Clinical Research Directory

Myocardial Perfusion CMR for Differentiating and Characterizing Hypertrophic Cardiomyopathy Phenotypes

This observational study aims to evaluate myocardial perfusion abnormalities using quantitative and qualitative cardiac magnetic resonance (CMR) perfusion imaging in patients with hypertrophic cardiomyopathy (HCM) phenotypes, including sarcomeric and non-sarcomeric HCM, Anderson-Fabry disease (AFD), and cardiac amyloidosis. The study will also include first-degree relatives of affected patients and genetic mutation carriers. By comparing myocardial blood flow and perfusion patterns across these different conditions, the study seeks to identify distinctive perfusion signatures that may improve diagnostic differentiation, support risk stratification, and provide insights into the role of ischemia in fibrosis progression, arrhythmias, and long-term outcomes.

Left Bundle Branch Pacing in Patients With Hypertrophic Cardiomyopathy After Myectomy

Pilot interventional randomized clinical trial to study the efficacy of left bundle branch pacing in patients with hypertrophic cardiomyopathy after myectomy for the prevention of progression of heart failure, prevent the occurrence of life-threatening rhythm disturbances and promote reverse remodeling of the LV. The aim of the study is to evaluate the comparative efficacy and safety of implantation of a cardioverter-defibrillator with left bundle branch block pacing and a dual-chamber cardioverter-defibrillator in patients with HCM and complete left bundle branch block after myectomy at high risk of SCD. Objectives of the study: 1. To analyze the safety of ICD implantation procedures with LBBB pacing in patients with HCM and LBBB after myectomy at high risk of SCD; 2. To develop a technique for LBBB lead implantation in patients with HCM and LBBB after myectomy; 3. To conduct a comparative analysis of QRS complex duration data based on ECG data before and after surgery, LV activation time, and pacing threshold based on postoperative programming data; 4. To conduct a comparative analysis of the functional class of CHF, NT-proBNP, the presence/absence of interventricular and intraventricular dyssynchrony, the degree of diastolic dysfunction, LVEF, and LV EDV based on echocardiography data before and 12 months after surgery; 5. Conduct a comparative analysis of QRS complex duration data based on ECG data, LV activation time, pacing threshold, the presence of recorded episodes of AF, VT, VF, antitachycardia and shock therapy according to programming data at 3, 6, and 12 months after surgery; 6. Assess quality of life before and 12 months after surgery using the KCCQ-12 questionnaire; 30 patients (15 patients in each group) will be randomly separated into 2 groups. All participants go through ICD programming at 3, 6, and 12 months after myectomy, assessment of left ventricular remodeling based on ECG and echocardiography, NT-proBNP, assessment of quality of life before surgery and 12 months after surgery.

Automated Reports Generation of Cardiovascular Magnetic Resonance Imaging

The goal of this observational study is to evaluate the accuracy, completeness, and clinical consistency of large language model-generated cardiac magnetic resonance (CMR) imaging reports compared with expert radiologist reports in patients undergoing routine clinical CMR examinations. The main question(s) it aims to answer are: Can automatically generated CMR reports produced by a large multimodal model accurately reflect key imaging findings and diagnoses when compared with reports written by experienced cardiovascular radiologists? How does the quality of generated reports perform in terms of clinical correctness, completeness, and linguistic clarity, as assessed by quantitative metrics and expert review? If there is a comparison group: Researchers will compare AI-generated CMR reports with ground-truth reports authored by board-certified cardiovascular radiologists to see if the automated system achieves comparable diagnostic accuracy and report quality across different cardiac pathologies. Participants will: Undergo standard-of-care cardiac MRI examinations as part of routine clinical practice. Have their anonymized CMR image data and corresponding radiologist reports retrospectively collected. Contribute data that will be used to generate automated CMR reports, which will then be evaluated against expert reports using objective metrics (e.g., diagnostic agreement, entity-level accuracy) and subjective clinical scoring by radiologists.

Effect of Henagliflozin on Myocardial Fibrosis in Non-Obstructive HCM: A Randomized, Double-Blind, Placebo-Controlled Trial Using 68Ga/18F-FAPI PET/CMR

his is a single-center, randomized, double-blind, placebo-controlled clinical trial designed to evaluate the effect of Henagliflozin (an SGLT2 inhibitor) on myocardial fibrosis burden in patients with non-obstructive hypertrophic cardiomyopathy (nHCM). The study will use 68 68 Ga/ 18 18 F-FAPI PET/CMR imaging to quantitatively assess changes in active fibroblast activity after 6 months of treatment. A total of 150 eligible adult patients with nHCM (FAPI-positive at baseline, NYHA class I-III) will be enrolled and randomized in a 1:1 ratio to either the Henagliflozin group (10 mg once daily) or the placebo group for a 6-month treatment period. The primary endpoint is the change in myocardial FAPI target-to-background ratio (ΔTBR) at 6 months. Secondary endpoints include changes in FAPI SUVmax, FAPI burden percentage (FAV%), cardiac structure and function parameters, 6-minute walk distance, NYHA classification, NT-proBNP levels, and quality-of-life scores. Exploratory analyses will assess clinical events over 12 months, such as heart failure hospitalization, atrial fibrillation, ventricular arrhythmias, and cardiovascular death. The study employs stratified block randomization based on baseline FAPI burden, central randomization and blinding via IWRS, independent core laboratory imaging evaluation, and an intention-to-treat analytical approach. It aims to provide early evidence for the anti-fibrotic effect of Henagliflozin in nHCM and to validate FAPI-PET/CMR as an imaging biomarker for fibrosis activity.

Stratification, Management, and Guidance of Hypertrophic Cardiomyopathy Patients Using Hybrid Digital Twin Solutions

The goal of SMASH-HCM is to develop a digital twin or virtual model of the heart and vascular system with sympathetic nerve control that integrates multi-scale and multi-organ spatiotemporal biophysical data from a multitude of sources. SMASH-HCM's digital twin powered platform will dramatically improve hypertrophic cardiomyopathy (HCM) patient stratification and disease management through stepwise deep phenotyping integrated in clinical and patient-guided workflows.

Efficacy of Mavacamten in Patients With Symptomatic Latent Obstructive Hypertrophic Cardiomyopathy

This study aimed to evaluate the efficacy and safety of Mavacamten compared to no treatment in patients with symptomatic latent obstructive hypertrophic cardiomyopathy. The trial was randomized into two groups: Mavacamten group and Non-Mavacamten group. Over the 30-week treatment period, patients underwent a series of assessments at predefined time points, including transthoracic echocardiography, electrocardiogram (ECG), Holter monitoring, NYHA functional classification, Kansas City Cardiomyopathy Questionnaire (KCCQ), and cardiac biomarkers.

An Observational Study of Subcutaneous Implantable Cardioverter Defibrillator in Patients with Hypertrophic Cardiomyopathy At High Risk of Sudden Cardiac Death

1. To observe the failure rate of S-ICD screening in HCM patients at high risk of SCD. 2. To observe the incidence of IAS in HCM patients with S-ICD implantation and explore the related factors of IAS.

Fabry Disease in High-risk Patients With Left Ventricular Hypertrophy: Prevalence and Implementation of a Clinical Score

This study aims to evaluate the prevalence of Fabry Disease (FD) among a cohort of high risk patients with left ventricular hypertrophy (LVH) presenting at the University Hospital Würzburg over the last 20 years. Fabry disease is a rare disease that is known to be consistently underdiagnosed due to its largely variable symptoms. Considering that an early Fabry diagnosis is crucial for maximum benefit from therapies available, screening for Fabry patients can contribute to preventing development and worsening of symptoms in Fabry patients with LVH. In addition, a positive diagnosis in a family member opens the possibility to diagnose further family members in an earlier stage of the disease, therefore allowing treatment of symptoms and organ manifestations before they become irreversible.

DZHK TORCH-Plus is a Registry for Patients With Cardiomyopathies and Serves as Source for Cardiovascular Research Studies

The DZHK TranslatiOnal Registry for CardiomyopatHies (DZHK TORCH) represents a unique resource of clinical data and high quality biological samples to enable innovative clinical and molecular studies on cardiomyopathies (CMP). As a multi-center German cardiomyopathy registry, TORCH has been prospectively admitting patients since December 2014. 2,300 patients were recruited as planned. Taken together, patient data showed that the prevalence of these diseases is much higher in men than in women, atrial fibrillation is common in all forms of CMPs as well as rare forms of disease indicate a higher risk and higher morbidity. This DZHK TORCH register is now to be expanded with a second phase (DZHK TORCH-Plus). The second phase DZHK TORCH-Plus consists of 4 main modules: 1. "Clinical phenotyping, follow-up \& biosampling" 2. "Genomics", 3. "Inflammation" and 4. "Biomarker". The central aims are 1) to significantly increase the number of probands (n = 4340) in order to better address the different types of CMPs, especially patients with rare CMP forms such as LVNC and ARVC or with probably molecularly explainable cardiomyopathies (familial DCM), 2) to prolong the longitudinal with a further follow-up to achieve sufficient events and thereby derive clinical recommendations for risk assessment, 3) to increase the number of probands with state-of-the-art phenotyping, 4) to pinpoint the effect of myocardial inflammation, fibrosis, gender and to determine or predict genotypes based for outcome, 5) to validate novel biomarkers developed in other DZHK studies, and 6) to foster active cooperation with international CMP registries and partners from industry.