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1.
Five cases of Marfan's syndrome with cardiovascular lesions are presented. Among these five patients, two with type I aortic dissection, one with true aneurysm of the ascending aorta and one with aortic regurgitation underwent composite grafting of the ascending aorta according to the Bentall technique. The fifth patient died preoperatively, due to extensive dissection of the transverse aorta. Marfan's syndrome is transmitted in an autosomal dominant manner. Fifty percent of the patients die at a mean age of 32 years, and 95 p. 100 of the deaths are caused by cardiovascular lesions. These lesions predominantly affect the left cardiac valves (aortic or mitral regurgitation) and the ascending aorta. Except in emergencies, replacement of the ascending aorta by a composite graft must be envisaged in subjects with Marfan's syndrome presenting with aortic regurgitation and dilatation of the aortic root.  相似文献   

2.
A 24-year-old pregnant woman with Marfan's syndrome delivered by cesarean section during the 38th week of gestation. Although aortic root dilatation did not increase during pregnancy, three months after delivery, the patient noticed a pulsatile abdominal mass. Aortic aneurysm was diagnosed and surgical replacement of the infrarenal abdominal aorta to the common iliac arteries and reconstruction of the inferior mesenteric artery were performed. Moreover, the patient subsequently developed a Stanford type B thoracic aortic dissection, even after more than four months of beta-blockade.  相似文献   

3.
OBJECTIVES: The purpose of this study was to use transesophageal echocardiography (TEE) to define the mechanisms of aortic regurgitation (AR) in acute type A aortic dissection so as to assist the surgeon in identifying patients with mechanisms of AR suitable for valve preservation. BACKGROUND: Significant AR frequently complicates acute type A aortic dissection necessitating either aortic valve repair or replacement at the time of aortic surgery. Although direct surgical inspection can identify intrinsically normal leaflets suitable for repair, it is preferable for the surgeon to correlate aortic valve function with the anatomy prior to thoracotomy. METHODS: We studied 50 consecutive patients with acute type A aortic dissection in whom preoperative TEE findings were considered by the surgeons in planning aortic valve surgery. Six patients did not undergo surgery (noncandidacy or refusal) and one patient had had a prior aortic valve replacement and therefore was excluded from the analysis. RESULTS: Twenty-seven patients had no or minimal AR and 22 had moderate or severe AR. In all, there were 16 with intrinsically normal leaflets who had AR due to one or more correctable aortic valve lesion: incomplete leaflet closure due to leaflet tethering in a dilated aortic root in 7; leaflet prolapse due to disrupted leaflet attachments in 8; and dissection flap prolapse through the aortic valve orifice in 5. Of these 16 patients, 15 had successful aortic valve repair whereas just 1 underwent aortic valve replacement after a complicated intraoperative course (unrelated to the aortic valve). Nine patients underwent aortic valve replacement for nonrepairable abnormalities, including Marfan's syndrome in four, bicuspid aortic valve in four, and aortitis in one. In patients undergoing aortic valve repair, follow-up transthoracic echocardiography at a median of three months revealed no or minimal residual AR, and clinical follow-up at a median of 23 months showed that none required aortic valve replacement. CONCLUSIONS: When significant AR complicates acute type A aortic dissection, TEE can define the severity and mechanisms of AR and can assist the surgeon in identifying patients in whom valve repair is likely to be successful.  相似文献   

4.
Between October 2000 and January 2002, 9 consecutive male patients with subacute or chronic aortic dissection underwent stent-graft placement. The indication for surgery was continuous pain or aneurysm development. One patient had a type A dissecting aortic aneurysm with a primary tear in the ascending thoracic aorta; the other 8 had type B dissection. Placement of an endovascular stent-graft was technically successful in 8 patients, and one underwent an open procedure for abdominal aortic fenestration. The entry site was sealed and the false lumen disappeared in 8 cases, and thrombosis of the false lumen was obtained. Rupture of an iliac artery dissecting aneurysm occurred in one patient 2 days after stent-graft placement; abdominal aortic fenestration with prosthetic replacement of the distal abdominal aorta was performed. One patient died of myocardial infarction 3 days after the stent-graft procedure. During a mean follow-up period of 7 months (1-16 months), one patient died of acute myocardial infarction at 11 months. It was concluded on the basis of these short-term results that endovascular repair of aortic dissection is a promising treatment, and abdominal aortic fenestration is a useful adjuvant procedure.  相似文献   

5.
AIMS: Intramural haematoma (IMH) forms part of the acute aortic syndrome presenting physiopathologic and evolutive patterns different from those of aortic dissection. The aim of this study was to determine the mortality and predictive factors of IMH in the first 3 months of evolution. METHODS AND RESULTS: Sixty-eight consecutive patients diagnosed of IMH (12 type A, 56 type B) were prospectively studied. Ten patients (eight type A, two type B) were surgically treated for clinical or haemodynamic evolution. Mortality rate was 19% (six type A and seven type B): five surgically treated (three type A, two type B) and eight medically treated (three type A, five type B). No relationship was observed between clinical variables and evolution. Maximum aortic diameter was greater in the group of patients who died (65.5+/-14.4 mm vs 46.0+/-7.6 mm; P<0.0001). Mortality rate in patients with aortic diameter >50 mm was 50% (P<0.0001). Significant periaortic bleeding was mortality-related (47%; P<0.005). Multivariate analysis showed only a significant relationship between mortality and maximum aortic diameter >50 mm (OR=11.33; P<0.005) and ascending aorta involvement (OR=11.18; P<0.05). CONCLUSION: Intramural haematoma mortality in the first 3 months of evolution is high (19%). Maximum aortic diameter >50 mm and ascending aorta involvement are predictive of early mortality.  相似文献   

6.
BACKGROUND: Replacement of the aortic valve and ascending aorta with a composite graft is the most common surgical treatment for aortic root aneurysms with or without aortic regurgitation (AR). In the early 90's reconstructive procedures of the aortic root have been described with encouraging results. This paper presents our experience with this technique. METHODS: Between January 2001 and May 2003, 28 patients (25 males, 3 females, mean age 60 years) with aortic root aneurysm were treated with reimplantation of the aortic valve. Twenty-two patients had AR > 2+, 5 had Marfan syndrome, 5 had an aortic arch aneurysm, 4 had type A aortic dissection, 2 patients had associated coronary artery disease, and one had mitral valve insufficiency. The only contraindication was primitive disease of the aortic leaflets. RESULTS: There was one perioperative death (type A aortic dissection) and 1 patient was discharged with mild to moderate AR requiring aortic valve replacement. The cardiopulmonary bypass and aortic cross-clamping times were 230 and 184 min respectively. No neurological events were recorded. During follow-up (mean 16.7 months, range 3-32 months) 1 patient died and one had mild AR. Freedom from reoperation and from AR at 24 months was 94 and 89% respectively. At multivariate analyses we did not find any correlation between Marfan syndrome, type A aortic dissection, grade of preoperative AR, and recurrence of AR. CONCLUSIONS: In our experience, valve-sparing surgery was feasible with a low mortality and morbidity and with good early results. It should be applied to all patients requiring aortic root surgery for aortic root aneurysm, a diseased aortic valve being the only contraindication.  相似文献   

7.
The authors report a series of 6 cases of recurrent dissection, 4 of which were treated surgically, out of a total of 64 acute dissections of the aorta referred to the CMC Foch, between January 1969 and October 1981. Three types of recurrent dissection were identified: --"de novo" recurrent dissection: a new dissection occurring in part of the aorta previously unaffected with a new intimal tear; --recurrent dissection due to extension of the previous dissection; --"in situ" recurrent dissection. Surgery is the treatment of choice because of the poor prognosis. Extensive resection (sometimes carried out in several stages) and deep hypothermia are valuable techniques when part of the aorta giving off arteries to vital organs has to be replaced. The use of GRF biological glue in the treatment of the original acute dissection has been a significant advance in the prophylaxis of recurrent dissection as it ensures better repair of the distal false lumen (27% persistent false lumens). With respect to an extensive replacement of the dissected vessel, the authors advise operation in several stages, especially in young subjects with Marfan's syndrome who are at high risk of recurrent dissection. All cases of acute dissection of the aorta, operated or not, should be followed up indefinitely with clinical and radiological examination, completed, when necessary, by an angioscan and an aortography.  相似文献   

8.
Acute aortic dissection during pregnancy   总被引:2,自引:0,他引:2  
We review the cases of two women with acute aortic dissection during their last trimester of pregnancy who underwent aortic root replacement. One patient with type A dissection had to undergo surgical intervention immediately, and required extracorporal membrane oxygenation for four days. The other patient with a pre-diagnosed Marfan's disease had suffered a type B dissection and had to undergo emergency operation after developing a type A dissection nine days later.  相似文献   

9.
OBJECTIVES: The purpose of this study was to examine clinical and echocardiographic predictors of outcome in a cohort of patients with Marfan's syndrome. BACKGROUND: Serial echocardiographic measurements of aortic root dimensions are an important clinical method for monitoring patients with Marfan's syndrome. However, there are few data on the prognostic importance of echocardiographic variables for risk stratification and timing of aortic root replacement. METHODS: In 89 consecutive patients with Marfan's syndrome (age range 1-54 years) clinical and serial echocardiographic data (n = 62) were evaluated as potential predictors of outcome (mean (range) follow up 4 (< 1-16) years). Aortic sinus diameter and an aortic ratio normalised for age and body surface area were examined using Kaplan-Meier life table and Cox regression analysis, with the end point defined as death or surgery for ascending aortic dissection and events defined as an end point or surgery for ascending aortic aneurysm. RESULTS: Overall actuarial survival at two and five years was 96% and 92% and event free survival was 85% and 76%, respectively. There were five deaths due to aortic dissection, four patients survived surgery for ascending dissection, and nine underwent root replacement with a composite graft for ascending aneurysm. Those with aortic events were older (35 (12) v 25 (13) years, P = 0.007) and had greater initial aortic root dimensions (47 (14) v 33 (8) mm, P < 0.0001) and ratios (1.6 (0.5) v 1.3 (0.2), P < 0.0001). In the 62 patients with serial echocardiographic follow up, the rate of aortic root dilatation was more rapid in those with events (15 (17) v 0 (3)%/year, P < 0.0001). Utilising a Cox proportional hazards model, the groups with an initial aortic ratio > or = 1.3 or an annual change in aortic ratio > or = 5% had a relative risk of an aortic complication of 2.7 and 4.1, respectively (95% confidence limits 1.5 to 4.8 and 1.8 to 9.3). Only one of 31 patients with an initial aortic ratio of < 1.3 and a rate of change of < 5% had an event (five year event free survival 97%). CONCLUSIONS: A low risk subgroup of patients with Marfan's syndrome can be identified as those with an aortic ratio < 1.3 and an annual change in aortic ratio of < 5%. These findings are helpful in optimising echocardiographic monitoring and risk stratification of patients with Marfan's syndrome.  相似文献   

10.
PURPOSE: To evaluate aortic diameter outcomes after stent-graft implantation for aortic dissection in the descending thoracic aorta. METHODS: Fifty patients with type A dissection after ascending aortic surgery (n = 10), type B dissection (n = 34), or intramural hematoma (n = 6) underwent stent-graft repair in 3 centers. Thrombosis and aortic diameter were analyzed by computed tomographic angiography at different levels of the aorta before stent-graft implantation, at discharge, and at follow-up. Measurements were standardized. RESULTS: In all, 67 stent-grafts were implanted for acute (n = 18) and chronic (n = 32) dissection. Stent-graft placement was successfully performed with high technical success (100%) despite 4 major complications (iliac thrombosis in 2 cases, aortic rupture, and a type A dissection) in 3 (6%) patients. Complete thrombosis of the thoracic false lumen was observed in 42% and 63% of cases at discharge and at follow-up (mean 15 months), respectively. At follow-up, the diameters of the entire aorta (mean 5 mm, p < 0.05) and the false lumen (mean 11 mm, p < 0.0001) decreased. Diameters of the abdominal aorta remained stable in association with persistent false lumen perfusion at this level. Aortic diameter results were better in the subgroup of patients with intramural hematoma compared to patients with Marfan syndrome. Three early deaths unrelated to the stent-graft procedure occurred; 2 patients with partial thrombosis of the false lumen died in follow-up secondary to aortic diameter growth. CONCLUSION: Complete thrombosis of the false lumen by stent-graft coverage of the entry tear results in decreased diameter of the entire aorta. In patients with partial thrombosis of the false lumen, the aneurysm continues to enlarge.  相似文献   

11.
主动脉腔内修复术治疗75例DeBakey Ⅲ型主动脉夹层   总被引:3,自引:0,他引:3  
目的 总结主动脉腔内修复术(EVAR)治疗DeBakey Ⅲ型主动脉夹层适应证的选择、临床疗效以及探讨并发症的防治原则.方法 2002年12月至2007年6月采用TALENT带膜支架施行EVAR治疗75例DeBakeyⅢ型主动脉夹层患者,男65例,女10例,平均年龄54.4±12.6(29~83)岁.1例29岁女性病因考虑为埃-当综合征,2例年青男性分别继发于原发性醛固酮增多症和外伤车祸.全部患者均经螺旋CT或磁共振血管造影检查确诊.全身麻醉下经股动脉置入带膜血管内支架,封堵原发破口,重复造影检查.结果 75例患者共置入81枚带膜支架.其中2例因近端破口接近左锁骨下动脉,先行颈总动脉-锁骨下动脉旁路移植术,再置入支架,支架近段覆盖左锁骨下动脉.25例支架置入后出现明显内漏,20例予球囊扩张,6例置入1枚短支架后内漏消失.支架置入技术成功率为100%.1例埃-当综合征女性患者术中出现股动脉、髂外动脉、髂总动脉广泛血管撕裂,失血性休克,经抢救好转恢复.2例术后早期死于夹层破裂.术后随访1~24个月,2例(包括1例埃-当综合征患者)术后半年发生猝死,1例术后1年复查CT发现支架远端出现破口,经外科手术治疗后恢复良好;其余存活者复查螺旋CT示假腔径缩小并见腔内血栓形成,真腔径增大,无瘤体破裂、内漏及支架移位等并发症发生.结论 EVAR治疗DeBakeyⅢ型主动脉夹层具有技术可靠、创伤小、术后恢复快、成功率高等优点,尤其适用于不能耐受传统开胸手术的老年高危患者.而对于年轻的主动脉夹层患者要注意排除埃-当综合征等遗传性疾病,用EVAR治疗埃-当综合征的血管并发症,目前尚未见报道,其手术并发症及疗效有待进一步观察.  相似文献   

12.
In a previous report we stated that two distinct types of aortic dissection exist, namely, the opened-communicated type (O type) which demonstrates communication between the true lumen and the false lumen, and the thrombosed type (T type, also known as aortic dissecting hematoma or as intramural hematoma). The latter, in its acute phase, shows no opacification of the false lumen through medical imagings such as computed tomography (CT) scans or angiography. We analyzed the pathoclinical features of the acute phase. Over a period of 14 years, a study was conducted of 371 patients who were classified as O type and 95 who were classified as T type. The mean age was significantly higher in T-type patients and the sex ratio showed a significantly higher proportion of males. As for atherosclerotic risk factors, such as hypertension and diabetes, hypertension was frequently evident and more frequently seen in T-type patients. The incidence of Marfan's syndrome was 11%, and was not observed in T-type patients. In 18 T-type patients (19%), complications such as cerebral ischemic episodes were observed. However, they were transient and not serious, sometimes indicating only minor surgery.Presented at The 39th Annual World Congress, International College of Angiology, Istanbul, Turkey, June 1997.  相似文献   

13.
Aim of the study was to elucidate causes of repeat operations and their outcomes in patients with the Marfan's syndrome. Between December 1972 and January 2009 60 patients with Marfan's syndrome were operated for aneurysm of the ascending aorta and aortic insufficiency. All patients were subjected to ascending aorta and aortic valve replacement with valve containing conduit. Five of 60 patients (8.3%) were subjected to 7 repeat operations (2 patients were reoperated twice). Causes of repeat interventions were: conduit dysfunction (n=3), abdominal aortic aneurysm (n=3), mitral valve prolapse (n=1). Three patients died: 2 during surgery because of impaired conduit function, one - in 1 year after repeat operation of mitral valve replacement for mitral valve prolapse with pronounced mitral insufficiency. In all cases cause of death was progressive heart failure. As an example a case of a female patient who had been subjected to repetitive surgeries because of malfunctioning conduit is presented in this paper. Main reason for reoperations was the Marfan's syndrome itself. Progression of this disease might continue after operation, alteration of the aortic wall promote dissection in regions not subjected to prosthetics. Application of prostheses especially designed for replacement of the ascending aorta excludes such causes of reoperations and death as disruption of old sutured of two parts conduits.  相似文献   

14.
OBJECTIVE—To evaluate survival and complication free survival in patients with Marfan's syndrome and to assess the possible influence of recently revised guidelines for prophylactic aortic root replacement in these patients.
METHODS—130 patients who had been attending one institution over 14 years were evaluated. Kaplan-Meier analysis was performed in 125 patients who did not present with aortic root dissection as the first sign of Marfan's syndrome, with the end points: death, aortic root dissection, and prophylactic aortic root replacement after diagnosis. In the patients developing aortic root dissection, current guidelines for prophylactic aortic root replacement were retrospectively applied to investigate the number of dissections that could theoretically have been prevented. The guidelines were: (1) aortic root diameter  55 mm, (2) positive family history of aortic dissections and aortic root diameter  50 mm, and (3) aortic root growth  2 mm/year. Outcomes following emergency surgery (15 patients) and prophylactic surgery of the aortic root (30 patients) were compared.
RESULTS—Five and 10 year survival after diagnosis was 95% and 88%, and the five and 10 year complication free survival was 78% and 66%, respectively. Thirteen patients developed dissection, 30 underwent prophylactic repair, and 82 had an uncomplicated course. Eleven dissections could theoretically have been prevented by application of the current guidelines. Five year survival following emergency and prophylactic repair of the aortic root was 51%, and 97%, respectively.
CONCLUSIONS—Survival in the Marfan's syndrome in the past 14 years seems satisfactory; with application of current guidelines, it has probably even improved. However, because of the high fatality rate in Marfan patients developing aortic root dissection, more extensive screening for Marfan's syndrome and a search for additional risk factors are desirable.


Keywords: Marfan's syndrome; aortic root dissection; survival  相似文献   

15.
Marfan's syndrome is a hereditary connective tissue disease, in which cardiovascular abnormalities (especially aortic root dilatation) are the most important cause of morbidity and mortality. In this report, we describe two 24-year-old twins, with a history of surgery for lens subluxation and severe cardiovascular manifestations secondary to Marfan's syndrome. One of the twins suffered a type A aortic dissection, which required replacement of the ascending aorta, and the other twin had an aneurysmal dilatation of the ascending aorta (46mm) and was prescribed medical treatment with atenolol and periodic controls to detect the presence of a critical diameter (50mm) that would indicate the need for prophylactic surgery.  相似文献   

16.
Surgical treatment of cardiovascular complications in patients with Marfan's syndrome is usually recommended with apprehension since the systemic nature of the disease predisposes to early and late complications. To define the incidence of these complications, 30 patients were evaluated after surgical treatment of aortic insufficiency and ascending aortic aneurysm at the Texas Heart Institute. To provide a minimal follow-up period of 5 years, only patients operated upon during of before 1968 were included in this series. There were 9 female and 21 male patients aged 4 to 80 years (mean 44 years). Aortic insufficiency was treated by valvuloplasty in 3 patients and by aortic valve replacement in 27. Graft replacement of the ascending aorta was required in 23 patients, and the aneurysm was excised and the aorta repaired by direct anastomosis in 7. Two patients were lost to follow-up study; 12 of the remaining 28 (42.8 percent) lived 5 years or more. The hospital mortality rate was 20 percent (6 of 30); the causes of death included dissection or rupture of the aorta in three patients, congestive heart failure in two and pulmonary embolism in one. The 24 survivors lived from 5 weeks to 9 years. Follow-up data were available on 22 of these patients. Ten of these (45.4 percent) died of late complications. Seven died suddenly, four of these had redissection, one patient had occlusion of the right coronary artery, and two had ventricular fibrillation of no apparent cause. The remaining three died of noncardiac causes. Although the risk of ascending aortic and aortic valve surgery in patients with Marfan's syndrome is high, 42.8 percent of the patients in our series survived 5 years or more. We believe that surgery should be recommended for patients with Marfan's disease who have dissection of the aorta or severe aortic regurgitation, or both.  相似文献   

17.
There are cases of dissecting aortic aneurysm in which thrombotic formation occurs in the false lumen at an early stage, preventing dissection of the vessel wall and enlargement of the aneurysm. We studied such early thrombotic obliteration of false lumina in 12 (28.6%) of 42 patients with dissecting aortic aneurysm who underwent transesophageal two-dimensional echocardiography in the acute phase from June 1986 to October 1989. It was the first employment of a transesophageal approach at our hospital. In this study, we examined the clinical profiles of these patients as well as the usefulness of transesophageal two-dimensional echocardiography in establishing the diagnosis of dissecting aortic aneurysm and characterizing the disease. The patients were classified as Type I (2 patients) and Type III (10 patients; 6 Type IIIa and 4 Type IIIb) according to DeBakey's classification. The minimum and mean intervals from the onset of symptoms to transesophageal two-dimensional echocardiography were 1.5 hours and a mean of 38.2 hours, respectively. These results indicated that thrombotic obliteration of the false lumen had already occurred at a very early stage in some patients. The maximum diameter of the descending aorta was mean 37.6 mm with only 2 patients having that of 40 mm or more. Since left intrapleural hemorrhage was observed in these 2 patients (1 received emergent replacement of the descending aorta), enlargement of the aortic diameter may be indicative of unpredictable outcome, even in patients with early thrombotic obliteration of the false lumen. The long-term clinical course was favorable in all patients, including those who were treated surgically, over a mean follow-up period of 14.5 months.(ABSTRACT TRUNCATED AT 250 WORDS)  相似文献   

18.
目的评价主动脉夹层腔内隔绝术治疗Stanford B型主动脉夹层动脉瘤的院内及长期临床疗效。方法选择行主动脉腔内隔绝术治疗Stanford B型主动脉夹层患者112例。经股动脉切开置入覆膜支架封堵胸主动脉破裂口,置入后冠状动脉造影检查证实疗效,术后随访平均(39±18)个月。分析其临床特点及疗效。结果手术成功112例,共置入覆膜支架119枚。左锁骨下动脉完全被封闭8例,合并严重狭窄病变的冠心病患者完成PCI 16例,主动脉腔内隔绝术后综合征19例,术后1个月内夹层破裂死亡3例。随访3个月,所有患者内膜破裂口封闭,胸降主动脉和腹主动脉真腔扩大,假腔内血栓形成,支架位置、形态正常。术后明显残余内漏10例,3个月自行封闭。术后6个月,再发升主动脉夹层3例,其中行升主动脉外科手术1例,截瘫1例,胃癌多器官转移死亡1例。术后1年迟发性内漏1例。结论腔内隔绝术治疗Stanford B型主动脉夹层动脉瘤的院内及长期疗效满意。  相似文献   

19.
Among 1,049 patients diagnosed with aortic dissection in a multinational registry, patients with Marfan's syndrome were typically younger and had unique presentations. Despite their younger age at presentation, patients with Marfan's syndrome and aortic dissection had a high mortality rate, similar to patients without Marfan's syndrome, an older patient cohort. Our data support the importance of aneurysm surveillance and prophylactic surgical intervention for patients with Marfan's syndrome to potentially reduce the risk of mortality.  相似文献   

20.
BACKGROUND. The value of transesophageal echocardiography in the assessment of patients with aortic dissection was studied. METHODS AND RESULTS. Group 1 (34 patients) represented all patients studied at our institution with this technique in whom aortic dissection was proven by aortography, surgery, or autopsy. Group 2 (27 patients) represented all patients studied with this technique at our institution in whom aortic dissection was excluded by aortography. Transesophageal echocardiography made a correct diagnosis of aortic dissection in 33 of 34 patients (sensitivity, 97%; specificity, 100%). It also correctly demonstrated the type of dissection in all 29 patients with aortographic or surgical proof. On the other hand, computed tomography scanning, performed in 24 of 34 patients in group 1, made a correct diagnosis in only 67% of patients and misclassified the type of dissection in 33%. Transesophageal echocardiography correctly identified involvement of the coronary arteries by aortic dissection in six of seven patients as well as absence of both left and right coronary artery involvement in 10 patients with aortic dissection. This technique was also useful in detecting communications between the true and false lumens, presence of thrombi in the false lumen, and, in two patients, localized dissection rupture with formation of a false aneurysm. In both groups 1 and 2, transesophageal echocardiography correctly identified patients with moderate to severe aortic regurgitation. CONCLUSIONS. Transesophageal echocardiography is very useful in the assessment of aortic dissection.  相似文献   

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