Clinical management of dilated cardiomyopathy: Current knowledge and future perspectives

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

Pathogenesis

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Pulmonary artery aneurysm and sarcoidosis

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

Changing mortality in dilated cardiomyopathy

Objective—To analyse the changes in mortality in dilated cardiomyopathy over the past 15 years and to identify the factors that might have influenced survival. Design—Follow up study of 235 patients (aged 16-70) systematically enrolled on a register from 1 January 1978 to 31 December 1992. Setting—Hospital department of cardiology. Patients—Three groups corresponding to three periods of 5 years: group 1 (diagnosis between 1 January 1978 and 31 December 1982) 26 patients; group 2 (diagnosis between 1 January 1983 and 31 December 1987) 65 patients; and group 3 (diagnosis between 1 January 1988 and 31 December 1992) 144 patients. Main outcome measures—Death or heart transplantation. Results—Two and four year survival was 73·8% and 53·8% in group 1, 87·7% and 72·3% in group 2, and 90·3% and 82·9% in group 3 (P = 0·02). During the 15 years of the study period the number of cases increased progressively and the baseline clinical characteristics changed (that is, patients were younger and less severely affected), partly explaining the improvement in survival. None the less, the three mortality curves tended to diverge progressively and the improvement in survival in the different groups was still significant after stratification for the severity of the disease, suggesting that treatment had a sustained effect. A progressively higher proportion of patients were treated with angiotensin converting enzyme (ACE) inhibitors and more recently with β blockers. In group 2, after stratification for the severity of heart failure, patients who were treated with ACE inhibitors showed a better survival than patients who were not. Furthermore, analysis of group 3 showed that β blockers had a significant additive effect with conventional therapy both by intention to treat and actual treatment. Four year survival in patients with mild and moderate to severe heart failure treated with β blockers, and usually digitalis and ACE inhibitors, was respectively 90% and 87·5%. Conclusions—The improvement in the survival of patients with dilated cardiomyopathy over the past 15 years may be explained by earlier diagnosis, new treatments, and a change in the clinical characteristics of the patients at enrolment

Prognostic relevance of pericardial effusion in STEMI patients treated by primary percutaneous coronary intervention: a 10-year single-centre experience

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 pericardial effusion experienced a higher incidence of free wall rupture (2.8% vs. 0.5%, P<0.0001) independently of the entity of pericardial effusion. 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