An observational study in Cardiomyopathies, Amyloidosis Cardiac and Fabry Disease, sponsored by IRCCS Azienda Ospedaliero-Universitaria di Bologna. Recruiting at 3 sites in Italy. Open to participants aged 12 Years and older. Per ClinicalTrials.gov, last updated 2025-02-07.
Sponsored by IRCCS Azienda Ospedaliero-Universitaria di Bologna · Observational
The study aims to identify new diagnostic and prognostic markers for CMP that can help predict disease progression. In particular, the study will focus on microRNAs (miRNAs) and spatial transcriptomics, which are emerging techniques that may provide insights into the underlying disease mechanisms. By understanding these markers, the investigators hope to improve the way the investigators diagnose and manage CMP, particularly in terms of predicting progression to heart failure or heart transplantation.
The study will evaluate patients with hypertrophic cardiomyopathy (e.g., sarcomeric forms, Anderson-Fabry disease, AL, and TTR cardiac amyloidosis), dilated cardiomyopathy and arrhythmogenic cardiomyopathy. These patients will undergo clinical evaluations, including ECG, echocardiograms, CMR, biopsy analysis, and genetic testing, as well as molecular studies such as transcriptomics and miRNA analysis. This comprehensive approach aims to identify potential new biomarkers for diagnosing and predicting the disease course.
This is a non-pharmacological, national multicenter cohort study with both retrospective and prospective components.
For the prospective study, patients with CMP will be consecutively enrolled from the outpatient or inpatient settings of the IRCCS Azienda Ospedaliero-Universitaria di Bologna (coordinating center), Azienda Ospedaliero-Universitaria Careggi di Firenze, and the Department of Cardiothoracic and Vascular Sciences, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome. Enrollment will begin upon receipt of favorable opinions from the respective Ethics Committees and general management approvals at each center until the planned sample size is reached.
For the retrospective study, all patients managed at the aforementioned centers prior to obtaining Ethics Committee approval and general management clearance will be included.
The structured collection of the data necessary for the evaluation of the objectives will be carried out by creating a specific electronic database in which retrospective data will be collected starting from January 1, 1990, and for the prospective observation period from the moment of ethical committee approval and receipt of the clearance from the general director until December 31, 2027. The electronic archive may be used to obtain or confirm new scientific evidence in the field of CMP, particularly with reference to diagnosis, prognosis, and therapy. The electronic archive will be compiled through the consultation of the clinical records (outpatient or hospitalization), from which data regarding demographics, remote and recent medical history, family history, any genetic investigations, instrumental tests, information about symptoms at onset, and therapy will be collected. Aside from the analysis of miRNAs on tissues, which will be carried out for the study and will not in any way affect the patients' diagnostic-therapeutic path, all instrumental exams and therapies that patients will undergo are part of standard clinical practice. Any genetic investigations that patients may undergo are part of the regular care pathway and will be performed when clinically indicated, or if a precise etiological diagnosis is necessary, with consequent therapeutic implications, in the differential diagnosis between various forms of CMP, and when, in the context of familial forms, it may be useful for family screening. Patients will be enrolled during hospitalization or at the outpatient visit. After obtaining informed consent, patients will be evaluated based on clinical needs. There is no minimum duration for following the patient. In a subgroup of patients enrolled in the prospective study, additional molecular evaluations will be carried out on plasma and cardiac tissue. The blood sample-taken as per clinical practice-will be processed within 4 hours through centrifugation. After isolation, the plasma will be divided into aliquots of 500 µL and stored at -80°C. In the subpopulation of the study undergoing endomyocardial biopsy (diagnostic) or cardiac surgery (e.g., myectomy, valve replacement, etc.), according to the standard-of-care, fresh cardiac tissue will be collected. The cardiac tissue (taken during endomyocardial biopsy/cardiac surgery performed according to clinical practice) will be partially immersed in formalin and sent, as per normal procedure, to pathology where it will be processed for paraffin embedding, and a small fragment will be frozen and immediately embedded in OCT (optimal cutting temperature) and stored at -80°C. RNA will then be extracted and analyzed using NGS (next-generation sequencing) of small RNA, ddPCR (droplet digital Polymerase Chain Reaction), and spatial transcriptomics.
553 studies on the registry are indexed under Cardiomyopathies; 152 are open to participants now.
This study's planned enrollment of 700 is above the median of 200 across 263 observational studies indexed under Cardiomyopathies.
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The study plans to enroll a population of 700 patients affected by CMP evaluated both in the inpatient and outpatient regime, at
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Exclusion Criteria:
Underlined terms in the criteria open these too. Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Informed consent
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
risk stratification
Accuracy of clinical, instrumental, histological, and molecular variables in identifying critical CMP patients or those at risk of organ transplantation. For each marker and their combinations, specificity, sensitivity, and AUC will be assessed. Unit of Measurement: sensitivity, specificity, and Area Under the Curve (AUC)
Time frame: Enrollment in the prospective phase and collection of data for the retrospective phase: 30 months; Follow-up: 2 years, Data analysis: 6 months
Gene expression profile (transcriptomics) and microRNA analysis
The tissues analyzed will consist of blood (plasma microRNA) and cardiac tissue (MicroRNA Quantification and Spatial Transcriptomics) on endomyocardial biopsy/cardiac surgery samples performed for diagnostic/therapeutic purposes. These analysis will allow us to evaluate transcripts specific to each cell and/or small groups of cells by mapping their location within the tissue organization. Gene expression data will be explored in association with the tissue images using dedicated analysis software Unit of Measurement: number of miRNA copies/µL
Time frame: Enrollment in the prospective phase: 30 months, Follow-up: 2 years, Data analysis: 6 months
AL (Light Chain Amyloidosis) vs TTR (Transthyretin) differential diagnosis in cardiac amyloidosis
Development of a diagnostic model integrating clinical (NYHA class), instrumental (left ventricle mass in g/m², BNP in pg/mL, Troponin in ng/dL), and molecular (miRNA copies/µL) variables to differentiate AL vs TTR cardiac amyloidosis. Unit of Measurement: sensitivity, specificity, and Area Under the Curve (AUC)
Time frame: Enrollment: 30 months; Data analysis: 6 months
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