Cardiac Magnetic Resonance in Primary Prevention of Sudden Cardiac Death

Sudden death accounts for 400,000 deaths annually in the United States. Most sudden deaths are cardiac and are related to arrhythmias secondary to structural heart disease or primary electrical abnormalities of the heart. Implantable cardioverter defibrillator significantly improves survival in patients at increased risk of life-threatening arrhythmias, but better selection of eligible patients is required to avoid unnecessary implantation and identify those patients who may benefit most from this therapy. Left ventricular (LV) ejection fraction (EF) measured by echocardiography has been considered the most reliable parameter for long-term outcome in many cardiac diseases. However, LVEF is an inaccurate parameter for arrhythmic risk assessment as patients with normal or mildly reduced LV systolic function could experience sudden cardiac death (SCD). Among other tools for arrhythmic stratification, magnetic resonance (CMR) provides the most comprehensive cardiac evaluation including in vivo tissue characterization and significantly aids in the identification of patients at higher SCD risk. Most of the evidence are related to late gadolinium enhancement (LGE), which was proven to detect cardiac fibrosis. LGE has been reported to add incremental value for prognostic stratification and SCD prediction across a wide range of cardiac diseases, including both ischemic and nonischemic cardiomyopathies. In addition, T1, T2 mapping and extracellular volume assessment were reported to add incremental value for arrhythmic assessment despite suffering from several technical limitations. CMR should be part of a multiparametric approach for patients' evaluation, and it will play a pivotal role in prognostic stratification according to the current evidence

Cardiac hamartoma

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Prevalence and prognostic significance of ischemic late gadolinium enhancement pattern in non-ischemic dilated cardiomyopathy

Background Typical late gadolinium enhancement (LGE) patterns in dilated cardiomyopathy (DCM) include intramyocardial and subepicardial distribution. However, the ischemic pattern of LGE (subendocardial and transmural) has also been reported in DCM without coronar y arter y disease (CAD), but its correlates and prognostic significance are still not known. On these bases, this study sought to describe the prevalence and prognostic significance of the ischemic LGE pattern in DCM. Methods A total of 611 DCM patients with available cardiac magnetic resonance were retrospectively analyzed. A composite of all-cause-death, major ventricular arrhythmias (MVAs), heart transplantation (HTx) or ventricular assist device (VAD) implantation was the primary outcome of the study. Secondary outcomes were a composite of sudden cardiac death or MVAs and a composite of death for refractory heart failure, HTx or VAD implantation. Results Ischemic LGE was found in 7% of DCM patients without significant CAD or history of myocardial infarction, most commonly inferior/inferolateral/anterolateral. Compared to patients with non-ischemic LGE, those with ischemic LGE had higher prevalence of hypertension and atrial fibrillation or flutter. Ischemic LGE was associated with worse long-term outcomes compared to non-ischemic LGE (36% vs 23% risk of primary outcome events at 5 years respectively, P = .006), and remained an independent predictor of primary outcome after adjustment for clinically and statistically significant variables (adjusted hazard ratio 2.059 [1.055-4.015], P = .034 with respect to non-ischemic LGE). Conclusions The ischemic pattern of LGE is not uncommon among DCM patients without CAD and is independently associated with worse long-term outcomes. (Am Heart J 2022;246:117-124.

Prognostic Significance of Feature-Tracking Right Ventricular Global Longitudinal Strain in Non-ischemic Dilated Cardiomyopathy

Aims: Left ventricular global longitudinal strain (GLS) by cardiac magnetic resonance feature tracking (CMR-FT) analysis has shown an incremental prognostic value compared to classical parameters in non-ischemic dilated cardiomyopathy (NICM). However, less is known about the role of right ventricular (RV) GLS. Our objective was to evaluate the prognostic impact of RV-GLS by CMR-FT analysis in a population of NICM patients.Methods: In this multicenter study, we examined NICM patients evaluated with a comprehensive CMR-FT study. Major cardiac events (MACEs) were considered as the study primary outcome measure and were defined as a composite of (a) cardiovascular death, (b) cardiac transplant or destination therapy ventricular assist device, (c) hospitalization for life-threatening ventricular arrhythmias or implantable cardiac defibrillator appropriate intervention. Heart failure (HF) related events, including hospitalizations and life-threatening arrhythmia-related events were considered as secondary end-points. Receiver operating time-dependent analysis were used to calculate the possible additional effect of RV-GLS to standard evaluation.Results: We consecutively enrolled 273 patients. During a median follow-up of 39 months, 41 patients (15%) experienced MACEs. RV-GLS and LV late gadolinium emerged as the strongest prognostic CMR-FT variables: their association provided an estimated 3-year MACEs rate of 29%. The addition of RV-GLS significantly improved the prognostic accuracy in predicting MACEs with respect to the standard evaluation including LGE (areas under the curve from 0.71 [0.66-0.82] to 0.76 [0.66-0.86], p = 0.03). On competing risk analysis, RV-GLS showed a significant ability to reclassify overall both HF-related and life-threatening arrhythmia-related events, regardless of LV and RV ejection fraction.Conclusions: In NICM patients, RV-GLS showed a significant prognostic role in reclassifying the risk of MACEs, incremental with respect to standard evaluation with standard prognostic parameters

Il Registro Regionale delle Morti Cardiache Improvvise in età giovanile del Friuli Venezia Giulia. Protocolli operativi e risultati di un progetto multidisciplinare

With the regional law n. 26 of December 30, 2020, the Friuli Venezia Giulia Region wanted to promote the establishment of the Regional Register of Sudden Cardiac Death, with the aim of favoring the study of all those deaths that occurred suddenly and unexpectedly under the age of 50 years in which it is not possible to trace the cause of death with certainty. Such dramatic events, difficult to quantify considering the complexity of data collection, are often accepted with resignation without any further investigation of the possible causes. The Regional Register of Sudden Cardiac Deaths of Friuli Venezia Giulia was born from this premise and from the awareness of the importance of going back with a rigorous scientific methodology and through a multidisciplinary approach, to the diagnosis of hereditary heart diseases which, when determined, allow the enrollment of relatives in a cardiological screening process and, therefore, primary prevention of potentially fatal events. The authors describe the operating procedures feeding the Regional Register and present the results of the first year of activity on 26 cases

Global longitudinal strain by CMR improves prognostic stratification in acute myocarditis presenting with normal LVEF

none25siPrognostic stratification of acute myocarditis (AM) presenting with normal left ventricular ejection fraction (LVEF) relies mostly on late gadolinium enhancement (LGE) characterization. Left ventricular peak global longitudinal strain (LV-GLS) measured by feature tracking analysis might improve prognostication of AM presenting with normal LVEF.Porcari, Aldostefano; Merlo, Marco; Baggio, Chiara; Gagno, Giulia; Cittar, Marco; Barbati, Giulia; Paldino, Alessia; Castrichini, Matteo; Vitrella, Giancarlo; Pagnan, Lorenzo; Cannatà, Antonio; Andreis, Alessandro; Cecere, Annagrazia; Cipriani, Alberto; Raafs, Anne; Bromage, Daniel I; Rosmini, Stefania; Scott, Paul; Sado, Daniel; Di Bella, Gianluca; Nucifora, Gaetano; Marra, Martina Perazzolo; Heymans, Stephane; Imazio, Massimo; Sinagra, GianfrancoPorcari, Aldostefano; Merlo, Marco; Baggio, Chiara; Gagno, Giulia; Cittar, Marco; Barbati, Giulia; Paldino, Alessia; Castrichini, Matteo; Vitrella, Giancarlo; Pagnan, Lorenzo; Cannatà, Antonio; Andreis, Alessandro; Cecere, Annagrazia; Cipriani, Alberto; Raafs, Anne; Bromage, Daniel I; Rosmini, Stefania; Scott, Paul; Sado, Daniel; Di Bella, Gianluca; Nucifora, Gaetano; Marra, Martina Perazzolo; Heymans, Stephane; Imazio, Massimo; Sinagra, Gianfranc

Inquadramento e gestione delle cardiomiopatie: il protocollo della Cardiologia di Trieste

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\u2019 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