論文ID: CJ-25-0517
A 21-year-old woman with systemic lupus erythematosus on hydroxychloroquine presented with pleuritic chest pain. Chest X-ray showed cardiomegaly (Figure 1A), and echocardiography revealed right heart enlargement, pericardial effusion, and severe pulmonary hypertension (PH). Pulmonary embolism was excluded by enhanced CT and ventilation–perfusion scan. Laboratory tests showed elevated level of B-type natriuretic peptide and mildly elevated liver enzymes without viral hepatitis or other chronic liver disease, including fatty liver, by abdominal ultrasound. She received intravenous diuretics for heart failure. On day 4, she underwent right heart catheterization. Her mean pulmonary arterial pressure (PAP) was 64 mmHg, but with normal pulmonary capillary wedge pressure, confirming Group 1 PH. Cardiac MRI (CMR) showed a reduced right ventricular ejection fraction (RVEF) of 18% (Figure 1B, Supplementary Movie 1), and increased myocardial native T1 values (Figure 1C,D). Although native T1 is not a specific marker of congestion, it reflects tissue characteristics such as fibrosis, edema, and inflammation. In this case, the myocardial and hepatic native T1 values were used as surrogate markers of congestion. She was treated with diuretics, steroid pulse therapy, and pulmonary vasodilators. Pericardial effusion and heart failure symptoms improved, and she was discharged.
(Panel 1) Images obtained during heart failure. ALT, alanine aminotransferase; AST, aspartate aminotransferase; BNP, B-type natriuretic peptide; FIB-4, fibrosis-4; LV, left ventricle; PAP, pulmonary arterial pressure; PCWP, pulmonary capillary wedge pressure; RAP, right atrial pressure; γ-GPT, γ-glutamyi transpeptidase; RV, right ventricle; T-Bil, total bilirubin. (Panel 2) Follow-up images obtained 6 months later.
At 6-month follow-up, chest X-ray (Figure 2A), CMR, and catheterization were repeated. Mean PAP had decreased to 34 mmHg, and RVEF had improved to 40% (Figure 2B, Supplementary Movie 2). Myocardial T1 values decreased, nearing normal in the septum (Figure 2C,D). Notably, the liver T1 value improved from 1,060 ms to 797 ms (Figure 1E,2E), indicating reversible hepatic congestion.
This case underscores the utility of MRI T1 mapping for detecting liver congestion, often missed by standard imaging. T1 mapping offers a sensitive, quantitative assessment of hepatic tissue changes related to hepatic congestion.1 Although liver enzyme abnormalities were mild, the T1 value markedly improved, highlighting its potential for monitoring cardiac and hepatic congestion beyond conventional biomarkers.
This work was supported in part by JSPS KAKENHI Grant Number 19K08501.
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Supplementary Movie 1. Cine CMR.
Supplementary Movie 2. Follow up cine CMR.
Please find supplementary file(s);
https://doi.org/10.1253/circj.CJ-25-0517
