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ims: Cardiac magnetic resonance (CMR) is recommended in the diagnosis of cardiomyopathies, but it is time-consuming, expensive, and limited in availability in some European regions. The aim of this study was to determine the use of CMR in cardiomyopathy patients enrolled into the European Society of Cardiology (ESC) cardiomyopathy registry [part of the EURObservational Research Programme (EORP)]. Methods and results: Three thousand, two hundred, and eight consecutive adult patients (34.6% female; median age: 53.0 ± 15 years) with cardiomyopathy were studied: 1260 with dilated (DCM), 1739 with hypertrophic (HCM), 66 with restrictive (RCM), and 143 with arrhythmogenic right ventricular cardiomyopathy (ARVC). CMR scans were performed at baseline in only 29.4% of patients. CMR utilization was variable according to cardiomyopathy subtypes: from 51.1% in ARVC to 36.4% in RCM, 33.8% in HCM, and 20.6% in DCM (P < 0.001). CMR use in tertiary referral centres located in different European countries varied from 1% to 63.2%. Patients undergoing CMR were younger, less symptomatic, less frequently had implantable cardioverter-defibrillator (ICD)/pacemaker implanted, had fewer cardiovascular risk factors and comorbidities (P < 0.001). In 28.6% of patients, CMR was used along with transthoracic echocardiography (TTE); 67.6% patients underwent TTE alone, and 0.9% only CMR. Conclusion: Less than one-third of patients enrolled in the registry underwent CMR and the use varied greatly between cardiomyopathy subtypes, clinical profiles of patients, and European tertiary referral centres. This gap with current guidelines needs to be considered carefully by scientific societies to promote wider availability and use of CMR in patients with cardiomyopathies.

The introduction of three-dimensional echocardiography and its evolution from time-consuming and cumbersome off-line reconstruction to real-time volumetric technique (real-time three-dimensional echocardiography) are one of the most significant advances in ultrasound imaging of the heart of the past decade. This imaging modality currently provides realistic views of cardiac valves capable of demonstrating the anatomy of various heart valve diseases in a unique, noninvasive manner. In addition, real-time three-dimensional echocardiography offers completely new views of the valves and surrounding structures, and allows accurate quantification of severity of valve disease. This article reviews the advantages of real-time three-dimensional echocardiography in assessing heart valves and shows also technological limitations in order to provide the scientific basis for its clinical use.

The definition of the athlete's heart includes the mechanisms of cardiac adaptation to training, characterized by the increase of internal chamber dimensions, ventricular wall thickness, and atrial chambers. The morphology of the athlete's heart is intermediate between concentric and eccentric left ventricular hypertrophy (LVH), in relation to the large prevalence of mixed sports activities and training protocols (including both aerobic and anaerobic exercise). Echocardiography is the tool of choice for the assessment of the athlete's heart and also for the differentiation of physiologic and pathologic LVH (hypertrophic cardiomyopathy and LVH due to arterial hypertension). The initial echocardiographic approach includes the quantitative analysis of the left ventricle, in order to calculate left ventricular mass, left ventricular mass index and relative wall thickness for diagnosing concentric or eccentric LVH. Tissue Doppler (pulsed or color modality) and strain rate imaging (Doppler or two-dimensional modality) may give additional information to the standard indices of systolic function. Diastolic function can be evaluated not only by standard Doppler transmitral inflow measurements but also using pulsed tissue Doppler, which may allow to distinguish the athlete's LVH from diastolic impairment of hypertensive patients or hypertrophic cardiomyopathy by the simple determination of myocardial early diastolic velocity. Also the morphological and functional features of the left atrium and of the right ventricle can be assessed in the athlete's heart by combining standard echocardiography with new echocardiographic technologies.

© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited., Aims: The interaction between common cardiovascular risk factors (CVRF) and hypertrophic cardiomyopathy (HCM) is poorly studied. We sought to explore the relation between CVRF and the clinical characteristics of patients with HCM enrolled in the EURObservational Research Programme (EORP) Cardiomyopathy registry. Methods and results: 1739 patients with HCM were studied. The relation between hypertension (HT), diabetes (DM), body mass index (BMI) and clinical traits was analyzed. Analyses were stratified according to the presence or absence of a pathogenic variant in a sarcomere gene.The prevalence of HT, DM and obesity (Ob) was 37%, 10%, and 21%, respectively. HT, DM and Ob were associated with older age (p

Aims The Cardiomyopathy Registry of the EURObservational Research Programme is a prospective, observational, and multinational registry of consecutive patients with four cardiomyopathy subtypes: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). We report the baseline characteristics and management of adults enrolled in the registry. ................................................................................................................................................................................................... Methods and results A total of 3208 patients were enrolled by 69 centres in 18 countries [HCM (n = 1739); DCM (n = 1260); ARVC (n = 143); and RCM (n = 66)]. Differences between cardiomyopathy subtypes (P < 0.001) were observed for age at diagnosis, history of familial disease, history of sustained ventricular arrhythmia, use of magnetic resonance imaging or genetic testing, and implantation of defibrillators. When compared with probands, relatives had a lower age at diagnosis (P < 0.001), but a similar rate of symptoms and defibrillators. When compared with the Long-Term phase, patients of the Pilot phase (enrolled in more expert centres) had a more frequent rate of familial disease (P < 0.001), were more frequently diagnosed with a rare underlying disease (P < 0.001), and more frequently implanted with a defibrillator (P = 0.023). Comparing four geographical areas, patients from Southern Europe had a familial disease more frequently (P < 0.001), were more frequently diagnosed in the context of a family screening (P < 0.001), and more frequently diagnosed with a rare underlying disease (P < 0.001). ................................................................................................................................................................................................... Conclusion By providing contemporary observational data on characteristics and management of patients with cardiomyopathies, the registry provides a platform for the evaluation of guideline implementation. Potential gaps with existing recommendations are discussed as well as some suggestions for improvement of health care provision in Europe.

Objectives The purpose of this paper was to determine whether microRNAs (miRNAs) involved in myocardial remodeling were differentially expressed in the blood of hypertrophic cardiomyopathy (HCM) patients, and whether circulating miRNAs correlated with the degree of left ventricular hypertrophy and fibrosis. Background miRNAs—small, noncoding ribonucleic acids (RNAs) that regulate gene expression by inhibiting RNA translation—modulate cellular function. Myocardial miRNAs modulate processes such as cardiomyocyte (CM) hypertrophy, excitation–contraction coupling, and apoptosis; non–CM-specific miRNAs regulate myocardial vascularization and fibrosis. Recently, the possibility that circulating miRNAs may be biomarkers of cardiovascular disease has been raised. Methods Forty-one HCM patients were characterized with conventional transthoracic echocardiography and cardiac magnetic resonance. Peripheral plasma levels of 21 miRNAs were assessed by quantitative real-time polymerase chain reaction and were compared with levels in a control group of 41 age- and sex-matched blood donors. Results Twelve miRNAs (miR-27a, -199a-5p, -26a, -145, -133a, -143, -199a-3p, -126-3p, -29a, -155, -30a, and -21) were significantly increased in HCM plasma. However, only 3 miRNAs (miR-199a-5p, -27a, and -29a) correlated with hypertrophy; more importantly, only miR-29a correlated also with fibrosis. Conclusions Our data suggest that cardiac remodeling associated with HCM determines a significant release of miRNAs into the bloodstream: the circulating levels of both cardiac- and non–cardiac-specific miRNAs are significantly increased in the plasma of HCM patients. However, correlation with left ventricular hypertrophy parameters holds true for only a few miRNAs (i.e., miR-199a-5p, -27a, and -29a), whereas only miR-29a is significantly associated with both hypertrophy and fibrosis, identifying it as a potential biomarker for myocardial remodeling assessment in HCM.

This study was designed to investigate whether, in patients with hypertrophic cardiomyopathy (HC), tilt-induced volume unloading triggers a peripheral reflex similar to that seen in patients with a history of vasovagal syncope or rather acts through an intrinsic cardiac mechanism secondary to diastolic dysfunction. Thirty-seven patients with HC (10 with and 27 without a history of syncope), 10 patients with vasovagal syncope, and 9 controls underwent 70 degrees head-up tilt for 45 minutes during continuous radionuclide monitoring of left ventricular function. We focused on the initial 5 minutes into the tilt test, well before symptoms occurred, to exclude that the observed hemodynamic changes were the consequence rather than the cause of syncope. HC patients with previous syncope and vasovagal patients experienced significant hypotension after the initial 5 minutes of tilt. Only HC patients with a history of syncope had a significant decrease in cardiac output, which began at the initial stage of the test. Systemic vascular resistance decreased in vasovagal patients, but increased in the HC syncopal group. Baseline peak filling rate was lower (2.4 +/- 0.5 vs 3.3 +/- 1.1 stroke counts/s, p = 0.03) and a "pseudonormal" or a restrictive pattern of left ventricular filling was more frequent (70% vs 26%, p = 0.02) in HC patients with than without a history of syncope. Thus, significant hypotension or frank syncope during orthostatic stress in HC patients with a history of syncope is due to an early decrease in cardiac output, which occurs well before the onset of symptoms; such impaired hemodynamic adaptation seems to be related to diastolic dysfunction.

Aims Asynchronous myocardial contraction adversely influences left ventricular (LV) function and is therefore associated with a poor prognosis in heart failure. Exercise-induced change in ventricular dyssynchrony may be an important determinant of dynamic changes in cardiac output and mitral regurgitation. Methods and results A prospective, longitudinal study was designed with pre-defined dyssynchrony index and outcome variables to test the hypothesis that dynamic dyssynchrony is associated with worse long-term event-free survival in patients with dilated cardiomyopathy (DCM) and ‘narrow’ QRS complex. One-hundred eighty patients (62 ± 8 years; 110 males) with NYHA class II–III, idiopathic DCM, ejection fraction ≤35%, and QRS duration

Aims: To evaluate left ventricular (LV) systolic and diastolic myocardial function, and their relation to coronary flow reserve in patients with non-insulin-dependent diabetes mellitus (DM) and microvascular angina. Methods and results: We selected a population of 45 normotensive patients with DM (56.3 ± 8.2 years; 25 males) with LV ejection fraction > 50% and microvascular angina (anginal pain, positive imaging stress test and normal coronary angiography). Thirty-five age- and sex-matched healthy controls were also enrolled. All the patients underwent standard echocardiography, Tissue Doppler (TDI), two-dimensional strain (2DSE) imaging, and coronary flow reserve (CFR) measurement. LV myocardial early diastolic peak velocities (Em) and peak systolic 2DSE were reduced in both interventricular septum (IVS) and LV lateral wall (p < 0.01) in DM, as well as CFR (1.89 ± 0.7 vs 2.55 ± 0.56, p < 0.0001) compared with controls. By multivariate analysis, the independent determinants of Em were glycated haemoglobin (β coefficient = - 0.36; p < 0.01) and age (β = - 0.46, p < 0.001), while global longitudinal strain was predicted by glycated haemoglobin (β = 0.48, P < 0.001) and by the duration of the disease (β = 0.38, P < 0.005). An independent association between LV global longitudinal strain and CFR (β coefficient = - 0.47, p < 0.001) in DM patients was also evidenced. Conclusions: TDI, 2DSE and CFR are valuable non-invasive and easy-repeatable tools for detecting LV myocardial and coronary function in DM patients with microvascular angina. © 2011 Published by Elsevier Ireland Ltd.

When applying echo-Doppler imaging for either clinical or research purposes it is very important to select the most adequate modality/technology and choose the most reliable and reproducible measurements. Quality control is a mainstay to reduce variability among institutions and operators and must be obtained by using appropriate procedures for data acquisition, storage and interpretation of echo-Doppler data. This goal can be achieved by employing an echo core laboratory (ECL), with the responsibility for standardizing image acquisition processes (performed at the peripheral echo-labs) and analysis (by monitoring and optimizing the internal intra- and inter-reader variability of measurements). Accordingly, the Working Group of Echocardiography of the Italian Society of Cardiology decided to design standardized procedures for imaging acquisition in peripheral laboratories and reading procedures and to propose a methodological approach to assess the reproducibility of echo-Doppler parameters of cardiac structure and function by using both standard and advanced technologies. A number of cardiologists experienced in cardiac ultrasound was involved to set up an ECL available for future studies involving complex imaging or including echo-Doppler measures as primary or secondary efficacy or safety end-points. The present manuscript describes the methodology of the procedures (imaging acquisition and measurement reading) and provides the documentation of the work done so far to test the reproducibility of the different echo-Doppler modalities (standard and advanced). These procedures can be suggested for utilization also in non referall echocardiographic laboratories as an "inside" quality check, with the aim at optimizing clinical consistency of echo-Doppler data.

Hypertrophic cardiomyopathy (HCM) is one of the most common inherited cardiomyopathy. The identification of patients with HCM is sometimes still a challenge. Moreover, the pathophysiology of the disease is complex because of left ventricular hyper-contractile state, diastolic dysfunction, ischemia and obstruction which can be coexistent in the same patient. In this review, we discuss the current and emerging echocardiographic methodology that can help physicians in the correct diagnostic and pathophysiological assessment of patients with HCM.

A 70-year-old man was admitted because of a 6-month history of progressive dyspnoea on exertion. The medical history showed that he suffered from patent ductus arteriosus (PDA) that was closed at 35 years of age by surgical ligation. Subsequently, up to year 1992, no evidence of residual left-to-right shunt was found. When he first came to our attention, we performed an echocardiographic test evidencing left ventricular dilation and contractile dysfunction and a recurrence of PDA. To exclude other possible causes of congestive heart failure, we performed several tests, including a coronary angiogram that showed coronary atherosclerosis without significant lesions. The haemodynamic study confirmed that the PDA was associated with a mild pulmonary hypertension with a QP: QS of 2: 1. The patient did not report any cardiovascular risk factor. Therefore, we concluded that PDA was responsible for congestive heart failure in this patient. We performed percutaneous closure of PDA, which was able to reverse left ventricular dilation and dysfunction, improving the patient's symptoms, at 1 month as well as 4 months after the interventional procedure. Although this kind of device is frequently used in the paediatric population, adult patients may present different challenges in proper management, such as poor visualization, calcification and pulmonary hypertension. In the description of the case reported here, we show that a PDA can present as congestive heart failure in the elderly. Percutaneous closure can be very effective in ameliorating left ventricular performance as well as symptoms.

We report herein a patient in whom a very large and old thrombus in the left atrium was detected by transesophageal echocardiography. The patient started warfarin and aspirin. After 2 years of therapy, transesophageal echocardiography showed the complete resolution of thrombus in the absence of clinical evidence of embolism. This case indicates that large and presumably organized thrombi may be dissolved by an anticoagulant therapy, although the lytic activity of warfarin has never been demonstrated. Transesophageal echocardiography helps in the identification and follow-up of such conditions and contributes to understanding of warfarin action in left atrial thrombosis. (C) 2003 Elsevier Ireland Ltd. All rights reserved.

Background— Myocardial interstitial fibrosis is a characteristic of hypertrophic cardiomyopathy (HCM). This study evaluates the collagen turnover in HCM and its impact on left ventricular (LV) diastolic function. Methods and Results— Thirty-six HCM patients and 14 sex- and age-matched controls were studied. Collagen turnover was assessed as follows. By radioimmunoassay, a byproduct of collagen III synthesis (PIIINP) and 3 peptides resulting from collagen I synthesis (PICP and PINP) and degradation (ICTP) were measured. By ELISA, matrix metalloproteinases (MMPs) were determined, as follows: active MMP-2; active MMP-9; and MMP-1 as active, free (as active MMP-1 plus its precursor), and total (as free MMP-1 plus MMP-1/tissue inhibitor complexes). Tissue inhibitor of metalloproteinases-1 (TIMP-1) was also assayed. All patients underwent echocardiography. The difference in duration between transmitral forward (A) and pulmonary venous retrograde (AR) waves (A−Ar) was considered an estimate of passive diastolic function. Furthermore, restrictive or pseudonormal LV filling patterns were considered to identify patients with passive diastolic dysfunction. Patients had higher levels of PIIINP, ICTP, MMP-2, MMP-9, and total TIMP-1 than did controls. PIIINP was inversely related to LV end-diastolic diameter. A−Ar was inversely related to PICP, PINP, and their differences with ICTP (estimates of collagen I buildup). Furthermore, A−Ar was directly related to MMP-1 and MMP-2. Conclusions— As compared with controls, collagen turnover is enhanced in HCM patients. As collagen I synthesis prevails over degradation and MMP-1 and MMP-2 are inhibited, passive diastolic dysfunction occurs in patients with HCM.

OBJECTIVES: We sought to assess the hemodynamics of exercise in patients with hypertrophic cardiomyopathy (HCM), with and without an exercise- induced abnormal blood pressure (BP) response, by ambulatory radionuclide monitoring of left ventricular (LV) function with the VEST device (Capintec Inc., Ramsey, New Jersey). BACKGROUND: Blood pressure fails to increase >20 mm Hg during exercise in about one-third of patients with HCM. This carries a high risk of sudden death. METHODS: Forty-three patients with HCM and 14 control subjects underwent maximal symptom- limited exercise on a treadmill during VEST. The VEST data were averaged for 1 min and analyzed at baseline, 3 min and peak exercise. The LV end- diastolic, end-systolic and stroke volumes, cardiac output and systemic vascular resistance were expressed as the percentage of baseline. RESULTS: Ejection fraction and stroke volume fell in patients with HCM, although they increased in control subjects (p < 0.001 and p = 0.002, respectively). Cardiac output increased significantly more in control subjects than in patients with HCM (p = 0.001). In 17 patients with HCM (39%) with an abnormal BP response, ejection fraction and stroke volume fell more (p = 0.032 and p = 0.009, respectively) and cardiac output increased less (p = 0.001) than they did in patients with HCM with a normal BP response. Systemic vascular resistance decreased similarly in patients with HCM, irrespective of the BP response. CONCLUSIONS: In patients with HCM with and without an abnormal BP response, abnormal hemodynamic adaptation to exercise was qualitatively similar but quantitatively different. An abnormal BP response was associated with exercise-induced LV systolic dysfunction. This causes hemodynamic instability, associated with a high risk of sudden cardiac death.

Objective: Redo operations for bioprosthesis malfunction can sometimes be technically very demanding and cardiac structures may be damaged. Excising only the leaflets of the damaged bioprosthesis and leaving the old ring in situ on which the ‘new’ mechanical valve is sutured can, in very selected cases, represent a solution. Methods: Twenty-two patients were operated on, with the valve-on-valve technique, from September 1991 through December 1992. There were three operative deaths. Results: The surviving 19 patients were followed-up from 83 to 98 months (mean 90.5 months.). There were two late deaths. The patients were examined clinically and with transthoracic and transesophageal echocardiograms. All patients were in good condition and the echocardiographic examinations showed no clinically important gradients across the prostheses. Conclusions: The valve-on-valve technique, in certain difficult situations, can give successful mid-term results. q 2000 Elsevier Science B.V. All rights reserved.

Background The origin of artifacts of the ascending aorta during transesophageal echocardiography has not been widely studied. This study was undertaken to investigate in vivo whether anatomic features could determine the appearance of artifacts. Methods and Results Transesophageal echocardiograms of 46 patients studied for suspected dissection with proven diagnosis (30 patients with and 16 without ascending aortic dissection) were reviewed. The incidence of artifacts was 46%, and it was similar in patients with and those without dissection (chi-square 0.516; P = not significant). Artifacts were located in the aortic lumen twice as far from the transducer as the atrial-aortic interface. The aortic diameter was larger in patients with than in those without artifacts (6.4 ± 1.1 vs 4.2 ± 0.9 cm, P 5 cm and an atrial-aortic ratio ≤0.6 predicted the artifact appearance with good sensitivity, specificity, positive and negative predictive values, and diagnostic accuracy; these parameters reached a value of 100% by analysis only of patients without dissection. Conclusions An ascending aortic diameter >5.0 cm that exceeds the left atrial diameter with an atrial-aortic ratio ≤0.6 creates in vivo the conditions for the reverberation of the atrial-aortic interface within the aorta. Therefore, in patients with such anatomic features, artifacts must be suspected in the presence of linear structures within the aorta. (Am Heart J 1999;137:967-73.)

The aim of this study was to assess whether left ventricular (LV) cavity size relates to functional impairment and syncope in patients with hypertrophic cardiomyopathy (HC). LV diastolic dysfunction influences functional limitation in HC. A reduced LV end-diastolic dimension may underlie impaired diastolic properties and be implicated in hemodynamic syncope. Eighty-two consecutive patients with HC (off drugs, in sinus rhythm) underwent echocardiography to measure LV end-diastolic dimension in the short-axis view (indexed to the body surface area) and radionuclide angiography (n = 50) to calculate peak filling rate (normalized to stroke counts/s). Patients in New York Heart Association functional classes II to IV had smaller LV end-diastolic dimension (23.2 +/- 2.6 vs 25.5 +/- 2.5 mm/M2, p = 0.0001) and lower peak filling rate (4.3 +/- 1.4 vs 5.1 +/- 1.3 stroke counts/s, p = 0.036) than those in New York Heart Association class I. LV end-diastolic diameter was correlated to peak filling rate (r = 0.37; p = 0.008). The most potent predictors of functional limitation were LV end-diastolic dimension (relative risk [RR] 0.63, confidence interval [CI] 0.45 to 0.88; p = 0.008), age (RR 1.09, CI 1.03 to 1.17; p = 0.003), and LV thickness score (RR 1.08, CI 1.02 to 1.13; p = 0.003). LV cavity size was smaller in patients with functional limitation irrespective of obstruction and hypertrophy. Patients with differed from those without a history of syncope for a smaller LV end-diastolic dimension (23.2 +/- 2.5 vs 25.0 +/- 2.7 mm/M2, p = 0.008), which was the only independent predictor of syncope (RR 0.77, CI 0.63 to 0.95; p = 0.013). Thus, a small LV cavity size is associated with functional limitation and history of syncope in HC.

Some studies have demonstrated that left ventricular (LV) diastolic function is the principal determinant of impaired exercise capacity in hypertrophic cardiomyopathy (HC). In this study we sought the capability of echocardiographic indexes of diastolic function in predicting exercise capacity in patients with HC. We studied 52 patients with HC while they were not on drugs;12 of them had LV tract obstruction at rest. Diastolic function was assessed by M-mode and Doppler echocardiography by measuring: (1) left atrial fractional shortening, and the slope of posterior aortic wall displacement during early atrial emptying on M-mode left atrial tracing; and (2) Doppler-derived transmitral and pulmonary venous flow velocity indexes. Exercise capacity was assessed by maximum oxygen consumption by cardiopulmonary test during cycloergometer upright exercise. Maximum oxygen consumption correlated with the left atrial fractional shortening (r = 0.63, p0.001), the slope of posterior aortic wall displacement during early atrial emptying (r = 0.55, p0.001), age (r = -0.50; p0.001), pulmonary venous diastolic anterograde velocity (r = 0.41, p0.01), and the systolic filling fraction (r = -0.43; p0.01). By stepwise multiple linear regression analysis, left atrial fractional shortening and the pulmonary venous systolic filling fraction were the only determinants of the maximum oxygen consumption (multiple r = 0.70; p0.001). Exercise capacity did not correlate with Doppler-derived transmitral indexes. Thus, in patients with HC, exercise capacity was determined by passive LV diastolic function, as assessed by the left atrial M-mode and Doppler-derived pulmonary venous flow velocities.