• Journal of Chest Surgery
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• v.35 no.7
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• pp.556-559
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• 2002

The presense of pectus excavatum in Marfan's syndrome may complicate cardiac operation by making midline sternotomy technically more difficult and limiting the operative exposure of the heart. We operated on a 33 year old male patient with Marfan's syndrome and severe pectus excavatum who had severe mitral regurgitation and moderate aortic regurgitation with 52mm aortic root dilation. The operative field was adequately exposed through a midline sternal incision with two sternal retactors. The patient underwent Bentall operation and mitral valve replacement. The repair of pectus excavatum was performed after completion of CPB and the administration of protamin. Permanent internal stabilization achieved by overlapping of the ends of lower ribs and reinforced with sternal closure wire.

• Journal of Chest Surgery
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• v.40 no.7 s.276
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• pp.508-511
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• 2007

The causes of aortic dissection are usually hypertension, connective tissue disease such as Marfan syndrome, congenital valvular abnormality such as bicuspid aortic valve, iatrogenic injury, pregnancy and drugs. Previous studies have shown that 50% of all dissections in women less than 40 years age were associated with pregnancy. Almost all aortic dissections during pregnancy occur during the third trimester or during labor and delivery. Marfan's syndrome is a particularly important predisposing factor for aortic dissection during pregnancy. We report here on a case of surgical treatment for acute type II aortic dissection in a Marfan syndrome patient who was 24 weeks pregnant, and we include a review of literature.

• Radiation Oncology Journal
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• v.32 no.3
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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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• v.34 no.9
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• pp.720-723
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• 2001

A case of Marfan’s syndrome with atresia of right coronary artery is reported. A 45-year-old woman, who was diagnosed as Marfan’s syndrome 1 year ago, came to the hospital complaining of acute chest pain. The patient showed arachynodactyly, pectus carinatum, and long and slender extremities. In echocardiography there were severe aortic regurgitation measured grade IV and aortic dilatation of ascending aorta maximally 5.9 cm in diameter. Mitral regurgitation was mild, but there were also moderate left ventricular dilation and moderately decreased ejection fraction of left ventricle. At operation, atresia of right coronary artery was found. We performed Bentall type operation with SJM 27mm valved conduit for left coronary artery, and Piehler’s modification for right coronary artery bypass using 6mm PTFE graft. The atretic portion of right coronary artery from the suspected right coronary ostium to distal coronary flow was about 4 cm in length. The combination of right coronary artery atresia and Marfan’s syndrome is very rare. The author describes the rare case, which is treated with combined technique of Bentall and Piehler modification for reconstruction of coronary circulation.

• Journal of Chest Surgery
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• v.19 no.3
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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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• v.48 no.1
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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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• v.19 no.2
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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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• v.40 no.6 s.275
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• pp.441-444
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• 2007

Sliding annuloplasty has been used for mitral valve repair in conjunction with posterior leaflet quadrangular resection to avoid systolic anterior motion of the anterior leaflet of the mitral valve. Herein, we report on a case of successful mitral valve repair with using the annular plication technique to facilitate sliding annuloplasty and extensive quadrangular resection was also done for treating a Marfan's syndrome patient who had an extremely redundant leaflet and a severely dilated annulus.

• Clinical and Experimental Pediatrics
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• v.59 no.2
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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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• v.18 no.3
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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.