Background: In patients with hypertrophic cardiomyopathy, the prognostic value of myocardial T1 and extracellular volume fraction for adverse cardiovascular events has not been well defined.
Methods: A total of 663 consecutive participants with hypertrophic cardiomyopathy who underwent 3T cardiovascular magnetic resonance were recruited. The follow-up end points included heart failure (HF)-related death, HF hospitalization, and sudden cardiac death or aborted sudden cardiac death.
Results: On Cox proportional hazards regression multivariable analyses, global native T1 excluding late gadolinium enhancement areas (hazard ratio [HR], 1.04 [95% CI, 0.99-1.09]; =0.094) and global extracellular volume fraction excluding late gadolinium enhancement (HR, 1.02 [95% CI, 0.95-1.10]; =0.565) were not associated with sudden cardiac death. Conversely, global native T1 (HR, 1.08 per 10 ms increase [95% CI, 1.02-1.16], =0.014; HR, 1.05 per 10 ms increase [95% CI, 1.01-1.09]; =0.009) and global extracellular volume fraction (HR, 1.23 per 1% increase [95% CI, 1.11-1.36], <0.001; HR, 1.10 per 1% increase [95% CI, 1.04-1.16]; <0.001) were independently associated with HF-related death and the composite end point of HF-related death or HF hospitalization in multivariable Cox models, respectively.
Conclusions: In this study of patients with hypertrophic cardiomyopathy, we found global native T1 and global extracellular volume fraction (excluding late gadolinium enhancement) to be both independently associated with HF-related events, but not sudden cardiac death in multivariable analysis. These findings are hypothesis-generating and will require external validation in larger cohorts.
Registration: URL: https://www.chictr.org.cn; Unique identifier: ChiCTR1900024094.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1161/CIRCIMAGING.124.017174 | DOI Listing |
Fabry disease (FD) is a lysosomal disorder due to alpha-galactosidase-A enzyme deficiency, accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3) which lead to proinflammatory effects. Males develop progressive hypertrophic cardiomyopathy (HCM) followed by fibrosis; females develop nonconcentric hypertrophy and/or early fibrosis. The inflammatory response to Gb3/lyso-Gb-3 accumulation is one of the suggested pathogenic mechanisms in FD cardiomyopathy when the secretion of inflammatory and transforming growth factors with infiltration of lymphocytes and macrophages into tissue promotes cardiofibrosis.
View Article and Find Full Text PDFSci Rep
March 2025
Department of Cardiovascular Ultrasound, The First Affiliated Hospital of China Medical University, 155 North Nanjing Street, Heping District, Shenyang, 110001, China.
Contemporary classification of hypertrophic cardiomyopathy (HCM) was mainly based on the site of myocardial hypertrophy and left ventricular outflow tract obstruction. A complementary classification based on left ventricular function could provide a powerful tool to identify individuals with high risk of adverse cardiovascular outcomes and guide individualized managements. Multi-dimensional echocardiographic parameters of left ventricular function derived from conventional echocardiography, tissue Doppler imaging, and speckle tracking echocardiography were obtained in 266 HCM patients and 169 healthy controls (HCs).
View Article and Find Full Text PDFArch Cardiovasc Dis
February 2025
Bordeaux University Hospital, 33000 Bordeaux, France; University of Bordeaux, 33000 Bordeaux, France; CIC-P 1401, 33600 Bordeaux-Pessac, France; Inserm 1045, 33600 Pessac, France.
Background: Patients with left ventricular hypertrophy (LVH) often maintain preserved left ventricular ejection fraction in the early stages of the disease. There is a need to identify simple and reliable variables beyond left ventricular ejection fraction to recognize those at risk of developing adverse clinical outcomes.
Aims: To examine left atrial (LA) strain in patients with hypertrophic cardiomyopathy (HCM), cardiac amyloidosis (CA) and Fabry disease (FD), pathologies known to cause LVH, and the relationship between LA strain and adverse clinical outcomes.
J Mol Cell Cardiol
March 2025
Department of Cardiology, Translational Research Center for Regenerative Medicine and 3D Printing Technologies, Guangdong Provincial Key Laboratory of Major Obstetric Diseases, Guangdong Provincial Clinical Research Center for Obstetrics and Gynecology, The Third Affiliated Hospital, Guangzhou Medical University, Guangzhou 510150, China. Electronic address:
Introduction: Lactylation is important for a variety of biological activities. It is reported that Class I histone deacetylases (HDAC1-3) are histone lysine delactylases. However, the role of lactylation in cardiac remodelling remains uncertain.
View Article and Find Full Text PDFEgypt Heart J
March 2025
Shri Vaishnav Vidyapeeth Vishwadvidyalaya, Indore, India.
Background: Cardiomyopathy is a heterogeneous group of myocardial disorders characterized by structural and functional abnormalities of the heart muscle. It is classified into primary (genetic, mixed, or acquired) and secondary categories, resulting in various phenotypes including dilated, hypertrophic, and restrictive patterns. Hypertrophic cardiomyopathy, the most common primary form, can cause exertional dyspnea, presyncope, and sudden cardiac death.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!