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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.

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

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.

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.

A cardiac magnetic resonance (CMR) imaging was performed 7 days after the admission of a 41-year-old woman referred for an acute coronary syndrome and normal coronary angiography, high-sensitive Tc troponin increase, and impaired left ventricle ejection fraction (LVEF). T2-weighted sequences showed increased sub-epicardial signal in anterior and inferior walls in favour of a myocardial …