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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.

Nonischemic-dilated cardiomyopathy (NIDCM) is an entity that gathers extremely heterogeneous diseases. This awareness, although leading to continuous improvement in survival, has increased the complexity of NIDCM patients' management. Even though the endorsed 'red-flags' approach helps clinicians in pursuing an accurate etiological definition in clinical practice, it is not clear when and how peripheral centers should interact with referral centers with specific expertise in challenging scenarios (e.g. postmyocarditis and genetically determined dilated cardiomyopathy) and with easier access to second-line diagnostic tools and therapies. This position paper will summarize each step in NIDCM management, highlighting the multiple interactions between peripheral and referral centers, from first-line diagnostic workup and therapy to advanced heart failure management and long-term follow-up.

Cardiomyopathies are primary myocardial disorders, genetically determined, with clinical onset between the third and the fifth decade of life. They represent the main causes of sudden cardiac death and heart failure in the youth. The more common myocardial diseases in clinical practice are dilated cardiomyopathy, arrhythmogenic cardiomyopathy and hypertrophic cardiomyopathy. Next generation sequencing techniques, recently available for genetics researches, together with the diffusion of advanced imaging techniques, permitted in the last years a deeper knowledge of these pathologies. Nevertheless, diagnosis, etiology and several aspects of patients’ clinical management remain complex and controversial. This review paper aims to propose some operative flow-charts, derived from scientific evidences and the internal protocol of the Cardiothoracovascular Department of Trieste Hospital, Italian referral Center for cardiomyopathies and heart failure, with more than 30 years of experience in diagnosis and management of patients who suffer from primary myocardial disorders.

Background Pericardial effusion is frequent in the acute phase of ST-segment elevation myocardial infarction. However, its prognostic role in the era of primary percutaneous coronary intervention is not completely understood. Methods We investigated the association between pericardial effusion, assessed by transthoracic echocardiography, and survival in a large cohort of ST-segment elevation myocardial infarction patients treated with primary percutaneous coronary intervention, enrolled in the Trieste primary percutaneous coronary intervention registry from January 2007 to March 2017. Multivariable analysis and a propensity score approach were performed. Results A total of 1732 ST-segment elevation myocardial infarction patients were included. Median follow-up was 45 (interquartile range 19–79) months. Pericardial effusion was present in 246 patients (14.2%). Thirty-day all-cause mortality was similar between patients with and without pericardial effusion (7.8% vs. 5.4%, P=0.15), whereas crude long-term survival was worse in patients with pericardial effusion (26.2% vs. 17.7%, P≤0.01). However, at multivariable analyses the presence of pericardial effusion was not associated with long-term mortality (hazard ratio 1.26, 95% confidence interval 0.86–1.82, P=0.22). Matching based on propensity scores confirmed the lack of association between pericardial effusion and both 30-day (hazard ratio 1, 95% confidence interval 0.42–2.36, P=1) and long-term (hazard ratio 1.14, 95% confidence interval 0.74–1.78, P=0.53) all-cause mortality. Patients with per effusion experienced a higher incidence of free wall rupture (2.8% vs. 0.5%, P Conclusions In acute ST-segment elevation myocardial infarction treated with primary percutaneous coronary intervention, the onset of pericardial effusion after ST-segment elevation myocardial infarction is not independently associated with short and long-term higher mortality. Free wall rupture has to be considered rare compared to the fibrinolytic era and occurs more frequently in patients with pericardial effusion, suggesting a close monitoring of these patients in the early post-primary percutaneous coronary intervention phase.

Dilated cardiomyopathy (DCM) is a primary heart muscle disease characterized by a progressive dilation and dysfunction of either the left or both ventricles. The management of DCM is currently challenging for clinicians. The persistent lack of knowledge about the etiology and pathophysiology of this disease continues to determine important fields of uncertainty in managing this condition. Molecular cardiology and genetics currently represent the most crucial horizon of increasing knowledge. Understanding the mechanisms underlying the disease allows clinicians to treat this disease more effectively and to further improve outcomes of DCM patients through advancements in etiologic characterization, prognostic stratification and individualized therapy. Left ventricular reverse remodeling predicts a lower rate of major cardiac adverse events independently from other factors. Optimized medical treatment and device implantation are pivotal in inducing left ventricular reverse remodeling. Newly identified targets, such as angiotensin-neprilysin inhibition, phosphodiesterase inhibition and calcium sensitizing are important in improving prognosis in patients affected by DCM.

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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.