• Journal of Chest Surgery
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• 제35권7호
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• pp.556-559
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• 2002

Marfan 증후군에 심한 누두흉이 동반된 경우 정중흉골절개시 개흉기를 걸기도 힘들고 대혈관과 심장의 노출이 어려울 수 있다. 저자들은 대동맥근확장의 직경이 52 mm, 중등도의 대동맥판 폐쇄부전증,고도의 승모판 폐쇄증, 누두흉을 가진 33세의 남자환자에서 대동맥근부의 근치술과 승모판 치환술을 시행하면서 누두흉을 성공적으로 치유했기에 보고한다. 정중절개에 의해 심장 및 대동맥에 접근했고 개심술 후 프로타민 투여 직후 시행된 변형된 늑연골의 절제와 흉골거상으로 수술후 안정된 흉벽을 형성할 수 있었다.

• Journal of Chest Surgery
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• 제40권7호
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• pp.508-511
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• 2007

일반적으로 대동맥 박리의 원인을 살펴보면 고혈압, Marfan 증후군과 같은 결체 조직성 질환, 이엽성 대동맥 판막과 같은 선천성 판막 질환, 침습적 시술, 임신, 마약 등 여러 가지이다. 이러한 대동맥 박리의 원인들 중 임신은 40세 이하 여성에서 나타나는 대동맥 박리 원인의 약 50%를 차지한다. 임신중의 대동맥 박리는 대부분 임신 제 3분기 또는 출산 중에 발생하고 이 중 약 반수에서 Marfan 증후군과 같은 결체 조직성 질환이 동반된다. 저자들은 임신 24주에 급성 제II형 대동맥 박리가 발생한 Marfan 증후군 환자에서 대동맥 판막을 포함한 상행 대동맥 치환술을 시행하고 추후 임신 38주에 제왕절개를 통해 건강한 태아를 출산한 치험 1예를 문헌 고찰과 함께 보고한다.

• Radiation Oncology Journal
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• 제32권3호
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• pp.208-212
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• 2014

Marfan syndrome is one of the collagen vascular diseases that theoretically predisposes patients to excessive radiation-induced fibrosis yet there is minimal published literature regarding this clinical scenario. We present a patient with a history of Marfan syndrome requiring radiation for a diagnosis of a right brachial plexus malignant nerve sheath tumor. It has been suggested that plasma transforming growth factor beta 1 (TGF-${\beta}1$) can be monitored as a predictor of subsequent fibrosis in this population of high risk patients. We therefore monitored the patient's TGF-${\beta}1$ level during and after treatment. Despite maintaining stable levels of plasma TGF-${\beta}1$, our patient still developed extensive fibrosis resulting in impaired range of motion. Our case reports presents a review of the literature of patients with Marfan syndrome requiring radiation therapy and the limitations of serum markers on predicting long-term toxicity.

• Journal of Chest Surgery
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• 제34권9호
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• pp.720-723
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• 2001

우관상동맥 폐쇄증을 동반한 Marfan 증후군환자를 보고한다. 환자는 45세 여자로 약 1년 전에 Marfan 증후군으로 진단받고, 최근 흉통을 주소로 내원하였다. 환자는 손가락과 발가락이 긴 지주증과 돌출흉이 있고 몸통에 비해 사지가 긴 전형적인 외형을 갖고 있었다. 시력이 매우 약하고 수정체의 아탈구가 있었다. 심초음파상 대동맥판막 폐쇄부전증은 경도로 있었고, 좌심실의 중등도 확장과 심박출율의 중등도 저하가 있었다. 수술시에 우관상동맥의 폐쇄증을 발견하였다. 대동맥판막 폐쇄부전증과 대동맥 확장증에 대해서 SJM 27mm composite graft를 이용하여 좌관상동맥에는 Bentall 술식과 우관상동맥에는 PTFE 6mm를 이용하여 변형된 Piehler 식 방법으로 수술을 하였다. 원래의 우관상동맥구로 추정되는 부분부터 우관상동맥 원위부의 혈류가 있는 곳까지의 결손부위는 약 4cm 가량 되었다. 관상동맥 폐색증과 Marfan 증후군의 조합은 매우 드문 질환으로 양측 관상동맥의 혈류 재건을 위하여 각기 다른 술식으로 수술하였다.

• Journal of Chest Surgery
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• 제19권3호
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• pp.500-505
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• 1986

Eight patients received operation for ascending aortic aneurysm and aortic regurgitation associated with Marfan`s syndrome from January 1984 to July 1986 at Seoul National University Hospital. The patients` age ranged from 29 to 51 years [mean 37.3 years]. Five patients were male and three were female. All of them showed some stigmata of skeletal system in Marfan`s syndrome. Three patients had dissecting aneurysm and five patients had fusiform aneurysm of ascending aorta. Two patients had concomitant fusiform aneurysm of abdominal aorta. All patients showed aortic regurgitation of grade III to IV. One patient received insertion of intraluminal ringed graft and resuspension of aortic valve, and seven patients received modified Bentall operation [Carol`s method]. There was no hospital death and all showed functional improvement in the 7.4 patient-years follow-up period.

• Journal of Chest Surgery
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• 제48권1호
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• pp.7-12
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• 2015

Background: Mitral regurgitation is one of the leading causes of cardiovascular morbidity in pediatric patients with Marfan syndrome. The purpose of this study was to contribute to determining the appropriate surgical strategy for these patients. Methods: From January 1992 to May 2013, six patients with Marfan syndrome underwent surgery for mitral regurgitation in infancy or early childhood. Results: The median age at the time of surgery was 47 months (range, 3 to 140 months) and the median follow-up period was 3.6 years (range, 1.3 to 15.5 years). Mitral valve repair was performed in two patients and four patients underwent mitral valve replacement with a mechanical prosthesis. There was one reoperation requiring valve replacement for aggravated mitral regurgitation two months after repair. The four patients who underwent mitral valve replacement did not experience any complications related to the prosthetic valve. One late death occurred due to progressive emphysema and tricuspid regurgitation. Conclusion: Although repair can be an option for some patients, it may not be durable in infantile-onset Marfan syndrome patients who require surgical management during infancy or childhood. Mitral valve replacement is a feasible treatment option for these patients.

• Journal of Chest Surgery
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• 제19권2호
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• pp.300-305
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• 1986

We have experienced two cases of ascending aorta aneurysm with aortic regurgitation in Marfan`s syndrome. There were abnormal findings in cardiovascular system associated with abnormalities of skeletal systems. They had total replacement of the ascending aorta and aortic valve with Bjork-Shiley`s aortic valve composite graft and reimplantation of coronary ostia on the graft. Their postoperative courses were uneventful and discharged with good clinical results for follow up.

• Journal of Chest Surgery
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• 제40권6호
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• pp.441-444
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• 2007

슬라이드 판막륜 성형술은 승모판막 폐쇄 부전증 환자에서 승모판막 성형술 후 드물게 발생하는 승모판막 전엽의 전방 운동의 발생을 막는 효과가 있다. 저자들은 과도한 판막륜 조직과 심각한 판막륜이완으로 인해 발생한 승모판막 역류를 보이는 말판 증후군 환자에서 벽측 판막륜 주름을 잡아 광범위한 사각절제술과 슬라이드 판막륜 성형술을 용이하게 하여 성공적인 판막 성형술을 시행하였기에 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제59권2호
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• pp.59-64
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• 2016

Purpose: Infantile Marfan syndrome (MFS) is a rare congenital inheritable connective tissue disorder with poor prognosis. This study aimed to evaluate the cardiovascular manifestations and overall prognosis of infantile MFS diagnosed in a tertiary referral center in Korea. Methods: Eight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated. Results: Their median age at the time of diagnosis was 2.5 months (range, 0-20 months). The median follow-up period was 25.5 months (range, 0-94 months). The median length at birth was 50.0 cm (range, 48-53 cm); however, height became more prominent over time, and the patients were taller than the 97th percentile at the time of the study. None of the patients had any relevant family history. Four of the 5 patients who underwent DNA sequencing had a fibrillin 1 gene mutation. All the patients with echocardiographic data of the aortic root had a z score of >2. All had mitral and tricuspid valve prolapse, and various degrees of mitral and tricuspid regurgitation. Five patients underwent open-heart surgery, including mitral valve replacement, of whom two required multiple operations. The median age at mitral valve replacement was 28.5 months (range, 5-69 months). Seven patients showed congestive heart failure before surgery or during follow-up, and required multiple anti-heart failure medications. Four patients died of heart failure at a median age of 12 months. Conclusion: The prognosis of infantile MFS is poor; thus, early diagnosis and timely cautious treatment are essential to prevent further morbidity and mortality.

• Journal of Chest Surgery
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• 제18권3호
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• pp.456-460
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• 1985

A case of Annuloaortic Ectasia associated with Marfan syndrome was treated by replacement of aorta and aortic valve with a valved conduit. A 26 years old man had suffered from palpitation and precordial pain. He had stigmata of Marfan`s syndrome. Aortogram and 2-D echocardiogram confirmed aneurysm of the ascending aorta with aortic insufficiency. Surgery was performed under the moderate hypothermia to 28oC. There was marked dilatation of the aortic annulus as well as sinus of Valsalva, with displacement of the coronary ostia. Aortic valve and aneurysm was replacement with 25mm, woven Dacron tubular graft, to which a 25mm, S.T. Jude Medical valve had been previously sutured. Right & left coronary ostia were anastomosed to the graft with the use of 3O Nylon pledget suture. The patient had a satisfactory post operation period with out specific complication.