• Korean Journal of Radiology
• /
• 제22권1호
• /
• pp.97-105
• /
• 2021

Objective: The present study aimed to investigate the association between myocardial blood flow (MBF) quantified by dynamic CT myocardial perfusion imaging (CT-MPI) and the increments in heart rate (HR) after stress in patients without obstructive coronary artery disease. Materials and Methods: We retrospectively included 204 subjects who underwent both dynamic CT-MPI and coronary CT angiography (CCTA). Patients with more than minimal coronary stenosis (diameter ≥ 25%), history of myocardial infarction/revascularization, cardiomyopathy, and microvascular dysfunction were excluded. Global MBF at stress was measured using hybrid deconvolution and maximum slope model. Furthermore, the HR increments after stress were recorded. Results: The median radiation dose of dynamic CT-MPI plus CCTA was 5.5 (4.5-6.8) mSv. The median global MBF of all subjects was 156.4 (139.8-180.4) mL/100 mL/min. In subjects with HR increment between 10 to 19 beats per minute (bpm), the global MBF was significantly lower than that of subjects with increment between 20 to 29 bpm (153.3 mL/100 mL/min vs. 171.3 mL/100 mL/min, p = 0.027). This difference became insignificant when the HR increment further increased to ≥ 30 bpm. Conclusion: The global MBF value was associated with the extent of increase in HR after stress. Significantly higher global MBF was seen in subjects with HR increment of ≥ 20 bpm.

• Investigative Magnetic Resonance Imaging
• /
• 제18권4호
• /
• pp.314-322
• /
• 2014

배경: 심장의 이완기능 이상은 비대성심근증 환자에서 흔히 발견되는 문제이다. 본 연구는 비대성심근증 환자의 심장 이완기능을 평가하는 데 있어, 승모판과 폐정맥의 유속을 이용한 MRI의 역할을 알아보고자 하였다. 방법과 결과: 승모판과 폐정맥의 유속을 이용한 위상차 MRI와 심초음파검사가 59명 (남:여 = 44:15, 평균 연령 = 51세)의 비대성심근증 환자에서 시행되었다. 49명의 환자가 심초음파검사에서 다양한 정도의 심장 이완기능 이상을 보였다; grade 1 (20명), grade 2 (27명), and grade 3 (2명). MRI로 계산한 승모판 유속인자들 (E, A, E/A ratios)은 심초음파검사로 구한 동일 인자들과 양의 상관관계를 보였다 (Pearson's r values = 0.47, 0.60, 0.75 for E, A, E/A, respectively, all P < 0.001). MRI로 폐정맥 유속정보를 이용하는 경우, pseudo-normalized pattern (8명)이 정상 충만 양상(filling pattern) (17명)과 구분될 수 있었고, 이들을 이용하여 이완기능 정도를 평가를 하는 경우, 심장 MRI가 심초음파검사와 중등도의 일치도를 보였다 (kappa value = 0.45, P < 0.001). 결론: 비대성심근증 환자에서 위상차 MRI를 이용한 심장 이완기능 평가는 임상적으로 적용 가능한 방법이며, 폐정맥 유속 분석을 추가하는 경우, 정상과 pseudo-normal을 구분하는데 유용하다.

• 대한핵의학회지
• /
• 제34권4호
• /
• pp.276-284
• /
• 2000

목적: 허혈성 심근병증 환자는 관동맥 우회로 이식술 위험도가 크므로 예민한 검사로 알려진 FDG PET으로 심근 생존능을 평가하여 수술 대상자를 선별하여야 한다. 이 연구에서는 FDG PET에 나타난 심근 생존능을 바탕으로 수술을 결정하고 관동맥우회로 수술 전 PET검사를 시행하여 수술 환자를 선택하고 수술 후 증상과 심장기능의 호전 여부를 평가하였다. 대상 및 방법: 11명 환자에서 Tl-201 휴식/24시간 지연 SPECT를 시행하고 FDG PET을 시행하여 생존 심근이 확인된 7명에서 관동맥 우회로 이식술을 시행하였다. 수술 후 환자의 흉통, 호흡곤란 증상의 호전과 좌심실 심박출계수의 호전을 조사하였다. 결과: 7명 모두 FDG PET과 Tl-201 SPECT에 생존심근이 확인되었고 6명에서는 Tl-201 휴식/지연 SPECT에서 찾지 못한 생존심근 분절이 FDG PET에서 발견되었다. 수술 전에는 흉통이 CCS 분류 IV 3명, III 2명, II 2명 이었는데 수술 후 6명은 증상이 없는 CCS I 이었고 한명은 CCS II 이었다($IV{\rightarrow}II$). 호흡곤란은 NYHA 분류 IV가 1명, III이 4명, II가 2명이었으며 수술후 6명은 NYHA I이 되었으며 한 명은 NYHA II ($IV{\rightarrow}II$)이었다. 수술 전 평균 좌심실 박출계수는 22%에서 수술 후 32%가 되었다. 결론: 허혈성 심근병증 환자에서 FDG PET으로 생존심근을 평가하여 관동맥 우회로 수술을 시행하였을 때 수술 후에 증상이 호전되고 좌심실 기능이 향상하였다. 허혈성 심근병증에서 FDG PET을 관동맥 우회로 수술로 호전될 환자를 선별하기 위한 생존심근 평가에 사용할 수 있을 것으로 생각한다., p=0.001). Tc-99m MDP 또는 Tc-99m DTPA에 의해 유도된 적응반응은 단백합성억제제인 cycloheximide에 의해 억제되었다($0.24{\pm}0.10\;vs.\;0.43{\pm}0.16$, p=0.001). Tc-99m MDP 신티그라피에 의한 적응반응 지수는 Tc-99m DTPA 신티그라피에 의한 적응반응지수에 비해 유의하게 높았다($0.10{\pm}0.06\;vs.\;0.24{\pm}0.05$, p=0.0001). 결론: Tc-99m MDP 신티그라피 또는 Tc-99m DTPA 신티그라피에 의한 저선량의 방사선 조사에 의하여 말초혈액 림프구에서 방사선 적응반응이 유도되었고, 적응반응의 정도는 Tc-99m MDP 골 신티그라피를 시행하는 환자에서 더욱 현저함을 알 수 있었다.3$으로 의미있는 차이를 보이지 않았다(p=0.43). 결론: 대단위 환자를 대상으로 한 저자들의 연구에서 Tc-99m MIBI 유방스캔은 원발성 유방의 진단에 유용하게 사용되어질 수 있으며 조기영상에서 구한 병변 대 배후방사능의 비는 악성과 양성의 감별에 보조적인 역할을 할 것으로 여겨지나 적절한 문턱값을 구하기 위해서는 추가적인 연구를 필요로 할 것으로 여겨진다. 그러나 1 cm 이하의 크기가 작은 유방암의 진단에 Tc-99m MIBI 유방스캔은 한계가 있으며 액와부 림프절의 전이 진단 역시 어려운 것으로 여겨지며 앞으로의 연구가 수반되어야 한다고 판단된다.의 관류지수와 재관류술 여부가 유의한 예측인자로 나타났다. 결론: 급성 심근경색 초기에 실시한 심근관류 SPECT 검사상 좌심실 확장군과 비확장군은 유의한 휴식기 관류지수의 차이를 보였고 또한 다변량분석에서 휴식기의 관류지수는 좌심실 확장을 예측할 수

• Journal of Chest Surgery
• /
• 제36권7호
• /
• pp.518-522
• /
• 2003

심근경색 후 좌심실 혈전증은 빈번하며 색전의 위험성이 있지만, 허혈성 심근증 환자에서 Dor 술식 후 좌심실 혈전증의 발생이나 치료법은 잘 알려져 있지 않다. 저자들은 45세 남자에서 관상동맥우회술과 Dor 술식 및 좌심실 혈전 제거술 후 좌심실 혈전이 재발하여 정맥내 헤파린 주사 및 경구 쿠마딘을 투여하여 좌심실 혈전의 소실이 있었기에 보고하고자 한다. 본 증례를 통해 저자들은 심첨부 심실벽의 이상운동을 가진 좌심실류가 동반된 좌심실 기능부전 환자들에서 Dor 술식 후 항응고제의 투여가 좌심실 혈전 발생의 예방 및 치료에 도움이 될 수 있을 것으로 사료된다.

• Clinical and Experimental Pediatrics
• /
• 제57권5호
• /
• pp.232-239
• /
• 2014

Purpose: Mitochondrial disease (MD) and Duchenne muscular dystrophy (DMD) are often associated with cardiomyopathy, but the myocardial variability has not been isolated to a specific characteristic. We evaluated the left ventricular (LV) mass by echocardiography to identify the general distribution and functional changes of the myocardium in patients with MD or DMD. Methods: We retrospectively evaluated the echocardiographic data of 90 children with MD and 42 with DMD. Using two-dimensional echocardiography, including time-motion (M) mode and Doppler measurements, we estimated the LV mass, ratio of early to late mitral filling velocities (E/A), ratio of early mitral filling velocity to early diastolic mitral annular velocity (E/Ea), stroke volume, and cardiac output. A "z score" was generated using the lambda-mu-sigma method to standardize the LV mass with respect to body size. Results: The LV mass-for-height z scores were significantly below normal in children with MD ($-1.02{\pm}1.52$, P<0.001) or DMD ($-0.82{\pm}1.61$, P =0.002), as were the LV mass-for-lean body-mass z scores. The body mass index (BMI)-for-age z scores were far below normal and were directly proportional to the LV mass-for-height z scores in both patients with MD (R =0.377, P<0.001) and those with DMD (R =0.330, P=0.033). The LV mass-for-height z score correlated positively with the stroke volume index (R =0.462, P<0.001) and cardiac index (R =0.358, P<0.001). Conclusion: LV myocardial atrophy is present in patients with MD and those with DMD and may be closely associated with low BMI. The insufficient LV mass for body size might indicate deterioration of systolic function in these patients.

• Journal of Genetic Medicine
• /
• 제16권2호
• /
• pp.55-61
• /
• 2019

Purpose: Genetic defects in the nuclear-encoded mitochondrial aminoacyl-tRNA synthetases were first identified as causes of various disorders in 2007. Variants in IARS2, which encodes a mitochondrial isoleucyl-tRNA synthetase, were first reported in 2014. These variants are associated with diverse phenotypes ranging from CAGSSS (CAtaracts, Growth hormone deficiency, Sensory neuropathy, Sensorineural hearing loss, and Skeletal dysplasia) and Leigh syndrome to isolated nonsyndromic cataracts. Here, we describe the phenotypic and genetic spectrum of Korean patients with IARS2-related disorders. Materials and Methods: Using whole-exome sequencing followed by Sanger sequencing, we identified five patients with IARS2 mutations. Their medical records and brain magnetic resonance images were reviewed retrospectively. Results: All five patients presented with developmental delay or regression before 18 months of age. Three patients had bilateral cataracts, but none had hearing loss or sensory neuropathy. No evidence of skeletal dysplasia was noted, but two had short stature. One patient had cardiomyopathy and another exhibited renal tubulopathy and hypoparathyroidism. Their brain imaging findings were consistent with Leigh syndrome. Interestingly, we found the recurrent mutations p.R817H and p.V105Dfs*7 in IARS2. Conclusion: To our knowledge, this is the first report of Korean patients with IARS2-related disorders. Our findings broaden the phenotypic and genotypic spectrum of IARS2-related disorders in Korea and will help to increase clinical awareness of IARS2-related neurodegenerative diseases.

• 대한한방내과학회지
• /
• 제23권2호
• /
• pp.181-190
• /
• 2002

The water extract of Omija-tang(OMJT) has been traditionally used for treatment of ischemic heart and brain damage in oriental medicine. However, little is known about the mechanism by which the water extract of OMJT protects cells from such damage. Therefore, this study was conducted to investigate the protective mechanisms of OMJT on $H_2O_2$-induced toxicity in H9c2 cardiomyoblast cells. Treatment of $H_2O_2$ markedly induced death of H9c2 cardiomyoblast cells in a dose-dependent manner. The characteristics of $H_2O_2$-induced death of H9c2 showed apparent apoptotic features, such as DNA fragmentation. However, OMJT significantly reduced both $H_2O_2$-induced cell death and chromatin fragmentation. The decrease of Bcl-XL expression by $H_2O_2$ was inhibited by OMJT. In addition, the increase of Bcl-XS and Bax expression were also inhibited by OMJT. In particular, Fas expression, which is generally recognized as cell death inducing signal by Fas/FasL interaction, was markedly increased by $H_2O_2$ in a time-dependent manner, whereas this increase was completely prevented by OMJT. The combined treatment of OMJT and $H_2O_2$ in H9c2 cells also reduced activation of caspase-9 and caspase-3 like protease. Taken together, this study indicates that the protective effects of the water extract of OMJT against oxidative damage may be mediated by the modulation of BcI-XL/S and Bax expression by way of the regulation of mitochondrial membrane potential and caspase cascades.

• Biomolecules & Therapeutics
• /
• 제24권4호
• /
• pp.363-370
• /
• 2016

Cardiovascular disease is the most common cause of death in diabetic patients. Hyperglycemia is the primary characteristic of diabetes and is associated with many complications. The role of hyperglycemia in the dysfunction of human cardiac progenitor cells that can regenerate damaged cardiac tissue has been investigated, but the exact mechanism underlying this association is not clear. Thus, we examined whether hyperglycemia could regulate mitochondrial dynamics and lead to cardiac progenitor cell dysfunction, and whether blocking glucose uptake could rescue this dysfunction. High glucose in cardiac progenitor cells results in reduced cell viability and decreased expression of cell cycle-related molecules, including CDK2 and cyclin E. A tube formation assay revealed that hyperglycemia led to a significant decrease in the tube-forming ability of cardiac progenitor cells. Fluorescent labeling of cardiac progenitor cell mitochondria revealed that hyperglycemia alters mitochondrial dynamics and increases expression of fission-related proteins, including Fis1 and Drp1. Moreover, we showed that specific blockage of GLUT1 improved cell viability, tube formation, and regulation of mitochondrial dynamics in cardiac progenitor cells. To our knowledge, this study is the first to demonstrate that high glucose leads to cardiac progenitor cell dysfunction through an increase in mitochondrial fission, and that a GLUT1 blocker can rescue cardiac progenitor cell dysfunction and downregulation of mitochondrial fission. Combined therapy with cardiac progenitor cells and a GLUT1 blocker may provide a novel strategy for cardiac progenitor cell therapy in cardiovascular disease patients with diabetes.

• Journal of Chest Surgery
• /
• 제18권2호
• /
• pp.182-192
• /
• 1985

[here are so many reports that pulsatile blood flow provides physiologic organ perfusions during cardiopulmonary bypass. So, we compared the recent 30 cases undergoing cardiac surgery by Cobe-Stckert pulsatile roller pump with another 30 cases by Polystan nonpulsatile roller pump. Pulsatile flow was applied during aortic-cross clamping period when synchronized to internal EKG simulator, and perfusion mode was changed to continuous nonpulsatile flow after declamping of aorta. Age, sex, weight, and disease entities were comparable and operative techniques were similar between two groups. 1. There were no differences in average ACC time, ECC time, and Operation time. 2. Postoperative artificial respiration time was 6hrs 30mins in nonpulsatile group and 4hrs 48mins in pulsatile group, and detubation time after ventilator weaning was 2hrs 44mins in nonpulsatile group and 1hrs 43mins in pulsatile group. 3. Average pulse pressure was 8mmHg in nonpulsatile group and 55mmHg in pulsatile group, and a mean arterial pressure was 66.0mmHg in nonpulsatile group and 60.7mmHg in pulsatile group. 4. Mean urine-output during ACC;ECC period was 9.717.3;9.913.2ml/kg/hr in nonpulsatile group and 14.215.0;15.817.5 in pulsatile group [p<0, 05], and thereafter progressive decrease of differences in urine output between two groups until POD 2, and lesser amounts of diuretics was needed in pulsatile group during same postoperative period. Serum BUN/Cr level showed no specific difference and urine concentration power was well preserved in both groups. 5. Plasma proteins and other Enzymes showed no differences between two groups, but serum GOT/GPT level was higher in nonpulsatile group till POD 2. 6. Serum Electrolytes showed no differences between two groups. 7. WBC, RBC, Platelet counts, Hgb and Hct were not different and Coagulogram was well preserved in both groups. 8. Plasma free Hgb level was 7.09mg% in pulsatile group compared with 3.48mg% in pulsatile group on POD 1 but was normalized on POD 2. Gross hemoglobinuria after ECC was noted in 6 cases [20%] of pulsatile group and 4 cases [13%] of nonpulsatile group. 9. In both groups, most patients were included in NYHA class III to IV [28 cases;93% in nonpulsatile group, 22 cases;73% in pulsatile group] preoperatively, and well improved to class I to 11[22 cases; 73% in nonpulsatile group, 30 cases; 100% in pulsatile group] postoperatively. There were 7 operative mortalities in nonpulsatile group only, which were 5 cases of TOF with hepatic failure, 1 case of multiple VSDs with low out-put syndrome, and 1 case of mitral valvular heart disease with cardiomyopathy. We concluded that the new, commercially available Cobe-Stckert pulsatile roller pump device was safe, simple, and reliable.

• Clinical and Experimental Pediatrics
• /
• 제56권3호
• /
• pp.125-129
• /
• 2013

Purpose: The use of implantable cardioverter defibrillators (ICDs) to prevent sudden cardiac death is increasing in children and adolescents. This study investigated the use of ICDs in children with congenital heart disease. Methods: This retrospective study was conducted on the clinical characteristics and effectiveness of ICD implantation at the department of pediatrics of a single tertiary center between 2007 and 2011. Results: Fifteen patients underwent ICD implantation. Their mean age at the time of implantation was $14.5{\pm}5.4$ years (range, 2 to 22 years). The follow-up duration was $28.9{\pm}20.4$ months. The cause of ICD implantation was cardiac arrest in 7, sustained ventricular tachycardia in 6, and syncope in 2 patients. The underlying disorders were as follows: ionic channelopathy in 6 patients (long QT type 3 in 4, catecholaminergic polymorphic ventricular tachycardia [CPVT] in 1, and J wave syndrome in 1), cardiomyopathy in 5 patients, and postoperative congenital heart disease in 4 patients. ICD coils were implanted in the pericardial space in 2 children (ages 2 and 6 years). Five patients received appropriate ICD shock therapy, and 2 patients received inappropriate shocks due to supraventricular tachycardia. During follow-up, 2 patients required lead dysfunction-related revision. One patient with CPVT suffered from an ICD storm that was resolved using sympathetic denervation surgery. Conclusion: The overall ICD outcome was acceptable in most pediatric patients. Early diagnosis and timely ICD implantation are recommended for preventing sudden death in high-risk children and patients with congenital heart disease.