Search

Objective. To evaluated possible clinical and instrumental, natural history and prognostic divergences in women and men with idiopathic dilated cardiomyopathy (IDCM). Patients and Methods. From 1988 to 2012, we evaluated 803 consecutive patients with IDCM recorded in the Heart Muscle Disease Registry of Trieste (Italy). All patients had serial follow-up evaluations at 6, 12, and 24 months, and subsequently every two years, or more frequently if clinically indicated. Results. Two hundred and twenty-seven patients (28%) were female. At first evaluation women were significantly older (48 vs. 45 years old, p = 0.008); presented more frequently left bundle branch block at ECG (38% vs. 28%, p = 0.01), smaller left ventricular end-diastolic indexed volume at echocardiography (85 vs. 93 ml/m2, p

Echocardiography is a reliable and reproducible method to assess non-invasively cardiac function in clinical and experimental research. Significant progress in the development of echocardiographic equipment and transducers has led to the successful translation of this methodology from humans to rodents, allowing for the scoring of disease severity and progression, testing of new drugs, and monitoring cardiac function in genetically modified or pharmacologically treated animals. However, as yet, there is no standardization in the procedure to acquire echocardiographic measurements in small animals. This position paper focuses on the appropriate acquisition and analysis of echocardiographic parameters in adult mice and rats, and provides reference values, representative images, and videos for the accurate and reproducible quantification of left ventricular function in healthy and pathological conditions. ispartof: CARDIOVASCULAR RESEARCH vol:117 issue:1 pages:43-59 ispartof: location:England status: published

Background: Titin (TTN)-related dilated cardiomyopathy (DCM) has a higher likelihood of left ventricular reverse remodelling compared with other genetic etiologies. No data regarding the evolution of right ventricular dysfunction (RVD) according to genetic background are available. Methods: Consecutive 104 DCM patients with confirmed pathogenic genetic variants (51 TTN-related DCM; 53 other genetic DCM) and a control group of 139 patients with negative genetic testing and available follow-up data at 12-24 months were analysed. RVD was defined as a right ventricular fractional area change (RVFAC) < 35%. The main study end point was the comparison of the evolution of RVD and the change of RVFAC throughout the follow-up according to etiology. A composite of all-cause mortality and heart transplantation was included as outcome measure. Results: At enrollment, RVD was present in 29.1% of genetically positive DCM without differences between genetic cohorts. At 14 months follow-up, 5.9% of TTN-related DCM patients vs 35.8% of other genetic DCM patients had residual RVD after treatment (P < 0.001). Accordingly, RVFAC significantly improved in the TTN-related DCM cohort and remained stably impaired in other genetic DCM patients. However, the evolution of RVD was similar between TTN-related DCM and patients without a genetic mutation. After adjusting for RVD at follow-up, no differences in the outcome measure were seen in the study cohorts. Conclusions: The evolution of RVD in DCM is heterogeneous in different genetic backgrounds. TTN-related DCM is associated with a higher chance of RVD recovery compared with other genetic etiologies.

The case highlights the value of contrast echocardiography in raising clinical suspicion of malignancy, allowing a diagnostic work‐up and the treatment of the primitive heart tumors., >The case highlights the value of contrast echocardiography in raising clinical suspicion of malignancy, allowing a diagnostic work‐up and the treatment of the primitive heart tumors. ​

Background: The early diagnosis of genetically determined dilated cardiomyopathy (DCM) could improve the prognosis in mutation carriers. Left ventricular global longitudinal strain (LV GLS) and peak left atrial longitudinal strain (PALS) are promising techniques for the detection of subtle systolic and diastolic dysfunction. We sought to evaluate the prevalence of subtle systolic and diastolic dysfunction by LV GLS and PALS in a cohort of genotype-positive phenotype-negative (GPFN) DCM relatives. Methods and results: In this retrospective study, we analyzed echocardiograms of forty-one GPFN relatives of DCM patients. They were compared with age and sex matched healthy individuals (control group). Reduced LV GLS and PALS were defined as >18% and

Various lung ultrasound (LUS) scanning modalities have been proposed for the detection of B-lines, also referred to as ultrasound lung comets , which are an important indication of extravascular lung water at rest and after exercise stress echo (ESE). The aim of our study was to assess the lung water spatial distribution ( comet map ) at rest and after ESE. We performed LUS at rest and immediately after semi-supine ESE in 135 patients (45 women, 90 men; age 62 ± 12 y, resting left ventricular ejection fraction = 41 ± 13%) with known or suspected heart failure or coronary artery disease. B-lines were measured by scanning 28 intercostal spaces (ISs) on the antero-lateral chest, 2nd–5th IS, along with the midaxillary (MA), anterior axillary (AA), mid-clavicular (MC) and parasternal (PS) lines. Complete 28-region, 16-region (3rd and 4th IS), 8-region (3rd IS), 4-region (3rd IS, only AA and MA) and 1-region (left 3rd IS, MA) scans were analyzed. In each space, the B-lines were counted from 0 = black lung to 10 = white lung. Interpretable images were obtained in all spaces (feasibility = 100 %). B-lines (>0 in at least 1 space) were present at ESE in 93 patients (69%) and absent in 42. More B-lines were found in the 3rd IS and along AA and MA lines. The B-line cumulative distribution was symmetric at rest (right/left = 1.10) and asymmetric with left lung predominance during stress (right/left = 0.67). The correlation of per-patient B-line number between 28-S and 16-S (R 2 = 0.9478), 8-S (R 2 = 0.9478) and 4-S scan (R 2 = 0.9146) was excellent, but only good with 1-S (R 2 = 0.8101). The average imaging and online analysis time were 5 s per space. In conclusion, during ESE, the comet map of lung water accumulation follows a predictable spatial pattern with wet spots preferentially aligned with the third IS and along the AA and MA lines. The time-saving 4-region scan is especially convenient during stress, simply dismissing dry regions and focusing on wet regions alone.

Aim: Contemporary survival trends in dilated cardiomyopathy (DCM) are largely unknown. The aim of this study is to investigate clinical descriptors, survival trends and the prognostic impact of aetiological characterization in DCM patients. Methods and results: Dilated cardiomyopathy patients were consecutively enrolled and divided into four groups according to the period of enrolment (1978-1984; 1985-1994; 1995-2004; and 2005-2015). A subset of patients with DCM of specific aetiology, enrolled from 2005 to 2015, was also analysed. Over a mean follow-up of 12 ± 8 years, 1284 DCM patients (52 in the 1978-1984 group, 326 in the 1985-1994 group, 379 in the 1995-2004 group, and 527 in the 2005-2015 group) were evaluated. Despite older age (mean age 51 ± 15, 43 ± 15, 45 ± 14, and 52 ± 15 years for the 1978-1984, 1985-1994, 1995-2004, and 2005-2015 groups, respectively; P < 0.001), most of the baseline clinical characteristics improved in the 2005-2015 group, suggesting a less advanced disease stage at diagnosis. Similarly, at competing risk analysis, the annual incidence of all outcome parameters progressively decreased over time (global P < 0.001). At multivariable analysis, the last period of enrolment emerged as independently associated with a reduction in all-cause mortality/heart transplantation (HTx)/ventricular assist device (VAD) implantation (1.46 events/100 patients/year), cardiovascular death/HTx/VAD implantation (0.82 events/100 patients/year) and sudden cardiac death (0.15 events/100 patients/year). Lastly, in 287 patients with DCM of specific aetiology, patients with environmental, toxic, or removable factors appeared to have different phenotypes and prognosis compared to those with genetic, post-myocarditis, or idiopathic DCM (P < 0.001). Conclusions: Contemporary survival trends in DCM significantly improved, mainly due to a reduction of cardiovascular events. Appropriate aetiological characterization might help in prognostication of DCM patients.

Objective The index of maximal systolic acceleration ([AImax]: maximal systolic acceleration of the Doppler waveform divided by peak systolic velocity) shows diagnostic accuracy in screening of renal artery stenosis. This study aimed to determine whether an upstream factor of resistance, such as aortic valve stenosis (AVS), can affect Doppler parameters detected in the peripheral arteries. Methods In this prospective study, we measured the AImax in non-stenotic renal interlobar arteries of 62 patients with AVS. Patients were divided into three groups on the basis of severity of valvulopathy as follows: mild-to-moderate AVS (M-AVS; n = 24), intermediate AVS (I-AVS; n = 15), and severe AVS (S-AVS; n = 23) based on Nishimura’s criteria. Results The AImax in the renal parenchymal arteries was significantly lower in the S-AVS group (8.9 ± 3.6 s−1) than in the M-AVS (15.3 ± 3.8 s−1) and I-AVS groups (16.7 ± 5.2 s−1). The AImax was positively correlated with the aortic valve area and inversely correlated with the tranvalvular aortic pressure gradient. After aortic valve replacement, the AImax significantly increased from 10.7 ± 4.0 s−1 at baseline to 19.3 ± 4.4 s−1. Conclusions Proximal resistance can lead to diagnostic bias of Doppler parameters that are applied in the diagnosis of peripheral vasculopathies, particularly in renal artery stenosis.

BACKGROUND: Tricuspid valve annuloplasty is the treatment of choice for tricuspid regurgitation (TR) secondary to left-sided heart valve disease (functional TR). METHODS: Between 1999 and 2014, 527 consecutive patients (mean age, 69.6 ± 9.5 years) with grade ≥ 1+ functional TR (graded from 0-3+) underwent tricuspid annuloplasty in addition to left-sided heart valve operations at the authors' institution. The operative risk (by the European System for Cardiac Operative Risk Evaluation II [EuroSCORE II]) was 10.4% ± 12.2%. Clinical data and echocardiographic studies were reviewed retrospectively during a mean follow-up of 5.2 ± 3.5 years. Risk factors for late repair failure were identified by multivariable analysis. RESULTS: Either suture (De Vega) or device annuloplasty was used in 14.8% and 85.2% of patients, respectively. Concomitant mitral or aortic valve surgery was performed in 92.6% and 35.9% of cases, respectively. There were 48 (9.1%) hospital deaths. The 10-year nonparametric estimates of freedom from all-cause death, cardiac and cerebrovascular deaths, and grade ≥ 2+ TR were 51.2% (95% confidence interval [CI], 47.8%-54.6%) 69.9% (95% CI, 67%-72.8%), and 77.8% (95% CI, 74.2%-81.4%), respectively. A left ventricular ejection fraction < 50% (P = 0.027), tricuspid annular diameter > 40 mm (P = 0.001), and use of De Vega annuloplasty (P = 0.019) were predictors of grade ≥ 2+ TR during the follow-up period. There was a strong link between grade ≥ 2+ TR and new left-sided valvular lesions (odds ratio, 5.3; P < 0.0001), primarily mitral regurgitation. CONCLUSIONS: After device annuloplasty and in the absence of preoperative left ventricular dysfunction and severe tricuspid annular dilatation, functional TR is generally controlled within grade 1+ during the follow-up period. Recurrent TR is associated with new left-sided valvular lesions.

Aims: Despite continuous efforts in improving the selection process, the rate of non-responders to cardiac resynchronization therapy (CRT) remains high. Recent studies on intraventricular blood flow suggested that the alignment of hemodynamic forces (HDFs) may be a reproducible biomarker of mechanical dyssynchrony. We aimed to explore the relationship between pacing-induced realignment of HDFs and positive response to CRT. Methods and results: We retrospectively analyzed 38 patients from the CRT database of our institution fulfilling the inclusion criteria for HDFs-related echocardiographic assessment early pre and post CRT implantation, with available mid-term follow-up (≥ 6 months) evaluation. Standard echocardiographic and deformation parameters early pre and post CRT implantation were integrated with the measurement of HFDs through novel methods based on speckle-tracking analysis. At midterm follow-up 71% of patients were classified as responders (reduction of Left Ventricular Systolic Volume Indexed ≥ 15%). Patients did not display significant changes between close evaluations pre and post-implant in terms of ejection fraction and strain metrics. A significant reduction of the ratio between the amplitudes of transversal and longitudinal force components was found. The variation of this ratio strongly correlates (R2 =0.60) with Left Ventricular (LV) end-systolic volume variation at mid-term follow up. Conclusion: Pacing-induced realignment of HDFs is associated with CRT efficacy at follow up. These preliminary results claim for dedicated prospective clinical studies testing the potential impact of HDFs study for patient selection and pacing optimization in CRT.

Dilated cardiomyopathy (DCM) is generally thought as a final common pathway of several conditions leading to the same clinical phenotype. Multiple imaging modalities play a fundamental role in recognizing the underlying pathological substrate in DCM. Areas covered: Echocardiography represents the first reliable and easily accessible diagnostic tool, allowing the identification of associated cardiac abnormalities, such as valve disease and highlighting features associated with an adverse prognosis. Recent advances in technology such as strain analysis and 3D-echocardiography have improved the diagnostic and prognostic capabilities of this technique. Cardiac magnetic resonance (CMR) is considered the gold standard for an accurate and reproducible assessment of ventricular volumes and ejection fraction. In addition, CMR allows us to perform tissue characterization that, through new sophisticated sequences, could be obtained even without gadolinium. Nuclear images could be useful to identify specific causes of left ventricular dysfunction, such as cardiac sarcoidosis and amyloidosis. Finally, endomyocardial biopsy is generally performed if acute myocarditis is suspected in high-risk patients. Expert commentary: Strengths and limitations are different for every method, but multiparametric evaluation of patients and family members could progressively improve current understanding of the disease. This is fundamental to specifically target therapy, allowing us to improve patients' prognosis.

The aim of the study was to assess the clinical and prognostic impact of early functional mitral regurgitation (FMR) improvement on the outcome of patients with idiopathic dilated cardiomyopathy (IDC). The prevalence and prognostic role of FMR improvement, particularly at early follow-up, in patients with IDC are still unclear. From 1988 to 2009, we enrolled 470 patients with IDC with available FMR data at baseline and after 6 ± 2 months. According to the evolution of FMR, patients were classified into 3 groups: stable absent-mild FMR, early FMR improvement (downgrading from moderate-severe to absent-mild), and persistence/early development of moderate-severe FMR. At baseline, 177 of 470 patients (38%) had moderate-severe FMR. Patients with early FMR improvement had significantly better survival rate-free from heart transplant with respect to those with persistence/early development of moderate-severe FMR (93%, 81%, and 66% vs 91%, 64%, and 52% at 1, 6, and 12 years, respectively; p = 0.044). At 6-month follow-up multivariate analysis, FMR improvement was associated with better prognosis (hazard ratio 0.78, 95% confidence interval [CI] 0.64 to 0.96, p = 0.02); the other independent predictors were male gender, heart failure duration, and early re-evaluation of the New York Heart Association class and left ventricle systolic function. This model provided more accurate risk stratification compared with the baseline model (Net Reclassification Index 80% at 12 months and 41% at 72 months). In conclusion, in a large cohort of patients with IDC receiving optimal medical treatment, early improvement of FMR was frequent (53%) and emerged as a favorable independent prognostic factor with an incremental short- and long-term power compared with the baseline evaluation.

In this report, we present a rare case of cardiac echinococcosis presenting with cerebral haemorrhage due to rupture of a mycotic aneurysm in a peripheral branch of the media cerebral artery. Further investigations lead to discovery of multiple cysts in the heart and liver. The complementary use of transthoracic and transoesophageal echocardiography, MRI and computed tomography lead to the final diagnosis of disseminated echinococcosis and allowed precise evaluation of the anatomical and structural characteristics of the cardiac mass, its boundaries and its relationship with the surrounding anatomic structures.

Background Patients with heart failure (HF) and severe symptomatic functional mitral regurgitation (FMR) may benefit from MitraClip implantation. With increasing numbers of patients being treated the success of procedure becomes a key issue. We sought to investigate the pre-procedural predictors of device failure in patients with advanced HF treated with MitraClip. Methods From April 2012 to November 2016, 76 patients with poor functional class (NYHA class III–IV) and severe left ventricular (LV) remodeling underwent MitraClip implantation at University Hospitals of Trieste and Bologna (Italy). Device failure was assessed according to MVARC criteria. Patients were subsequently followed to additionally assess the patient success after 12months. Results Mean age was 67±12years, the mean Log-EuroSCORE was 23.4±16.5%, and the mean LV end-diastolic volume index and ejection fraction (EF) were 112±33ml/m 2 and 30.6±8.9%, respectively. At short-term evaluation, device failure was observed in 22 (29%) patients. Univariate predictors of device failure were LVEF, LV and left atrial volumes and anteroposterior mitral annulus diameter. Annulus dimension (OR 1.153, 95% CI 1.002–1.327, p =0.043) and LV end-diastolic volume (OR 1.024, 95% CI 1.000–1.049, p =0.049) were the only variables independently associated with the risk of device failure at the multivariate model. Conclusions Pre-procedural anteroposterior mitral annulus diameter accurately predicted the risk of device failure after MitraClip in the setting of advanced HF. Its assessment might aid the selection of the best candidates to percutaneous correction of FMR.

Arrhythmic risk stratification in idiopathic dilated cardiomyopathy (IDC) remains a major concern. As the ventricles remodel in time, risk factors for arrhythmic death may change. A cohort of 710 patients with idiopathic dilated cardiomyopathy, without previous ventricular arrhythmias, was retrospectively studied to understand how risks vary in time. The primary end point was a composite of sudden cardiac death, ventricular fibrillation, sustained ventricular tachycardia, and appropriate implantable cardioverter-defibrillator interventions. The prediction of the arrhythmic outcome was assessed dynamically through landmark analysis. Patients were assessed at baseline, short term (12 months, interquartile range 6 to 18), and long-term (72 months, interquartile range 60 to 84). The strongest risk predictors at each evaluation were combined in 3 multivariate models. During a median follow-up of 102 months, 80 patients (11%) experienced the primary end point. At baseline, QRS duration (p = 0.008), disease duration (p

Hypertensive hypokinetic cardiomyopathy (HHC) is defined by left ventricular (LV) systolic dysfunction with a history of systemic hypertension as the only possible cause. Although commonly encountered in clinical practice, its characterization and differences with true idiopathic dilated cardiomyopathy (IDC) are lacking. The aim of this study was to characterize the clinical instrumental features and the natural history of HHC. We analyzed the data of 4,191 patients referred to our center for newly diagnosed LV systolic dysfunction from 2005 to 2010. Of them, 310 presented idiopathic LV systolic dysfunction (LV ejection fraction

Background The role of percutaneous mitral valve repair (PMVR) in patients with end-stage heart failure (HF) and functional mitral regurgitation (FMR) is unclear. Methods Seventy-five consecutive patients with FMR grade ≥ 3 + and severe HF symptoms despite optimal medical therapy and resynchronization therapy underwent PMVR with the MitraClip system (Abbott, Abbott Park, IL, USA) at 3 centers. Clinical evaluation, echocardiography and pro-BNP measurement were performed at baseline and at 6-month. Results Mean age was 67 ± 11 years, logistic EuroSCORE = 23 ± 18%, left ventricle ejection fraction (LVEF) 30 ± 9%. In 6 patients (8%) PMVR was performed as a bridge to heart transplant; many patients were dependent from iv diuretics and/or inotropes. Rate of serious adverse in-hospital events was 1.3% (1 patient who died after conversion to cardiac surgery). Sixty-three patients (84%) were discharged with MR ≤ 2 +. At 6-month, 4 patients died (5%), 80% had MR ≤ 2 + and 75% were in New York Heart Association class ≤ II. Median pro-BNP decreased from 4395 pg/ml to 2594 pg/ml (p = 0.04). There were no significant changes in LV end-diastolic volume (222 ± 75 ml vs. 217 ± 79, p = 0.19), end-systolic volume (LVESV, 154 ± 66 ml vs. 156 ± 69, p = 0.54) and LVEF (30 ± 9% vs. 30 ± 12%, p = 0.86). Significant reverse remodeling (reduction of LVESV ≥ 10%) was observed in 25%, without apparent association with baseline characteristics. The number of hospitalizations for HF in comparison with the 6 months before PMVR were reduced from 1.1 ± 0.8 to 0.3 ± 0.6 (p

Objectives The study sought to provide an insight into the prevalence, characterization and possible reliable indicators of early sudden cardiac death/malignant ventricular arrhythmias (SCD/MVAs) in a large cohort of dilated cardiomyopathy (DCM). Background DCM generally affects young individuals and is characterized by an unpredictable prognosis with a non-negligible risk of SCD/MVAs, particularly in early stages of disease. Methods From 1988 to 2014, 952 patients with DCM were consecutively included in the Heart Muscle Disease Registry of Trieste. Results Globally, 20 patients (2.1% of the overall population) experienced SCD/MVAs within the first 6 months after enrollment (primary endpoint). At baseline they showed a worse functional class (New York Heart Association functional class III to IV 42% vs. 22%, p = 0.038), a longer QRS complex duration (127 ± 41 ms vs. 108 ± 33 ms; p = 0.013) and a larger indexed left ventricular end-systolic volume (LVESVI) (82 ± 49 ml/m 2 vs. 67 ± 34 ml/m 2 ; p = 0.049), as compared to patients without early SCD/MVAs. Beta-blockers were less tolerated (59% vs. 83% in patients with no early SCD/MVAs; p = 0.008), mostly due to hemodynamic intolerance. At multivariate analysis, LVESVI (odds ratio [OR]: 1.012; 95% confidence interval [CI]: 1.000 to 1.024; p = 0.043) and QRS complex duration (OR: 1.017; 95% CI: 1.003 to 1.030; p = 0.015) were independently associated with the primary endpoint, whereas beta-blockers demonstrated a protective effect (OR: 0.169, CI: 0.048 to 0.593; p = 0.006). Conclusions Patients with DCM present a significant risk of major arrhythmic events in the first phase of the disease. Baseline LVESVI, QRS duration, and intolerance to beta-blocker therapy might be useful tools in the arrhythmic early risk assessment.

Objectives Annuloplasty bands and rings are widely used to treat functional tricuspid regurgitation (TR). However, the question as to which is the ideal annuloplasty device remains open. Early and late outcomes of tricuspid valve annuloplasty with flexible band (B-TVA) or rigid ring (R-TVA) are compared in the present study. Methods Between 1999 and 2014, 462 consecutive patients (mean age, 69.2 ± 9.5 years) with grade ≥1+ functional TR (graded from 0 to 3+) underwent either B-TVA (n = 345; mean EuroSCORE II 9.2 ± 10.8%) or R-TVA (n = 117; mean EuroSCORE II 12 ± 13.4%) in addition to other cardiac procedures at the authors' institution. Results One-to-one propensity score-matched analysis resulted in 98 pairs with similar baseline characteristics and operative risk. Hospital mortality was 7.5% after B-TVA and 12% after R-TVA (P = 0.14). R-TVA was associated with higher rates of low cardiac output (10.1 vs 17.9%, P = 0.025) and transient complete atrioventricular block (10.3 vs 17.2%, P = 0.046). Among the matched pairs, there were no significant differences in hospital mortality (5.1 vs 9.2%, P = 0.27) and perioperative complications. Both in overall series and matched pairs, between B-TVA and R-TVA patients, there were no significant differences in freedom from all-cause death (P = 0.29 and 0.91), cardiac and cerebrovascular deaths (P = 0.63 and 0.87) and grade ≥2+ TR (P = 0.68 and 0.77). Right atrial and tricuspid valve reverse remodelling combined with right ventricular reverse remodelling occurred after R-TVA but not after B-TVA. Conclusions B-TVA and R-TVA are equally effective in the treatment of functional TR. However, R-TVA causes over time a more complete right heart reverse remodelling.

Dilated cardiomyopathy (DC) is the final common pathway of different pathogenetic processes and presents a significant prognostic heterogeneity, possibly related to its etiologic variety. The characterization and long-term prognosis of postmyocarditic dilated cardiomyopathy (PM-DC) remain unknown. This study assesses the clinical-instrumental evolution and long-term prognosis of a large cohort of patients with PM-DC. We analyzed 175 patients affected with DC consecutively enrolled from 1993 to 2008 with endomyocardial biopsy (EMB) data available. PM-DC was defined in the presence of borderline myocarditis at EMB or persistent left ventricular dysfunction 1 year after diagnosis of active myocarditis at EMB. Other patients were defined as affected by idiopathic dilated cardiomyopathy (IDC). Analysis of follow-up evaluations was performed at 24, 60, and 120 months. We found 72 PM-DC of 175 enrolled patients (41%). Compared with IDC, patients with PM-DC were more frequently females and less frequently presented a familial history of DC. No other baseline significant differences were found. During the long-term follow-up (median 154, first to third interquartile range 78 to 220 months), patients with PM-DC showed a trend toward slower disease progression. Globally, 18 patients with PM-DC (25%) versus 49 with IDC (48%) experienced death/heart transplantation (p = 0.045). The prognostic advantage for patients with PM-DC became significant beyond 40 months of follow-up. At multivariable time-dependent Cox analysis, PM-DC was confirmed to have a global independent protective role (hazard ratio 0.53, 95% confidence interval 0.28 to 0.97, p = 0.04). In conclusion, PM-DC is characterized by better long-term prognosis compared with IDC. An exhaustive etiologic characterization appears relevant in the prognostic assessment of DC.

OBJECTIVES: In this study, the authors analyzed the prognostic role of right ventricular systolic function (RVF) longitudinal trends in a large cohort of patients affected by dilated cardiomyopathy (DCM). BACKGROUND: RVF is a known prognostic predictor in DCM; however, whether RVF changes over time to better predict the long-term disease progression has not been investigated. METHODS: From 1993 to 2008, we analyzed 512 patients with DCM (46 years of age [36 to 55 years of age], left ventricular ejection fraction 32% [25% to 41%]) with a potential follow-up of ≥72 months and available data at baseline and at least 1 pre-specified follow-up evaluation (i.e., 6, 24, 48, or 72 months). RV dysfunction was defined as RV fractional area change

Background An important number of patients with idiopathic dilated cardiomyopathy have dramatically improved left ventricular function with optimal treatment; however, little is known about the evolution and long‐term outcome of this subgroup, which shows apparent healing. This study assesses whether real healing actually exists in dilated cardiomyopathy . Methods and Results Persistent apparent healing was evaluated among 408 patients with dilated cardiomyopathy receiving tailored medical treatment and followed over the very long‐term. Persistent apparent healing was defined as left ventricular ejection fraction ≥50% and indexed left ventricular end‐diastolic diameter ≤33 mm/m 2 at both mid‐term (19±4 months) and long‐term (103±9 months) follow‐up. At mid‐term, 63 of 408 patients (15%) were apparently healed; 38 (60%; 9% of the whole population) showed persistent apparent healing at long‐term evaluation. No predictors of persistent apparent healing were found. Patients with persistent apparent healing showed better heart transplant–free survival at very long‐term follow‐up (95% versus 71%; P =0.014) compared with nonpersistently normalized patients. Nevertheless, in the very long term, 37% of this subgroup experienced deterioration of left ventricular systolic function, and 5% died or had heart transplantation. Conclusions Persistent long‐term apparent healing was evident in a remarkable proportion of dilated cardiomyopathy patients receiving optimal medical treatment and was associated with stable normalization of main clinical and laboratory features. This condition can be characterized by a decline of left ventricular function over the very long term, highlighting the relevance of serial and individualized follow‐up in all patients with dilated cardiomyopathy , especially considering the absence of predictors for long‐term apparent healing.

Background— Analysis of right ventricular (RV) regional dysfunction by cardiac magnetic resonance (CMR) imaging in arrhythmogenic RV cardiomyopathy (ARVC) may be inadequate because of the complex contraction pattern of the RV. Aim of this study was to determine the use of RV strain and dyssynchrony assessment in ARVC using feature-tracking CMR analysis. Methods and Results— Thirty-two consecutive patients with ARVC referred to CMR imaging were included. Thirty-two patients with idiopathic RV outflow tract arrhythmias and 32 control subjects, matched for age and sex to the ARVC group, were included for comparison purpose. CMR imaging was performed to assess biventricular function; feature-tracking analysis was applied to the cine CMR images to assess regional and global longitudinal, circumferential, and radial RV strains and RV dyssynchrony (defined as the SD of the time-to-peak strain of the RV segments). RV global longitudinal strain (−17±5% versus −26±6% versus −29±6%; P P =0.001), and global radial strain (18 [12–26]% versus 22 [15–32]% versus 27 [20–39]%; P =0.015) were significantly lower and SD of the time-to-peak RV strain in all 3 directions were significantly higher among patients with ARVC compared with patients with RV outflow tract arrhythmias and controls. RV global longitudinal strain >−23.2%, SD of the time-to-peak RV longitudinal strain >113.1 ms, and SD of the time-to-peak RV circumferential strain >177.1 ms allowed correct identification of 88%, 75%, and 63% of ARVC patients with no or only minor CMR criteria for ARVC diagnosis. Conclusions— Strain analysis by feature-tracking CMR helps to objectively quantify global and regional RV dysfunction and RV dyssynchrony in patients with ARVC and provides incremental value over conventional cine CMR imaging.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disease characterized by myocyte loss and fibro-fatty tissue replacement. Diagnosis of ARVC remains a clinical challenge mainly at its early stages and in patients with minimal echocardiographic right ventricular (RV) abnormalities. ARVC shares some common features with other cardiac diseases, such as RV outflow ventricular tachycardia, Brugada syndrome, and myocarditis, due to arrhythmic expressivity and biventricular involvement. The identification of ARVC can be often challenging, because of the heterogeneous clinical presentation, highly variable intra- and inter-family expressivity and incomplete penetrance. This genotype-phenotype "plasticity" is largely unexplained. A familial history of ARVC is present in 30% to 50% of cases, and the disease is considered a genetic cardiomyopathy, usually inherited in an autosomal dominant pattern with variable penetrance and expressivity; in addition, autosomal recessive forms have been reported (Naxos disease and Carvajal syndrome). Diagnosis of ARVC relays on a scoring system, with major or minor criteria on the Revised Task Force Criteria. Implantable cardioverter defibrillators (ICDs) are increasingly utilized in patients with ARVC who have survived sudden death (SD) (secondary prevention). However, there are few data available to help identifying ARVC patients in whom the prophylactic implantation of an ICD is truly warranted. Prevention of SD is the primary goal of management. Pharmacologic treatment of arrhythmias, catheter ablation of ventricular tachycardia, and ICD are the mainstay of treatment of ARVC.

we report a prospective study to define the epidemiological, clinical, microbiological, echocardiographic characteristics and the out-come of IE in different age groups in order to evaluate age-related differences.

n/a

Heart failure is a complex syndrome characterized by impaired emptying and/or impaired filling of the heart chambers. The use of parameters of diastolic function has provided novel tools for risk stratification and management of patients with heart failure. This study evaluated the potential correlation between apoptosis at time of death and left ventricular (LV) diastolic function after acute myocardial infarction. We selected, at routine postmortem examination, 14 subjects who died 10 to 62 days after an acute myocardial infarction and had an available echocardiographic report from the most recent hospital admission. The apoptotic rate was calculated at the region bordering the infarct, using co-localization of in situ end-labeling for deoxyribonucleic acid fragmentation and immunohistochemistry for caspase-3. Transthoracic echocardiographic studies were retrospectively reevaluated and pulse-wave Doppler spectra of mitral inflow were analyzed. LV diastolic function was assessed by measuring the ratio of E peak velocity to A peak velocity and E-wave deceleration time; a ratio of E peak velocity to A peak velocity ≥2 and deceleration time

n/a

In this chapter describes clinical and imaging assessment in diagnosis and patient management of other cardiomyopathies (CMP) not included among the previously defined main groups of CMP. Most of these unclassified CMP are characterized by frequent reversibility of myocardial dysfunction after adequate treatment. The peculiar form called left ventricular noncompaction is also addressed.

Background Genotype–phenotype correlations are poorly characterised in arrhythmogenic right ventricular cardiomyopathy (ARVC). We investigated whether carriers of rare variants in desmosomal genes (DC) and titin gene ( TTN ) display different phenotypes and clinical outcomes compared with non-carriers (NT-ND). Methods and results Thirty-nine ARVC families (173 subjects, 67 affected) with extensive follow-up (mean 9 years), prospectively enrolled in the International Familial Cardiomyopathy Registry since 1991, were screened for rare variants in TTN and desmosomal genes ( DSP, PKP2, DSG2, DSC2 ). Multiple clinical and outcome variables were compared between three genetic groups ( TTN, DC, NT-ND) to define genotype–phenotype associations. Of the 39 ARVC families, 13% (5/39) carried TTN rare variants (11 affected subjects), 13% (5/39) DC (8 affected), while 74% (29/39) were NT-ND (48 affected). When compared with NT-ND, DC had a higher prevalence of inverted T waves in V2-3 (75% vs 31%, p=0.004), while TTN had more supraventricular arrhythmias (46% vs 13%, p=0.013) and conduction disease (64% vs 6% p

The aims of this study were to investigate the clinical outcomes of patients with low-gradient aortic stenosis despite preserved left ventricular ejection fraction and to assess reliable prognostic clinical-instrumental features in patients experiencing or not experiencing aortic valve replacement (AVR). Clinical-laboratory and echocardiographic data from 167 patients (median age 78 years, interquartile range 69 to 83) with aortic valve areas 70 years showed a trend toward being a prognostic predictor (p = 0.082). In conclusion, in patients with low-gradient aortic stenosis despite a preserved left ventricular ejection fraction, AVR was strongly correlated with a better prognosis. Patients with atrial fibrillation associated with advanced New York Heart Association class had the worst prognosis if treated medically but at the same time a relative better benefit from surgical intervention.

Infiltrative/storage cardiomyopathies (CMP) are diseases characterized by the deposition of pathological substances in the heart muscle. In particular, the term infiltrative refers to intercellular infiltration, whereas storage refers to intracellular deposition within the myocardium. As the infiltration/storage usually involves multiple organs, these CMP should be considered part of a systemic disorder. Despite variable etiology and pathological mechanisms, this group of CMP shares some common features, such as myocardial hypertrophy and progressive myocardial stiffness, with diastolic and systolic dysfunction. Heart failure is the main clinical manifestation, but arrhythmias and conduction defects are common. Accurate clinical evaluation is therefore crucial for correctly targeting further examinations. Moreover, cardiac imaging plays a major role not only in differential diagnosis but also in prognostic stratification. The impact of identifying cardiac involvement and the underlying pathology reflects upon treatment and prognosis.

Studies relating to arrhythmogenic right ventricular cardiomyopathy (ARVC) have demonstrated that mutations in genes encoding different components of the desmosome, a major cell-adhesion structure, are the main genetic cause of the disease, which is histologically characterized by right ventricular myocellular atrophy with characteristic fibro-fatty replacement [1]. From a structural point of view, as observed by imaging techniques such as echocardiography (Fig. 7.1) and cardiac magnetic resonance imaging, the pathological hallmark of the disease is a thin right ventricle usually with bulges and sacculations, which are typically located in the “triangle of dysplasia” (apex, outflow tract and subtricuspid areas). Symptomatic or asymptomatic arrhythmias of right ventricular origin are usually present, and in more advanced cases a severe right ventricular enlargement with systolic dysfunction can be present.

Pulmonary artery aneurysm unassociated to congenital heart disease and pulmonary hypertension is exceedingly rare. Its pathogenesis and correct management remain unknown. Sarcoidosis is a systemic disease that can exceptionally involve large vessels, leading to stenosis and dilatation. Pulmonary artery aneurysm has never been described in association with sarcoidosis. Surgical approach should prevent aneurysm rupture, but it is not known when surgery should be preferred to strict medical follow-up. In this report we present a case of large pulmonary artery aneurysm associated to systemic sarcoidosis underlining problematic management of diseases 'forgotten' by evidence based medicine.

We would like to thank Chemla et al .1 for their interest in our recently published work. Their letter, however, raises some critical concerns that should be appropriately addressed. In fact, as expected, in our patient population the mean bias of the modified formula is nearly zero, due to the property of the least squares regression optimization algorithm. Therefore, the standard deviation is comparable between the two formulas. In the validating sample, comparable absolute …

BACKGROUND AND AIM: Insulin resistance is recognized as the pathophysiological hallmark of non-alcoholic fatty liver disease (NAFLD). A relation between insulin sensitivity and left ventricular morphology and function has been reported in essential hypertension, where a high prevalence of NAFLD has been recently found. We investigated the inter-relationship between left ventricular morphology/function, metabolic parameters and NAFLD in 86 never-treated essential hypertensive patients subdivided in two subgroups according to the presence (n = 48) or absence (n = 38) of NAFLD at ultrasonography. METHODS AND RESULTS: The two groups were similar as to sex, age and blood pressure levels. No patient had diabetes mellitus, obesity, hyperlipidemia, or other risk factors for liver disease. Body mass index, waist circumference, triglycerides, glucose, insulin, homeostasis model of assessment index for insulin resistance (HOMA-IR), aspartate aminotransferase and alanine aminotransferase were higher and adiponectin levels were lower in patients with NAFLD than in patients without NAFLD, and were associated with NAFLD at univariate analysis. Patients with NAFLD had similar prevalence of left ventricular hypertrophy compared to patients without NAFLD, but a higher prevalence of diastolic dysfunction (62.5 vs 21.1%, P < 0.001), as defined by E/A ratio 220 ms. Diastolic dysfunction (P = 0.040) and HOMA-IR (P = 0.012) remained independently associated with NAFLD at backward multivariate analysis. CONCLUSIONS: Non-alcoholic fatty liver disease was associated with insulin resistance and abnormalities of left ventricular diastolic function in a cohort of patients with essential hypertension, suggesting a concomitant increase of metabolic and cardiac risk in this condition.