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Background: The early diastolic paradoxical midventricular flow is suggestive of apical aneurysm (AA) formation in hypertrophic cardiomyopathy (HCM). We aimed to determine whether early diastolic paradoxical midventricular flow may be a useful screening tool in patients, following the time progression of HCM to the aneurysmal stage. Methods: One hundred twenty-one HCM patients with dominant hypertrophy in the mid and apical segments, based on echocardiography and/or cardiovascular magnetic resonance, were selected from our HCM database, which comprises 1,332 patients. They were further stratified according to the presence of AA. All imaging studies in a period of 16 years (2005-2021) were considered for time progression. Midventricular Doppler (pulsed-wave, continuous-wave, color, and color M mode) were analyzed. Results: Thirty-five patients (29% of the study group and 2.6% of all HCM patients) had AA. Early diastolic paradoxical midventricular flow had a sensitivity of 92% and specificity of 98.6% for the detection of AA in the study group. In 108 patients, follow-up echocardiography was performed (median, 5 [3-9] studies). Sixteen patients (15%) with 10 [7-12] years of follow-up displayed progressive time changes in left ventricle (LV) apical morphology and/or mid-LV flow. Ten patients (9%) progressed to an AA, during 7 [4-11] years of follow-up. Patients progressing to AA were younger (P = .009), with more severe LV hypertrophy (P = .01) and more often a significant mid-LV systolic gradient (≥30 mm Hg, P < .001). A wall thickness over 20 mm had 70% sensitivity and 69% specificity in detecting evolution toward AA. With significant systolic gradient, sensitivity was 80% and specificity was 62%. Furthermore, patients with AA had a higher incidence of ventricular tachycardia (log-rank P = .03). Conclusions: Early diastolic paradoxical midventricular flow reliably detects AA presence and should prompt extra imaging studies. In HCM with mid and apical dominant involvement there is a progressive trend toward aneurysm formation, especially in patients with wall thickness over 20 mm and significant mid-LV systolic gradient (≥30 mm Hg), which can be monitored through serial Doppler studies.

BACKGROUND: Implantable cardioverter-defibrillators (ICDs) are frequently used for primary and secondary prevention in patients with cardiomyopathies due to different etiologies. However, long-term outcome studies in patients with noncompaction cardiomyopathy (NCCM) are scarce.OBJECTIVES: This study summarizes the long-term outcome of ICD therapy in patients with NCCM compared with those with dilated cardiomyopathy (DCM) or hypertrophic cardiomyopathy (HCM).METHODS: Prospective data from our single-center ICD registry were used to analyze the ICD interventions and survival in patients with NCCM (n = 68) compared with patients with DCM (n = 458) and patients with HCM (n = 158) from January 2005 to January 2018.RESULTS: This NCCM population with an ICD for primary prevention comprised 56 (82%) patients with a median age of 43 years and 52% males, compared with 85% in patients with DCM and 79% in patients with HCM (P = 0.20). During a median follow-up of 5 years (IQR: 2.0-6.9 years), appropriate and inappropriate ICD interventions were not significantly different. Nonsustained ventricular tachycardia during Holter monitoring in patients with NCCM was the only significant risk factor for appropriate ICD therapy in patients with NCCM, with a HR of 5.29 (95% CI: 1.12-24.96). The long-term survival was significantly better in the univariable analysis in the NCCM group. However, there was no difference in multivariable Cox regression analyses between the cardiomyopathy groups.CONCLUSIONS: At 5 years of follow-up, the rate of appropriate and inappropriate ICD interventions in NCCM was comparable to that in DCM or HCM. In multivariable analysis, no differences in survival were found between the cardiomyopathy groups.

Aims: The aim of the present study was to compare the rate of actionable arrhythmic events between patients with hypertrophic cardiomyopathy (HCM) who are monitored with an insertable cardiac monitor (ICM) or Holter monitoring. Methods: We studied 50 patients (mean age 52 years, 72% men) with HCM at low or intermediate risk for sudden cardiac death (SCD), of whom 25 patients received an ICM between November 2014 and February 2019. We retrospectively identified a control group of 25 patients who were matched on age, sex, and HCM Risk-SCD score category. The mean HCM Risk-SCD score was 3.41 ± 1.31 and 3.31 ± 1.43 for the ICM and Holter groups, respectively. The primary endpoint was an actionable event which was defined as an arrhythmic event resulting in a change in patient management. The secondary endpoint was the occurrence of ventricular tachycardia (VT). Results: The cumulative actionable event rate at 30 months was higher in the ICM group (51 vs. 27%, log-rank p value n = 3). Overall, 4 implantable cardioverter-defibrillators were implanted for primary prevention (n = 2 in each group). The cumulative rate of VT episodes at 30 months was similar between groups (23% [ICM group] vs. 42% [Holter group], log-rank p value = 0.71). Furthermore, the characteristics of VT were similar between groups with regard to the number of beats and rate. Conclusions: In adults with HCM, an ICM will detect more arrhythmic events requiring an intervention than a conventional Holter strategy. In contrast, the diagnostic yield of detecting VT seems similar for both groups.

Purpose Poor left ventricular (LV) function may affect the physiological intraventricular blood flow and physiological vortex formation. The aim of this study was to investigate the pattern of intraventricular blood flow dynamics in patients with LV assist devices (LVADs) using echocardiographic particle image velocimetry. Materials and methods This prospective study included 17 patients (mean age 57 ± 11 years, 82% male) who had received an LVAD (HeartMate 3, Abbott Laboratories, Chicago, Illinois, USA) because of end-stage heart failure and poor LV function. Eleven (64%) patients had ischemic cardiomyopathy, and six patients (36%) had nonischemic cardiomyopathy. All patients underwent echocardiography, including intravenous administration of an ultrasound-enhancing agent (SonoVue, Bracco, Milan, Italy). Echocardiographic particle image velocimetry was used to quantify LV blood flow dynamics, including vortex formation (Hyperflow software, Tomtec imaging systems Gmbh, Unterschleissheim, Germany). Results Contrast-enhanced ultrasound was well tolerated in all patients and was performed without adverse reactions or side effects. The LVAD function parameters did not change during or after the ultrasound examination. The LVAD flow was on average 4.3 ± 0.3 L/min, and the speed was 5247 ± 109 rotations/min. The quantification of LV intraventricular flow demonstrated substantial impairment of vortex parameters. The energy dissipation, vorticity, and kinetic energy fluctuation indices were severely impaired. Conclusions Echo particle velocimetry is safe and feasible for the quantitative assessment of intraventricular flow in patients with an LVAD. The intraventricular LV flow and vortex parameters are severely impaired in these patients.

BACKGROUND There is overlap in genetic causes and cardiac features in noncompaction cardiomyopathy (NCCM), hypertrophic cardiomyopathy (HCM), and dilated cardiomyopathy (DCM).OBJECTIVES The goal of this study was to predict phenotype and outcome in relatives according to the clinical features and genotype of NCCM index cases.METHODS Retrospective DNA and cardiac screening of relatives of 113 families from 143 index patients were used to classify NCCM cases according to the cardiac phenotype. These cases were classified as isolated NCCM, NCCM with left ventricular (LV) dilation (DCM), and NCCM with LV hypertrophy (HCM).RESULTS In 58 (51%) families, screening identified 73 relatives with NCCM and 34 with DCM or HCM without NCCM. The yield of family screening was higher in families with a mutation (p CONCLUSIONS The phenotype of relatives may be predicted according to the NCCM phenotype and the mutation of index patients. NCCM phenotypes were related to outcome. In this way, clinical and genetic features of index patients may help prediction of outcome in relatives. (C) 2019 by the American College of Cardiology Foundation.

Background: Sex disparities are common in hypertrophic cardiomyopathy (HCM). Previous research has shown that at time of myectomy, women are older, have greater impairment of diastolic function and more advanced cardiac remodeling. The clinical impact of these differences is unknown. Method: This study included 162 HCM patients (61% men) who underwent septal myectomy. Time to treatment was calculated in relation to symptom onset and diagnosis. Pre- and post-operative echocardiographic data were collected. Sex differences were assessed at baseline and in time-to-event survival analyses for the composite endpoint of all-cause mortality, cardiac transplantation, re-intervention and aborted sudden cardiac death. Results: Women were generally older at time of myectomy (57 vs. 49 years, p < 0.01), with similar time to treatment as measured from symptom onset (2.3 [1.3–6.0] vs. 2.8 [1.1–5.3] years, p > 0.05), but a shorter time since diagnosis compared to men (2.6 [1.2–7.0] vs. 4.3 [2.4–8.3] years, p = 0.02). Mean wall thickness and left atrial diameter were the same for men and women, but were higher in women when correcting for body surface area (absolute: 20 vs. 19 mm, 48 vs 46 mm, p ≥ 0.05; corrected: 9.7 vs. 11.2 mm/m2, 23.4 vs. 26.3 mm/m2, p < 0.01). After 5.9 [3.0–9.1] years, 15% of men and 8% of women had reached the composite endpoint (p > 0.05). Conclusion: In conclusion, although women present later in life and seem to have more advanced disease on echocardiography, time until myectomy was similar and clinical outcomes after myectomy are favourable for both men and women.

Pathogenic gene mutations are found in about 50% of patients with hypertrophic cardiomyopathy (HC). Previous studies have shown an association between sarcomere mutations and medium-term outcome. The association with long-term outcome has not been described. The aim of this cohort study was to assess the long-term outcomes of patients with genotype positive (G+) and genotype negative (G-) HC. The study population consisted of 626 patients with HC (512 probands and 114 relatives) who underwent phenotyping and genetic testing from 1985 to 2014. End points were all-cause mortality, cardiovascular (CV) mortality, heart failure (HF)-related mortality, and sudden cardiac death/aborted sudden cardiac death (SCD/aborted SCD). Kaplan-Meier and multivariate Cox regression analyses were performed. A pathogenic mutation was detected in 327 patients (52%). G+ probands were younger than G- probands (46 ± 15 vs 55 ± 15 years, p0.001), had more non sustained ventricular tachycardia (34% vs 13%; p0.001), more often a history of syncope (14% vs 7%; p = 0.016), and more extreme hypertrophy (maximal wall thickness ≥30 mm, 7% vs 1%; p0.001). G- probands were more symptomatic (New York Heart Association ≥II, 73% vs 53%, p0.001) and had higher left ventricular outflow tract gradients (42 ± 39 vs 29 ± 33 mm Hg, p = 0.001). During 12 ± 9 years of follow-up, G+ status was an independent risk factor for all-cause mortality (hazard ratio [HR] 1.90, 95% CI 1.14 to 3.15; p = 0.014), CV mortality (HR 2.82, 95% CI 1.49 to 5.36; p = 0.002), HF-related mortality (HR 6.33, 95% CI 1.79 to 22.41; p = 0.004), and SCD/aborted SCD (HR 2.88, 95% CI 1.23 to 6.71; p = 0.015). In conclusion, during long-term follow-up, patients with G+ HC are at increased risk of all-cause death, CV death, HF-related death, and SCD/aborted SCD.

Despite multiple studies on the diagnostic and prognostic use of dobutamine myocardial perfusion imaging (MPI), information on its long-term prognostic value is scarce. The aim of this study was to assess the value of this technique for the prediction of very long-term outcome. A total of 721 patients with limited exercise capacity underwent dobutamine MPI for the evaluation of suspected or known coronary artery disease. 719 of 721 patients attended follow-up (99.7%). Twenty-eight patients who underwent early coronary revascularization were excluded from analysis. Endpoints were all-cause mortality, cardiac death, nonfatal infarction, and coronary revascularization. Kaplan-Meier survival curves were constructed, and univariate and multivariate analyses were performed to identify predictors of long-term outcome. The mean age of patients was 60 +/- A 11 years, and 61% were male. Myocardial perfusion abnormalities were present in 381 patients (55%) and included fixed defects in 190 patients (27%) and reversible defects in 191 patients (28%). During a median follow-up time of 14 years (range 0-16), 295 deaths occurred (43%), of which 158 were cardiac deaths (23%). Nonfatal myocardial infarction occurred in 35 (5%), and late coronary revascularization was performed on 133 patients (19%). An abnormal dobutamine MPI provided significant prognostic information for prediction of cardiac death, hard cardiac events, and MACE, after adjustment for significant clinical variables and stress test variables. Dobutamine MPI provides incremental prognostic information for the prediction of cardiovascular outcomes in patients with limited exercise capacity. The long-term prognosis of patients with limited exercise capacity who have an abnormal dobutamine MPI is significantly worse than those with a normal MPI.

Objectives The aim of this study was to compare outcomes of alcohol septal ablation (ASA) in young and elderly patients with obstructive hypertrophic cardiomyopathy (HCM). Background The American College of Cardiology Foundation/American Heart Association guidelines reserve ASA for elderly patients and patients with serious comorbidities. Information on long-term age-specific outcomes after ASA is scarce. Methods This cohort study included 217 HCM patients (age 54 ± 12 years) who underwent ASA because of symptomatic left ventricular outflow tract obstruction. Patients were divided into young (age ≤55 years) and elderly (age >55 years) groups and matched by age in a 1:1 fashion to nonobstructive HCM patients. Results Atrioventricular block following ASA was more common in elderly patients (43% vs. 21%; p = 0.001), resulting in pacemaker implantation in 13% and 5%, respectively (p = 0.06). Residual left ventricular outflow tract gradient, post-procedural New York Heart Association functional class, and necessity for additional septal reduction therapy was comparable between age groups. During a follow-up of 7.6 ± 4.6 years, 54 patients died. The 5- and 10-year survival following ASA was 95% and 90% in patients age ≤55 years and 93% and 82% in patients age >55 years, which was comparable to their control groups. The annual adverse arrhythmic event rate following ASA was 0.7%/year in young patients and 1.4%/year in elderly patients, which was comparable to their control groups. Conclusions ASA is similarly effective for reduction of symptoms in young and elderly patients; however, younger patients have a lower risk of procedure-related atrioventricular conduction disturbances. The long-term mortality rate and risk of adverse arrhythmic events following ASA are low, both in young and elderly patients, and are comparable to age-matched nonobstructive HCM patients.

Patients with familial hypercholesterolemia (FH) have high levels of LDL-C, owing to defective uptake of these particles by LDL receptors in the liver. Consequently, FH patients have a high risk of cardiovascular disease (CVD). However, among these patients, there is marked variance in age of onset of CVD and a proportion of untreated patients do not develop CVD at all. Statin treatment can greatly reduce risk, and since it is not yet possible to precisely predict which FH patients will develop CVD, all patients initiate treatment once the diagnosis has been made. The purpose of this article is to provide an update of clinical and genetic risk factors influencing CVD risk in FH patients, and to discuss future lines of research that could uncover improved methods of treatment for heterozygous FH patients.

Severely symptomatic patients with obstructive hypertrophic cardiomyopathy (HC) may benefit from surgical myectomy. In patients with enlarged mitral leaflets and mitral regurgitation, myectomy can be combined with anterior mitral leaflet extension (AMLE) to stiffen the midsegment of the leaflet. The aim of this study was to evaluate the long-term results of myectomy combined with AMLE in patients with obstructive HC. This prospective, observational, single-center cohort study included 98 patients (49 +/- 14 years, 37% female) who underwent myectomy combined with AMLE from 1991 to 2012. End points included all-cause mortality and change in clinical and echocardiographic characteristics. Mortality was compared with age- and gender-matched patients with nonobstructive HC and subjects from the general population. Long-term follow-up was 8.3 +/- 6.1 years. There was no operative mortality, and New York Heart Association class was reduced from 2.8 +/- 0.5 to 1.3 +/- 0.5 (p

Although the mechanism of ischemic mitral regurgitation (MR) is understood, the echocardiographic picture of ischemic MR is not homogeneous. Ninety-two consecutive patients with chronic ischemic MR due to restricted motion were divided into two groups according to tethering pattern: the asymmetric group with predominant posterior tethering of both leaflets (54 patients) and the symmetric one with predominant apical tethering of both leaflets (38 patients). The mitral deformation indexes, LV global (volume, function and sphericity) and local (papillary muscle displacements and regional wall motion score index) remodeling were evaluated. All indexes of global LV remodeling were significantly higher in the symmetric than asymmetric group (all p

Hyperglycemia is associated with increased mortality in cardiac patients. However, the predictive value of admission- and average glucose levels in patients admitted to an intensive cardiac care unit (ICCU) has not been described.Observational study of patients admitted to the ICCU of a tertiary medical center in whom glucose levels were measured at and during admission. Over a 19-month period, 1713 patients were included. Mean age was 63±14 years, 1228 (72%) were male, 228 (17%) had known diabetes. Median (interquartile) glucose levels at admission were 7.9 (6.5-10.1) mmol/l; median glucose levels during ICCU admission (873 patients with three or more measurements) were 7.3 (6.7-8.3) mmol/l. Cox regression analysis was performed including the variables age, gender, admission diagnosis, length of stay, prior (cardio)vascular disease and diabetes.A 1 mmol/l increase in admission glucose level (above 9 mmol/l) was associated with a 10% (95% confidence interval (CI): 7 -13%) increased risk for all-cause mortality. A 1 mmol/l higher average glucose level (above 8 mmol/l) was an additional independent predictor of mortality (HR 1.11, 95% CI: 1.03 - 1.20). At 30 days, 16.8% (97/579) of the patients with an admission glucose level in the highest tertile (9.8 mmol/L) had died vs 5.2% (59/1134) of those with a lower admission glucose level.In a high risk ICCU population, both high admission glucose levels as well as high average glucose levels during hospitalization were independently associated with increased mortality, even when accounting for other risk factors and parameters of disease severity.

To the Editor: In a recent study by Blais and colleagues,1 the authors reported the use of dobutamine stress echocardiography (DSE) in patients with low-output, low-gradient aortic valve stenosis. Clinical decision making regarding these patients is challenging, and the clinical advantage of DSE is in distinguishing truly and pseudosevere aortic stenosis and evaluating contractile reserve.2–4 The authors have not made specific recommendations about the safety of DSE in these patients, but they have stated that “in a significant proportion of …

Objective: To assess whether tissue Doppler myocardial imaging (TDI) indices can predict postoperative left ventricular function in patients with mitral regurgitation (MR) after surgical correction. Methods: 84 patients ( mean (SD) age 54.3 (10.8) years) with asymptomatic severe MR, an end systolic diameter, 45 mm, and an ejection fraction (EF). 60% were subdivided in two groups: 43 patients with a postoperative EF reduction, 10% ( group 1) and 41 patients with a postoperative EF reduction greater than or equal to 10% ( group 2). TDI systolic indices of the lateral annulus were analysed preoperatively to assess myocardial systolic wave (Sm) velocity, myocardial precontraction time (PCTm), myocardial contraction time (CTm), and the PCTm: CTm ratio. Results: Postoperative EF decreased significantly ( from 67 ( 5)% to 60 (5.5)%, p = 0.0001). Group 2 had a higher PCTm, CTm, and PCTm: CTm ratio and a lower Sm velocity than group 1 ( PCTm 100.4 ( 19) ms v 82 (21.8) ms, p = 0.004; CTm 222 (3.1) ms v 215 (2.3) ms, p = 0.01; PCTm: CTm 0.45 (0.08) v 0.38 (0.09), p = 0.001; Sm velocity 10.4 (1.1) cm/s v 13 (1.3) cm/s, p = 0.0001). Multivariate regression analysis showed that the combination of PCTm: CTm ratio greater than or equal to 40 ms and Sm velocity less than or equal to10.5 cm/s was the main independent predictor of postoperative EF reduction greater than or equal to 10% ( sensitivity 78%, specificity 95%). Conclusions: TDI systolic indices can predict postoperative left ventricular function in patients with asymptomatic MR undergoing surgical correction. RI Schinkel, Arend/A-8673-2010 Objective: To assess whether tissue Doppler myocardial imaging (TDI) indices can predict postoperative left ventricular function in patients with mitral regurgitation (MR) after surgical correction. Methods: 84 patients ( mean (SD) age 54.3 (10.8) years) with asymptomatic severe MR, an end systolic diameter, 45 mm, and an ejection fraction (EF). 60% were subdivided in two groups: 43 patients with a postoperative EF reduction, 10% ( group 1) and 41 patients with a postoperative EF reduction greater than or equal to 10% ( group 2). TDI systolic indices of the lateral annulus were analysed preoperatively to assess myocardial systolic wave (Sm) velocity, myocardial precontraction time (PCTm), myocardial contraction time (CTm), and the PCTm: CTm ratio. Results: Postoperative EF decreased significantly ( from 67 ( 5)% to 60 (5.5)%, p = 0.0001). Group 2 had a higher PCTm, CTm, and PCTm: CTm ratio and a lower Sm velocity than group 1 ( PCTm 100.4 ( 19) ms v 82 (21.8) ms, p = 0.004; CTm 222 (3.1) ms v 215 (2.3) ms, p = 0.01; PCTm: CTm 0.45 (0.08) v 0.38 (0.09), p = 0.001; Sm velocity 10.4 (1.1) cm/s v 13 (1.3) cm/s, p = 0.0001). Multivariate regression analysis showed that the combination of PCTm: CTm ratio greater than or equal to 40 ms and Sm velocity less than or equal to10.5 cm/s was the main independent predictor of postoperative EF reduction greater than or equal to 10% ( sensitivity 78%, specificity 95%). Conclusions: TDI systolic indices can predict postoperative left ventricular function in patients with asymptomatic MR undergoing surgical correction.

Background: In ischaemic cardiomyopathy, raised plasma concentrations of natriuretic peptides are associated with a poor long term prognosis, while the presence of contractile reserve is a favourable sign.Objective: To assess the relation between plasma natriuretic peptides and contractile reserve.Design: Prospective observational study.Setting: Tertiary referral centre.Patients: 66 consecutive patients undergoing low dose dobutamine stress echocardiography to evaluate contractile reserve in regions with contractile dysfunction at rest, divided into two groups: group 1, 31 patients with ischaemic cardiomyopathy (left ventricular ejection fraction ⩽ 40%) and heart failure symptoms; group 2, 35 patients with normal left ventricular function.Main outcome measures: Plasma atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP), measured using immunoradiometric assays. Contractile reserve was defined as an improvement in segmental wall motion score during infusion of low dose dobutamine.Results: Plasma ANP and BNP concentrations were higher in group 1 than in group 2 (mean (SD): ANP, 17.8 (32.8) v 7.2 (9.7), p < 0.005; BNP, 24.4 (69.0) v 5.0 (14.3) pmol/l, respectively; p < 0.001). In group 1, the presence of contractile reserve was inversely related to ANP and BNP levels; however, patients with contractile reserve had lower ANP and BNP concentrations than patients without contractile reserve (ANP, 14.2 (9.1) v 24.2 (44.2), p < 0.05; BNP, 20.2 (25.5) v 37.5 (93.8) pmol/l, respectively; p < 0.05).Conclusions: Plasma natriuretic peptide concentrations are raised in patients with left ventricular dysfunction, but in the presence of preserved myocardial contractile reserve, relatively low levels of ANP and BNP are present.

Contrast-enhanced ultrasound (CEUS) is increasingly being used to evaluate patients with known or suspected atherosclerosis. The administration of a microbubble contrast agent in conjunction with ultrasound results in an improved image quality and provides information that cannot be assessed with standard B-mode ultrasound. CEUS is a high-resolution, noninvasive imaging modality, which is safe and may benefit patients with coronary, carotid, or aortic atherosclerosis. CEUS allows a reliable assessment of endocardial borders, left ventricular function, intracardiac thrombus and myocardial perfusion. CEUS results in an improved detection of carotid atherosclerosis, and allows assessment of high-risk plaque characteristics including intraplaque vascularization, and ulceration. CEUS provides real-time bedside information in patients with a suspected or known abdominal aortic aneurysm or aortic dissection. The absence of ionizing radiation and safety of the contrast agent allow repetitive imaging which is particularly useful in the follow-up of patients after endovascular aneurysm repair. New developments in CEUS-based molecular imaging will improve the understanding of the pathophysiology of atherosclerosis and may in the future allow to image and directly treat cardiovascular diseases (theragnostic CEUS). Familiarity with the strengths and limitations of CEUS may have a major impact on the management of patients with atherosclerosis.

Percutaneous transluminal septal myocardial ablation (PTSMA) using microsphere embolization instead of alcohol is a new interventional technique to treat patients with obstructive hypertrophic cardiomyopathy (HCM). Six HCM patients (age 68.4±6.3) with severe left ventricular outflow tract (LVOT)