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Hypertrophic cardiomyopathy (HCM) is a genetic disease characterized by unexplained left ventricular hypertrophy (LVH), diastolic dysfunction, and increased sudden-death risk. Early detection of the phenotypic expression of the disease in genetic carriers without LVH (Gen+/Phen-) is crucial for emerging therapies. This clinical study aims to identify echocardiographic predictors of phenotypic development in Gen+/Phen-. Sixteen Gen+/Phen- (one subject with troponin T, six with myosin heavy chain-7, and nine with myosin-binding protein C3 mutations), represented the study population. At first and last visit we performed comprehensive 2D speckle-tracking strain echocardiography. During a follow-up of 8 ± 5 years, five carriers developed LVH (LVH+). At baseline, these patients were older than those who did not develop LVH (LVH-) (30 ± 8 vs. 15 ± 8 years, p = 0.005). LVH+ had reduced peak global strain rate during the isovolumic relaxation period (SRIVR) (0.28 ± 0.05 vs. 0.40 ± 0.11 1/s, p = 0.048) and lower global longitudinal strain (GLS) (-19.8 ± 0.4 vs. -22.3 ± 1.1%; p < 0.0001) than LVH- at baseline. SRIVR and GLS were not correlated with age (overall, p > 0.08). This is the first HCM study investigating subjects before they manifest clinically significant or relevant disease burden or symptomatology, comparing at baseline HCM Gen+/Phen- subjects who will develop LVH with those who will not. Furthermore, we identified highly sensitive, easily obtainable, age- and load-independent echocardiographic predictors of phenotype development in HCM gene carriers who may undergo early preventive treatment.
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Cardiomiopatía Hipertrófica , Ecocardiografía , Hipertrofia Ventricular Izquierda , Mutación , Humanos , Masculino , Femenino , Ecocardiografía/métodos , Hipertrofia Ventricular Izquierda/genética , Hipertrofia Ventricular Izquierda/diagnóstico por imagen , Hipertrofia Ventricular Izquierda/etiología , Adulto , Cardiomiopatía Hipertrófica/genética , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Persona de Mediana Edad , Adolescente , Cadenas Pesadas de Miosina/genética , Troponina T/genética , Heterocigoto , Proteínas Portadoras/genética , Adulto Joven , Fenotipo , Miosinas Cardíacas/genéticaRESUMEN
AIMS: Fractional flow reserve (FFR) has never been investigated in patients with reduced ejection fraction and associated coronary artery disease (CAD). We evaluated the impact of FFR on the management strategies of these patients and related outcomes. METHODS AND RESULTS: From 2002 to 2010, all consecutive patients with left ventricular ejection fraction (LVEF) ≤50% undergoing coronary angiography with ≥1 intermediate coronary stenosis [diameter stenosis (DS)% 50-70%] treated based on angiography (Angiography-guided group) or according to FFR (FFR-guided group) were screened for inclusion. In the FFR-guided group, 433 patients were matched with 866 contemporary patients of the Angiography-guided group. For outcome comparison, 617 control patients with LVEF >50% were included. After FFR, stenotic vessels per patient were significantly downgraded compared with the Angiography-guided group (1.43 ± 0.98 vs. 1.97 ± 0.84; P < 0.001). This was associated with lower revascularization rate (52% vs. 62%; P < 0.001) in the FFR-guided vs. the Angiography-guided group. All-cause death at 5 years of follow-up was significantly lower in the FFR-guided as compared with Angiography-guided group [22% vs. 31%. HR (95% CI) 0.64 (0.51-0.81); P < 0.001]. Similarly, rate of major adverse cardiovascular and cerebrovascular events (MACCE: composite of all-cause death, myocardial infarction, revascularization, and stroke) was significantly lower in the FFR-guided group [40% vs. 46% in the Angiography-guided group. HR (95% CI) 0.81 (0.67-0.97); P = 0.019]. Higher rates of death and MACCE were observed in patients with reduced LVEF compared with the control cohort. CONCLUSIONS: In patients with reduced LVEF and CAD, FFR-guided revascularization was associated with lower rates of death and MACCE at 5 years as compared with the Angiography-guided strategy. This beneficial impact was observed in parallel with less coronary artery bypass grafting and more patients deferred to percutaneous coronary intervention or medical therapy.
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Enfermedad de la Arteria Coronaria , Estenosis Coronaria , Reserva del Flujo Fraccional Miocárdico , Intervención Coronaria Percutánea , Angiografía Coronaria , Humanos , Volumen Sistólico , Resultado del Tratamiento , Función Ventricular IzquierdaRESUMEN
Ischemic stroke represents one of the most important health problems in industrialized countries, both for epidemiological and socio-economic impact. The presence of thrombi in the aorta is rare and its treatment has not been uniquely defined. Here we report the case of an 82-years-old man with aortic thrombosis and acute ischemic stroke.
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Enfermedades de la Aorta , Accidente Cerebrovascular Isquémico , Accidente Cerebrovascular , Trombosis , Anciano de 80 o más Años , Aorta/diagnóstico por imagen , Enfermedades de la Aorta/complicaciones , Humanos , Masculino , Accidente Cerebrovascular/complicaciones , Trombosis/etiologíaRESUMEN
Objectives: Increased arterial stiffness is associated with advanced arteriosclerosis, abnormal left ventricular (LV) geometry and function. Whether increased arterial stiffness is associated with incident cardiovascular (CV) event (MACE), independent of other markers of target organ damage needs to be clarified. Methods: We selected hypertensive participants of the Campania Salute Network free of prevalent CV disease, with available echocardiogram and carotid ultrasound, ejection fraction ≥50%, and ≤ stage III Chronic Kidney Disease (n = 6907). Median follow-up was 63 months. End-point was incident MACE (fatal and non-fatal stroke and myocardial infarction, sudden cardiac death, carotid stenting and heart failure requiring hospitalization). Arterial stiffness was assessed from ratio of brachial pulse pressure/stroke index (i.e. normalized for body height in meter to 2.04 power) (PP/SVi). High PP/SVi (n = 980) was defined as >95th sex-specific percentile of the normal distribution from a reference normal population (>2.63/>2.82 mmHg/ml in men and women, respectively). Results: Patients with high PP/SVi were more likely to be women, older, diabetic, with higher systolic blood pressure (BP) and heart rate, more LV concentric geometry, left atrial dilatation and more carotid plaque (all p < .01). At given increase in SVi, patients with high PP/SVi exhibited two-fold increase in PP compared to normal PP/SVi. In Cox regression, patients with high PP/SVi had 63% increased hazard of MACE [95% CI (1.02-2.59) p = .04], independently of significant effect of older age, male sex, carotid plaque and less frequent anti-RAS therapy. Conclusions: In treated hypertensive patients, high PP/SVi predicted increased rate of MACE, independent of common confounders.
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Presión Sanguínea/fisiología , Enfermedades Cardiovasculares/fisiopatología , Hipertensión/diagnóstico , Volumen Sistólico/fisiología , Rigidez Vascular , Adulto , Anciano , Enfermedades Cardiovasculares/etiología , Femenino , Estudios de Seguimiento , Humanos , Hipertensión/epidemiología , Masculino , Persona de Mediana Edad , Infarto del Miocardio , Pronóstico , Sistema de Registros , Accidente CerebrovascularRESUMEN
Background: Hypertension is a leading cause of chronic kidney disease (CKD) and a decrease in glomerular filtration rate (GFR) is associated with a higher prevalence of hypertension and an increased proportion of suboptimal blood pressure (BP) control. Methods: To investigate characteristics associated with GFR decline, we selected 4539 hypertensive patients from the Campania Salute Network (mean age 53 ± 11 years) with at least 3 years of follow-up (FU) and no more than Stage III CKD. GFR was calculated at baseline and at the last available visit using the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation. GFR decline was defined as a ≥30% decrease from initial GFR for patients in Stage III CKD or by a composite ≥30% decrease from baseline and a final value of <60 for those < with Stage III or higher CKD. Results: At a mean FU of 7.5 years, 432 patients (10%) presented with GFR decline. Those patients were older, more likely to be diabetic, with lower GFR and ejection fraction, higher systolic and lower diastolic BP and higher left ventricular (LV) mass and relative wall thickness at baseline; during FU, patients with GFR decline exhibited higher systolic BP, took more drugs and developed more atrial fibrillation (all P < 0.02). The probability of GFR decline was independently associated with older age, prevalent diabetes, baseline lower GFR, higher systolic BP during FU, FU duration, increased LV mass and incident AF with no impact from antihypertensive and antiplatelet medications. Conclusions: During antihypertensive therapy, kidney function declines in patients with initially lower GFR, increased LV mass and suboptimal BP control during FU.
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Antihipertensivos/uso terapéutico , Fibrilación Atrial/fisiopatología , Diabetes Mellitus/fisiopatología , Tasa de Filtración Glomerular , Hipertensión/complicaciones , Hipertensión/tratamiento farmacológico , Insuficiencia Renal Crónica/epidemiología , Adulto , Presión Sanguínea , Femenino , Humanos , Italia/epidemiología , Masculino , Persona de Mediana Edad , PrevalenciaRESUMEN
PURPOSE: Atherosclerosis is a systemic disease and coronary artery disease is frequently associated with peripheral artery disease. As aortic and mitral valvular calcification (VC) share some etiopathogenetic mechanisms with atherosclerosis, we analyzed the risk profile and the echocardiographic characteristics of patients admitted for first acute coronary syndrome (ACS) to investigate whether the presence of VC could be a marker of asymptomatic hemodynamically significant peripheral atherosclerosis. METHODS: A total of 151 patients admitted for ACS without previous history of cardiovascular disease were consecutively enrolled. The presence of VC was identified by echocardiography; a carotid stenosis ≥50% by ultrasound identified carotid artery disease (CarAD); an ankle-brachial index ≤0.9 or ≥1.4 identified lower extremity artery disease (LEAD). Significant peripheral atherosclerosis was defined by the presence of CarAD and/or LEAD. RESULTS: Peripheral atherosclerosis was diagnosed in 82 (54.3%) patients; isolated CarAD in 24, isolated LEAD in 20, both diseases in 38 patients. VC was present in 103 (68.2%) patients. By multivariate analysis, age (OR = 1.059, 95% CI 1.007-1.113, P = 0.025), diabetes mellitus (OR = 5.068, 95% CI 1.480-17.351, P = 0.010), VC (OR = 7.422, 95% CI 2.421-22.880, P < 0.001), and multivessel CAD (OR = 3.317, 95% CI 1.281-8.586, P = 0.013) were the only independent predictors of having peripheral atherosclerosis. C-statistic for VC was not inferior to that obtained by age (0.728, 95% CI 0.649-0.797 vs. 0.800, 95% CI 0.727-0.861, P = 0.101) and to that obtained by the combination of multivessel CAD with diabetes (0.750; 95% CI 0.673-0.817, P = 0.635), and, furthermore, it was higher than that obtained by diabetes alone (0.620, 95% CI 0.538-0.698, P = 0.036). CONCLUSION: Ruling out the presence of significant peripheral atherosclerosis should be routinely considered in patients with ACS showing VC at echocardiography.
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Síndrome Coronario Agudo/diagnóstico por imagen , Enfermedades de las Arterias Carótidas/diagnóstico por imagen , Ecocardiografía/métodos , Enfermedades de las Válvulas Cardíacas/diagnóstico por imagen , Enfermedad Arterial Periférica/diagnóstico por imagen , Calcificación Vascular/diagnóstico por imagen , Síndrome Coronario Agudo/complicaciones , Válvula Aórtica/diagnóstico por imagen , Biomarcadores , Enfermedades de las Arterias Carótidas/complicaciones , Extremidades/irrigación sanguínea , Extremidades/diagnóstico por imagen , Femenino , Enfermedades de las Válvulas Cardíacas/complicaciones , Historia Antigua , Humanos , Persona de Mediana Edad , Válvula Mitral/diagnóstico por imagen , Enfermedad Arterial Periférica/complicaciones , Reproducibilidad de los Resultados , Sensibilidad y Especificidad , Calcificación Vascular/complicacionesRESUMEN
Background: Sex-specific differences in left ventricular (LV) geometry might help in developing tailored strategies for hypertension management. Objectives: The purpose of the study was to evaluate sex-related differences in LV geometry at baseline and over time in hypertension. Methods: From a prospective registry, we included hypertensives without prevalent cardiovascular disease, incident myocardial infarction, chronic kidney disease > stage III, and with normal LV ejection fraction. LV mass index >115 g/m2 in males and >95 g/m2 in females, identified LV hypertrophy (LVH). Relative wall thickness ≥0.43 defined LV concentric geometry. LVH in presence of concentric geometry was defined as concentric LVH, whereas relative wall thickness <0.43 was categorized as eccentric. Concentric geometry, or LVH, identified LV remodeling. Results: Six thousand four hundred twenty-seven patients (age 53 ± 11 years, 43% females) were included. At baseline, females showed lower prevalence of normal geometric pattern and higher prevalence of LVH than males (50% vs 72%, P < 0.001; 47% vs 23%, P < 0.001, respectively), with a higher prevalence of eccentric LVH (40% vs 18%, P < 0.001). Female sex was independently associated with LV remodeling (OR: 2.36; 95% CI: 2.12-2.62; P < 0.001). At long-term follow-up (mean 6.1 years, IQR: 2.8-8.6 years), prevalence of LV remodeling increased in both sexes, although a normal LV geometry remained less frequent in females than males (43% vs 67%, P < 0.001), with differences persisting in eccentric (41% vs 21%, P < 0.001) and concentric LVH (11% vs 5%, P < 0.001). Conclusions: We found sex-related differences in LV geometry among hypertensives. Females have higher risk of LV remodeling at baseline compared with males, with differences persisting at long-term follow-up.
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INTRODUCTION: Delay in arterial hypertension (AH) diagnosis and late therapy initiation may affect progression towards hypertensive-mediated organ damage (HMOD) and blood pressure (BP) control. AIM: We aimed to assess the impact of time-to-therapy on BP control and HMOD in patients receiving AH diagnosis. METHODS: We analysed data from the Campania Salute Network, a prospective registry of hypertensive patients (NCT02211365). At baseline visit, time-to-therapy was defined as the interval between the first occurrence of BP values exceeding guidelines-directed thresholds and therapy initiation; HMOD included left ventricular hypertrophy (LVH), carotid plaque, or chronic kidney disease. Optimal BP control was considered for average values < 140/90 mmHg. Low-risk profile was defined as grade I AH without additional cardiovascular risk factors. RESULTS: From 14,161 hypertensive patients, we selected 1,627 participants who were not on antihypertensive therapy. This population was divided into two groups based on the median time-to-therapy (≤ 2 years n = 1,009, > 2 years n = 618). Patients with a time-to-therapy > 2 years had higher risk of HMOD (adjusted odds ratio, aOR:1.51, 95%, CI:1.19-1.93, p < 0.001) due to increased risks of LVH (aOR:1.43, CI:1.12-1.82, p = 0.004), carotid plaques (aOR:1.29, CI:1.00-1.65, p = 0.047), and chronic kidney disease (aOR:1.68, CI:1.08-2.62, p = 0.022). Time-to-therapy > 2 years was significantly associated with uncontrolled BP values (aOR:1.49, CI:1.18-1.88, p < 0.001) and higher number of antihypertensive drugs (aOR:1.68, CI:1.36-2.08, p < 0.001) during follow-up. In low-risk subgroup, time-to-therapy > 2 years did not impact on BP control and number of drugs. CONCLUSIONS: In hypertensive patients, a time-to-therapy > 2 years is associated with HMOD and uncontrolled BP.
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Hypertrophic Cardiomyopathy (HCM) presents a complex diagnostic and prognostic challenge due to its heterogeneous phenotype and clinical course. Artificial Intelligence (AI) and Machine Learning (ML) techniques hold promise in transforming the role of Electrocardiography (ECG) in HCM diagnosis, prognosis, and management. AI, including Deep Learning (DL), enables computers to learn patterns from data, allowing for the development of models capable of analyzing ECG signals. DL models, such as convolutional neural networks, have shown promise in accurately identifying HCM-related abnormalities in ECGs, surpassing traditional diagnostic methods. In diagnosing HCM, ML models have demonstrated high accuracy in distinguishing between HCM and other cardiac conditions, even in cases with normal ECG findings. Additionally, AI models have enhanced risk assessment by predicting arrhythmic events leading to sudden cardiac death and identifying patients at risk for atrial fibrillation and heart failure. These models incorporate clinical and imaging data, offering a comprehensive evaluation of patient risk profiles. Challenges remain, including the need for larger and more diverse datasets to improve model generalizability and address imbalances inherent in rare event prediction. Nevertheless, AI-driven approaches have the potential to revolutionize HCM management by providing timely and accurate diagnoses, prognoses, and personalized treatment strategies based on individual patient risk profiles. This review explores the current landscape of AI applications in ECG analysis for HCM, focusing on advancements in AI methodologies and their specific implementation in HCM care.
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INTRODUCTION: No data are available on the diagnostic algorithms recommended by guidelines for the assessment of diastolic dysfunction (DD) in patients with arterial hypertension. AIM: To fill this gap, we evaluated diastolic function in hypertensive patients with and without LVH matched with healthy subjects by applying 2016 American Society of Echocardiography-European Association of Cardiovascular Imaging Guidelines for the evaluation of LV diastolic function. METHODS: 717 healthy and hypertensives with normal LV ejection fraction and with and without LV hypertrophy (LVH), matched 1:1:1 from two prospective registries, represented the study population. RESULTS: By applying algorithm A, indeterminate pattern was found in 0.4% of healthy, in 6.3% of hypertensives without LVH, and in 21% with LVH (overall p < 0.05 vs. healthy). DD was absent in healthy, however present in 2 and 8% of hypertensives without and with LVH (p = 0.06 and p = 0.001 vs. healthy, respectively). By applying algorithm B, no cases of indeterminate pattern were found. DD was observed in 2.9% of healthy, 7 and 10.5% of hypertensives without and with LVH (p < 0.05 vs. healthy). CONCLUSIONS: The use of algorithm A should be limited only to truly normal subjects, whereas algorithm B should be applied to all patients with hypertension, even without comorbidities and irrespective of LVH.
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Algoritmos , Diástole , Hipertensión , Hipertrofia Ventricular Izquierda , Valor Predictivo de las Pruebas , Sistema de Registros , Función Ventricular Izquierda , Humanos , Hipertensión/fisiopatología , Hipertensión/diagnóstico , Masculino , Femenino , Persona de Mediana Edad , Hipertrofia Ventricular Izquierda/fisiopatología , Hipertrofia Ventricular Izquierda/diagnóstico por imagen , Hipertrofia Ventricular Izquierda/diagnóstico , Anciano , Estudios de Casos y Controles , Disfunción Ventricular Izquierda/fisiopatología , Disfunción Ventricular Izquierda/diagnóstico por imagen , Presión Arterial , Guías de Práctica Clínica como Asunto , Adulto , Volumen Sistólico , Estudios Prospectivos , Reproducibilidad de los ResultadosRESUMEN
Background: In obstructive hypertrophic cardiomyopathy (HOCM), disopyramide is used in patients who remain symptomatic despite ß-blockers or verapamil. However, effectiveness of disopyramide therapy has not been clearly established due to inconsistent definition of responders and the insufficient length of follow-ups reported in literature. To address these shortcomings, we have conducted a retrospective analysis from detailed databases with long follow-up, from two HCM Referral Centers. Methods: 62 symptomatic HOCM patients (43% women, age 52 ± 14 years) with left ventricular (LV) outflow tract gradient (LVOTG) ≥ 50 mmHg at rest or during provocation, were recruited from two Italian Centers. Disopyramide was added as second-line therapy in the patients in whom symptoms persisted despite classic pharmacologic treatment. Patients in NYHA class > II at baseline who reached NYHA class II or I, and patients in NYHA class II at baseline who reached NYHA class I or symptoms stabilization were defined as responders. Results: At follow-up, (mean 4.4 years, IQR 1.1-6.6 years), 47 patients (76%) were responders, whereas 15 (24%) were no-responders. Responders showed larger LV diastolic volume index (LVEDVi) at baseline as compared to no-responders (61 ± 14 vs. 49 ± 16â ml, respectively, p = 0.018), and, at follow-up, reached lower LVOTG than no-responders (43 ± 32 vs. 66 ± 28â mmHg, respectively, p = 0.013), with a LVOTG <50 mmHg more represented in responders than in no-responders (75% vs. 25%, respectively; p = 0.004). No side effects requiring discontinuation of the therapy were recorded. Conclusion: HOCM patients treated with disopyramide as second-line therapy in a quite long-follow-up showed a significant improvement of symptoms, which avoided SRT in up to 70% of them. Moreover, our data suggest that a larger LVEDVi at baseline identify the subgroup of patients who benefit the most from the therapy in terms of symptoms and reduction of LVOTG below 50 mmHg during treatment. We will discuss specific situations where disopyramide may be preferred over myosin inhibition to ensure that effective therapeutic options are fully considered and not prematurely dismissed.
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BACKGROUND AND AIM: Atrial fibrillation (AF) is the most common sustained arrhythmia in hypertrophic cardiomyopathy (HCM) with significant effects on outcome. We aim to compare the left atrial (LA) diameter measurement with HCM-AF Score in predicting atrial fibrillation (AF) development in HCM. METHODS: From the regional cohort of the Campania Region, Italy, 519 HCM patients (38% women, age45 ± 17 years) without history of AF, were enrolled in the study. The primary clinical endpoint was the development of AF, defined as at least 1 episode documented by ECG. RESULTS: During the follow-up (mean 8 ± 6, IQ range 2.5-11.2 years), 99 patients (19%) developed AF. Patients who developed AF were more symptomatic, had higher prevalence of ICD implantation, had larger LA diameter, greater left ventricular (LV) maximal wall thickness and LV outflow tract obstruction (p < 0.01). Both LA diameter and HCM-AF score were higher in patients who developed AF versus those who did not (LA diameter 49 ± 7 versus 43 ± 6 mm; HCM-AF score 22 ± 4 versus 19 ± 4; p < 0.0001); however, ROC curve analysis demonstrated that LA diameter had a significant greater area under the curve than HCM-AF Score (p < 0.0001). At 5 years follow-up, a LA diameter > 46 mm, showed a similar accuracy in predicting AF development of HCM-AF score ≥ 22, which identifies patients at high risk to develop AF. CONCLUSION: Our analysis shows that LA diameter, a worldwide and simple echocardiographic measure, is capable alone to predict AF development in HCM patients.
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Apéndice Atrial , Fibrilación Atrial , Cardiomiopatía Hipertrófica , Humanos , Femenino , Masculino , Fibrilación Atrial/diagnóstico por imagen , Fibrilación Atrial/epidemiología , Atrios Cardíacos , Cardiomiopatía Hipertrófica/diagnóstico , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Ventrículos Cardíacos , Factores de RiesgoRESUMEN
BACKGROUND: Women have a lower risk for cardiovascular (CV) disease compared to men. Whether this difference is influenced by the presence of hypertension-mediated organ damage is unknown. OBJECTIVE: To assess whether the presence of carotid plaque (CP) impacts the sex difference in risk for CV events in treated hypertensive patients. METHODS: From the Campania Salute Network Registry 2419 women and men <51 years of age with treated hypertension and free from prevalent CV disease were included. The presence of CP was identified by Doppler ultrasound (intima-media thickness≥1.5 mm). The primary outcome was a composite of fatal and non-fatal stroke or myocardial infarction, sudden death, TIA, myocardial revascularization, de novo angina, and atrial fibrillation. RESULTS: Among patients without CP at baseline (n = 1807), women were older, with higher systolic blood pressure, serum cholesterol level and prevalence of LVH but lower serum triglycerides and eGFR, compared to men (all p < 0.001). Among patients with CP (n = 612), women were older, used higher number of antihypertensive drugs, had higher serum cholesterol level and prevalence of left ventricular hypertrophy (LVH), but had lower serum triglycerides and eGFR compared to men (all p < 0.001). During follow-up, women without CP had a lower risk for CV disease than men (hazard ratio, HR, 0.51, 95 % confidence intervals, CI, 0.27-0.99, p = 0.04) after accounting for cardiovascular risk factors, LVH, and antihypertensive treatment. In contrast, among patients with CP, women had similar risk for CV disease compared with men (HR 1.3, 95 % CI, 0.59-2.9, p = 0.48). CONCLUSIONS: Our findings suggest that the presence of CP in young patients with treated hypertension offsets the CV disease protection in women. TRIAL REGISTRATION: NCT02211365.
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Hypertrophic cardiomyopathy (HCM) is mainly caused by sarcomeric mutations which may affect myocardial mechano-energetic efficiency (MEE). We investigated the effects of sarcomeric mutations on MEE. A non-invasive pressure/volume (P/V) analysis was performed. We included 49 genetically screened HCM patients. MEEi was calculated as the ratio between stroke volume and heart rate normalized by LV mass. Fifty-seven percent (57%) HCM patients carried a sarcomeric mutation. Patients with and without sarcomeric mutations had similar LV ejection fraction, heart rate, LV mass, and LV outflow gradient. Younger age at diagnosis, family history of HCM, and lower MEEi were associated with presence of sarcomeric mutation (p = 0.017; p = 0.001 and p = 0.0001, respectively). Lower MEEi in HCM with sarcomeric mutation is not related to significant differences on filling pressure as shown on P/V analysis. Sarcomeric mutations determine a reduction of the LV pump performance as estimated by MEEi in HCM. Lower MEEi may predict a positive genetic analysis.
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Aortic stenosis (AS) is a valvular heart disease that significantly contributes to cardiovascular morbidity and mortality worldwide. The condition is characterized by calcification and thickening of the aortic valve leaflets, resulting in a narrowed orifice and increased pressure gradient across the valve. AS typically progresses from a subclinical phase known as aortic sclerosis, where valve calcification occurs without a transvalvular gradient, to a more advanced stage marked by a triad of symptoms: heart failure, syncope, and angina. Echocardiography plays a crucial role in the diagnosis and evaluation of AS, serving as the primary non-invasive imaging modality. However, to minimize misdiagnoses, it is crucial to adhere to a standardized protocol for acquiring echocardiographic images. This is because, despite continuous advances in echocardiographic technology, diagnostic errors still occur during the evaluation of AS, particularly in classifying its severity and hemodynamic characteristics. This review focuses on providing guidance for the imager during the echocardiographic assessment of AS. Firstly, the review will report on how the echo machine should be set to improve image quality and reduce noise and artifacts. Thereafter, the review will report specific emphasis on accurate measurements of left ventricular outflow tract diameter, aortic valve morphology and movement, as well as aortic and left ventricular outflow tract velocities. By considering these key factors, clinicians can ensure consistency and accuracy in the evaluation of AS using echocardiography.
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Aortic stenosis (AS) can often coexist with other valvular diseases or be combined with aortic regurgitation (AR), leading to unique pathophysiological conditions. The combination of affected valves can vary widely, resulting in a lack of standardized diagnostic or therapeutic approaches. Echocardiography is crucial in assessing patients with valvular heart disease (VHD), but careful consideration of the hemodynamic interactions between combined valvular defects is necessary. This is important as it may affect the reliability of commonly used echocardiographic parameters, making the diagnosis challenging. Therefore, a multimodality imaging approach, including computed tomography or cardiac magnetic resonance, is often not just beneficial but crucial. It represents the future of diagnostics in this intricate field due to its unprecedented capacity to quantify and comprehend valvular pathology. The absence of definitive data and guidelines for the therapeutic management of AS in the context of multiple valve lesions makes this condition particularly challenging. As a result, an individualized, case-by-case approach is necessary, guided primarily by the recommendations for the predominant valve lesion. This review aims to summarize the pathophysiology of AS in the context of multiple and mixed valve disease, with a focus on the hemodynamic implications, diagnostic challenges, and therapeutic options.
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Hypertrophic cardiomyopathy (HCM) is a genetic disease with heterogeneous clinical presentation and prognosis. Within the broad phenotypic expression of HCM, there is a subgroup of patients with a left ventricular (LV) apical aneurysm, which has an estimated prevalence between 2% and 5%. LV apical aneurysm is characterized by an area of apical dyskinesis or akinesis, often associated with regional scarring. To date, the most accepted pathomechanism of this complication is, in absence of coronary artery disease, the high systolic intra-aneurysmal pressure, which, combined with impaired diastolic perfusion from lower stroke volume, results in supply-demand ischemia and myocardial injury. Apical aneurysm is increasingly recognized as a poor prognostic marker; however, the efficacy of prophylactic anticoagulation and/or intracardiac cardioverted defibrillator (ICD) in improving morbidity and mortality is not yet clearly demonstrated. This review aims to elucidate the mechanism, diagnosis and clinical implication of LV aneurysm in patients with HCM.
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Cardiac surgery is necessary in almost 50% of patients with endocarditis. Early surgery, i.e., the surgery performed during the first hospitalization, is required in the following cases: heart failure secondary to valve regurgitation; S. aureus, fungal organism, or other highly resistant organism infection; heart block, annular or aortic abscess, or destructive penetrating lesions; evidence of persistent infection as manifested by persistent bacteremia or fevers lasting >5 days after onset of appropriate antimicrobial therapy. A 62-year-old man developed a fever (38 °C) 3 days after a transaortic electrophysiological study; blood cultures were positive for S. aureus, and were sensitive to vancomycin and ceftaroline. Antibiotic therapy was started, controlling the fever and the patient's infective and inflammatory profiles well; however, 3 days later, acute aortic regurgitation developed. At transesophageal echocardiography (TEE), a rare condition was revealed-vegetation was attached to the aortic wall, impeding correct aortic valve closure. Cardiac operation was carried out and the time for surgery was discussed; based on the patient's clinically stable condition, and on the infection, which was controlled well by antibiotics therapy, surgery was not performed in emergency circumstance (within 24-48 h)-rather, it was programmed during the hospitalization. A TEE surveillance was initiated, and after 7 days, TEE revealed a new picture, with images of an aortic abscess with small perforation in the right atrium, requiring emergency surgery, carried out 20 h later. In our case, the rapid evolution of the vegetation attached to the aortic wall suggested the following: (1) that the time for the surgery cannot be guided only by clinical procedure but must also be guided by imaging pictures; (2) that strictly TEE surveillance is mandatory in patients with aortic endocarditis not initially referred for emergency surgery.
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Background: The influence of age and gender on strain-imaging-derived myocardial work (MW) was recently investigated in healthy subjects. No information is available on the impact of heart rate (HR) on MW. Methods: 177 healthy subjects (47% men, mean age 42 years) underwent an echo-Doppler exam, including quantification of global longitudinal strain (GLS). Cuff blood pressure was used as a surrogate of left ventricular peak pressure to estimate global work index (GWI), global constructive work (GCW), global wasted work (GWW) and global work efficiency (GWE). Statistical analyses were performed according to age and HR tertiles. Results: GWW was higher in the third HR tertile, i.e., ≥74 bpm (74.7 ± 33.6 mmHg %) than in the first HR tertile (<66 bpm) (61.0 ± 32.5 mmHg %) (p < 0.02). In the pooled population, by adjusting for systolic blood pressure, GLS, E/e' ratio and left atrial volume index, age was independently associated with GCW (ß = 0.748) and GWI (ß = 0.685) (both p < 0.0001) and HR with GWW (ß = 0.212, p = 0.006) and GWE (ß = −0.204, p = 0.007). Conclusions: In healthy subjects age shows a mild influence on GCW. HR exerts an independent negative impact on GWW and GWE: the higher HR the greater wasted work and lower myocardial efficiency.
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BACKGROUND: Exaggerated variability of blood pressure (BP) poses additional stress on cardiovascular system independent of BP average value, increasing risk of target organ damage (HMOD) and cardiovascular events. We assessed the impact of visit-to-visit variability (VVV) of BP on development of cardiovascular events and HMOD. METHODS: Standard deviation (SD) and coefficient of variability of mean SBP and DBP were calculated in 3555 patients from the Campania Salute Network registry, with available echocardiogram and more than six visits during follow-up. Values from the first visit were excluded. The impact of VVV of BP on cardiovascular events, and mediation of HMOD were assessed at final visit. RESULTS: Mean number of visits was 11â±â6 with mean interval between visits of 9.1â±â3.7 months. Mean visit-to-visit SD during follow-up was 13â±â5 for systolic and 8â±â3âmmHg for DBP; coefficients of variability were 9.7â±â3.5 and of 9.6â±â3.2, respectively. In multivariable analysis, left ventricular mass at follow-up was correlated with systolic VVV of BP independently of significant effect of age, BMI, mean SBP during follow-up and initial left ventricular mass. Follow-up GFR was inversely associated with systolic and diastolic VVV, independently of significant effect of age, mean glucose and SBP during follow-up, and initial GFR. In Cox regression, high VVV of BP was also associated with increased risk of cardiovascular events (hazard ratio 1.49, 95% confidence interval 1.08-2.06, Pâ=â0.015), independently of significant effect of HMOD. CONCLUSION: VVV is associated with prevalent HMOD and development of cardiovascular events, independently of mean BP value during follow-up and HMOD.