cardiomyopathy

心肌病
  • 文章类型: Journal Article
    据报道,选择性5-羟色胺再摄取抑制剂(SSRIs)和5-羟色胺和去甲肾上腺素再摄取抑制剂(SNRIs)会引起应激性心肌病(SC)。本研究在公开的美国食品和药物管理局不良事件报告系统(FAERS)数据库中评估了SSRI/SNRI使用与心肌病发生之间的关联。使用不成比例分析和似然比检验来确定与SSRIs或SNRIs相关的风险以及SC的发生率。使用从FAERS数据库获得的2012年至2022年之间的数据。该研究确定了132个与SSRIs或SNRIs相关的SC个体病例安全性报告(ICSR)。文拉法辛(48%)和氟西汀(27%)是ICSR中最常见的抗抑郁药。大约80%的SC病例报告为女性,45-65岁的人被确定为高危人群。文拉法辛(比率量表信息成分[RSIC]2.54,95%CI2.06-3.04)和氟西汀(RSIC3.20,95%CI2.31-4.47)均与SC相关,文拉法辛的似然比估计值为3.55(p=0.02),氟西汀的似然比估计值为4.82(p=0.008)。心肌病发病的中位时间为20天,其中48.33%的患者报告住院。文拉法辛和氟西汀与SC风险相关,尤其是中年妇女。使用SSRIs或SNRIs联合其他5-羟色胺能药物时,应谨慎行事。
    Selective serotonin reuptake inhibitors (SSRIs) and serotonin and norepinephrine reuptake inhibitors (SNRIs) are reported to cause stress cardiomyopathy (SC). This study evaluated the association between SSRI/SNRI use and the occurrence of cardiomyopathy in the publicly available U.S. Food and Drug Administration Adverse Event Reporting System (FAERS) database. Disproportionate analysis and likelihood ratio tests were used to identify risk associated with SSRIs or SNRIs and the incidence of SC, using data from between from 2012 to 2022 acquired from the FAERS database. The study identified 132 individual case safety reports (ICSRs) of SC associated with SSRIs or SNRIs. Venlafaxine (48%) and fluoxetine (27%) were the most common antidepressants of the ICSRs. Approximately 80% of SC cases were reported in females, with individuals aged 45-65 years identified as a high-risk population. Both venlafaxine (ratio-scale information component [RSIC] 2.54, 95% CI 2.06-3.04) and fluoxetine (RSIC 3.20, 95% CI 2.31-4.47) were associated with SC, with likelihood ratio estimates of 3.55 (p = 0.02) for venlafaxine and 4.82 (p = 0.008) for fluoxetine. The median time to cardiomyopathy onset was 20 days, with hospitalization reported in 48.33% of patients. Venlafaxine and fluoxetine were associated with SC risk, particularly in middle-aged women. Caution should be exercised when using SSRIs or SNRIs combined with other serotonergic medications.
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  • 文章类型: Case Reports
    右室流出道(RVOT)阻塞是肥厚型心肌病(HCM)患者心室肥厚的罕见并发症。这项研究提出了一例罕见的HCM患者,该患者患有严重的RVOT梗阻,通过使用mavacampen成功缓解。
    Right ventricular outflow tract (RVOT) obstruction is a rare complication of ventricular hypertrophy in patients with hypertrophic cardiomyopathy (HCM). This study presents an unusual case of a patient with HCM with severe RVOT obstruction that was relieved successfully through the use of mavacamten.
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  • 文章类型: Case Reports
    使用抗疟药羟氯喹是系统性红斑狼疮患者的标准治疗方法。它有助于减少疾病相关的损害,防止疾病发作,并提高总体生存率。羟氯喹的作用机制包括干扰细胞的溶酶体降解导致液泡的积累。视网膜病变是羟氯喹的良好描述的不良反应,因此需要长期使用后与眼科医生进行筛查。虽然很少报道,羟氯喹的心脏不良反应也可能发生。在这份报告中,我们介绍了一例23岁女性患者,该患者使用羟氯喹治疗,可能是由于Libman-Sacks心内膜炎导致卒中,经胸超声心动图发现患有严重肥厚型心肌病.
    The use of the antimalarial drug hydroxychloroquine is a standard treatment in patients with systemic lupus erythematosus. It helps reduce disease-associated damage, prevents disease flare, and improves overall survival. The mechanism of action of hydroxychloroquine includes interference with lysosomal degradation of cells leading to the accumulation of vacuoles. Retinopathy is a well-described adverse effect of hydroxychloroquine, thus requiring screening with an ophthalmologist after prolonged use. Although rarely reported, cardiac adverse effects of hydroxychloroquine can also occur. In this report, we present a case of a 23-year-old woman with systemic lupus erythematosus on hydroxychloroquine who presented with stroke possibly due to Libman-Sacks endocarditis and was found to have severe hypertrophic cardiomyopathy on transthoracic echocardiogram.
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  • 文章类型: Case Reports
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  • 文章类型: Editorial
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  • 文章类型: Editorial
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  • 文章类型: Journal Article
    左心室致密化不全(LVNC)的特征是左心室(LV)壁的过度小梁。
    作者旨在研究出生时2至4岁有和无LVNC儿童的LV功能和形态的变化,并描述一级亲属中LVNC的患病率。
    有和没有LVNC(匹配1:4)的儿童在2至4岁以及一级亲属中进行了超声心动图检查。对LVNC进行了盲目评估,并将其定义为在≥1个LV节段中≥2的非致密心肌与致密心肌的比率。小梁表示为具有LVNC的节段的数量占节段总数的百分比。
    总共,14(中位年龄3岁,71%男性)出生时患有LVNC的16名儿童和无LVNC的56名儿童(中位年龄4岁,71%男性),37名LVNC儿童的一级亲属(平均年龄31岁,46%的男性)和146名无儿童的一级亲属(中位年龄33岁,包括50%的男性)。在患有LVNC的儿童中,小梁(8%对13%,P=0.81)和左心室射血分数(50%vs49%,P=0.91)从出生到随访没有变化,但LV射血分数低于无LVNC的儿童(49%vs60%,P<0.001)。在LVNC儿童的亲属中,与无亲属的无LVNC儿童相比,37例中的11例(30%)符合LVNC标准(P<0.001)。
    在2到4岁时,出生时诊断为LVNC的儿童与无LV功能障碍或小梁形成程度的儿童相比,收缩功能降低.在LVNC儿童的一级亲属中,30%符合标准。
    UNASSIGNED: Left ventricular noncompaction (LVNC) is characterized by excessive trabeculations of the left ventricular (LV) wall.
    UNASSIGNED: The authors aimed to examine changes in LV function and morphology in 2 to 4-year-old children with and without LVNC at birth and to describe the prevalence of LVNC in first-degree relatives.
    UNASSIGNED: Echocardiograms in children with and without LVNC (matched 1:4) were performed at 2 to 4 years and in first-degree relatives. LVNC was blindly assessed and defined as a ratio of non-compact to compact myocardium of ≥2 in ≥1 LV segment. Trabeculations were expressed as a percentage of the number of segments with LVNC out of the total number of segments.
    UNASSIGNED: In total, 14 (median age 3 years, 71% male) of 16 children with LVNC at birth and 56 children without (median age 4 years, 71% male), 37 first-degree relatives of children with LVNC (median age 31 years, 46% male) and 146 first-degree relatives of children without (median age 33 years, 50% male) were included. In children with LVNC, trabeculation (8% vs 13%, P = 0.81) and LV ejection fraction (50% vs 49%, P = 0.91) were unchanged from birth to follow-up but LV ejection fraction was lower compared to children without LVNC (49% vs 60%, P < 0.001). In relatives of children with LVNC, 11 of 37 (30%) fulfilled LVNC criteria compared to no relatives to children without LVNC (P < 0.001).
    UNASSIGNED: At 2 to 4 years, children with LVNC diagnosed at birth had reduced systolic function compared to children without but did not have progression of LV dysfunction or extent of trabeculations. In first-degree relatives to children with LVNC, 30% fulfilled criteria.
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  • 文章类型: Editorial
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  • 文章类型: Journal Article
    关于肥厚型心肌病(HCM)患者亲属的疾病表达的证据很少。这些信息对家庭筛查计划具有重要意义,遗传咨询,和受影响家庭的管理。
    这项研究的目的是调查在公认的HCM基因中携带致病性/可能致病性(P/LP)变异的索引患者亲属的疾病表达和外显率。
    共有453名连续且无关的HCM指数患者接受了临床和遗传学调查。共有903名基因型阳性指数患者的亲属被邀请进行临床调查和基因检测。恍惚,疾病表达,在携带P/LP变异的个体中调查了主要不良心脏事件(MACE)的发生率.
    40%(183/453)的索引患者携带P/LP变异。有P/LP变异的指数患者的所有亲属中有百分之八十四(757/903)可用于调查,其中54%(407/757)携带P/LP变体。亲属中HCM的外显率为39%(160/407)。患有HCM和指数患者的亲属在相似的年龄(43±18岁vs46±15岁;P=0.11)。在8年的随访中,MACE的临床特征和发生率没有差异。
    在识别的疾病基因中携带P/LP变异的索引患者和受影响亲属中HCM的疾病表达相似,经历MACE的风险相等。这些发现为支持基因型阳性HCM家庭的家庭筛查和随访提供了证据,以改善管理并减少亲属中不良疾病并发症的数量。
    UNASSIGNED: Little evidence is available on the disease expression in relatives of index patients with hypertrophic cardiomyopathy (HCM). This information has important implications for family screening programs, genetic counseling, and management of affected families.
    UNASSIGNED: The purpose of this study was to investigate the disease expression and penetrance in relatives of index patients carrying pathogenic/likely pathogenic (P/LP) variants in recognized HCM genes.
    UNASSIGNED: A total of 453 consecutive and unrelated HCM index patients underwent clinical and genetic investigations. A total of 903 relatives of genotype-positive index patients were invited for clinical investigations and genetic testing. Penetrance, disease expression, and incidence rates of major adverse cardiac events (MACEs) were investigated in individuals carrying P/LP variants.
    UNASSIGNED: Forty percent (183/453) of index patients carried a P/LP variant. Eighty-four percent (757/903) of all relatives of index patients with P/LP variants were available for the investigation, of whom 54% (407/757) carried a P/LP variant. The penetrance of HCM among relatives was 39% (160/407). Relatives with HCM and index patients were diagnosed at a similar age (43 ± 18 years vs 46 ± 15 years; P = 0.11). There were no differences in clinical characteristics or incidence rates of MACE during 8 years of follow-up.
    UNASSIGNED: The disease expression of HCM among index patients and affected relatives carrying P/LP variants in recognized disease genes was similar, with an equal risk of experiencing MACE. These findings provide evidence to support family screening and follow-up of genotype-positive HCM families to improve management and diminish the number of adverse disease complications among relatives.
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  • 文章类型: Journal Article
    蒽环类药物是受心脏毒性限制的有效化疗药物。空间心室梯度(SVG)是与不良心血管结局相关的电异质性的标志,包括心脏性猝死和心力衰竭(HF)。
    这项研究的目的是评估化疗前的SVG值是否与蒽环类药物相关的HF或心肌病(CM)的风险相关。
    我们分析了1992年至2019年在一个学术医疗中心接受癌症治疗的回顾性队列中,蒽环类药物开始前6个月内获得的12导联心电图。发作性HF和CM由ICD-9/10代码定义,并通过图表审查确认。心电图是根据基线心电图构建的,并计算SVG。蒽环类抗生素相关HF或CM的累积发病率在SVG矢量方向和大小上回归,死亡为竞争性风险。
    在889例患者中(47%为男性;平均年龄58±16岁;71%为血液系统恶性肿瘤),化疗前SVG幅度较大与多变量调整后HF或CM风险降低相关,亚风险比为每1SD增加0.76(95%CI:0.59-0.96;P=0.024)。SVG矢量方向,特别是一个更向左的VGx,与HF或CM的风险降低相关,亚风险比为0.77/1SD增加(95%CI:0.61-0.96;P=0.023)。
    在一个大型回顾性队列研究中,较大的SVG幅度和较向左的SVG方向与蒽环类药物心脏毒性风险降低相关。结合SVG的改进的心脏风险分层算法可以个性化癌症和心脏保护治疗。
    UNASSIGNED: Anthracyclines are effective chemotherapies that are limited by cardiotoxicity. The spatial ventricular gradient (SVG) is a marker of electrical heterogeneity linked to adverse cardiovascular outcomes, including sudden cardiac death and heart failure (HF).
    UNASSIGNED: The purpose of this study was to assess if SVG values before chemotherapy are associated with the risk of anthracycline-associated HF or cardiomyopathy (CM).
    UNASSIGNED: We analyzed 12-lead electrocardiograms obtained within 6 months before initiation of anthracyclines in a retrospective cohort treated for cancer between 1992 and 2019 at a single academic medical center. Incident HF and CM were defined by ICD-9/10 codes and confirmed by chart review. Vectorcardiograms were constructed from baseline electrocardiograms, and the SVG was calculated. The cumulative incidence of anthracycline-associated HF or CM was regressed on SVG vector orientation and magnitude with death as a competing risk.
    UNASSIGNED: In 889 patients (47% male; mean age 58 ± 16 years; 71% hematologic malignancies), larger SVG magnitude prechemotherapy was associated with decreased risk of HF or CM after multivariable adjustment, with a subhazard ratio of 0.76 per 1 SD increase (95% CI: 0.59-0.96; P = 0.024). SVG vector orientation, specifically a more leftward oriented VGx, was associated with decreased risk of HF or CM with a subhazard ratio of 0.77 per 1 SD increase (95% CI: 0.61-0.96; P = 0.023).
    UNASSIGNED: Larger SVG magnitude and more leftward SVG orientation were associated with a decreased risk of anthracycline cardiotoxicity in a large retrospective cohort. Improved cardiac risk stratification algorithms incorporating the SVG could personalize both cancer and cardioprotective therapy.
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