• Journal of The Korean Dental Society of Anesthesiology
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• v.13 no.4
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• pp.209-214
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• 2013

Multiple caries in a pediatric patient often requires dental treatment under general anesthesia, especially when the patient is suffering from a systemic disease. The patient was a 6-year-old boy with Marfan syndrome and needed extensive dental treatment. Marfan syndrome is an inherited disorder resulting from mutations in Fibrillin-1 gene. Patients are known to have mainly cardiovascular, ocular, and musculoskeletal problems. Although clinical symptoms of the syndrome are age-realted, thus hindering early diagnosis of the disease in young children, our patient had been confirmed by a gene study at a younger age. Medical history of the patient revealed moderate to severe mitral regurgitation and aortic root dilatation, which required mitral valve replacement surgery with a mechanical valve. As a result, the patient was taking warfarin post-operatively and changes in medication had to be made before the dental treatment. Also, prophyalctic antibiotics had to be given before the treatment for prevention of (to prevent the) infective endocarditis. With careful control of the medications and bleeding tendency, general anesthesia and the treatment were done successfully without any complications.

• Journal of Chest Surgery
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• v.34 no.7
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• pp.557-558
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• 2001

We operated on a Marfan patient who had Stanford type A acute aortic dissection, aortic root aneurysm, and aortic regurgitation. The Yacoub-David aortic root remodeling procedure which preserves native aortic valve and replaces all three aortic sinuses and ascending aorta by a Dacron graft, was applied for this patient. A 24mm Hemashield graft was designed to three tongues at the aortic root end to meet the shape of the Valsalva sinuses. The patient recovered from the procedure uneventfully and there was no aortic regurgitation posto-peratively.

• Journal of Chest Surgery
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• v.30 no.12
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• pp.1197-1204
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• 1997

Between April 1981 and June 1996, 65 patients had aortic root replacement at our institution. Disease entities were pure aortic annuloectasia in 31 patients(47.7%), Stanford type A aortic dissection with annuloectasia in 8(43.1%), atherosclerotic aneurysm with aortic regurgitation in 4(6.2%), and paravalvular leakage after aortic valve replacement in 2(3.1 %). 34 patients(52.3%) had the clinical stigmata of the Marfan syndrome. The operative procedures were Bentall operation in 61 patients(93.8%); 3 of conventional procedure and 58 of Cabrol's modification, aortic valve-sparing operation in 2(3.1 %), and root replacement with homograft in 2(3.1%). Hospital deaths occurred in 3 patients(4.8%) because of uncontrolled bleeding(1) and bypass weaning failure due to low cardiac output(2), and all had emergency operation with Cabrol's procedure. Postoperative complications developed in 19(29.2%) patients and most of them were transient. Surviving 62 patients have been followed up to cumulative total 315.0 patient-years(mean 60.2 $\pm$42.4 months). Late deaths occurred in 7 patients(11.3%), aneurysmal changes of remaining aorta were detected in 12 patients(19.4%). Actuarial survival rate at 10 years was 72.0 $\pm$ 9.7%, and the subsequent aortic operation-free rate at 10 years was 68.0$\pm$ 8.9% In a multivariate analysis, Marfan syndrome, emergency operation, preoperative dissection, combined arch replacement, and total circulatory arrest emerged as significant risk factors for hospital death or subsequent aortic operation. Over 60 years of age was the only risk factor for late death. Our 16 years'cummulative experience shows that aortic root replacement, mainly by means of Cabrol's procedure, can be applied successfully to variety of aortic root disease. However, long-term follow up will be needed to determine the late result of aortic valve-saving operation and root replacement with homograft. When dissection is present or the distal native aorta is diseased in'Marfan patients, close follow-up is necessary because of the subsequent aneurysmal change of remaining aorta.

• Journal of Yeungnam Medical Science
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• v.33 no.1
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• pp.44-47
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• 2016

Primary dissection of the renal artery is rare. Spontaneous renal artery dissection can be associated with diseases such as medial degeneration, neurofibromatosis, syphilitic arteritis, tuberculosis, polyarteritis nodosa, Marfan syndrome, fibromuscular dysplasia, or Ehlers-Danlos syndrome (EDS). Among these causes, EDS related renal artery dissection is very rare worldwide and has not been previously reported in Korea. EDS are a group of heritable connective tissue disorders characterized by fragility of the skin and hypermobility of the joints. We describe the case history of a young man who presented with left side flank pain, hypermobility of the hand joints and showed left renal artery dissection on computed tomography and angiography that turned out to be the first complication of vascular type EDS.

• Journal of Chest Surgery
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• v.29 no.12
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• pp.1360-1365
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• 1996

From September 1992 to May 1996, 38 patients ranging in age from 23 to 78, were operated for aortic dissection at Asan medical center There were 21 men and 17 women. The underlying aortic pathology were acute aortic dissection in 23, chronic aortic dissection in 15. Eight patients had Martian syndrome. In 34 cases of DeBakey type I, II patients, femoral artery and vein and/or right atrial auricle were used as cannulation site. With deep hypothermic c rculatory arrest (esophageal temperature 12 $\pm$ 2.5$^{\circ}C$) and retrograde cerebral perfusion of cold oxygenated blood through SVC, we replaced the ascending aorta and the part of arch if necessary. The mean duration of the total circulatory arrest time was 25 $\pm$ 1.7 mintstuts. In 4 cases of DeBakey type III patients, we replaced descending thoracic aorta or thoracoabdomlnal aorta without shunt or bypass under normothermia with an average 30: 1.5 minutesaortic cross clamp time. One death(2.6%) occurred on the twenty-second postoperative day owing to asphyxia related to ulcer bleeding. Postoperative complications were myocardial infarction with transient left peroneal palsy in 1 case, transient lower extremity weakness in 1 case and prolonged ventilatory support in 1 case. Two patients required reoperation due to retrograde extended dissection and aortic insufuciency. There was no late death with an average 25 months follow-up period.

• Journal of Chest Surgery
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• v.33 no.11
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• pp.886-893
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• 2000

배경: 흉복부 대동맥류는 질환 자체가 광범위하고 수술 중 척수를 포함한 각종 장기의 보호 및 허혈 방지라는 면에서 아직도 수술 합병증이 높은 질환이다. 본 연구에서는 그 동안의 임상경험을 바탕으로 치료성적, 합병증 발생, 사망률 등을 검토하였다. 대상 및 방법: 1990년 1월부터 1999년 12월까지 흉복부동맥류로 수술을 시행한 38명의 환자를 대상으로 의무 기록 검토을 통한 후향적 분석을 시행하였다. 결과: 남자가 22명, 여자가 16명, 평륜 연령은 46.2$\pm$12.3세였고, 크로포드 진단분류 상 1형 이 13례(34.2%), 2형이 19례(50%), 3형이 4례(11%), 4형이 2례(4.8%)였다. 만성 대동맥박리증에 관련된 경우가 29례(76.3%)로 가장 많았고, Marfan 증후군이 동반된 경우가 9례(23.7%)에서 있었다. 원위부 대동맥 관류를 35례에서 시행하였으며, 고동맥-고정맥 심폐바이패스를 31례에서, 좌심방-고동맥 바이패스를 4례에서 시행하였다. 심폐바이패스를 이용한 31례중 4례에서 초저체온하 완전순환정지를 이용하였다. 대동맥 차단 중 복강내 주요 대동맥 분지로의 선택적 관류를 시행하였다. 대동맥류의 최대직경은 평균 8.2$\pm$2.4cm이었고, 동맥류가 파열된 경우는 11례(28.9%)에서 있었다. 전례에서 인조혈관을 이용한 대동맥의 치환술을 시행하였다. 단계적으로 흉복부 대동맥을 겸자하면서 인공혈관을 문합하였고, Adamkiewicz 동맥으로 생각되는 부위의 늑간 동맥을 문합해주었는데, 8번째부터 12번째 흉추 사이의 늑간 동맥 중 역류혈류가 나타나고 상대적으로 내경이 큰 것들을 문합해 주었다. 술 후 조기사망은 3례에서 있었으며, 사인은 심폐기 이탈 실패 (2례), 저혈압 및 산증(1례) 등이었다. 조기 합병증으로는 애성 5례, 출혈 5례, 창상간염 3례, 장기간의 인공호흡기보조가 3례 등이 있었다. 치명적인 조기 합병증인 하지마비는 2례(5.3%)에서 발생하였고, 이 중 1례는 대동맥의 심한 석회화로 늑간동맥을 문합해 주지 못했던 경우였다. 35명의 환자에서 평균 추적기간은 103.1$\pm$6.1 개월, 2년 생존율은 93.8%, 5년 생존율은 86.1%, 8년 생존율은 80.7%였다. 추척기간 동안 4례의 만기사망이 관찰되었고, 사인은 2례에서는 갑작스런 의식소실이 발생하여 규명하기 어려웠으며, 대동맥-식도루 발생에 의한 경우가 1례, 경동맥류 파열에 의한 경우가 1례 등이었다. 만기 합병증으로는 복부 대동맥류(2례), 상행 대동맥 및 대동맥 근부 확장(1례), 대동맥-늑막루(1례), 창상 부위 탈장(1례), 역행성 사정(1례) 등이 있었다. 결론: 저자들은 흉복부대동맥류의 수술에 있어 심폐우 회술 혹은 좌심방-고동맥 바이패스를 통한 원위부 관류 및 저체온법, 수술 시 척수의 혈류공급과 관련된 늑간동맥의 연결 등으로 주요 신경합병증의 발생을 줄이고 좋은 성적을 거두고 있다고 판단하였으며 향후 임상 경험 축적과 함께 보다 정련된 위험인자의 분석이 필요하다고 본다.

• Journal of Chest Surgery
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• v.31 no.1
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• pp.13-19
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• 1998

Aortic aneurysm has poor prognosis and high mortality, but the incidence of aortic aneurysm is in increasing state. From July, 1986 to July, 1996, we operated on 25 patients with aortic aneurysm and analysed the clinical results and relations between the duration from symptoms onset to operation(Sx-Op), the duration from admission to operation(Adm-Op), preoperative blood pressure, preoperative heart rate and postoperative mortality, retrospectively. The patients were classified as dissecting aneurysm(10 cases), abdominal aortic aneurysm(9 cases), Marfan's syndrome(3 cases), descending thoracic aortic aneurysm(3 cases). The operative technique were graft interposition in 17 cases, Bentall's operation in 4 cases, aneurysm bypass in 2 cases, and wrapping of aorta in 2 cases. Seven patients died of several causes, bleeding in 5 cases, acute renal failure in 1 case and respiratory failure in another one case. Before 1992, the early stage of operation, 6 mortality among 14 operated patients occurred, and after then 1 mortality among 11 operated patients occurred. Eighteen survivors were followed up from 1 to 118 months(mean 50.6 months), and total follow up was 911 patient-months. During the follow up period one patient died of melena 30 months after operation. The other patients did not complain chest pain or dyspnea. The surgical mortality was improved in the late period, and the major cause of death was intraoperative or postoperative bleeding. The Sx-Op duration, the Adm-Op duration, preoperative blood pressure and preoperative heart rate were proven to have no statistical relations with postoperative mortality.

• Journal of Chest Surgery
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• v.35 no.2
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• pp.102-112
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• 2002

This study was undertaken to analyze the outcome of composite valve graftreplacement(CVGR) for the treatment of aneurysms of the ascending aorta involving the aortic root. Material and Method: Between April 1995 and June 2001, 56 patients had replacement of the ascending aorta and aortic root with a composite graft valve and were reviewed retrospectively. Aortic regurgitation was present in 50 patients(89%), Marfan's syndrome in 18 patients(32%), and bicuspid aortic valve in 7(12.5%). The indications for operation were annuloaortic ectasia(AAE) in 30 patients(53.6%), aortic dissection in 13(23.2%), aneurysms of the ascending aorta involving aortic root in 11(19.6%), and aortitis in 2(3.6%). Cardiogenic shock due to the aortic rupture was present in 2 patients. Nine patients(16%) had previous operations on the ascending aorta or open heart surgery. The operative techniques used for CVGR were the aortic button technique in 51 patients(91%), the modified Cabrol technique in 4, and the classic Bentall technique in 1. The concomitant procedures were aortic arch replacement in 24 patients(43%), coronary artery bypass graft in 8(14.3%), mitral valve repair in 2, redo mitral valve replacement in 1, and the others in 7 The mean time of circulatory arrest, total bypass, and aortic crossclamp were 21$\pm$14 minutes, 186$\pm$68 minutes, and 132$\pm$42 minutes, respectively. Result: Early mortality was 1.8%(1/56). The postoperative complications were left ventricular dysfunction in 16 patients(28.6%), reoperation for bleeding in 7(12.5%), pericardial effusion in 2, and the others in 7. Fifty-three patients out of 55 hospital survivors were followed up for a mean of 23.2 $\pm$ 18.7 months(1-75 months). There were two late deaths(3.8%) including one death due to the traumatic cerebral hemorrhage, and CVGR-related late mortality was 1.9%. The 1- and 6-year actuarial survival was 98.1$\pm$1.9% and 93.2$\pm$5.1%, respectively. Two patients required reoperation for complication of CYGR(3.8%) and two other patients required subsequent operations for dissection of the remaining thoracoabdominal aorta. The 1- and 6-year actuarial freedom from reoperation was 97.8$\pm$2.0% and 65.3$\pm$26.7%, respectively.

• Journal of Chest Surgery
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• v.38 no.7 s.252
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• pp.483-488
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• 2005

Aortic valve-sparing procedures could reduce valve-related morbidity, but may increase operative risks; therefore, these procedures could not be performed routinely. We attempted to find out the early results while focusing on the operative risks associated with these procedures in our hospital. Material and Method: From May 1996 to July 2003, 26 patients underwent these procedures including 15 patients with Marfan syndrome and 1 patient with Behcet disease. There were 17 men and 9 women with mean age of $37.9\pm19.2$ years (range: 6 months-74 years). Ten patients had ascending aortic dissection, 18 patients had more than moderate degree of aortic valve insufficiency (AI). Two types of valve-sparing procedures were performed: valve reimplantation in 14 and root remodeling in 12 patients. Associated procedures were performed as follows: aortic valve plasty in 6, mitral valve plasty in 5, hemi-arch replacement in 4, total arch replacement in 2, coronary artery bypass surgery in 1 and Maze procedure in 1 patient(s). Result: In four patients, valve-sparing procedures were converted to Bentall procedures during operation. Including these patients, there was no operative deaths, 3 patients underwent re-operation due to bleeding, 1 patient had permanent pacemaker. The median duration of ICU stay was 45.5 hours, the median duration of hospital stay was 10.5 days. In 22 patients excluding 4 converted patients, intraoperative transesophageal echocardiogram (TEE) showed less than mild degree of AI in all except one who had not received intra-operative TEE in the beginning and showed moderate degree of AI at discharge. The mean duration of follow-up was $21.2\pm27.4$ months. All patients were alive except one who died during other departmental surgery. In 3 patients, more than moderate degree of AI was recurred, but there were no reoperation. Conclusion: Aortic valve-sparing procedures could be performed relatively safely in selected patients who had annuloaortic ectasia.