• Journal of Chest Surgery
• /
• 제36권7호
• /
• pp.463-471
• /
• 2003

대동맥판막 치환술과 동반시행한 승모판막 성형술의 장기 결과에 대해서는 잘 알려져 있지 않다. 본 연구는 대동맥판막 및 승모판막 질환에서의 대동맥판막 치환술과 동반시행한 승모판막 성형술의 조기 및 장기 수술 결과에 대해 알아보고자 하였다. 대상 및 방법: 1990년 9월부터 2002년 4월까지 세종병원에서 대동맥판막 치환술과 승모판막 성형술을 받은 45명의 환자를 대상으로 후향적으로 조사하였다. 남녀비는 28 : 17명이었고 평균 연령은 47$\pm$17세였다. 심방세동은 12명에서 있었고 3명은 과거에 심장수술을 받았다. 승모판막 질환은 폐쇄부전 34명, 협착 3명, 폐쇄부전 및 협착 혼합형이 8명이었다. 승모판막 질환의 원인으로는 류머치스성 24명, 퇴행성 11명, 기능성 8명, 허혈성과 심내막염이 각각 1명이었으며 수술 소견상 판륜확장 31명, 건삭확장 19명, 판첨비후 19명, 교련응합 13명, 건삭융합 10명, 건삭판열 6명 등이었다. 수술은 모든 환자에서 대동맥판막(기계판막 32명, 조직판막 12명, 폐동맥자가판막 1명)을 치환하였고 승모판막 성형술은 판륜성형술이 32명에서, 판첨성형술은 29명에서 54건이 시행되었다. 총체외순환시간 및 대동맥차단시간은 각각 204$\pm$62분, 153$\pm$57분이었다. 결과: 조기사망은 1명(2.2%)으로 술 후 저심박증후군으로 사망하였다. 57$\pm$37개월을 추적조사한 결과 1명(2.3%)이 암으로 사망하였으며 10년 actuarial survival 96$\pm$4%였다. 승모판 폐쇄부전은 11명에서 II혹은 III를 보였고, 중등도의 승모판 협착은 3명에서 있었으며 판막 관련 재수술은 2명에서 승모판막 질환으로 1명에서 대동맥판막 질환으로 필요하였다. 승모판막 폐쇄부전과 협착으로부터의 자유도는 각각 64$\pm$11%와 86$\pm$8%였으며 재수술로부터의 자유도는 89$\pm$7%였다. 결론: 대동맥판막 치환술과 동반시행한 승모판막 성형술은 양호한 조기 및 장기 생존율을 보이며 향후 승모판막 폐쇄부전 및 협착 재발률을 더 낮추기 위해서는 특히 류머치스성 승모판막 질환 경우에는 성형술에 대한 적절한 술기 및 적응증의 선택이 중요하리라 생각된다.

• Journal of Chest Surgery
• /
• 제30권1호
• /
• pp.92-96
• /
• 1997

구강 생식기의 궤양, 안구와 피부 병변을 특징으로 하는 베체씨 병의 예후는 중추 신경계, 위장관, 혈 관계의 합병증에 의존된다. 심장의 병변 특히 대동맥 판막 폐쇄부전증은 매우 드물고, 일반적으로 혈역 학적 불안정성은 개심술을 통하여 치료된다. 그러나 많은 환자에서 인공 판막의 이탈, 판막 주변 누출. 전도 장애, 가성 동맥류 등의 심각한 합병증이 보고되어 왔다. 이런 합병증을 막기 위하여 많은 노력이 있었으며 그중 하나는 동종 이식편읜 사용이었다. 서울대학교병원 흉부외과에서는 베체씨 대동맥염으로 인해 인조 판막 이탈이 있었던 39세 남자 환자에서 동종 이식편을 이용한 대동맥 근위부 치환술을 시도하여 좋은 결과를 얻었기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
• /
• 제50권4호
• /
• pp.235-241
• /
• 2017

Background: The Ross/Ross-Konno procedure is considered a good option for irreparable aortic valve disease in pediatric patients because of its hemodynamic performance and potential for growth of the pulmonary autograft. This study is a review of the long-term results of our 20-year experience with the Ross and Ross-Konno operations in a single institution. Methods: Between June 1995 and January 2016, 16 consecutive patients (mean age, $6.0{\pm}5.9years$; range, 16 days to 17.4 years) underwent either a Ross operation (n=9) or a Ross-Konno operation (n=7). The study included 12 males and 4 females, with a median follow-up period of 47 months (range, 6 to 256 months). Results: There were no cases of in-hospital or late mortality. Six reoperations were performed in 5 patients. Four patients underwent right ventricular-pulmonary artery (RV-PA) conduit replacement. Two patients underwent concomitant replacement of the pulmonary autograft and RV-PA conduit 10 years and 8 years after the Ross operation, respectively. The rate of freedom from adverse outcomes of the pulmonary autograft was 88% and 70% at 5 and 10 years, respectively. The rate of freedom from valve-related reoperations was 79% and 63% at 5 and 10 years, respectively. Conclusion: Pulmonary autografts demonstrated good durability with low mortality. The Ross/Ross-Konno procedure is a good option that can be performed safely in pediatric patients with aortic valve disease, even in a small-volume center.

• Journal of Chest Surgery
• /
• 제32권6호
• /
• pp.588-590
• /
• 1999

오늘날 말기 신부전 환자에서의 심장수술은 보편화 되는 추세이며, 만성 신부전 환자들의 수가 늘어남에 따라 이들 환자에 대한 적절한 치료법 개발이 필요하게 되었다. 이 환자들에게는 관상동맥질환 뿐만 아니라 판막질환도 드물지 않다. 이들에게 시행되는 복막투석은 체외순환에 다소의 지장은 있을수 있지만 수술 전후에 적절한 대책을 준비한다면 더 이상 심장수술의 걸림돌이 되지 않는다. 저자는 심한 승모판막 폐쇄부전과 만성신부전을 동반한 33세의 무뇨증 여자환자에서 기계판막치환술을 시행하였다. 환자는 수술전에 복막투석을 주기적으로 함으로써 수분 및 전해질 균형을 적절히 유지시켰고, 수술후에도 혈액 생화학적 검사결과를 수시로 예의검토하면서 복막투석을 계속시행함으로써 무사히 회복될 수 있었다.

• Journal of Genetic Medicine
• /
• 제11권2호
• /
• pp.74-78
• /
• 2014

Sotos syndrome (SS, OMIM 117550) is characterized by prenatal and postnatal overgrowth with multiple congenital anomalies. However, there have been few cases of growth retardation caused by renal failure from infancy. We report a case of dysplasia of the bilateral kidneys with renal failure and poor postnatal growth. A 2-month-old boy visited the emergency room owing to poor oral intake and abdominal distension. He was born at the gestational age of 38 weeks with a birth weight of 4,180 g. After birth, he had feeding difficulty and abdominal distension. Upon physical examination, his height and weight were in less than the 3rd percentile, while his head circumference was in the 50th percentile on the growth curve. He also showed a broad and protruding forehead and high hairline. Blood laboratory tests showed severe azotemia; emergent hemodialysis was needed. Abdominal ultrasonography revealed bilateral renal dysplasia with multiple cysts and diffuse bladder wall thickening. A posterior urethral valve was suggested based on vesicoureterography and abdominal magnetic resonance findings. Results of a colon study to rule out congenital megacolon did not reveal any specific findings. The conventional karyotype of the patient was 46, XY. Array comparative genomic hybridization study revealed a chromosome 5q35 microdeletion including the NSD1 gene, based on which SS was diagnosed. We describe a case of SS presenting with end stage renal disease due to posterior urethral valve. The typical somatic overgrowth of SS in the postnatal period was not observed due to chronic renal failure that started in the neonatal period.

• Journal of Chest Surgery
• /
• 제56권4호
• /
• pp.255-261
• /
• 2023

Background: The surgical threshold for bicuspid aortic valve (BAV)-related aortopathy is a matter of debate due to its uncertain etiology and prognosis. This study investigated the prognosis of unrepaired BAV aortopathy in patients undergoing surgical aortic valve replacement (SAVR). Methods: We retrospectively analyzed data from 720 patients (age, 60.8±11.5 years; 246 women) who underwent SAVR for BAV disease without aortic repair between 2005 and 2020 at Asan Medical Center. The clinical endpoints were defined as occurrences of sudden death, aortic dissection or rupture, and elective aortic repair. To estimate postoperative changes in the dimensions of the unrepaired aorta, the individual annual aortic expansion rate was calculated. Multiple linear regression models were used to evaluate the risk of aortic expansion. Results: The mean ascending aortic diameter was 39.5±4.6 mm, and 299 patients (41.5%) had a baseline ascending aorta diameter >40 mm. During 70.0±68.3 months of follow-up, the mean annual aortic expansion rate was 0.39±1.96 mm/yr, no aortic dissection or rupture was observed, and sudden deaths were reported in 12 patients (0.34% per person-year). Linear regression analysis revealed no significant correlation between the baseline ascending aortic diameter and postoperative aortic expansion (R²=0.004, β=-0.84, p=0.082). Conclusion: In selected patients undergoing SAVR for a BAV (<55 mm), the risk of adverse aortic events was very low. As this observation contradicts current practice guidelines advocating for proactive aortic replacement in dilated ascending aortas measuring >45 mm, the study results need further validation by studies involving larger populations or randomized controlled trials.

• Korean Circulation Journal
• /
• 제52권8호
• /
• pp.623-631
• /
• 2022

Background and Objectives: Pulmonary valve replacement (PVR) is the most common operation in adults with congenital heart disease (CHD). There is controversy regarding the best bioprosthesis. We compare the performance of stented bioprosthetic valves (the Mosaic [Medtronic^TM] porcine pericardial against Carpentier Perimount Magna Ease [Edwards^TM] bovine) in pulmonary position in patients with CHD. Methods: Between January 1999 and December 2019, all the PVRs were identified from hospital databases in 2 congenital heart centres in Spain. Valve performance was evaluated using clinical and echocardiographic criteria. Propensity score matching was used to balance the 2 treatment groups. Results: Three hundred nineteen patients were retrospectively identified. After statistical adjustment, 79 propensity-matched pairs were available for comparison Freedom from reintervention for the porcine cohort was 98.3%, 96.1%, and 91.9% at 3, 5, and 10 years and 100%, 98%, and 90.8% for the bovine cohort (p=0.88). Freedom from structural valve degeneration (SVD) for the porcine cohort was 96.9%, 92.8% and 88.7% at 3, 5, and 10 years and 100%, 98%, and 79.1% for the bovine cohort (p=0.38). Bovine prosthesis was associated with a reintervention hazard ratio (HR), 1.12; 95% confidence intervals (CIs), 0.24-5.26; p=0.89 and SVD HR, 1.69 (0.52-5.58); p=0.38. In the first 5 years, there was no difference in outcomes. After 5 years, the recipients of the bovine bioprosthesis were at higher risk for SVD (reintervention HR, 2.08 [0.27-16.0]; p=0.49; SVD HR, 6.99 [1.23-39.8]; p=0.03). Conclusions: Both bioprosthesis have similar outcomes up to 5 years, afterwards, porcine bioprosthesis seem to have less SVD.

• Journal of Chest Surgery
• /
• 제53권6호
• /
• pp.411-413
• /
• 2020

A 34-year-old man who had undergone aortic valve replacement 8 years ago underwent an additional Bentall operation due to mechanical valve dehiscence 2 years later. Subsequently, he was diagnosed with Behçet disease and Batter syndrome. A week after being hospitalized again due to chest pain and dyspnea, a large pseudo-aneurysm was detected on computed tomography. Because of the excessively large size of the pseudo-aneurysm, surgical treatment seemed very risky. Therefore, we planned to perform thoracic endovascular aortic repair (TEVAR) and treated him successfully. However, the patient experienced recurrence of the same symptoms 4 months later, and was found to have type IV endoleak. He received a TEVAR procedure again, and it was successful.

• Journal of Chest Surgery
• /
• 제32권3호
• /
• pp.255-261
• /
• 1999

배경: 심방세동은 모든 부정맥중에서 가장 흔하고 승모판질환을 가진 환자의 79%를 차지한다. 이 연구에서는 승모판 수술을 시행하는 환자의 심방세동이 냉동절제 없이 시행한 Maze 술식으로 제거되는지를 관찰하였다. 대상 및 방법: 1997년 5월에서 1998년 4월까지 승모판 질환과 심방세동이 있는 14명의 환자에서 냉동절제 없이 Maze III 술식을 시행하였다. 남자는 6례, 여자는 8례였고, 평균 나이는 46.2$\pm$10.7세 였다. 승모판막 협착증 11례, 그리고 승모판막 폐쇄부전 3례였다. 동반 질환으로 대동맥판막 질환 4례, 삼첨판막 폐쇄부전이 1례, 그리고 심방중격결손증이 2례였다. 경흉적 심초음파 검사상 좌심방의 평균 크기는 54.7$\pm$5.3 mm였고, 좌심방내에서 혈전이 관찰된 경우는 2례였다. 수술후 심초음파 검사상 승모판막을 통과하는 혈류를 측정함으로써 초기 충전파의 최고 속도와 심방수축파의 최고 속도 사이의 비(A/E ratio)를 계산하였다. 수술은 대동맥판막 치환술 4례, DeVega 판륜성형술 1례, 그리고 심방중격결손 폐쇄술 2례를 동반하여 승모판막 치환술을 13례에서 시행하였고, 나머지 1례는 Maze III 술식만 시행하였다. 결과: 5례(38%)의 경우에서 일시적인 심방세동이 재발하였고, 수술후 평균 38.8$\pm$23.5일에 flecainide 또는 amiodarone의 투여로 정상동방결절율동으로 전환되었다. 수술후 합병증은 일시적인 접합부율동 6례, 일시적인 심방세동 5례, 출혈로 인한 재수술 3례, , 술후 심막절개증후군 1례, 편측 성대마비 1례, 정신증 1례, 그리고 심근경색이 1례였다. 수술후 A/E비는 0.43$\pm$0.22였고, A파가 9(64%)례에서 양성이었다. 수술후 3개월에서 14개월(평균 추적관찰, 8.1개월)동안 14례에서 모두 정상 동방결절율동으로 전환되었고, 9례(64%)에서 좌심방의 수축을 관찰할수 있었으며, 11례(79%)에서는 항부정맥제를 투여하지 않고 있다. 결론: Maze III 술식에서 냉동절제를 시행하지 않더라도 승모판막 질환과 동반된 심방세동에 효과적인 수술임을 알 수 있었다.

• 한국임상수의학회지
• /
• 제37권3호
• /
• pp.153-156
• /
• 2020

A 14-year-old intact female Cocker Spaniel dog, weighing 6.8 kg, presented with chief complaints of severe cough, dyspnea, anorexia, and exercise intolerance. It had the characteristics of a blood clot, the evidence of mitral regurgitation, a high left atrial/aortic root ratio, and pericardial effusion with a left atrial rupture due to myxomatous mitral valve degeneration (MMVD) was strongly suspected. Traditional therapy (pimobendan, furosemide, enalapril, and spironolactone) for heart failure with MMVD was provided twice a day orally, and partial pericardiectomy was performed for pericardial effusion. The medical prescriptions for MMVD were continued, and the patient's heart disease was well-controlled. However, it suddenly died 3 months after the operation.