• Journal of mucopolysaccharidosis and rare diseases
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• 제4권1호
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• pp.21-25
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• 2018

Fabry disease is a hereditary lysosomal storage disorder caused by the reduction or absence of lysosomal enzyme alpha-galactosidase A and the accumulation of glycosphingolipids, such as globotriaosylceramide (Gb3), in various organs, including the heart. The prevention of cardiac involvement in Fabry disease can only be achieved by enzyme replacement therapy (ERT), and the method of assessing the efficacy of ERT should be confirmed. Changes in the electrocardiogram, such as the shortening of PQ interval, prolongation of QTc and repolarization abnormalities as well as left ventricular hypertrophy in voltage criteria, can be used to identify Fabry disease patients; however, the usefulness of electrocardiograms for evaluating the efficacy of ERT is limited. The assessment of left ventricular hypertrophy using echocardiography has been established to evaluate the efficacy of ERT during long-term period. A new technique involving speckled tracking method might be useful for detecting early cardiac dysfunction and identifying the effect of ERT for a relatively short period. The estimation of left ventricular hypertrophy using cardiac magnetic resonance (CMR) is also useful for assessing the efficacy of ERT. Identifying late gadolinium enhancement in CMR may affect the effectiveness of ERT, and the new technique of T1 mapping might be useful for monitoring the accumulation of Gb3 during ERT. Histopathology in cardiac biopsy specimens is another potentially useful method for identifying the accumulation of GB3; however, the use of histopathology to evaluate of the efficacy of ERT is limited because of the invasive nature of an endomyocardial biopsy.

• 한국전자통신학회논문지
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• 제5권2호
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• pp.133-137
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• 2010

본 연구에서는 128채널 3차원 심장전기도 시스템의 영상화를 PC 기P반에서 구현하였다. 데이터를 받는 시간을 제외하더라도 실제적으로 구간을 설정하고 분석하는 시간은 1분도 걸리지 않아 짧은 시간 내에 분석해야 하는 심장 수술시 매우 유용함을 알 수 있었다. 특히 PC 기반으로 구현되었기 때문에 이동은 물론 데이터 보관이 용이하다. 또한 수술후에도 언제든지 데이터를 불러내어 분석 할 수 있으며 원하는 결과를 쉽게 확인할 수 있다.

• Korean Journal of Radiology
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• 제20권6호
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• pp.1001-1002
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• 2019

• Journal of Cardiovascular Imaging
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• 제30권3호
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• pp.202-211
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• 2022

BACKGROUND: This study aims to investigate normal changes throughout aging of the heart in cardiac magnetic resonance (CMR) imaging in healthy volunteers. While type 2 diabetes mellitus is a frequent finding in the elderly population, also the influence of this circumstance in otherwise healthy persons is part of our study. METHODS: In this prospective single-center trial, 75 healthy subjects in distinct age groups and 10 otherwise healthy diabetics were enrolled. All subjects underwent functional, flow sensitive, native T2- and T1-mapping in a 1.5T CMR scanner. RESULTS: No differences in right and left ventricular ejection fractions were observed between aging healthy groups. Bi-ventricular volumes lowered significantly (p<0.001) between the age groups. There was also a significant decrease in myocardial T1 values, aortic distensibility, and left ventricular peak diastolic strain rates. There were no differences in T2 mapping and the other deformation parameters. Patients with type 2 diabetes mellitus had lower end-diastolic volume indexes; all the other measurements were comparable. CONCLUSIONS: Aging processes in the healthy heart involve a decrease in ventricular volumes, with ejection fractions remaining normal. Stiffening of the myocardium and aorta and a decrease in T1 values are potential indications of age-related remodeling. Type 2 diabetes mellitus seems to have no major influence on aging processes of the heart.

• 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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• 제2권3호
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• pp.121-131
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• 2001

Echocardiography and catheterization angiography suffer certain limitations in the evaluation of congenital heart diseases in adults, though these are overcome by MRI, in which a wide field-of view, unlimited multiplanar imaging capability and three-dimensional contrast-enhanced MR angiography techniques are used. In adults, recently introduced fast imaging techniques provide cardiac MR images of sufficient quality and with less artifacts. Ventricular volume, ejection fraction, and vascular flow measurements, including pressure gradients and pulmonary-to-systemic flow ratio, can be calculated or obtained using fast cine MRI, phase-contrast MR flow-velocity mapping, and semiautomatic analysis software. MRI is superior to echocardiography in diagnosing partial anomalous pulmonary venous connection, unroofed coronary sinus, anomalies of the pulmonary arteries, aorta and systemic veins, complex heart diseases, and postsurgical sequelae. Biventricular function is reliably evaluated with cine MRI after repair of tetralogy of Fallot, and Senning's and Mustard's operations. MRI has an important and growing role in the morphologic and functional assessment of congenital heart diseases in adolescents and adults.

• Korean Journal of Radiology
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• 제24권9호
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• pp.838-848
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• 2023

Objective: To quantitatively analyze the cardiac magnetic resonance imaging (CMR) characteristics of chemotherapy-related cardiac dysfunction (CTRCD) and explore their prognostic value for major adverse cardiovascular events (MACE). Materials and Methods: A total of 145 patients (male:female = 76:69, mean age = 63.0 years) with cancer and heart failure who underwent CMR between January 2015 and January 2021 were included. CMR was performed using a 3T scanner (Siemens). Biventricular functions, native T1 T2, extracellular volume fraction (ECV) values, and late gadolinium enhancement (LGE) of the left ventricle (LV) were compared between those with and without CTRCD. These were compared between patients with mild-to-moderate CTRCD and those with severe CTRCD. Cox proportional hazard regression analysis was used to evaluate the association between the CMR parameters and MACE occurrence during follow-up in the CTRCD patients. Results: Among 145 patients, 61 had CTRCD and 84 did not have CTRCD. Native T1, ECV, and T2 were significantly higher in the CTRCD group (1336.9 ms, 32.5%, and 44.7 ms, respectively) than those in the non-CTRCD group (1303.4 ms, 30.5%, and 42.0 ms, respectively; P = 0.013, 0.010, and < 0.001, respectively). They were not significantly different between patients with mild-to-moderate and severe CTRCD. Indexed LV mass was significantly smaller in the CTRCD group (65.0 g/m² vs. 78.9 g/mm²; P < 0.001). According to the multivariable Cox regression analysis, T2 (hazard ratio [HR]: 1.14, 95% confidence interval [CI]: 1.01-1.27; P = 0.028) and quantified LGE (HR: 1.07, 95% CI: 1.01-1.13; P = 0.021) were independently associated with MACE in the CTRCD patients. Conclusion: Quantitative parameters from CMR have the potential to evaluate myocardial changes in CTRCD. Increased T2 with reduced LV mass was demonstrated in CTRCD patients even before the development of severe cardiac dysfunction. T2 and quantified LGE may be independent prognostic factors for MACE in patients with CTRCD.

• 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.

• Korean Journal of Radiology
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• 제24권12호
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• pp.1200-1220
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• 2023

Dilated cardiomyopathy (DCM) is one of the most common types of non-ischemic cardiomyopathy. DCM is characterized by left ventricle (LV) dilatation and systolic dysfunction without coronary artery disease or abnormal loading conditions. DCM is not a single disease entity and has a complex historical background of revisions and updates to its definition because of its diverse etiology and clinical manifestations. In cases of LV dilatation and dysfunction, conditions with phenotypic overlap should be excluded before establishing a DCM diagnosis. The differential diagnoses of DCM include ischemic cardiomyopathy, valvular heart disease, burned-out hypertrophic cardiomyopathy, arrhythmogenic cardiomyopathy, and non-compaction. Cardiac magnetic resonance (CMR) imaging is helpful for evaluating DCM because it provides precise measurements of cardiac size, function, mass, and tissue characterization. Comprehensive analyses using various sequences, including cine imaging, late gadolinium enhancement imaging, and T1 and T2 mapping, may help establish differential diagnoses, etiological work-up, disease stratification, prognostic determination, and follow-up procedures in patients with DCM phenotypes. This article aimed to review the utilities and limitations of CMR in the diagnosis and assessment of DCM.

• Investigative Magnetic Resonance Imaging
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• 제21권3호
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• pp.131-138
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• 2017

Purpose: To optimize the timing of scans using cardiac magnetic resonance contrast-enhanced timing robust angiography (CMR-CENTRA) for electroanatomic mapping (EAM) of the right atrium (RA) and left atrium (LA) in patients with atrial fibrillation (AF). Materials and Methods: Fifty patients with AF (38 men; mean age, $59.6{\pm}9.3years$) underwent CMR-CENTRA in preparation for EAM. The CMR-CENTRA data were acquired at five different scan times: 0 seconds, 5 seconds, 10 seconds, 15 seconds, and 20 seconds after an intravenous injection of contrast media. To evaluate the degree of contrast enhancement, right atrial relative contrast (RA-RC) and left atrial relative contrast (LA-RC) on the CMR-CENTRA scans were assessed at each time point. The three-dimensional (3D) reconstruction of the RA and LA for the EAM system was performed using the CMR-CENTRA data. Results: A CMR-CENTRA at a scan time of 10 seconds showed significantly greater LA-RC (P < 0.05) compared with all other scan times. A CMR-CENTRA at a scan time of 15 seconds showed significantly greater RA-RC (P < 0.05) compared with all other scan times. In the 3D reconstruction of the RA, the success rates of CMR-CENTRA at scan times of 10 seconds and 15 seconds were 18% and 100%, respectively. In the 3D reconstruction of the LA, the success rates of CMR-CENTRA at 10- and 15-second scan times were 100%. Conclusion: The CMR-CENTRA data acquired at 15 seconds after the injection of contrast media is appropriate for the preparation of an EAM system that is focused on the RA and LA in patients with AF.