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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 <50%): 136 (44%) had a history of systemic hypertension and were defined HHC. The remaining 174 patients were considered IDC. Compared with patients with IDC, those with HHC were older (63 ± 11 vs 47 ± 14 years, p <0.001), with worse comorbidity profile, higher blood pressure, and increased LV mass. During follow-up, patients with HHC showed earlier and higher proportion of LV reverse remodeling (46% vs 21% at 6 months' follow-up). Moreover, they had a better long-term survival free from cardiovascular death/ventricular assist device/heart transplant/malignant ventricular arrhythmias (5.1 vs 12.6 in HHC and IDC, p = 0.03). Indeed, their mortality was mainly driven by noncardiovascular causes (at 10 years 9.6% vs 1.7% in HHC and IDC, p <0.001). In conclusion, HHC has a high prevalence among patients with "idiopathic" LV dysfunction. The natural history of patients with HHC is characterized by a rapid response to optimal therapy for heart failure, a favorable cardiovascular outcome, and a relevant incidence of noncardiovascular events., (Copyright © 2016 Elsevier Inc. All rights reserved.)

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., (Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2016 Elsevier Inc. All rights reserved.)

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 <35% at 2-dimensional echocardiography. The primary outcome measure was a composite of death or heart transplantation., Results: At enrollment, 103 (20%) patients had RV dysfunction. During follow-up, 89 of them (86%, 17% of the overall cohort) normalized RVF at a median time of 6 months, whereas 38 of the remaining 409 patients with normal baseline RVF (9%; 7% of the overall population) exhibited a new-onset RV dysfunction (median time: 36 months). RVF normalization was significantly associated with subsequent left ventricular reverse remodeling that was observed at a median time of 24 months (odds ratio: 2.49; 95% confidence interval [CI]: 1.17 to 5.3; p = 0.018). At baseline multivariate analysis, RV dysfunction was independently associated with the primary outcome measure (hazard ratio: 1.71; 95% CI: 1.02 to 2.85; p = 0.0413). At time-dependent model, RVF revaluation over time maintained an independent predictive value (hazard ratio: 2.83; 95% CI: 1.57 to 5.11; p = 0.0006)., Conclusions: Patients with DCM frequently present RV dysfunction at first evaluation. However, a complete RVF recovery is largely observed early after optimization of medical therapy and predates subsequent left ventricular reverse remodeling. Systematic revaluation of patients including RVF throughout regular follow-up conferred additive long-term prognostic value to the baseline evaluation., (Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Constrictive pericarditis is a rare pericardial disorder that causes an impairment of cardiac filling and frequently heart failure. The clinical presentation is non-specific and the differential diagnosis includes myocardial diseases, particularly restrictive cardiomyopathy. Echocardiography has a central role in the initial diagnosis. Some peculiar signs, such as abnormal inspiratory shift of the interventricular septum, increased respiratory variations of transmitral, transtricuspid and hepatic vein flow velocities and the normality of early diastolic relaxation velocity (e') at tissue Doppler, increase the likelihood of the disease. These signs are an expression of increased ventricular interdependence and dissociation between intrathoracic and intracardiac pressures typical of pericardial constriction. For further diagnosis, computed tomography and magnetic resonance are used to identify the presence of pericardial thickening. Invasive cardiac catheterization is indicated in dubious cases and in the assessment of the severity of hemodynamic abnormalities, especially in cases with surgical indication. Pericardiectomy is indicated in symptomatic patients meeting the diagnostic criteria of constrictive pericarditis and is able to improve the prognosis.

Dilated cardiomyopathy (DCM) is a relatively rare primary heart muscle disease with genetic or post-inflammatory etiology. In the last decade, the incidence and prevalence of the disease have significantly increased as a consequence of an earlier diagnosis supported by extensive familial screening programs and by the improvement in diagnostic techniques. Moreover, current therapeutic strategies have deeply modified the prognosis of DCM with a dramatic reduction in mortality. A significant number of patients with DCM present an impressive response to pharmacological and non-pharmacological therapy in terms of left ventricular reverse remodeling (reduction in ventricular size with improvement of systolic function), which confers a more favorable prognosis in the long term. However, the identification of patients with an increased likelihood of improvement after therapeutic optimization remains a challenging issue; in particular the assessment of arrhythmic risk carries important implications. Finally, the long-term follow-up of patients showing a significant left ventricular functional recovery under optimal treatment is still poorly known. Hence, the aim of the present review is to provide an insight into the clinical evolution/long-term follow-up of DCM, which should be actually considered a dynamic process rather than a static and chronic disease. Left ventricular reverse remodeling should be considered a key therapeutic goal, mostly associated with a long-standing recovery, but cannot be considered the expression of permanent "healing", confirming the need for a systematic and careful follow-up over time in this setting.

Background and Aim of the Study: The Carpentier-McCarthy-Adams IMR ETlogix annuloplasty ring was specifically designed to treat ischemic mitral regurgitation (IMR) associated with asymmetric mitral annular dilation and leaflet tethering. The study aim was to review, retrospectively, the results of mitral annuloplasty with this asymmetric ring in a representative number of patients., Methods: Between January 2005 and July 2012, the IMR ETlogix ring was implanted in 190 consecutive patients (mean age 69.5 +/- 7.6 years) with grade > or =2+ IMR (graded from 0 to 3+). Preoperatively, 37 patients (19.5%) were in NYHA class IV, and 73 (38.4%) suffered from unstable angina. The operative risk according to the European System for Cardiac Operative Risk Evaluation II was 15.6 +/- 14.5%. Using two- dimensional echocardiography, postoperative changes in mitral annular diameter (MAD) and tenting height (TH) of the mitral valve in four-chamber, two-chamber and long-axis views, were assessed at mid-systole., Results: Thirty-eight patients (20.0%) received one or more concomitant major cardiac surgical procedure(s) other than, or in addition to, coronary artery bypass grafting or tricuspid valve annuloplasty. Nineteen (10.0%) hospital deaths occurred, and one patient underwent immediate reoperation for residual MR. During the follow up (mean 4.8 +/- 2.1 years) there were 26 cardiac deaths, 14 non-cardiac deaths, and three mitral valve replacements. The seven-year actuarial survival, freedom from grade > or =2+ MR and reoperation were 62.0%, 93.1% and 97.6%, respectively. Renal impairment (p = 0.012) and extracardiac arteriopathy (p = 0.047) were predictors of death; bilateral internal thoracic artery grafting was a protective factor (p = 0.033). Heart failure symptoms were improved (p <0.01). Left ventricular reverse remodeling was achieved in 50.6% of patients. The MAD and TH were each decreased in all three echocardiographic views (p < 0.001), the reductions being greater in the long-axis view., Conclusion: By restoring the mitral apparatus geometry and competence, asymmetric annuloplasty with the IMR ETlogix ring provides good mid-term outcomes and helps left ventricular reverse remodeling in IMR.

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<0.001). When compared with the NT-ND group, the DC group experienced a worse prognosis (67% vs 11%, p=0.03) and exhibited a lower survival free from death or heart transplant (59% vs 95% at 30 years, and 31% vs 89% at 50 years, HR 9.66, p=0.006), while the TTN group showed an intermediate survival curve (HR 4.26, p=0.037)., Conclusions: TTN carriers display distinct phenotypic characteristics including a greater risk for supraventricular arrhythmias and conduction disease. Conversely, DC are characterised by negative T waves in anterior leads, severe prognosis, high mortality and morbidity., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Aims: ACE-inhibitors, β-blockers, implantable cardioverter-defibrillator (ICD) and cardiac resynchronization therapy (CRT) improved prognosis of heart failure. We sought to analyse the long-term prognostic impact of evidence-based integrated therapeutic strategies in patients with idiopathic dilated cardiomyopathy (IDCM)., Methods and Results: From 1978 to 2007, 853 IDCM patients (45 ± 15 years, 72% males) were enrolled and classified as follows: Group 1, 110 patients (12.8%) enrolled during 1978-1987; Group 2, 376 patients (44.1%) enrolled during 1988-1997; Group 3, 367 patients (43.1%) enrolled during 1998-2007. ACE-inhibitors/angiotensin receptor blockers were administered in 34%, 93%, and 93% (P <0.001), and β-blockers in 11%, 82%, and 86% (P <0.001) in Groups 1, 2, and 3, respectively; ICDs were implanted in 2%, 14%, and 13% (P = 0.005); mean time to device implantation was lower in Group 3. At 8 years, heart transplant (HTx)-free survival rates were 55%, 71%, and 87% in Groups 1, 2, and 3, respectively (P <0.001). Similar progressive improvement was found for pump-failure death (DHF)/HTx, while survival free from sudden death (SD) was significantly improved only in Group 3. Multivariable models considering competing risk indicated early diagnosis (i.e. a baseline less advanced disease stage) and tailored medical therapy (HR 0.44, CI 95% 0.19-0.98) as independent protectors against DHF/HTx. Concerning SD, lower left ventricular ejection fraction emerged as a predictor, while ICD was the only therapy with a protective role (HR 0.08, CI 95% 0.01-0.61). Treatment with digitalis emerged as a predictor of both DHF/HTx and SD., Conclusions: An effective management and evidence-based integrated therapeutic approach progressively and significantly improved the long-term prognosis of IDCM during the last three decades., (© 2013 The Authors. European Journal of Heart Failure © 2013 European Society of Cardiology.)

Background/objective: Left ventricular (LV) circumferential or longitudinal shortening may be impaired in patients with type 2 diabetes mellitus (DM). In the present study, patients with type 2 DM without myocardial ischemia and combined impairment of circumferential and longitudinal (C+L) shortening were studied to assess the prevalence and factors associated with this condition., Methods: Data from 386 patients with type 2 DM enrolled in the SHORTening of midWall and longitudinAl left Ventricular fibers in diabEtes study were analyzed. One hundred twenty healthy subjects were used to define C+L dysfunction. Stress-corrected midwall shortening and mitral annular peak systolic velocity were considered as indexes of C+L shortening and classified as low if <89% and <8.5 cm/s, respectively (10th percentiles of controls)., Results: Combined C+L dysfunction was detected in 66 patients (17%). The variables associated with this condition were lower glomerular filtration rate (OR 0.98 [95% CI 0.96 to 0.99], greater LV mass (OR 1.05 [95% CI 1.02 to 1.08]), high pulmonary artery wedge pressure (OR 1.23 [95% CI 1.04 to 1.44]) and mitral annular calcifications (OR 3.35 [95% CI 1.71 to 6.55]). Considering the entire population, the relationship between stress-corrected midwall shortening and peak systolic velocity was poor (r=0.20), and the model was linear. The relationship was considerably closer and nonlinear in patients with combined C+L dysfunction (r=0.61; P<0.001), having the best fit by cubic function., Conclusions: Combined C+L dysfunction was present in one-sixth of patients with type 2 DM without myocardial ischemia. This condition was associated with reduced renal function, worse hemodynamic status and structural LV abnormalities, and may be considered a preclinical risk factor for heart failure.

Background and Aims: Cardiac amyloidosis (CA) is usually characterised by a poor outcome in the short-term; clinical and instrumental features are heterogeneous and could characterise subgroups with different prognoses. The aim of our study was to describe a subgroup of patients with CA showing an impressive favourable long-term survival., Methods: Out of 50 patients (males 65%, 63 ± 11 years) with an echocardiographic and bioptic diagnosis of CA observed from 1991 to 2009, we selected a subgroup of patients surviving more than 50 months from diagnosis (group 1). We described their features at enrolment and during follow-up, comparing them with patients surviving less than 12 months (group 2)., Results: We found seven patients (14%) belonging to group 1 and 26 (52%) to group 2. Four out of seven long term survivors suffered from AL amyloidosis, in one case the underlying aetiology was a chronic inflammatory disease, while in two cases remained unknown. At enrolment, group 1 patients showed higher systolic blood pressure with respect to group 2 (140 ± 25 vs. 112 ± 18 mmHg, respectively, p=0.011), and a less thick interventricular septum (IVS) (IVS thickness > 15 mm in 29% vs. 69% of patients, p = 0.049). No patient of group 1 presented left ventricular restrictive filling pattern (0 vs. 31% in group 1 and 2 respectively, p = 0.035), atrial fibrillation (0 vs. 35%, p = 0.024), or progression towards a more severe disease during follow-up., Conclusions: A not negligible proportion of patients with CA can have a long-term survival. They showed a less severe disease at diagnosis, with substantial stability over time. Further studies on larger populations are necessary to understand the mechanisms underlying this more favourable natural history of the disease., (Copyright © 2013 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Background and Aim of the Study: For patients who require aortic root replacement but are unwilling or unable to receive anticoagulants, a composite conduit was assembled intraoperatively that contained a stented biological valve sutured inside a vascular tube graft, rather than at its extremity. This simple modification of the Bentall concept may provide several advantages. The results obtained with this conduit over an 11-year period were analyzed., Methods: Between May 2001 and April 2012, 101 consecutive patients (mean age 68.3 +/- 9.2 years) underwent aortic root replacement with the bioprosthetic valved conduit. Aortic pathologies included degenerative disease in 61 patients (60.4%), atherosclerosis in 20 (19.8%), annuloaortic ectasia in 12 (11.9%), porcelain aorta in four (4.0%), and acute dissection in four (4.0%). The whole ascending aorta was replaced in 79 patients (78.2%); a hemiarch reconstruction and a total arch replacement were added in 18 (17.8%) and four (4.0%) patients, respectively. Hypothermic circulatory arrest was performed in 60 cases (59.4%). Forty patients (39.6%) underwent additional cardiac procedures. All perioperative data were collected prospectively., Results: There were five (5.0%) hospital deaths. During a mean follow up of 3.8 +/- 2.4 years there were two non-valve-related cardiac deaths and five noncardiac deaths. The seven-year actuarial survival was 79.2% (95% CI 67.0-91.4%). Bioprosthetic structural dysfunction occurred in only one patient; reoperation was easily performed by replacing the valve within the vascular graft. In the remaining 88 patients (87.1%), echocardiographic assessment showed a low transaortic mean pressure gradient (7.2 +/- 4.7 mmHg) and left ventricular wall mass reduction (p = 0.0002)., Conclusion: This valved conduit is a safe and durable option for replacing the aortic root, thus facilitating the technique of implantation and simplifying reoperation in the case of valve failure.

Aims: To identify a correction of the modified Bernoulli formula used to estimate systolic and mean pulmonary artery pressure [sPAP and mPAP; respectively: sPAP = 4 × TRv (tricuspid regurgitation velocity)(2)+ RAP (right atrial pressure); and mPAP = 0.61sPAP + 2], applicable in the follow-up of pulmonary hypertension (PH) patients., Methods and Results: From January 1979 to December 2009, 60 patients with precapillary (class I and IV) and 'out of proportion' PH were consecutively enrolled in the PH Registry of Trieste. All patients underwent both echocardiographic and right heart catheter evaluation. We used a simple-linear-regression method in order to compare sPAP and mPAP Doppler-estimated values with the respective right-heart catheterization invasive variables. The comparison of the estimated with the traditional modified Bernoulli formula echo-Doppler data and the effective invasive values confirmed a significant association between them (for sPAP P< 0.001; for mPAP P= 0.006). Simple-linear-regression-derived formulas were sPAP = 1.07 × (4TRv(2)+ RAP) + 7.4 (1) and mPAP = 1.1 × (0.61sPAP + 2) + 2.5 (2). These regression-corrected formulas were validated in an external population of PH patients., Conclusion: Our data suggest that formulas (1) and (2) could be more reliable with respect to the traditional modified Bernoulli equation, when estimating echocardiographically sPAP and mPAP in patients with PH confirmed by right-heart catheterization.

Although right ventricular (RV) failure is the main cause of death in patients with pulmonary arterial hypertension (PAH), there is insufficient data about the effects of PAH treatment on RV geometry and function mainly because the RV assessment has been hampered by its complex crescentic shape, large infundibulum, and its trabecular nature. Echocardiography is a widely available imaging technique particularly suitable for follow-up studies, because of its non-invasive nature, low cost, and lack of ionizing radiation or radioactive agent. Real-time three-dimensional echocardiography (RT3DE) has been shown to be accurate in assessing RV and left ventricular (LV) volumes, stroke volumes, and ejection fractions in comparison with cardiac magnetic resonance imaging. In this review, we describe RV structural and functional changes which occur in patients with PAH and strengths and weaknesses of current non-invasive imaging techniques to assess them. Finally, we describe an ongoing multicentre, prospective observational study involving seven centres expert in treating patients with PAH from four different countries. Investigators will use conventional and advanced echo parameters from RT3DE and speckle-tracking echocardiography to assess the extent of LV and RV remodelling before symptom onset and during pharmacological treatment in patients with PAH. Seventy patients who will survive for at least 1 year will be recruited. All the participating institutions will perform comprehensive standard 2D and Doppler as well as RT3DE examinations with a pre-defined imaging protocol. Measurements will be performed at the core echocardiography laboratory by experienced observers who will be unaware of each patient's treatment assignment and whether the examination was a baseline or a follow-up study. Enrolment duration is expected to be 1 year.

Aim: To study frequency and incremental prognostic value of restrictive filling pattern (RFP) in hypertrophic cardiomyopathy (HCM)., Methods and Results: Eighty-seven consecutive HCM patients (64% men, mean age 45 +/- 19 years) underwent physical and Doppler echocardiographic evaluation at our centre from March 1993 to February 2001. Mean length of follow-up was 96 +/- 54 months. RFP was found in 14 patients (16%) at index evaluation. Patients with RFP had higher NYHA class, more frequent signs of heart failure and lower left ventricular ejection fraction (P = 0.018, P = 0.002 and P = 0.001, respectively). During follow-up, cardiac death plus heart transplantation was significantly higher in HCM patients with RFP than in those without RFP (P = 0.0001). NYHA class (HR = 5.95, 95% CI: 1.34-26.38, P = 0.019), indexed left atrial diameter (HR = 1.68, 95% CI: 1.01-2.82, P = 0.047) and RFP (HR = 2.94, 95% CI: 1.25-6.88, P = 0.01) were selected as predictors of cardiac death or heart transplantation in a multivariate proportional hazard model. The AUC of ROC curve from multivariate regression models for predicting adverse outcome significantly improved from 0.76 considering only NYHA class to 0.84 after inclusion of RFP and indexed left atrial diameter (P = 0.01)., Conclusions: RFP is rare, but not exceptional, in HCM. Echo-Doppler evaluation of filling pattern confers additional prognostic power to clinical stratification.

We report a case of a 57 year-old woman with Starr-Edwards model 6120 mitral valve replacement and Kay-Shiley bioprosthetic tricuspid valve replacement in 1968 at Niguarda Hospital in Milan. The mitral caged-ball has proved its excellent durability and its good hemodynamic performance in many patients, even if subject to high tendency to thrombosis. In literature there is no evidence of durability of this prosthesis longer than 35 years. Our patient after 39 years from mitral valve replacement lives a happy and fulfilling life (NYHA II), with no evidence of hemolysis, ball variance, symptomatic embolization or major bleeding.

The triad of pneumonia, meningitis, and endocarditis due to Streptococcus pneumoniae is known as Austrian syndrome. We report a case with an aortic-right atrium fistula in a 39-year-old woman who had undergone splenectomy for Hodgkin's lymphoma. The review of literature shows that the prevalence of Austrian syndrome is decreasing from 19% to 3% of patients with pneumococcal endocarditis in recent years. This case emphasizes that diagnosis of endocarditis should be considered early in every patient with pneumococcal meningitis or bacteremia, particularly in immunocompromised patients.

Background: Autopsy studies report a frequency of myocarditis ranging from 0.11 to 5.5% in the general population, reaching almost 50% in selected groups. Myocarditis is often undiagnosed and the incidence of fatal course myocarditis has never been evaluated. The aim of our study was to assess the frequency of fatal course myocarditis in a consecutive series of autopsies and to describe the clinical, histological and morphologic features of the disease., Methods: From January 1, 1995 to January 31, 1996, 2560 autopsies were performed, and 143 cases of active myocarditis were diagnosed (5.6%)., Results: In 39 cases (1.5%; 12 males; 4/39 aged < or = 35 years) active myocarditis was identified as the final cause of death. Only in 1 case was myocarditis suspected ante-mortem. The histological pattern was lymphocytic in 64% of cases. A mixed inflammatory infiltration was found in 33% and a granulomatous infiltration in 3%. In 49% of cases myocarditis was localized in both ventricles and the interventricular septum. The clinical presentation of myocarditis was heart failure in 18/39 patients (46%), cardiac arrest in 4/39 patients (10%) and syncope and chest pain in 1/39 patient (3%). The mean creatine phosphokinase levels were 890 +/- 2742 IU/I (assessed in 11/39 patients, 28%) but they were increased only in 7/39 (18%). ECG (performed in 29/39 patients, 74%) showed sinus rhythm in 16/39 patients (55%, > 100 b/min in 41%), atrioventricular or interventricular conduction defects in 10/39 patients (34%) and a pathological Q wave in 4/39 patients (14%). At echocardiography (performed in 7/39 patients, 18%), right and/or left ventricular dysfunction was found to be present in 5 cases (71%) and a pericardial effusion in 4 cases (57%)., Conclusions: Myocarditis is underdiagnosed ante-mortem. A high index of clinical suspicion is mandatory for prompt diagnosis and treatment of this fatal disease seen also in the young.

Dilated cardiomyopathy (DCM), a heart muscle disease characterized by ventricular dilation and dysfunction, is a leading cause of mortality and morbidity. In the present paper we will consider the main results of studies on the natural history of DCM in 581 consecutive patients prospectively enrolled and systematically followed in the Heart Muscle Disease Registry of Trieste in the last 25 years. In the last decades prognosis of DCM significantly improved over time, mainly as a consequence of optimized treatment with ACE-inhibitors and beta-blockers. However, a strong heterogeneity of prognosis was observed among patients both in familial and sporadic cases. Early diagnosis and treatment allowed to recognize two distinct subgroups, one with a rapidly progressive downhill course, high mortality and urgent indication to heart transplantation, another with a more favorable outcome. Long-term optimized treatment with ACE-inhibitors (in 90% of cases) and beta-blockers (in 87% of cases) was associated with a remarkable clinical improvement in 50% of patients and apparent "healing" in 16% of cases. A systematic and accurate echocardiographic follow-up showed in these cases a significant improvement of the left ventricular ejection fraction (LVEF) with "reverse remodeling", frequently associated with a decrease of severity of functional mitral regurgitation and regression of the restrictive filling pattern. The response to optimal treatment showed a strong relation to long-term outcome. The 8-year transplant-free survival, starting from the evaluation at 2 years, was 31% in patients with persistent NYHA class III-IV, 64% in NYHA class I-II and LVEF < or = 40%, 83% in NYHA class I-II and LVEF > 40% and 94% in patients with apparent "healing" (p < 0.0001). Long-term follow-up showed a significant clinical progression of the disease in 33% of cases, independently of the initial clinical response to treatment. Predictive factors of a favorable response to beta-blocker treatment associated with ACE-inhibitors were a history of mild hypertension, an early diagnosis and treatment and the presence of sinus tachycardia. The risk of sudden death was increased particularly in patients with long-term persistent or progressive left ventricular dilation and dysfunction. A rigorous pharmacological approach (optimization of beta-blockers, withdrawal or decrease of dosage of digitalis), and selective non-pharmacological strategy (automated implantable cardioverter-defibrillators for primary prevention in high-risk patients) are potentially effective to decrease the incidence of sudden death during long-term follow-up. In conclusion, the Heart Muscle Disease Registry of Trieste gave us in the last 25 years new insights into the natural history of DCM, underlying the importance of a rigorous and systematic approach both at clinical presentation and during long-term follow-up on optimized medical treatment.

Dilated cardiomyopathy (DCM), a heart muscle disease characterized by ventricular dilation and dysfunction, is a leading cause of mortality and morbidity. In the present paper we will consider the main results of studies on the natural history of DCM in 581 consecutive patients prospectively enrolled and systematically followed in the Heart Muscle Disease Registry of Trieste in the last 25 years. In the last decades prognosis of DCM significantly improved over time, mainly as a consequence of optimized treatment with ACE-inhibitors and beta-blockers. However, a strong heterogeneity of prognosis was observed among patients both in familial and sporadic cases. Early diagnosis and treatment allowed to recognize two distinct subgroups, one with a rapidly progressive downhill course, high mortality and urgent indication to heart transplantation, another with a more favorable outcome. Long-term optimized treatment with ACE-inhibitors (in 90% of cases) and beta-blockers (in 87% of cases) was associated with a remarkable clinical improvement in 50% of patients and apparent "healing" in 16% of cases. A systematic and accurate echocardiographic follow-up showed in these cases a significant improvement of the left ventricular ejection fraction (LVEF) with "reverse remodeling", frequently associated with a decrease of severity of functional mitral regurgitation and regression of the restrictive filling pattern. The response to optimal treatment showed a strong relation to long-term outcome. The 8-year transplant-free survival, starting from the evaluation at 2 years, was 31% in patients with persistent NYHA class III-IV, 64% in NYHA class I-II and LVEF < or = 40%, 83% in NYHA class I-II and LVEF > 40% and 94% in patients with apparent "healing" (p < 0.0001). Long-term follow-up showed a significant clinical progression of the disease in 33% of cases, independently of the initial clinical response to treatment. Predictive factors of a favorable response to beta-blocker treatment associated with ACE-inhibitors were a history of mild hypertension, an early diagnosis and treatment and the presence of sinus tachycardia. The risk of sudden death was increased particularly in patients with long-term persistent or progressive left ventricular dilation and dysfunction. A rigorous pharmacological approach (optimization of beta-blockers, withdrawal or decrease of dosage of digitalis), and selective non-pharmacological strategy (automated implantable cardioverter-defibrillators for primary prevention in high-risk patients) are potentially effective to decrease the incidence of sudden death during long-term follow-up. In conclusion, the Heart Muscle Disease Registry of Trieste gave us in the last 25 years new insights into the natural history of DCM, underlying the importance of a rigorous and systematic approach both at clinical presentation and during long-term follow-up on optimized medical treatment.

Typical morphological and functional abnormalities of dilated cardiomyopathy can be well evaluated non-invasively by the echocardiographic and echo-Doppler study. However it is important to remember that left ventricular dilation and depressed systolic function can be found also in other diseases that may mimic idiopathic dilated cardiomyopathy (coronary heart disease, myocarditis, biventricular dysplasia, advanced hypertrophic cardiomyopathy, hypertensive or valvular heart diseases). Unusual echocardiographic findings for dilated cardiomyopathy, i.e. a mildly dilated and/or hypertrophic left ventricle, segmental rather than diffuse wall motion abnormalities, predominant right ventricular dilation and dysfunction or severe valvular abnormalities should increase the clinical suspicion of a different disease. Transesophageal and dobutamine stress echocardiography can be employed in selected patients. However, for definitive diagnosis invasive hemodynamic study with coronary angiography and sometimes endomyocardial biopsy is often required. The diagnostic role of other non-invasive studies, such as magnetic resonance, computed tomography and nuclear techniques, is not well known, considering the cost and the limited availability of these clinical tools.