UNASSIGNED: Alcohol septal ablation (ASA) has been more commonly applied in medical refractory hypertrophic obstructive cardiomyopathy (HOCM) compared with septal myectomy (SM), however its potential to create a proarrhythmic substrate is increased.
UNASSIGNED: A systematic search was performed in PubMed, EMBASE, Web of Science, and the Cochrane Library from inception to October 2020. Fixed or random effects models were used to estimate the risk ratios (RR) for ventricular arrhythmia events or other outcomes between the SM and ASA cohorts.
UNASSIGNED: Twenty studies with 8025 patients were included. Pool analysis showed that the incidence of ventricular tachycardia (VT)/ventricular fibrillation (VF), which included appropriate implantable cardioverter defibrillator (ICD) intervention, was significantly higher in the ASA cohort than that in the SM cohort (ASA vs SM: 10% (345/3312) vs 5% (161/3227) (RR = 1.98, 95% CI (confidence interval), 1.65-2.37; p < 0.00001, I 2 = 0%). In both groups, more than 90% of VT/VF events occurred in the early phase (during the procedure, during hospitalization or within 30 days after the procedure) (ASA: 94.20%; SM: 94.41%). Further subgroup analysis also showed that the ASA group had a higher incidence of VT/VF in both the early phase (RR = 1.94, 95% CI, 1.61-2.33; p < 0.0001, I 2 = 0%) and the late phase (RR = 2.80, 95% CI, 1.00-7.89; p = 0.05, I 2 = 33%). Furthermore, although the risks of sudden cardiac death (SCD) were similar between the ASA and SM groups, a higher incidence of sudden cardiac arrest (SCA), which included SCD and resuscitated SCA, was observed in the ASA group (RR = 2.30, 95% CI, 1.35-3.94; p = 0.002, I 2 = 0%).
UNASSIGNED: In patients with HOCM, those who received ASA showed a higher incidence of VF/VT and SCD combined with resuscitated SCA. The majority of VT/VF occurred in the early phase.

Hypertrophic cardiomyopathy (HCM) is a genetic disorder in which left ventricular outflow tract obstruction critically affects symptoms and prognosis. Traditionally, left ventricular outflow tract obstruction was primarily attributed to septal hypertrophy with systolic anterior motion of the mitral valve. However, recent evidence highlights significant contributions from the mitral valve and papillary muscle anomalies, as well as an apical-basal muscle bundle observed in HCM patients. Accurate morphological assessment is essential when considering septal reduction therapy. While transesophageal echocardiography and cardiac magnetic resonance are recommended for assessing the anomalous structures, four-dimensional computed tomography offers superior spatial resolution and multiplanar reconstruction capabilities. These features enable the evaluation of details of the morphological anomalies, such as the apical-basal muscle band, papillary muscle anomalies, subaortic stenosis, and right ventricular outflow tract obstruction. Based on the detailed assessment of these morphological features, four-dimensional computed tomography has been utilized for planning of surgical correction in a comprehensive HCM center. This approach facilitates the intervention strategies and may improve outcomes in septal reduction therapy for obstructive HCM.

UNASSIGNED: Hypertrophic cardiomyopathy (HCM) is a heterogeneous genetic heart disease with an estimated prevalence in the general population of 0.2% to 0.6%. Clinically, HCM can range from no symptoms to severe symptoms such as heart failure or sudden cardiac death. Currently, the management of HCM involves lifestyle modifications, familial screening, genetic counseling, pharmacotherapy to manage symptoms, sudden cardiac death risk assessment, septal reduction therapy, and heart transplantation for specific patients. Multicenter randomized controlled trials have only recently explored the potential of cardiac myosin inhibitors (CMIs) such as mavacamten as a directed pharmacological approach for managing HCM.
UNASSIGNED: We will assess the existing medical treatments for HCM: beta-blockers, calcium channel blockers, disopyramide, and different CMIs. We will also discuss future HCM pharmacotherapy guidelines and underline this patient population\'s unfulfilled needs.
UNASSIGNED: Mavacamten is the first-in-class CMI approved by the FDA to target HCM pathophysiology specifically. Mavacamten should be incorporated into the standard therapy for oHCM in case of symptom persistence despite using maximally tolerated beta blockers and/or calcium channel blockers. Potential drug-drug interactions should be assessed before initiating this drug. More studies are needed on the use of CMIs in patients with kidney and/or liver failure and pregnant/breastfeeding patients.

OBJECTIVE: To compare early and late outcomes of septal myectomy in patients with obstructive hypertrophic cardiomyopathy who presented with residual or recurrent left ventricular outflow tract (LVOT) obstruction after previous septal-reduction therapy (SRT).
METHODS: From January 1989 to March 2022, 145 patients underwent reintervention by septal myectomy for residual LVOT obstruction after previous SRT; 72 patients had previous alcohol septal ablation (ASA) and 73 had previous surgical septal myectomy. Baseline patient characteristics, echocardiographic parameters, and surgical outcomes were compared between these 2 groups.
RESULTS: Patients who had previous ASA were more likely to be male (50.0% vs 30.1%; P = .015), be older (median age 57.5 years vs 48.3 years; P < .001), and have a greater body mass index (32.7 kg/m2 vs 30.0 kg/m2; P = .011). After repeat SRT by septal myectomy, there was no significant difference in the incidence of postoperative complete heart block, necessitating permanent pacemaker, between the 2 groups (8.3% vs 2.7%; P = .151). One (0.7%) patient died within 30 days of surgery. Over a median follow-up of 7.5 years (interquartile range, 3.0-13.8), there were 20 deaths. Kaplan-Meier 5-, 10-, and 15-year survival rates were 100%, 91%, and 76% for the previous septal myectomy group, and 93%, 81%, and 64% for the previous ASA group (P = .207).
CONCLUSIONS: Septal myectomy for residual or recurrent LVOT obstruction in patients who had previous ASA is safe, with an acceptably low rate of postoperative complete heart block. Surgical outcomes and late survival rates in patients with complete heart block ASA were satisfactory and comparable with patients who underwent repeat myectomy.

OBJECTIVE: The \"2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy\" provides recommendations to guide clinicians in the management of patients with hypertrophic cardiomyopathy.
METHODS: A comprehensive literature search was conducted from September 14, 2022, to November 22, 2022, encompassing studies, reviews, and other evidence on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, the Agency for Healthcare Research and Quality, and other selected databases relevant to this guideline. Additional relevant studies, published through May 23, 2023, during the guideline writing process, were also considered by the writing committee and added to the evidence tables, where appropriate.
UNASSIGNED: Hypertrophic cardiomyopathy remains a common genetic heart disease reported in populations globally. Recommendations from the \"2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy\" have been updated with new evidence to guide clinicians.

UNASSIGNED: The \"2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy\" provides recommendations to guide clinicians in the management of patients with hypertrophic cardiomyopathy.
UNASSIGNED: A comprehensive literature search was conducted from September 14, 2022, to November 22, 2022, encompassing studies, reviews, and other evidence on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, the Agency for Healthcare Research and Quality, and other selected databases relevant to this guideline. Additional relevant studies, published through May 23, 2023, during the guideline writing process, were also considered by the writing committee and added to the evidence tables, where appropriate.
UNASSIGNED: Hypertrophic cardiomyopathy remains a common genetic heart disease reported in populations globally. Recommendations from the \"2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy\" have been updated with new evidence to guide clinicians.

暂无摘要。

UNASSIGNED: Hypertrophic cardiomyopathy (HCM) is a genetic disease that can cause left ventricular outflow tract (LVOT) obstruction. This study analyzed the efficacy of radiofrequency ablation (RA) in improving clinical and hemodynamic factors in patients receiving obstructive HCM refractory treatment. This evaluation was necessary because of the small number of studies on the effectiveness of this technique for obstructive HCM in the existing literature.
UNASSIGNED: We used the PubMed, Embase, and Science Direct databases to identify randomized clinical trials and observational studies addressing the clinical and hemodynamic outcomes before and after RA in patients with HCM.
UNASSIGNED: We selected six articles published between 2011 and 2022, comprising 304 patients (mean age: 45 years).
UNASSIGNED: We performed a bias assessment using the ROBINS I tool, and meta-analysis processing was performed using the STATA program (v.16.0).
UNASSIGNED: The left ventricular outflow tract (LVOT) gradient at rest and with stimulation decreased by 58.78 mmHg (p = 0.001) and 70.38 mmHg (total effect Z = 21.62; p < 0.0001), respectively. Additionally, the New York Heart Association (NYHA) functional class decreased by 0.43 (p = 0.001), indicating symptomatic and hemodynamic improvements. Furthermore, we observed a significant reduction in septal thickness (by 4 mm; p = 0.001).
UNASSIGNED: RA improved the NYHA functional class and LVOT gradient at rest and with stimulation and reduced septal thickness. These results suggest that RA is effective in patients refractory to pharmacological therapy and unsuitable for alcohol septal ablation or myectomy. However, more studies, including randomized clinical trials, should be conducted to define the role of RA in interventional therapies.

BACKGROUND: Left ventricular outflow tract (LVOT) obstruction is a source of morbidity in hypertrophic cardiomyopathy (HCM) and a life-threatening complication of transcatheter mitral valve replacement (TMVR) and transcatheter aortic valve replacement (TAVR). Available surgical and transcatheter approaches are limited by high surgical risk, unsuitable septal perforators, and heart block requiring permanent pacemakers.
OBJECTIVE: The authors report the initial experience of a novel transcatheter electrosurgical procedure developed to mimic surgical myotomy.
METHODS: We used septal scoring along midline endocardium (SESAME) to treat patients, on a compassionate basis, with symptomatic LVOT obstruction or to create space to facilitate TMVR or TAVR.
RESULTS: In this single-center retrospective study between 2021 and 2023, 76 patients underwent SESAME. In total, 11 (14%) had classic HCM, and the remainder underwent SESAME to facilitate TMVR or TAVR. All had technically successful SESAME myocardial laceration. Measures to predict post-TMVR LVOT significantly improved (neo-LVOT 42 mm2 [Q1-Q3: 7-117 mm2] to 170 mm2 [Q1-Q3: 95-265 mm2]; P < 0.001; skirt-neo-LVOT 169 mm2 [Q1-Q3: 153-193 mm2] to 214 mm2 [Q1-Q3: 180-262 mm2]; P < 0.001). Among patients with HCM, SESAME significantly decreased invasive LVOT gradients (resting: 54 mm Hg [Q1-Q3: 40-70 mm Hg] to 29 mm Hg [Q1-Q3: 12-36 mm Hg]; P = 0.023; provoked 146 mm Hg [Q1-Q3: 100-180 mm Hg] to 85 mm Hg [Q1-Q3: 40-120 mm Hg]; P = 0.076). A total of 74 (97.4%) survived the procedure. Five experienced 3 of 76 (3.9%) iatrogenic ventricular septal defects that did not require repair and 3 of 76 (3.9%) ventricular free wall perforations. Neither occurred in patients treated for HCM. Permanent pacemakers were required in 4 of 76 (5.3%), including 2 after concomitant TAVR. Lacerations were stable and did not propagate after SESAME (remaining septum: 5.9 ± 3.3 mm to 6.1 ± 3.2 mm; P = 0.8).
CONCLUSIONS: With further experience, SESAME may benefit patients requiring septal reduction therapy for obstructive hypertrophic cardiomyopathy as well as those with LVOT obstruction after heart valve replacement, and/or can help facilitate transcatheter valve implantation.

UNASSIGNED: Artificial intelligence-enhanced electrocardiography (AI-ECG) can identify hypertrophic cardiomyopathy (HCM) on 12-lead ECGs and offers a novel way to monitor treatment response. While the surgical or percutaneous reduction of the interventricular septum (SRT) represented initial HCM therapies, mavacamten offers an oral alternative.
UNASSIGNED: To evaluate biological response to SRT and mavacamten.
UNASSIGNED: We applied an AI-ECG model for HCM detection to ECG images from patients who underwent SRT across three sites: Yale New Haven Health System (YNHHS), Cleveland Clinic Foundation (CCF), and Atlantic Health System (AHS); and to ECG images from patients receiving mavacamten at YNHHS.
UNASSIGNED: A total of 70 patients underwent SRT at YNHHS, 100 at CCF, and 145 at AHS. At YNHHS, there was no significant change in the AI-ECG HCM score before versus after SRT (pre-SRT: median 0.55 [IQR 0.24-0.77] vs post-SRT: 0.59 [0.40-0.75]). The AI-ECG HCM scores also did not improve post SRT at CCF (0.61 [0.32-0.79] vs 0.69 [0.52-0.79]) and AHS (0.52 [0.35-0.69] vs 0.61 [0.49-0.70]). Among 36 YNHHS patients on mavacamten therapy, the median AI-ECG score before starting mavacamten was 0.41 (0.22-0.77), which decreased significantly to 0.28 (0.11-0.50, p <0.001 by Wilcoxon signed-rank test) at the end of a median follow-up period of 237 days.
UNASSIGNED: The lack of improvement in AI-based HCM score with SRT, in contrast to a significant decrease with mavacamten, suggests the potential role of AI-ECG for serial monitoring of pathophysiological improvement in HCM at the point-of-care using ECG images.