Hypertrophic Cardiomyopathy: New Concepts and Therapies

Maron, Barry J.; Rowin, Ethan J.; Maron, Martin S.

doi:10.1146/annurev-med-042220-021539

Cited by 35 publications

(26 citation statements)

References 77 publications

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“…In contrast to DCM, HCM is characterized by severe hypertrophy of the left ventricle leading to left ventricular outflow obstruction, heart failure, ventricular arrhythmias, and sudden cardiac death ( Maron et al, 2022a ; Maron et al, 2022b ; Maron et al, 2022c ). hiPSC-CMs have also been utilized to model a variety of HCM mutations.…”

Section: Maturation Of Ca 2+ Handling In Hipsc Car...mentioning

confidence: 99%

Cardiac calcium regulation in human induced pluripotent stem cell cardiomyocytes: Implications for disease modeling and maturation

Erñst

Bidwell

Dora

et al. 2023

Front. Cell Dev. Biol.

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Human induced pluripotent stem cell cardiomyocytes (hiPSC-CMs) are based on ground-breaking technology that has significantly impacted cardiovascular research. They provide a renewable source of human cardiomyocytes for a variety of applications including in vitro disease modeling and drug toxicity testing. Cardiac calcium regulation plays a critical role in the cardiomyocyte and is often dysregulated in cardiovascular disease. Due to the limited availability of human cardiac tissue, calcium handling and its regulation have most commonly been studied in the context of animal models. hiPSC-CMs can provide unique insights into human physiology and pathophysiology, although a remaining limitation is the relative immaturity of these cells compared to adult cardiomyocytes Therefore, this field is rapidly developing techniques to improve the maturity of hiPSC-CMs, further establishing their place in cardiovascular research. This review briefly covers the basics of cardiomyocyte calcium cycling and hiPSC technology, and will provide a detailed description of our current understanding of calcium in hiPSC-CMs.

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Section: Maturation Of Ca 2+ Handling In Hipsc Car...mentioning

confidence: 99%

Cardiac calcium regulation in human induced pluripotent stem cell cardiomyocytes: Implications for disease modeling and maturation

Erñst

Bidwell

Dora

et al. 2023

Front. Cell Dev. Biol.

View full text Add to dashboard Cite

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“…According to epidemiological investigations, the prevalence of HCM is approximately 1 per 200 in America and 800 per million in China. It is considered the main cause of sudden cardiac death in young people and even athletes ( 1 – 3 ). The current mainstream opinion regards mutations in genes encoding proteins of the cardiomyocytic contractile apparatus as the major pathogenesis of HCM.…”

Section: Introductionmentioning

confidence: 99%

Global research trends of hypertrophic cardiomyopathy from 2000 to 2022: Insights from bibliometric analysis

Zheng

et al. 2023

Front. Cardiovasc. Med.

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ObjectivesTo analyze the global research trends of hypertrophic cardiomyopathy (HCM) from 2000 to 2022 and explore new frontiers in this field.MethodsWe reviewed the literature in the Web of Science Core Collection database from January 2000 to August 2022 using the retrieval strategy of medical subject headings combined with text words. We focused on articles and reviews that were published in English. Relevant data of the target publications, such as title, authors, organizations, abstract, keywords, published date, journal, and number of citations, were collected. The R software with the “bibliometrix” and VOSviewer software was used to process and visualize the information.ResultsAmong a total of 20,581 records related to HCM, 13,427 from 103 countries and regions, 8,676 affiliations, and 46,645 researchers were included. Most of the publications in this field were from the United States, followed by Japan, the United Kingdom, and China. We also report the top 10 institutions and most influential researchers, cited articles, and highest-frequency keywords (echocardiography, heart failure, sudden cardiac death, genetics, atrial fibrillation, magnetic resonance imaging/cardiac magnetic resonance, prognosis, mutation, arrhythmia, late gadolinium enhancement). In addition, keywords trend analysis indicated that the novel medicine Mavacamten, genetic diagnosis, and cardiac magnetic resonance have attracted the most attention for the treatment and diagnosis of HCM over the past five years.ConclusionThe present study reports on the global research trends of HCM over the past two decades using bibliometric analysis. It may enlighten new frontiers in the diagnosis, treatment, and risk prevention of HCM.

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“…The disease has obvious clinical heterogeneity, ranging from asymptomatic patients with a positive genotype to patients with typical symptoms of angina pectoris or heart failure and even to the extreme state with sudden cardiac death as the first symptom. It is also the main cause of sudden cardiac death in young people and athletes, according to epidemiological investigations ( 1 , 2 ). HCM can be divided into two phenotypes, obstructive and non-obstructive, based on the hemodynamic characteristics.…”

Section: Introductionmentioning

confidence: 99%

Alcohol Septal Ablation or Septal Myectomy? An Updated Systematic Review and Meta-Analysis of Septal Reduction Therapy for Hypertrophic Obstructive Cardiomyopathy

Zheng

Yang

et al. 2022

Front. Cardiovasc. Med.

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ObjectiveTo evaluate the safety and effectiveness of alcohol septal ablation (ASA) and septal myectomy (SM) for the treatment of hypertrophic obstructive cardiomyopathy.MethodsWe searched the PubMed, MEDLINE, EMBASE, and CBM databases for observational research articles related to ASA and SM published from the establishment of the databases to November 2021. All ultimate selected articles were highly related to our target. The Newcastle-Ottawa Scale was used to evaluate the literature quality. A fixed or random effect model was performed in the meta-analysis depending on the heterogeneity of the included studies. The Mantel-Haenszelt test with relative risk ratio (RR) and 95% confidence interval (CI) was used to measure the effect indicator of binary data, while the inverse variance method with weighted mean difference (WMD) and 95% CI was used to measure the effect indicator of continuous data.ResultsA totally of 3,647 cases (1,555 cases treated with ASA and 2,092 cases treated with SM) were included. The results of the systematic review indicated no statistically significant difference in postoperative all-cause mortality (RR = 0.82; 95% CI: 0.65–1.04; P = 0.10) between patients treated with ASA and SM, but both the reduction in the postoperative left ventricular outflow tract pressure gradient (WMD = 9.35 mmHg, 95% CI: 5.38–13.31, P < 0.00001) and the post-operative improvement on cardiac function, assessed by the grade of New York Heart Association (NYHA), compared to pre-operative measurements (WMD = 0.13; 95% CI: 0.00–0.26; P < 0.04) in the ASA group were slightly inferior to those in the SM group. In addition, both the risk of pacemaker implantation (RR = 2.83, 95% CI: 2.06–3.88; P < 0.00001) and the risk of reoperation (RR = 11.23, 95% CI: 6.21–20.31; P < 0.00001) are recorded at a higher level after ASA procedure.ConclusionBoth ASA and SM have a high degree of safety, but the reduction in the postoperative left ventricular outflow tract pressure gradient and the improvement on cardiac function are slightly inferior to SM. In addition, both the risk of pacemaker implantation and the risk of reoperation are recorded at a higher level after ASA procedure. The operative plan should be chosen through multidisciplinary discussions in combination with the wishes of the patients and the actual clinical situation.

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Hypertrophic Cardiomyopathy: New Concepts and Therapies

Cited by 35 publications

References 77 publications

Cardiac calcium regulation in human induced pluripotent stem cell cardiomyocytes: Implications for disease modeling and maturation

Cardiac calcium regulation in human induced pluripotent stem cell cardiomyocytes: Implications for disease modeling and maturation

Global research trends of hypertrophic cardiomyopathy from 2000 to 2022: Insights from bibliometric analysis

Alcohol Septal Ablation or Septal Myectomy? An Updated Systematic Review and Meta-Analysis of Septal Reduction Therapy for Hypertrophic Obstructive Cardiomyopathy

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