02; NSVT: HR 12.1, 95% CI 4.02-36.5, p < 0.0001). In conclusion, CMR evidence of LV involvement is a strong independent predictor of cardiac events in patients with definite, borderline or possible ARVC diagnosis. Abnormal CMR has very high negative predictive value for hard cardiac events

BACKGROUND The aim of present study was to assess left ventricular (LV) myocardial deformation and changes over time in patients with acute myocarditis (AM) with preserved ejection fraction detected by late gadolinium enhancement (LGE) magnetic resonance imaging. METHODS Thirty-five male patients with AM diagnoses and preserved systolic function based on cardiac magnetic resonance imaging (MRI) were prospectively enrolled. On admission, echocardiography with measurements of global and segmental longitudinal (LS) strains was performed both at the endocardial (ENDO) and epicardial (EPI) levels. Findings were compared to 25 control subjects. Twenty-six patients were also monitored over a 22-month follow-up (FU group). RESULTS On admission, global ENDO-LS was poorer in magnitude in AM (-19.2 ± 3.1) than in controls (-24.0 ± 1.05) (P

Background Few studies have explored prognosis in patients with previous myocardial infarction (MI) with mild-moderate (MM) left ventricular (LV) dysfunction (D). The aim of our study was to investigate whether combining LV parameters obtained by cardiac magnetic resonance (CMR) improves risk stratification of patients with previous MI and MM-LV-D. Methods In 418 consecutive patients (63.3±11.3years old, female 12.9%) with previous MI, we quantified LVEF, volumes and wall motion score index (WMSI) and measured the infarct extent by late gadolinium enhancement (LGE). According to LVEF, patients were considered with normal LVEF (>55%), MM-LV-D (LVEF>30 and ≤55%) and severe (S) LV-D (LVEF ≤30). Results During follow-up (median, 39.7months) cardiac events (cardiac death or appropriate intra-cardiac defibrillator shocks) occurred in 17/99 of patients with S-LV-D, in 15/201 with MM-LV-D, and in only 1/118 of those with normal LV-EF. After adjustment for age, an extent of LGE >11.3%, a dilated LV (male >112ml/m 2 ; female >92ml/m 2 ) and a WMSI>1.59 were associated with adverse cardiac events in patients with MM-LV-D. In patients with MM-LV-D, when each of these 3 factors was observed, the prognosis was worse respect to those with 1–2 factors and no factor ( p =0.035 and p =0.004, respectively). Prognosis was similar ( p =0.61) between MM-LV-D patients with all 3 factors and those with S-LV-dysfunction. CONCLUSIONS. A multiparametric CMR approach, which includes LGE, dilated LV and WMSI, permits to identify post MI patients with MM-LV-D with a risk of cardiac events similar to those with S-LV-D. Further multicenter studies are needed to confirm our data.

Background The prognostic role of cardiac magnetic resonance (CMR) and late gadolinium enhancement (LGE) has not been clarified in acute myocarditis (AM) with preserved left ventricular (LV) ejection fraction (EF). Objectives This study sought to evaluate the role of CMR and LGE in the prognosis of AM with preserved LVEF. Methods This study analyzed data from ITAMY (ITalian multicenter study on Acute MYocarditis) and evaluated CMR results from 386 patients (299 male; mean age 35 ± 15 years) with AM and preserved LVEF. Clinical follow-up was performed for a median of 1,572 days. A clinical combined endpoint of cardiac death, appropriate implantable cardioverter-defibrillator firing, resuscitated cardiac arrest, and hospitalization for heart failure was used. Results Among the 374 patients with suitable images, LGE involved the subepicardial layer inferior and lateral wall in 154 patients (41%; IL group), the midwall layer of the anteroseptal wall in 135 patients (36%; AS [anteroseptal] group), and other segments in 59 patients (16%; other-LGE group), and it was absent in 26 patients (no-LGE group). The AS group had a greater extent of LGE and a higher LV end-diastolic volume index than other groups, but levels of inflammatory markers were lower than in the other groups. Kaplan-Meier curve analysis indicated that the AS group had a worse prognosis than the other groups (p Conclusions In patients with AM and preserved LVEF, LGE in the midwall layer of the AS myocardial segment is associated with a worse prognosis than other patterns of presentation.

Cardiac magnetic resonance (CMR) has emerged as a reliable and accurate diagnostic tool for the evaluation of patients with cardiac disease in several clinical settings and with proven additional diagnostic and prognostic value compared with other imaging modalities. This document has been developed by the working group on the 'application of CMR' of the Italian Society of Cardiology to provide a perspective on the current state of technical advances and clinical applications of CMR and to inform cardiologists on how to implement their clinical and diagnostic pathways with the inclusion of this technique in clinical practice. The writing committee consisted of members of the working group of the Italian Society of Cardiology and two external peer reviewers with acknowledged experience in the field of CMR.

Impairment of the adaptive mechanisms that increase cardiac output during exercise can translate to a reduced functional capacity. We investigated cardiovascular adaptation to exertion in asymptomatic hypertensive patients, aiming to identify the early signs of cardiac and vascular dysfunction.We enrolled 54 subjects: 30 patients (45.1±11.9years, 19 males) and 24 age-matched healthy controls (44.4±9.6years, 14 males). Speckle-tracking echocardiography (STE) and echo-tracking were performed at rest and during exertion to assess myocardial deformation and arterial stiffness.E/E' increased from rest to peak exercise more in patients than in controls (peak stage: p=0.024). Global longitudinal strain increased significantly from rest to peak stage in controls (p=0.011) whereas it remained unchanged in patients (p=0.777). Left atrial (LA) reservoir was significantly increased throughout the exercise only in controls (p=0.001) whereas it was almost unchanged in patients (p=0.293). LA stiffness was significantly higher in patients than in controls both at rest (p=0.023) and during exercise (p0.001). Beta index and pulse wave velocity (PWV) increased during exercise in both groups, showing higher values in patients in each step.Our study showed a more pronounced maladaptation during exercise, with respect to rest, of the cardiovascular system with impaired cardiac-vessel coupling in hypertensive patients compared to healthy subjects. Exercise echocardiography implemented by STE and echo-tracking is invaluable in the early detection of these cardiovascular abnormalities.

The current document was developed by the working group on the 'application of cardiac magnetic resonance' of the Italian Society of Cardiology to provide a perspective on the current state of technical advances and clinical cardiac magnetic resonance applications and to inform cardiologists how to implement their clinical and diagnostic pathway with the introduction of this technique in the clinical practice. Appropriateness criteria were defined using a score system: score 1-3 = inappropriate (test is not generally acceptable and is not a reasonable approach for the indication), score 4-6 = uncertain (test may be generally acceptable and may be a reasonable approach for the indication but more research and/or patient information is needed to classify the indication definitively) and score 7-9 = appropriate (test is generally acceptable and is a reasonable approach for the indication).

We thank Dr. Corrado and colleagues for the letter regarding our study ([1][1]). We enrolled patients with definite diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) based on 2010 Task Force criteria ([1][1]). As previously described, we found that a relevant percentage of patients

Objectives The aim of this study was to assess the diagnostic accuracy of bone scintigraphy in a large multicenter cohort of patients with cardiac amyloidotic involvement and Phe64Leu transthyretin (TTR) mutation. Background Diagnostic accuracy of bone scintigraphy for transthyretin-related cardiac amyloidosis (TTR-CA) is considered extremely high, enabling this technique to be the noninvasive diagnostic standard for TTR-CA. Nevertheless, this approach has not been systematically validated across the entire spectrum of TTR mutations. Methods A total of 55 patients with Phe64Leu TTR mutation were retrospectively analyzed and evaluated between 1993 and 2018 at 7 specialized Italian tertiary centers. Cardiac involvement was defined as presence of an end-diastolic interventricular septum thickness ≥12 mm, without other possible causes of left ventricular hypertrophy (i.e., arterial hypertension or valvulopathies). A technetium-99m (99mTc)–diphosphonate (DPD) or 99mTc–hydroxyl-methylene-diphosphonate (HMDP) bone scintigraphy was reviewed, and visual scoring was evaluated according to Perugini’s method. Results Among 26 patients with definite cardiac involvement, 19 underwent 99mTc-DPD or 99mTc-HMDP bone scintigraphy. Of them, 17 (89.5%) patients had low or absent myocardial bone tracer uptake, whereas only 2 (10.5%) showed high-grade myocardial uptake. The sensitivity and the accuracy of bone scintigraphy in detecting TTR-CA were 10.5% and 37%, respectively. Patients with cardiac involvement and low or absent bone tracer uptake were similar to those with high-grade myocardial uptake in terms of age, sex, and electrocardiographic and echocardiographic findings. Conclusions The sensitivity of bone scintigraphy (DPD and HMDP) in detecting TTR-CA is extremely low in patients with Phe64Leu TTR mutation, suggesting the need to assess diagnostic accuracy of bone scintigraphy to identify cardiac involvement across a wider spectrum of TTR mutations.

Background: Late gadolinium enhancement (LGE) is an important prognostic marker in hypertrophic cardiomyopathy and an extent >15% it is associated with high risk of sudden cardiac death. We proposed a novel method, the LGE-dispersion mapping, to assess heterogeneity of scar, and evaluated its prognostic role in patients with hypertrophic cardiomyopathy. Methods: One hundred eighty-three patients with hypertrophic cardiomyopathy and a low- or intermediate 5-year risk of sudden cardiac death underwent cardiac magnetic resonance imaging. A parametric map was generated from each LGE image. A score from 0 to 8 was assigned at every pixel of these maps, indicating the number of the surrounding pixels having different quality (nonenhancement, mild-enhancement, or hyperenhancement) from the central pixel. The Global Dispersion Score (GDS) was calculated as the average score of all the pixels of the images. Results: During a median follow-up time of 6 (25th–75th, 4–10) years, 22 patients had hard cardiac events (sudden cardiac death, appropriate implantable cardioverter-defibrillator therapy, resuscitated cardiac arrest, and sustained ventricular tachycardia). Kaplan-Meier analysis showed that patients with GDS>0.86 had worse prognosis than those with lower GDS ( P 0.86 was the only independent predictor of cardiac events (hazard ratio, 9.9 [95% CI, 2.9–34.6], P =0.0003). When compared with LGE extent >15%, GDS improved the classification of risk in these patients (net reclassification improvement, 0.39 [95% CI, 0.11–0.72], P Conclusions: LGE-dispersion mapping is a marker of scar heterogeneity and provides a better risk stratification than LGE presence and its extent in patients with hypertrophic cardiomyopathy and a low-intermediate 5-year risk of sudden cardiac death.

Background: Cardiac magnetic resonance (CMR) is widely used to assess tissue and functional abnormalities in arrhythmogenic right ventricular cardiomyopathy (ARVC). Recently, a ARVC risk score was proposed to predict the 5-year risk of malignant ventricular arrhythmias in patients with ARVC. However, CMR features such as fibrosis, fat infiltration, and left ventricular (LV) involvement were not considered. Objectives: The authors sought to evaluate the prognostic role of CMR phenotype in patients with definite ARVC and to evaluate the effectiveness of the novel 5-year ARVC risk score to predict cardiac events in different CMR presentations. Methods: A total of 140 patients with definite ARVC were enrolled (mean age 42 ± 17 years, 97 males) in this multicenter prospective registry. As per study design, CMR was performed in all the patients at enrollment. The novel 5-year ARVC risk score was retrospectively calculated using the patient's characteristics at the time of enrollment. During a median follow-up of 5 years (2 to 8 years), the combined endpoint of sudden cardiac death, appropriate implantable cardioverter-defibrillator intervention, and aborted cardiac arrest was considered. Results: CMR was completely negative in 14 patients (10%), isolated right ventricular (RV) involvement was found in 58 (41%), biventricular in 52 (37%), and LV dominant in 16 (12%). During the follow-up, 48 patients (34%) had major events, but none occurred in patients with negative CMR. At Kaplan-Meier analysis, patients with LV involvement (LV dominant and biventricular) had a worse prognosis than those with lone RV (p < 0.0001). At multivariate analysis, the LV involvement, a LV-dominant phenotype, and the 5-year ARVC risk score were independent predictors of major events. The estimated 5-year risk was able to predict the observed risk in patients with lone RV but underestimated the risk in those with LV involvement. Conclusions: Different CMR presentations of ARVC are associated with different prognoses. The 5-year ARVC risk score is valid for the estimation of risk in patients with lone-RV presentation but underestimated the risk when LV is involved.

V122I is one of more than 130 mutations in transthyretin gene associated with hereditary TTR (ATTRv) amyloidosis. Main clinical expression is an infiltrative pseudohypertrophic cardiomyopathy with mild or no neurological symptoms. It is particularly common among African-Americans (prevalence: 3%-4%). We report 12 subjects from seven unrelated Caucasian families hailing from Sicily and carrying the V122I mutation. One patient was homozygous for V122I and in another family two subjects also carried the E89Q variant in compound heterozygosity. All the subjects underwent neurologic/neurophysiologic evaluation and cardiologic baseline tests; in five of them, cardiac magnetic resonance and/or (99 m) Tc-DPD scintigraphy were performed. Three of 12 subjects were asymptomatic carriers. Of the remaining nine subjects, in four of nine patients, the nerve conduction studies revealed a polyneuropathy; in one of them, this represents the only sign of disease after 5 years of follow-up. In eight of nine subjects, we found a hypertrophic restrictive cardiomyopathy and cardiac failure, associated with a carpal tunnel syndrome. Although in non-Afro-American individuals V122I prevalence is low, subjects carrying this mutation have been identified in the United Kingdom, Italy, and France. Our report describes a large cohort of V122I Caucasian patients from a non-endemic area, confirming the possible underestimation of this mutation in the non-African population. Moreover, it highlights the heterogeneity in the genotype-phenotype correlation of ATTRv mutations, suggesting that the presence of a polyneuropathy has to be identified as soon as possible, since available treatments are, in Europe, so far authorized only for ATTRv amyloid peripheral neuropathy.

Current task force criteria (TFC) of cardiac magnetic resonance (CMR) for the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC/D) were generated by comparing probands (mean age of 44 years) to healthy participants of the multi-ethnic study of atherosclerosis (mean age of 60 years). These age differences may be a selection bias because right ventricular end-diastolic volume index decreases 4.6% per decade. Moreover, fat infiltration and late gadolinium enhancement were not included. We evaluated the diagnostic accuracy of TFC using the same methodology used by the task force but comparing probands and age- and gender-matched healthy controls and considering also other morphofunctional and tissue abnormalities detected by CMR. Forty-seven probands with previous diagnosis of ARVC/D (excluding probands if CMR was used for diagnosis) were compared with 216 age- and gender-matched healthy controls. TFC had optimal specificity (100%) but poor sensitivity (20% for major and 13% for minor criteria). The presence of any pre- and post-contrast signal abnormalities had 100% specificity and 81% sensitivity. The best diagnostic accuracy (98%) was achieved by the combined evaluation of any right ventricular wall motion abnormality (excluding hypokinesia) with any signal abnormality (including left ventricular fat infiltration and late gadolinium enhancement) yielding a 100% specificity and 96% sensitivity. Left ventricular was involved in 45% of the probands. Current TFC for CMR presented optimal specificity but poor sensitivity to identify patient with ARVC/D. Signal and wall motion parameters of CMR should be considered together to achieve the best diagnostic accuracy for the diagnosis of ARVC/D.

Purpose To define reference values of cardiac volumes, dimensions, and new morpho-functional parameters normalized for age, gender, and body surface area by cine-bSSFP (balanced steady-state free-precession) magnetic resonance (MR). Materials and Methods We enrolled 308 healthy subjects subdivided by gender and by six age classes: class I, >15–20 years; class II, >20–30 years; class III, >30–40 years; class IV, >40–50 years; class V, >50–60 years; and class VI >60 years. Dimensional, volumetric and morpho-functional parameters of the left (LV) and right (RV) ventricles were measured using cine-bSSFP MRI at 1.5T. Results The LV and RV end-diastolic volume indexes (EDVi) were inversely related to age (P < 0.0001 r = –0.34 and P < 0.0001 r = –0.37, respectively). In addition, the LV mass index decreased with age (P = 0.0004, r = –0.21). The LV longitudinal shortening was not significantly different among groups: ≥15% in all populations (95% confidence interval [CI]: 16–31). The sphericity index measured in end-diastole was higher in females than in males (P < 0.03): the upper limit was 40% for males and 42% for females. The normality cutoff of LV global function index was ≥33% in males and ≥35% in females. The end-diastolic volume (EDV) of RV and LV was balanced (RV/LV ratio 0.85–1.15) without differences in the population. The LV EDV/mass was 1.0–1.8 in males and 1.0–2.1 in females. Conclusion This study provides potential age- and gender-specific reference. Evidence level 2 J. Magn. Reson. Imaging 2016.

Objectives: Left atrial (LA) function is an important marker of hemodynamic status in cardiac amyloidosis (CA), and its characterization may provide relevant prognostic information. We sought to assess the prevalence and prognostic impact of LA dysfunction by cardiac magnetic resonance (CMR) in patients with CA. Methods: We performed CMR in 80 consecutive patients with CA, including 38 with AL (47%) and 42 with ATTR (53%). LA function was assessed by acquiring short axis cine steady-state free precession (SSFP) covering the entire chamber. The atrial emptying fraction (AEF) was calculated as the ratio between the difference of LA maximal and minimal volume to LA maximal volume, expressed as percentage. Severe atrial dysfunction was defined as AEF ≤ 14%. Results: Mean AEF was 18% (13–35%). Overall, AEF ≤ 14% was present in 19 patients (24%), including 21% of those in sinus rhythm (SR) with no history of atrial fibrillation (AF). After a median of 3 years (IQR 2–4), 36 patients (44%) died of cardiac causes. Patients with AEF ≤ 14% showed increased cardiac mortality, with an independent OR of 4.2 (95 IC 2.1–8.2, P

Background Cardiac magnetic resonance (CMR) is widely used to confirm the diagnosis of acute myocarditis (AM) in the acute setting. CMR is often repeated after 6 months to assess the evolution of myocardial involvement. However, the clinical and prognostic role of 6-month CMR is unknown. Objectives This multicenter study aimed to evaluate the clinical and prognostic role of 6-month repetition of CMR in patients with AM. Methods In a subgroup of 187 patients from the ITAMY (ITAlian study in MYocarditis) registry, CMR was performed within the first week after symptom onset (CMR-I) and repeated after 6 months (CMR-II). Results Myocardial edema was detected in all the patients at CMR-I and persisted in 31 (16%) at CMR-II. LGE was detected in 182 (96%) patients at CMR-I and in 164 (86%) at CMR-II. At CMR-II, 20 (11%) patients presented a complete recovery from edema and LGE, 30 (16%) patients had edema with LGE, and 137 (73%) presented LGE without edema. LGE disappeared completely in 18 (10%) patients, the number of LGE segments decreased in 87 (46%), unchanged in 58 (31%), and increased in 26 (14%). During a median clinical follow-up of 7 years (25th to 75th percentile: 6 to 8 years) cardiac events occurred in 22 patients. At Kaplan-Meier curves, patients with LGE and without edema had worse prognosis than others (p Conclusions In the acute setting, LGE does not mean definite fibrosis, and it may disappear at 6 months. The presence of LGE without edema at 6-month CMR is associated with worse prognosis, particularly when distributed with a midwall septal pattern. LGE without edema could represent definite fibrosis whereas the presence of edema suggests a residual chance of recovery.

Sudden cardiac death (SCD) is the most life-threating complication of hypertrophic cardiomyopathy. Guidelines of the European Society of Cardiology (ESC) suggest the implantation of an implantable cardioverter defibrillator in primary prevention according to a 5-year risk SCD score >= 6%. The aim of the study is to evaluate the prognostic role of late gadolinium enhancement (LGE) in patients with a 5-year risk SCD score = 4 and = 10% was the best threshold to predict major arrhythmic events (area under the curve: 0.74). Kaplan-Meier curves showed that patients with LGE >= 10% had a worse prognosis than those with lower extent (p < 0.0001). LGE extent was the best independent predictor of hard cardiac events (hazard ratio 1.05; 95% confidence interval [CI] 1.03 to 107; p < 0.0001). The estimates 5-year risk of hard cardiac event was 2.5% (95% CI 0.8 to 4.2) in patients with LGE extent = 10%. In conclusion, this study demonstrates as the extent of LGE >= 10% is able to recognize additional patients at increased risk for malignant arrhythmic episodes in a population with low-to-intermediate ESC SCD risk score. (C) 2019 Elsevier Inc. All rights reserved.

We sought to evaluate the role of cardiac magnetic resonance imaging (CMR) in the evaluation of diastolic function by a combined assessment of left ventricular (LV) and left atrial (LA) function in a cohort of subjects with various degrees of diastolic dysfunction (DD) detected by echocardiography. Forty patients with different stages of DD and 18 healthy controls underwent CMR. Short-axis cine steady-state free precession images covering the entire LA and LV were acquired. Parameters of diastolic function were measured by the analysis of the LV and LA volume/time (V/t) curves and the respective derivative dV/dt curves. At receiver operating characteristic (ROC) curve analysis, the peak of emptying rate A indexed by the LV filling volume with a cut-off of 3.8 was able to detect patients with grade I DD from other groups (area under the curve [AUC] 0.975, 95% confidence interval [CI] 0.86–1). ROC analysis showed that LA ejection fraction with a cut-off of ≤36% was able to distinguish controls and grade I DD patients from those with grade II and grade III DD (AUC 0.996, 95% CI 0.92–1, p < 0.001). The isovolumetric pulmonary vein transit ratio with a cut-off of 2.4 allowed class III DD to be distinguished from other groups (AUC 1.0, 95%CI 0.93–1, p < 0.001). Analysis of LV and LA V/t curves by CMR may be useful for the evaluation of DD. • Combined atrial and ventricular volume/time curves allow evaluation of diastolic function. • Atrial emptying fraction allows distinction between impaired relaxation and restrictive/pseudo-normal filling. • Isovolumetric pulmonary vein transit ratio allows distinction between restrictive and pseudo-normal filling.

We thank Dr. Vasques-Novoa and colleagues for their comments on our paper ([1][1]). In our study, we enrolled only stable patients with acute myocarditis and preserved ejection fraction. Presentation, management, and prognosis of our patients is very different from patients with septic

We read with great interest the article by Henein MY and Colleagues [1] showing that in patients affected by transthyretin (TTR) cardiac amyloidosis (CA): (1) left atrial (LA) myocardial function i...

BACKGROUND We sought to assess left atrial (LA) morphology and function in patients with transthyretin cardiac amyloidosis (TTR-CA) and hypertrophic cardiomyopathy (HCM). Primarily, longitudinal deformation (reservoir) and pump function were the focus of vector-velocity strain echocardiography imaging. METHODS AND RESULTS The study group comprised 32 patients (mean age 57.7±15.4 years, 16 in each group), and 15 healthy controls. Diagnosis of TTR-CA was based on echocardiography and either gadolinium-enhanced (LGE) cardiac magnetic resonance (cMRI) or radionuclide imaging. At baseline, there were no differences in age, body surface area, blood pressure and risk factors among the groups. Left ventricular (LV) mass was greater in patients than in controls, and slight LA dilatation was found in the TTR-CA group. LA reservoir was 14.1±4.7% in TTR-CA, 20.0±5.6% in HCM, and 34.0±11.8% in controls (

AimWe aimed to evaluate the usefulness of left atrial (LA) mechanics and stiffness over global left ventricular (LV) longitudinal strain (GLS) for risk stratification in severe aortic stenosis (AS).MethodsFrom a cohort of 89 prospective asymptomatic patients with severe AS and normal LV ejection fraction, 82 (32 men, mean age 73±10 years) truly asymptomatic patients, scheduled after a negative exercise echocardiogram, were enrolled. Forty age- and gender-matched prospective, asymptomatic subjects served as controls. Predefined end points were the occurrence of symptoms (dyspnea, angina, syncope), and death during follow-up.ResultsAt study entry, patients had: impaired LV GLS (p=0.001), reduced LA reservoir (p0.001), lower LA reservoir (p

Background Isolated left ventricular diverticulum, a rare cardiac malformation, can be asymptomatic or associated with systemic embolization, ventricular arrhythmias, or sudden death. MRI allows for the detection of diverticuli and the distinction between fibrous and muscular types using the delayed enhancement technique. Aim To evaluate the prevalence of left ventricular diverticuli in nonselected consecutive patients who had undergone MRI. Methods In a total of 3273 consecutive patients who had undergone cardiac magnetic resonance from January 2001 to December 2005, isolated ventricular diverticuli were found in 25 patients (0.76%), with no apparent cardiac disease. A delayed enhancement technique was used to distinguish fibrous and muscular types. The prevalence of complications was evaluated with a follow-up of 52 ± 8 months. Results The site of the left ventricular diverticuli was the septum in 10 (37%) cases, inferior wall in 6 (22%) cases, lateral wall in 4 (15%) cases, and apical in 7 (26%) cases. In two cases, multiple diverticuli were found. In delayed enhancement images, a partial or total fibrous diverticulum was found in 6 (24%) patients. At follow-up, clinical complications were recorded in 6 (24%) patients; 2 (8%) patients had arrhythmic complications and 4 (12%) patients had embolic complications of presumed cardiac origin. In addition, two patients (8%) had nonsustained ventricular tachycardia diagnosed using 24-h ECG Holter monitoring. Fibrous type diverticuli were associated with a higher incidence of arrhythmic complications than the muscular type. Conclusion The prevalence of isolated ventricular diverticuli detected by MRI is higher than that previously reported with other imaging techniques. Fibrous diverticuli are associated with a higher incidence of arrhythmic complications.

We aimed to evaluate the role of two-dimensional speckle tracking imaging (2DSTI) in detecting early changes of myocardial deformation in patients affected by thalassemia major (TM) and its relation with myocardial iron overload (MIO) detected by T2* cardiovascular magnetic resonance (CMR). We studied 28 TM patients (15 males, 37.4 ± 10 years). All patients underwent CMR and echocardiography in the same day. Segmental and global T2* values were measured. Values of global longitudinal strain (GLS) were derived from the three apical views, while radial and circumferential strain were obtained as average strain from the short axis views at basal, mid and apical level. Six patients (21.4%) showed significant MIO (global heart T2*

Aims Bicuspid aortic valve (BAV) is associated with aortic wall alterations. We aimed to detect any correlation between aortic elasticity and genetic and biomolecular patterns of elastin. Methods and results Forty-nine BAV patients (mean age: 38 ± 17.05) were prospectively enrolled. A blood sample was drawn for analysis of a single nucleotide polymorphism of elastin gene (ELN rs2071307) responsible for misfolding of elastin, and for the amount of elastin soluble fragments (ESF) in the plasma. Aortic dimensions and elastic properties were determined by echocardiography, aortic stiffness (AS) by M-mode analysis, and longitudinal strain (LS) of the ascending aorta (AA) by speckle-tracking echocardiography; values of aortic strain were compared with 45 age-matched subjects (mean age: 33 ± 9.67) with tricuspid aortic valve (TAV). BAV patients had greater aortic dimensions [Valsalva sinus (P = 0.004), sinotubular junction (P = 0.013), AA (P

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Acute myocarditis (AM) is commonly found in everyday clinical practice. Differential diagnosis between various causes of myocardial damage with non-obstructive coronary arteries can be cumbersome for clinician. Moreover, AM may be provoked by a number of different causes and clinical presentation can be heterogeneous with potential overlap going from asymptomatic or subclinical to severe heart failure, arrhythmias, and death. Cardiac magnetic resonance (CMR) over the last decades has proven to be the diagnostic technique of choice since it allows identifying AM with excellent diagnostic accuracy. Latest technological advancement with parametric imaging such as T1 and T2 mapping further increases sensitivity and provides additional help towards a correct diagnosis. CMR however is no longer to be considered as a mere diagnostic tool but also as a powerful source of prognostic information. Scientific evidence has corroborated CMR's role beyond diagnosis demonstrating how late gadolinium enhancement (LGE) presence is a powerful predictor of cardiac events and how the presence of septal LGE is to be considered of worst prognosis regardless of LGE extension even in patients with preserved global systolic function. CMR should be routinely performed in all patients with AM suspicion since its diagnostic and prognostic role is of paramount important and could modify therapeutic strategy and subsequent clinical decisions.

OBJECTIVES: Right ventricular (RV) myocarditis (MY) is unrecognized, and its prevalence is unknown. We evaluated the prevalence of RV involvement in acute MY and its association with cardiac events (cardiac death, cardiac arrest, ventricular assist device, transplantation, and appropriate ICD intervention). METHODS: We enrolled 151 patients who underwent cardiac magnetic resonance for clinical suspicion of acute MY. The CMR protocol included T2-STIR images for edema detection, post-contrast cine-SSFP for hyperemia detection and late gadolinium enhancement (LGE) images. RESULTS: Signs of RV MY were found in 27 patients (17.8%): RV edema at T2-STIR in all of these 27 patients; RV LGE was detected in 11 patients (7.3%). The median RV myocardial segment involved was 2 (1-3). In 13 patients, RV edema was in direct continuity with LV edema of septum and inferior wall or with anterior septum and anterior wall. In 2 patients RV myocarditis was found without any signs of LV involvement. Patients with RV MY had higher RV end-diastolic volume index (p = 0.04) and RV mass index (p = 0.03), and lower RV ejection fraction (p

In recent years, the role of left ventricular ejection fraction (EF) as the gold standard parameter for the evaluation of systolic function has been questioned, and many efforts have been concentrated in the clinical validation of new noninvasive tools for the study of myocardial contractility. Improvement in the accuracy of speckle-tracking echocardiography has resulted in a large amount of research showing the ability of two-dimensional strain to overcome EF limitations in the majority of primary and secondary heart diseases. Currently, global longitudinal strain (GLS) is considered the most accurate and sensitive parameter for the assessment of early left ventricular dysfunction. This review summarizes the advantages that this measurement can provide in several clinical settings. Moreover, the important cautions that should be considered in making the choice to use GLS also are addressed. Finally, a special focus on bull’s-eye polar maps for the assessment of regional changes of longitudinal function and the usefulness of these maps in the differential diagnosis of several diseases is provided.

Background: Myocardial bridging (MB) occurs when a segment of an epicardial coronary artery takes an intra– myocardial course, thus leading to systolic compression. Most myocardial bridges involve the left anterior descending artery and are observed in 14–35% of patients. Different pathophysiological mechanisms can induce symptoms secondary to myocardial ischemia: systolic coronary compression, diastolic dysfunction associated with aging and coronary atherosclerosis, LV hypertrophy, vasospasm, microvascular and endothelial dysfunction, plaque development proximal to the bridge. Methods: We performed a literature review of MB, with a particular emphasis on electrocardiographic manifestations. Results: Stable angina–like chest pain is the usual presentation and MB should be suspected in patients atlowriskforcoronaryatherosclerosiswhich referthissymptomorwhichpresentmyocardial ischemia at instrumental examinations. ECG changes are not specific for MB and resting ECG is often normal or presents ST segment anomalies. Exercise stress test often shows non specific signs of ischemia, conduction disturbances or arrhythmias which do not allow the distinction between myocardial bridging and other causes of myocardial ischemia; angina often appears during exercise, even in the absence of ECG changes. Myocardial perfusion deficits at scintigraphy are neither obligatory nor specific. Although the clinical significance of MB is still debated, MB has been associated with acute coronary syndrome, coronary vasospasm, and even sudden cardiac death. Conclusion: Although MB may lead to myocardial ischemia during stress, its clinical presentation and electrocardiographic findings are not specific. Ann Noninvasive Electrocardiol 2014;00(0):1–5 coronary artery anomaly; myocardial bridging; myocardial ischemia