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2.
Echocardiography ; 41(5): e15834, 2024 May.
Article En | MEDLINE | ID: mdl-38784981

OBJECTIVES: Endocardial global longitudinal strain (endo-GLS) measured with echocardiography (echo) has been demonstrated to be associated with myocardial fibrosis (MF) and is a prognostic predictor in patients with hypertrophic cardiomyopathy (HCM). Late gadolinium enhancement cardiac magnetic resonance (LGE-CMR) imaging showed that MF is primarily located in the myocardial layer of the extremely hypertrophic septal or ventricular wall. We hypothesized that GLS of the myocardial layer (myo-GLS) is more strongly correlated with the extent of LGE (%LGE) and is a more powerful prognostic factor than endo-GLS. METHODS: A total of 177 inpatients (54.0 [IQR: 43.0, 64.0] years, female 37.3%) with HCM were retrospectively included from May 2019 to April 2021. Among them, 162 patients underwent echocardiographic examination and contrast-enhanced CMR within 7 days. Myo-GLS and %LGE were blindly assessed in a core laboratory. All the patients were followed after they were discharged. RESULTS: During a mean follow-up of 33.77 [IQR 30.05, 35.40] months, 14 participants (7.91%) experienced major adverse cardiac events (MACE). The MACE (+) group showed lower absolute endo-GLS and myo-GLS than the MACE (-) group. Myo-GLS was more associated with %LGE (r = -.68, P < .001) than endo-GLS (r = -.64, P < .001). Cox multivariable analysis indicated that absolute myo-GLS was independently associated with MACE (adjusted hazard ratio = .75, P < .05). Myo-GLS was better than endo-GLS at detecting MACE (+) patients (-8.64%, AUC .939 vs. - 16.375%, AUC .898, P < .05). CONCLUSIONS: Myo-GLS is a stronger predictor of MACE than endo-GLS in patients with HCM and is highly correlated with %LGE.


Cardiomyopathy, Hypertrophic , Echocardiography , Magnetic Resonance Imaging, Cine , Humans , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/physiopathology , Female , Male , Middle Aged , Retrospective Studies , Magnetic Resonance Imaging, Cine/methods , Echocardiography/methods , Adult , Prognosis , Predictive Value of Tests , Contrast Media , Heart Ventricles/diagnostic imaging , Heart Ventricles/physiopathology , Follow-Up Studies , Myocardium/pathology , Global Longitudinal Strain
3.
Circ Heart Fail ; 17(5): e011435, 2024 May.
Article En | MEDLINE | ID: mdl-38695186

Hypertrophic cardiomyopathy is the most common genetic cardiac disease and is characterized by left ventricular hypertrophy. Although this hypertrophy often associates with sarcomeric gene mutations, nongenetic factors also contribute to the disease, leading to diastolic dysfunction. Notably, this dysfunction manifests before hypertrophy and is linked to hypercontractility, as well as nonuniform contraction and relaxation (myofibril asynchrony) of the myocardium. Although the distribution of hypertrophy in hypertrophic cardiomyopathy can vary both between and within individuals, in most cases, it is primarily confined to the interventricular septum. The reasons for septal thickening remain largely unknown. In this article, we propose that alterations in muscle fiber geometry, present from birth, dictate the septal shape. When combined with hypercontractility and exacerbated by left ventricular outflow tract obstruction, these factors predispose the septum to an isometric type of contraction during systole, consequently constraining its mobility. This contraction, or more accurately, this focal increase in biomechanical stress, prompts the septum to adapt and undergo remodeling. Drawing a parallel, this is reminiscent of how earthquake-resistant buildings are retrofitted with vibration dampers to absorb the majority of the shock motion and load. Similarly, the heart adapts by synthesizing viscoelastic elements such as microtubules, titin, desmin, collagen, and intercalated disc components. This pronounced remodeling in the cytoskeletal structure leads to noticeable septal hypertrophy. This structural adaptation acts as a protective measure against damage by attenuating myofibril shortening while reducing cavity tension according to Laplace Law. By examining these events, we provide a coherent explanation for the septum's predisposition toward hypertrophy.


Cardiomyopathy, Hypertrophic , Humans , Cardiomyopathy, Hypertrophic/physiopathology , Myocardial Contraction/physiology , Animals , Ventricular Remodeling/physiology , Heart Septum/physiopathology , Heart Septum/diagnostic imaging , Heart Septum/pathology , Ventricular Septum/physiopathology , Ventricular Septum/diagnostic imaging
4.
J Am Heart Assoc ; 13(9): e032553, 2024 May 07.
Article En | MEDLINE | ID: mdl-38700034

BACKGROUND: Previous studies have shown that women with hypertrophic obstructive cardiomyopathy (HCM) have worse long-term outcomes irrespective of intervention. However, the outcomes of patients undergoing alcohol septal ablation (ASA) based on sex have not been described. Hence, this study aimed to evaluate pressure changes and long-term mortality in patients with HCM undergoing ASA based on sex. METHODS AND RESULTS: This is a single-center retrospective study evaluating hemodynamic changes and long-term mortality in patients with HCM treated with ASA according to sex. A total of 259 patients were included (aged 68.4±11.9 years, 62.2% women). Women had higher age and baseline pressures at the time of ASA, with a greater percent reduction in mean left atrial pressure (men versus women: 2.2% versus 15.9%, respectively; P=0.02). Women had better survival (median survival rate of men versus women: 8.6 versus 12.5 years, respectively; P=0.011). On Cox multivariable regression, predictors of mortality were age (per group change <60 years, 61-70 years, 71-80 years, and >80 years; hazard ratio [HR], 1.45 [95% CI, 1.10-1.91], P=0.008), female sex (HR, 0.59 [95% CI, 0.35-0.99], P=0.048), chronic kidney disease (HR, 1.88 [95% CI, 1.06-3.33], P=0.031), and left ventricular outflow tract gradient reduction ≤86% (HR, 1.91 [95% CI, 1.14-3.19], P=0.014). CONCLUSIONS: Women with HCM undergoing ASA are older and have higher left-sided baseline pressures compared with men yet have better survival. Further studies exploring the mechanisms of differential outcomes according to sex in patients with HCM undergoing ASA are needed.


Ablation Techniques , Cardiomyopathy, Hypertrophic , Ethanol , Humans , Cardiomyopathy, Hypertrophic/surgery , Cardiomyopathy, Hypertrophic/mortality , Cardiomyopathy, Hypertrophic/physiopathology , Cardiomyopathy, Hypertrophic/complications , Female , Male , Aged , Retrospective Studies , Ethanol/adverse effects , Middle Aged , Sex Factors , Ablation Techniques/methods , Aged, 80 and over , Treatment Outcome , Heart Septum/surgery , Risk Factors , Time Factors , Age Factors
5.
BMC Cardiovasc Disord ; 24(1): 255, 2024 May 16.
Article En | MEDLINE | ID: mdl-38755595

BACKGROUND: Ventricular tachycardia (VT) is the primary cause of sudden cardiac death in patients with hypertrophic cardiomyopathy (HCM). However, the strategy for VT treatment in HCM patients remains unclear. This study is aimed to compare the effectiveness of catheter ablation versus antiarrhythmic drug (AAD) therapy for sustained VT in patients with HCM. METHODS: A total of 28 HCM patients with sustained VT at 4 different centers between December 2012 and December 2021 were enrolled. Twelve underwent catheter ablation (ablation group) and sixteen received AAD therapy (AAD group). The primary outcome was VT recurrence during follow-up. RESULTS: Baseline characteristics were comparable between two groups. After a mean follow-up of 31.4 ± 17.5 months, the primary outcome occurred in 35.7% of the ablation group and 90.6% of the AAD group (hazard ratio [HR], 0.29 [95%CI, 0.10-0.89]; P = 0.021). No differences in hospital admission due to cardiovascular cause (25.0% vs. 71.0%; P = 0.138) and cardiovascular cause-related mortality/heart transplantation (9.1% vs. 50.6%; P = 0.551) were observed. However, there was a significant reduction in the composite endpoint of VT recurrence, hospital admission due to cardiovascular cause, cardiovascular cause-related mortality, or heart transplantation in ablation group as compared to that of AAD group (42.9% vs. 93.7%; HR, 0.34 [95% CI, 0.12-0.95]; P = 0.029). CONCLUSIONS: In HCM patients with sustained VT, catheter ablation reduced the VT recurrence, and the composite endpoint of VT recurrence, hospital admission due to cardiovascular cause, cardiovascular cause-related mortality, or heart transplantation as compared to AAD.


Anti-Arrhythmia Agents , Cardiomyopathy, Hypertrophic , Catheter Ablation , Recurrence , Tachycardia, Ventricular , Humans , Tachycardia, Ventricular/diagnosis , Tachycardia, Ventricular/physiopathology , Tachycardia, Ventricular/mortality , Tachycardia, Ventricular/therapy , Tachycardia, Ventricular/etiology , Tachycardia, Ventricular/surgery , Anti-Arrhythmia Agents/therapeutic use , Anti-Arrhythmia Agents/adverse effects , Catheter Ablation/adverse effects , Catheter Ablation/mortality , Male , Female , Middle Aged , Cardiomyopathy, Hypertrophic/mortality , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/physiopathology , Cardiomyopathy, Hypertrophic/surgery , Cardiomyopathy, Hypertrophic/therapy , Treatment Outcome , Time Factors , Adult , Retrospective Studies , Risk Factors , Aged , Heart Rate , China
6.
J Am Heart Assoc ; 13(9): e032520, 2024 May 07.
Article En | MEDLINE | ID: mdl-38686858

BACKGROUND: Symptomatic limitations in apical hypertrophic cardiomyopathy may occur because of diastolic dysfunction with resultant elevated left ventricular filling pressures, cardiac output limitation to exercise, pulmonary hypertension (PH), valvular abnormalities, and/or arrhythmias. In this study, the authors aimed to describe invasive cardiac hemodynamics in a cohort of patients with apical hypertrophic cardiomyopathy. METHODS AND RESULTS: Patients presenting to a comprehensive hypertrophic cardiomyopathy center with apical hypertrophic cardiomyopathy were identified (n=542) and those who underwent invasive hemodynamic catheterization (n=47) were included in the study. Of these, 10 were excluded due to postmyectomy status or incomplete hemodynamic data. The mean age was 56±18 years, 16 (43%) were women, and ejection fraction was preserved (≥50%) in 32 (91%) patients. The most common indication for catheterization was dyspnea (48%) followed by suspected PH (13%), and preheart transplant evaluation (10%). Elevated left ventricular filling pressures at rest or exercise were present in 32 (86%) patients. PH was present in 30 (81%) patients, with 6 (20%) also having right-sided heart failure. Cardiac index was available in 25 (86%) patients with elevated resting filling pressures. Of these, 19 (76%) had reduced cardiac index and all 6 with right-sided heart failure had reduced cardiac index. Resting hemodynamics were normal in 8 of 37 (22%) patients, with 5 during exercise; 3 of 5 (60%) patients had exercise-induced elevation in left ventricular filling pressures. CONCLUSIONS: In patients with apical hypertrophic cardiomyopathy undergoing invasive hemodynamic cardiac catheterization, 86% had elevated left ventricular filling pressures at rest or with exercise, 81% had PH, and 20% of those with PH had concomitant right-sided heart failure.


Cardiac Catheterization , Cardiomyopathy, Hypertrophic , Hemodynamics , Humans , Female , Cardiomyopathy, Hypertrophic/physiopathology , Cardiomyopathy, Hypertrophic/complications , Middle Aged , Male , Aged , Hemodynamics/physiology , Adult , Ventricular Function, Left/physiology , Stroke Volume/physiology , Retrospective Studies , Hypertension, Pulmonary/physiopathology , Hypertension, Pulmonary/diagnosis , Apical Hypertrophic Cardiomyopathy
7.
Europace ; 26(4)2024 Mar 30.
Article En | MEDLINE | ID: mdl-38588067

AIMS: Typical electrocardiogram (ECG) features of apical hypertrophic cardiomyopathy (ApHCM) include tall R waves and deep or giant T-wave inversion in the precordial leads, but these features are not always present. The ECG is used as the gatekeeper to cardiac imaging for diagnosis. We tested whether explainable advanced ECG (A-ECG) could accurately diagnose ApHCM. METHODS AND RESULTS: Advanced ECG analysis was performed on standard resting 12-lead ECGs in patients with ApHCM [n = 75 overt, n = 32 relative (<15 mm hypertrophy); a subgroup of which underwent cardiovascular magnetic resonance (n = 92)], and comparator subjects (n = 2449), including healthy volunteers (n = 1672), patients with coronary artery disease (n = 372), left ventricular electrical remodelling (n = 108), ischaemic (n = 114) or non-ischaemic cardiomyopathy (n = 57), and asymmetrical septal hypertrophy HCM (n = 126). Multivariable logistic regression identified four A-ECG measures that together discriminated ApHCM from other diseases with high accuracy [area under the receiver operating characteristic (AUC) curve (bootstrapped 95% confidence interval) 0.982 (0.965-0.993)]. Linear discriminant analysis also diagnosed ApHCM with high accuracy [AUC 0.989 (0.986-0.991)]. CONCLUSION: Explainable A-ECG has excellent diagnostic accuracy for ApHCM, even when the hypertrophy is relative, with A-ECG analysis providing incremental diagnostic value over imaging alone. The electrical (ECG) and anatomical (wall thickness) disease features do not completely align, suggesting that future diagnostic and management strategies may incorporate both features.


Cardiomyopathy, Hypertrophic , Electrocardiography , Humans , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/physiopathology , Electrocardiography/methods , Male , Female , Middle Aged , Aged , Predictive Value of Tests , Reproducibility of Results , Adult , ROC Curve , Logistic Models , Case-Control Studies , Multivariate Analysis , Magnetic Resonance Imaging , Area Under Curve , Diagnosis, Differential , Ventricular Remodeling , Apical Hypertrophic Cardiomyopathy
8.
Catheter Cardiovasc Interv ; 103(6): 1023-1034, 2024 May.
Article En | MEDLINE | ID: mdl-38639143

BACKGROUND: The clinical efficacy and safety of alcohol septal ablation (ASA) for obstructive hypertrophic cardiomyopathy (HCM) have been well-established; however, less is known about outcomes in patients undergoing preemptive ASA before transcatheter mitral valve replacement (TMVR). AIMS: The goal of this study is to characterize the procedural characteristics and examine the clinical outcomes of ASA in both HCM and pre-TMVR. METHODS: This retrospective study compared procedural characteristics and outcomes in patient who underwent ASA for HCM and TMVR. RESULTS: In total, 137 patients were included, 86 in the HCM group and 51 in the TMVR group. The intraventricular septal thickness (mean 1.8 vs. 1.2 cm; p < 0.0001) and the pre-ASA LVOT gradient (73.6 vs. 33.8 mmHg; p ≤ 0.001) were higher in the HCM group vs the TMVR group. The mean volume of ethanol injected was higher (mean 2.4 vs. 1.7 cc; p < 0.0001). The average neo-left ventricular outflow tract area increased significantly after ASA in the patients undergoing TMVR (99.2 ± 83.37 mm2 vs. 196.5 ± 114.55 mm2; p = <0.0001). The HCM group had a greater reduction in the LVOT gradient after ASA vs the TMVR group (49.3 vs. 18 mmHg; p = 0.0040). The primary composite endpoint was higher in the TMVR group versus the HCM group (50.9% vs. 25.6%; p = 0.0404) and had a higher incidence of new permanent pacemaker (PPM) (25.5% vs. 18.6%; p = 0.3402). The TMVR group had a higher rate of all-cause mortality (9.8% vs. 1.2%; p = 0.0268). CONCLUSIONS: Preemptive ASA before TMVR was performed in patients with higher degree of clinical comorbidities, and correspondingly is associated with worse short-term clinical outcomes in comparison to ASA for HCM patients. ASA before TMVR enabled percutaneous mitral interventions in a small but significant minority of patients that would have otherwise been excluded. The degree of LVOT and neoLVOT area increase is significant and predictable.


Ablation Techniques , Cardiac Catheterization , Cardiomyopathy, Hypertrophic , Ethanol , Heart Valve Prosthesis Implantation , Mitral Valve , Humans , Retrospective Studies , Male , Ethanol/administration & dosage , Ethanol/adverse effects , Cardiomyopathy, Hypertrophic/diagnostic imaging , Cardiomyopathy, Hypertrophic/mortality , Cardiomyopathy, Hypertrophic/therapy , Cardiomyopathy, Hypertrophic/surgery , Cardiomyopathy, Hypertrophic/physiopathology , Female , Treatment Outcome , Ablation Techniques/adverse effects , Ablation Techniques/mortality , Aged , Cardiac Catheterization/adverse effects , Cardiac Catheterization/mortality , Cardiac Catheterization/instrumentation , Middle Aged , Risk Factors , Heart Valve Prosthesis Implantation/adverse effects , Heart Valve Prosthesis Implantation/instrumentation , Heart Valve Prosthesis Implantation/mortality , Time Factors , Mitral Valve/diagnostic imaging , Mitral Valve/physiopathology , Mitral Valve/surgery , Recovery of Function , Aged, 80 and over , Heart Septum/diagnostic imaging , Heart Septum/surgery , Mitral Valve Insufficiency/diagnostic imaging , Mitral Valve Insufficiency/physiopathology , Mitral Valve Insufficiency/surgery , Mitral Valve Insufficiency/mortality
9.
BMC Cardiovasc Disord ; 24(1): 230, 2024 Apr 27.
Article En | MEDLINE | ID: mdl-38678215

BACKGROUND: Unidentified heart failure occurs in patients with multiple myeloma when their heart was involved. CMR with late gadolinium enhancement (LGE) and T1 mapping can identify myocardial amyloid infiltrations. PURPOSE: To explore the role of CMR with late gadolinium enhancement (LGE) and T1 mapping for detection of multiple myeloma patients'heart. MATERIAL AND METHODS: A total of 16 MM patients with above underwent CMR (3.0-T) with T1 mapping (pre-contrast and post-contrast) and LGE imaging. In addition, 26 patients with non-obstructive hypertrophic cardiomyopathy and 26 healthy volunteers were compared to age- and sex-matched healthy controls without a history of cardiac disease, diabetes mellitus, or normal in CMR. All statistical analyses were performed using the statistical software GraphPad Prism. The measurement data were represented by median (X) and single sample T test was adopted. Enumeration data were represented by examples and Chi-tested was adopted. All tests were two-sided, and P values < 0.05 were considered statistically significant. RESULTS: In MM group, LVEF was lower than healthy controls and higher than that of non-obstructive hypertrophic cardiomyopathy group, but without statistically significant difference (%: 49.1 ± 17.5 vs. 55.6 ± 10.3, 40.4 ± 15.6, all P > 0.05). Pre-contrast T1 values of MM group were obviously higher than those of healthy controls and non-obstructive hypertrophic cardiomyopathy group (ms:1462.0 ± 71.3vs. 1269.3 ± 42.3, 1324.0 ± 45.1, all P < 0.05). 16 cases (100%) in MM group all had LGE. CONCLUSION: LGE joint T1 mapping wider clinical use techniques and follow-up the patients'disease severity.


Cardiomyopathy, Hypertrophic , Contrast Media , Magnetic Resonance Imaging, Cine , Multiple Myeloma , Predictive Value of Tests , Stroke Volume , Ventricular Function, Left , Humans , Multiple Myeloma/diagnostic imaging , Multiple Myeloma/diagnosis , Male , Female , Middle Aged , Contrast Media/administration & dosage , Aged , Cardiomyopathy, Hypertrophic/diagnostic imaging , Cardiomyopathy, Hypertrophic/physiopathology , Case-Control Studies , Myocardium/pathology , Adult , Heart Failure/diagnostic imaging , Heart Failure/physiopathology , Heart Failure/diagnosis , Heart Failure/etiology
11.
Clin Ther ; 46(4): 368-373, 2024 Apr.
Article En | MEDLINE | ID: mdl-38508915

PURPOSE: Hypertrophic cardiomyopathy (HCM) is an under-recognized genetic cardiac disorder affecting the muscles and contractility of the heart, which in turn can result in heart failure symptoms, arrhythmia, and sudden cardiac death. Previously, pharmacotherapy options for HCM were not disease-specific, often poorly tolerated, and overall inadequate for optimal management. This narrative review discusses the pharmacology of the novel drug mavacamten, the clinical trials supporting its use, and considerations for its use in clinical practice. METHODS: PubMed and ClinicalTrials.gov were searched for the key words mavacamten and Camzyos to identify currently active clinical trials and clinical trials published between January 2015 and March 2023. Data from EXPLORER-HCM were included, as EXPLORER-HCM led to approval by the US Food and Drug Administration of the use of mavacamten, along with data from VALOR-HCM, which provided additional evidence for use. Publications that were not randomized, controlled trials were not included in this review. FINDINGS: The findings from this review suggest that mavacamten is an effective treatment for patients with persistently symptomatic obstructive HCM and may decrease the need for septal reduction therapy. Mavacamten use was associated with improved exercise capacity, left ventricular outflow tract obstruction, and New York Heart Association functional class, and with a decreased frequency of septal reduction therapy. IMPLICATIONS: HCM is associated with significant morbidity and mortality, independent of other disease states. Mavacamten is a novel treatment option for patients with HCM and offers an additional option for patients with persistent symptoms who previously had limited treatment options. The use of mavacamten in patients with obstructive HCM may improve exercise capacity, and decrease symptoms and the need for septal reduction therapy. There is potential for mavacamten to be indicated for use in patients with nonobstructive HCM in the future, pending findings from Phase III trials in this population.


Cardiomyopathy, Hypertrophic , Humans , Cardiomyopathy, Hypertrophic/drug therapy , Cardiomyopathy, Hypertrophic/physiopathology , Treatment Outcome , Clinical Trials as Topic , Benzylamines , Uracil/analogs & derivatives
12.
Heart Vessels ; 39(6): 524-538, 2024 Jun.
Article En | MEDLINE | ID: mdl-38553520

The efficacy of convolutional neural network (CNN)-enhanced electrocardiography (ECG) in detecting hypertrophic cardiomyopathy (HCM) and dilated HCM (dHCM) remains uncertain in real-world applications. This retrospective study analyzed data from 19,170 patients (including 140 HCM or dHCM) in the Shinken Database (2010-2017). We evaluated the sensitivity, positive predictive rate (PPR), and F1 score of CNN-enhanced ECG in a ''basic diagnosis'' model (total disease label) and a ''comprehensive diagnosis'' model (including disease subtypes). Using all-lead ECG in the "basic diagnosis" model, we observed a sensitivity of 76%, PPR of 2.9%, and F1 score of 0.056. These metrics improved in cases with a diagnostic probability of ≥ 0.9 and left ventricular hypertrophy (LVH) on ECG: 100% sensitivity, 8.6% PPR, and 0.158 F1 score. The ''comprehensive diagnosis'' model further enhanced these figures to 100%, 13.0%, and 0.230, respectively. Performance was broadly consistent across CNN models using different lead configurations, particularly when including leads viewing the lateral walls. While the precision of CNN models in detecting HCM or dHCM in real-world settings is initially low, it improves by targeting specific patient groups and integrating disease subtype models. The use of ECGs with fewer leads, especially those involving the lateral walls, appears comparably effective.


Cardiomyopathy, Hypertrophic , Electrocardiography , Neural Networks, Computer , Humans , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/physiopathology , Cardiomyopathy, Hypertrophic/complications , Electrocardiography/methods , Retrospective Studies , Male , Female , Middle Aged , Predictive Value of Tests , Adult , Aged
13.
Int J Cardiovasc Imaging ; 40(4): 921-930, 2024 Apr.
Article En | MEDLINE | ID: mdl-38448705

The relationship between left ventricular (LV) torsion and myocardial fibrosis (MF) in hypertrophic cardiomyopathy (HCM) patients with preserved ejection fraction was still not well understood. New developments in cardiac magnetic resonance (CMR) enable a much fuller assessment of cardiac characteristics. This study sought to assess the impact of HCM on myocardial function as assessed by LV torsion and its relationship with MF. HCM (n = 79) and healthy controls (n = 40) underwent CMR. According to whether there was late gadolinium enhancement (LGE), patients were divided into LGE+ group and LGE- group. LV torsion and torsion rate were measured by CMR feature-tracking (CMR-FT). MF was quantitatively evaluated through LGE imaging. LGE was present in 44 patients (56%). Compared with healthy controls, torsion increased in the LGE- group (P < 0.001). Compared with LGE+ group, torsion was higher in the LGE- group (P < 0.001). There was no significant difference in torsion between LGE+ group and healthy controls. Correlation analysis showed that torsion was correlated with LGE% (r = - 0.443) and LGE mass (r = - 0.435) respectively. On multivariable logistic regression analysis, LV torsion was the only feature that was independently associated with the presence of LGE (OR 0.130; 95% CI 0.040 to 0.420, P = 0.01). The best torsion value associated with MF was 1.91 (sensitivity 60.0%, specificity 77.3%, AUC = 0.733). In HCM patients with preserved ejection fraction, CMR-FT derived LV torsion analysis holds promise for myocardial fibrosis detection.


Cardiomyopathy, Hypertrophic , Contrast Media , Fibrosis , Magnetic Resonance Imaging, Cine , Myocardium , Predictive Value of Tests , Stroke Volume , Torsion, Mechanical , Ventricular Function, Left , Humans , Male , Female , Cardiomyopathy, Hypertrophic/physiopathology , Cardiomyopathy, Hypertrophic/diagnostic imaging , Cardiomyopathy, Hypertrophic/complications , Middle Aged , Myocardium/pathology , Adult , Aged , Case-Control Studies , Retrospective Studies , Reproducibility of Results , Biomechanical Phenomena
14.
Heart ; 110(11): 792-799, 2024 May 10.
Article En | MEDLINE | ID: mdl-38388470

OBJECTIVE: The objective is to evaluate the 5-year follow-up results of percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) for hypertrophic obstructive cardiomyopathy (HOCM), including clinical status, electrocardiographic and echocardiographic characteristics. METHODS: 27 patients (age: 44.3±15.5 years; 67% men, 33% women) with severely symptomatic HOCM who underwent PIMSRA from October 2016 to September 2017 were included. Their clinical status, resting and exercise stress echocardiography, electrocardiography and cardiac MRI (CMRI) after long-term follow-up were assessed. RESULTS: One patient died of intracerebral haemorrhage 1 year post procedurally. The New York Heart Association class, Canadian Cardiovascular Society class and exercise-induced syncopal attacks improved significantly in 26 patients (all p<0.01). Left ventricular (LV) outflow tract gradients revealed sustained reduction (resting: from 95.0 to 9.0 mm Hg, p<0.001; post exercise: from 130.5 to 21.0 mm Hg, p<0.001). The echocardiographic evaluation revealed decreased septal thickness, LV posterior wall thickness and left atrial (LA) diameter (all p<0.001). CMRI data revealed decrease in LV mass index and LA volume index and increase in LV end-diastolic volume index and stroke volume index between baseline and long-term follow-up (all p<0.05). The global longitudinal strain of LV improved from (-11.9%±3.7%) before the procedure to (-13.1%±3.9%) at the last check (p<0.001). Malignant ventricular arrhythmia and heart failure events were not observed. CONCLUSIONS: PIMSRA can effectively alleviate symptoms in patients with HOCM and improve their hemodynamics in the long term. TRIAL REGISTRATION NUMBER: NCT02888132.


Cardiomyopathy, Hypertrophic , Adult , Female , Humans , Male , Middle Aged , Cardiomyopathy, Hypertrophic/physiopathology , Cardiomyopathy, Hypertrophic/surgery , Cardiomyopathy, Hypertrophic/diagnostic imaging , Catheter Ablation/methods , Electrocardiography , Follow-Up Studies , Heart Septum/surgery , Heart Septum/diagnostic imaging , Magnetic Resonance Imaging, Cine/methods , Radiofrequency Ablation/methods , Time Factors , Treatment Outcome , Ventricular Function, Left/physiology
15.
Heart Fail Rev ; 29(3): 663-674, 2024 May.
Article En | MEDLINE | ID: mdl-38308002

Over the last years, there has been a growing interest in the clinical manifestations and outcomes of cardiomyopathies in women. Peripartum cardiomyopathy is the only women-specific cardiomyopathy. In cardiomyopathies with X-linked transmission, women are not simply healthy carriers of the disorder, but can show a wide spectrum of clinical manifestations ranging from mild to severe manifestations because of heterogeneous patterns of X-chromosome inactivation. In mitochondrial disorders with a matrilinear transmission, cardiomyopathy is part of a systemic disorder affecting both men and women. Even some inherited cardiomyopathies with autosomal transmission display phenotypic and prognostic differences between men and women. Notably, female hormones seem to exert a protective role in hypertrophic cardiomyopathy (HCM) and variant transthyretin amyloidosis until the menopausal period. Women with cardiomyopathies holding high-risk features should be referred to a third-level center and evaluated on an individual basis. Cardiomyopathies can have a detrimental impact on pregnancy and childbirth because of the associated hemodynamic derangements. Genetic counselling and a tailored cardiological evaluation are essential to evaluate the likelihood of transmitting the disease to the children and the possibility of a prenatal or early post-natal diagnosis, as well as to estimate the risk associated with pregnancy and delivery, and the optimal management strategies.


Cardiomyopathies , Humans , Female , Cardiomyopathies/therapy , Cardiomyopathies/diagnosis , Cardiomyopathies/physiopathology , Cardiomyopathies/genetics , Pregnancy , Pregnancy Complications, Cardiovascular/therapy , Pregnancy Complications, Cardiovascular/physiopathology , Pregnancy Complications, Cardiovascular/diagnosis , Pregnancy Complications, Cardiovascular/genetics , Cardiomyopathy, Hypertrophic/therapy , Cardiomyopathy, Hypertrophic/genetics , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/physiopathology , Genetic Counseling/methods , Disease Management
18.
Can J Cardiol ; 40(5): 907-920, 2024 May.
Article En | MEDLINE | ID: mdl-38244986

Hypertrophic cardiomyopathy (HCM) is a primary heart muscle disease characterized by left ventricular hypertrophy that can be asymptomatic or with presentations that vary from left ventricular outflow tract obstruction, heart failure from diastolic dysfunction, arrhythmias, and/or sudden cardiac death. Children younger than 1 year of age tend to have worse outcomes and often have HCM secondary to inborn errors of metabolism or syndromes such as RASopathies. For children who survive or are diagnosed after 1 year of age, HCM outcomes are often favourable and similar to those seen in adults. This is because of sudden cardiac death risk stratification and medical and surgical innovations. Genetic testing and timely cardiac screening are paving the way for disease-modifying treatment as gene-specific therapies are being developed.


Cardiomyopathy, Hypertrophic , Humans , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/physiopathology , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/therapy , Child , Death, Sudden, Cardiac/prevention & control , Death, Sudden, Cardiac/etiology , Genetic Testing/methods
19.
Can J Cardiol ; 40(5): 876-886, 2024 May.
Article En | MEDLINE | ID: mdl-38286174

Hypertrophic cardiomyopathy (HCM) is the most prevalent genetically inherited cardiovascular disorder in adults and a significant cause of heart failure and sudden cardiac death. Historically, atrial fibrillation (AF) has been considered as a critical aspect in HCM patients as it is considered to be a marker of disease progression, escalates the frequency of heart failure hospitalisations, increases the risk of thromboembolic events, and worsens quality of life and outcome. Increasing evidence suggests that AF is the result of a subtle long-standing process that starts early in the history of HCM. The process of left atrial dilation accompanied by morphologic and functional remodelling is the quintessential prerequisite for the onset of AF. This review aims to describe the current understanding of AF pathophysiology in HCM, emphasising the role of left atrial myopathy in its development. In addition, we discuss risk factors and management strategies specific to AF in the context of HCM, providing insights into the complexities and challenges of treating this specific patient population.


Atrial Fibrillation , Cardiomyopathy, Hypertrophic , Heart Atria , Humans , Atrial Fibrillation/physiopathology , Atrial Fibrillation/diagnosis , Atrial Fibrillation/complications , Atrial Fibrillation/therapy , Atrial Fibrillation/etiology , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/physiopathology , Cardiomyopathy, Hypertrophic/complications , Heart Atria/physiopathology , Risk Factors , Atrial Remodeling/physiology , Disease Management
20.
Can J Cardiol ; 40(5): 860-868, 2024 May.
Article En | MEDLINE | ID: mdl-38110174

The mitral valve (MV) plays an important role in the pathophysiology of hypertrophic obstructive cardiomyopathy (HOCM). Dynamic left ventricular outflow tract (LVOT) obstruction, caused by systolic anterior motion (SAM), is a common occurrence in most patients with hypertrophic cardiomyopathy and is directly associated with the MV apparatus. First line therapy for HOCM patients is pharmacological, and surgical intervention is often indicated for patients who do not respond to medical therapy. Emerging research on mitral disease in HOCM, specifically mitral regurgitation (MR), demonstrates that these patients frequently do not respond to standard therapeutic options, and can benefit from MV interventions. In this review, we describe the involvement of the MV in the pathogenesis of HOCM, discuss medical therapy, and explore available mitral procedures. Surgical myectomy, often combined with various modifications to the MV apparatus, is frequently necessary to achieve a durable resolution of LVOT obstruction and SAM-related MR. Alcohol septal ablation, an alternative to surgical myectomy, will be briefly mentioned. We also emphasize the role of transcatheter edge-to-edge repair (TEER) as a promising and novel therapeutic option for HOCM patients. Over time, TEER has established itself as an effective and safe procedure, demonstrating success across a spectrum of anatomical variations. The leaflet modification and movement restriction achieved through TEER help reduce SAM and, consequently, have the potential to alleviate LVOT obstruction and SAM-related MR. Furthermore, we propose a treatment algorithm for cases where TEER is a potential course of action for patients who are at high risk for other interventions.


Cardiomyopathy, Hypertrophic , Mitral Valve Insufficiency , Mitral Valve , Ventricular Outflow Obstruction , Humans , Cardiomyopathy, Hypertrophic/therapy , Cardiomyopathy, Hypertrophic/physiopathology , Cardiomyopathy, Hypertrophic/surgery , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/complications , Mitral Valve/surgery , Mitral Valve/diagnostic imaging , Mitral Valve Insufficiency/surgery , Mitral Valve Insufficiency/diagnosis , Mitral Valve Insufficiency/physiopathology , Ventricular Outflow Obstruction/surgery , Ventricular Outflow Obstruction/etiology , Ventricular Outflow Obstruction/physiopathology , Ventricular Outflow Obstruction/diagnosis , Cardiac Surgical Procedures/methods
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