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Ann Thorac Surg 2008;86:1821-1825. doi:10.1016/j.athoracsur.2008.08.026
© 2008 The Society of Thoracic Surgeons

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Original Articles: Adult Cardiac

Surgery for Marfan Patients With Acute Type A Dissection Using a Stented Elephant Trunk Procedure

LiZhong Sun, MDa,*,*, RuiDong Qi, MDa,*, Qian Chang, MDa, JunMing Zhu, MDa, YongMin Liu, MDa, ChunTao Yu, MDa, HaiTao Zhang, MDa, Bin Lv, MDb, Jun Zheng, MDa, LiangXin Tian, MDa, JinGuo Lu, MDb

a Department of Cardiovascular Surgery, Cardiovascular Institute and Fuwai Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
b Department of Radiology, Cardiovascular Institute and Fuwai Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China

Accepted for publication August 11, 2008.

* Address correspondence to Dr Sun, Department of Cardiovascular Surgery, Cardiovascular Institute and Fuwai Hospital, 167 Beilishi Rd, Beijing, 100037, China (Email: slzh_2005{at}yahoo.com.cn).


    Abstract
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Footnotes
 References
 
Background: The purpose of the study was to assess the efficacy of total arch replacement combined with stented elephant trunk implantation for Marfan patients with acute Stanford type A aortic dissection involving the aortic arch.

Methods: Between January 2004 and April 2006, 13 consecutive Marfan patients (4 female, 9 male) with acute type A aortic dissection involving the aortic arch underwent total arch replacement combined with implantation of a stented elephant trunk. Aortic dissection extending to the iliac artery was seen in 10 patients, and to the abdominal aorta in 3 patients. Ages ranged from 17 to 65 years (mean, 39 ± 13). Computed tomography was done to evaluate the residual false lumen in the descending aorta.

Results: All patients survived and were discharged from hospital. One patient with thrombosis of the innominate artery suffered cerebral infarction and recovered during follow-up. One patient had ischemia of the left upper limb postoperatively, but recovered after axillary to axillary artery bypass. There was 1 death during the mean follow-up period of 27 ± 10 months. Complete thrombus formation was observed in 84.6% of patients (11 of 13) around the stented elephant trunk, and in 69.2% of patients (9 of 13) at the diaphragmatic level.

Conclusions: Total arch replacement combined with stented elephant trunk implantation for Marfan patients with acute type A aortic dissection involving the aortic arch results in less late dilatation of the dissected descending aorta. That prolongs the reoperation interval or reduces the number of late thoracoabdominal aortic replacements, unless there is a patent false lumen around the stented elephant trunk.


    Introduction
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Footnotes
 References
 
Repair of acute type A aortic dissection is challenging. Issues regarding aortic enlargement and late reoperation after repair of acute type A dissection have been raised, particularly for patients with Marfan syndrome [1]. Total replacement of the arch combined with a stented elephant trunk for Stanford type A aortic dissection could cause thrombosis of the false lumen, promote healing of the remaining dissected aorta, and thereby decrease the need for late reoperation [2]. In this study, we retrospectively reviewed our experience of total replacement of the arch combined with implantation of a stented elephant trunk for patients with Marfan syndrome with acute type A aortic dissection involving the aortic arch.


    Patients and Methods
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Footnotes
 References
 
A consecutive series of 13 Marfan patients (4 female and 9 male) aged 17 to 65 years (median, 39 ± 13) with acute Stanford type A aortic dissection involving the aortic arch underwent total arch replacement combined with implantation of a stented elephant trunk in our hospital from January 2004 to April 2006. The technique was approved by the Institutional Review Board of the Chinese Academy of Medical Science and Peking Union Medical College. Informed consent was obtained from each patient before surgery.

The diagnosis of Marfan syndrome was based on revised diagnostic criteria [3]. Surgery was done during the acute stage (within 2 weeks of the onset of acute aortic dissection). Aortic dissection extending to the iliac artery was observed in 10 patients, and to the abdominal aorta in 3 patients. Pericardial effusion was observed in 5 patients, acute left-side heart failure in 1 patient, infective endocarditis in 1, pulmonary infection in 1, chronic nephritis in 1, retina detachment in 1, pleural effusion in 1, thrombosis of the innominate artery in 1, diabetes mellitus in 2 patients, and hypertension in 6. The primary tear was located in the ascending aorta in 12 patients, and in the aortic arch in 1 patient. The primary tear of the aortic dissection was resected in all cases. Clinical profiles of the patients undergoing total arch replacement combined with elephant trunk implantation are listed in Table 1.


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Table 1 Clinical Profiles of Marfan Patients With Acute Type A Aortic Dissection
 
Patients received preoperative computed tomography (CT) with contrast enhancement. Postoperative CT was done routinely to assess the residual false lumen in patients who survived surgery before discharge, 3 or 6 months after surgery, and once each year after discharge. Marfan patients were recommended to use β-blockers for routine medical treatment after surgical discharge, even if they had relatively normal blood pressure.

Surgical Technique
After induction of general anesthesia, arterial blood pressure in the radial artery and femoral artery was measured. The right axillary artery was exposed through a right subclavicular incision. A standard median sternotomy was done in all patients. After systemic heparinization, total cardiopulmonary bypass (CPB) was instituted by cannulating the right axillary artery and the right atrium. Cannulation of the right axillary artery was routinely used for CPB and selective cerebral perfusion (SCP). The arterial line was bifurcated for the right axillary artery and for antegrade perfusion through one limb of a four-branched prosthetic graft. The surgical technique has previously been described in detail by our research group [2].

After establishing CPB, cooling was initiated and CPB flow was maintained between 2.2 L · min–1 · m–2 and 2.4 L · min–1 · m–2. Brachiocephalic arteries were dissociated from surrounding tissue during the cooling period and exposed. After cross-clamping the ascending aorta, aortic root procedures were done (if necessary). Myocardial protection was based on intermittent antegrade perfusion of cold-blood cardioplegic solution. Circulatory arrest was established when the nasopharyngeal temperature reached 18° to 22°C. Selective cerebral perfusion was started through the right axillary artery, and the brain was perfused at approximately 5 to 10 mL · kg–1 · min–1.

Ascending aorta, aortic arch, and proximal descending thoracic aorta were opened. The intimal tear was resected if it was located in the ascending aorta, in the transverse arch, or in the proximal descending thoracic aorta. The descending aorta was transected below the origin of the left subclavian artery. A catheter sheath containing a stent graft (MicroPort Medical [Shanghai] Co, Ltd, China) of 10 cm length was inserted into the true lumen of the descending thoracic aorta in a bound, compressed state (Fig 1). In patients with acute type A dissection, appropriate sizing of the stented elephant trunk was close to the diameter of the proximal descending aorta of normal persons matched for age, sex, and height. The diameter of the stent graft was larger than that of the true lumen, and was smaller than that of the entire aorta as determined by preoperative CT and intraoperative judgment.


Figure 1
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Fig 1. Stented graft. (A) Catheter sheath containing the stented graft in a bound and compressed state. (B) Stent graft springs open and expands completely.

 
Great care must be taken not to create a new intimal tear when implanting the stent graft owing to the weakness and fragility of the aortic wall in Marfan patients with acute aortic dissection. The stent graft must not be expanded, particularly in the Marfan patient with a small true lumen of the descending aorta. After implantation into the correct location, one hand gripped the shaft (handle) to which the surgical stent graft was attached; the other hand pulled the draw ring, which in turn released the self-expanding stent. The shaft, draw ring, and wire were removed after the stent "sprang open" and expanded. The surgical stent graft was distended to match the descending aorta. The proximal edge of the residual descending aorta was trimmed to match the proximal end of the stent graft. The graft was fixed in the true lumen of the descending thoracic aorta by the self-expanding stent to make the true lumen expandable. The distal end of the four-branched prosthetic graft was anastomosed to the descending aorta containing the intraluminal stented graft. Air was eliminated from the descending aorta when the anastomoses were completed. Blood perfusion of the lower body was started through the perfusion limb of the four-branched prosthetic graft. One limb of the prosthetic graft was then anastomosed to the left common carotid artery in an end-to-end fashion. After the anastomosis was accomplished, SCP was discontinued, CPB gradually resumed to normal flow, and rewarming started. Anastomosis to the left subclavian artery and the innominate artery was done in the same fashion. The proximal end of the prosthetic graft was then anastomosed to the ascending aorta.


    Results
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Footnotes
 References
 
Surgical Data
Patients underwent total replacement of the arch combined with stented elephant trunk implantation using hypothermic SCP. Cardiopulmonary bypass time was 140 to 261 minutes (mean, 189 ± 44); aortic cross-clamp time was 85 to 176 minutes (mean, 129 ± 30); and SCP time was 17 to 75 minutes (mean, 31 ± 16).

Concomitant procedures, as summarized in Table 1, were Bentall procedure in 10 patients; aortic valve replacement with ascending aorta replacement in 1 patient; aortic valve repair with ascending aorta replacement in 1 patient; and Bentall procedure with coronary artery bypass grafting in 1 patient.

Morbidity and Mortality
In-hospital death was not observed. Cerebral infarction occurred in 1 male patient who had thrombosis of the innominate artery after surgery, but he recovered during follow-up. Left upper limb ischemia caused by ligation of the left subclavian artery was noted in 1 male patient; he recovered after axillary to axillary artery bypass. Paraplegia was not observed in any patient.

Imaging
Patients underwent CT during follow-up. Complete formation of a thrombus around the stented elephant trunk was observed in 11 of 13 patients (84.6%). Patent false lumen around the stented elephant trunk was observed in 2 of 13 patients (15.4%). The descending aorta returned to normal in 7 of 11 patients (63.6%; Fig 2). Complete formation of a thrombus extending to the level of the diaphragm was observed in 9 of 13 patients (69.2%: Fig 2). Diameter of the descending aorta distal to the stented graft decreased in 7 patients and increased in 3 patients. The preoperative false lumen of the descending aorta was 2.17 ± 0.21 cm. Compared with preoperative data, the diameter of the descending aorta was 3.04 ± 0.25 cm (versus 3.42 ± 0.24 cm, except for 2 patients with a patent false lumen). We observed an increase in the diameter of the abdominal aorta at the level of the superior mesenteric artery in patients with thrombus formation around the stented elephant trunk, except in 1 patient. Compared with preoperative data, mean enlargement of the abdominal aorta was only 0.387 cm per year at the level of the superior mesenteric artery.


Figure 2
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Fig 2. Computed tomography scans of a patient with acute type A aortic dissection involving the aortic arch 2 weeks (A, B, and C) and 30 months (D, E, and F) after surgery. The true lumen in the descending aorta was resumed (A versus D, B versus E, and C versus F). The stented graft expanded to its full diameter (A versus D), and the aorta wall had been remodeled to a normal shape owing to thrombus absorption (A versus D, B versus E). The false lumen was obliterated with thrombus at the diaphragmatic level (C versus F).

 
Follow-Up
All patients completed follow-up, which was 14 to 46 months (mean, 27 ± 10). One patient with axillary to axillary artery bypass died 14 months after surgery from an unknown cause. Severe complications during follow-up were not observed.


    Comment
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Footnotes
 References
 
Surgical morbidity and mortality in patients with acute Stanford type A aortic dissection has decreased owing to improvements in surgical technique, anesthesia, and perioperative care. Total replacement of the arch has been recommended for patients with acute type A aortic dissection involving the aortic arch [4–7], but the dissected descending aorta was untreated. The distal false lumen remains patent in most patients with acute type A aortic dissection after total arch replacement [4, 6, 8]. Late reoperation for dilatation of a patent false lumen was required [9]. The elephant trunk procedure was introduced by Borst and colleagues [10] to facilitate staged aortic replacement, but many patients fail to return for the second operation because of aneurysm rupture. Kato and associates [11] developed the stented elephant trunk technique for repair of acute type A aortic dissection. Some modifications of the stented elephant trunk technique were made by our research group, and the initial results were encouraging [2].

Marfan patients had a significantly higher reoperation rate than non-–Marfan patients (38.8% versus 13%) [12]. The weakened abnormal aortic tissue in patients with Marfan syndrome was the primary cause of multiple surgical reconstructions. The outcome of the initial procedure influences the likelihood of late reoperation [12]. The rate of reoperation was significantly higher among patients with residual dissection than among those without residual dissection [13]. Total replacement of the arch was recommended for Marfan patients with acute type A aortic dissection involving the aortic arch during the initial procedure [14]. Significant differences in the rate of reoperation were not found between Marfan patients with and without concomitant total arch replacement. The cause may be correlated with the persistent patent false lumen in the dissected descending aorta [13]. Subsequent thoracoabdominal replacement was needed for most Marfan patients with acute type A aortic dissection involving the aortic arch after initial replacement of the ascending aorta and aortic arch [1]. Surgery for late thoracoabdominal aneurismal dilatation still continued to carry considerable mortality and morbidity. Surgeons were confronted with the increased risk of the late reoperation.

We wondered if we could reduce the likelihood of late reoperation or prolong the reoperation interval by inducing thrombosis of the false lumen in the dissected descending aorta. The stent elephant trunk was implanted in the descending aorta during total arch replacement. Satisfactory results were obtained. Complete formation of a thrombus of the false lumen in the descending aorta was observed in 84.6% of Marfan patients. Postoperative CT showed that the descending aorta had returned to a normal level in most Marfan patients. No patient required reoperation during follow-up. These data demonstrated that this technique could prolong the reoperation interval or reduce the likelihood of late reoperation. This technique would make it safer and easier to carry out the anastomosis between the distal end of the stent graft and Dacron prosthesis if late thoracoabdominal aortic replacement was required.

A patent false lumen around the stented elephant trunk was observed in 2 patients. In contrast to non-Marfan patients, Marfan patients had a high prevalence of patent false lumen using this technique during the same period (15.4% versus 1.9%; unpublished data). Thrombus obliteration of the false lumen at the level of the diaphragm was lower in Marfan patients than it was in non-Marfan patients (69.2% versus 84.6%; unpublished data). Using postoperative CT, patients with Marfan syndrome showed lower thrombus absorption of the false lumen in the descending aorta compared with non-Marfan patients (53.8% versus 86.5%, unpublished data). Weakness and fragility of the aortic wall may disturb thrombotic closure of the false lumen in the descending aorta, and may be the primary cause of continued false lumen patency in Marfan patients with this technique. Major reentry into the descending aorta may be associated with continued false lumen patency. There was residual dissection in the abdominal aorta, but mean enlargement of the abdominal aorta was only 0.387cm per year at the superior mesenteric artery level during follow-up.

There is greater debate over the management of aortic dissection in Marfan patients using the stent graft. Fixation zones for endografts are prone to future dilatation and endoleaks due to weakness and fragility of the aortic wall in patients with Marfan syndrome. Endovascular stent graft treatment is not recommended for Marfan patients. Endovascular stent graft treatment of Marfan patients with aortic dissection was not done in our center.

We tried to carry out total arch replacement or proximal descending aorta replacement combined with stented elephant trunk implantation in Marfan patients. There are several advantages of this technique over endovascular stent graft treatment. Firstly, the stented elephant trunk in the descending aorta was fixed to the distal end of the four-branched prosthetic graft using the suture line. The stent renders the sutures more solidly between the stented elephant trunk and the aortic wall, decreasing the risk of dangerous endoleaks. Secondly, the diameter of the stented elephant trunk was close to the diameter of the proximal descending aorta of normal persons matched for age, sex, and height. The radial force exerted by the stented elephant trunk was relatively low, and injury to the aortic wall caused by the stented elephant trunk was small. Thirdly, surgery was done under deep hypothermic circulatory arrest with SCP; that avoided injury to the aortic wall due to endografting under aortic pulsation. Attachment to the aortic wall of the endograft is achieved by the radial force of the stent graft in patients undergoing endovascular stent graft treatment. It increases injury to the aortic wall. Endoleaks occurred because of the dilatation of fixation zones for endografts. Recommendations for use of the stent graft state a maximal oversizing of 10% to 15% compared with fixation zones for endografts. The larger the size of the stent graft, the stronger the radial force it gives to the fragile aortic wall; that increases injury to the aortic wall in Marfan patients.

The primary limitation of this study was that comparisons between the total arch replacement group and the total arch replacement with stented elephant trunk group were not made. The number of patients was small, and the data preliminary. Our experience of this technique for Marfan patients was limited, with a mean follow-up of 27 ± 10 months. The effectiveness of this technique must be validated with longer follow-up.

In conclusion, we demonstrated the effectiveness of this technique for Marfan patients with acute type A aortic dissection involving the aortic arch. Thrombus formation in the false lumen of the dissected descending aorta was obtained in most Marfan patients.

Total arch replacement combined with stented elephant trunk implantation (1) results in less late dilatation of the dissected descending aorta; (2) prolongs the reoperation interval or reduces the number of late thoracoabdominal aortic replacements except in a patent false lumen around the stented elephant trunk; (3) facilitates late thoracoabdominal aortic replacement; and (4) thereby decreases the associated mortality and morbidity.

Addendum
From April 2006 to July 2007, 4 Marfan patients with acute type A dissection underwent the procedure described in this paper. Complete thrombus formation extending to the level of the diaphragm was observed in 2 patients. The distal end of the stent graft entering the false lumen was observed in the other 2 patients. Preoperative CT showed a very small true lumen of the descending aorta, which was subtotally compressed by a large false lumen. Owing to weakness and fragility of the aortic wall, continuous compression of the stented graft resulted in disruption of the intimal layer after the stent graft was sprung open and expanded in the small true lumen of the descending aorta. The distal end of the stent graft entered the false lumen. We think that the small true lumen of the descending aorta, abnormal fragile tissue, and an inexperienced surgeon were the main causes of this complication in Marfan patients with acute type A dissection. To avoid this severe complication, we recommend this technique be done in Marfan patients with the true lumen of the descending aorta greater than 10 mm in diameter.


    Footnotes
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Footnotes
 References
 
* Drs Sun and Qi contributed equally to this work. Back


    References
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Footnotes
 References
 

  1. Halstead JC, Meier M, Etz C, et al. The fate of the distal aorta after repair of acute type A aortic dissection J Thorac Cardiovasc Surg 2007;133:127-135.[Abstract/Free Full Text]
  2. Liu ZG, Sun LZ, Chang Q, et al. Should the "elephant trunk" be skeletonized?. Total arch replacement combined with stented elephant trunk implantation for Stanford type A aortic dissection. J Thorac Cardiovasc Surg 2006;131:107-113.[Abstract/Free Full Text]
  3. De Paepe A, Devereux RB, Dietz HC, Hennekam RC, Pyeritz RE. Revised diagnostic criteria for the Marfan syndrome Am J Med Genet 1996;62:417-426.[Medline]
  4. Takahara Y, Sudo Y, Mogi K, Nakayama M, Sakurai M. Total aortic arch grafting for acute type A dissection: analysis of residual false lumen Ann Thorac Surg 2002;73:450-454.[Abstract/Free Full Text]
  5. Tan ME, Dossche KM, Morshuis WJ, Kelder JC, Waanders FG, Schepens MA. Is extended arch replacement for acute type A aortic dissection an additional risk factor for mortality? Ann Thorac Surg 2003;76:1209-1214.[Abstract/Free Full Text]
  6. Ochiai Y, Imoto Y, Sakamoto M, et al. Long-term effectiveness of total arch replacement for type A aortic dissection Ann Thorac Surg 2005;80:1297-1302.[Abstract/Free Full Text]
  7. Hirotani T, Kameda T, Kumamoto T, Shirota S. Results of a total aortic arch replacement for an acute aortic arch dissection J Thorac Cardiovasc Surg 2000;120:686-691.[Abstract/Free Full Text]
  8. Hirotani T, Nakamichi T, Munakata M, Takeuchi S. Routine extended graft replacement for an acute type A aortic dissection and the patency of the residual false channel Ann Thorac Surg 2003;76:1957-1961.[Abstract/Free Full Text]
  9. Zierer A, Voeller RK, Hill KE, Kouchoukos NT, Damiano RJ, Moon MR. Aortic enlargement and late reoperation after repair of acute type A aortic dissection Ann Thorac Surg 2007;84:479-487.[Abstract/Free Full Text]
  10. Borst HG, Walterbusch G, Schaps D. Extensive aortic replacement using "elephant trunk" prosthesis Thorac Cardiovasc Surg 1983;31:37-40.[Medline]
  11. Kato M, Ohnishi K, Kaneko M, et al. New graft-implanting method for thoracic aortic aneurysm or dissection with a stented graft Circulation 1996;94:II188-II193.[Medline]
  12. Bachet JE, Termignon JL, Dreyfus G, et al. Aortic dissection. Prevalence, cause, and results of late reoperations. J Thorac Cardiovasc Surg 1994;108:199-206.[Abstract/Free Full Text]
  13. Tagusari O, Ogino H, Kobayashi J, et al. Should the transverse aortic arch be replaced simultaneously with aortic root replacement for annuloaortic ectasia in Marfan syndrome? J Thorac Cardiovasc Surg 2004;127:1373-1380.[Abstract/Free Full Text]
  14. Bachet J, Larrazet F, Goudot B, et al. When should the aortic arch be replaced in Marfan patients? Ann Thorac Surg 2007;83(Suppl):774-779.

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