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Introduction Left ventricular non compaction (LVNC) is a heterogeneous entity with uncertain prognosis. Cardiac magnetic resonance (CMR) is widely used in the diagnosis of LVNC. However, its role in risk stratification has not been well established. Purpose Therefore, the aim of our study was to identify prognostic CMR variables in LVNC. Methods We conducted a retrospective longitudinal multicentre cohort study of consecutive patients fulfilling CMR LVNC criteria. The endpoints were heart failure (HF), ventricular arrhythmias (VA), systemic embolisms (SE) and all-cause mortality. Biventricular volumes, ejection fraction (LVEF and RVEF) as well as late gadolinium enhancement (LGE) were analysed. Results A total of 310 patients were included: age was 44.4±19 and 43% female. LVEF was 47% ± 15%, RVEF was 48±12 and 28 patients (9%) presented LGE. After a median follow-up of 3.8 2.5 years, 40 patients (13%) presented HF, 31 (10%) had VA, SE occurred in 6 (2%) and 3 patients (1%) died. Baseline characteristics of patients with and without HF and VA are described in Table 1. In univariate analysis, LVEF, LV volumes, LGE, and RVEF were associated with both HF and VA risk. In multivariate analysis, LVEF was the only variable independently associated with HF (HR 0.932, CI 95% 0.88–0.97, p 0.003). Patients with an LVEF >35% were at very low risk of HF (Figure 1A). With regards to VA, LGE was the only independent predictor (HR 2.64, IC 95% 1.059–6.61, p 0.003) (Figure 1B). In LGE negative patients, the arrhythmic risk was higher among those with an LVEF Conclusions In a large multicentre retrospective LVNC study, left ventricular ejection fraction and late gadolinium enhancement were the main predictors of cardiovascular events. Patients with an LVEF Funding Acknowledgement Type of funding sources: None.

Introduction Accurate and reproducible assessment of aortic diameters and their growth rate is of key importance for the management of patients with thoracic aortic aneurysms [1,2]. It has been recently shown that image registration permits the assessment of progressive aortic dilation on ECG-gated contrast-enhanced CT angiography, outperforming manual quantification and allowing for 3D aortic size and growth mapping [3]. However, exposure to radiation makes it convenient to limit the use of CT for serial follow-up, especially in young patients. Self-navigated 3D whole-heart CMR acquisitions provides excellent image quality overcoming these limitations [4]. Purpose To evaluate the accuracy and reproducibility of registration-based assessment of aortic dilation using self-navigated 3D whole-heart CMR acquisitions. Methods Fifteen patients with two self-navigated 3D whole-heart CMR images obtained at least 1 year apart were included. Aortic root and thoracic aorta diameters were measured by 2 independent observers both manually (multiplanar reconstruction) and with the registration-based technique. To perform registration-based assessment, the aorta was semi-automatically segmented and typical anatomical landmarks were placed by each observer at baseline [3]. Geometrical mapping between baseline and follow-up acquisitions was obtained using deformable image registration, and applied to the baseline aortic surface points to obtain their location at follow-up. Finally, aortic diameters and their growth rate were automatically measured and used to calculated 3D aortic dilation maps. Agreement between techniques and their inter-observer reproducibility were calculated. Results Patients age was 27.2±14.5 years and 40% were male. Mean follow-up duration was 2.7±1.6 years. Compared to manual assessment, the registration-based technique presented low bias and excellent agreement for aortic diameters (Table 1), and low bias and moderate agreement for growth rates both in the aortic root and the thoracic aorta (Table, Fig. 1A). The techniques presented similar inter-observer reproducibility in the assessment of aortic diameters (Table 1), while the registration-based method demonstrated much higher inter-observer reproducibility in the assessment of growth rates in the aortic root and the thoracic aorta (Table 1, Fig. 1A and B). Three-dimensional mapping of thoracic aortic diameters and growth was highly reproducible (mean regional ICC=0.90 for diameters; 0.82 for growth rate). Conclusion The assessment of the dilation rate of the thoracic aorta via registration of serial self-navigated 3D whole-heart CMR acquisitions is accurate and reproducible in the aortic root and the thoracic aorta. Thus, it allows to assess local aortic growth without the drawbacks of CT. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Instituto de Salud Carlos III and Ministerio de Ciencia e Innovaciόn (Spain)

Introduction Marfan syndrome (MFS) is a connective tissue disorder related to FBN1 mutations. The main cardiac feature of this syndrome is the enlargement and the dissection of the ascending aorta. Close monitoring of the aortic root diameters and its preventive replacement has allowed improving life expectancy of these patients in the last decades. However, there is paucity of data about the outcome of aortic dissection affecting the descending aorta and its markers of poor evolution. Purpose To characterize patients with MFS and dissection involving the descending aorta. To compare baseline characteristics and the clinical outcome between patients with isolated type B dissection (Group B) and patients with type A dissection with residual B dissection (Group A). Methods Retrospective longitudinal analysis of patients that attended our Marfan unit between 2001 and 2021 with an aortic event involving the descending aorta. Clinical data were collected from electronic health records. Results From the global cohort of adults with Marfan syndrome seen at our centre, we identified a total of 37 adults (11.5% of the overall cohort) that had an acute aortic event with involvement of the descending aorta. 20 (54.1%) were type A aortic events and 17 (45.9%) were isolated type B dissections. Cardiovascular risk factors were similarly distributed in both groups but there was a significant higher percentage of women in Group B (35.0% vs 76.5%, p=0.012) (Figure 1). Acute management of the aortic event implied surgery in 17 (85.0%) patients in Group A and in 5 (29.4%) patients in Group B (p=0.001). In Group B, interventions were 3 TEVAR and 2 open surgeries. 2 (5.4%) patient died during the acute phase, 1 (5.0%) in Group A and 1 (5.9%) in Group B (p=ns).The remaining 35 patients (19 in Group A, 16 in Group B) were followed-up for 10.4±6.7 years. 10 patients died during follow-up (6 in Group A, 4 in Group B). In Group A, 2 patients died of sudden death, 2 patients died in the perioperative setting of aortic surgery, and 2 patients died of an aortic rupture. In Group B, 3 patients died in the perioperative setting of aortic surgery and 1 of an intracranial hemorrhage. The proportions of aortic events during follow-up (death, distal redissection, proximal dissection, distal intervention or aortic root surgery) in both groups are described in Figure 2. Median survival free of reintervention or death (non-related to initial event) was 7.5 years in the overall cohort; 8.2 years in Group A and 2.2 years in Group B (log rank p=0.04) (Figure 2). Conclusions Patients with Marfan syndrome and aortic dissection involving the descending aorta have a poor clinical outcome, with high rates of reintervention, redissection or death. Although Group B required less often surgery during the acute phase, worst survival free of reintervention or death was observed during follow-up. Funding Acknowledgement Type of funding sources: None.

Introduction Abnormal aortic flow patterns in patients with a connective tissue disorder (CTD), such as Marfan or Loeys-Dietz syndrome, may contribute to aortic root dilation [1,2]. Valve sparing aortic root replacement, which is effective in reducing the risk of aortic dissection in case of severe dilation, may also normalize flow patterns beyond the replaced aorta and potentially slow its progressive aortic dilation. Purpose To assess aortic flow dynamics in patients with a CTD by 4D flow cardiovascular magnetic resonance (CMR) before and after valve sparing aortic root replacement, and to compare the results with those of healthy volunteers (HV). Methods Patients with Marfan or Loeys-Dietz syndrome underwent two non-contrast enhanced 4D flow CMR, one before and another after undergoing valve sparing aortic root replacement. Healthy volunteers matched for age, sex and BSA were also included for comparison. Maximum velocity, in-plane rotational flow (IRF), systolic flow reversal ratio (SFRR) and wall shear stress (WSS) magnitude and its axial and circumferential components were obtained at 24 planes covering the thoracic aorta from the sinotubular junction to the descending aorta at the diaphragmatic level [3–5]. Results Sixteen patients and 21 healthy volunteers were included. Demographic and clinical data is presented in Table. The mean time between the CMR prior and posterior to surgery was 15 months. Compared to HV, patients with CTD before intervention presented lower maximum velocity at the proximal ascending aorta (Fig. 1A), lower IRF and circumferential WSS at the arch and the proximal descending aorta (Fig. 1B and F), lower magnitude and axial WSS at the proximal ascending and descending aorta (Fig. 1E and D), and increased SFRR at the proximal descending aorta (Fig. 1C). The intervention completely restored maximum velocity and partially-restored physiological helical flow and circumferential WSS, but barely improved axial WSS and SFRR. Conclusion Valve sparing aortic root replacement in patients with Marfan or Loeys-Dietz syndrome partially restore to physiological level both in-plane rotational flow and circumferential wall shear stress in the descending aorta. This flow normalization may contribute to prevent progressive dilation after the surgery. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Instituto de Salud Carlos III (Spain) (PI17/00381)Spanish Society of Cardiology (SEC/FEC-INV-CLI 20/015)

Background In different clinical scenarios (i.e., obesity, COPD, exercise, mechanical ventilation), the swings in intrathoracic pressure are much larger, and end-expiratory (Pee) pressure can be significantly greater than atmospheric pressure. In these scenarios, the intravascular (Piv) pulmonary pressure can overestimate the true transmural (tm) value and it is recommended to read the average of Piv over a few respiratory cycles (Pmrc) [1–3]. Purpose To analyze the respiratory swings and the effect of esophageal pressure (PES) (as a surrogate of intrathoracic pressure) on the reading of Piv tracings during the RHC at rest in COPD and interstitial lung disease (ILD) candidates for lung transplantation (LTx). Methods Thirty-one COPD (15) and ILD (16) candidates for LTx underwent RHC. End-expiratory and mean respiratory cycle measurements were obtained. The respiratory swing was estimated as the difference between maximum-minimum values of Piv. Ten patients (5 COPD/5 ILD) underwent simultaneous RHC and PES (Micro-balloon Esophageal Catheter, LATITUDE) to assess the Ptm (Piv − PES) [4]. Results Both demographic (11F/20M, 60±7 yrs, 25±4 kg/m2) and hemodynamic data (mPAP 24±9 mmHg, pulmonary arterial occlusion pressure [PAOP] 8.6±4 mmHg, right atrial pressure [RAP] 5.2±3.9 mmHg, pulmonary vascular resistance 3.5±2.6 Wu) did not show significant differences between ILD and COPD except the cardiac index (ILD: 2.8±0.8 vs. COPD: 2.4±0.3 L/min/m2). Intravascular RAPee and PAOPee were higher than mrc values in both groups (Fig 1). However, transmural RAPee and PAOPee were similar to and correlated with (r=0.62 and 0.69, respectively; p Conclusion End-expiratory intravascular RAP and PAOP overestimates the mean respiratory cycle pressures in COPD and ILD candidates for LTx. Averaging pulmonary vascular pressure tracings over the respiratory cycle would be accurate in COPD but could underestimate transmural values in ILD candidates for LTx. The reading of mean respiratory cycle pressure could not be enough to correct the pulmonary pressures measurement error associated with the presence of large swings of intrathoracic pressure. Funding Acknowledgement Type of funding sources: None.

Introduction Left ventricular noncompaction (LVNC) is a poorly defined entity with LV ejection fraction (LVEF) being the main predictor of major adverse cardiovascular events (MACE). Left ventricular hemodynamic forces (LVHDF) have been recently demonstrated to be promising markers of sub-clinical dysfunction and potential predictors of disease outcome. Purpose To determine in a large cohort of LNVC the LVHDF parameters and its long-term prognostic value. Methods Retrospective, longitudinal, multicentre cohort study including consecutive patients with LVNC from 2000 to 2018. CMR was performed at 1.5T and LVHDF were analyzed with a prototype software (Medis Suite Qstrain). Systolic LVHDF were decomposed into “apex-base” (long-LVHDF) and “lateral-septal” (radial-LVHDF). MACE was defined as a composite of heart failure (HF), ventricular arrhythmias (VA), systemic embolisms (SE) and/or all-cause mortality. Results A total of 158 patients were included, age was 53±4.3y and 85 (53.8%) were men. Median LVEF was 44 (IQR 34–55)%, with 61.4% having a LVEF 50%, on univariate regression long-LVHDF Conclusions LVHDF in patients LVNC were quantified for the first time, with an adequate correlation with LVEF. Both radial and longitudinal LVHDF were significantly reduced in patients with MACE, however, only misalignment of systolic longitudinal-LVHDF predicted outcomes. In patients with LVEF >50%, reduced longitudinal-LVHDF was associated with prognosis, while LVEF was not, and may serve as an additional tool for risk stratification. Funding Acknowledgement Type of funding sources: None.

Background Cardiac affectation in amyloidosis is presented among 50% patients what implies worse prognostic. Early diagnosis and prognostic stratification is mandatory after the appearance of new therapies that could modify the course of the disease. Purpose The aim of the present study was to evaluate the main outcome predictors with multiparametric approach including clinical, laboratory, electrocardiographic and imaging parameters in patients with cardiac amyloidosis (CA). Methods We recruited patients (p) prospectively followed in our cardiomyopathy unit, for a mean period of 10 years (from 2010 to 2020) who were diagnosed with CA. Baseline clinical, laboratory and echocardiographic data were obtained. We collected cardiovascular risk factors and previous events. We performed a multimodality imaging study including echocardiogram (TTE) and cardiac magnetic resonance (CMR). TTE hallmarks of CA were recorded, as well as CMR parameters of cardiac function, myocardial mass and tissue characterization. Finally we evaluated the parameters related with mortality in the follow-up. Results A total of 98 p were included, with a mean age of 67.5±16.9 years old. Mean follow-up was 42,2±32 months. 22 p (24,4%) had light-chain amyloidosis (AL), 34 p (37,8%) wild type transthyretin amyloidosis (ATTRwt) and 34 p (37,8%) familiar transthyretin amyloidosis (ATTRm). 43p (47.78%) died in the follow-up. Factors related with mortality in the the follow-up where the age (HR 1.08, ppresence of hypertension (HR 2.81, p=0,002) diabetes (HR 3.12, p=0,001) and previous stroke (HR 2.69, p=0,01); NYHA class at diagnosis (HR 2.49, p0,001) and extension of late gadolinium enhancement (HR 1,06, p=0,03) at CMR. When evaluated within a multivariate analysis in a Cox regression model, the independent variables associated with mortality were NYHA class at diagnosis (p=0.002) and amyloidosis type (p=0.002) ATTRm had a better prognosis than ATTRwt and AL (Log-rank p Conclusion Mortality in cardiac amyloid patients is high. Clinical parameters like hypertension, diabetes, previous stroke and NYHA class at diagnosis; laboratory parameters like BNP and creatinine, main systolic and diastolic function at TTE, presence of auricular fibrosis and extension of late gadolinium enhancement at CRM are related with mortality in the follow-up. Among different types of CA, patients with ATTRm had a better prognosis, which may have therapeutic implications with the appearance of new therapies. Funding Acknowledgement Type of funding sources: None. Mortality

Background Cardiac affectation in amyloidosis is presented among 50% patients what implies worse prognostic. Early diagnosis and prognostic stratification is mandatory after the appearance of new therapies that could modify the course of the disease. Purpose The aim of our study was to assess the prognostic value of cardiac magnetic resonance parameters, including Tissue deformation, in cardiac amyloidosis patients. Methods We recruited patients (p) prospectively followed in our cardiomyopathy unit, for a mean period of 10 years (from 2010 to 2020) who were diagnosed with CA. Baseline clinical, laboratory and echocardiographic data were obtained. Contrast-enhanced CMR was performed on a 1.5 T clinical scanner. Routine analysis was performed with a semi-automatic software for volumetric analysis. When late gadolinium enhancement was present, percentage of area of enhancement (LGE) was calculated.All strain parameters were measured off-line using dedicated software. Left ventricular circumferential strain (CS) measurements were obtained using mid-ventricular level short-axis cine views. Longitudinal strain (LS) derived from cine SSFP of 2-, 3-, and 4-chamber long axis views.Finally we evaluated the CMR parameters related with the combined event of mortality and heart failure in the follow-up. Results A total of 98 p were included, with a mean age of 67.5±16.9 years old. Mean follow-up was 42,2±32 months. 22 p (24,4%) had light-chain amyloidosis (AL), 34 p (37,8%) wild type transthyretin amyloidosis (ATTRwt) and 34 p (37,8%) familiar transthyretin amyloidosis (ATTRm). 59p (60,8%) died or present heart failure during the follow-up. Patients who died or present heart failure in the follow-uo had lower left ventricular ejection fraction (48,25±12,2% vs 56,13±11,03%, p=0,003), higher myocardial mass (156,05±54,8g vs 120,84±56,1g, p=0,007), higher LGE (8,6±6,8% vs 4,4±2%), worst GLS (−14,76±6,1% vs −18,67±6,2%) and worst GCS (−23,3±9,7% vs −30,04±9,1%). Both, GLS and GCS were independently associated with the combined event of dead or heart failure when evaluated within a multivariate analysis in a Cox regression model, but GCS was the stronger predictor of events in the follow-up over other CMR parameters like LGE an myocardial mass (p Conclusion Mortality and appearance of heart failure in cardiac amyloid patients is high. The assessment of myocardial strain parameters by CMR tissue-tracking in this population is useful to predict adverse outcomes in the follow-up. Particularly, GCS, stratify patients in subgroups with different risk of events, with added value to classical CMR parameters. Funding Acknowledgement Type of funding sources: None. Death & Heart Failure by GCS

Background COVID19 has been related to elevated CVB and biventricular dysfunction during hospitalization. However, it is unknown whether patients with biomarker elevation exhibit long-lasting abnormalities in cardiac function. Purpose To determine, using advanced echocardiography, the prevalence and type of cardiovascular sequelae after COVID19 infection with marked elevation of cardiovascular biomarkers (CVB), and their prognostic implications. Methods All patients admitted from March 1st to May 25th, 2020 to a tertiary referral hospital were included. Patients with cardiovascular disease antecedent, death during admission, or the first 30 days after discharge were excluded. Patients with hs-TnI >45 ng/L, NT-proBNP >300 pg/ml, and D-dimer >8000 ng/ml were separated based on each CVB elevation and matched with COVID controls (three biomarkers within the normal range) based on intensive care requirements and age. Results From a total of 2025 hospitalized COVID19 patients, 80 patients with significantly elevated CVB and 29 controls were finally included. No differences in baseline characteristics were observed among groups, but elevated CVB patients were sicker. Follow-up echocardiograms showed no differences among groups regarding LVEF or RV diameters, but TAPSE was lower if hs-TnI or D-dimer were elevated. Hs-TnI patients also had lower global myocardial work and global longitudinal strain. The presence of an abnormal echocardiogram was more frequent in the elevated CVB group compared to controls (23.8 vs 10.3%, P=0.123) but mainly associated with mild abnormalities in deformation parameters. Management did not change in any case and no major cardiovascular events except deep vein thrombosis occurred after a median follow-up of 7 months (Figure 1). Conclusions Minimal abnormalities in cardiac structure and function are observed in COVID19 survivors without previous cardiovascular diseases who presented a significant CVB rise at admission, with no impact on patient management or short-term prognosis. These results do not support a routine screening program after discharge in this population. Funding Acknowledgement Type of funding sources: None. Figure 1

Introduction Left ventricular noncompaction (LVNC) is a poorly defined entity with heterogeneous prognosis. LV ejection fraction (LVEF) is one of the main predictors of major adverse cardiovascular events (MACE). However, outcomes of LVNC patients with preserved LVEF (pEF) remain uncertain. Purpose The aim of our study was to determine the incidence and predictors of MACE in LVNC patients with pEF as well as to assess the evolution of LVEF throughout follow-up. Methods We conducted a retrospective, longitudinal, multicentre cohort study. Consecutive patients with transthoracic echocardiography (TTE) and/or cardiac magnetic resonance (CMR) diagnostic criteria for LVNC and initially pEF (LVEF≥50%) were recruited. MACE were defined as a composite of heart failure (HF), ventricular arrhythmias (VA), systemic embolisms (SE) and/or all-cause mortality. Progressive systolic dysfunction was defined as an LVEF Results A total of 305 patients from 12 centres were included from 2000 to 2018. Age was 38±19 years, 165 (54%) were men and 185 (61%) were probands. LVEF was 62±8% and 8% had late gadolinium enhancement (LGE). During a median follow-up of 4.7 (IQR 2.1–7.4) years, MACE occurred in 40 (13%) patients with an incidence rate of 2.96 (95% CI 2.17–4.04) events per 100 person-years: 8 HF, 27 VA, 3 SE and 5 deaths. LVEF by TTE (HR 0.95, 95% CI 0.90–0.99, p=0.035) and age (HR 1.02, 95% CI 1.01–1-04, p=0.04) were the only variables independently associated with the endpoint. Patients with lower limit LVEF values showed an increased risk of MACE (Figure 1). Among probands, those with family aggregation presented a higher incidence of MACE compared to nonfamilial cases (HR 2.74, p=0.043). A positive genotype was not associated. Sixty-one (21%) patients experienced progressive systolic dysfunction: 31 (11%) had an LVEF Conclusions Patients with left ventricular noncompaction and preserved ejection fraction carry a moderate risk of major adverse cardiovascular events and progressive systolic dysfunction. LVEF remains the main predictor of outcomes in this subgroup. Patients with lower limit LVEF values are at increased risk, probably suggesting subclinical systolic dysfunction. Therefore, they should be carefully monitored. Funding Acknowledgement Type of funding sources: None. Figure 1Figure 2

Background Aortic dissection (AD) is the most devastating complication of thoracic aortic disease (1). In the chronic phase, yearly clinical and imaging follow-up of the maximum aortic diameter is recommended, since indication for thoracic endovascular aortic repair or surgery is suggested by guidelines in case of thoracic aortic enlargement or false lumen (FL) aneurysms (2). Most of the reported parameters related adverse events in chronic AD are focused on morphological variables (3) and not on the haemodynamics and biomechanics of the FL. Purpose To evaluate the relationship between aortic growth rate and anatomical variables, flow patterns and aortic stiffness in patients with chronic type B AD. Methods Forty-one patients with chronic type B aortic dissection, no connective tissue disorders and with an imaging follow-up including two computed tomography angiograms (CTA) acquired at least 3 years apart underwent contrast-enhanced 4D-flow CMR and MR angiography (MRA). The FL volume was segmented from MRA, and velocity data inside the 3D volume of the FL was extracted from 4D-flow CMR and used for parameter quantification. Retrograde systolic and diastolic flow, wall shear stress (WSS) and in-plane rotational flow (IRF) were calculated at 8 equidistant planes in the distal descending aorta (DAo), from the pulmonary bifurcation to the diaphragmatic level, and averaged values were used [4]. Aortic stiffness in the FL was assessed in terms of pulse wave velocity (PWV), which was calculated from the third supraortic trunk to the diaphragmatic level on 4D-flow CMR [5]. The percentage of thrombus in the FL was calculated as the ratio of thrombus and FL volumes on MRA. Dominant entry tear area was quantified on the baseline CTA (Figure 1). Aortic growth rate (GR) was defined as the difference between final and baseline aortic diameters as measured on CTA divided by follow-up duration. Results Anatomical, haemodynamic and biomechanical parameters are shown in Table. Twenty-five patients have repaired type A AD with residual entry tear and 16 have type B AD. Mean follow-up duration was of 4.9±2.7 years. In bivariate analysis, WSS, IRF and PWV were positively related to GR, whereas dominant entry tear area and percentage of thrombus in the FL showed a positive tendency with GR (Table) (Figure). In multivariate analysis IRF, PWV, dominant entry tear area and thrombus in the FL were positively and independently associated with GR (Table). Retrograde systolic and diastolic flow were not related to GR while WSS tended to statistical significance. Conclusions In-plane rotational flow, regional aortic stiffness, dominant entry tear area and percentage of thrombus in the false lumen are positively and independently related to aortic growth rate in patients with chronic type B aortic dissection. Further prospective studies are needed to confirm if the assessment of these parameters may help to identify patients at higher risk of adverse clinical events. Funding Acknowledgement Type of funding sources: None. Table 1Figure 1

Background Mitral annular disjunction (MAD) is a structural abnormality of the mitral annular fibrosus characterized by a separation between the atrial wall-mitral valve junction, and the left ventricular attachment (1). It has been associated with mitral valve prolapse (MVP) (2) but also, with arrhythmias and sudden cardiac death (SCD) (3). There is no evidence of its prevalence and clinical significance in patients with syndromic hereditary aortopathies. Purpose To evaluate the prevalence of MAD, PMV, and the combination of both in patients with syndromic hereditary thoracic aortic disease (HTAD) including Marfan (MFS), Loeys-Dietz (LDS) and vascular Ehlers-Danlos syndromes (vEDS), and its relationship with arrhythmias, SCD, mitral regurgitation (MR) severity and the need for mitral surgery at the follow-up. Methods Adult patients with syndromic HTAD seen at our specialized unit were retrospectively included. The presence of MAD, MVP, and significant MR at first echocardiogram were evaluated. Electronic medical records were reviewed to register the occurrence of arrhythmic events and the need of mitral surgery. Last echocardiogram available was also assessed to evaluate MR progression. Results A total of 295 patients were included (235 MFS, 42 LDS and 18 vEDS). Mean age at baseline was 39.0±14.4 and 52.9% were female. MAD was present in 87 (37.0%) of MFS, 6 (14.3%) of LDS and was not present in vEDS (p In MFS, the presence of MAD was significantly associated with MVP (p≤0.001) (Table 1). However, 14 (6.0%) of patients had isolated MAD (Table 2). At baseline, significant MR was observed in 18 (24.7%) of patients with concurrent MAD and MVP and was not present in patients with isolated MAD (Table 2). MVP (OR 16.85 IC 4.43 – 64.07) but not MAD (p=0.607), was associated with significant MR in the multivariate analysis. A second echocardiogram was available in 220 patients at ≥1 year (mean 4.1±1.4 years). Overall, 25 (11.4%) presented significant progression of MR, 0 in the isolated MAD group, 13 (19.4%) in the MAD/MVP group and 6 (20.0%) of the isolated MVP (p=0.007). After a mean clinical follow-up of 7.5±3.2 years, 10 patients required mitral surgery (6 prosthesis, 4 valvuloplasty), 22 (9.4%) presented atrial fibrillation, flutter or supraventricular tachycardia (SVT), and 2 (0.9%) SCD. After adjustment for the presence of MVP and time of follow-up, MAD was not associated with progression of MR (p=0.529) need for mitral surgery (p=0.096), atrial fibrillation-flutter or SVT (p=0.510) nor SCD. (p=0.997). Conclusions The prevalence of MAD in syndromic HTAD is high, especially in Marfan syndrome, and absent in vEDS. In this retrospective observational study, the presence of MAD in Marfan was not associated with mitral regurgitation evolution or arrhythmic events. Funding Acknowledgement Type of funding sources: None. Characteristics of MFS patientsPresence of significant MR by groups.

Background Left ventricular noncompaction (LVNC) is associated with an increased risk of systemic embolisms (SE). However, incidence and risk factors are not well established. Purpose To evaluate the rate of SE in LVNC and describe risk factors. Methods LNVC patients were included in a multicentric registry. Those with SE were considered for the analysis. Results 514 patients with LVNC from 10 Spanish centres were recruited from 2000 to 2018. During a median follow-up of 4.2 years (IQR 1.9–7.1), 23 patients (4.5%) had a SE. Patients with SE (Table 1) were older at diagnosis, with no differences in gender and had similar cardiovascular risk factors. They were more frequently under oral anticoagulation (OAC). Besides, they had a more reduced LVEF, and more dilated LV and left atrium (LA). Late gadolinium enhancement (LGE) was more frequent, altogether suggesting a more severe phenotype. Patients with SE had non-significantly higher rates of hospitalization for heart failure (33% vs 24%, p=0.31) and atrial fibrillation (35% vs 19%, p=0.10). In multivariate analysis, only LA diameter was an independent predictor of SE (OR 1.04, p=0.04). A LA diameter>45 mm had an independent 3 fold increased risk of SE (OR 3.04, p=0.02) (Image 1). Table 1 Systemic embolisms (n=23) No systemic embolisms (n=491) p Men, n (%) 15 (65) 289 (56) 0.52 Median age at diagnosis (IQR), yr 60 (48–76) 48 (30–64) 0.02 Median follow up (IQR), yr 5.9 (3.1–7.8) 4.2 (1.8–7.1) 0.18 Hypertension, % 8 (33) 118 (24) 0.31 Diabetes mellitus, % 3 (14) 39 (8) 0.41 OAC, % 19 (83) 118 (24) 0.01 LVEF (SD), % 37 (15) 48 (17) 0.01 LVEDD (SD), mm 58 (11) 54 (10) 0.04 LVESD (SD), mm 45 (13) 38 (11) 0.01 LA diameter (SD), mm 46 (9) 39 (9) 0.01 LVEDV CMR (SD), mL 193 (75) 163 (70) 0.12 LVESV CMR (SD), mL 121 (64) 85 (64) 0.04 LGE, % 9 (40) 88 (18) 0.04 Conclusions LVNC carries a moderate mid-term risk of SE, which appears to be irrespective of atrial fibrillation and associated with age, LV dilatation and systolic dysfunction and mainly LA dilatation. This subgroup of patients should be considered for oral anticoagulation in primary prevention.

Background Late gadolinium enhancement (LGE) is the clinical reference standard for estimation of infarct extension and prediction of functional recovery following reperfused acute ST-segment elevation myocardial infarction (STEMI). Nevertheless, myocardial edema, microvascular obstruction and intramyocardial hemorrhage as well as the timing of image acquisition after contrast administration may influence the extent of LGE and underestimate the potential for recovery. Dobutamine stress testing has been recommended to more accurately predict functional recovery when infarct transmurality is between 25 to 75%. We hypothesized that cardiac magnetic resonance (CMR) tissue tracking strain analysis may provide additional value to LGE for the prediction of functional recovery. Methods In 370 patients with STEMI who underwent successful primary percutaneous revascularization and were studied with CMR within 3–5 days of the event, peak systolic longitudinal (LS), circumferential (CS) and radial (RS) strain were analyzed with routine SSFP images of 3 long-axis and a stack of short-axis slices (Tissue Tracking, CVI42®, Figure panel A and B). Inversion-recovery echogradient sequences were analyzed 20 minutes after contrast administration for LGE transmurality (Panel C). All per-segment analysis was performed according to the AHA 16-segment model. CMR was repeated at 6 months and functional recovery was defined as persistent normokinesia or improvement of wall motion score from baseline to 6-month CMR. Results At baseline CMR, of a total of 5920 segments 70.4% were normokinetic, 7.2% were hypokinetic, 21.9% were akinetic and 0.6% were dyskinetic. All strain parameters decreased significantly with worsening wall motion. At follow-up, 81.5% of the segments showed functional recovery. All strain parameters were significantly associated with functional recovery (p CS analysis in an inferior STEMI Conclusions Acute CMR tissue tracking strain analysis complements LGE assessment for prediction of functional recovery following an STEMI. The combination of LGE infarct transmurality under 50% and a CS strain higher than −10.7% showed the highest accuracy for prediction of recovery of function.