Structural abnormalities in hypertrophic cardiomyopathy beyond left ventricular hypertrophy by multimodality imaging evaluation.

BACKGROUND: Echocardiography- and cardiovascular magnetic resonance (CMR)-based studies have revealed a wide range of phenotypic manifestations in hypertrophic cardiomyopathy (HCM) apart from hypertrophy. This study sought to comprehensively describe a number of structural abnormalities in HCM beyond hypertrophy, by multimodality imaging. METHODS: A total of 100 HCM patients were prospectively enrolled, undergoing standard and contrast echocardiography, and CMR. Morphological characteristics involving mitral valve leaflets (MVL), subvalvular apparatus, and left ventricular cavity and wall were investigated. Seventy healthy volunteers served as control population. RESULTS: As assessed by echo, MVLs were longer in HCM patients than in controls (anterior method 1: 24[22,28] vs 19[18,20] mm, P < 0.01; anterior method 2: 27[24, 29] vs 21[19, 23] mm, P < 0.01; posterior: 15[12,19] vs 14[13,15] mm, P < 0.01). Abnormal chordal attachment to anterior MVL, anterior papillary muscle displacement, and accessory apical-basal muscle bundle were present in 42 (42%), 61 (61%), and 35 (35%) patients, respectively (P values vs controls <0.01); direct papillary muscle insertion into MVL and hypertrabeculation were found in two and five patients, respectively. Contrast echocardiography (n = 94) detected myocardial crypts in 15 patients (16%). Overall, 83% of HCM subjects had at least one of these phenotypic manifestations. Echocardiography and CMR agreement for MVL length was poor, while for structural characteristics was moderate to substantial (Cohen's Kappa: 0.53-1.00). Except for posterior MVL length and hypertrabeculation, the phenotypic characteristics studied had acceptable reproducibility by echocardiography and CMR. CONCLUSIONS: Structural abnormalities in HCM beyond hypertrophy are significantly common. Multimodality imaging approach to these HCM facets by echocardiography and CMR is feasible and desirable.

Three-Dimensional Transesophageal Echocardiography in the Anatomical Assessment of Isolated Parachute Mitral Valve in an Adult Patient.

Parachute mitral valve (PMV) is a rare congenital anomaly of the mitral valve apparatus usually evidenced in infants and young children. Adult presentation is extremely rare and is generally mild in terms of mitral stenosis. A 73-year-old woman was admitted to the emergency department due to progressive dyspnea, with NYHA functional class IV symptoms on presentation. The echocardiographic examination identified a PMV with moderate mitral stenosis and a secondary smaller subvalvular mitral orifice. The report shows the usefulness of three-dimensional transesophageal echocardiography in the detection and quantification of this rare anomaly.

Prevalence of Fabry disease in patients with left ventricular hypertrophy and renal involvement (PrEFaCe). / Prevalencia de la enfermedad de Fabry en los pacientes con hipertrofia ventricular izquierda e insuficiencia renal (PrEFaCe).

INTRODUCTION AND AIMS: Fabry disease (FD) causes glycosphingolipid accumulation in the vascular endothelium, with predominantly cardiac and renal involvement. Its prevalence in patients with concomitant involvement of these two organs is unknown. The objective of the study was to determine the prevalence of FD in patients with left ventricular hypertrophy and any degree of chronic kidney disease. PATIENTS AND METHODS: Patients with ventricular thickness ≥13mm and kidney disease from 29 Spanish hospitals were included. Sociodemographic variables and target organ involvement of FD were collected. Laboratory determinations of EF were carried out, with an enzymatic activity test±genetic test in men and direct genetic test in women. RESULTS: Eight hundred ninety-eight patients with left ventricular hypertrophy and chronic kidney disease were included. The presence of heart failure and cardiorenal syndrome was common (46.1% and 40.1%). Three patients (2 men and 1 woman) were diagnosed with FD, based on the presence of a pathogenic variant in the GLA gene and classic signs of FD, resulting in a prevalence of 0.33% (CI 95% 0.06-1%). Six patients (0.66%) presented genetic variants of unknown significance, without showing classic signs of FD, while in 13 patients (3.2%) performing the blood test was impossible. CONCLUSIONS: FD is an important cause of left ventricular hypertrophy and chronic kidney disease. Genetic diagnosis is crucial for avoiding biases and ensuring accurate identification of FD, especially in women. The results support the inclusion of this disease in the differential diagnosis of patients with ventricular hypertrophy ≥13mm and chronic kidney disease.

Beta-blockers and renin-angiotensin system inhibitors for Takotsubo syndrome recurrence: a network meta-analysis.

INTRODUCTION: Takotsubo syndrome (TTS) is an acute heart failure syndrome, featured by transient left ventricular systolic dysfunction. Recurrences of TTS are not infrequent and there is no standard preventive therapy. The aim of this study was to evaluate in a network meta-analysis if beta-blockers (BB) and ACE inhibitors/angiotensin receptor blockers (ACEi/ARBs), in combination or not, can effectively prevent TTS recurrences. METHODS: We performed a systematic network meta-analysis, using MEDLINE/EMBASE and the Cochrane Central Register of Controlled Trials for clinical studies published between January 2010 and September 2022. We considered all those studies including patients receiving medical therapy with BB, ACEi/ARBs. The primary outcome was TTS recurrence. RESULTS: We identified 6 clinical studies encompassing a total of 3407 patients with TTS. At 40±10 months follow-up, TTS recurrence was reported in 160 (4.7%) out of 3407 patients. Mean age was 69.8±2 years and 394 patients (11.5%) out of 3407 were male. There were no differences in terms of TTS recurrence when comparing ACEi/ARBs versus control (OR 0.83; 95% CI 0.47 to 1.47, p=0.52); BB versus control (OR 1.01; 95% CI 0.63 to 1.61, p=0.96) and ACEi/ARBs versus BB (OR 0.88; 95% CI 0.51 to 1.53, p=0.65).Combination of BB and ACEi/ARBs was also not effective in reducing the risk of recurrence versus control (OR 0.91; 95% CI 0.58 to 1.43, p=0.68) vs ACEi/ARBs (OR 0.79; 95% CI 0.46 to 1.34, p=0.38)) and vs BB (OR 0.77; 95% CI 0.49 to 1.21, p=0.26). CONCLUSIONS: Our study did not find sufficient statistical evidence regarding combination therapy with BB and ACEi/ARBs in reduction of TTS recurrence.

Contrast-Enhanced Echocardiographic Measurement of Left Ventricular Wall Thickness in Hypertrophic Cardiomyopathy: Comparison with Standard Echocardiography and Cardiac Magnetic Resonance.

BACKGROUND: Left ventricular wall thickness (LVWT) measurement is key in the diagnostic and prognostic assessment of hypertrophic cardiomyopathy (HCM). Recent investigations have highlighted discrepancies in LVWT by cardiac magnetic resonance (CMR) and standard echocardiography (S-Echo) in this condition. The aim of this study was to elucidate the role of contrast-enhanced echocardiography (C-Echo) to optimize LVWT measurement in patients with HCM. METHODS: Fifty patients with HCM were prospectively enrolled, undergoing S-Echo, C-Echo, and CMR. Blinded LVWT measurements were performed according to a 16-segment left ventricular model using all three imaging techniques. Agreement between both echocardiographic modalities and CMR (as the reference technique) at the segmental level was tested using Bland-Altman analyses. Reproducibility on segmental measurements by S-Echo and C-Echo was also investigated. RESULTS: Patients' mean age was 47 ± 21 years, and 35 (70%) were men. Maximal mean LVWT by S-Echo (20.1 ± 3.8 mm) was greater than the values derived using C-Echo (17.6 ± 4.0 mm, P < .01) and CMR (17.7 ± 4.5 mm, P < .01), with no statistically significant difference between the latter two. Segmental Bland-Altman models demonstrated globally smaller bias and narrower 95% limits of agreement for C-Echo compared with S-Echo. Across all left ventricular segments, LVWT by C-Echo was 2.4 mm lower (range, 1.0-2.5 mm) than that derived by S-Echo, which accounted for a 25% intertechnique difference. Regarding maximal LVWT, the mean absolute difference between C-Echo and S-Echo was 3.0 ± 1.9 mm (range, 0.0-7.9 mm), which represented a 15% intertechnique change. Data analyses demonstrated globally less intra- and interobserver variability in segmental LVWT derived from C-Echo compared with S-Echo. CONCLUSIONS: C-Echo rendered LVWT measurements closer to those derived by the reference technique (CMR) and improved reproducibility compared with S-Echo. C-Echo represents a suitable tool for LVWT measurement in patients with HCM as an alternative to CMR whenever this is not available or possible.

Clinical and echocardiographic course in tako-tsubo cardiomyopathy: Long-term follow-up from a multicenter study.

OBJECTIVES: To jointly describe clinical characteristics, ECG and echocardiographic findings, and adverse cardiovascular events in patients with tako-tsubo cardiomyopathy (TC) in the long-term. METHODS: Longitudinal multicenter study including retrospective analysis of clinical and ECG data, and follow-up evaluation with clinical interview, electrocardiogram and echocardiogram. RESULTS: Data from 66 cases of TC were available for analysis of clinical and adverse cardiovascular events, and 56 of them completed the follow-up visit including electrocardiogram and echocardiogram. Most patients (97%) were asymptomatic or oligosymptomatic (NYHA I [58%] or II [39%], respectively) at follow-up (median time: 3.7 [1.8-6.6] years). The vast majority of individual QRS complex and repolarization abnormalities had disappeared (87% with no ECG abnormalities at follow-up). On echocardiography, left ventricular ejection fraction was ≥50% in all patients (mean: 63±6%). Wall motion abnormalities were observed in 4 patients (7%; 3 with apical wall motion abnormalities and 1 with mild global hypokinesia). Long-term outcomes were as follows: 4 deaths (6%), 2 cardiovascular and 2 non-cardiovascular; no atrial fibrillation development; no stroke events; 5 acute recurrence events of TC (8%). Globally, 57 patients (86%) had a clinical course free from adverse cardiovascular events. CONCLUSIONS: After a long period following the admission event, patients discharged from TC remain asymptomatic or minimally symptomatic, and feature a low prevalence of both ECG and left ventricular wall motion abnormalities; moreover, the latter lead to a very mild impairment of ejection fraction. Among cardiovascular adverse events, recurrence of the TC event appears to play the most significant role.

Direct oral anticoagulants in patients with hypertrophic cardiomyopathy and atrial fibrillation.

BACKGROUND: Chronic anticoagulation with vitamin K antagonists (VKAs) is recommended in patients with hypertrophic cardiomyopathy (HCM) and atrial fibrillation (AF). Direct oral anticoagulants (NOACs) are an alternative to VKAs but there are limited data to support their use in HCM. We sought to describe the pattern of use, thromboembolic events, bleeding and quality of life in patients with HCM and AF treated with NOACs. METHODS: Data from patients treated with NOACs (n=99) and VKA (n=433) at 9 inherited cardiac diseases units were retrospectively collected. Annual rates of embolic events, serious bleeding and death were analysed and compared. Quality of life and treatment satisfaction were evaluated with SF-36 and SAFUCA questionnaires in 80 NOAC-treated and 57 VKA-treated patients. RESULTS: After median follow-up of 63 months (IQR: 26-109), thromboembolic events (TIA/stroke and peripheral embolism) occurred in 10% of patients on oral anticoagulation. Major/clinically relevant bleeding occurred in 3.8% and the global mortality rate was 23.3%. Thromboembolic event rate was 0.62 per 100patient-years in the NOAC group vs. 1.59 in the VKA group [subhazard ratio (SHR) 0.32;95%CI:0.04-2.45; p=0.27]. Major/clinically relevant bleeding occurred in 0.62 per 100person-years in the NOAC group vs. 0.60 in the VKA group (SHR 1.28;95%CI 0.18-9.30; p=0.85). Quality of life scores were similar in both groups; however, NOAC-treated patients achieved higher scores in the SAFUCA. CONCLUSIONS: HCM patients with AF on NOACs showed similar embolic and bleeding rates to those on VKA. Although quality of life was similar in both groups, the NOAC group reported higher treatment satisfaction.

Three-dimensional Speckle Tracking Echocardiography in Light Chain Cardiac Amyloidosis: Examination of Left and Right Ventricular Myocardial Mechanics Parameters.

INTRODUCTION AND OBJECTIVES: The study of myocardial mechanics has a potential role in the detection of cardiac involvement in patients with amyloidosis. This study aimed to characterize 3-dimensional-speckle tracking echocardiography-derived left and right ventricular myocardial mechanics in light chain amyloidosis and examine their relationship with brain natriuretic peptide. METHODS: In patients with light chain amyloidosis, left ventricular longitudinal and circumferential strain (n=40), and right ventricular longitudinal strain and radial displacement (n=26) were obtained by 3-dimensional-speckle tracking echocardiography. Brain natriuretic peptide levels were determined. RESULTS: All myocardial mechanics measurements showed differences when compared by brain natriuretic peptide level tertiles. Left and right ventricular longitudinal strain were highly correlated (r=0.95, P<.001). Left ventricular longitudinal and circumferential strain were reduced in patients with cardiac involvement (-9±4 vs -16±2; P<.001, and -24±6 vs -29±4; P=.01, respectively), with the most prominent impairment at the basal segments. Right ventricular longitudinal strain and radial displacement were diminished in patients with cardiac involvement (-9±3 vs -17±3; P<.001, and 2.7±0.8 vs 3.8±0.3; P=.002). On multivariate analysis, left ventricular longitudinal strain was associated with the presence of cardiac involvement (odds ratio = 1.6; 95% confidence interval, 1.04 to 2.37; P=.03) independent of the presence of brain natriuretic peptide and troponin I criteria for cardiac amyloidosis. CONCLUSIONS: Three-dimensional-speckle tracking echocardiography-derived left and right ventricular myocardial mechanics are increasingly altered as brain natriuretic peptide increases in light chain amyloidosis. There appears to be a strong association between left ventricular longitudinal strain and cardiac involvement, beyond biomarkers such as brain natriuretic peptide and troponin I.

Investigation of global and regional myocardial mechanics with 3-dimensional speckle tracking echocardiography and relations to hypertrophy and fibrosis in hypertrophic cardiomyopathy.

BACKGROUND: In hypertrophic cardiomyopathy (HCM), heterogeneous myocardial hypertrophy and fibrosis are responsible for abnormalities of left ventricular (LV) function. We aimed to characterize LV global and regional myocardial mechanics in HCM, according to segmental hypertrophy and fibrosis. METHODS AND RESULTS: Fifty-nine patients with HCM underwent standard echocardiography, 3-dimensional speckle tracking echocardiography, and cardiac magnetic resonance with late gadolinium enhancement (LGE); all 3 tests were <24 hours apart. Longitudinal, circumferential, and area strains were investigated according to the extent of LGE (no LGE, LGE<10%, and LGE≥10%), segmental thickness (≥15 versus <15 mm), and segmental LGE (LGE versus non-LGE). Attenuated global longitudinal strain showed association with extent of hypertrophy (indexed LV mass, r=0.32, P=0.01; maximum LV wall thickness, r=0.34, P=0.009; number of segments≥15 mm, r=0.44, P<0.001), whereas enhanced global circumferential strain was correlated to LV global functional parameters (indexed end-systolic volume, r=0.47, P<0.001; ejection fraction, r=-0.75, P<0.001). Parameters of global myocardial mechanics showed no association with the extent of LGE; in contrast, the extent of LGE was associated with the extent of hypertrophy. All 3 deformation parameters were attenuated both in segments≥15 mm in thickness and in those with LGE; adjusted analysis demonstrated that segmental presence of LGE was associated with additional attenuation in myocardial deformation. CONCLUSIONS: Both hypertrophy and fibrosis contribute to regional impairment of myocardial shortening in HCM. The extent of hypertrophy is the primary factor altering global myocardial mechanics. Circumferential myocardial shortening seems to be directly involved in preservation of LV systolic performance in HCM.

Evaluation of myocardial mechanics with three-dimensional speckle tracking echocardiography in heart transplant recipients: comparison with two-dimensional speckle tracking and relationship with clinical variables.

AIMS: Two-dimensional speckle-tracking echocardiography (2D-STE) is limited by its inability to track tissue motion in three dimensions. This is particularly relevant in heart transplant recipients, in whom marked translational motion of the transplanted heart is present. We aimed to compare 3-dimensional (3D)- and 2D-STE-derived strain parameters, and to identify clinical features associated with myocardial mechanics in transplant recipients. METHODS AND RESULTS: In 36 heart transplant recipients, global and regional left-ventricular (LV) longitudinal and circumferential strain (LSt and CSt), and radial displacement (RDisp) were obtained by 3D- and 2D-STE, and their results were compared. 3D-STE deformation from a subset of transplant recipients with preserved ejection fraction was compared with a control group of 25 subjects matched by gender, age, history of hypertension, and ejection fraction. Associations between global LSt and CSt and clinical, echocardiographic, and haemodynamic parameters in transplant recipients were investigated. 3D-STE yielded lower magnitude of global LSt compared with 2D-STE (-13 ± 3 vs. -16 ± 3%, P < 0.001). The inferolateral wall was a source of variation between 3D- and 2D-STE both for LSt and CSt. Inferolateral wall 3D-STE-derived RDisp was greater than that observed in control subjects (7.4 ± 1.2 vs. 6.5 ± 1.7 mm, P = 0.03), while anteroseptal RDisp was lower than controls (4.2 ± 1.0 vs. 7.3 ± 1.6 mm, P < 0.001). Multiple regression analysis demonstrated that 3D-STE-derived LSt was independently associated with NYHA class (P < 0.001), while 2D-STE-derived LSt was not. CONCLUSION: Examination of LV mechanics by 3D- and 2D-STE deformation parameters in heart transplant recipients yields significantly discordant results. 3D-STE-derived LSt is independently associated with NYHA class, suggesting a clinically important relationship between functional status and myocardial mechanics.

Prognostic value of different serum biomarkers for left ventricular remodelling after ST-elevation myocardial infarction treated with primary percutaneous coronary intervention.

BACKGROUND: Mortality derived from ST-elevation myocardial infarction (STEMI) has decreased due to primary percutaneous coronary intervention (PCI). Paradoxically, the incidence of heart failure secondary to left ventricular remodelling (LVR) is on the rise due to the survival derived from reperfusion strategies. The aim of this study was to assess the prognostic value for LVR of biomarkers involved in several pathophysiological mechanisms activated during STEMI treated with primary PCI. METHODS: 112 patients with STEMI undergoing primary PCI were evaluated. LVR was defined as a ≥20% increase in the left ventricular end-diastolic volume at 6-month follow-up assessed using echocardiogram as compared with that at discharge. Blood samples were obtained for glucose, N-terminal pro-brain natriuretic peptide, troponin T (TnT), matrix metalloproteinase 9, procollagen type-I N-terminal propeptide and high-sensitivity C reactive protein (hs-CRP). RESULTS: 24 patients (21%) developed LVR. Higher levels of maximum TnT, and matrix metalloproteinase 9 and hs-CRP at discharge, were detected as independent risk factors for LVR (OR 1.310, p=0.03; OR 1001, p=0.04; OR 1.040, p=0.04, respectively). Both TnT and hs-CRP showed significant ability to distinguish patients who developed LVR from those who did not, being the values that yielded the greatest sensitivity and specificity as follows: TnT 7.0 µg/l (73%, 84%), hs-CRP 30 mg/l (59%, 85%). CONCLUSIONS: Myocardial necrosis, as measured by released TnT, and inflammation state evident due to circulating levels of CRP are factors that may play a major role in the development of LVR following STEMI treated with primary PCI.