• Journal of Chest Surgery
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• 제48권6호
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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.

• 대한영상의학회지
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• 제82권2호
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• pp.429-434
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• 2021

아밀로이드증은 세포 외 공간에 비정상적인 단백질이 축적되는 것을 특징으로 하는 전신 질환이며, 심근을 포함한 다양한 기관을 침범한다. 저자들은 심장 아밀로이드증 환자에서 항암 화학 요법 및 줄기세포 이식 후 심장 자기공명영상의 추적검사에서 조영 전 T1 및 T2 수치와 세포 외 부피 분율의 감소를 보인 사례를 보고한다. 조영 전 T1 이완시간 및 세포 외 부피 분율은 아밀로이드증 환자에서 조직의 아밀로이드 축적 정도와 밀접한 관련이 있다. 이 사례를 통해 T1 지도화 심장 자기공명영상 기법이 심장 아밀로이드증의 치료 반응 모니터링에 비침습적이며 정량적인 도구로서 큰 역할을 할 수 있는 가능성을 확인할 수 있다.

• Investigative Magnetic Resonance Imaging
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• 제17권1호
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• pp.33-40
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• 2013

목적 : 심장 아밀로이드증 환자의 심장 자기공명영상의 지연 조영증강영상에서 좌심근의 조영증강 패턴과 다른 심장부위의 조영증강 유무를 평가하고자 하였다. 대상 및 방법 : 2009년 9월부터 2011년 6월 사이 심장 아밀로이드증이 의심되어 심장 자기공명영상을 촬영한 9명의 환자를 후향적으로 분석하였다. 좌심근의 조영증강 여부 및 패턴, 그리고 좌우심방 및 우심실의 조영증강 유무를 평가하였다. 또한 심방중격 두께, 좌심방 혈액신호 강도에 대한 심방중격과 심외막 지방의 상대적 신호강도 비를 계산하여 허혈성 심질환 대조군 16명과 Wilcoxon rank sum test를 통해 통계적으로 비교분석 하였다. 결과 : 총 9명중 8명에서 좌심근 조영증강소견을 보였으며, 8명중 4명의 환자에서 심내막하 원형성, 나머지 4명에서 미만성 패턴을 보였다. 심방중격 두께는 심장 아밀로이드증 환자에서 허혈성 심질환 환자에 비해서 증가되어 있었고 (p = 0.02), 심장 아밀로이드증 환자군에서 심방중격 대 혈액 신호 강도비 (p = 0.0002) 및 심외막 지방 대 혈액 신호 강도비 (p = 0.0006) 모두 의미있게 높았다. 결론 : 좌심실의 심내막하 원형성 혹은 미만성 조영증강 패턴은 심장 아밀로이드증 환자의 지연 조영 심장 자기공명영상의 특징적인 소견이며, 심방중격 및 심외막 지방 대 혈액 신호 강도비 신호강도가 상대적으로 증가된다.

• Korean Journal of Radiology
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• 제25권5호
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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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• 제23권2호
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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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• 제23권6호
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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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• 제22권6호
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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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• 제1권1호
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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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• 제16권2호
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• pp.189-194
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• 2012

40세 남성에서 상대정맥의 폐쇄와 우폐동맥 협착을 동반한 종격동 종괴가 발견되어 병리학적 소견에 경화성 종격동염으로 진단되었고, 6개월 후 심장 자기공명영상(MRI)에서 비허혈성 심근병증으로 의심된 심장림프종의 예를 보고하고자 한다. 심장 초음파에서 제한적 양상의 심장 생리현상을 보였고 구혈율이 매우 감소되어 있었다. MRI에서 전반적인 심근비대와 조영제 주입 후 광범위한 지연조영증강이 있어 비허혈성 심근병증이 의심되었으며 심장근육 생검 후 림프종으로 확진되었다. 조영제 주입후 MRI에서 심근 기저부는 침범되지 않았고 전반적인 심내막 보존이 있었으므로 아밀로이드증과 구별되는 양상이고 종괴를 형성하지 않았기 때문에 심장종양 보다는 심근병증에 더 유사한 영상 소견을 보인 특이한 증례로서 심장림프종의 자기공명영상에서의 특징을 문헌고찰과 함께 보고하고자 한다.

• Korean Journal of Radiology
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• 제23권12호
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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.