• 대한영상의학회지
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• 제81권2호
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• pp.302-309
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• 2020

파브리병(Fabry disease)은 매우 드문 X-연관 유전 대사 질환으로 알파 갈락토시다아제(alpha galactosidase A)의 결핍으로 인하여 다양한 세포 및 기관에 글리코스핑고지질(glycosphingolipid)의 축적을 초래하는 질환이다. 심장 침범이 비교적 흔하며 비정상적인 지질침착으로 인한 심근 염증, 좌심실 비대 및 심근 섬유증을 일으킨다. 심장 침범은 환자 예후를 결정하는 중요한 요인이므로 이를 진단하는 것은 매우 중요하다. 심장 자기공명영상은 심실의 기능, 부피 측정을 위한 표준기법으로 알려져 있으며 심근의 조직 변화를 볼 수 있는 유용한 기법이다. 특히 최근 많이 쓰이는 T1 지도화 기법을 통한 심근 조영 전 T1 수치를 이용하여 파브리병의 심장 침범을 조기 진단할 수 있으며 자기공명영상을 이용한 심근 질량 측정으로 치료 모니터링을 할 수 있다. 심장 자기공명영상은 파브리병 환자에서 다양한 역할을 할 수 있을 것으로 생각되며 이에 대해 정리해보고자 한다.

• The Korean Journal of Physiology and Pharmacology
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• 제28권4호
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• pp.335-344
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• 2024

Diphenyleneiodonium (DPI) has been widely used as an inhibitor of NADPH oxidase (Nox) to discover its function in cardiac myocytes under various stimuli. However, the effects of DPI itself on Ca²⁺ signaling and contraction in cardiac myocytes under control conditions have not been understood. We investigated the effects of DPI on contraction and Ca²⁺ signaling and their underlying mechanisms using video edge detection, confocal imaging, and whole-cell patch clamp technique in isolated rat cardiac myocytes. Application of DPI suppressed cell shortenings in a concentration-dependent manner (IC₅₀ of ≅0.17 µM) with a maximal inhibition of ~70% at ~100 µM. DPI decreased the magnitude of Ca²⁺ transient and sarcoplasmic reticulum Ca²⁺ content by 20%-30% at 3 µM that is usually used to remove the Nox activity, with no effect on fractional release. There was no significant change in the half-decay time of Ca²⁺ transients by DPI. The L-type Ca²⁺ current (I_Ca) was decreased concentration-dependently by DPI (IC₅₀ of ≅40.3 µM) with ≅13.1%-inhibition at 3 µM. The frequency of Ca²⁺ sparks was reduced by 3 µM DPI (by ~25%), which was resistant to a brief removal of external Ca²⁺ and Na⁺. Mitochondrial superoxide level was reduced by DPI at 3-100 µM. Our data suggest that DPI may suppress L-type Ca²⁺ channel and RyR, thereby attenuating Ca²⁺-induced Ca²⁺ release and contractility in cardiac myocytes, and that such DPI effects may be related to mitochondrial metabolic suppression.

• Investigative Magnetic Resonance Imaging
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• 제24권3호
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• pp.141-153
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• 2020

Purpose: Myocardial T1 and T2 relaxation times are affected by technical factors such as cardiovascular magnetic resonance platform/vendor. We aimed to validate T1 and T2 mapping sequences using a phantom; establish reference T1, T2, and extracellular volume (ECV) measurements using two sequences at 3T in normal Koreans; and compare the protocols and evaluate the differences from previously reported measurements. Materials and Methods: Eleven healthy subjects underwent cardiac magnetic resonance imaging (MRI) using 3T MRI equipment (Verio, Siemens, Erlangen, Germany). We did phantom validation before volunteer scanning: T1 mapping with modified look locker inversion recovery (MOLLI) with 5(3)3 and 4(1)3(1)2 sequences, and T2 mapping with gradient echo (GRE) and TrueFISP sequences. We did T1 and T2 mappings on the volunteers with the same sequences. ECV was also calculated with both sequences after gadolinium enhancement. Results: The phantom study showed no significant differences from the gold standard T1 and T2 values in either sequence. Pre-contrast T1 relaxation times of the 4(1)3(1)2 protocol was 1142.27 ± 36.64 ms and of the 5(3)3 was 1266.03 ± 32.86 ms on the volunteer study. T2 relaxation times of GRE were 40.09 ± 2.45 ms and T2 relaxation times of TrueFISP were 38.20 ± 1.64 ms in each. ECV calculation was 24.42% ± 2.41% and 26.11% ± 2.39% in the 4(1)3(1)2 and 5(3)3 protocols, respectively, and showed no differences at any segment or slice between the sequences. We also calculated ECV from the pre-enhancement T1 relaxation time of MOLLI 5(3)3 and the post-enhancement T1 relaxation time of MOLLI 4(1)3(1)2, with no significant differences between the combinations. Conclusion: Using phantom-validated sequences, we reported the normal myocardial T1, T2, and ECV reference values of healthy Koreans at 3T. There were no statistically significant differences between the sequences, although it has limited statistical value due to the small number of subjects studied. ECV showed no significant differences between calculations based on various pre- and post-mapping combinations.

• Investigative Magnetic Resonance Imaging
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• 제17권3호
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• pp.200-206
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• 2013

목적: 자기공명 심장영상을 이용한 좌심실 파라미터 측정에 있어, 자동화 방식을 적용하였을 경우에 나타나는 효과를 분석하고 이를 수동 및 반자동 방식을 적용했을 경우 나타나는 결과와 비교하였다. 대상과 방법: 1.5T 자기공명 심장영상 촬영을 시행한 62명의 환자를 대상으로, 심장 주기당 20상의 단축 항정상태 자유세차 동영상과 심첨2방 및 심첨4방 영상을 얻었다. 심내막 경계와 심외막 경계를 수동, 자동, 반자동 방식으로 각각 구하여 이를 바탕으로 이완말기와 수축말기 용적, 박출 계수, 일회 박출량, 좌심실 질량을 계산하고 각 방식간 평균값 차이를 일원분산분석법을 이용 통계적 분석하였다. 결과: 이완말기와 수축말기 용적의 경우에는 세 방식으로 측정한 결과는 통계적으로 유의하게 다르지 않았다. (P = .399 and .145). 그러나, 박출 계수, 일회 박출량, 좌심실 질량의 경우에는 통계적으로 유의하게 다르게 나타났으며 (P=.001, < 001, < 001) 자동화 방식으로 측정한 측정치가 다른 두 방식에 비해 일관되게 큰 결과치를 보였다. 결론: 자동화 방식을 적용하여 측정한 좌심실의 박출 계수, 일회 박출량, 좌심실 질량의 측정치는 수동, 반자동 방식에 비해 과장된 값을 나타낸다. 자동화 방식으로 많은 노력을 절감할 수 있으나, 임상적으로 민감한 케이스에 대해서는 이에 더하여 수동적 교정을 고려해야 할 것이다.

• Clinical and Experimental Pediatrics
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• 제57권7호
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• pp.297-303
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• 2014

Transcatheter closure of atrial septal defects has become a popular procedure. The availability of a preprocedural imaging study is crucial for a safe and successful closure. Both the anatomy and morphology of the defect should be precisely evaluated before the procedure. Three-dimensional (3D) echocardiography and cardiac computed tomography are helpful for understanding the morphology of a defect, which is important because different defect morphologies could variously impact the results. During the procedure, real-time 3D echocardiography can be used to guide an accurate closure. The safety and efficiency of transcatheter closures of atrial septal defects could be improved through the use of detailed imaging studies.

• Nuclear Medicine and Molecular Imaging
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• 제43권3호
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• pp.207-214
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• 2009

Positron emission tomogrpahy (PET) represents the most advanced scintigraphic imaging technology. With the increase in availability of PET, the clinical use of PET has grown in medical fields. This can be employed for cardiovascular research as well as for clinical applications in patients with various cardiovascular disease. PET allows non-invasive functional assessment of myocardial perfusion, substrate metabolism and cardiac innervation and receptors as well as gene expression in vivo. PET is regarded as the gold standard for the detection of myocardial viability, and it is the only method available for the quantitative assessment of myocardial blood flow. This review focuses on the clinical applications of myocardial perfusion PET in coronary artery disease.

• Journal of Veterinary Science
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• 제22권4호
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• pp.57.1-57.7
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• 2021

In a 3-year-old Samoyed, aortic bulging was found on radiography during a general check-up. On echocardiography, turbulent flow was found in left ventricular outflow tract (LVOT) with high velocity (6.1 m/s). A linear structure was attached to the interventricular septum and connected to the chordae tendineae reaching the papillary muscle. A part of the structure moved during cardiac cycle, similar to mitral motion. This dog was diagnosed with LVOT obstruction caused by accessory mitral valve tissue (AMVT). This is the first report of AMVT in veterinary medicine. AMVT should be considered as a possible cause of LVOT obstruction in dogs.

• 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.

• Clinical Endoscopy
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• 제56권6호
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• pp.681-692
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• 2023

In general, gastroesophageal reflux disease (GERD) is diagnosed clinically based on typical symptoms and/or response to proton pump inhibitor treatment. Upper gastrointestinal endoscopy is reserved for patients presenting with alarm symptoms, such as dysphagia, odynophagia, significant weight loss, gastrointestinal bleeding, or anorexia; those who meet the criteria for Barrett's esophagus screening; those who report a lack or partial response to proton pump inhibitor treatment; and those with prior endoscopic or surgical anti-reflux interventions. Newer endoscopic techniques are primarily used to increase diagnostic yield and provide an alternative to medical or surgical treatment for GERD. The available endoscopic modalities for the diagnosis of GERD include conventional endoscopy with white-light imaging, high-resolution and high-magnification endoscopy, chromoendoscopy, image-enhanced endoscopy (narrow-band imaging, I- SCAN, flexible spectral imaging color enhancement, blue laser imaging, and linked color imaging), and confocal laser endomicroscopy. Endoscopic techniques for treating GERD include esophageal radiofrequency energy delivery/Stretta procedure, transoral incisionless fundoplication, and endoscopic full-thickness plication. Other novel techniques include anti-reflux mucosectomy, peroral endoscopic cardiac constriction, endoscopic submucosal dissection, and endoscopic band ligation. Currently, many of the new endoscopic techniques are not widely available, and their use is limited to centers of excellence.

• 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.