Aortic valve calcification (AVC), as measured by gold-standard computed tomography (CT) Agatston score, provides an anatomic assessment of aortic stenosis (AS) severity and is a key predictor of AS progression and need for valve replacement. AVC detection and quantification from transthoracic echocardiography (TTE) could expand AS early diagnosis and risk stratification, currently limited by CT availability and radiation exposure. A multi-view video-based deep learning framework was developed using 1166 TTE aortic valve videos from 187 TTE studies acquired in 110 patients with available AVC score by CT. EchoAVC architecture includes a feature extraction model followed by a quality-aware model that aggregates video-level information to obtain patient-level predictions for AVC detection and quantification (score). The framework was validated internally with 173 TTE studies from 86 patients across seven centres, and externally using 430 TTE studies from 280 patients across four different centres. The associations between EchoAVC estimations and AS severity, progression, and need for aortic valve replacement were examined. EchoAVC demonstrated excellent performance in AVC detection (AUROC 0.98, accuracy 94.4%), and quantification (R = 0.64) in the external multi-centre testing set. EchoAVC score was correlated with echocardiographic AS severity descriptors, including aortic valve mean pressure gradient (ρ = 0.749) and peak velocity (ρ = 0.757), and predicted future increase in mean pressure gradient (ρ = 0.382), peak velocity (ρ = 0.433) and calcium score by CT (ρ = 0.650). In 361 patients followed for a median of 3.8 years, 139 underwent aortic valve replacement. Baseline presence and extent of AVC as predicted by EchoAVC showed strong risk-stratification power for aortic valve replacement, remarkably in line with those obtained by CT, and incremental over TTE AS descriptors. EchoAVC was further tested in routine clinical practice images, confirming strong associations with AS severity and progression, including stratification for incident AS in previously unaffected individuals (p<0.001). EchoAVC enables accurate and non-invasive detection and quantification of AVC, offering substantial diagnostic and prognostic value for aortic stenosis progression and need for valve replacement. This technique holds promise as a scalable tool for early detection and clinical management of aortic valve stenosis. * Aortic valve calcification can be detected and quantified on standard, two-dimensional transthoracic echocardiography * EchoAVC score is associated with echocardiographic metrics of aortic stenosis severity * EchoAVC predicts progressive increase in aortic stenosis severity and need for aortic valve replacement * This openly available deep learning framework can be used to estimate aortic valve calcium in patients at risk of or presenting aortic stenosis to predict aortic stenosis progression * EchoAVC may help identify patients likely to have high aortic valve calcium and prioritize them for confirmatory CT assessment, thereby supporting earlier identification of calcific aortic valve disease in clinical pathways.

BACKGROUND:Ascending aorta dilation and aortic valve degeneration are common complications in patients with bicuspid aortic valve. Several retrospective studies have suggested the benefit of statins in reducing these complications. This study aimed to determine whether atorvastatin treatment is effective in reducing the growth of aortic diameters in bicuspid aortic valve and if it slows the progression of valve calcification. METHODS:In a randomized clinical trial, 220 patients with bicuspid aortic valve (43 women; 46±13 years of age) were included and treated with either 20 mg of atorvastatin per day or placebo for 3 years. Inclusion criteria were ≥18 years of age, nonsevere valvular dysfunction, nonsevere valve calcification, and ascending aorta diameter ≤50 mm. Computed tomography and echocardiography studies were performed at baseline and after 3 years of treatment. RESULTS:During follow-up, 28 patients (12.7%) discontinued medical treatment (15 on atorvastatin and 13 taking placebo). Thus, 192 patients completed the 36 months of treatment. Low-density lipoprotein cholesterol levels decreased significantly in the atorvastatin group (median [interquartile range], -30 mg/dL [-51.65 to -1.75 mg/dL] versus 6 mg/dL [-4, 22.5 mg/dL]; P<0.001). The maximum ascending aorta diameter increased with no differences between groups: 0.65 mm (95% CI, 0.45-0.85) in the atorvastatin group and 0.74 mm (95% CI, 0.45-1.04) in the placebo group (P=0.613). Similarly, no significant differences were found for the progression of the aortic valve calcium score (P=0.167) or valvular dysfunction. CONCLUSIONS:Among patients with bicuspid aortic valve without severe valvular dysfunction, atorvastatin treatment was not effective in reducing the progression of ascending aorta dilation and aortic valve calcification during 3 years of treatment despite a significant reduction in low-density lipoprotein cholesterol levels. REGISTRATION:URL: https://www.clinicaltrialsregister.eu; Unique identifier: 2015-001808-57. URL: https://www.clinicaltrials.gov; Unique identifier: NCT02679261.

AIMS:To assess the progression of the disease and evolution of the main echocardiographic variables for quantifying AS in patients with severe low-flow low-gradient (LFLG) AS compared to other severe AS subtypes. METHODS AND RESULTS:Longitudinal, observational, multicenter study including consecutive asymptomatic patients with severe AS (aortic valve area, AVA < 1.0 cm²) and normal left ventricle ejection fraction (LVEF ≥ 50%). Patients were classified according to baseline echocardiography into: HG (high gradient; mean gradient ≥ 40 mmHg), NFLG (normal-flow low-gradient; mean gradient < 40 mmHg, indexed systolic volume (SVi) > 35mL/m2), or LFLG (mean gradient < 40 mmHg, SVi ≤ 35 mL/m²). AS progression was analyzed by comparing patients' baseline measurements and their last follow-up measurements or those taken prior to aortic valve replacement (AVR). Of the 903 included patients, 401 (44.4%) were HG, 405 (44.9%) NFLG, and 97 (10.7%) LFLG. Progression of the mean gradient in a linear mixed regression model was greater in low-gradient groups: LFLG vs. HG (regression coefficient 0.124, P = 0.005) and NFLG vs. HG (regression coefficient 0.068, P = 0.018). No differences were observed between the LFLG and NFLG groups (regression coefficient 0.056, P = 0.195). However, AVA reduction was slower in the LFLG group compared to the NFLG (P < 0.001). During follow-up, in conservatively-managed patients, 19.1% (n = 9) of LFLG patients evolved to having NFLG AS and 44.7% (n = 21) to having HG AS. In patients undergoing AVR, 58.0% (n = 29) of LFLG baseline patients received AVR with a HG AS. CONCLUSION:LFLG AS shows an intermediate AVA and gradient progression compared to NFLG and HG AS. The majority of patients initially classified as having LFLG AS changed over time to having other severe forms of AS, and most of them received AVR with a HG AS.

The 2020 ESC atrial fibrillation (AF) guidelines suggest the novel 4S-AF scheme for the characterization of AF. Imaging techniques could be helpful for this objective in everyday clinical practice, and information derived from these techniques reflects basic aspects of the pathophysiology of AF, which may facilitate treatment decision-making, and optimal management of AF patients. The aim of this review is to provide an overview of the mechanisms associated with atrial fi-brosis and to describe imaging techniques that may help the management of AF patients in clinical practice. Transthoracic echocardiography is the most common procedure given its versatility, safety, and simplicity. Transesophageal echocardiogra-phy provides higher resolution exploration, and speckle tracking echocardiography can provide incremental functional and prognostic information over conventional echocardiographic parameters. In addition, LA deformation imaging, including LA strain and strain rate, are related to the extent of fibrosis. On the other hand, multidetector-row computed tomography and cardiac magnetic resonance provide higher resolution data and more accurate assessment of the dimensions, structure, and spatial relationships of the LA. Imaging is central when deciding on catheter ablation or cardioversion, and helps in selecting those patients who will really benefit from these procedures. Moreover, imaging enhances the understanding of the underlying mechanisms of atrial remodeling and might assists in refining the risk of stroke, which help to select the best medical therapies/interventions.In summary, evaluation of LA enlargement, LA remodeling and fibrosis with imaging techniques adds clinical and prognostic information and should be assessed as a part of routine comprehensive AF evaluation.

The aim of the present investigation is to study the relationship of ventricular global longitudinal strain (GLS) and ultrasound lung comets (ULC) formation to establish a link between extravascular pulmonary water formation and cardiac contractile dysfunction. This is a prospective observational study including 14 active military divers. The subjects performed two sea dives of 120 min each with a semi-closed SCUBA circuit at 10 m depth. Divers were examined at baseline, 15 min (D1) and 60 min (D2) after diving. The evaluation included pulmonary and cardiac echography (including speckle tracking techniques). Blood samples were drawn at baseline and after diving, assessing hs-TnT and Endothelin-1. ULC were detected in 9 (64.2%) and 8 (57.1%) of the subjects after D1 and D2 respectively. No differences were found in right and left ventricular GLS after both immersions (RV: Baseline:  – 17.9 4.9 vs. D1:  – 17.2 6.5 and D2:  – 16.7 5.8 s−1; p = 0.757 and p = 0.529; LV: Baseline:  – 17.0 2.3 vs. D1:  – 17.4 2.1 and D2:  – 16.9 2.2 s−1; p = 0.546 and p = 0.783). However, a decrease in atrial longitudinal strain parameters have been detected after diving (RA: Baseline: 35.5 9.2 vs. D1: 30.3 12.8 and D2: 30.7 13.0 s−1; p = 0.088 and p = 0.063; LA: Baseline: 39.0 10.0 vs. D1: 31.6 6.1 and D2: 32.4 10.6 s−1; p = 0.019 and p = 0.054). In the present study, no ventricular contractile dysfunction was observed. However, increase pulmonary vasoconstriction markers were present after diving.

ObjectivesTo determine the risk of mortality and need for aortic valve replacement (AVR) in patients with low-flow low-gradient (LFLG) aortic stenosis (AS).MethodsA longitudinal multicentre study including consecutive patients with severe AS (aortic valve area [AVA] < 1.0 cm2) and normal left ventricular ejection fraction (LVEF). Patients were classified as: high-gradient (HG, mean gradient ≥ 40 mmHg), normal-flow low-gradient (NFLG, mean gradient < 40 mmHg, indexed systolic volume (SVi) > 35 ml/m2) and LFLG (mean gradient < 40 mmHg, SVi ≤ 35 ml/m2).ResultsOf 1,391 patients, 147 (10.5%) had LFLG, 752 (54.1%) HG, and 492 (35.4%) NFLG. Echocardiographic parameters of the LFLG group showed similar AVA to the HG group but with less severity in the dimensionless index, calcification, and hypertrophy. The HG group required AVR earlier than NFLG (p < 0.001) and LFLG (p < 0.001), with no differences between LFLG and NFLG groups (p = 0.358). Overall mortality was 27.7% (CI 95% 25.3–30.1) with no differences among groups (p = 0.319). The impact of AVR in terms of overall mortality reduction was observed the most in patients with HG (hazard ratio [HR]: 0.17; 95% CI: 0.12–0.23; p < 0.001), followed by patients with LFLG (HR: 0.25; 95% CI: 0.13–0.49; p < 0.001), and finally patients with NFLG (HR: 0.29; 95% CI: 0.20–0.44; p < 0.001), with a risk reduction of 84, 75, and 71%, respectively.ConclusionsParadoxical LFLG AS affects 10.5% of severe AS, and has a lower need for AVR than the HG group and similar to the NFLG group, with no differences in mortality. AVR had a lower impact on LFLG AS compared with HG AS. Therefore, the findings of the present study showed LFLG AS to have an intermediate clinical risk profile between the HG and NFHG groups.

OBJECTIVES The Edwards Intuity valve is a rapid deployment aortic prosthesis that favours less invasive approaches. However, evidence about the clinical behaviour of their smaller sizes is scarce. Herein, we studied haemodynamic behaviours and clinical outcomes of small Intuity prostheses (19-21 mm) in comparison to larger Intuity prostheses (>21 mm). METHODS This is an observational study including patients implanted with an Edwards Intuity rapid deployment aortic prosthesis. Patients with prosthesis sizes 19-21 and >21 mm were included. Baseline and perioperative variables, as well as adverse events during the follow-up were recorded and compared between groups. RESULTS A total of 122 patients (37% female, mean age 75 ± 4.5 years) were included, of whom 54 (45%) were implanted with a small prosthesis and 68 (55%) with a prosthesis >21 mm. There were no significant differences between patients with small Intuity prostheses and patients with larger prostheses regarding in-hospital mortality (2% vs 4%, P = 0.43) or mortality during the follow-up (3.41 vs 2.45 per 100 patients-years; P = 0.58). Survival in the small Intuity valve group was 95% at 1 year and 83% at 6 years, whereas in the larger Intuity valve group was 96% at 1 year and 78% at 6 years. The presence of a small prosthesis did not influence mid-term survival (log-rank P-value = 0.62). CONCLUSIONS This study showed good clinical performance of Intuity aortic prostheses with appropriate mid-term survival in patients with the small aortic annulus. Thus, the Edwards Intuity rapid deployment aortic prosthesis may be considered as a potential option in patients with the small aortic annulus.

Abstract Background and objectives Low-flow low-gradient (LFLG) aortic stenosis portends bad prognosis in different series. The objective of this study was to evaluate the evolution of this entity in our country. Methods We included 1394 consecutive patients evaluated between 2008-2016 with severe AS (AVA <1 cm²) and ejection fraction> 50% from 14 Spanish centres. The results (aortic valve intervention and mortality) were compared using the Kaplan-Meier survival analysis. Results Three groups based on gradient and flow status were established (high gradient: HG, normal flow under gradient: NFLG, low gradient low flow: LFLG). No significant demographic or clinical differences between groups were observed. After a follow-up of 61.52 months (IQR 43.5-86.5), 551 (73.8%) HG, 268 (35.4%) with NFLG and 81 (57.9%) LFLG received intervention, with a later surgery/TAVI indication in the LFLG group compared with HG group (p = 0.001) (Figure 1). The analysis of the Kaplan-Meier mortality curves showed no significant differences. Conclusions Patients with LFLG aortic stenosis with normal ejection fraction received less and later aortic valve intervention than the HG group with no significant differences in mortality. Abstract P754 Figure. Time to surgery

Los avances en el campo de la imagen cardiovascular han seguido aumentando de forma exponencial en este último año. Las técnicas de imagen cardiaca siguen buscando el origen fisiopatológico de un gran número de enfermedades cardiovasculares para determinar nuevas dianas terapéuticas, o anticiparse a la enfermedad detectando la alteración miocárdica y ateroesclerótica subclínica. El amplio desarrollo de las nuevas técnicas de imagen cardiaca ha permitido extender su uso en cada vez más sustratos patológicos: la deformación miocárdica se establece como un factor pronóstico de utilidad en pacientes con cáncer, enfermedad valvular o insuficiencia cardiaca; las secuencias avanzadas de cardiorresonancia se muestran prometedoras para el estudio de las aortopatías, miocardiopatías y cardiopatías congénitas, e incluso en un futuro próximo puede que la inteligencia artificial resuelva dudas y facilite las tareas diarias de los laboratorios de imagen cardiaca de nuestro entorno. Sin duda, uno de los campos más destacados es el intervencionismo estructural percutáneo, que evoluciona de forma imparable, con el objetivo de extenderse a una población cada vez menos restringida y anticiparse, en ciertas ocasiones, al acto quirúrgico en pacientes de alto riesgo.

Objective Bicuspid aortic valve (BAV) is the most common congenital heart disease. This study aimed to determine the prevalence rate of BAV in first-degree relatives (FDR) and the inheritance pattern according to different morphotypes and aortic dilation. Methods BAV probands were consecutively studied at eight tertiary referral centres. After sequential screening, FDR were included in the study. The BAV morphotype, aortic dilation and aortic phenotype were assessed by transthoracic echocardiography. Results Seven hundred and twenty-four FDR of 256 BAV probands agreed to undergo family screening. The prevalence of BAV was 6.4% in FDR (9.2% in men, 3.5% in women, p=0.002). Aortic dilation was diagnosed in 9.6% of FRD with tricuspid aortic valves (TAV), with a root phenotype in 2.7% and tubular in 6.9% and more frequently in the presence of arterial hypertension (OR 4.48; CI 95% 2.51 to 7.99; p=0.0001) and valvular regurgitation (OR 5.87, CI 95% 1.37 to 25.16; p=0.025). The heritability (h2 ) of BAV was highly significant (0.47; p=0.002); however, no concordance was observed among valve morphotypes. Aortic dilation heritability was not significant. Conclusions The BAV prevalence rate in FDR was low (6.4%) but aortic dilation was observed in 9.6% of FDR with TAV. The heritability of BAV was high without concordance in valve morphotypes, and aortic dilation heritability was not observed. Patients with BAV should be made aware of its familial pattern.

425 consecutive patients underwent a surgical AVR for isolated AS with a biological or mechanical prosthesis: BAV = 135 patients (31.8%),TAV = 290 patients (68.2%).Exclusion criteria included coronary artery disease and associated significant valvulopathy.Patients who underwent the Ross procedure (national referrals) or a transcatheter AVR (program started in 2010) were also excluded to avoid any selection bias.Results: BAV patients were younger than TAV patients (61 years vs 75 years, p<0.001).Mean gradient was higher in BAV vs TAV (54 mmHg vs 50 mmHg respectively, p=0.049).At the time of surgery, the proportion of patients with decreased LVEF (<45%) was higher in the BAV group compared to the TAV group: 16.3% (22/135) vs 8.9% (26/290) respectively, p=0.026.LV dilatation was also more frequent in the BAV group (22.3% vs 14%, p=0.037).The overall early mortality rate after AVR was 3.7% (16/425), without significant difference between the 2 groups. Conclusion:Our study shows that patients with stenotic BAV are at risk of impaired LV systolic function before surgical AVR compared to TAV patients, despite a younger age and a similar clinical tolerance.This adds to the fact that BAV is associated with an intrinsic cardiomyopathy.Further investigations are needed to determine more accurate criteria for the indication of AVR in this population.

Objective Bicuspid aortic valve (BAV) is associated with early valvular dysfunction and proximal aorta dilation with high heterogeneity. This study aimed to assess the determinants of these complications. Methods Eight hundred and fifty-two consecutive adults diagnosed of BAV referred from cardiac outpatient clinics to eight echocardiographic laboratories of tertiary hospitals were prospectively recruited. Exclusion criteria were aortic coarctation, other congenital disorders or intervention. BAV morphotype, significant valve dysfunction and aorta dilation (≥2 Z-score) at sinuses and ascending aorta were established. Results Three BAV morphotypes were identified: right–left coronary cusp fusion (RL) in 72.9%, right–non-coronary (RN) in 24.1% and left–non-coronary (LN) in 3.0%. BAV without raphe was observed in 18.3%. Multivariate analysis showed aortic regurgitation (23%) to be related to male sex (OR: 2.80, p<0.0001) and valve prolapse (OR: 5.16, p<0.0001), and aortic stenosis (22%) to BAV-RN (OR: 2.09, p<0.001), the presence of raphe (OR: 2.75, p<0.001), age (OR: 1.03; p<0.001), dyslipidaemia (OR: 1.77, p<0.01) and smoking (OR: 1.63, p<0.05). Ascending aorta was dilated in 76% without differences among morphotypes and associated with significant valvular dysfunction. By contrast, aortic root was dilated in 34% and related to male sex and aortic regurgitation but was less frequent in aortic stenosis and BAV-RN. Conclusions Normofunctional valves are more prevalent in BAV without raphe. Aortic stenosis is more frequent in BAV-RN and associated with some cardiovascular risk factors, whereas aortic regurgitation (AR) is associated with male sex and sigmoid prolapse. Although ascending aorta is the most commonly dilated segment, aortic root dilation is present in one-third of patients and associated with AR. Remarkably, BAV-RL increases the risk for dilation of the proximal aorta, whereas BAV-RN spares this area.

There is a crescent interest on normal adult echocardiographic values and the introduction of new deformation imaging and 3D parameters pose the issue of normative data. A multitude of nomograms has been recently published, however data are often fragmentary, difficult to find, and their strengths/limitations have been never evaluated.AIMS:(I) to provide a review of current echocardiographic nomograms; (II) to generate a tool for easy and fast access to these data. A literature search was conducted accessing the National Library of Medicine using the keywords: 2D/3D echocardiography, strain, left/right ventricle, atrial, mitral/tricuspid valve, aorta, reference values/nomograms/normal values. Adding the following keywords, the results were further refined: range/intervals, myocardial velocity, strain rate and speckle tracking. Forty one published studies were included. Our study reveals that for several of 2D/3D parameters sufficient normative data exist, however, a few limitations still persist. For some basic parameters (i.e., mitral/tricuspid/pulmonary valves, great vessels) and for 3D valves data are scarce. There is a lack of studies evaluating ethnic differences. Data have been generally expressed as mean values normalised for gender and age instead of computing models incorporating different variables (age/gender/body sizes) to calculate z scores. To summarize results a software (Echocardio-Normal Values) who automatically calculate range of normality for a broad range of echocardiographic measurements according to age/gender/weight/height, has been generated. We provide an up-to-date and critical review of strengths/limitation of current adult echocardiographic nomograms. Furthermore we generated a software for automatic, easy and fast access to multiple echocardiographic normative data.

Las mutaciones en MYBPC3 son causa de miocardiopatía hipertrófica (MCH). A pesar de que la mayoría de ellas producen una proteína truncada, la gravedad del fenotipo es diversa. Se describe el fenotipo clínico de una nueva mutación en MYBPC3, p.Pro108Alafs*9, presente en 13 familias del sur de España, y se compara con la mutación de MYBPC3 con mayor prevalencia en dicha región (c.2308 + 1 G > A). Se estudió a 107 familiares de 13 casos índice que tenían diagnóstico de MCH y portaban la mutación p.Pro108Alafs*9. Se realizó un análisis del árbol genealógico, junto con una evaluación clínica y determinación del genotipo. Se identificó en total a 54 portadores de la mutación p.Pro108Alafs*9, de los que 39 tenían MCH. Hubo 5 casos de muerte súbita en las 13 familias. La penetrancia de la enfermedad aumentaba a medida que se incrementaba la edad, y los pacientes con MCH fueron con más frecuencia varones, y estos contrajeron la enfermedad más precozmente que las mujeres. El fenotipo fue similar en la p.Pro108Alafs*9 y la c.2308 + 1 G > A, pero se observaron diferencias en varios factores de riesgo y en la supervivencia. Hubo tendencia a mayor masa ventricular izquierda en la p.Pro108Alafs*9 que en la c.2308 + 1G > A. La resonancia magnética cardiaca reveló una extensión y un patrón de fibrosis similares en ambas. La mutación p.Pro108Alafs*9 se asoció a MCH, alta penetrancia y aparición de la enfermedad a mediana edad. Mutations in MYBPC3 are the cause of hypertrophic cardiomyopathy (HCM). Although most lead to a truncating protein, the severity of the phenotype differs. We describe the clinical phenotype of a novel MYBPC3 mutation, p.Pro108Alafs*9, present in 13 families from southern Spain and compare it with the most prevalent MYBPC3 mutation in this region (c.2308 + 1 G > A). We studied 107 relatives of 13 index cases diagnosed as HCM carriers of the p.Pro108Alafs*9 mutation. Pedigree analysis, clinical evaluation, and genotyping were performed. A total of 54 carriers of p.Pro108Alafs*9 were identified, of whom 39 had HCM. There were 5 cases of sudden death in the 13 families. Disease penetrance was greater as age increased and HCM patients were more frequently male and developed disease earlier than female patients. The phenotype was similar in p.Pro108Alafs*9 and in c.2308 + 1 G > A, but differences were found in several risk factors and in survival. There was a trend toward a higher left ventricular mass in p.Pro108Alafs*9 vs c.2308 + 1G > A. Cardiac magnetic resonance revealed a similar extent and pattern of fibrosis. The p.Pro108Alafs*9 mutation is associated with HCM, high penetrance, and disease onset in middle age. Full English text available from: www.revespcardiol.org/en

En el presente documento se sintetizan el concepto de ecocardioscopia, los aspectos técnicos fundamentales relacionados con esta técnica y sus objetivos diagnósticos. Además, se define cómo deben ser la formación y la capacitación en ecocardioscopia. Todo ello, avalado por la Sociedad Española de Medicina Interna (SEMI), la Sociedad Española de Medicina Familiar y Comunitaria (semFYC), la Sociedad Española de Neurología (SEN) y la Sociedad Española de Cardiología (SEC).This document summarizes the concept of focused cardiac ultrasound, the basic technical aspects related to this technique, and its diagnostic objectives. It also defines training requisites in focused cardiac ultrasound. This consensus document has been endorsed by the Spanish Society of Internal Medicine (SEMI), the Spanish Society of Family and Community Medicine (semFYC), the Spanish Society of Neurology (SEN), and the Spanish Society of Cardiology (SEC).Full English text available from: www.revespcardiol.org/en