• Title/Summary/Keyword: Marfan′s syndrome

Search Result 33, Processing Time 0.02 seconds

Clinical Analysis of 58 Cases of Aortic Dissecting Aneurysm (해리성대동맥류 58례에 대한 임상적 고찰)

  • 정철하
    • Journal of Chest Surgery
    • /
    • v.27 no.1
    • /
    • pp.31-35
    • /
    • 1994
  • Dissecting aortic aneurysm is a life threatening condition which necessitates prompt diagnosis and management. Between January 1987 and September 1993,58 patients was admitted to our department. Mean age at admission was 53 years.[range 25-82]. Clinical findings included chest pain in 48 cases[83%],renal failure in 12[20%],aortic insufficiency in 11[19%] and stroke in 9[15%]. Predisposing factors were hypertension in 50 cases[86%],Marfan`s syndrome in 6[10%] and diabetes melitus in 1 [2%]. 23 patients[ type A 13,type B 10 ] underwent surgical treatment. Surgical technique for type A included graft replacement of ascending aorta in 7 cases,graft replacement and aortic valve resuspension in 3,and Bentall`s operation in 3 cases. Type B patients were operated when specific indications applied. There were three [Two in type A and 1 in type B] deaths in the operation group and nine [ 5 in type A and 4 in type B] deaths in the medical group. These results support our current policy in the treatment of dissecting aortic aneurysm.

  • PDF

Clinical Study of Surgical Treatment of Aortic Aneurysm (대동맥류의 수술적 치료에 관한 임상적 연구)

  • Ryu, Ji-Yun;Jeon, Hong-Ju;Jo, Gwang-Hyeon
    • Journal of Chest Surgery
    • /
    • v.30 no.3
    • /
    • pp.300-307
    • /
    • 1997
  • We have experienced 25 cases of aortic aneurysm from October 1987 to January 1996. Patients ranged in age from 26yrs to 73yrs(mean age 52. Syrs). There were 13 males and 12 females. Eighteen cases were thoracic aneurysm and seven were abdom nal aneurysm. The cause of aneurysm were dissecting in 16cases aneurysms and non-dissecting in 9 cases. Risk factors of aortic aneurysm were hypertension, hypercholesterolemia, Marfan's syndrome. In thoracic aneurysm patients, 1'S cases of dissecting aneurysm underwent aneurysmectomy and replacement of vessel interposition graft with or without coronary artery implantation on the graft. 6 cases of non-dissecting aneurysm underwent operation with same policy as dissecting aneurysm. In 7 case of abdominal aneurysm,all patients underwent aneurysmectomy and graft interposition with straight i)r Y graft. Thcre were 5 postoperative death(mortality 20%). Several cases of complications were improved with proper managements. All survivors showed improvement in clinical symptom and sign and discharged without specific complications.

  • PDF

Surgical Experience of Aortic Root Replacement (대동맥근부치환술의 임상경험)

  • Kim, Hyun-jo;Ahn, Hyuk
    • Journal of Chest Surgery
    • /
    • v.30 no.12
    • /
    • pp.1197-1204
    • /
    • 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.

  • PDF

Surgical Treatment of Annuloaortic Ectasia - Review of 4 cases - (Annuloaortic ectasia의 외과적 치험)

  • Lee, S.;Ahn, W.S.;Kim, B.Y.;Lee, J.H.;Yu, H.S.
    • Journal of Chest Surgery
    • /
    • v.24 no.1
    • /
    • pp.36-40
    • /
    • 1991
  • Between November, 1981 and July, 1989, 4 patients, 3 male and 1 female patients ranging in age from 36 to 45 years, were operated on for aortic insufficiency associated with uncomplicated annuloaortic ectasia. All patients were in New York Heart Association class III. Two patients had clinical stigmata of the Marfan syndrome. The surgical treatment consisted of. supracoronary replacement of ascending aorta with vascular graft and replacement of the aortic valve in our first case. and composite graft replacement of the ascending aorta and aortic valve with reimplantation of the coronary arteries in subsequent 3 cases. Our first patient developed aneurysm of proximal aorta and pseudoaneurysm of distal aortic anastomosis 5 years postoperatively. One patient among the three patients with Ben-tall operation, died of ventricular fibrillation and myocardial failure during immediate postoperative period. Remaining 2 patients were in NYHA class I with follow-up of 16 months and 20 months respectively.

  • PDF

Surgical Treatment for Dissecting Aneurysm of the Aorta using Sutureless Intraluminal graft (무봉합 혈관내 인조이식혈관을 이용한 박리성 대동맥류의 수술요법)

  • 이재원
    • Journal of Chest Surgery
    • /
    • v.18 no.2
    • /
    • pp.305-313
    • /
    • 1985
  • Surgical therapy for dissection of the aorta has had a high mortality. One contributing factor has been hemorrhage from the prosthesis and the suture lines. Recently, a new method of treatment with an intraluminal graft that requires no end-to-end anastomosis has been developed. Of the four patients with dissecting aneurysm of the aorta treated by inserting sutureless ringed intraluminal graft at the Department of Thoracic and Cardiovascular Surgery, S.N.U.H., three were DeBakey type I [one with associated aortic insufficiency] and the other was DeBakey type III. Suspected etiology of the dissection was Marfan`s syndrome in one and hypertension in the others. Total cardiopulmonary bypass was utilized in repairing dissecting aneurysms of the ascending aorta [type A] and simple aortic crossclamping was used for the patient with dissecting aneurysm of the descending aorta. The basic technique consists of inserting the whole ringed graft into the true lumen of the dissected aorta and circumferentially ligating the aorta against the groove in the rings. The proximal ring of the graft effectively stabilized the flail aortic valve in patient with aortic insufficiency associated with dissection of the ascending aorta. There were no hospital deaths and one patient with type III dissecting aneurysm developed postoperative paraparesis and renal insufficiency which was resolved. Follow-up has been from 1 month to 16 months with no evidence of prosthetic problems, such as erosion, migration, or thrombosis.

  • PDF

Clinical Analysis of Surgery for Aortic Disease (대동맥 질환 수술의 임상적 고찰)

  • 안정태
    • Journal of Chest Surgery
    • /
    • v.28 no.10
    • /
    • pp.906-911
    • /
    • 1995
  • From January 1991 to January 1995, 11 patients with aortic diseases underwent various surgical repairs. The age at operation ranged from 26 years to 63 years[ mean=50.9 years . The disease entities included 8 aortic dissections[ type I in 4, type II in 2 and type III in 2 cases , 2 Marfan`s syndrome with annuloaortic ectasia and 1 desecending thoracic aortic aneurysm The operative procedures we tried were 3 Bentall`s operation, 5 graft replacement of ascending aorta, and 3 graft interposition in descending thoracic aorta.Overall hospital mortality rate is 36.3%[4/11 . And causes of death are pump weaning failure in 2 cases and multiorgan failure in 2 cases. It was that 2 sternal dehiscence & mediastinitis, 1 acute renal failure, 2 hypoxic brain damages and 2 postoperative psychosis were complicated. Recently we tried surgical repair of aortic dissection five out of 6 cases using total circulatory arrest with deep hypothermia at 14$^{\circ}C$. Total circulatory arrest time ranged from 18 to 26 minutes[ mean 22.2 minutes , and mean aortic cross-clamping time was 48.2 minutes. One of 5 patient died on the 7th postoperative day due to multiorgan failure. Mortality of patients with TCA was 20%[1/5 , and it of remainders was 50%[3/6 . Our result for surgical repair using total circulatory arrest with deep hypothermia is satisfactory on the basis of our clinical data.

  • PDF

Surgical Management of Aneurysm of the Ascending Aorta with Aortic Insufficiency (대동맥판부전이 동반된 상행대동맥류의 외과적 치험)

  • 박만실
    • Journal of Chest Surgery
    • /
    • v.26 no.6
    • /
    • pp.457-462
    • /
    • 1993
  • We retrospectively studied 34 patients who underwent operations of ascending aortic aneurysm and aortic valve replacement from August 1979 to July 1992 at the Yonsei Cardiovascular Center. Eight patients underwent supracoroanry non-composite graft replacement and separate aortic valve replacement[group I]. Twenty six patients underwent valved composite graft replacement with reimplantation of coronary arteries[group II]. Two cases in group II died within 1 month after the operation. Among the 32 survivors 28 patients have been followed up for an average of 59 months ranging from 1 months to 159 months. During the follow up periods, a pseudoaneurysm around the ascending aorta and a newly developed dissecting aneurysm in remaining aorta were noted in group II. There were 6 late deaths: 2 cases in group I and 4 cases in group II. Three cases among the 6 late deaths have stigmata of Marfan`s syndrome[1 cases in group I and 2 case in group II]. There was no statistically significant difference in actuarial survival rates between group I and group II[p > 0.05]. This study suggests that non-composite supracoronary graft interposition with separate aortic valve replacement is a safe surgical technique in patients who have normal aortic annulus and normal position of coronary ostia. However in degenerative disease such as cystic medial necrosis, composite graft replacement is recommended because this procedure eliminates entire abnormal tissue.But it seems to be important that the suture technique and strict follow-up in patients with diseased aortic wall.

  • PDF

Effects of Quinacrine HCl (Atabrine) on Spontaneous Pneumothorax (자연기흉(自然氣胸)에 대(對)한 Quinacrine HCl의 효과(效果)(®Atabrine))

  • Lee, N.S.;Kim, H.J.;Song, Y.J.;Kim, H.M.
    • Journal of Chest Surgery
    • /
    • v.9 no.1
    • /
    • pp.98-101
    • /
    • 1976
  • Clinical observations were made on a total 104 cases of spontaneous pneumothorax during the period of 5 years from MAR. 1971 to MAR. 1976 at the Dept. of Chest Surgery, Korea University Medical College. The ratio of male to female cases was 7.4 : 1 in male predominance, and age distribution was 73% of the total cases within the 15-40 age range. There were 56 patients (53.8%) of right sided pneumothorax and 44 patients (42.3%) of left sided pneumothorax, 8 patients presented bilateral spontaneous pneumothorax. The etiologic factors were tuberculous origin in 42.3%, rupture of bullae or blebs in 15.4%, acute pulmonary infection in 3.8%, pulmonary paragonimiasis in 2.9%, cysticercosis 1%, Marfan's syndrome in 1.9%, unknown causes in 32.7%. In all 104 cases, closed chest tube thoracostomy were performed for reexpansion of collapsed lung. In 50 cases of 104 cases, intrapleural instillation of Quinacrine HCl (Atabrine, Winthrop Laboratories, New York, N.Y.) thru the chest tube was used to control of recurrent pneumothorax, and compared observation with 54 cases control group of closed chest tube thoracostomy only. In both groups, 1) cure rates were 63% in control group and 88% in Atabrine group. 2) average duration of inserted chest tube were 5.6 days in control group and 5.7 days in Atabrine group.

  • PDF

Surgical Treatment of the Aortic Aneurysm (대동맥류의 외과적 치료)

  • 김학제;조원민;김태식;이송암;김욱진;손영상;최영호
    • Journal of Chest Surgery
    • /
    • v.31 no.1
    • /
    • pp.13-19
    • /
    • 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.

  • PDF

Comparision of Inclusion TechnicRue with Open Technique in Patients with Bentall Procedures (Bentall수술에서 inclusion technique 과 open technique의 비교)

  • 김정택;문준호
    • Journal of Chest Surgery
    • /
    • v.30 no.5
    • /
    • pp.506-511
    • /
    • 1997
  • To understand the clinical results of aortic root replacement with either inclusion or open technique, we analysed 53 patients who underwent replacement of the aortic root with composite graft between October,1980, and May, 1995. Annuloaortic ectasia was the most common indication for operation(29 patients), follwed by aortic dissection(22 patients). Among 53 patients, 19(35%) had Marfan syndrome. Three patients died during hospitalization (Mortality: 5.5%). The follow up was possible in 48 patients(Follow-up rate; 94%,mean duration;37 months). The actuarial survival rate at 24 months was 95% in open technique group, and 87% in inclusion technique group. Late complications developed in 10 patients. Dissecting aneurysm in the remaining aorta was noted in 3 patients with inclu ion 1,schnique, and a pseudoaneurysm from coronary artery anastomosis site developed in a patient with inclusion technique. In conclusion, there was no statistical differences in survival for 24 months between inclusion technique and open technique group. But late problems in the remaining aorta or death from unknown cause occurred with moderate frequency : careful follow-up after aortic root replacement thought to be important for long term survival.

  • PDF