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Dilated cardiomyopathy is a primary disease of the heart muscle, which affects relatively young patients with a low comorbidity profile. It is characterized by structural and/or functional abnormalities leading to systolic dysfunction of the left ventricle or of both ventricles, often associated with dilatation, in the absence of an ischaemic, valvular, or pressure overload cause sufficient to explain the phenotype. Although the prognosis of the disease has greatly improved over the last few decades, prognostic stratification remains a fundamental objective, especially about the prediction of potentially life-threatening arrhythmic events. An accurate diagnostic work-up and an appropriate aetiopathogenetic characterization affect the patients’ outcome and represent the essential basis of an adequate prognostic stratification. It is necessary to adopt a multiparametric approach, especially when the aim is the prediction of arrhythmic risk; it includes an integration of medical history and physical examination with cardiac imaging and genetic testing, in order to obtain a personalized diagnosis and therapeutic strategies. Furthermore, the evaluation should be repeated at every clinical check-up, considering the dynamic trend of the pathology and the arrhythmic risk changes over time. This article aims to illustrate how, starting from an exhaustive aetiological and clinical–instrumental characterization, including all diagnostic methods available at present time, it is possible to obtain a tailored diagnostic evaluation and stratification of the arrhythmic risk as accurate as possible.

A 27-year-old woman, without previous cardiological history, was admitted at our emergency department in the clinical context of an acute pericarditis. Echocardiography documented mild pericardial effusion (8 mm) without any sign of hemodynamic impact. The patient was then admitted at our Cardiology Unit. During the hospitalization a complete transthoracic echocardiogram showed a normal biventricular morphology and function, normal valvular function and found an anechoic structure on the postero-lateral left atrial wall (1.75×1.5 cm). To better characterize this accidental finding, a transesophageal echocardiography was performed unmasking a dilated coronary sinus with anomalous course. A subsequent cardiac computed tomography was performed showing anomalous systemic venous return with the persistency of the left superior vena cava draining into the coronary sinus. No other congenital anomalies were found. PLSVC is present in approximatively 0.5% of individuals in the general population and is the most frequent congenital malformation of the thoracic venous return. In about 90% coexists with the right superior vena cava and co-occur in almost 40% of patients with other congenital heart abnormalities such as atrial septal defect, aortic coarctation, bicuspid aortic valve, tetralogy of Fallot, transposition of the great vessels, anomalous connections of the pulmonary veins, dextroversion and cor triatatum. Patients usually are asymptomatic. Nevertheless, this congenital malformation may cause chest discomfort, cardiac arrhythmias, thromboembolic events, decreased exercise tolerance and syncope. Moreover, the correct identification of this condition has important clinical implications. In fact, it is a relative contraindication to the administration of retrograde cardioplegia during cardiac surgery. Furthermore, the placement of central venous-access line, pulmonary artery catheterization or the implantation of pacemaker/resynchronization leads could be difficult or can result in incorrect positioning. In these cases, it is recommended to access to the right heart through the right subclavian vein instead the left one. Legend: *: Anomalous course of coronary sinus; LA: Left Atrium; LV: Left Ventricle; Ao: Aorta; RA: Right Atrium; R-SVC: Right superior vena cava; L-SVC: Left superior vena cava; LSPV: Left superior pulmonary vein.

A 41-year-old man, without previous cardiological history, presented at the emergency department with hypotension and multi-organ failure. Laboratory testing showed elevated white cell blood count (16780/mcL; normal value 4000-11000/mcL; 90% neutrophils), high-sensibility troponin I (23247 ng/L; normal value After initial hemodynamic stabilization with endovenous inotropes, diuretics, and mechanical circulatory support (intra-aortic balloon pump), coronary angiography was performed showing normal coronary arteries. Since fulminant myocarditis was suspected and magnetic resonance was not feasible due to the patient's instability, an endomyocardial biopsy (EMB) was performed with the evidence of myocardial edema, initial myocardial fibrosis, and the concomitant presence of lymphocytic and eosinophilic endo-myocarditis (despite normal eosinophilic blood count) without evidence of active viral replication. After careful exclusion of latent or active infections, high doses of corticosteroids and azathioprine were initiated, with rapid laboratoristic and clinical response. EKG showed AV block regression and QRS width's normalization, so the pacemaker was removed. LVEF rapidly improved and was completely normalized at discharge after two weeks. In the early follow-up, a cardiac magnetic resonance (CMR) was performed with evidence of persistency of mildly dilated LV, normalization of LV wall thickness and biventricular function, a diffuse increase of T1 relaxation time, increased T2 signal of the anterior and septal walls, and intramural anteroseptal late gadolinium enhancement (LGE). Fulminant myocarditis is a rare presentation of acute myocarditis, with low incidence but high mortality. The inflammation of the myocardium itself leads to acute heart failure, cardiogenic shock, and cardiac arrhythmias, including sinus arrest, AV block, ventricular tachycardia, and ventricular fibrillation during the acute phase. In our case, AV block could be an expression of anteroseptal involvement, documented at CMR. This localization has already been associated with worse clinical presentation and could be a marker of poor prognosis. Diagnosis and treatment of fulminant myocarditis require EMB, to obtain information about the etiology and active viral replication in the heart tissue. In our case, the florid inflammatory state and the absence of viral replication allowed prompt treatment with immunosuppressors, with a quick and full recovery of biventricular function in only two weeks.

Background Chemotherapy-induced dilated cardiomyopathy (CI-DCM) is a well-recognized phenotype of non-ischemic dilated cardiomyopathy (DCM), characterized by poor outcomes. However, a detailed comparison between idiopathic DCM (iDCM) and CI-DCM is still lacking. Methods All consecutive DCM patients enrolled in the Trieste Muscle Heart Disease Registry were analyzed. CI-DCM and iDCM were defined according to current recommendations. The primary study outcome measure was all-mortality death and secondary outcomes were a) a composite of cardiovascular death/heart-transplantation/ventricular-assist-device implantation, and b) major ventricular arrhythmias. Results The study included 551 patients (499 iDCM and 52 CI-DCM). At enrolment, compared to iDCM, CI-DCM patients were older (51±14 years vs 58±3 years respectively, p Conclusions In a well-selected DCM cohort, patients with a chemotherapy-induced aetiology had a higher incidence of all-cause mortality compared to iDCM. Conversely, the incidence of life-threatening ventricular arrhythmic events was higher among patients with iDCM. Funding Acknowledgement Type of funding sources: None.

Chemotherapy can lead to chemotherapy-induced dilated cardiomyopathy (CI-DCM), recognized as one of the Non-ischaemic Dilated Cardiomyopathy (DCM) phenotypes characterized by worse outcome. Evidences on a direct comparison between idiopathic-DCM (iDCM) and CI-DCM still lack. We included all the consecutive patients enrolled in the Trieste Muscle Heart Disease Registry. C-DCM was defined according to current recommendations. Uni- and multivariable analysis and Kaplan-Meier were performed. The primary outcome was all-cause death and the secondary outcomes were cardiac death and a composite of heart failure hospitalization, heart transplantation, ventricular assist-device implantation and major ventricular arrhythmias. The study included 511 patients (499 patients affected by iDCM and 52 patients affected by CI-DCM). Compared to iDCM, CI-DCM patients were older (51 ± 14 years vs. 58 ± 3 years respectively, P

A case of abdominal mass strongly suspected at examination of being a primary ovarian tumour with abnormal echographic features is reported. At laparotomy, it proved to be a uterine fibroleiomyoma of extraordinary dimensions.