An observational study in Hypertrophic Cardiomyopathy (HCM) and Cardiac Myosin Inhibitors, sponsored by Centro Cardiologico Monzino. Active, not recruiting at 1 site in Italy. Open to participants aged 18 Years and older. Per ClinicalTrials.gov, last updated 2026-07-10.
Sponsored by Centro Cardiologico Monzino · Observational
Hypertrophic cardiomyopathy (HCM) is one of the leading causes of sudden cardiac death (SCD), particularly in young and middle-aged individuals. Current arrhythmic risk stratification mainly relies on clinical and imaging-based models, including the ESC HCM Risk-SCD score and guideline-recommended risk markers. However, these approaches show only moderate predictive accuracy at the individual level, highlighting the need for novel biomarkers able to improve risk prediction.
Cardiac magnetic resonance (CMR) plays a central role in phenotypic characterization and prognostic assessment of HCM, particularly through the evaluation of late gadolinium enhancement (LGE), a marker of myocardial fibrosis. Recent studies suggest that radiomic analysis of LGE images can identify quantitative features of myocardial scar heterogeneity that provide additional prognostic information beyond conventional fibrosis burden assessment. Radiomics applied to pre-contrast cine CMR sequences may also capture quantitative features related to myocardial shape, texture, and contractile dynamics, potentially associated with myocardial disarray, mechanical alterations, and electromechanical instability.
Integration of CMR radiomics with genetic data may allow a more comprehensive characterization of the arrhythmic substrate in HCM. In obstructive hypertrophic cardiomyopathy (oHCM), left ventricular outflow tract obstruction is a major determinant of symptoms and prognosis. Mavacamten, a selective cardiac myosin inhibitor, has been shown to significantly reduce LVOT gradient and improve symptoms and cardiac remodeling. However, it remains unknown whether CMR radiomics can detect phenotypic changes associated with mavacamten treatment and whether these changes may contribute to dynamic reassessment of arrhythmic risk.
This is a prospective, multicenter, non-profit observational study enrolling adult patients diagnosed with hypertrophic cardiomyopathy (HCM) who undergo cardiac magnetic resonance (CMR) imaging as part of routine clinical practice.
The study aims to evaluate the role of CMR radiomics in predicting arrhythmic risk in patients with HCM and to investigate phenotypic evolution in patients with obstructive hypertrophic cardiomyopathy (oHCM) treated with Mavacamten. Specifically, the study will assess whether radiomic features extracted from CMR imaging can improve the identification of patients at risk of malignant ventricular arrhythmic events and whether radiomic analysis can detect phenotypic modifications associated with mavacamten treatment that may contribute to longitudinal reassessment of arrhythmic risk.
Eligible participants will include adult patients with a diagnosis of hypertrophic cardiomyopathy, including both obstructive and non-obstructive forms, who undergo clinically indicated CMR examinations. In addition, the study will include a subgroup of patients with obstructive HCM initiating treatment with mavacamten, for whom both baseline CMR assessment and longitudinal follow-up CMR evaluation will be available.
Approximately 2,000 patients are expected to be enrolled in the study. Clinical data, standard CMR parameters, and radiomic features extracted from imaging sequences will be collected and analyzed in order to develop predictive models for arrhythmic risk stratification and to evaluate potential imaging biomarkers associated with disease progression and treatment-related phenotypic changes.
347 studies on the registry are indexed under Cardiomyopathy, Hypertrophic; 110 are open to participants now.
This study's enrollment of 2,000 is above the median of 259 across 168 observational studies indexed under Cardiomyopathy, Hypertrophic.
Browse Cardiomyopathy, Hypertrophic studies →Centro Cardiologico Monzino is the lead sponsor of 69 studies on the registry; 24 are open to participants now.
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Adult patients with a diagnosis of hypertrophic cardiomyopathy (HCM), including both obstructive and non-obstructive forms, undergoing clinically indicated cardiac magnetic resonance (CMR) imaging as part of routine clinical practice will be included in the study.
The study will also include a subgroup of patients with obstructive hypertrophic cardiomyopathy (oHCM) initiating treatment with mavacamten, for whom both baseline CMR assessment and follow-up CMR evaluation will be available.
For the subgroup of patients with obstructive HCM treated with mavacamten only:
Exclusion Criteria:
Adult patients diagnosed with hypertrophic cardiomyopathy (HCM), including both obstructive and non-obstructive forms, undergoing clinically indicated cardiac magnetic resonance (CMR) imaging as part of routine clinical practice will be enrolled in the study. The study will also include a subgroup of patients with obstructive hypertrophic cardiomyopathy (oHCM) initiating treatment with mavacamten, for whom both baseline CMR assessment and longitudinal follow-up CMR evaluation will be available
Composite of malignant ventricular arrhythmic events
* Sustained ventricular tachycardia * Ventricular fibrillation * Appropriate implantable cardioverter-defibrillator (ICD) therapy * Sudden cardiac death Events will be adjudicated based on clinical records, device interrogation data, and death certificates where available.
Time frame: 60 months
Longitudinal changes in radiomic features in mavacamten-treated patients
Time frame: 60 months
Predictive performance of radiomic models
Time frame: 60 months
Incremental prognostic value over ESC and AHA/ACC models
Time frame: 60 months
Association between genotype and arrhythmic risk
Time frame: 60 months
This study is active, not recruiting, as verified in Jun 2026. You cannot join it, but the record below documents what was studied.
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Centro Cardiologico Monzino