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Immune-checkpoint inhibitor-associated myocarditis (ICI-myocarditis) often presents with arrhythmias, but the prognostic value of early electrocardiogram findings is unclear. Although ICI-myocarditis and acute cellular rejection (ACR) following cardiac transplantation use similar treatment strategies, differences in arrhythmia burden are unknown.To evaluate the association of electrocardiogram findings in ICI-myocarditis with myocarditis-related mortality and life-threatening arrhythmia.A total of 125 cases of ICI-myocarditis were identified retrospectively across 49 hospitals worldwide; 50 cases of grade 2R or 3R ACR were included as comparators. Two cardiologists blinded to clinical data interpreted electrocardiograms. Associations between electrocardiogram features, myocarditis-related mortality and the composite of myocarditis-related mortality and life-threatening arrhythmias were examined. Adjusted hazard ratios (aHRs) were calculated.The cohort had 78 (62.4%) men; median (interquartile range) age was 67 (58-76) years. At 30 days, myocarditis-related mortality was 20/124 (16.1%), and 28/124 (22.6%) met the composite endpoint. Patients who developed complete heart block (aHR by subdistribution hazards model [aHR(sh)] 3.29, 95% confidence interval [CI] 1.24-8.68; P=0.02) or life-threatening cardiac arrhythmias (aHR(sh) 6.82, 95% CI: 2.87-16.21; P0.001) had a higher risk of myocarditis-related mortality. Pathological Q waves (aHR(sh) 3.40, 95% CI: 1.38-8.33; P=0.008), low QRS voltage (aHR(sh) 6.05, 95% CI: 2.10-17.39; P0.001) and Sokolow-Lyon index (aHR(sh)/mV 0.54, 95% CI: 0.30-0.97; P=0.04) on admission electrocardiogram were also associated with increased risk of myocarditis-related mortality. These associations were mirrored in the composite outcome analysis. Compared with ACR, ICI-myocarditis had a higher incidence of life-threatening cardiac arrhythmias (15/125 [12.0%] vs 1/50 [2%]; P=0.04) and third-degree heart block (19/125 [15.2%] vs 0/50 [0%]; P=0.004).Electrocardiograms in ICI-myocarditis with ventricular tachycardias, heart block, low-voltage and pathological Q waves were associated with myocarditis-related mortality and life-threating arrhythmia. Arrhythmia burden in ICI-myocarditis exceeds that of ACR after heart transplant.

International audience; Background: Structural changes and myocardial fibrosis quantification by cardiac imaging have become increasingly important to predict cardiovascular events in patients with mitral valve prolapse (MVP). In this setting, it is likely that an unsupervised approach using machine learning may improve their risk assessment.Objectives: This study used machine learning to improve the risk assessment of patients with MVP by identifying echocardiographic phenotypes and their respective association with myocardial fibrosis and prognosis.Methods: Clusters were constructed using echocardiographic variables in a bicentric cohort of patients with MVP (n = 429 patients, 54 ± 15 years) and subsequently investigated for their association with myocardial fibrosis (assessed by cardiac magnetic resonance) and cardiovascular outcomes.Results: Mitral regurgitation (MR) was severe in 195 (45%) patients. Four clusters were identified: cluster 1 comprised no remodeling with mainly mild MR, cluster 2 was a transitional cluster, cluster 3 included significant left ventricular (LV) and left atrial (LA) remodeling with severe MR, and cluster 4 included remodeling with a drop in LV systolic strain. Clusters 3 and 4 featured more myocardial fibrosis than clusters 1 and 2 (P < 0.0001) and were associated with higher rates of cardiovascular events. Cluster analysis significantly improved diagnostic accuracy over conventional analysis. The decision tree identified the severity of MR along with LV systolic strain 42 mL/m2 as the 3 most relevant variables to correctly classify participants into 1 of the echocardiographic profiles.Conclusions: Clustering enabled the identification of 4 clusters with distinct echocardiographic LV and LA remodeling profiles associated with myocardial fibrosis and clinical outcomes. Our findings suggest that a simple algorithm based on only 3 key variables (severity of MR, LV systolic strain, and indexed LA volume) may help risk stratification and decision making in patients with MVP. (Genetic and Phenotypic Characteristics of Mitral Valve Prolapse; NCT03884426 and Myocardial Characterization of Arrhythmogenic Mitral Valve Prolapse [MVP STAMP]; NCT02879825).

Background Acute myocarditis (AM) is thought to be a rare cardiovascular complication of COVID-19, although minimal data are available beyond case reports. We aim to report the prevalence, baseline characteristics, in-hospital management, and outcomes for patients with COVID-19–associated AM on the basis of a retrospective cohort from 23 hospitals in the United States and Europe. Methods A total of 112 patients with suspected AM from 56963 hospitalized patients with COVID-19 were evaluated between February 1, 2020, and April 30, 2021. Inclusion criteria were hospitalization for COVID-19 and a diagnosis of AM on the basis of endomyocardial biopsy or increased troponin level plus typical signs of AM on cardiac magnetic resonance imaging. We identified 97 patients with possible AM, and among them, 54 patients with definite/probable AM supported by endomyocardial biopsy in 17 (31.5%) patients or magnetic resonance imaging in 50 (92.6%). We analyzed patient characteristics, treatments, and outcomes among all COVID-19–associated AM. Results AM prevalence among hospitalized patients with COVID-19 was 2.4 per 1000 hospitalizations considering definite/probable and 4.1 per 1000 considering also possible AM. The median age of definite/probable cases was 38 years, and 38.9% were female. On admission, chest pain and dyspnea were the most frequent symptoms (55.5% and 53.7%, respectively). Thirty-one cases (57.4%) occurred in the absence of COVID-19–associated pneumonia. Twenty- one (38.9%) had a fulminant presentation requiring inotropic support or temporary mechanical circulatory support. The composite of in-hospital mortality or temporary mechanical circulatory support occurred in 20.4%. At 120 days, estimated mortality was 6.6%, 15.1% in patients with associated pneumonia versus 0% in patients without pneumonia (P=0.044). During hospitalization, left ventricular ejection fraction, assessed by echocardiography, improved from a median of 40% on admission to 55% at discharge (n=47; P Conclusions AM occurrence is estimated between 2.4 and 4.1 out of 1000 patients hospitalized for COVID-19. The majority of AM occurs in the absence of pneumonia and is often complicated by hemodynamic instability. AM is a rare complication in patients hospitalized for COVID-19, with an outcome that differs on the basis of the presence of concomitant pneumonia.

Background MessengerRNA (mRNA) COVID-19 vaccination has been associated with a higher-than-expected occurrence of acute myocarditis. Scarce information is available on mid-term prognosis and changes in cardiac function, volumes, and tissue characterization on cardiac magnetic resonance (CMR). Methods Retrospective, multicenter study including patients with a definite diagnosis of acute myocarditis within 30 days from mRNA COVID-19 vaccination, with a confirmed myocarditis diagnosis based on endomyocardial biopsy (EMB) or autopsy or by the coexistence of positive biomarkers (troponin >99th upper reference limit or elevated creatine kinase myocardial band [CK-MB]) and cardiac MRI findings consistent with AM according to the 2018 updated Lake Louise Criteria. Results 77 patients (median age 25 years [IQR 20-35], 15% female) were included and followed-up for 147 days [IQR 74-215]. Follow-up CMR was available in n=49 patients and showed no changes in biventricular ejection fraction (EF) as compared to CMR at diagnosis (left ventricular EF: 59%[55-65]vs. 60%[57-64], p=0.507, right ventricular EF: 56%[52-62]vs. 57%[52-61], p=0.563, respectively). Late gadolinium enhancement was present in all patients at diagnosis and persisted in only n=39 (79.6%) at follow-up (p=0.001), generally sparing the anterior wall and the septum. N=10 (20.4%) had a persistent edema based on T2-weighted short tau inversion recovery (STIR) sequences, with predominant involvement of inferior or inferior-lateral walls. The proportion of patients with increased T1 and T2 mapping signals significantly decreased at follow-up (n=13 (68%) vs. n=4 (13%),p No differences in morpho-functional CMR parameters based on the type of vaccine administered were found (BNT162b2 Pfizer/BioNTech®, n=36, 73.5%, m-RNA-1273 Moderna®, n=13, 26.5%). Among patients with available follow-up (N=75, 97.4%), no major adverse cardiovascular events nor myocarditis recurrence or death were reported. Conclusions At mid-term follow-up, patients who experienced an acute myocarditis after a mRNA COVID-19 vaccine had preserved biventricular EF. The rate and localization of residual scar or edema on CMR is in line with classic viral myocarditis with a good prognosis. This new piece of information should further reassure patients who experience acute myocarditis after mRNA COVID-19 vaccination.

Objectives This study aimed to identify a subset of patients based on echocardiographic parameters who might have benefited from transcatheter correction using the Mitraclip system in the MITRA-FR (Percutaneous Repair with the MitraClip Device for Severe Functional/Secondary Mitral Regurgitation) trial. Background It has been suggested that differences in the degree of mitral regurgitation (MR) and left ventricular (LV) remodeling may explain the conflicting results between the MITRA-FR and the COAPT (Cardiovascular Outcomes Assessment of the MitraClip Percutaneous Therapy for Heart Failure Patients with Functional Mitral Regurgitation) trials. Methods In a post hoc analysis, we evaluated the interaction between the intervention and subsets of patients defined based on MR severity (effective regurgitant orifice [ERO], regurgitant volume [RVOL] and regurgitant fraction [RF]), LV remodeling (end-diastolic and end-systolic diameters and volumes) and combination of these parameters with respect to the composite of death from any cause or unplanned hospitalization for heart failure at 24 months. Results We observed a neutral impact of the intervention in subsets with the highest MR degree (ERO ≥30 mm2, RVOL ≥45 ml or RF ≥50%) as in patients with milder MR degree. The same was seen in subsets with the milder LV remodeling using either diastolic or systolic diameters or volumes. When parameters of MR severity and LV remodeling were combined, there was still no benefit of the intervention including in the subset of patients with an ERO/end-diastolic volume ratio ≥ 0.15 despite similar ERO and LV end-diastolic volume compared with COAPT patients. Conclusions In the MITRA-FR trial, we could not identify a subset of patients defined based on the degree of the regurgitation, LV remodeling or on their combination, including those deemed as having disproportionate MR, that might have benefited from transcatheter correction using the Mitraclip system. (Multicentre Study of Percutaneous Mitral Valve Repair MitraClip Device in Patients With Severe Secondary Mitral Regurgitation [MITRA-FR]; NCT01920698).

International audience; Purpose: Immune checkpoint blocker (ICB) associated myocarditis (ICB-myocar-ditis) may present similarly and/or overlap with other cardiac pathology including acute cor-onary syndrome presenting a challenge for prompt clinical diagnosis.Methods: An international registry was used to retrospectively identify cases of ICB-myocarditis. Presence of coronary artery disease (CAD) was defined as coronary artery steno-sis >70% in patients undergoing coronary angiogram.Results: Among 261 patients with clinically suspected ICB-myocarditis who underwent a coro-nary angiography, CAD was present in 59/261 patients (22.6%). Coronary revascularization was performed during the index hospitalisation in 19/59 (32.2%) patients. Patients undergoing coro-nary revascularization less frequently received steroids administration within 24 h of admission compared to the other groups (p = 0.029). Myocarditis-related 90-day mortality was 9/17 (52.7%) in the revascularised cohort, compared to 5/31 (16.1%) in those not revascularized and 25/156 (16.0%) in those without CAD (p = 0.001). Immune-related adverse event-related 90-day mortality was 9/17 (52.7%) in the revascularized cohort, compared to 6/31 (19.4%) in those not revascularized and 31/156 (19.9%) in no CAD groups (p = 0.007). All-cause 90-day mortality was 11/17 (64.7%) in the revascularized cohort, compared to 13/31 (41.9%) in no revas-cularization and 60/158 (38.0%) in no CAD groups (p = 0.10). After adjustment of age and sex, coronary revascularization remained associated with ICB-myocarditis-related death at 90 days (hazard ratio [HR] = 4.03, 95% confidence interval [CI] 1.84-8.84, p < 0.001) and was margin-ally associated with all-cause death (HR = 1.88, 95% CI, 0.98-3.61, p = 0.057).Conclusion: CAD may exist concomitantly with ICB-myocarditis and may portend a poorer outcome when revascularization is performed. This is potentially mediated through delayed diag-nosis and treatment or more severe presentation of ICB-myocarditis.

Inflammatory cardiomyopathies, also known as "myocarditis" are inflammatory pathologies affecting the myocardium and characterized by vast etiological and clinical heterogeneity. They can be asymptomatic, particularly in viral forms, or be responsible for sudden death, particularly in subjects under 35 years olds. Due to insufficient sensitivity and specificity of imaging and biology, the gold standard is histopathological and is performed on an endomyocardial biopsy or on explanted heart samples in a transplant context. Their classification has considerably evolved and is now based on the identification of a predominant cell pattern such as lymphocytic, neutrophilic or eosinophilic polynuclear, giant cell or granulomatous myocarditis. These different patterns will guide the etiological diagnosis, prognosis and the therapies to be implemented. Due to the importance of viral etiologies, this morphological analysis must be complemented by a virological analysis based on PCR with viral load quantification. In addition, some authors have been able to demonstrate the occurrence of myocarditis in patients with arrhythmogenic cardiomyopathy of genetic origin. The aim of this chapter is to review the current state of knowledge on inflammatory cardiomyopathies and their management.

Background: Acute myocarditis (AM) is thought to be a rare cardiovascular complication of COVID-19, although minimal data are available beyond case reports. We aim to report the prevalence, baseline characteristics, in-hospital management, and outcomes for patients with COVID-19–associated AM on the basis of a retrospective cohort from 23 hospitals in the United States and Europe. Methods: A total of 112 patients with suspected AM from 56 963 hospitalized patients with COVID-19 were evaluated between February 1, 2020, and April 30, 2021. Inclusion criteria were hospitalization for COVID-19 and a diagnosis of AM on the basis of endomyocardial biopsy or increased troponin level plus typical signs of AM on cardiac magnetic resonance imaging. We identified 97 patients with possible AM, and among them, 54 patients with definite/probable AM supported by endomyocardial biopsy in 17 (31.5%) patients or magnetic resonance imaging in 50 (92.6%). We analyzed patient characteristics, treatments, and outcomes among all COVID-19–associated AM. Results: AM prevalence among hospitalized patients with COVID-19 was 2.4 per 1000 hospitalizations considering definite/probable and 4.1 per 1000 considering also possible AM. The median age of definite/probable cases was 38 years, and 38.9% were female. On admission, chest pain and dyspnea were the most frequent symptoms (55.5% and 53.7%, respectively). Thirty-one cases (57.4%) occurred in the absence of COVID-19–associated pneumonia. Twenty-one (38.9%) had a fulminant presentation requiring inotropic support or temporary mechanical circulatory support. The composite of in-hospital mortality or temporary mechanical circulatory support occurred in 20.4%. At 120 days, estimated mortality was 6.6%, 15.1% in patients with associated pneumonia versus 0% in patients without pneumonia ( P =0.044). During hospitalization, left ventricular ejection fraction, assessed by echocardiography, improved from a median of 40% on admission to 55% at discharge (n=47; P Conclusions: AM occurrence is estimated between 2.4 and 4.1 out of 1000 patients hospitalized for COVID-19. The majority of AM occurs in the absence of pneumonia and is often complicated by hemodynamic instability. AM is a rare complication in patients hospitalized for COVID-19, with an outcome that differs on the basis of the presence of concomitant pneumonia.

BACKGROUND The risk of adverse cardiovascular events in patients with acute myocarditis (AM) and desmosomal gene variants (DGV) remains unknown.OBJECTIVES The purpose of this study was to ascertain the risk of death, ventricular arrhythmias, recurrent myocarditis, and heart failure (main endpoint) in patients with AM and pathogenic or likely pathogenetic DGV.METHODS In a retrospective international study from 23 hospitals, 97 patients were included: 36 with AM and DGV (DGV[+]), 25 with AM and negative gene testing (DGV[-]), and 36 with AM without genetics testing. All patients had troponin elevation plus findings consistent with AM on histology or at cardiac magnetic resonance (CMR). In 86 patients, CMR changes in function and structure were re-assessed at follow-up.RESULTS In the DGV(+) AM group (88.9% DSP variants), median age was 24 years, 91.7% presented with chest pain, and median left ventricular ejection fraction (LVEF) was 56% on CMR (P = NS vs the other 2 groups). Kaplan-Meier curves demonstrated a higher risk of the main endpoint in DGV(+) AM compared with DGV(-) and without genetics testing patients (62.3% vs 17.5% vs 5.3% at 5 years, respectively; P < 0.0001), driven by myocarditis recurrence and ventricular arrhythmias. At follow-up CMR, a higher number of late gadolinium enhanced segments was found in DGV(+) AM. CONCLUSIONS Patients with AM and evidence of DGV have a higher incidence of adverse cardiovascular events compared with patients with AM without DGV. Further prospective studies are needed to ascertain if genetic testing might improve risk stratification of patients with AM who are considered at low risk. (J Am Coll Cardiol HF 2022;10:714-727) (c) 2022 by the American College of Cardiology Foundation.

International audience; Purpose: This study aimed at determining the diagnostic implications of indirect signs of infection at FDG-PET-i.e., hypermetabolisms of the spleen and/or bone marrow (HSBM)-when documented in patients with known or suspected infective endocarditis (IE).Methods: HSBM were defined by higher mean standardized uptake values comparatively to that of the liver on FDG-PET images from patients with a high likelihood of IE and prospectively included in a multicenter study.Results: Among the 129 included patients, IE was ultimately deemed as definite in 88 cases. HSBM was a predictor of definite IE (P = 0.014; odds ratio (OR) 3.2), independently of the criterion of an abnormal cardiac FDG uptake (P = 0.0007; OR 9.68), and a definite IE was documented in 97% (29/30) of patients showing both HSBM and abnormal cardiac uptake, 78% (7/9) of patients with only abnormal cardiac uptake, 67% (42/63) of patients with only HSBM, and 37% (10/27) of patients with neither one.Conclusion: In this cohort with a high likelihood of IE, HSBM is an additional albeit indirect sign of IE, independently of the criterion of an abnormal cardiac uptake, and could reinforce the suspicion of IE in the absence of any other infectious, inflammatory, or malignant disease.

Aims The MITRA-FR trial showed that among symptomatic patients with severe secondary mitral regurgitation, percutaneous repair did not reduce the risk of death or hospitalization for heart failure at 12 months compared with guideline-directed medical treatment alone. We report the 24-month outcome from this trial. Methods and results At 37 centres, we randomly assigned 304 symptomatic heart failure patients with severe secondary mitral regurgitation (effective regurgitant orifice area >20 mm2 or regurgitant volume >30 mL), and left ventricular ejection fraction between 15% and 40% to undergo percutaneous valve repair plus medical treatment (intervention group, n = 152) or medical treatment alone (control group, n = 152). The primary efficacy outcome was the composite of all-cause death and unplanned hospitalization for heart failure at 12 months. At 24 months, all-cause death and unplanned hospitalization for heart failure occurred in 63.8% of patients (97/152) in the intervention group and 67.1% (102/152) in the control group [hazard ratio (HR) 1.01, 95% confidence interval (CI) 0.77-1.34]. All-cause mortality occurred in 34.9% of patients (53/152) in the intervention group and 34.2% (52/152) in the control group (HR 1.02, 95% CI 0.70-1.50). Unplanned hospitalization for heart failure occurred in 55.9% of patients (85/152) in the intervention group and 61.8% (94/152) in the control group (HR 0.97, 95% CI 0.72-1.30). Conclusions In patients with severe secondary mitral regurgitation, percutaneous repair added to medical treatment did not significantly reduce the risk of death or hospitalization for heart failure at 2 years compared with medical treatment alone.

BackgroundDiagnostic and patients’ management modifications induced by whole-body 18F-FDG-PET/CT had not been evaluated so far in prosthetic valve (PV) or native valve (NV) infective endocarditis (IE)-suspected patients.MethodsIn sum, 140 consecutive patients in 8 tertiary care hospitals underwent 18F-FDG-PET/CT. ESC-2015-modified Duke criteria and patients’ management plan were established jointly by 2 experts before 18F-FDG-PET/CT. The same experts reestablished Duke classification and patients’ management plan immediately after qualitative interpretation of 18F-FDG-PET/CT. A 6-month final Duke classification was established.ResultsAmong the 70 PV and 70 NV patients, 34 and 46 were classified as definite IE before 18F-FDG-PET/CT. Abnormal perivalvular 18F-FDG uptake was recorded in 67.2% PV and 24.3% NV patients respectively (P < .001) and extracardiac uptake in 44.3% PV and 51.4% NV patients. IE classification was modified in 24.3% and 5.7% patients (P = .005) (net reclassification index 20% and 4.3%). Patients’ managements were modified in 21.4% PV and 31.4% NV patients (P = .25). It was mainly due to perivalvular uptake in PV patients and to extra-cardiac uptake in NV patients and consisted in surgery plan modifications in 7 patients, antibiotic plan modifications in 22 patients and both in 5 patients. Altogether, 18F-FDG-PET/CT modified classification and/or care in 40% of the patients (95% confidence interval: 32–48), which was most likely to occur in those with a noncontributing echocardiography (P < .001) or IE classified as possible at baseline (P = .04), while there was no difference between NV and PV.ConclusionsSystematic 18F-FDG-PET/CT did significantly and appropriately impact diagnostic classification and/or IE management in PV and NV-IE suspected patients.Clinical Trials RegistrationNCT02287792.

Background: Mitral valve prolapse (MVP) is a frequent disease that can be complicated by mitral regurgitation (MR), heart failure, arterial embolism, rhythm disorders, and death. Left ventricular (LV) replacement myocardial fibrosis, a marker of maladaptive remodeling, has been described in patients with MVP, but the implications of this finding remain scarcely explored. We aimed at assessing the prevalence, pathophysiological and prognostic significance of LV replacement myocardial fibrosis through late gadolinium enhancement (LGE) by cardiac magnetic resonance in patients with MVP. Methods: Four hundred patients (53±15 years of age, 55% male) with MVP (trace to severe MR by echocardiography) from 2 centers, who underwent a comprehensive echocardiography and LGE cardiac magnetic resonance, were included. Correlates of replacement myocardial fibrosis (LGE+), influence of MR degree, and ventricular arrhythmia were assessed. The primary outcome was a composite of cardiovascular events (cardiac death, heart failure, new-onset atrial fibrillation, arterial embolism, and life-threatening ventricular arrhythmia). Results: Replacement myocardial fibrosis (LGE+) was observed in 110 patients (28%; 91 with myocardial wall including 71 with basal inferolateral wall, 29 with papillary muscle). LGE+ prevalence was 13% in trace-mild MR, 28% in moderate MR, and 37% in severe MR, and was associated with specific features of mitral valve apparatus, more dilated LV and more frequent ventricular arrhythmias (45% versus 26%, P P =0.009) and moderate-severe MR (odds ratio, 2.28 [95% CI, 1.21–4.31], P =0.011). LGE+ was associated with worse 4-year cardiovascular event–free survival (49.6±11.7 in LGE+ versus 73.3±6.5% in LGE–, P P =0.002) were associated with poor outcome. Conclusions: LV replacement myocardial fibrosis is frequent in patients with MVP; is associated with mitral valve apparatus alteration, more dilated LV, MR grade, and ventricular arrhythmia; and is independently associated with cardiovascular events. These findings suggest an MVP-related myocardial disease. Last, cardiac magnetic resonance provides additional information to echocardiography in MVP.

Outcomes research; Risk factors Investigación de resultados; Factores de riesgo Recerca dels resultats; Factors de risc Bioprosthetic heart valves (BHVs) are commonly used to replace severely diseased heart valves but their susceptibility to structural valve degeneration (SVD) limits their use in young patients. We hypothesized that antibodies against immunogenic glycans present on BHVs, particularly antibodies against the xenoantigens galactose-α1,3-galactose (αGal) and N-glycolylneuraminic acid (Neu5Gc), could mediate their deterioration through calcification. We established a large longitudinal prospective international cohort of patients (n = 1668, 34 ± 43 months of follow-up (0.1–182); 4,998 blood samples) to investigate the hemodynamics and immune responses associated with BHVs up to 15 years after aortic valve replacement. Early signs of SVD appeared in

Funding Acknowledgements Type of funding sources: None. Background Lamin A/C cardiomyopathy (CM) is an inherited disease due to LMNA gene mutation with particular phenotype that associates conduction disorders, frequent atrial fibrillation and life-threatening ventricular arrhythmias, with normal or altered ventricular systolic function. Cardiac magnetic resonance (CMR) studies suggest frequent late gadolinium enhancement (LGE) involving septal mid-myocardium. Aims To assess the added value of CMR to conventional clinical features of Lamin A/C CM for the prediction of a positive LMNA gene testing. Methods We performed a retrospective monocentric study in all index patients referred for genetic testing for a clinical suspicion of Lamin A/C CM. Clinical, ECG and imaging data including CMR at time of genetic testing in patients with a positive test (LMNA+) and patients without (LMNA-) were compared. The diagnostic performances of relevant parameters for the prediction of a positive LMNA gene testing were analyzed in several logistic regression models. Results 90 patients were included (55 LMNA+, 35 LMNA-).49% had significant left ventricular (LV) dilatation on echocardiography,57% had a LV ejection fraction (LVEF) Conclusion CMR, particularly septal mid-myocardium LGE, carries good diagnostic accuracy to predict a positive LMNA gene testing in clinically suspected Lamin A/C CM with increased specificity when added to conventional red flags. Abstract Figure. Logistic regression models performances

Background Mitral valve prolapse (MVP) is a common condition in the general population, which can be associated to non-specific ECG abnormalities described initially as ST segment depression, T waves flattening or inversion, especially in the inferior leads. Lately, this type of ECG abnormalities has been reported in patients with MVP and ventricular arrhythmias (VA) or sudden death (SD). However, the prevalence of ECG abnormalities has never been studied in a large series of patients, and the link between ECG abnormalities, VA and SD to echocardiography examination has never been prospectively assessed. Objective To study the prevalence of ECG abnormalities including ventricular arrhythmias in MVP patients and their link with echocardiographic characteristics. Methods All patients (n=731, MVP = 486, Controls = 245) were prospectively enrolled and underwent a comprehensive echocardiography and ECG. In MVP patients 81 had minimal systolic displacement (MSD), 92 isolated MVP, 108 mild-moderate MR, and 196 severe MR. A comprehensive qualitative and quantitative analysis of ECG obtained from rest ECG, 24-hours ECG recording or exercise stress tests, was carried out. Mean follow-up was 4.4 years. Results The mains ECG abnormalities were an inversion of T wave in the inferior leads found in 12 MVP vs 1 control (2.5 vs 0.4%, P=0.047) or a QRS notch (5.1 vs 2.9%, P=0.13). In bileaflet MVP T wave inversion was more frequent as compared with other MVP patients (3.8 vs 0.8%, P=0.039). In addition there was a progressive prolongation of PR interval, QRS duration and increase QT dispersion associated with worsening of MR and heart chambers remodeling. None of ECG findings were significantly linked with the presence of MVP only. Out of 731 individuals, 27 (3.7%) had an history of VA or SD. In a multivariate analysis, bileaflet prolapse and mitral annulus disjunction were associated with VA or SD, whereas none of ECG criterion was associated with. Conclusion In this large prospective series of MVP patients, prevalence of inferior leads ECG abnormalities is very low. Prolongation of atrio-ventricular and ventricular conduction, as well increased QT dispersion is associated with worsening of MR and LV remodeling. Bileaflet prolapse and mitral annulus disjunction are associated with VA or SD. Funding Acknowledgement Type of funding source: None

Background Calcific aortic valve stenosis (CAVS) is the most common valvular heart disease. Although a familial component has been described, the heritability remains unknown. This study aimed to determine the heritability of CAVS and to compare the prevalence of bicuspid aortic valve (BAV) relatives among families with tricuspid and bicuspid aortic valve phenotype and to a control cohort. Methods Probands were recruited following aortic valve replacement in three French university hospitals. All relatives who agreed to participate were enrolled in the study. Families were defined as tricuspid aortic valve (TAV) or BAV families based on the aortic valve phenotype observed in the proband. As control group, we used probands diagnosed with mitral valve prolapse and their families. All recruited probands and relatives underwent comprehensive transthoracic echocardiography to determine aortic valve morphology and CAVS status. Additive genetic heritability was performed with SOLAR genetic statistics package. Prevalence of BAV relatives in each sub-group and Prevalence Ratio were calculated. Results Within the 2371 relatives coming from 138 CAVS families, the heritability of CAVS was significant (h2=0.49, p Conclusions The heritability of aortic valve stenosis in this population confirms the genetic effect in determining aortic stenosis for all aortic valve phenotype and for tricuspid aortic valve. The bicuspid enrichment in TAV families suggests common genetic predisposition of aortic valve stenosis in some families. Taking all together, these findings encourage echocardiography screening of relatives of affected individuals. Funding Acknowledgement Type of funding source: None

Inflammatory cardiomyopathies, also known as "myocarditis" are inflammatory pathologies affecting the myocardium and characterized by vast etiological and clinical heterogeneity. They can be asymptomatic, particularly in viral forms, or be responsible for sudden death, particularly in subjects under 35 years olds. Due to insufficient sensitivity and specificity of imaging and biology, the gold standard is histopathological and is performed on an endomyocardial biopsy or on explanted heart samples in a transplant context. Their classification has considerably evolved and is now based on the identification of a predominant cell pattern such as lymphocytic, neutrophilic or eosinophilic polynuclear, giant cell or granulomatous myocarditis. These different patterns will guide the etiological diagnosis, prognosis and the therapies to be implemented. Due to the importance of viral etiologies, this morphological analysis must be complemented by a virological analysis based on PCR with viral load quantification. In addition, some authors have been able to demonstrate the occurrence of myocarditis in patients with arrhythmogenic cardiomyopathy of genetic origin. The aim of this chapter is to review the current state of knowledge on inflammatory cardiomyopathies and their management.

It remains unknown whether β-blockers are useful and safe in acute myocardial infarction (MI). Owing to its pharmacological profile and vasodilating action, nebivolol (N) is useful in MI. The aim of the present study was to assess in rat whether early nebivolol treatment could be beneficial in MI. It remains unknown whether β-blockers are useful and safe in acute MI. On day (D) 0, male Sprague-Dawley rats underwent left coronary artery ligation (MI) or simple thoracotomy (SHAM). On D1 and D2, the rats were treated with either nebivolol (5 mg.kg-1 .day-1 , MI-N and Sham-N) or vehicle (V, MI-V and Sham-V). On D3, heart rate, left ventricle (LV) intrinsic contractility (PESmid) and arterial elastance were measured. Cardiac and aortic β-Adrenoceptor (AR) subtype mRNA were quantified using real time quantitative RT-qPCR. Catecholamine response was assessed on isolated heart and aortic rings with isoproterenol. PESmid was decreased in MI without worsening the decrease nebivolol. In LV, β1 - and β3 -AR mRNA were respectively decreased and increased in all MI. β3 -AR mRNA increase was partly limited by nebivolol. Ex vivo, basal contractility was less decreased in MI-N than in MI-V. Isoproterenol response was only altered in MI-V. In MI aorta, Nebi prevented β2 - and β3 -AR mRNA increases. In addition, Acetylcholine-induced relaxation was lowered in MI-V but preserved with nebivolol. We demonstrated an early modulation of cardiovascular β3 -AR transcription early MI. Despite its putative negative inotropic properties, nebivolol did not worsen cardiac function in basal conditions and preserved LV catecholamine response.

This study aimed to identify a subset of patients based on echocardiographic parameters who might have benefited from transcatheter correction using the MitraClip system in the MITRA-FR (Percutaneous Repair with the MitraClip Device for Severe Functional/Secondary Mitral Regurgitation) trial.It has been suggested that differences in the degree of mitral regurgitation (MR) and left ventricular (LV) remodeling may explain the conflicting results between the MITRA-FR and the COAPT (Cardiovascular Outcomes Assessment of the MitraClip Percutaneous Therapy for Heart Failure Patients with Functional Mitral Regurgitation) trials.In a post hoc analysis, we evaluated the interaction between the intervention and subsets of patients defined based on MR severity (effective regurgitant orifice [ERO], regurgitant volume [RVOL] and regurgitant fraction [RF]), LV remodeling (end-diastolic and end-systolic diameters and volumes) and combination of these parameters with respect to the composite of death from any cause or unplanned hospitalization for heart failure at 24 months.We observed a neutral impact of the intervention in subsets with the highest MR degree (ERO ≥30 mmIn the MITRA-FR trial, we could not identify a subset of patients defined based on the degree of the regurgitation, LV remodeling or on their combination, including those deemed as having disproportionate MR, that might have benefited from transcatheter correction using the MitraClip system. (Multicentre Study of Percutaneous Mitral Valve Repair MitraClip Device in Patients With Severe Secondary Mitral Regurgitation [MITRA-FR]; NCT01920698).

Introduction Left ventricular (LV) replacement myocardial fibrosis has been described in MVP. We aimed at assessing the prevalence, pathophysiological and prognostic significance of LV replacement myocardial fibrosis through late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) in MVP Method Four hundred patients (53 ± 15 years, 55% male) with MVP underwent echocardiography and CMR. Correlates of replacement myocardial fibrosis (LGE + ), influence of MR degree, and ventricular arrhythmia were assessed. The primary outcome was a composite of cardiovascular events. Results LGE+ was observed in 110 patients (28%; 91 myocardial wall including 71 basal inferolateral wall, 29 papillary muscle). LGE+ prevalence was 13% in trace-mild MR, 28% in moderate and 37% in severe MR, and was associated with specific features of mitral valve apparatus, more dilated LV and more frequent ventricular arrhythmias (45 vs. 26%, P Fig. 1 ). Conclusion LV replacement myocardial fibrosis is frequent in patients with MVP, is associated with mitral valve apparatus alteration, more dilated LV, MR grade, ventricular arrhythmia, and is independently associated with cardiovascular events. These findings suggest a MVP-related myocardial disease. Finally, CMR provides additional information to echocardiography in MVP.

Background Mitral valve prolapse (MVP) is a frequent pathology that can be complicated by mitral regurgitation, heart failure, rhythm disorders, arterial embolism and death. The aim of this study was to evaluate the prognostic interest of ventricular volumes, right ventricular ejection fraction (RVEF) and late gadolinium enhancement (LGE) assessed by cardiac magnetic resonance (CMR) imaging on cardiovascular morbi-mortality in a cohort of patients with MVP Methods We examined the prognostic value of CMR imaging in 237 patients with MVP (no to severe mitral regurgitation) included between 2010 and 2019. All patients underwent a comprehensive echocardiography. The main endpoint was a composite endpoint of cardiovascular death, heart failure, new onset atrial fibrillation, arterial embolism. Results Among the 237 patients (63% male), 97 (41%) had LGE (75 myocardial wall, 10 papillary muscle tip and 12 both locations). Factors associated with LGE in multivariable analysis were age (OR 1.02, P = 0.037), left ventricular (LV) mass (OR 1.01, P = 0.008) and pulmonary artery systolic pressure (PAPS, OR 1.02, P = 0.069). Follow-up was censored at the time of surgery or percutaneous repair. In univariate analysis NYHA class, LV mass, left atrial volume, PAPS, LV end-diastolic and end-systolic volumes, chordal rupture and MR degree were associated with outcome. RVEF and biventricular dysfunction (LV EF < 60% and RV EF < 40%) were also associated with impaired event-free survival (36.0 ± 17.0% vs 65.4± 5.8%, P = 0.019). Finally, LGE was associated with a decreased event-free survival (55.6 ± 9.9% vs 70.7± 6.9%, P = 0.002). In multivariablee analysis, moderate to severe mitral regurgitation (HR : 2.14 [1.44-3.19], P Conclusion CMR imaging provides additional prognostic information to echocardiography in the study of MVP. Myocardial fibrosis of the left ventricle and right ventricular function as assessed by CMR imaging are predictors of cardiovascular morbidity and mortality in MVP.

Funding Acknowledgements PHRCI mitral 2012 Background Filamin-A mitral valve prolapse/dystrophy (FLNA-MVP) phenotype associates moderate MVP and a paradoxical restricted motion in diastole. Purpose We aimed to assess the association of MVP with restricted motion in diastole in MVP patients (restricted MVP). Methods We prospectively enrolled 433 MVP probands (57 ± 16 years). Patients underwent a clinical examination and a comprehensive echocardiographic analysis of mitral valve apparatus. Results Among the 433 probands, 27 (6.2%, 95% CI 3.9-8.5) had both a MVP and a doming aspect in diastole. Patients with restricted MVP exhibited shorter posterior chordae tendinaes (24.8 ± 6.3 vs 27.2 ± 5.9 mm, P = 0.037), and a shorter distance between papillary muscle (PM) tips and mitral annulus (anterior PM: P = 0.0001; posterior PM: P = 0.009). Anterior mitral valve leaflet was lengthened (15.5 ± 2.4 vs 14.3 ± 2.6 mm/m², P = 0.018), but leaflet thickness, leaflet prolapse, and mitral valve annulus did not differ between the 2 groups. Bicuspid aortic valve was more frequent in patients with restricted phenotype (14.8 vs 2.9%, P Conclusion Restricted MVP is a quite frequent finding in MVP patients and is associated with PM tips location closer to mitral annulus. Restricted MVP can be regarded as a third type of MVP beside myxomatous Barlow disease and fibro-elastic deficiency MVP.

Aims Several data suggest that acute myocarditis could be related to genetic variants involved in familial cardiomyopathies, particularly arrhythmogenic cardiomyopathy, but the management of patients with acute myocarditis and their families regarding their risk for having an associated inherited cardiomyopathy is unclear. Methods and results Families with at least one individual with a documented episode of acute myocarditis and at least one individual with a cardiomyopathy or a history of sudden death were included in the study. Comprehensive pedigree, including genetic testing, and history of these families were analysed. Six families were included. Genetic analysis revealed a variant in desmosomal proteins genes in all the probands [five in desmoplakin (DSP) gene and one in desmoglein 2 gene]. In the five families identified with a DSP variant, genetic testing was triggered by the association of an acute myocarditis with a single case of apparently isolated dilated cardiomyopathy or sudden death. Familial screening identified 28 DSP variant carriers; 39% had an arrhythmogenic left ventricular (LV) cardiomyopathy phenotype. Familial histories of sudden death were frequent, and a remarkable phenotype of isolated LV late gadolinium enhancement on contrast‐enhanced cardiac magnetic resonance without any other structural abnormality was found in 38% of asymptomatic mutation carriers. None of the DSP variant carriers had imaging characteristics of right ventricle involvement meeting current Task Force criteria for arrhythmogenic right ventricular cardiomyopathy. Conclusions Comprehensive familial screening including genetic testing in case of acute myocarditis associated with a family history of cardiomyopathy or sudden death revealed unknown or misdiagnosed arrhythmogenic variant carriers with left‐dominant phenotypes that frequently evade arrhythmogenic right ventricular cardiomyopathy Task Force criteria. In view of our results, acute myocarditis should be considered as an additional criterion for arrhythmogenic cardiomyopathy, and genetic testing should be advised in patients who experience acute myocarditis and have a family history of cardiomyopathy or sudden death.

Background The link between acute myocarditis (AM) and familial cardiomyopathy remains unclear. Purpose To assess the clinical significance of AM in families with cardiomyopathy. Methods and results We describe the pedigree of 6 families with at least one familial case of AM and a familial history of cardiomyopathy or sudden death (SD). AM was defined as an infarct-like clinical presentation with normal coronary arteries and myocardial inflammation (MI) documented by cardiac magnetic resonance (CMR), or as an autopsy proven AM. Detailed familial pedigrees are shown in the picture. In family 1 to 5, genetic testing was triggered by the association of a documented case of AM with an index case of cardiomyopathy or early SD. In this setting, all genetic testing revealed a mutation in the desmoplakin (DSP) gene. In family 1, patient II.1 (15 y.o) was diagnosed with AM, 6 months after his sister died suddenly at the age of 12. In family 2, patient II.4 (17 y.o) was diagnosed with AM. His mother had a DCM, with a CMR revealing the presence of MI. In family 3, patient IV.3 (22 y.o) died suddenly from an AM, attested by post-mortem autopsy. Her aunt had a DCM. In family 4, patient II.4 (41 y.o) had an AM, progressing toward a DCM. Her mother had died suddenly at the age of 39, and her niece had a DCM. In family 5, patient V.16 (9 y.o) presented 4 recurrent episodes of AM. Her cousin's mother had a DCM. In family 6, patient IV.3 had 3 episodes of AM, his father had previously been diagnosed with an arrythmogenic right ventricular cardiomyopathy (ARVC) with a desmoglein 2 (DSG2) mutation. Table shows detailed genotype-phenotype relationship in all mutation carriers screened in the 6 families. Phenotypes observed in mutation carriers Mutation DCM ARVC AM Isolated LGE (no cardiomyopathy, no AM) Family 1 (n=3) DSP 0 0 1 1 Family 2 (n=3) DSP 1 0 1 0 Family 3 (n=11) DSP 5 0 1 0 Family 4 (n=3) DSP 2 0 1 0 Family 5 (n=7) DSP 2 0 1 3 Family 6 (n=5) DSG2 0 1 1 1 Family Pedigrees Conclusion AM is strongly associated to desmosomal mutations when a familial history of cardiomyopathy is present, particularly in DSP gene. In these families, DCM phenotype and SD are frequent, and a notable proportion of isolated LGE suggestive of myocardial fibrosis is present in asymptomatic relatives. These results highlight the need for a comprehensive familial screening in case of AM.

Background Filamin-A mitral valve prolapse/dystrophy (FLNA-MVP) phenotype associates MVP and a paradoxical restricted motion in diastole. Purpose We aim to assess the association of mitral valve prolapse to restricted motion in diastole in MVP patients (restricted MVP). Methods We prospectively enrolled 475 MVP probands (64±13 years) and controls relatives. Patients underwent a clinical examination and a comprehensive echocardiographic analysis of mitral valve apparatus. Results Among 475 consecutive probands, 48 (10.1%, 95% CI 7.7–13.3) had both a MVP and a doming aspect in diastole. Patients with restricted MVP exhibited shorted chordae tendinaes, and a shorter distance between papillary muscle tip and mitral annulus. Compared with controls, mitral valve leaflets were lenghtened, thickened and mitral valve annulus was enlarged. The prevalence of polyvalvular disease and bicuspid aortic valve was not increased in restricted MVP patients compared with conventional MVP. Familial form of restricted MVP was identified even in the absence of Filamin-A mutation. Conclusion Restricted MVP is a quite frequent finding in MVP patients and is associated with unique features of the MV apparatus. Restricted MVP can be regarded as a third type of MVP beside myxomatous Barlow disease and fibro-elastic deficiency MVP. Acknowledgement/Funding PHRC I Mitral, Fédération Française de Cardiologie, Fondation Coeur et recherche

Background Structural valve degeneration (SVD) remains a major complication of aortic bioprostheses. Purpose We aimed to assess the mode of SVD leading to bioprosthetic aortic valve failure (BVF) in a large series of patients. Methods Between 2010 and 2017, we prospectively enrolled 261 consecutive patients with BVF related to SVD. All patients underwent a clinical work-up. Explanted bioprostheses were analysed for assessing the mechanism of SVD. Results The delay from surgery to SVD diagnosis was 8.5±3.3 (1.7 to 21.4) years, 10 years after exclusion of a specific type of bioprosthesis. Of the 261 SVD patients, 150 (57%) had mainly a stenotic type, and 111 (43%) a regurgitant type. In regurgitant SVD bioprosthesis was more frequently porcine (19 vs 7%, P=0.002), prosthesis diameter was larger (23.2±2.5 vs 21.6±1.9 mm; P Conclusion Structural valve degeneration remains a matter of concern in current practice with a mean delay of 8 to 10 years after surgery. Beside classical SVD with extensive calcification process other types of SVD can be observed with minimal calcification.

International audience; AIMS: Percutaneous mitral valve repair has become an alternative to conventional surgery in patients suffering primary mitral regurgitation (MR) with a contraindication to surgery and could benefit patients at high surgical risk. The aim of the MITRA-HR study is to raise the level of evidence supporting the use of the MitraClip(R) device in primary MR patients with a pre-defined high risk for surgery. METHODS AND RESULTS: MITRA-HR is a prospective, multicentre, randomised study designed to compare mitral valve repair using MitraClip(R) with conventional surgery in patients with severe primary mitral regurgitation at high risk for surgery. The surgical risk is defined by age, Society of Thoracic Surgeons (STS) risk estimate score, frailty, major organ system dysfunction, and procedure-specific impediments. The study will enrol 330 patients randomised between conventional surgery and MitraClip(R) with a 1:1 ratio. The composite primary endpoint includes all-cause mortality, unplanned rehospitalisation for cardiovascular reasons, and mitral valve reintervention at 12 months. The main secondary safety endpoint is a major adverse event composite assessment evaluated 30 days after the procedure. CONCLUSIONS: The randomised MITRA-HR study is designed to provide additional supportive evidence of non-inferiority in efficacy and superiority in safety for percutaneous mitral valve repair using MitraClip(R) compared to conventional surgery in high surgical risk patients.

International audience; Multimodality imaging plays an important role in the initial evaluation, diagnosis and management of patients suspected of having a cardiomyopathy. Beyond functional and anatomical information, multimodality imaging provides important variables that facilitate risk stratification and prognosis evaluation. Whatever the underlying suspected cardiomyopathy, echocardiography is the most common initial imaging test used to establish the presence of cardiomyopathy, by depicting structural and functional abnormalities. However, echocardiographic findings are non-specific, and therefore have a limited role in identifying the underlying aetiology. Cardiac magnetic resonance imaging allows characterization of myocardial tissue, which can be of great help in identifying the aetiology of the cardiomyopathy. When a specific aetiology is suspected, particularly inflammation, 18F-fluorodeoxyglucose positron emission tomography is recommended. The clinician should be capable of selecting the appropriate imaging techniques for each clinical scenario. Each technique has strengths and weaknesses, which should be known. In order to improve diagnostic performance, and as proposed by the European Association for Cardiovascular Imaging, cardiovascular imaging groups must be composed of experts from all modalities. The future of multimodality imaging in the diagnosis and management of cardiomyopathies will also involve evolution of its use in care, teaching and research. Training goals for future cardiac imaging experts must be defined; academic and industry partnerships should enable the connection to be made between imaging data and clinical data on the one hand and outcomes on the other hand, using big-data analysis and artificial intelligence.

Background Primary mitral regurgitation (MR) is the second cause of valvular surgery in Europe. Early surgery seems to improve long term prognosis, raising the need of prognostic markers. Purpose Evaluate the prognostic value of %VO2max, assessed by cardiopulmonary echocardiography exercise test (CPEET), in terms of free-of-cardiovascular-event survival. Methods The patients underwent CPEET for primary MR between march 2012 and march 2019. Clinical follow-up of a composite cardiovascular event (cardiovascular death, heart failure progression ≥ 1 NYHA stage or unplanned hospitalization for heart failure, de novo atrial fibrillation, systemic embolic event, symptomatic ventricular tachycardia). Results 180 patients were analysed, with a median follow-up of 33 months. Free-of-events survival at 48 months was significantly associated with % VO2max 180s (42.3% vs. 58.1%, P = 0.001) and with maximal effort sPAP > 60 mmHg (42.2% vs. 67.8% P = 0.006) in univariate analysis. In multivariate analysis the impairment of %VO2max Conclusion An impairment of functional capacity, assessed by a cardiopulmonary-echocardiography-exercise-test with a %VO2max

Background In patients who have chronic heart failure with reduced left ventricular ejection fraction, severe secondary mitral-valve regurgitation is associated with a poor prognosis. Whether percutaneous mitral-valve repair improves clinical outcomes in this patient population is unknown. Methods We randomly assigned patients who had severe secondary mitral regurgitation (defined as an effective regurgitant orifice area of >20 mm2 or a regurgitant volume of >30 ml per beat), a left ventricular ejection fraction between 15 and 40%, and symptomatic heart failure, in a 1:1 ratio, to undergo percutaneous mitral-valve repair in addition to receiving medical therapy (intervention group; 152 patients) or to receive medical therapy alone (control group; 152 patients). The primary efficacy outcome was a composite of death from any cause or unplanned hospitalization for heart failure at 12 months. Results At 12 months, the rate of the primary outcome was 54.6% (83 of 152 patients) in the intervention grou...

Background: Filamin-A ( FLNA ) mutations have been associated with the development of mitral valve prolapse and a unique mitral valve features described as a paradoxical restrictive leaflets motion in diastole has been recently described using a comprehensive echocardiographic screening. Polyvalvular diseases have also been reported in these patients, especially affecting the aortic valve. Objectifs: The objective of this study was to perform a comprehensive echocardiographic analysis of the aortic valve (AV) and the proximal aortic root of patients with FLNA mutations, and assess the impact of the aortic disease on outcomes. Methods: We included in this analysis 256 subjects (42±22 years, 136 men, 76 mutated: FLNA+) with confirmed genetic status, from 5 FLNA families. Comprehensive echocardiographic characterization of the aortic valve and the proximal aortic root, including the measurement of the aortic annulus, sinuses of Valsalva, sinotubular junction and ascending aorta, was performed in FLNA+ patients vs control relatives. Results: Overall, 47 subjects (18%) presented an aortic valve alteration: 40 (53%) of FLNA+ compared to 7 (4%) FLNA- subjects (p2 ; p=0.02), sinuses of Valsalva (17.8±2.5 vs 16.2±1.9 mm/m 2 ; p2 ; p Conclusion: The FLNA -mutated patients presented aortic valve disease more frequently, including a higher prevalence of bicuspid valve, stenosis, and regurgitation owing to either cusp prolapsed or restrictive motion. This unique features described in this population was associated with worse clinical outcomes, especially in FLNA+ males. Management and decision making should be done according to the features of these patients with polyvalvular diseases

Introduction Structural valve degeneration (SVD) of surgical bioprostheses (BP) is increasingly diagnosed. We aimed to reappraise aortic BP SVD mode, determinants, effect of treatment and outcome. Method A total of 262 SVD patients (78 ± 9 years, 53.4% males) were enrolled. The mode and determinants of SVD were investigated by echocardiography, surgical and macroscopic evaluation. Mortality and the effect of treatment were assessed. Results The predominant mode of SVD was stenosis (58%). The annualized rate of increase in mean gradient was 4.6 ± 3.2 mmHg/year in stenotic SVD. In multivariable analysis, predictive factors of an earlier SVD were age (P 3000 (HR 2.22, P = 0.004). Conclusion The rate of SVD progression is 4 to 5 mmHg/year on average. BP type and post-operative hemodynamic are predictors of faster SVD. NoCalcif accounts for > 15% of SVD. Leaflet fibrosis is a component of SVD. Redo-surgery and VinV are associated with a better outcome, independently of failure mode, and should be considered in most SVD patients.

Secondary mitral regurgitation (MR) is frequent valvular heart disease and conveys worse prognostic. Therapeutic surgical or percutaneous options are available in the context of severe symptomatic secondary MR, but the best approach to treat these patients remains unclear, given the lack of clear clinical evidence of benefit. A comprehensive evaluation of the mitral valve apparatus and the left ventricle (LV) has the ability to clearly define and characterize the disease, and thus determine the best option for the patient to improve its clinical outcomes, as well as quality of life and symptoms. The current report reviews the mitral valve (MV) anatomy, the underlying mechanisms associated with secondary MR, the related therapeutic options available, and finally the usefulness of a multimodality imaging approach for the planning of surgical or percutaneous mitral valve intervention.

Introduction Filamin-A mitral valve prolapse/dystrophy (FLNA-MVP) phenotype associates moderate MVP and a paradoxical restricted motion in diastole. We aim to assess the association of MVP with restricted motion in diastole in MVP patients (restricted MVP). Method We prospectively enrolled 475 MVP probands (64 ± 13 years). Patients underwent a clinical examination and a comprehensive echocardiographic analysis of mitral valve apparatus. Results Among the 475 probands, 48 (101%, 95% CI 7.7–13.3) had both a MVP and a doming aspect in diastole. Patients with restricted MVP exhibited shorter chordae tendinaes, and a shorter distance between papillary muscle tip and mitral annulus. Compared with controls, mitral valve leaflets were lengthened, thickened and mitral valve annulus was enlarged. The prevalence of polyvalvular disease and bicuspid aortic valve was not increased in restricted MVP patients compared with conventional MVP. Familial form of restricted MVP was identified even in the absence of Filamin-A mutation. Conclusion Restricted MVP is a quite frequent finding in MVP patients and is associated with unique features of the MV apparatus. Restricted MVP can be regarded as a third type of MVP beside myxomatous Barlow disease and fibro-elastic deficiency MVP.

Introduction Structural valve degeneration (SVD) of bioprostheses is a common and serious complication in patients undergoing aortic valve replacement. SVD pathophysiology remains unclear.18F-sodium fluoride (NaF) and 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) have respectively shown that an active metabolic phenomenon of calcification rather than an inflammatory process contribute to native aortic valve stenosis. We studied the respective value of NaF and FDG PET to explore the potential mechanisms involved in SVD. Method SVD patients underwent NaF PET to explore potential active calcification process, FDG PET to explore potential inflammatory process and thoracic CT. Tracer uptake was quantitatively measured by the bioprosthesis tissue-to-background ratio of standardized uptake values (TBR). Echocardiographic parameters, bioprosthesis calcium scoring on CT, and qualitative pattern of NaF and FDG activity on bioprostheses were analyzed. Results Twenty-one patients were included. Calcium score was higher in patients with significant NaF visual uptake versus patients without (1065 ± 505 vs. 462 ± 320, P = 0.015). The median NaF TBR (3.49, [2.33–5.04]) was significantly higher than FDG (1.34, [1.20–1.47]). Patients with NaF TBR greater than the median value had a higher calcium score (1059 ± 550 vs. 566 ± 363, P = 0.05), and showed a tendency to have more severe hemodynamic stenosis. Picture shows an example of patient with SVD (A); CT (B) established localizations of calcium deposits; NaF PET/CT (C) show intense uptake adjacent to calcifications; FDG PET/CT (D) show no uptake ( Fig. 1 ). Conclusion These results suggest a link between SVD severity and active calcification activity and opens new perspectives on the assessment of SVD pathophysiology through NaF PET quantification of ongoing mineral burden.

Tako-Tsubo cardiomyopathy, first described in 1990 by Sato in Japan, has recently gained increasing consideration when reported in non-Japanese patients, including the United States and Europe. Typical presentation mimics acute coronary syndrome, with acute chest pain and/or dyspnoea, associated to electrocardiographic changes and moderate cardiac biomarkers release, but in which coronary angiography reveals no coronary arteries lesions and echocardiography or left ventriculography shows a reversible left ventricle systolic dysfunction. Prognosis is good, in contrast to acute coronary syndrome, provided that the patients survive the possible life-threatening acute presentation, with correction of the left ventricle systolic dysfunction within several days or weeks. As noted in several reviews, 3.5% to 10% of the patients have a recurrence during the first few years after the initial presentation. Here, we described a case of a 60-year-old female who had three episodes of Tako-Tsubo always preceded by severe emotional stress suggesting a potential common etiopathogenesis.

Purpose Organic mitral regurgitation (MR) is a frequent heart valve disease which influences ventricular functions and impacts cardiovascular outcome. Right Ventricular Free Wall Longitudinal Strain (RVLS) is a good prognosticator in heart failure. Hence, we aimed to study its predictive value in organic MR. Methods and results Two hundred fifty-eight patients (59 ± 15 years, 154 males) with mild to severe organic MR were prospectively enrolled with a complete echocardiography including RV free wall and LV global longitudinal strain assessment. Out of 181 patients with moderate to severe MR, 101 had normal LV and RV function (NoDysf), 40 patients (22%) had an isolated LV dysfunction (≤ −20%, LVdysf), 17 (9%) had an isolated RV dysfunction (≤ −21%, RVdysf), 23 (13%) had a biventricular dysfunction (BiVdysf). Biventricular dysfunction was strongly associated with worse structural (tricuspid annulus diameter: 22.2 ± 3.5 mm/m2 vs. 19.2 ± 3.5 mm/m2, P = 0.0003) and functional RV remodeling (RV fractional shortening: 27.6 ± 9.6% vs. 38.3 ± 11.4%, P Conclusion In organic MR, we identified by echocardiographic strain modality a sub-group of patients (BiVDysf), which portends a higher cardiovascular risk. The adjunction of RV longitudinal strain assessment in the clinical work-up of these patients could improve the evaluation of cardiovascular risk and may be helpful in the clinical-decision making process.