Unequivocal interpretation of dobutamine stress echocardiography in low-flow, low-gradient aortic stenosis by right parasternal view.

We present the case of a 77-years-old man with aortic valve stenosis (AS) and reduced left ventricular ejection fraction, in whom right parasternal view provided the best hemodynamic evaluation of AS severity during dobutamine stress echocardiography.

The Heart Muscle and Valve Involvement in Marfan Syndrome, Loeys-Dietz Syndromes, and Collagenopathies.

The inherited connective tissue disorders (Marfan syndrome, Loeys-Dietz syndrome [LDS], and Ehlers-Danlos syndrome [EDS]) involve connective tissue of various organ systems. These pathologies share many common features, nonetheless compared to Marfan syndrome, LDS' cardiovascular manifestations tend to be more severe. In contrast, no association is reported between LDS and the presence of ectopia lentis. The EDS are currently classified into thirteen subtypes. There is substantial symptoms overlap between the EDS subtypes, and they are associated with an increased incidence of cardiovascular abnormalities, such as mitral valve prolapse and aortic dissection.

Clinical impact of mitral regurgitation in aortic valve stenosis: Insight from effective regurgitant orifice area.

PURPOSE: Mechanisms leading to heart failure (HF) symptoms in aortic valve stenosis (AS) are contentious. We examined the impact of secondary mitral regurgitation (MR) on the symptomatic status in patients with AS. METHODS: Outpatients performing echocardiography with any degree of AS, without organic mitral valve disease, mitral valve intervention, or aortic insufficiency were enrolled. MR was quantitatively defined through mitral effective regurgitant orifice area (EROA) using the proximal isovelocity surface area method. Patients were divided into two groups (New York Heart Association [NYHA] class I-II vs. NYHA class III-IV). RESULTS: Five hundred and eighty-four patients were enrolled (484 NYHA I-II, 100 NYHA III-IV). More symptomatic patients had smaller aortic valve area (AVA), lower left ventricular ejection fraction (LVEF) and stroke volume, higher E/E', and LV global afterload. MR was present in 178 (30%) patients and EROA was <.20 cm2 in 158 (89%). NYHA III-IV patients showed higher prevalence of MR (78% vs 21%, P < 0.0001) and larger EROA (.13±.08 cm2 vs .09±.07 cm2 , P < 0.0001). An association between EROA and symptoms was present in the total cohort and in subgroups with preserved LVEF, AVA ≥ 1 and <1 cm2 , EE' 8-14 and ≥14 (P < 0.05 for all). EROA was associated with severe symptoms after adjustment for LVEF, E/E', and AVA in the overall population (OR 1.10 [1.06-1.15]; P < 0.0001) and in the 516 patients with preserved LVEF (OR 1.13 [1.08-1.19]; P < 0.0001). CONCLUSION: In patients with AS, greater EROA values are associated with HF symptoms, even though MR degree is far from the threshold of MR severity. Therefore, even a mild MR represents a supportive marker of HF symptoms presence.

Impact of Regular Physical Activity on Aortic Diameter Progression in Paediatric Patients with Bicuspid Aortic Valve.

Patients with bicuspid aortic valve (BAV) have an increased risk of aortic dilation and aortic dissection or rupture. The impact of physical training on the natural course of aortopathy in BAV patients remains unclear. The aim of this study was to evaluate the impact of regular physical activity on aortic diameters in a consecutive cohort of paediatric patients with BAV. Consecutive paediatric BAV patients were evaluated and categorized into two groups: physically active and sedentary subjects. Only the subjects with a complete 2-year follow-up were included in the study. To evaluate the potential impact of physical activity on aortic size, aortic diameters were measured at the sinus of Valsalva and mid-ascending aorta using echocardiography. We defined aortic diameter progression the increase of aortic diameter ≥ 10% from baseline. Among 90 BAV patients (11.5 ± 3.4 years of age, 77% males), 53 (59%) were physically active subjects. Compared to sedentary, physically active subjects were not significantly more likely to have > 10% increase in sinus of Valsalva (13% vs. 8%, p-value = 0.45) or mid-ascending aorta diameter (9% vs. 13%, p-value = 0.55) at 2 years follow-up, both in subjects with sinus of Valsalva diameter progression (3.7 ± 1.0 mm vs. 3.5 ± 0.8 mm, p-value = 0.67) and in those with ascending aorta diameter progression (3.0 ± 0.8 mm vs. 3.2 ± 1.3 mm, p-value = 0.83). In our paediatric cohort of BAV patients, the prevalence and the degree of aortic diameter progression was not significantly different between physically active and sedentary subjects, suggesting that aortic dilation is unrelated to regular physical activity over a 2-year period.

Prevalence, clinical correlates, and burden of undiagnosed aortic stenosis in older patients: a prospective study in a non-cardiologic acute hospital ward.

BACKGROUND: The epidemiology of aortic stenosis (AS) in older patients admitted to non-cardiologic acute hospital wards and the effect of AS on mid-term survival are incompletely reported. In a cohort of very old patients admitted to an acute geriatric unit (AGU), we aimed to assess: (1) the prevalence of newly and previously diagnosed AS; and (2) the association between AS severity and patients' 6-month mortality. METHODS: The patients consecutively admitted in two AGU rooms from February 2016 to February 2018 were assessed with echocardiography and AS severity was defined according to standard criteria. We assessed frailty using a 34-item Frailty Index (34-FI), which was operationalized using health variable information, and the Clinical Frailty Scale (CFS). Vital status at 6 months was extracted from Regional Register of Birth and Death. RESULTS: Two hundred and three patients (mean age 84.5 ± 6.0 SD, female gender 56.1%) were included. Of these, 57 (28.1%) had AS, mild in 9 (4.5%), moderate in 32 (16.1%) and severe in 16 (8.1%). A new diagnosis of AS was obtained in 42 (73.7%) patients, of whom 33 (78.6%) had moderate or severe AS. At 6 months, 61 (28.9%) patients died. In multiple regression models, after adjusting for covariates, frailty, as assessed with both FI and CFS, was independent predictor of 6-month mortality whereas AS was not. CONCLUSIONS: Among older patients admitted to non-cardiologic acute hospital wards, AS was common and frequently underdiagnosed. The severity of AS was not associated with increased 6-month mortality, whereas frailty was the most important predictor.

Clinical significance of family history and bicuspid aortic valve in children and young adult patients with Marfan syndrome.

BACKGROUND: Marfan syndrome is an autosomal dominant disorder of the connective tissue, whose cardinal features affect eyes, musculoskeletal, and cardiovascular system. Despite prevalence and natural history of cardiovascular manifestation are well known in adults, little is known about children and young adult patients. The aim of this study was to describe a well-characterised cohort of consecutive children and young patients with marfan syndrome, looking at the impact of family history and presence of bicuspid aortic valve on disease severity. METHODS: A total of 30 consecutive children and young patients with Marfan syndrome were evaluated. All patients underwent a comprehensive clinical-instrumental-genetic evaluation. Particular attention was posed to identify differences in prevalence of cardiovascular abnormalities between patients with and without family history of Marfan syndrome or bicuspid aortic valve. RESULTS: Of these 30 patients, family history of Marfan syndrome and bicuspid aortic valve were present in 76 and 13%, respectively. Compared to patients with family history of Marfan syndrome, those without showed higher prevalence of aortic sinus dilation (87 versus 32%, p-value = 0.009), greater aortic sinus diameters (4.2 ± 2.1 versus 1.9 ± 1.1 z score, p-value = 0.002), and higher rate of aortic surgery during follow-up (37 versus 0%, p-value = 0.002). Compared to patients with tricuspid aortic valve, those with bicuspid aortic valve were younger (3.2 ± 4.3 versus 10.7 ± 6.8 years old, p-value = 0.043), showed greater aortic sinus diameters (4.2 ± 0.9 versus 2.2 ± 1.6 z score, p-value = 0.033), and underwent more frequently aortic root replacement (50 versus 4%, p-value = 0.004). CONCLUSIONS: In our cohort of patients with Marfan syndrome, the absence of family history and the presence of bicuspid aortic valve were associated to severe aortic phenotype and worse prognosis.

Awareness and appropriateness of the management of preclinical heart failure in outpatient clinics in Italy: Insights from the VASTISSIMO study - EValuation of the AppropriateneSs of The preclInical phase (Stage A and Stage B) of Heart FaIlure Management in Outpatient Clinics in Italy.

A key factor in cardiovascular prevention is the detection and appropriate management of preclinical heart failure (HF), but information on the subject is scarce. We designed VASTISSIMO as a prospective, observational study to investigate Outpatient Clinic Cardiologists' skills in detecting and managing preclinical HF in Italy. Quality scores were used to assess the appropriateness of clinical management according to guideline recommendations. The feasibility of making a diagnosis of preclinical HF in a cardiology outpatient clinical setting, cardiologists' awareness of preclinical HF and consistency between physician's perceived risk of HF and the patient's classification into the preclinical HF Stages A [(SAHF) or B (SBHF)] have been investigated. Consistency was defined acceptable if the concordance between perceived risk and actual risk was >70%. Out of 3322 patients included in the study data necessary for identifying SBHF were collected in 2106 (63.4%). Many SBHF patients had their risk underestimated: 16.2% of those with previous acute myocardial infarction (AMI), 23.1% with left ventricular hypertrophy (LVH) at ECG/echocardiography, 30% with systolic/diastolic dysfunction, and 14.3% with valve disease. Cardiologists' awareness of preclinical HF in the outpatient setting should be improved. This is a critical area of cardiovascular prevention that requires attention to improve good clinical practice and adherence to guidelines.

Role of TGFBR1 and TGFBR2 genetic variants in Marfan syndrome.

OBJECTIVE: Genetic variants in transforming growth factor beta (TGF-ß) receptors type 1 (TGFBR1) and type 2 (TGFBR2) genes have been associated with different hereditary connective tissue disorders sharing thoracic aortic aneurysm and dissection (TAA/D). Mutations in both TGFBR1/2 genes have been described in patients with TAA/D and Marfan syndrome (MFS), and they are associated consistently with Loeys-Dietz syndrome. The existing literature shows discordant data resulting from mutational screening of TGFBR1/2 genes in patients with MFS. The aim of the study was to investigate the role of TGFBR1/2 genetic variants in determining and/or modulating MFS clinical phenotype. METHODS: We investigated 75 unrelated patients with MFS referred to the Center for Marfan Syndrome and Related Disorders (Careggi University Hospital, Florence) who were subjected to FBN1 and TGFBR1/2 Sanger mutational screening. RESULTS: Forty-seven patients with MFS (63%) carried a pathogenetic FBN1 mutation. No pathogenetic mutations were detected in TGFBR1/2 genes. Ten common polymorphisms were identified in TGFBR2 and 6 in TGFBR1. Their association with cardiovascular manifestations was evaluated. Carriers of the A allele of rs11466512, delA allele of c.383delA or delT allele of c.1256-15del1T polymorphisms had a trend toward or significantly reduced z-scores (median [interquartile range (IQR)], 2.2 [1.13-4.77]; 2.1 [1.72-3.48]; 2.5 [1.85-3.86]) with respect to homozygous patients with wild-type MFS (median [IQR], 4.20 [2.39-7.25]; 3.9 [2.19-7.00]; 3.9 [2.14-6.93]). Carriers of the A allele of the rs2276767 polymorphism showed a trend toward increased z-score (median [IQR], 4.9 [2.14-7.16]) with respect to patients with wild-type MFS (median [IQR], 3.3 [1.75-5.45]). The protective effect of TGFBR1/2 genetic score including all the 4 variants was also evaluated. Patients with MFS with two or more protective alleles included in the score had statistically significant reduced aortic z-scores (median [IQR], 2.20 [1.48-3.37]) with respect to patients with 1 or no protective alleles (median [IQR], 4.20 [2.48-7.12]; P = .007). Patients with severe aortic manifestations (aortic z-score ≥ 2 or aortic surgery) showed a significantly lower prevalence of subjects with two or more protective alleles included in the genetic score (29.7%) than patients with no or milder cardiovascular involvement (63.6%; P = .029). The genetic score protective effect on global aortic manifestations severity (aortic z-score ≥ 2 or aortic surgery) was also observed at the logistic regression analysis adjusted for the presence of FBN1 gene mutations (odds ratio, 0.21; 95% CI, 0.05-0.84; P = .028). CONCLUSIONS: In conclusion, our data reappraise the role of TGFBR1 and TGFBR2 as major genes in patients with MFS, and suggest that TGFBR1/2 genetic variants (in particular when evaluated as a burden by score) might play a role in modulating the severity of cardiovascular manifestation in MFS.

Echocardiographic advances in hypertrophic cardiomyopathy: Three-dimensional and strain imaging echocardiography.

In the recent past, new ultrasound technologies, such as three-dimensional echocardiography and strain imaging echocardiography, raised up in clinical practice leading to a better assessment of cardiac morphology and performance. These tools may assess regional cardiac mechanics, detecting clinical and subclinical myocardial dysfunction in different settings such as ischemic heart disease, cardiomyopathies, and heart valve diseases. Interesting results derive from patients affected from hypertrophic cardiomyopathy (HCM). Particularly, the mentioned techniques are progressively redefining the role of echocardiography in diagnostic evaluation of HCM variants such as apical HCM, detection of the underlying conditions of increased wall thickness, assessment of subclinical myocardial impairment, and potentially refine risk stratification and prognosis. In this review, we describe the clinical uses of these methodologies and the perspective application in HCM patients.

Echocardiographic assessment of left ventricular systolic function: from ejection fraction to torsion.

Assessment of left ventricular (LV) systolic function is the cornerstone of the echocardiographic examination. There are many echocardiographic parameters that can be used for clinical and research purposes, each one with its pros and cons. The LV ejection fraction is the most used one due to its feasibility and predictability, but it also has many limits, related to both the imaging technique used for calculation and to the definition itself. LV longitudinal function is expression of subendocardial fibers contraction. Because the subendocardium is often involved early in many pathological processes, its analysis has been a fertile field for the development of sensitive parameters. Longitudinal function can be evaluated in many ways, such as M-mode echocardiography, tissue Doppler imaging, and speckle tracking echocardiography. This latter is a relatively new tool to assess LV function through measurement of myocardial strain, with a high temporal and spatial resolution and a better inter- and intra-observer reproducibility compared to Doppler strain. It is angle independent, not affected by translation cardiac movements, and can assess simultaneously the entire myocardium along all the three-dimensional geometrical (longitudinal, circumferential, and radial) axes. Speckle tracking echocardiography also allows the analysis of LV torsion. The aim of this paper was to review the main echocardiographic parameters of LV systolic function and to describe its pros and cons.

Value of Combined Circumferential and Longitudinal Left Ventricular Systolic Dysfunction to Predict Adverse Outcome in Patients with Asymptomatic Aortic Stenosis.

BACKGROUND AND AIM OF THE STUDY: Patients with asymptomatic aortic stenosis (AS) may have left ventricular systolic dysfunction (LVSD) defined as an impairment of the circumferential and/or longitudinal (C&L) myocardial fibers, despite a preserved left ventricular ejection fraction (LVEF). An assessment was made as to whether the combined LVSD of C&L fibers has a prognostic impact in asymptomatic AS. METHODS: A total of 200 asymptomatic AS patients was analyzed. Midwall shortening and mitral annular peak systolic velocity were considered as indices of C&L function and classified as low if <16.5% and <8.5 cm/s, respectively. The primary outcome was a composite of major cardiovascular events (MACE), including aortic valve-related and ischemic cardiovascular-related events. RESULTS: During a 25-month follow up period, MACE occurred in 69 patients (35%),while 46 of 72 patients (64%) had C&L LVSD and 23 of 128 patients (18%) had not (p <0.001). Cox analysis identified C&L LVSD as an independent MACE predictor, together with aortic transvalvular peak gradient, E/E' ratio and excessive left ventricular mass. C&L-LVSD also predicted the occurrence of aortic valve-related events and ischemic cardiovascular-related events analyzed separately. A receiver operating characteristic curve analysis showed that the area under the curve (AUC) for C&L LVSD in predicting MACE was 0.77, significantly higher (p = 0.002, z-statistic) than the AUCs of C&L fibers considered individually (0.64 and 0.63, respectively). CONCLUSION: C&L-LVSD provides additional prognostic information into traditional risk factors for patients with asymptomatic AS.

Does treatment assignment influence the prognosis of patients with symptomatic severe aortic stenosis?

OBJECTIVE: Aortic valve replacement (AVR) is the standard therapy in patients with symptomatic aortic stenosis (AS). In high surgical risk patients, alternative therapeutic options to medical treatment (MT) such as trans-catheter aortic valve implantation (TAVI) or balloon aortic valvuloplasty (BAV) have been proposed. In this study we evaluated whether treatment assignment influences per se the prognosis of these subjects. PATIENTS AND METHODS: Criteria for treatment assignment were based on patient's clinical conditions, Logistic EuroSCORE and other co-morbidities ignored by EuroSCORE. Due to baseline clinical differences between patients with diverse treatment assignment, we used propensity score matching to achieve balance. RESULTS: 368 patients were studied: 141 underwent AVR, 127 TAVI, 49 BAV and 51 MT. 84 events (deaths for all causes) occurred during 14 months of follow-up: 11 AVR (8%), 26 TAVI (20%), 18 MT (35%), 29 BAV group (59%). Traditional Cox analysis identified treatment assignment as independent predictor of events (HR 1.82 [CI 1.10-3.25]) together with lower left ventricular ejection fraction, impaired renal function and history of heart failure. Matched Cox analysis by propensity score confirmed treatment assignment as an independent prognosticator of events (HR 1.90 [CI 1.27-2.85]), and showed similar rate events in TAVI and AVR patients, while it was significantly increased in BAV and MT patients. CONCLUSIONS: Treatment assignment may influence outcome of symptomatic patients with AS.

Combined circumferential and longitudinal left ventricular systolic dysfunction in patients with asymptomatic aortic stenosis.

BACKGROUND: Early detection of left ventricular (LV) systolic dysfunction is pivotal in the management of patients with aortic stenosis (AS). LV circumferential and/or longitudinal shortening may be impaired in these patients despite LV ejection fraction is preserved. We focused on prevalence and factors associated with combined impairment of circumferential and longitudinal shortening (C&L) in asymptomatic AS patients. METHODS: Echocardiographic and clinical data from 200 patients with asymptomatic AS of any degree without history of heart failure and normal LV ejection fraction were analyzed. C&L were evaluated by mid-wall shortening (MS) and tissue Doppler mitral annular peak systolic velocity (S'), and classified low if <16.5% and if <8.5 cm/sec, respectively (10th percentiles of controls). RESULTS: Combined C&L dysfunction was detected in 72 patients (36%). The variables associated with this condition were higher LV mass (OR 1.02 [CI 1.01-1.04], P = 0.03), concentric LV geometry (OR 4.30 [CI 1.79-10.34], P = 0.001), increasing pulmonary artery wedge pressure (by E/e' ratio; OR 1.11 [CI 1.04-1.19], P = 0.001). The relation of MS and peak S' was linear and slightly significant in the whole population (r = 0.23; F statistic=0.001), absent in patients with C&L dysfunction (r = 0.04; F = ns), negative (linear model) in the subgroup of patients without C&L dysfunction (r = -0.22; F = 0.02). CONCLUSIONS: C&L dysfunction is present in more than one-third of patients with asymptomatic AS and is associated with concentric LV geometry and higher degree of diastolic dysfunction. The relation between MS and peak S' largely varies in the subgroups with different C&L function.

Identification of fibrillin 1 gene mutations in patients with bicuspid aortic valve (BAV) without Marfan syndrome.

BACKGROUND: Bicuspid aortic valve (BAV) is the most frequent congenital heart disease with frequent involvement in thoracic aortic dilatation, aneurysm and dissection. Although BAV and Marfan syndrome (MFS) share some clinical features, and some MFS patients with BAV display mutations in FBN1, the gene encoding fibrillin-1, the genetic background of isolated BAV is poorly defined. METHODS: Ten consecutive BAV patients [8 men, age range 24-42 years] without MFS were clinically characterized. BAV phenotype and function, together with evaluation of aortic morphology, were comprehensively assessed by Doppler echocardiography. Direct sequencing of each FBN1 exon with flanking intron sequences was performed on eight patients. RESULTS: We detected three FBN1 mutations in two patients (aged 24 and 25 years) displaying aortic root aneurysm ≥50 mm and moderate aortic regurgitation. In particular, one patient had two mutations (p.Arg2726Trp and p.Arg636Gly) one of which has been previously associated with variable Marfanoid phenotypes. The other patient showed a pArg529Gln substitution reported to be associated with an incomplete MFS phenotype. CONCLUSIONS: The present findings enlarge the clinical spectrum of isolated BAV to include patients with BAV without MFS who have involvement of FBN1 gene. These results underscore the importance of accurate phenotyping of BAV aortopathy and of clinical characterization of BAV patients, including investigation of systemic connective tissue manifestations and genetic testing.

Association of left ventricular longitudinal and circumferential systolic dysfunction with diastolic function in hypertension: a nonlinear analysis focused on the interplay with left ventricular geometry.

BACKGROUND: The relationships of left ventricular (LV) longitudinal and circumferential systolic dysfunction with diastolic performance in hypertensive patients have never been compared. METHODS AND RESULTS: In 532 asymptomatic hypertensive patients, circumferential function was assessed with the use of midwall fractional shortening (mFS) and stress-corrected mFS (SCmFS), whereas longitudinal function was assessed with the use of left atrioventricular plane displacement (AVPD) and systolic mitral annulus velocity (s'). Early diastolic annular velocity (e') and the E/e' ratio were measured. Global longitudinal and circumferential strain were determined in a subset of 210 patients. e' was linearly related to all systolic indexes (AVPD: R = 0.40; s': R = 0.39; mFS: R = 0.16; SCmFS: R = 0.17; all P < .0001), but the correlations were stronger with longitudinal indexes than with circumferential ones (P < .0001). E/e' was nonlinearly related to AVPD (R = -0.49; P < .0001) and s' (R = -0.34; P < .0001) and showed no relationship with mFS and SCmFS. Longitudinal indexes were superior to circumferential ones in predicting e' <8 cm/s, E/e' <8, and E/e' ≥13. The effect of LV geometry on LV diastolic function was evident among patients with preserved systolic longitudinal function, but was blunted among patients with impaired longitudinal function. In multivariable analyses, only longitudinal indexes remained associated with e' and E/e'. Analyses using strains provided similar results. CONCLUSIONS: In asymptomatic hypertensive subjects, LV diastolic performance is independently associated with longitudinal systolic dysfunction, but not with circumferential systolic dysfunction. Subtle longitudinal systolic impairment plays a role in mediating the effect of LV geometry on diastolic performance. These findings may support the need of critically revising the concept of isolated diastolic dysfunction in these patients.

Unsupervised machine learning for risk stratification and identification of relevant subgroups of ascending aorta dimensions using cardiac CT and clinical data.

The potential of precision population health lies in its capacity to utilize robust patient data for customized prevention and care targeted at specific groups. Machine learning has the potential to automatically identify clinically relevant subgroups of individuals, considering heterogeneous data sources. This study aimed to assess whether unsupervised machine learning (UML) techniques could interpret different clinical data to uncover clinically significant subgroups of patients suspected of coronary artery disease and identify different ranges of aorta dimensions in the different identified subgroups. We employed a random forest-based cluster analysis, utilizing 14 variables from 1170 (717 men/453 women) participants. The unsupervised clustering approach successfully identified four distinct subgroups of individuals with specific clinical characteristics, and this allows us to interpret and assess different ranges of aorta dimensions for each cluster. By employing flexible UML algorithms, we can effectively process heterogeneous patient data and gain deeper insights into clinical interpretation and risk assessment.

Diagnostic Contexts of Echocardiographic Nonapical Window.

The long-established utility of multiwindow interrogation in echocardiography (suprasternal notch, right and left sternal border, apex, and subxiphoid) is sometimes not systematically implemented in routine practice. This case series emphasizes the pivotal importance of such practice for the systematic assessment of aortic valve stenosis and in the evaluation of left ventricular outflow tract and the aorta.

Peak atrial longitudinal strain and risk stratification in moderate and severe aortic stenosis.

AIMS: We sought to investigate the association of left atrial strain with the outcome in a large cohort of patients with at least moderate aortic stenosis (AS). METHODS AND RESULTS: We analysed 467 patients (mean age 80.6 ± 8.2 years; 51% men) with at least moderate AS and sinus rhythm. The primary study endpoint was the composite of all-cause mortality and hospitalizations for heart failure. After a median follow-up of 19.2 (inter-quartile range 12.5-24.4) months, 96 events occurred. Using the receiver operator characteristic curve analysis, the cut-off value of peak atrial longitudinal strain (PALS) more strongly associated with outcome was <16% {area under the curve (AUC) 0.70 [95% confidence interval (CI): 0.63-0.78], P < 0.001}. The Kaplan-Meier curves demonstrated a higher rate of events for patients with PALS < 16% (log-rank P < 0.001). On multivariable analysis, PALS [adjusted HR (aHR) 0.95 (95% CI 0.91-0.99), P = 0.017] and age were the only variables independently associated with the combined endpoint. PALS provided incremental prognostic value over left ventricular (LV) global longitudinal strain, LV ejection fraction, and right ventricular function. Subgroup analysis revealed that impaired PALS was also independently associated with outcome in the subgroups of paucisymptomatic patients [aHR 0.98 (95% CI 0.97-0.98), P = 0.048], moderate AS [aHR 0.92, (95% CI 0.86-0.98), P = 0.016], and low-flow AS [aHR 0.90 (95% CI 0.83-0.98), P = 0.020]. CONCLUSION: In our patients with at least moderate AS, PALS was independently associated with outcome. In asymptomatic patients, PALS could be a potential marker of sub-clinical damage, leading to better risk stratification and, potentially, earlier treatment.

The role of echocardiography in sports cardiology: An expert opinion statement of the Italian Society of Sports Cardiology (SIC sport).

Transthoracic echocardiography (TTE) is routinely required during pre-participation screening in the presence of symptoms, family history of sudden cardiac death or cardiomyopathies <40-year-old, murmurs, abnormal ECG findings or in the follow-up of athletes with a history of cardiovascular disease (CVD). TTE is a cost-effective first-line imaging modality to evaluate the cardiac remodeling due to long-term, intense training, previously known as the athlete's heart, and to rule out the presence of conditions at risk of sudden cardiac death, including cardiomyopathies, coronary artery anomalies, congenital, aortic and heart valve diseases. Moreover, TTE is useful for distinguishing physiological cardiac adaptations during intense exercise from pathological behavior due to an underlying CVD. In this expert opinion statement endorsed by the Italian Society of Sports Cardiology, we discussed common clinical scenarios where a TTE is required and conditions falling in the grey zone between the athlete's heart and underlying cardiomyopathies or other CVD. In addition, we propose a minimum dataset that should be included in the report for the most common indications of TTE in sports cardiology clinical practice.

Aortopathy and aortic valve surgery in patients with bicuspid aortic valve with and without raphe.

AIM: To evaluate the association between raphe in bicuspid aortic valve (BAV) patients and valve dysfunction, aortopathy and aortic valve surgery in the REBECCA registry [REgistro della valvola aortica Bicuspide della Società Italiana di ECocardiografia e CArdiovascular Imaging (SIECVI)]. METHODS: Prevalence of aortic valve dysfunction and aortopathy was investigated in BAV patients with and without raphe. Aortic valve dysfunction (regurgitation or stenosis) was categorized as mild, moderate and severe. Aortopathy was defined as annulus ≥14 mm/m2; root ≥20 mm/m2; sinotubular junction ≥16 mm/m2; ascending aorta ≥17 mm/m2, and classified in Type A, isolated ascending aorta dilatation; Type B, aortic root and ascending aorta dilatation; and Type C, isolated aortic root dilatation. RESULTS: Overall, 695 patients with BAV were enrolled; 520 (74.8%) with raphe and 175 (25.2%) without raphe. BAV patients with raphe presented more frequently with moderate or severe aortic stenosis than BAV patients without raphe (183 [35.2%] vs 34 [19.4%], p < 0.001). A higher prevalence of aortopathy, particularly Type B, was observed in patients with vs without raphe. At multivariable analysis, raphe was a predictor of aortic valve surgery at three-year follow-up (odds ratio 2.19, 95% confidence interval 1.08-4.44, p < 0.001). CONCLUSIONS: Patients with BAV and raphe have a higher prevalence of significant aortic stenosis, aortopathy, especially Type B, and a higher risk of undergoing aortic valve surgery at three-year follow-up.