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

• Journal of Chest Surgery
• /
• v.34 no.9
• /
• pp.720-723
• /
• 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
• /
• v.35 no.7
• /
• pp.556-559
• /
• 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
• /
• v.34 no.8
• /
• pp.604-610
• /
• 2001

Stanford type A aortic dissection after graft replacement of ascending aorta and/or aortic arch required careful follow-up due to progression of the enlarged false lumen or the recurrence of dissection. From June 1984 to June 200, 124 patients underwent operations for type A aortic dissection. Among them, 6 patients underwent reoperation due to recurred aneurysm or dissection. We evaluated that the causes of reoperation, including Marfan syndrome, the approach and result of reoperation, and strategy to reduce the risk of reoperation. Material and method: The first operation was done on acute stage in 4 cases, and chronic stage in 2 cases. There were Marfan syndromes in 3 cases. The entry site was the ascending aorta for all cases except one who underwent Bentall operation(n=3) or ascending aorta graft replacement(n=2). In one case, Bentall operation and total arch replacement was performed due to chronic type A dissection with multiple fenestrations. Mean interval of reoperation was 67.6months(range 5 months to 14 year 4months) after the first operation. Reoperations were performed with recurrence of dissection(n=4), threatening aneurysmal evolution of persisting dissection(n=1), and false aneurysm with infection(n=1). The redo operation involved the hemiarch in 1 case, distal ascending to total arch and descending thoracic aorta in 4 cases, and only descending thoracic aorta in 1 case. Result: There were Marfan syndromes in 18 patients. The mean age in type A dissection was 56.7 years and that in the first operation of reoperationc ases was 32.2 years. Especially in 3 patients with Marfan syndrome, the mean age was 29 years.

• Journal of Chest Surgery
• /
• v.40 no.6 s.275
• /
• pp.441-444
• /
• 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.

• Journal of Chest Surgery
• /
• v.31 no.12
• /
• pp.1234-1237
• /
• 1998

Patients with aortic root disease, frequently seen in Marfan syndrome have progressive dilatation of the aortic sinuses and dilatation and distortion of the aortic annulus, leading to aortic incompetence. They are currently treated with composite graft replacement of the ascending aorta and aortic valve and reimplantation of the coronary arteries. Recently, we experienced an aortic root replacement with aortic valve preservation in a patient with annuloaortic ectasia. The ascending aorta and sinus was excised except the aortic annulus and aortic valve. The aortic valve was reimplanted inside of a collagen-impregnated tubular Dacron graft. The coronary arteries were also reimplanted. The patient was followed up for six months and reevaluated with the echocardiography. Postoperative Doppler echocardiography revealed normal aortic valve function. With this technique, it is possible to preserve the native aortic valve if the aortic leaflets are anatomically normal.

• Journal of Chest Surgery
• /
• v.19 no.3
• /
• pp.500-505
• /
• 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
• /
• v.28 no.1
• /
• pp.65-68
• /
• 1995

Pectus excavatum occasionally occurs in patients who have underlying cardiac disease, especially Marfan syndrome. This report describes a patient with pectus excavatum who had ascending aortic aneurysm with aortic regurgitation and anterior leaflet prolapse of mitral valve. This patient underwent replacement of aortic valve and ascending aorta with 25 mm SJM valved conduit graft[Bentall operation with Cabrol shunt , and mitral valve replacement with SJM 31 mm, the pectus excavatum was corrected at the time of completion of the intracardiac operation with the modified sternal turnover. This procedure offered excellent operative exposure for the inracardiac operation with prevention of low cardiac output after operation due to depressed sternum and maintained chest wall stability resulting good cosmetic chest wall appearance. This patient recovered and discharged in good postoperative result with minimal temporary peroneal nerve palsy in his left leg.

• Journal of Yeungnam Medical Science
• /
• v.6 no.1
• /
• pp.179-184
• /
• 1989

The Marfans syndrome is a generalized connective tissue disease involving eye, musculoskeletal system, cardiovascular system, and inherited autosomal dominant with various expression type. The cardiovascular complications such as aortic aneurysm, aortic dissection, aortic regurgitation, mitral regurgitation and aortic dissection which usually occurs in previously normal sized aorta are poor prognostic factors. However, the aortic dissection which developed in patient with Marfan syndrome and aortic aneurysm was rare. We experienced one case of dissecting aneurysm in patient diagnosed as previous aoritc aneurysm, aortic regurgitation, and Marfan syndrome, receiving successful operation.

• Journal of Chest Surgery
• /
• v.42 no.5
• /
• pp.639-644
• /
• 2009

Characterized by unique phenotypic features such as aortic aneurysm/dissection, hypertelorism, bifid uvula/cleft palate and generalized tortuosity in the arterial system, Loeys-Dietz syndrome is a newly described aggressive connective tissue disorder associated with mutation in the gene encoding transforming growth factor-$\beta$ receptor type I or type II. Some phenotypic manifestations of Loeys-Dietz syndrome overlap with those of Marfan syndrome or Ehlers-Danlos syndrome type IV. However, due to its more malignant pathophysiologic nature, physicians should be alert to Loeys-Dietz syndrome. High suspicion, early diagnosis, preventive surgery and serial imaging assessments are warranted for optimal management of Loeys-Dietz syndrome. We present here a case of a young patient with Loeys-Dietz syndrome who had aortic rupture, bifid uvula and hypertelorism. We also present a review of the medical literature.