Journal Article

Derivation and validation of diagnostic models for myocardial fibrosis in duchenne muscular dystrophy: assessed by multi-parameter cardiovascular magnetic resonance

Fu, Hang, Xu, Ke, Cai, Xiao-tang, Xu, Hua-yan, Xu, Rong, Zhou, Zi-qi, Guo, Ying-kun

2023

Overview

Background Gadolinium-enhanced cardiovascular magnetic resonance (CMR) is the most widely used approach for diagnosing myocardial fibrosis with late gadolinium enhancement (LGE) in cardiomyopathy associated with Duchenne muscular dystrophy. Given the limitations and safety of gadolinium use, we wanted to develop and evaluate multi-parametric pre-contrast CMR models for the diagnosis of LGE and investigate whether they could be utilised as surrogates for LGE in DMD patients. Methods A total of 136 DMD patients were prospectively recruited and separated into LGE − and LGE + groups. In the first subset of patients (derivation cohort), regression models for the diagnosis of LGE were built by logistic regression using pre-contrast sequence parameters. In a validation cohort of other patients, the models’ performances were evaluated. Results EF, native T1 and longitudinal strain alone, as well as their combinations form seven models. The model that included EF, native T1 and longitudinal strain had the best diagnostic value, but there was no significant difference in diagnostic accuracy among the other models except EF. In the validation cohort, the diagnosis outcomes of models were moderate consistent with the existence of LGE. The longitudinal strain outperformed the other models in terms of diagnostic value (sensitivity: 83.33%, specificity: 54.55%). Conclusions Pre-contrast sequences have a moderate predictive value for LGE. Thus, pre-contrast parameters may be considered only in a specific subset of DMD patients who cannot cooperate for long-time examinations and have contradiction of contrast agent to help predict the presence of LGE. Trial registration number (TRN) ChiCTR1800018340 Date of registration 20180107

• Publisher: BioMed Central,BioMed Central Ltd,Springer Nature B.V,BMC
• Subject: Cardiac function / Cardiomyopathies - complications / Cardiomyopathies - diagnostic imaging / Cardiomyopathy / Cardiovascular disease / Cardiovascular magnetic resonance / Complications and side effects / Contrast Media / Diagnosis / Duchenne muscular dystrophy / Duchenne's muscular dystrophy / Fibrosis / Gadolinium / Heart / Heart diseases / Human Genetics / Humans / Late gadolinium enhancement / Longitudinal strain / Magnetic resonance imaging / Magnetic Resonance Spectroscopy / Medical diagnosis / Medical research / Medicine / Medicine & Public Health / Methods / Muscular Dystrophy, Duchenne - diagnostic imaging / Myocardium - pathology / Native T1 / Patient compliance / Pharmacology/Toxicology / Rare cardiovascular diseases / Rare diseases / Regression analysis / Risk factors / Software / Ventricular Function, Left