Hypertrophic Cardiomyopathy

IJCS

Editorial

Cardiac Myosin Inhibitors in Patients with Hypertrophic Cardiomyopathy

Marcelo Imbroinise Bittencourt et al. Ricardo Mourilhe-Rocha

In recent years, we have witnessed a significant shift in the management of symptomatic Hypertrophic Cardiomyopathy (HCM). For decades, treatment relied on medications that provided hemodynamic support without directly addressing sarcomeric hypercontractility, the primary pathophysiological driver of the disease. The emergence of Cardiac Myosin Inhibitors (CMIs), particularly mavacamten and aficamten, has transformed this landscape.^, In an article published in this issue of the International Journal of Cardiovascular Sciences, Fogaça PHT et al. present an updated systematic review and meta-analysis that reinforces the efficacy of this class of medications while providing important insights into their effects in both obstructive and non-obstructive HCM.

The study pooled data from six randomized controlled trials. Five included patients with obstructive HCM, whereas one – the MAVERICK-HCM trial – included patients with non-obstructive HCM.^,,- The inclusion of non-obstructive patients represents a distinctive aspect of this analysis, as the benefits of CMIs in the absence of left ventricular outflow tract (LVOT) obstruction remain less well established.

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IJCS

Brief Communication

Beyond Hypertrophic Cardiomyopathy: Rethinking Ventricular Thickening in the Spectrum of Cardiomyopathies

Fábio Fernandes et al. Murillo Oliveira Antunes Felix José Alvares Ramires Vinicius Machado Correia … Viviane Tiemi Hotta Juliano Novaes Cardoso Luciano Nastari Barbara Maria Ianni Marcelo Luiz Campos Vieira Charles Mady Jeffrey B. Geske Adelaide M. Arruda-Olson Alan Silva Martins Guilherme José dos Santos Ferreira Vagner Madrini Junior

Hypertrophic cardiomyopathy: potentially misleading nomenclature

In pathology, hypertrophy is the increase in cell size.³ In the heart, this suggests hypertrophy of cardiomyocytes, which can induce an anchoring bias, leading physicians to prioritize the diagnosis of sarcomeric hypertrophic cardiomyopathy, while diseases such as cardiac amyloidosis — where thickening is due to extracellular amyloid accumulation — are underdiagnosed.

This problem is exacerbated in echocardiographic reports, which often use “hypertrophic cardiomyopathy” nonspecifically. Although echocardiography is useful, it does not differentiate between thickening due to cellular hypertrophy or interstitial accumulation (infiltrative or storage diseases), making the term inadequate and potentially harmful in guiding clinical investigation and decisions.

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IJCS

ORIGINAL ARTICLE

Efficacy of Cardiac Myosin Inhibitors in Symptomatic Hypertrophic Cardiomyopathy: An Updated Meta-Analysis and Systematic Review

Pedro Henrique Tavares Fogaça et al. Gabriel Grando Alves Camila Sales Sales Fagundes Fernanda Lavarda Scheinpflug … João Candido Tavares Fogaça Mateus Diniz Marques

Abstract

Background:

This study evaluates the efficacy of cardiac myosin inhibitors (CMI), which appear to be an innovative alternative for hypertrophic cardiomyopathy (HCM), a disease lacking highly effective pharmacologic treatments.

Objective:

To provide updated efficacy data on CMI (aficamten or mavacamten) in symptomatic HCM.

Materials and methods:

We searched PubMed, Embase, and Cochrane databases for randomized controlled trials comparing CMI to placebo in symptomatic HCM, reporting: (1) New York Heart Association (NYHA) functional class improvement; (2) change from baseline in Kansas City Cardiomyopathy Questionnaire clinical summary score (KCCQ-CSS); (3) percent change from baseline in left ventricular outflow tract (LVOT) gradient at rest and after Valsalva maneuver; and (4) serious adverse events. Heterogeneity was examined with I² statistics, and p values < 0.05 indicated sta tistical significance. Continuous endpoints were analyzed using pooled mean difference (MD), and binary endpoints were analyzed using odds ratios (OR), both with 95% confidence intervals (CI).

Results:

We included six RCTs with 826 patients, 443 of whom received CMI. Compared to placebo, NYHA class improvement was more frequent in patients receiving CMI (OR: 4.10; 95% CI: 2.79 to 6.02; p < 0.00001), with significant differences in KCCQ-CSS change (MD: 7.15; 95% CI: 4.21 to 10.10; p < 0.00001), as well as in LVOT gradient change at rest (MD: −38.25; 95% CI: −46.76 to −29.74; p < 0.00001) and after Valsalva maneuver (MD: −46.49; 95% CI: −54.70 to −38.27; p < 0.00001). There were no significant differences in serious adverse events (OR: 0.64; 95% CI: 0.31 to 1.31; p = 0.22).

Conclusion:

CMI shows greater efficacy than placebo in treating symptomatic HCM.

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IJCS

Original Article

Long-Term Clinical Outcomes of Catheter Ablation in Hypertrophic Cardiomyopathy and Atrial Fibrillation: A Systematic Review and Meta-Analysis

Acácio Fernandes Cardoso et al. Fernanda Cristina Poscai Ribeiro Luiz Claudio Behrmann Martins Glaucylara Reis Geovanini … Cynthia Aparecida da Silva Rocha Nemer Luiz Pichara Áleff Mascarenhas da Silva

Abstract

Background:

Patients with hypertrophic cardiomyopathy (HCM) are at increased risk of atrial fibrillation (AF). However, the benefits of catheter ablation (CA) for reducing adverse clinical outcomes in this population are not well established.

Objective:

To assess the long-term impact of CA on clinical outcomes in patients with HCM and AF through a systematic review and meta-analysis.

Methods:

Controlled studies of patients with HCM and AF undergoing CA were identified through PubMed/MEDLINE, Embase, LILACS, and Cochrane. Meta-analysis employed a random-effects model in R Studio, version 4.3.2, with p < 0.05 considered significant.

Results:

Four retrospective cohort studies met inclusion criteria, all with long follow-up period (5.52 ± 0.91 years). Arrhythmia-free survival after multiple CA ranged from 36.3% to 82%. Combined event analysis (mortality, stroke, and heart failure) yielded odds ratio 0.22 (95% confidence interval 0.08 to 0.60; p < 0.05; I² = 64%). In individual analysis, only heart failure hospitalizations were significantly lower in the CA group (odds ratio 0.32; 95% confidence interval 0.13 to 0.82; p = 0.01).

Conclusions:

This study suggests that CA may improve clinical outcomes in patients with HCM and AF, particularly by improving heart failure hospitalizations. Studies with greater methodological rigor are needed to prove this hypothesis, since only four studies were included in this systematic review.

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ABC

Original Article

Friedreich's Ataxia: Cardiac Evaluation of 25 Patients with Clinical Diagnosis and Literature Review

Lilian Maria José Albano et al. Silvana Angelina Dório Nishioka Regina Lucia Moysés Jaqueline Wagenführ … Débora Bertola Sofia Mizuho Miura Sugayama A. Kim Chong

OBJECTIVE – Cardiac evaluation (clinical, electrocardiographic and echocardiographic) of 25 Brazilian patients with clinical diagnosis of Friedreich’s ataxia (FA) related to the frequency and the size of GAA repeats (unstable expansion of trinucleotide repeats that results in the disease). METHODS – Clinical and cardiac study including electrocardiogram and echocardiogram of all patients and molecular analysis to detect the frequency and the size of GAA expansion, by polymerase chain reaction analysis. RESULTS – Homozygous GAA expansion was detected in 17 patients (68%) — all typical cases. In 8 (32%) cases (6 atypical and 2 typical), no GAA expansion was observed, therefore it was not considered Friedreich’s ataxia. All patients with GAA expansion (100%) had electrocardiographic abnormalities, and only 25% of the cases without GAA expansion had some abnormality on this exam. However, only 6% of all patients revealed some signals/symptoms suggestive of cardiac involvement. CONCLUSION – A molecular analysis is essential to confirm the diagnosis of Friedreich’s ataxia; however, an adequate cardiac evaluation, including an electrocardiogram, was extremely useful to better screening the patients which should perform these molecular analysis.

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Cardiac Myosin Inhibitors in Patients with Hypertrophic Cardiomyopathy

Beyond Hypertrophic Cardiomyopathy: Rethinking Ventricular Thickening in the Spectrum of Cardiomyopathies

Hypertrophic cardiomyopathy: potentially misleading nomenclature

Efficacy of Cardiac Myosin Inhibitors in Symptomatic Hypertrophic Cardiomyopathy: An Updated Meta-Analysis and Systematic Review

Abstract

Background:

Objective:

Materials and methods:

Results:

Conclusion:

Long-Term Clinical Outcomes of Catheter Ablation in Hypertrophic Cardiomyopathy and Atrial Fibrillation: A Systematic Review and Meta-Analysis

Abstract

Background:

Objective:

Methods:

Results:

Conclusions:

Friedreich's Ataxia: Cardiac Evaluation of 25 Patients with Clinical Diagnosis and Literature Review

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