• 제목/요약/키워드: Rare

검색결과 11,854건 처리시간 0.15초

Blood-Blister Aneurysms of the Internal Carotid Artery in Tibetan and Han Populations : A Retrospective Observational Study

  • Bowen Huang;Yanming Ren;Hao Liu;Anqi Xiao;Lunxin Liu;Hong Sun;Yi Liu;Hao Li;Lu Ma;Chang-Wei Zhang;Chao-Hua Wang;Min He;Yuekang Zhang;Chao You;Jin Li
    • Journal of Korean Neurosurgical Society
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    • 제67권3호
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    • pp.345-353
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    • 2024
  • Objective : Blood-blister aneurysms (BBAs) of the internal carotid artery (ICA) are challenging lesions with high morbidity and mortality rates. Although research on BBAs is well documented in different populations, the study of BBAs in the Tibetan population is extremely rare. This study aimed to evaluate the characteristics of BBAs and analyze the treatment modalities and long-term outcomes in the Tibetan population in comparison with the Han population. Methods : The characteristics of patients with BBAs of the ICA from January 2009 to January 2021 at our institution were reviewed. The features of aneurysms, treatment modalities, complications, and follow-up outcomes were retrospectively analyzed. Results : A total of 130 patients (41 Tibetan and 89 Han patients) with BBAs of the ICA who underwent treatment were enrolled. Compared with the Han group, the Tibetan group significantly demonstrated a high ratio of BBAs among ICAs (8.6%, 41/477 vs. 1.6%, 89/5563; p<0.05), a high ratio of vasospasm (34.1%, 14/41 vs. 6.7%, 6/89; p=0.001), a high risk of ischemic events (43.9%, 18/41 vs. 22.5%, 20/89; p<0.05), and a low ratio of good outcomes (modified Rankin scale, 0-2) at the 1-year follow-up (51.2%, 21/41 vs. 74.2%, 66/89; p<0.05). The multivariate regression model showed that ischemic events significantly contributed to the prediction of outcomes at 1 year. Further analysis revealed that microsurgery and vasospasm were associated with ischemic events. Conclusion : In comparison with Han patients, the Tibetan population had a high ratio of BBA occurrence, a high incidence of ischemic events, and a high ratio of poor outcomes. The endovascular approach showed more benefits in BBA patients.

Differentiation between Clear Cell Sarcoma of the Kidney and Wilms' Tumor with CT

  • Choeum Kang;Hyun Joo Shin;Haesung Yoon;Jung Woo Han;Chuhl Joo Lyu;Mi-Jung Lee
    • Korean Journal of Radiology
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    • 제22권7호
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    • pp.1185-1193
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    • 2021
  • Objective: Clear cell sarcoma of the kidney (CCSK) is the second-most common but extremely rare primary renal malignancy in children after Wilms' tumor. The aims of this study were to evaluate the imaging features that could distinguish between CCSK and Wilms' tumor and to assess the features with diagnostic value for identifying CCSK. Materials and Methods: We reviewed the initial contrast-enhanced abdominal-pelvic CT scans of children with CCSK and Wilms' tumor between 2010 to 2019. Fifty-eight children (32 males and 26 females; age, 0.3-10 years), 7 with CCSK, and 51 with Wilms' tumor, were included. The maximum tumor diameter, presence of engorged perinephric vessels, maximum density of the tumor (Tmax) of the enhancing solid portion, paraspinal muscle, contralateral renal vein density, and density ratios (Tmax/muscle and Tmax/vein) were analyzed on the renal parenchymal phase of contrast-enhanced CT. Fisher's exact tests and Mann-Whitney U tests were conducted to analyze the categorical and continuous variables, respectively. Logistic regression and receiver operating characteristic curve analyses were also performed. Results: The age, sex, and tumor diameter did not differ between the two groups. Engorged perinephric vessels were more common in patients in the CCSK group (71% [5/7] vs. 16% [8/51], p = 0.005). Tmax (median, 148.0 vs. 111.0 Hounsfield unit, p = 0.004), Tmax/muscle (median, 2.64 vs. 1.67, p = 0.002), and Tmax/vein (median, 0.94 vs. 0.59, p = 0.002) were higher in the CCSK compared to the Wilms' group. Multiple logistic regression revealed that engorged vessels (odds ratio 13.615; 95% confidence interval [CI], 1.770-104.730) and Tmax/muscle (odds ratio 5.881; 95% CI, 1.337-25.871) were significant predictors of CCSK. The cutoff values of Tmax/muscle (86% sensitivity, 77% specificity) and Tmax/vein (71% sensitivity, 86% specificity) for the diagnosis of CCSK were 1.97 and 0.76, respectively. Conclusion: Perinephric vessel engorgement and greater tumor enhancement (Tmax/muscle > 1.97 or Tmax/vein > 0.76) are helpful for differentiating between CCSK and Wilms' tumor in children aged below 10 years.

Comparison of Radiological Tumor Response Based on iRECIST and RECIST 1.1 in Metastatic Clear-Cell Renal Cell Carcinoma Patients Treated with Programmed Cell Death-1 Inhibitor Therapy

  • Bingjie Zheng;Ji Hoon Shin;Hailiang Li;Yanqiong Chen;Yuan Guo;Meiyun Wang
    • Korean Journal of Radiology
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    • 제22권3호
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    • pp.366-375
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    • 2021
  • Objective: To evaluate the radiological tumor response patterns and compare the response assessments based on immune-based therapeutics Response Evaluation Criteria in Solid Tumors (iRECIST) and RECIST 1.1 in metastatic clear-cell renal cell carcinoma (mccRCC) patients treated with programmed cell death-1 (PD-1) inhibitors. Materials and Methods: All mccRCC patients treated with PD-1 inhibitors at Henan Cancer Hospital, China, between January 2018 and April 2019, were retrospectively studied. A total of 30 mccRCC patients (20 males and 10 females; mean age, 55.6 years; age range, 37-79 years) were analyzed. The target lesions were quantified on consecutive CT scans during therapy using iRECIST and RECIST 1.1. The tumor growth rate was calculated before and after therapy initiation. The response patterns were analyzed, and the differences in tumor response assessments of the two criteria were compared. The intra- and inter-observer variabilities of iRECIST and RECIST 1.1 were also analyzed. Results: The objective response rate throughout therapy was 50% (95% confidence interval [CI]: 32.1-67.9) based on iRECIST and 30% (95% CI: 13.6-46.4) based on RECIST 1.1. The time-to-progression (TTP) based on iRECIST was longer than that based on RECIST 1.1 (median TTP: not reached vs. 170 days, p = 0.04). iRECIST and RECIST 1.1 were discordant in 8 cases, which were evaluated as immune-unconfirmed PD based on iRECIST and PD based on RECIST 1.1. Six patients (20%, 6/30) had pseudoprogression based on iRECIST, of which four demonstrated early pseudoprogression and two had delayed pseudoprogression. Significant differences in the tumor response assessments based on the two criteria were observed (p < 0.001). No patients demonstrated hyperprogression during the study period. Conclusion: Our study confirmed that the iRECIST criteria are more capable of capturing immune-related atypical responses during immunotherapy, whereas conventional RECIST 1.1 may underestimate the benefit of PD-1 inhibitors. Pseudoprogression is not rare in mccRCC patients during PD-1 inhibitor therapy, and it may last for more than the recommended maximum of 8 weeks, indicating a limitation of the current strategy for immune response monitoring.

고등학교 지구과학 탐구활동에서 소그룹의 상호작용 양식에 따른 학생들의 반성적 탐구의 특성 (Students' Characteristics of the Reflective Inquiry Dispositions According to the Modes of Interaction of Small Group in High School Earth Science Inquiry Class)

  • 박미라;정진우;정철
    • 한국과학교육학회지
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    • 제26권7호
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    • pp.843-855
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    • 2006
  • 본 연구는 지구과학 탐구활동에서 그룹의 상호작용 양식에 따라 학생들의 반성적 탐구는 어떤 특징을 보이는지를 알아보고, 수업의 3가지 맥락들은 반성적 탐구활동에서 어떻게 관련되는지를 알아봄으로써 학생들의 반성적 탐구의 실제를 이해하고 반성적 탐구를 촉진하기 위한 탐구활동 수업의 시사점을 얻고자 하였다. 이를 위해 고등학교 1학년 1개 학급을 선정해서 본 연구에서 개발한 반성적 탐구활동수업을 실시하고 수업을 녹화 전사해서 언어행동분석틀과 수업상황의 3가지 맥락으로 분석하였다. 사전 검사를 통해 알아낸 학습자 특성을 기초로 그룹을 나누어 수업을 실시하였으며 면담을 실시하여 수업분석에 참고하였다. 반성적 탐구활동수업에서 그룹 상호작용양식에 따른 반성적 탐구의 차이는 조화적 상호작용양식을 보이는 그룹에서는 전개와 정리단계에서 자료와 과제맥락이 연결된 반성적 탐구가 자주 나타난 반면, 대립적 상호작용양식 그룹에서는 자료맥락과 연결된 반성적 탐구가 아주 드물고 반성적 탐구척도 값도 2 배 이상 낮게 나타났다. 즉 조화적 그룹이 양적으로 더 많은 반성적 탐구진술을 했으며 자료맥락과 과제맥락을 연결하는 더 의미 있는 반성적 탐구를 했다는 것을 알 수 있었다. 탐구능력과 인지양식에 있어서 이질그룹은 정의적 측면에서 대립적 상호작용양식을 보여 반성적 탐구에 가장 부정적인 영향을 끼쳤고, 인지양식과 탐구능력 모두에서 동질그룹은 조화적 상호작용양식을 보여 반성적 탐구에 긍정적인 영향을 주었다는 것을 알 수 있었다. 이러한 결과로부터 반성적 탐구활동을 촉진시키기 위한 그룹조직의 시사점을 얻을 수 있었다.

동해 울릉분지 퇴적물에서 망간산화물과 철산화물 환원율 추정 (Estimate of Manganese and Iron Oxide Reduction Rates in Slope and Basin Sediments of Ulleung Basin, East Sea)

  • 최유정;김동선;이태희;이창복
    • 한국해양학회지:바다
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    • 제14권3호
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    • pp.127-133
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    • 2009
  • 동해 울릉분지에서 망간산화물과 철산화물의 유기물 분해 기여율을 정량화하기 위하여 6개의 박스형 시추퇴적물을 채집하였다. 수심이 2,000 m 이상인 울릉분지 표층퇴적물에서 유기탄소 함량은 2.6% 가량으로 육상기원 유기물 유입이 많은 흑해와 용승 해역인 칠레 주변해역들과 비슷했다. 울릉분지에 위치한 정점들에서 망간산화물 함량은 퇴적물 최상부에서 2% 이상으로 매우 높았고, 철산화물 함량은 울릉분지 표층 $1{\sim}4cm$ 내외에서 2% 가량의 최고값을 나타냈다. 그러나 대륙사면 정점들에서 망간산화물과 철산화물 함량은 분지처럼 높은 값을 보이지 않았다. 동해 울릉분지에서 2% 이상으로 높은 망간산화물 함량은 높은 유기탄소 함량과 사면보다 비교적 느린 퇴적속도 때문이거나 망간 이온이 저탁류를 통해 사면에서 분지로 이동한 것으로 예상된다. 망간산화물 환원율은 $0.3{\sim}0.57\;mmol\;m^{-2}\;day^{-1}$의 범위를 보였고, 철산화물 환원율은 $0.10{\sim}0.24\;mmol\;m^{-2}\;day^{-1}$의 범위를 나타냈다. 울릉분지에서 금속산화물은 유기물 분해에 $13{\sim}26%$ 정도를 차지하였으며, 이는 칠레 용승 해역과 유사하고, 덴마크 연안보다 낮은 값이다.

열대 해역 지표종 Ornithocercus 속 와편모조의 출현 동향으로 본 한반도 온난화 (The Korean Peninsula Warming Based on Appearance Trend of Tropical Dinoflagellate Species, Genus Ornithocercus)

  • 김형신;정민민;이준백
    • 한국해양학회지:바다
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    • 제13권4호
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    • pp.303-307
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    • 2008
  • 제주도 서부해역인 차귀도 연안 해역에서 2006년 10월부터 2007년 11월 사이 5회에 걸쳐 식물 플랑크톤의 일종인 와편모조의 종 조성 변화를 조사하였으며 총 32종의 와편모조가 관찰되었다. 이들 와편모조 중 19종 즉, Amphisolenia bidentata, Ceratium gravidum, C. hexacanthum, C. platycorne, C. praelongum, C. ranipes, C. reflexum, C. geniculatum, Dissodinium elegans, D. bicorne, Ornithocercus heteroporus, O. magnificus, O. quadratus, O. splendidus, O. steinii, Protoperidinium excentricum, Pseliodinium vaubanii, Ptychodiscus noctiluca, Pyrocystis hamulus 등의 종은 한국 연안 해역에서는 매우 드물게 출현이 보고된 종이거나 미기록 종이었다. 그리고 채집된 시료에서 열대 해역 지표종인 Ornithocercus 속의 월별 단위체적당 출현량을 분석한 결과 2006년 10월, 2007년 2, 4, 9 및 11월에 각각 28, 22, 0, 3 및 $502\;cells/m^3$이었다. 또한 월별 총 와편모조류 출현량에 대한 Ornithocercus 속의 점유율은 각각 8, 3.9, 0, 0.2 및 97.9%로 2007년 11월에 최고치를 나타내었다. 이상의 연구 결과는 심각하게 진행되고 있는 지구 온난화에 발맞추어 한반도 연안 해역 역시 온난화 되어지고 있음을 부분적으로 나타내고 있다고 판단된다.

중심성 교뇌수초용해 환자의 치료 경과에 따른 보행 기능의 정량적 평가 (Case Report: Quantitative Evaluation of Gait Function Following Treatment Progression in a Patient with Central Pontine Myelinolysis)

  • 지상호;강선이;김지우;소영조;이상관;김철현
    • 대한한방내과학회지
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    • 제45권2호
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    • pp.190-198
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    • 2024
  • Introduction: Central pontine myelinolysis (CPM) is a rare neurological disorder marked by symmetric nerve fiber damage, commonly following rapid hyponatremia correction, with no established treatment and a poor prognosis. The condition, often linked to alcoholism, malnutrition, and various diseases, lacks comprehensive studies on its impact on gait. This research aims to quantitatively analyze gait changes in CPM patients receiving both traditional Korean and conventional rehabilitation treatments, addressing a gap in current understanding of CPM management. Case presentation: A 56-year-old male diagnosed with CPM following an initial misdiagnosis underwent combined electoracupuncture and rehabilitative treatment at ○○ university Korean medical center, resulting in significant gait improvements. A treadmill gait analysis system was used to measure changes in key gait parameters at 2-week intervals, and the patient's progress was documented. Conclusions: The quantitative analysis revealed significant gait improvements. Foot rotation decreased from 8.9° to 6.4° (right) and from 11.1° to 7.2° (left); lateral symmetry improved from -7.8 to 0.8; step length increased from 21 cm to 44 cm (right) and from 19 cm to 44 cm (left); and velocity increased from 1.2 m/s to 2.7 m/s. These findings highlight decreased foot rotation and lateral symmetry, along with increased step length and velocity, suggesting a positive outcome of the treatment regimen. Notably, the patient experienced no adverse effects related to the treatments. Despite limitations, including the singe case focus and lack of prior gait-focused CPM research, this case report provides valuable insights into effective CPM management strategies, paving the way for future research in this domain.

개인의 업무 네트워크 특성에 따른 동시적 CMC의 영향 : 매체 동시성 이론 관점 (The Effect of Synchronous CMC Technology by Task Network: A Perspective of Media Synchronicity Theory)

  • 김민수;박철우;양희동
    • Asia pacific journal of information systems
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    • 제18권3호
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    • pp.21-43
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    • 2008
  • The task network which is formed of different individuals can be recognized as a social network. Therefore, the way to communicate with people inside or outside the network has considerable influence on their outcome. Moreover, the position on which a member stands in a network shows the different effects of the information systems supporting communication with others. In this paper, it is to be studied how personal CMC (computer-mediated communication) tools affect the mission that those who work for a network perform through diverse task networks. Especially, we focused on synchronicity of CMC. On this score, the perspective of Media Synchronicity Theory was taken that had been suggested by criticizing Media Richness Theory. It is the objective, from this perspective, to find which characteristics of networks make the value of IT supporting synchronicity high. In the research trends of social networks, there have been two traditional perspectives to explain the effect of network: embeddedness and diversity ones. These differ from the aspect which type of social network can provide much more economic benefits. As similar studies have been reported by various researchers, these are also divided into the bonding and bridging views which are based on internal and external tie, respectively, Size, density, and centrality were measured as the characteristics of personal task networks. Size means the level of relationship between members. It is the total number of other colleagues who work with a specific member for a certain project. It means, the larger the size of task network, the more the number of coworkers who interact each other through the job. Density is the ratio of the number of relationships arranged actually to the total number of available ones. In an ego-centered network, it is defined as the ratio of the number of relationship made really to the total number of possible ones between members who are actually involved each other. The higher the level of density, the larger the number of projects on which the members collaborate. Centrality means that his/her position is on the exact center of whole network. There are several methods to measure it. In this research, betweenness centrality was adopted among them. It is measured by the position on which one member stands between others in a network. The determinant to raise its level is the shortest geodesic that represents the shortest distance between members. Centrality also indicates the level of role as a broker among others. To verify the hypotheses, we interviewed and surveyed a group of employees of a nationwide financial organization in which a groupware system is used. They were questioned about two CMC applications: MSN with a higher level of synchronicity and email with a lower one. As a result, the larger the size of his/her own task network, the smaller its density and the higher the level of his/her centrality, the higher the level of the effect using the task network with CMC tools. Above all, this positive effect is verified to be much more produced while using CMC applications with higher-level synchronicity. Among the a variety of situations under which the use of CMC gives more benefits, this research is considered as one of rare cases regarding the characteristics of task network as moderators by focusing ITs for the operation of his/her own task network. It is another contribution of this research to prove empirically that the values of information system depend on the social, or comparative, characteristic of time. Though the same amount of time is shared, the social characteristics of users change its value. In addition, it is significant to examine empirically that the ITs with higher-level synchronicity have the positive effect on productivity. Many businesses are worried about the negative effect of synchronous ITs, for their employees are likely to use them for personal social activities. However. this research can help to dismiss the concern against CMC tools.

다형성 암종의 임상적 고찰 (Clinical Characteristics of Pleomorphic Carcinoma)

  • 이덕헌;박훈;박창권;권건영;박남희;최세영;이광숙;유영선
    • Journal of Chest Surgery
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    • 제36권6호
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    • pp.397-403
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    • 2003
  • 폐의 다형성 암종은 일반적으로 드문 폐암종의 아형으로 알려져 있다. 1999년 WHO 폐암분류의 기준을 적용하여 계명대학교 동산의료원에서 진단된 42예의 다형성 암종에 대한 임상적 특징에 대해 알아보고자 하였다. 대상 및 방법: 1992년 1월부터 2001년 12월까지 본원에서 폐암으로 폐 절제술을 시행한 256예 중, 광학현미경적 재검색과 면역조직화학염색을 실시하여 다형성 암종으로 진단한 42예를 대상으로, 연구를 시행하였다. 결과: 42예 중 남자는 31예, 여자는 11예였고, 나이는 26세부터 77세까지였다. 임상증상은 기침, 객혈, 객담이 주로 나타났으며, 진단 시 병기는 Ia 3예, (7%), Ib 16예(38%), IIa 1예 (2%), IIb 8예(19%), IIIa 15예(35%), IIIb 1예(2%)였고, 이 중 림프절 전이가 없는 N0 경우는 23예(54%), 림프절 전이가 있는 N1과 N2인 경우는 19예(46%)로 나타났다. 원격전이가 발생한 환자는 총 19예로 뇌 5예(26%),뼈 4예(21%),근육 4예(21%), 림프절 2예(10%), 간, 난소, 편측 폐, 부신이 각 1예씩 발생하였다. 종양의 크기는 1 cm부터 11 cm까지 분포하였고 평균 5.85 cm이었다. 42예의 환자에서 조사 기간 중 생존자는 11예(26%), 사망자는 31예(74%)이었다. Kaplan-Meier method에 의한 이들의 전체 2년, 5년 생존율은 각각 26%와 13%로 조사대상 256예에서 다형성암종을 제외한 증례의 2년생존율 44%와 5년 생존율 34%에 비해서 유의하게 낮은 것으로 조사되었다(p<0.002). 나이와 종양의 크기, 병기 I과 병기 II 이상, N0 N1 이상의 생존율 비교에서는 유의한 차이는 없었다. 술 후 원격전이가 발생한 증례는 모두 사망하였고 이들은 원격전이가 없는 증례에 비하여 생존율이 유의하게 낮게 나타났다(p<0.002). 걸론: 1999년 WHO 폐암분류에 따른 새로운 진단 기준을 적용할 경우 다형성 암종은 이전의 WHO 폐암분류에서보다 높은 발생률을 보이며, 술 후 이들의 생존율은 다른 비소세포암종의 유형보다 유의하게 낮은 것을 확인할 수 있었다.

대동맥판륜 확장증, 심실중격결손 및 대동맥판막 폐쇄부전과 동반된 우심실 저형성 환자에서 대동맥근부 치환술 -1예 보고 (Aortic Root Replacement in Patient of Right Ventricular Hypoplasia with Annuloaortic Ectasia, Ventricular Septal Defect and Aortic Regurgitation - Report of 1 case -)

  • 백만종;나찬영;오삼세;김웅한;황성욱;이철;장윤희;조원민;김재현;서홍주;강상수;문현수;박영관;김종환
    • Journal of Chest Surgery
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    • 제36권7호
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    • pp.510-513
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    • 2003
  • 우심실 저형성 환자에서 심실중격결손과 대동맥판막 폐쇄부전 및 대동맥판륜 확장증이 동반된 경우는 매우 드물다. 저자들은 우심실 저형성 환자에서 심실중격결손, 대동맥판막 폐쇄부전 및 대동맥판륜 확장증이 동반되어 composite graft를 이용한 근부치환술을 시행한 증례를 보고한다. 환자는 19세 남자로 심장초음파 및 심도자 검사에서 직경 2 cm크기의 막성 심실중격결손과 심한 대동맥판막 폐쇄부전증, 그리고 대동맥판륜 확장증이 동반되어 있었으며 우심실은 bipartite 형태로 심한 저형성을 보였고 Qp/Qs는 1.2였다. 수술소견 상 우관상 및 비관상 판첨 변연부 길이의 현저한 확장, 판첨의 비후와 수축, 그리고 교련부의 융합 및 석회화가 있었다. 수술은 대동맥근부를 통해 자가 심낭 패취를 이용한 심실중격결손 폐쇄술 및 composite graft를 이용한 button Bentall 술식과 우심실 유출로 부위의 비후된 근육을 절제하여 주었다. 환자는 수술 직후 일시적인 심폐기 이탈의 어려움이 있었으며 술 후 14일째 특별한 합병증 없이 퇴원하였다.