Risk Stratification and Outcome of Patients with Hypertrophic Cardiomyopathy

A special issue of Medicina (ISSN 1648-9144). This special issue belongs to the section "Cardiology".

Deadline for manuscript submissions: closed (1 June 2023) | Viewed by 4819

Special Issue Editors


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Guest Editor
Clinic for Cardiology, University Clinical Center of Serbia, Visegradska 26, Belgrade, Serbia
Interests: hypertrophic cardiomyopathy; coronary flow reserve; coronary physiology; echocardiography
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Co-Guest Editor
Clinic for Cardiology, University Clinical Center of Serbia, Visegradska 26, Belgrade, Serbia
Interests: hypertrophic cardiomyopathy; stress echocardiography; coronary physiology; echocardiography

Special Issue Information

Dear Colleagues,

Hypertrophic cardiomyopathy is the most common inherited cardiac disease which is associated with increased cardiovascular morbidity and mortality. As a result of heterogeneous clinical courses and phenotypes of hypertrophic cardiomyopathy, risk stratification remains challenging, and is mainly focused on sudden cardiac death. However, certain echocardiographic and clinical parameters used for sudden cardiac death prediction have limited prognostic value in predicting other hypertrophic cardiomyopathy-related cardiovascular events, such as the progressive deterioration of left ventricular systolic function with heart failure development or an ischemic stroke. Furthermore, it is significant that some hypertrophic cardiomyopathy patients without "traditional" risk markers can nevertheless experience fatal events or significant clinical deterioration. Therefore, clinical research should be focused on identifying other potential predictors in order to optimize hypertrophic cardiomyopathy risk assessment and patient management.

Therefore, this Special Issue aims to collect a series of articles, specifically related to the identification of cardiovascular predictors which are associated with increased morbidity and mortality in patients with hypertrophic cardiomyopathy.

Dr. Milorad Tesic
Dr. Vojislav Giga
Guest Editors

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Keywords

  • hypertrophic cardiomyopathy
  • prognosis
  • adverse cardiac outcome
  • risk stratification

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Published Papers (2 papers)

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Research

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11 pages, 1439 KiB  
Article
Prognostic Value of Mitral Regurgitation in Patients with Primary Hypertrophic Cardiomyopathy
by Milorad Tesic, Lazar Travica, Vojislav Giga, Ivana Jovanovic, Danijela Trifunovic Zamaklar, Dejana Popovic, Djordje Mladenovic, Marija Radomirovic, Jelena Vratonjic, Nikola Boskovic, Srdjan Dedic, Olga Nedeljkovic Arsenovic, Srdjan Aleksandric, Stefan Juricic, Branko Beleslin and Ana Djordjevic Dikic
Medicina 2023, 59(10), 1798; https://doi.org/10.3390/medicina59101798 - 9 Oct 2023
Viewed by 1673
Abstract
Background and Objectives: Mitral valve pathology and mitral regurgitation (MR) are very common in patients with hypertrophic cardiomyopathy (HCM), and the evaluation of mitral valve anatomy and degree of MR is important in patients with HCM. The aim of our study was [...] Read more.
Background and Objectives: Mitral valve pathology and mitral regurgitation (MR) are very common in patients with hypertrophic cardiomyopathy (HCM), and the evaluation of mitral valve anatomy and degree of MR is important in patients with HCM. The aim of our study was to examine the potential influence of moderate or moderately severe MR on the prognosis, clinical presentation, and structural characteristics of HCM patients. Materials and Methods: A prospective study examined 176 patients diagnosed with primary asymmetric HCM. According to the severity of the MR, the patients were divided into two groups: Group 1 (n = 116) with no/trace or mild MR and Group 2 (n = 60) with moderate or moderately severe MR. All patients had clinical and echocardiographic examinations, as well as a 24 h Holter ECG. Results: Group 2 had significantly more often the presence of the obstructive type of HCM (p < 0.001), syncope (p = 0.030), NYHA II class (p < 0.001), and atrial fibrillation (p = 0.023). Also, Group 2 had an enlarged left atrial dimension (p < 0.001), left atrial volume index (p < 0.001), and indirectly measured systolic pressure in the right ventricle (p < 0.001). Patients with a higher grade of MR had a significantly higher E/e′ (p < 0.001) and, as a result, higher values of Nt pro BNP values (p < 0.001) compared to Group 1. Kaplan–Meier analysis demonstrated that the event-free survival rate during a median follow-up of 88 (IQR 40–112) months was significantly higher in Group 1 compared to Group 2 (84% vs. 45% at 8 years; log-rank 20.4, p < 0.001). After adjustment for relevant confounders, the presence of moderate or moderately severe MR remained as an independent predictor of adverse outcomes (HR 2.788; 95% CI 1.221–6.364, p = 0.015). Conclusions: The presence of moderate or moderately severe MR was associated with unfavorable long-term outcomes in HCM patients. Full article
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Review

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27 pages, 1351 KiB  
Review
Hypertrophic Cardiomyopathy: Genetic Foundations, Outcomes, Interconnections, and Their Modifiers
by Mila Glavaški, Lazar Velicki and Nataša Vučinić
Medicina 2023, 59(8), 1424; https://doi.org/10.3390/medicina59081424 - 4 Aug 2023
Cited by 3 | Viewed by 2768
Abstract
Hypertrophic cardiomyopathy (HCM) is the most prevalent heritable cardiomyopathy. HCM is considered to be caused by mutations in cardiac sarcomeric protein genes. Recent research suggests that the genetic foundation of HCM is much more complex than originally postulated. The clinical presentations of HCM [...] Read more.
Hypertrophic cardiomyopathy (HCM) is the most prevalent heritable cardiomyopathy. HCM is considered to be caused by mutations in cardiac sarcomeric protein genes. Recent research suggests that the genetic foundation of HCM is much more complex than originally postulated. The clinical presentations of HCM are very variable. Some mutation carriers remain asymptomatic, while others develop severe HCM, terminal heart failure, or sudden cardiac death. Heterogeneity regarding both genetic mutations and the clinical course of HCM hinders the establishment of universal genotype–phenotype correlations. However, some trends have been identified. The presence of a mutation in some genes encoding sarcomeric proteins is associated with earlier HCM onset, more severe left ventricular hypertrophy, and worse clinical outcomes. There is a diversity in the mechanisms implicated in the pathogenesis of HCM. They may be classified into groups, but they are interrelated. The lack of known supplementary elements that control the progression of HCM indicates that molecular mechanisms that exist between genotype and clinical presentations may be crucial. Secondary molecular changes in pathways implicated in HCM pathogenesis, post-translational protein modifications, and epigenetic factors affect HCM phenotypes. Cardiac loading conditions, exercise, hypertension, diet, alcohol consumption, microbial infection, obstructive sleep apnea, obesity, and environmental factors are non-molecular aspects that change the HCM phenotype. Many mechanisms are implicated in the course of HCM. They are mostly interconnected and contribute to some extent to final outcomes. Full article
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