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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.

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.

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.

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.

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

[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.