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Sacubitril/valsartan reduces heart failure (HF)-related hospitalizations and cardiovascular mortality in PARADIGM-HF and has become a foundational treatment for HF with reduced ejection fraction (HFrEF). However, data of its routine real-world use are limited, and evidence from Italian settings is lacking. The REAL.IT study aimed to characterize the demographics, pharmacotherapy, clinical characteristics and outcomes of sacubitril/valsartan-treated Italian patients with HFrEF. Electronic medical records of patients initiating sacubitril/valsartan from October 2016 to June 2019 at nine specialized hospital outpatient HF centers across Italy were reviewed. Overall, 924 adults (mean age 64.5 years, 84.6% male) were included. At baseline, 38.7% had an ischemic HF etiology, 45.9% hypertension, 23.2% atrial fibrillation, 25.4% diabetes mellitus, 26.1% an implantable cardioverter-defibrillator and 31.9% coronary artery bypass grafting. There were no clear patterns of patient selection over time. During follow-up, NYHA class improved in 37.5% of patients after a mean of 5.3 ± 3.8 months; 36.1% and 16.7% of patients were in NYHA class III during characterization and after one year of follow-up, respectively. Left ventricular ejection fraction (LVEF) improved ≥5% in 56.3% of patients at one year; 39.7% had ≥30% reduction of N-terminal pro-B-type natriuretic peptide; 2.2% had hyperkalemia during characterization and 2.6% during follow-up; and 3.8% had hypotension during characterization and 12% during follow-up. A total of 50 (5.8%) of patients had device implantation (ICD/CRT) during follow-up. HF-related hospitalization was recorded in 19.6% of patients during follow-up; 3.8% of patients died, approximately 1.3% from cardiovascular causes. Our real-world data confirm the favorable effectiveness and tolerability of sacubitril/valsartan observed in pivotal randomized controlled trials.

The psychometric properties of the core disease-specific 14-item Italian HeartQoL health-related quality of life questionnaire have been evaluated in this study. The Italian version of the HeartQoL, the MacNew questionnaire, and the Hospital Anxiety and Depression Scale were completed by 472 patients (angina, N = 183; myocardial infarction, N = 167; or ischemic heart failure, N = 122) who were recruited in five Italian centers (Florence, Veruno, Turin, Udine, and Naples) between 2015 and 2017. Patients with myocardial infarction reported significantly higher HeartQoL scores than patients with angina or ischemic heart failure. Floor and ceiling effects were always minor on the HeartQoL global scale and physical subscale with moderate ceiling effects on the emotional subscale in the total group and in patients with myocardial infarction. The bifactorial structure of the original HeartQoL questionnaire was confirmed with strong physical, emotional, and global scale H coefficients (> 0.50). The HeartQoL scales demonstrated optimal internal consistency (Cronbach’s alpha > 0.84). Convergent and divergent validity were confirmed. Discriminative validity was not confirmed for age, largely confirmed for sex, and fully confirmed for anxiety, depression, and distress. The Italian HeartQoL questionnaire demonstrated adequate key psychometric attributes of internal consistency reliability and validity in Italian-speaking patients with ischemic heart disease.

Background Left cardiac sympathetic denervation (LCSD) is an established therapy for refractory ventricular arrhythmias (VAs) in channelopathies. A multicentric American and Indian case series suggested a greater efficacy of bilateral denervation (BCSD) in patients with structural heart disease (SHD). Purpose To describe our single-center experience with BCSD in SHD. Methods Nine patients (78% male, mean 55±18 yrs, mean LVEF 31±14%) with SHD and refractory VAs underwent BCSD. All had a Video-Assisted Thoracoscopic Surgery (VATS), in 2 cases associated with the robotic technique. The underlying cardiomyopathy (CMP) was non-ischemic (NICMP) in most cases (n=5, 55%), ischemic in 2 cases, arrhythmogenic right ventricular (ARVC) in one and related to lamin A/C deficiency in one. All patients had an ICD, 44% (n=4) a CRT-D. NYHA functional class I was present in 4 patients, the rest were in NYHA class II (n=3) or III (n=2). Three patients were candidates to heart transplant/LV assistance device. The arrhythmic burden pre BCSD included in 7 pts (78%) a history of electrical storm (ES); the median number of shocks/patient in the 12 months before BCSD was 5 (IQ range 3–18). Except for 2 patients with previous thyrotoxicosis, the remaining were either on amiodarone (n=6) or on sotalol (n=1) before BCSD. Main BCSD indications were represented by drug refractory fast VT in 7 pts (cycle Results No major complication occurred. One patient (NICMP, NYHA II), has an uneventful follow up (FU) of less than 1 month and was excluded from the efficacy analysis. The median FU in the remaining 8 patients is 10 months (IQ range 6–19), during which the median number of shocks/patients was 0.5 (IQ range 0–3). Overall, 4 patients (50%) had ICD shock recurrences. Two cases (mean LVEF 17.5%, NYHA class III) had an ES during severe hemodynamic instability and subsequently died because of cardiogenic shock respectively 1 and 7 months after BCSD. One case had three, not consecutive ICD shocks 20 months after BCSD in the setting of severe amiodarone-induced thyrotoxicosis. Finally, one patient received a single intra-hospital ICD shock 5 days after BCSD before reintroduction of full-dose beta-blockers. The figure summarizes ICD shocks burden in the 6 months before and after BCSD. Among the 5 patients with NICMP/ARVC (4 in NYHA class I), only 1 had a single ICD shock recurrence. ICD shocks pre versus post BCSD, n=8 Conclusions Our case series, although numerically small, has a good follow-up and is the first reported in Europe. The results are in agreement with the suggested remarkable efficacy of BCSD in patients with good functional capacity and fast VAs. Therefore, cardiac sympathetic denervation should always be considered in patients with SHD and refractory ventricular tachyarrhythmias, especially in case VT ablation is either not indicated or fails.

Heart transplantation is a life-saving therapy for some patients admitted for acute myocarditis. However, controversial exists about the major risk of rejection following heart transplantation in specific types of myocarditis. Because of relatively few data on the post heart transplant outcomes, we report the long-term follow-up of a 39-year-old patient with a previous history of ulcerative colitis, which rapidly worsened heart failure until an emergency heart transplant in 2004.The clinical course was complicated by many episodes of rejection; lastly, after the development of severe cardiac allograft vasculopathy, re-heart transplantation was needed. The main findings of this case are: 1) inflammatory aetiology should always be suspected in patients with concomitant autoimmune disease that developing rapidly progressing heart failure; 2) patients with inflammatory myocardial disease undergoing heart transplantation should also undergo strict immunological surveillance; 3) the option of performing the re-heart transplant in a patient with a so complex management in the first one could be uncertain, but in this case the young age and lack of noncardiac comorbidities were effective to favour the survivor after two immunologically so challenging heart transplantation.

Danon disease is characterized by hypertrophic cardiomyopathy, skeletal myopathy, and intellectual disability due to deficiency of the lysosome-associated membrane protein-2 (LAMP-2). Although heart transplantation is considered an option for end stage Danon cardiomyopathy, scarce information is available about long term follow up. We report on long term follow up (14.7 years, IQ range 9–21 years) of 4 patients, transplanted for Danon disease cardiomyopathy, showing two LAMP-2 gene variants, the novel c.815T > C and the previously reported c.294G > A. We have also analysed previous published paper on this topic comparing available data from different follow up. Being a skeletal and cardiac muscle disease, with systemic effects, long term results about HTx are indispensable to justify any treatments in this subset of patients.

To identify differences in clinical epidemiology, in-hospital management and 1-year outcomes among patients hospitalized for acute heart failure (AHF) and enrolled in the European Society of Cardiology Heart Failure Long-Term (ESC-HF-LT) Registry, stratified by clinical profile at admission.The ESC-HF-LT Registry is a prospective, observational study collecting hospitalization and 1-year follow-up data from 6629 AHF patients. Among AHF patients enrolled in the registry, 13.2% presented with pulmonary oedema (PO), 2.9% with cardiogenic shock (CS), 61.1% with decompensated heart failure (DHF), 4.8% with hypertensive heart failure (HT-HF), 3.5% with right heart failure (RHF) and 14.4% with AHF and associated acute coronary syndromes (ACS-HF). The 1-year mortality rate was 28.1% in PO, 54.0% in CS, 27.2% in DHF, 12.8% in HT-HF, 34.0% in RHF and 20.6% in ACS-HF patients. When patients were classified by systolic blood pressure (SBP) at initial presentation, 1-year mortality was 34.8% in patients with SBP85 mmHg, 29.0% in those with SBP 85-110 mmHg, 21.2% in patients with SBP 110-140 mmHg and 17.4% in those with SBP140 mmHg. These differences tended to diminish in the months post-discharge, and 1-year mortality for the patients who survived at least 6 months post-discharge did not vary significantly by either clinical profile or SBP classification.Rates of adverse outcomes in AHF remain high, and substantial differences have been found when patients were stratified by clinical profile or SBP. However, patients who survived at least 6 months post-discharge represent a more homogeneous group and their 1-year outcome is less influenced by clinical profile or SBP at admission.

Cardiovascular involvement is common in acromegaly and can lead to development of acromegalic cardiomyopathy, characterized by concentric biventricular hypertrophy with a progressive impairment of diastolic and systolic function. The onset of heart failure and arrhythmias are related to poor prognosis. We report on a case of a 48-year-old man with acromegalic cardiomyopathy caused by pituitary adenoma. Despite the successful trans-sphenoidal resection of the tumour, the patient was rehospitalized for ventricular arrhythmic storms that led to cardiogenic shock, which required mechanical hemodynamic support with intra-aortic balloon pump, venoarterial extracorporeal membrane oxygenation, and urgent heart transplantation.

Heart failure is the leading cause of hospitalization among the elderly. This study compares clinical characteristics, management, and prognosis of octogenarians (OLD) with younger (YOUNG) patients in the Italian Survey on Acute Heart Failure (AHF).A nationwide, prospective, observational study on AHF was done. Two hundred six Italian departments with intensive cardiac care units enrolled 2,807 patients in 3 months.Octogenarians (mean age 84 +/- 4 years) represented 28% of enrollees. Females were 50% in the OLD group versus 36% in the YOUNG group (P.0001). Risk factors such as obesity, diabetes, and smoking were more frequent in the YOUNG group. Comorbidities such as anemia and renal dysfunction were more common in the OLD group (64% vs 53%, P.0001, and 56% vs 43%, P.0001). More octogenarians were admitted with cardiogenic shock and pulmonary edema, whereas younger patients presented more frequently in New York Heart Association class III to IV (P = .002). Left ventricular ejection fraction was measured in 90% of octogenarians versus 93% of the younger ones and was preserved in 41% of the OLD group versus 31% of the YOUNG group (P.0001). Coronary angiography was performed in 20% of the YOUNG group and 10% of the OLD group. In-hospital mortality was twice as high in the OLD group (11.8% vs 5.6%, P.001). In multivariable analysis, the strongest predictors of this event were use of inotropic agents, advanced age (or =80 years), and elevated troponin at admission.Octogenarians represent more than one fourth of the admissions for AHF and have a more severe clinical presentation. Their management is less aggressive, and treatments recommended by guidelines are underused. In-hospital mortality is high in the OLD group independently of left ventricular ejection fraction.

cause of sudden cardiac death. The 5–10% rate of outof-hospital cardiac arrest is ascribed to VF in absence of organic heart disease [5, 12]. The trigger role of the ventricular premature beats (VPBs) arising from the distal Purkinje system has been identified by recent studies, which demonstrated the feasibility of eliminating or decreasing the incidence of idiopathic VF by catheter ablation of this trigger [3, 7–9, 15, 17]. However, multiple conditions like electrolyte imbalance, catecholamines, fever or drug use may facilitate induction of idiopathic VF [8, 11, 13, 14]. We report the case of a healthy woman who presented with a dramatic storm of more than 50 nonsustained and sustained VF episodes in the absence of a detectable underlying heart disease but with the presence of left bundle branch block. The morphology of initiating VBPs suggests the role of specific triggers arising from the distal Purkinje system. The concomitant presence of mild hypocalcemia without evident causes could have a possible and previously not described role in generating recurrent VF episodes.

OBJECTIVE: Heart failure (HF) is a major health problem resulting in a high financial burden for the healthcare system. Many previous HF management programmes reduced adverse clinical outcomes and costs, but they usually involved several professional figures as well as huge investments, requiring resources and budgets not often available in our healthcare system. We evaluated the effects of our HF management programme, which included patient education and regular outpatient contact with the HF team, on re-hospitalisation and death, optimising the few resources already available at our hospital. METHODS: Two hundred consecutive patients admitted to the internal medicine department with a diagnosis of HF were randomised to the intervention group (nurse-led education programme, facilitated telephone communication and follow-up visits with an internist at 15 days, 1 and 6 months) or to the usual care group (follow-up by their primary care physician). The primary endpoints were all-cause readmissions and all-cause deaths during the 6-month post-discharge period. RESULTS: There were 81 all-cause hospital readmissions in the intervention group and 82 in the control group (P = NS). Fourteen patients (14%) in the intervention group and eight patients (8%) in the control group died during the study period (P = NS). Unplanned outpatient visits were less frequent in the intervention group than in the control group (39 [28%] versus 99 [72%], P < 0.001). CONCLUSIONS: The present low-cost HF management programme reduced unplanned outpatient visits but proved ineffective in reducing subsequent readmissions and in improving clinical status. More intense follow-up monitoring and more resources are needed to achieve better results.

Patient-centered treatment outcomes such as health-related quality of life are recommended in clinical care and research studies. Health-related quality of life questionnaires need to be validated in the language of the target population. The reliability and validity of the Italian version of the MacNew Questionnaire was determined in patients with angina, myocardial infarction, or ischemic heart failure. Sociodemographic and clinical data were collected on 298 patients [angina, n = 88; MI, n = 106; heart failure, n = 104; mean age, 64.8 (±10.6) years] at three centers in Italy. MacNew mean scores were higher (p

Idiopathic left ventricular aneurysm (LVA) is a very rare clinical condition. This article describes a patient with idiopathic LVA associated with episodes of ventricular tachycardia and ventricular fibrillation. Clinical and instrumental examinations did not reveal the pathogenesis of the aneurysm. The malignant clinical course suggests that an aggressive antiarrhythmic treatment, including ICD implantation, may be warranted.

Background: Acute myocarditis (AM) may occasionally have an infarct-like presentation. The aim of the present study was to investigate the relation between electrocardiographic (ECG) findings in this group of patients and myocardial damage assessed by cardiac magnetic resonance imaging (MRI) with the late gadolinium enhancement (LGE) technique. Hypothesis: Myocardial damage may be associated with ECG changes in infarct-like AM. Methods: Forty-one consecutive patients (36 males; mean age, 36 ± 12 years) with diagnosis of AM according to cardiac MRI Lake Louise criteria and infarct-like presentation were included. The relation between site of ST-segment elevation (STE), sum of STE (sumSTE), time to normalization of STE, and development of negative T wave with the extent of LGE (expressed as % of left ventricular mass [%LV LGE]), was evaluated. Results: Most (80%) patients presented with inferolateral STE; mean sumSTE was 5 ± 3 mm. Normalization of STE occurred within 24 hours in 20 (49%) patients. Development of negative T wave occurred in 28 (68%) patients. Cardiac MRI showed LGE in all patients; mean %LV LGE was 9.6 ± 7.2%. Topographic agreement between site of STE and LGE was 68%. At multivariate analysis, sumSTE (β = 0.42, P 24 hours (β = 0.39, P < 0.001), and development of negative T wave (β = 0.49, P < 0.001) were independently related to %LV LGE. Conclusions: Analysis of the site of STE underestimates the extent of myocardial injury among patients with infarct-like myocarditis. However, some ECG features (ie, sumSTE, normalization of STE >24 hours, and development of negative T wave) may help to identify patients with larger areas of myocardial damage. The authors have no funding, financial relationships, or conflicts of interest to disclose.

A 43-year-old woman with a familial history of Danon disease and early sudden death was clinically followed because of carrier of LAMP-2 mutation. She was symptomatic for palpitations. Clinical evaluation and electrocardiogram were normal ( Panel A ), while Holter monitoring revealed non-sustained ventricular tachycardia (VT; Panel B ). Conventional two- and three-dimensional echocardiograms were unremarkable (see Supplementary …

Danon disease is a rare X-linked dominant metabolic disorder caused by a primary deficiency in lysosome-associated membrane protein 2. It is characterized by the development of cardiac disease, skeletal myopathy and cognitive disorder. Due to the rarity of Danon disease, physicians may be unfamiliar with the phenotype, confusing it with hypertrophic cardiomyopathy or other causes of left ventricular hypertrophy. The present report demonstrates the clinical value of cardiac magnetic resonance imaging for the diagnostic work-up of Danon disease.

[Figure][1] [![Graphic][3] ][3][![Graphic][4] ][4][![Graphic][5] ][5] A 48-year-old woman with schizophrenia was referred to our department because of chest pain while driving, electrocardiographic changes, and increased cardiac troponin I. A routine chest X-ray showed 6

Patients affected by heart failure have a compromised quality of life (QOL) and in the last few years “health related quality of life” has become an important outcome indicator for the evaluation of heart failure treatment. Methods: Translation into Italian of the Left Ventricular Dysfunction Questionnaire (LVD-36), a new, 36-item, disease-specific health status instrument for patients with congestive heart failure, and its subsequent validation by administration to 50 consecutive patients in our heart failure outpatient clinic. The Italian LVD-36 was compared to the “The Minnesota Living with Heart Failure Questionnaire” (MLHF). Results: The Italian version of the LVD-36 correlates well with MLHF for ejection fraction (EF), NYHA class I and II, etiology and therapy. Since, however, the LVD-36 has only one domain, it may be able to offer more synthetic information than MLHF about patients’ status. Conclusions: The Italian version of the LVD-36 appears to be a reliable instrument for assessing patients’ QOL and the degree of limitations imposed on them by the disease. It is short, clear and easy to complete. In patients with heart failure the LVD-36 correlates well with the MLHF and may be considered a new disease- specific instrument to estimate changes in health status, and an useful support in optimizing therapeutic options.

Prevalence of isolated left ventricular (LV) diastolic dysfunction has been reported to be as high as one-third of all heart failure (HF) cases, with an increasing prevalence in the elderly population. However, there is a paucity of prospective data about the prevalence and prognosis of isolated LV diastolic dysfunction in an unselected population of patients hospitalized with HF. Therefore, we prospectively evaluated 179 consecutive patients discharged from our hospital with HF to assess the prevalence of systolic versus diastolic LV dysfunction among patients hospitalized with HF and to compare their demographics, clinical features, self-perceived quality of life (QOL), and 6-month readmission rate and mortality. Among them, 133 (59% men, median age 74 years) showed in sinus rhythm and had no significant primary valvular disease. LV diastolic dysfunction was diagnosed on the basis of the European Study Group on Diastolic HF echocardiographic criteria. QOL was assessed at hospital discharge and 6-month follow-up visit using the Minnesota Living with HF questionnaire. Survival of patients with HF was compared with that of age- and sex-matched general population. In all, 29 patients (22%) had isolated LV diastolic dysfunction and 102 (78%) had prevalent LV systolic dysfunction (ie, LV ejection fraction

The present case report refers to a 65-year-old male patient with subocclusion of the right coronary artery who had an inferior myocardial infarction that was treated with coronary angioplasty. The patient subsequently developed intractable angina pectoris in the absence of critical coronary stenosis at serial coronary angiography. Doppler wire velocity demonstrated an impaired coronary flow reserve. The patient died of cardiogenic shock. Postmortem examination revealed amyloid involvement of the media of the epicardial coronary arteries and severe amyloid deposition in the media and adventitia with obstruction of the lumen of the intramyocardial coronary arteries. Widespread ischemic areas were present in the myocardium with only slight amyloid deposition. In this patient myocardial infarction and unstable angina were a rare initial manifestation of primary amyloidosis.

[1] Cooper A, Calvert N, SKinner J, et al. Nice guideline: chest pain of recent onset: assessment of recent onset chest pain or discomfort of suspected cardiac origin; 2010. available at:http://www.nice.org.uk/nicemedia/live/12947/47931/47931.pdf. [2] Bourdillon PJ. NICE and chest pain diagnosis. Exercise ECG useful in finding coronary artery disease. BMJ 2010;340:c1971. [3] Underwood SR. NICE and chest pain diagnosis. W(h)ither the exercise ECG? BMJ 2010;340:c2387. [4] Timmis A. NICE and chest pain diagnosis. NICE replies. BMJ 2010;340:c2391. [5] Nijjer SS, Eseonu KC, Baker CS. A survey on the latest NICE guidance for diagnosis of chest pain: a challenge to NICE. Int J Cardiol 2010;145:611–3. [6] Shewan LG, Coats AJ. Ethics in the authorship and publishing of scientific articles. Int J Cardiol 2010;144:1–2.

Aims To assess prospectively the value of cardiac magnetic resonance imaging in patients with apparently idiopathic premature contractions arising from the right ventricular outflow tract. Methods We compared magnetic resonance imaging scans in 19 patients (13 males and six females, mean age 44 years) with frequent (>100 per hour), monomorphic (left bundle branch block and inferior axis morphology) extrasystoles, and in 10 volunteers (four males and six females, mean age 36·7 years) without structural heart disease. Magnetic resonance imaging studies (1 or 1·5 Tesla) included spinecho and gradient-echo sequences in the standard planes. The presence of structural and dynamic abnormalities of the right and left ventricles, such as reduced wall thickness, systolic bulging, and decreased systolic thickening, were evaluated. In addition, end-diastolic diameters of the right ventricular outflow tract were measured in the transverse plane. Results The dimensions of the right ventricular outflow tract were wider in patients with extrasystoles compared to the control group. Mean anteroposterior and transverse diameters were 39·6±4·6 mm vs 29·9±4·8 mm ( P

Background: Atrial fibrillation (AF) and Heart Failure (HF) share many risk factors. CHA2DS and CHA2DS2Vasc scores predict stroke occurrence and mortality in AF patients. Aim of this study was to evaluate whether these scores can also predict mortality in different subsets of HF patients. Methods: ESC-HF Pilot survey was a prospective multicentre observational study run in 136 Cardiology Centres in 12 European Countries. From October 2009 to May 2010, 5118 patiens were included and followed up for one year. CHADS and CHADVASC scores were calculated for each patient. Patients were classified in three groups according to their CHADS score (Group A CHADS 1, Group B CHADS 2-3, Group C CHADS >3) and their CHADSVasc score (Group D CHADSVasc 1, Group E CHADSVasc 2-3, Group F CHADSVasc >3). All-cause 1-year mortality rate was calculated in three subsets of interest: Patients with or without AF, patients with preserved vs reduced left ventricular function, patients admitted for acute heart failure vs outpatients with chronic heart failure Results: Table shows one year all-cause mortality rate in these three subsets of patient split according to their CHADS and CHADVASC score, showing a clear relation between increasing scores and one year mortality. Multivariate anaysis showed that compared to Group A (CHADS score of 1), the odds of mortality were 1.78 (95% CI 1.3-2.4) in Group B (CHADS score 2-3) and 3.04 (95% CI 2.2-4.3) in Group C (CHADS score >3). Compared to Group D (CHADSVasc score 1), the odds of mortality were 2.83 (95% CI 1.5-5.5) in Group E (CHADSVasc score 2-3) and 5.00 (95% CI 2.6-9.5) in Group F (CHADSVasc score >3). View this table: Mortality by CHADS and CHADSVasc Score Conclusions: Both CHA2DS and CHA2DS2Vasc scores are independently associated with one year all-cause mortality in a wide variety of heart failure patients.