• Journal of Chest Surgery
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• v.48 no.6
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• pp.422-425
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• 2015

Amyloid deposits in the heart are not exceptional in systemic amyloidosis. The clinical manifestations of cardiac amyloidosis may include restrictive cardiomyopathy, characterized by progressive diastolic and eventually systolic biventricular dysfunction; arrhythmia; and conduction defects. To the best of our knowledge, no previous cases of isolated tricuspid regurgitation as the initial manifestation of cardiac amyloidosis have been reported. We describe a rare case of cardiac amyloidosis that initially presented with severe tricuspid regurgitation in a 42-year-old woman who was successfully treated with tricuspid valve replacement. Unusual surgical findings prompted additional evaluation that established a diagnosis of plasma cell myeloma.

• Journal of the Korean Society of Radiology
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• v.82 no.2
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• pp.429-434
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• 2021

Amyloidosis is a multisystemic disease characterized by the accumulation of abnormal proteins in extracellular spaces in various organs, with frequent involvement of the myocardium. We report a case of a patient who had cardiac amyloidosis with a trend of reduction in native T1 and T2 values and extracellular volume fraction on serial cardiac magnetic resonance imaging after chemotherapy and stem cell transplantation. The native T1 value and the extracellular volume fraction are closely associated with tissue amyloid burden in amyloidosis patients. This case demonstrated that cardiac magnetic resonance imaging may be used as a non-invasive and quantitative biomarker in the treatment monitoring of amyloidosis.

• Investigative Magnetic Resonance Imaging
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• v.17 no.1
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• pp.33-40
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• 2013

Purpose : To evaluate late gadolinium enhancement (LGE) pattern of left ventricular (LV) myocardium and presence or absence of LGE in other regions of the heart on cardiac magnetic resonance (CMR) imaging in patients diagnosed with cardiac amyloidosis. Materials and Methods: From 2009 to 2011, 9 patients who were suspected cardiac amyloidosis underwent CMR. We retrospectively analyzed the presence or absence of LGE and enhancement pattern in LV myocardium, and the presence or absence of LGE in other chambers as well. Also we measured interatrial septal thickness (IST), relative signal intensities of atrial septum and epicardial fat over the left atrial (LA) cavity on delayed enhanced images. MRI parameters in these patients were compared to those of control group of patients with ischemic heart disease by Wilcoxon rank sum test. Results: Of nine patients, LGE were found in 8; subendocardial circumferential pattern in 4 and diffuse pattern in 4. LGE in right ventricle was observed in 7. IST was significantly increased in patients with cardiac amyloidosis (P = 0.02). Ratio of atrial septum to LA cavity and ratio of epicardial fat to LA cavity showed a significant difference (P = 0.0002 and P = 0.0006, respectively). Conclusion: In LGE CMR, subendocardial or diffuse enhancement pattern is a typical finding for patients with cardiac amyloidosis. Atrial septum and epicardial fat show relatively increased signal intensities over LA blood cavity.

• Korean Journal of Radiology
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• v.25 no.5
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• pp.426-437
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• 2024

Objective: Cardiac magnetic resonance (CMR) is a diagnostic tool that provides precise and reproducible information about cardiac structure, function, and tissue characterization, aiding in the monitoring of chemotherapy response in patients with light-chain cardiac amyloidosis (AL-CA). This study aimed to evaluate the feasibility of CMR in monitoring responses to chemotherapy in patients with AL-CA. Materials and Methods: In this prospective study, we enrolled 111 patients with AL-CA (50.5% male; median age, 54 [interquartile range, 49-63] years). Patients underwent longitudinal monitoring using biomarkers and CMR imaging. At follow-up after chemotherapy, patients were categorized into superior and inferior response groups based on their hematological and cardiac laboratory responses to chemotherapy. Changes in CMR findings across therapies and differences between response groups were analyzed. Results: Following chemotherapy (before vs. after), there were significant increases in myocardial T2 (43.6 ± 3.5 ms vs. 44.6 ± 4.1 ms; P = 0.008), recovery in right ventricular (RV) longitudinal strain (median of -9.6% vs. -11.7%; P = 0.031), and decrease in RV extracellular volume fraction (ECV) (median of 53.9% vs. 51.6%; P = 0.048). These changes were more pronounced in the superior-response group. Patients with superior cardiac laboratory response showed significantly greater reductions in RV ECV (-2.9% [interquartile range, -8.7%-1.1%] vs. 1.7% [-5.5%-7.1%]; P = 0.017) and left ventricular ECV (-2.0% [-6.0%-1.3%] vs. 2.0% [-3.0%-5.0%]; P = 0.01) compared with those with inferior response. Conclusion: Cardiac amyloid deposition can regress following chemotherapy in patients with AL-CA, particularly showing more prominent regression, possibly earlier, in the RV. CMR emerges as an effective tool for monitoring associated tissue characteristics and ventricular functional recovery in patients with AL-CA undergoing chemotherapy, thereby supporting its utility in treatment response assessment.

• Investigative Magnetic Resonance Imaging
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• v.23 no.2
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• pp.100-113
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• 2019

Sarcoidosis is a multisystem disease characterized by noncaseating granulomas. Cardiac involvement is known to have poor prognosis because it can manifest as a serious condition such as the conduction abnormality, heart failure, ventricular arrhythmia, or sudden cardiac death. Although early diagnosis and early treatment is critical to improve patient prognosis, the diagnosis of CS is challenging in most cases. Diagnosis usually relies on endomyocardial biopsy (EMB), but its diagnostic yield is low due to the incidence of patchy myocardial involvement. Guidelines for the diagnosis of CS recommend a combination of clinical, electrocardiographic, and imaging findings from various modalities, if EMB cannot confirm the diagnosis. Especially, the role of advanced imaging such as cardiac magnetic resonance (CMR) imaging and positron emission tomography (PET), has shown to be important not only for the diagnosis, but also for monitoring treatment response and prognostication. CMR can evaluate cardiac function and fibrotic scar with good specificity. Late gadolinium enhancement (LGE) in CMR shows a distinctive enhancement pattern for each disease, which may be useful for differential diagnosis of CS from other similar diseases. Effectively, T1 or T2 mapping techniques can be also used for early recognition of CS. In the meantime, PET can detect and quantify metabolic activity and can be used to monitor treatment response. Recently, the use of a hybrid CMR-PET has introduced to allow identify patients with active CS with excellent co-localization and better diagnostic accuracy than CMR or PET alone. However, CS may show various findings with a wide spectrum, therefore, radiologists should consider the possible differential diagnosis of CS including myocarditis, dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy, amyloidosis, and arrhythmogenic right ventricular cardiomyopathy. Radiologists should recognize the differences in various diseases that show the characteristics of mimicking CS, and try to get an accurate diagnosis of CS.

• Korean Journal of Radiology
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• v.23 no.6
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• pp.581-597
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• 2022

Left ventricular (LV) wall thickening, or LV hypertrophy (LVH), is common and occurs in diverse conditions including hypertrophic cardiomyopathy (HCM), hypertensive heart disease, aortic valve stenosis, lysosomal storage disorders, cardiac amyloidosis, mitochondrial cardiomyopathy, sarcoidosis and athlete's heart. Cardiac magnetic resonance (CMR) imaging provides various tissue contrasts and characteristics that reflect histological changes in the myocardium, such as cellular hypertrophy, cardiomyocyte disarray, interstitial fibrosis, extracellular accumulation of insoluble proteins, intracellular accumulation of fat, and intracellular vacuolar changes. Therefore, CMR imaging may be beneficial in establishing a differential diagnosis of LVH. Although various diseases share LV wall thickening as a common feature, the histologic changes that underscore each disease are distinct. This review focuses on CMR multiparametric myocardial analysis, which may provide clues for the differentiation of thickened myocardium based on the histologic features of HCM and its phenocopies.

• Korean Journal of Radiology
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• v.22 no.6
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• pp.880-889
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• 2021

Objective: This study aimed to investigate the regional amyloid burden and myocardial deformation using T1 mapping and strain values in patients with cardiac amyloidosis (CA) according to late gadolinium enhancement (LGE) patterns. Materials and Methods: Forty patients with CA were divided into 2 groups per LGE pattern, and 15 healthy subjects were enrolled. Global and regional native T1 and T2 mapping, extracellular volume (ECV), and cardiac magnetic resonance (CMR)-feature tracking strain values were compared in an intergroup and interregional manner. Results: Of the patients with CA, 32 had diffuse global LGE (group 2), and 8 had focal patchy or no LGE (group 1). Global native T1, T2, and ECV were significantly higher in groups 1 and 2 than in the control group (native T1: 1384.4 ms vs. 1466.8 ms vs. 1230.5 ms; T2: 53.8 ms vs. 54.2 ms vs. 48.9 ms; and ECV: 36.9% vs. 51.4% vs. 26.0%, respectively; all, p < 0.001). Basal ECV (53.7%) was significantly higher than the mid and apical ECVs (50.1% and 50.0%, respectively; p < 0.001) in group 2. Basal and mid peak radial strains (PRSs) and peak circumferential strains (PCSs) were significantly lower than the apical PRS and PCS, respectively (PRS, 15.6% vs. 16.7% vs. 26.9%; and PCS, -9.7% vs. -10.9% vs. -15.0%; all, p < 0.001). Basal ECV and basal strain (2-dimensional PRS) in group 2 showed a significant negative correlation (r = -0.623, p < 0.001). Group 1 showed no regional ECV differences (basal, 37.0%; mid, 35.9%; and apical, 38.3%; p = 0.184). Conclusion: Quantitative T1 mapping parameters such as native T1 and ECV may help diagnose early CA. ECV, in particular, can reflect regional differences in the amyloid deposition in patients with advanced CA, and increased basal ECV is related to decreased basal strain. Therefore, quantitative CMR parameters may help diagnose CA and determine its severity in patients with or without LGE.

• Korean Journal of Radiology
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• v.1 no.1
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• pp.33-37
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• 2000

Objective: To describe the radiologic findings of migrating lobar atelectasis of the right lung. Materials and Methods: Chest radiographs (n = 6) and CT scans (n = 5) of six patients with migrating lobar atelectasis of the right lung were analyzed retrospectively. The underlying diseases associated with lobar atelectasis were bronchogenic carcinoma (n = 4), bronchial tuberculosis (n = 1), and tracheobronchial amyloidosis (n = 1). Results: Atelectasis involved the right upper lobe (RUL) (n = 3) and both the RUL and right middle lobe (RML) (n = 3). On supine anteroposterior radiographs (n = 5) and on an erect posteroanterior radiograph (n = 1), the atelectatic lobe(s) occupied the right upper lung zone, with a wedge shape abutting onto the right mediastinal border. On erect posteroanterior radiographs (n = 6), the heavy atelectatic lobe(s) migrated downward, forming a peri- or infrahilar area of increased opacity and obscuring the right cardiac margin. Erect lateral radiographs (n = 4) showed inferior shift of the anterosuperiorly located atelectatic lobe(s) to the anteroinferior portion of the hemithorax. Conclusion: Atelectatic lobe(s) can move within the hemithorax according to changes in a patient s position. This process involves the RUL or both the RUL and RML.

• Investigative Magnetic Resonance Imaging
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• v.16 no.2
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• pp.189-194
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• 2012

We report a case of cardiac lymphoma in a 40-year-old man, who had a mediastinal mass which was diagnosed as sclerosing mediastinitis pathologically. The mediastinal mass caused right pulmonary arterial stenosis. The patient developed myocardial hypertrophy and echocardiography showed restrictive physiology and severely decreased left ventricle ejection fraction, 6 months later. MRI showed global left ventricular myocardial hypertrophy and diffuse late gadolinium hyperenhancement after administration of contrast material. Thus, non-ischemic cardiomyopathy was suspected on MRI. However, pathology confirmed the myocardial abnormality as lymphoma after myocardial biopsy. Because a basal part of the left ventricle and global subendocardial myocardium were not involved on contrast-enhanced delayed MRI, the MRI abnormalities could be differentiated from amyloidosis and other myocardial diseases. The peculiar non-mass forming diffuse hypertrophy pattern of cardiac lymphoma has not been known in the MRI literature.

• Korean Journal of Radiology
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• v.23 no.12
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• pp.1251-1259
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• 2022

Objective: T1 mapping provides valuable information regarding cardiomyopathies. Manual drawing is time consuming and prone to subjective errors. Therefore, this study aimed to test a DL algorithm for the automated measurement of native T1 and extracellular volume (ECV) fractions in cardiac magnetic resonance (CMR) imaging with a temporally separated dataset. Materials and Methods: CMR images obtained for 95 participants (mean age ± standard deviation, 54.5 ± 15.2 years), including 36 left ventricular hypertrophy (12 hypertrophic cardiomyopathy, 12 Fabry disease, and 12 amyloidosis), 32 dilated cardiomyopathy, and 27 healthy volunteers, were included. A commercial deep learning (DL) algorithm based on 2D U-net (Myomics-T1 software, version 1.0.0) was used for the automated analysis of T1 maps. Four radiologists, as study readers, performed manual analysis. The reference standard was the consensus result of the manual analysis by two additional expert readers. The segmentation performance of the DL algorithm and the correlation and agreement between the automated measurement and the reference standard were assessed. Interobserver agreement among the four radiologists was analyzed. Results: DL successfully segmented the myocardium in 99.3% of slices in the native T1 map and 89.8% of slices in the post-T1 map with Dice similarity coefficients of 0.86 ± 0.05 and 0.74 ± 0.17, respectively. Native T1 and ECV showed strong correlation and agreement between DL and the reference: for T1, r = 0.967 (95% confidence interval [CI], 0.951-0.978) and bias of 9.5 msec (95% limits of agreement [LOA], -23.6-42.6 msec); for ECV, r = 0.987 (95% CI, 0.980-0.991) and bias of 0.7% (95% LOA, -2.8%-4.2%) on per-subject basis. Agreements between DL and each of the four radiologists were excellent (intraclass correlation coefficient [ICC] of 0.98-0.99 for both native T1 and ECV), comparable to the pairwise agreement between the radiologists (ICC of 0.97-1.00 and 0.99-1.00 for native T1 and ECV, respectively). Conclusion: The DL algorithm allowed automated T1 and ECV measurements comparable to those of radiologists.