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Morphological, Functional, and Tissue Characterization of Silent Myocardial Involvement in Patients With Primary
Pan Jiang1, Zehao Feng1, Li Sheng1
1State Key Laboratory for Oncogenes and Related Genes, Shanghai Cancer Institute; Division of Gastroenterology and Hepatology, Division of Cardiology, Key Laboratory of Coronary Heart Disease, Shanghai Municipal Education Commission; Key Laboratory of Gastroenterology and Hepatology, Ministry of Health, Shanghai Institute of Digestive Disease, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Insights
Primary biliary cholangitis (PBC) patients show silent heart damage before cirrhosis. Cardiovascular magnetic resonance (CMR) reveals early myocardial impairment, enabling timely interventions for better outcomes.
Area of Science:
- Cardiology
- Hepatology
- Medical Imaging
Background:
- Cirrhotic cardiomyopathy is a severe complication in end-stage primary biliary cholangitis (PBC).
- Early-stage PBC may involve silent myocardial impairment before cirrhosis or cardiac symptoms.
- The EARLY-MYO-PBC study investigates subclinical cardiac changes in early PBC.
Purpose of the Study:
- To identify silent myocardial impairment in primary biliary cholangitis (PBC) patients without cardiac manifestations.
- To characterize early cardiac involvement using multi-modality imaging.
- To establish imaging biomarkers for early detection of cardiac abnormalities in PBC.
Main Methods:
- Prospective, multi-center study involving 112 subjects (56 PBC patients, 56 controls).
- Cardiovascular magnetic resonance (CMR) was used for cardiac imaging.
- Demographic, serologic, and imaging data were collected from participants without prior heart disease or symptoms.
Main Results:
- PBC patients exhibited subclinical myocardial changes on CMR, including hyperdynamic left ventricular ejection fraction and myocardial edema.
- Elevated extracellular matrix indices and impaired myocardial viability (positive late gadolinium enhancement) were observed in PBC patients.
- A distinct mid-wall "stripe" pattern on late gadolinium enhancement was identified as specific to PBC, with gp210 positivity, low hemoglobin, and BMI as independent predictors.
Conclusions:
- Clinically silent cardiac impairment with specific CMR patterns exists in early-stage PBC.
- CMR can identify early myocardial impairment in PBC patients.
- Findings support early screening and potential timely therapeutic interventions for cardiac involvement in PBC.
Background & Aims:
Cirrhotic cardiomyopathy is a major complication and cause of morbidity in end-stage primary biliary cholangitis (PBC). However, it is unclear whether there is clinically silent myocardial involvement at the early stage of PBC before cirrhosis and cardiac manifestations. This prospective, three-center, multi-modality cardiac imaging study on the early identification of myocardial impairment in PBC (EARLY-MYO-PBC) was designed to identify silent myocardial impairment in PBC patients without cardiac manifestations.
Methods:
A total of 112 subjects (56 with PBC and 56 age- and sex-matched controls) undergoing cardiovascular magnetic resonance (CMR) were enrolled. Demographic, serologic, and cardiac imaging data were prospectively collected. All participants had no cardiac discomfort or previous heart disease and had normal electrocardiographic findings.
Results:
Subclinical myocardial involvement, as evidenced by cardiac morphologic, functional, and tissue characterization changes on CMR, including hyperdynamic left ventricular (LV) ejection fraction (median, 75% in PBC patients vs 69% in controls, P = .029), subclinical myocardial edema by T2-short tau inversion recovery (21% vs 2% in controls, P = .001), elevated extracellular matrix indices (30% vs 26% in controls, P < .001), and impaired myocardial viability by positive late gadolinium enhancement (LGE) (36%), was detected in PBC patients. Importantly, a mid-wall "stripe" at the LV septum was identified as a PBC-specific LGE pattern that differs from other known cardiomyopathies. In multivariate analysis, gp210 positivity (odds ratio [OR] = 9.909, P = .010), lower hemoglobin (OR = 0.919, P = .004), and body mass index (OR = 0.638, P = .005) were independent predictors of cardiac abnormalities in PBC.
Conclusions:
This study demonstrates clinically silent cardiac impairment with specific CMR patterns in PBC, allowing optimal screening for early myocardial impairment and potentially timely therapies. (Trial registration no.: NCT03545672).
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