HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Shigeyuki Aomi Akimasa Hashimoto Hitoshi Koyanagi Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Niinami, H. Articles by Koyanagi, H. Search for Related Content PubMed PubMed Citation Articles by Niinami, H. Articles by Koyanagi, H.

Ann Thorac Surg 1999;67:1864-1867
© 1999 The Society of Thoracic Surgeons

Extensive aortic reconstruction for aortic aneurysms in Marfan syndrome

^a Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women’s Medical College, Tokyo, Japan

Address reprint requests to Dr Niinami, Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women’s Medical College, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, Japan

Presented at the Aortic Surgery Symposium VI, April 30–May 1, 1998, New York, NY.

	Abstract

Top Abstract Introduction Patients and methods Results Comment References

 
Background. Marfan syndrome patients frequently develop aneurysms or dissections involving multiple segments of the aorta, and occasionally require staged replacement of the entire aorta. This study reviews the surgical outcome of patients with Marfan syndrome who underwent extensive aortic reconstruction. Extensive reconstruction is defined as reconstruction of more than two segments of the ascending, arch, descending thoracic, or abdominal aorta.

Methods. From March 1973 to December 1997, 101 patients with Marfan syndrome underwent aortic operation. Twenty-six patients (25.7%) had extensive aortic reconstruction. All 26 patients suffered from aortic dissection: 13 patients had Stanford type A and 13 had type B dissection. Twenty-three patients (88.4%) had annuloaortic ectasia and aortic regurgitation. Surgical procedures included composite valve graft replacement (n = 23, 88.4%), aortic arch reconstruction (n = 15, 57.7%), graft replacement of the descending thoracic aorta (n = 6, 23.1%), and graft replacement of the thoracoabdominal aorta (n = 16, 61.5%). Five patients (19.2%) had total thoracoabdominal aortic replacement, and three patients (11.5%) had replacement of the entire aorta. Twenty-one patients (80.8%) required multiple operations.

Results. Follow-up was complete in all patients. The 30-day survival rate was 88.5%. None of the survivors had paraplegia or paraparesis. The overall long-term survival rate was 88.5 ± 6% at 1 year, and 81.7 ± 9% at 9 years.

Conclusions. Aortic surgery prolongs survival in patients with Marfan syndrome, and currently there is a relatively low associated morbidity and mortality even for aggressive surgical treatment.

	Introduction

Top Abstract Introduction Patients and methods Results Comment References

 
Marfan syndrome is an autosomal dominant disorder of connective tissue characterized by abnormalities involving the skeletal, ocular, and cardiovascular systems [1]. The cardiovascular features typically include progressive dilatation of the proximal aorta leading to aortic dissection or rupture. In addition, the dilatation of the aortic annulus can cause aortic valve incompetence and regurgitation. The cardiovascular complications of the syndrome lead to a reduced life expectancy for affected individuals if left untreated [2]. With improvements in operative techniques, anesthetic care, and perioperative management, surgical repair of aortic aneurysms in patients with Marfan syndrome can be carried out with minimal early and late mortality [3, 4]. Marfan patients also frequently develop aneurysms or dissections involving multiple segments of the aorta, and occasionally require staged replacement of the entire aorta [3].

Complications arising from thoracoabdominal aortic replacement are a primary cause of late mortality in patients with aortic dissection. In particular, thoracoabdominal aortic replacement in patients with Marfan syndrome is challenging due to weakened aortic tissue and the predominance of extensive aneurysms and associated dissections. However, recent data suggest that current techniques for neurological protection have led to a reduced incidence of paraplegia and paraparesis [5, 6]. We retrospectively reviewed our experience with 26 consecutive extensive aortic reconstructions for aortic aneurysms in Marfan patients in order to evaluate the results of surgery using contemporary methods.

	Patients and methods

Top Abstract Introduction Patients and methods Results Comment References

 
From March 1973 to December 1997, 101 patients with Marfan syndrome, diagnosed according to published guidelines, underwent cardiovascular operations at the Heart Institute of Japan [7]. Twenty-six patients (25.7%) had extensive aortic reconstruction. In this study, extensive aortic reconstruction refers to reconstruction of more than two segments of the ascending aorta, aortic arch, descending thoracic aorta, or abdominal aorta; we excluded infrarenal abdominal aortic aneurysm repair. Sixteen patients (61.5%) were men and 10 patients were women. The ages ranged from 23 to 59 years (mean 39.3 ± 12.1).

Twenty-three patients (88.4%) had annuloaortic ectasia and aortic regurgitation. All 26 patients suffered from aortic dissection: 13 patients (50%) had Stanford type A, and 13 patients type B dissection. Indications for graft replacement of the thoracoabdominal aortic segment included impending rupture, ischemic complications related to dissection, or an aortic diameter measuring more than 5.5 cm as determined by computed tomography or magnetic resonance imaging. No operation was performed for acute dissection. Extensive thoracoabdominal aortic aneurysms (Crawford type I and type II) were present in most of the patients (96.2%). One patient (3.8%) with type B dissection presented with rupture.

Operative techniques
Surgical techniques used to repair aortic aneurysms have previously been described in detail [8, 9]. Table 1 summarizes all procedures performed in this group of patients. All patients undergoing surgical treatment before 1990 for aortic valvular insufficiency or aortic root aneurysm underwent composite valve graft replacement using the original Bentall technique. Since 1990, coronary arteries have been reanastomosed using a transversely placed small Dacron tube graft in order to achieve tension-free reattachment. Reconstruction of the aortic arch was performed along with composite graft replacement in 12 patients. In patients with total arch replacement (n = 11), retrograde cerebral perfusion via the superior cava was used as an adjunct for brain protection requiring profound hypothermic circulatory arrest (18°C to 20°C). In patients with major abdominal branches perfused from the false lumen of the aortic dissection, a fenestration technique or a double-barrel repair was utilized at the time of distal anastomosis of the aortic arch reconstruction. Since 1990, we have been using the elephant trunk technique for a staged operation [10].

Follow-up and statistical analysis
Current follow-up data were available in all, yielding a total of 80.3 patient-years for analysis. The follow-up interval in the 23 operative survivors was from 1 month to 10 years, with a mean of 3.1 years. The information on all patients was confirmed either by contacting the patients in our outpatient clinic, or by telephone in January 1998. No patients were lost during follow-up.

Any death within 30 days after operation for extensive aortic reconstruction was considered an early death. Postoperative survival was analyzed by the Kaplan-Meier actuarial method. Noted values are mean ± standard deviation.

	Results

Top Abstract Introduction Patients and methods Results Comment References

 
One or more subsequent cardiovascular operations were required in 21 patients (80.7%); two patients (7.7%) had a third operation. The indications for subsequent operations were late aortic sequelae (defined as aneurysmal dilatation or recurrent dissection distant from the initial repair) associated with the natural evolution of aortic pathology in patients with Marfan syndrome. Recently, we have scheduled subsequent procedures as a part of a staged operation (n = 7). The mean interval between the initial and second operation was 4.3 ± 5.0 years; it was 2.1 ± 2.2 years in patients with staged operations. In 2 patients who had third operations, both staged, the duration between the second and third procedures was 1 month and 3 years.

Twenty-three patients (88.4%) had aortic root replacement: an original Bentall procedure in 9 patients, and a modified Bentall in 14; all had mechanical prostheses. One patient developed pseudoaneurysm formation after an original Bentall operation. Among the survivors, there were no episodes of structural valve deterioration, prosthetic valve endocarditis, or valve thrombosis. No patient had episodes of thromboembolism, or anticoagulant-related hemorrhagic complications. Aortic arch reconstruction was performed in 15 patients (57.7%); total arch replacement in 11 patients, and hemiarch repair in 4 patients. In all 11 patients who had total aortic arch replacement, retrograde cerebral perfusion with profound hypothermic circulatory arrest was carried out. In 5 of 11 patients who had total arch replacement, the elephant trunk technique was utilized. In 3 patients with total arch replacement, a fenestration was created for the perfusion of the false lumen. Six patients (23.1%) had graft replacement of the descending thoracic aorta, and 16 patients (61.5%) had graft replacement of the thoracoabdominal aorta. Five patients (19.2%) had total thoracoabdominal aortic replacement, and 3 patients (11.5%) had replacement of the entire aorta.

The early mortality in this study was 11.5% (3/26 patients). The causes of death were cardiac failure, pulmonary bleeding, and brain hemorrhage. Renal failure requiring hemodialysis occurred in 1 patient (3.8%). All three deaths, and one case of renal failure, occurred in patients who had undergone Crawford type II thoracoabdominal aneurysm repairs. Of those who died, 2 patients had total thoracoabdominal aortic replacement with left heart bypass, and 1 had total aortic replacement supported by CPB with normothermia for the reconstruction of a thoracoabdominal aortic aneurysm. In 22 patients who had thoracic or thoracoabdominal aneurysm repair, the average number of reconstructed intercostal arteries was 2.4 ± 2.3 (0 to 8). None of the 23 survivors had paraplegia or paraparesis.

One patient (3.8%) died during the interval of follow-up. This patient was diagnosed as a type B dissection and had total thoracoabdominal aortic replacement. The cause of death was rupture of the ascending aorta. The overall long-term survival rate, including early mortality, was 88.5 ± 6% at 1 year, and 81.7 ± 9% at 9 years (Fig 1).

View larger version (14K): [in this window] [in a new window]   Fig 1. Actuarial survival for all patients, including early deaths in the hospital. The numbers in parentheses represent the numbers of patients at risk at each interval.

	Comment

Top Abstract Introduction Patients and methods Results Comment References

For the reconstruction of the aortic arch in patients with type A dissection, we have been using retrograde cerebral perfusion with profound hypothermic circulatory arrest since 1990 [8]. Compared with antegrade cerebral perfusion, retrograde cerebral perfusion without cannulation of aortic arch branches provides an excellent operative view and shortens the duration of arch repair. In all patients with Marfan syndrome who require total arch replacement, we use a graft with three side branches for reconstruction of the neck vessels in order to exclude as much aortic tissue as possible.

Our approach to Marfan syndrome patients with thoracoabdominal aortic aneurysm due to aortic dissection is aggressive because of the risks of progressive aortic dilatation, further dissection, and possible rupture. In this study, 22 of 26 patients had descending thoracic or thoracoabdominal aortic graft replacement (84.6%). Eight patients in this group (36.4%) had total thoracoabdominal aortic replacement, including 3 patients who had replacement of the entire aorta.

Paraplegia remains one of the most devastating complications after graft replacement of the descending thoracic or thoracoabdominal aorta [13]. In this series, no patients had paraplegia or paraparesis. Although our previous data suggested a relatively low incidence of mortality and morbidity for the surgical treatment of type B dissection using left heart bypass, this technique requires the use of sequential aortic clamping to minimize the duration of spinal cord ischemia, and this will not be possible in all patients [9, 14]. We have recently introduced profoundly hypothermic CPB with or without a period of circulatory arrest in order to achieve effective protection of the brain, spinal cord, kidneys, and the abdominal viscera. This technique is indicated in the presence of an unsafe proximal aorta, and when extensive thoracic or thoracoabdominal aortic disease is present and the risk of developing paraplegia is judged to be increased. In particular, patients with Marfan syndrome have a weakened aortic wall, so ideally the proximal aorta should not be clamped in cases of type B dissection. Also, this technique makes surgery easier and faster because of better operative visibility and less tissue mobilization. In this study, three cases of early mortality occurred mainly because of relatively long operating times due to intraoperative bleeding. Although the number of patients in whom profound hypothermic CPB was used is relatively small, there has been no mortality since introduction of this technique.

The goal for the treatment of aortic disease in Marfan patients may be replacement of the entire aorta. Svensson et al reported that untreated valvular disease, aneurysms, and pseudoaneurysms caused 39.3% of the late deaths after aortic surgery in patients with Marfan syndrome [15]. In this series, we had one late death, in a patient who had total thoracoabdominal replacement after type B dissection: theoretically, the whole of the diseased portion of the aorta was replaced, but a new aneurysm of the ascending aorta developed, and the patient died of rupture. New lesions must be searched for and treated aggressively because Marfan syndrome is not just a disease of the proximal aorta but rather affects the entire aorta. It is recommended that patients with Marfan syndrome undergo prophylactic aortic graft replacement when the aortic diameter exceeds 5.5 cm, because the probability of aortic dissection or rupture increases with greater diameters [11]. Our current strategy for patients with Marfan syndrome is an elective staged operation to achieve total thoracoabdominal replacement or entire aortic replacement.

In this series, we demonstrated the ability to prolong survival in patients with Marfan syndrome through aortic operations with a low associated mortality and morbidity. This supports the aggressive use of elective aortic surgery. From the variety and multiplicity of aortic procedures undertaken in most of our patients, we have shown the frequent need for multiple aortic operations, and have confirmed the necessity for lifelong cardiovascular surveillance.

	References

Top Abstract Introduction Patients and methods Results Comment References

 

1. Pyeritz R.E., McKusick V.A. The Marfan syndrome: diagnosis and management. N Engl J Med 1979;300:772-777.[Medline]
2. Murdoch J.L., Walker B.A., Halpern B.L., et al. Life expectancy and causes of death in Marfan syndrome. N Engl J Med 1972;286:804-808.
3. Crawford E.S. Marfan’s syndrome: broad spectral surgical treatment cardiovascular manifestations. Ann Surg 1983;198:487-505.[Medline]
4. Gott V.L., Pyeritz R.E., Cameron D.E., et al. Composite graft repair of Marfan aneurysm of the ascending aorta: results in 100 patients. Ann Thorac Surg 1991;52:38-45.[Abstract]
5. Coselli J.S., LeMaire S.A., Poli de Figueiredo L., et al. Paraplegia after thoracoabdominal aortic aneurysm repair: is dissection a risk factor?. Ann Thorac Surg 1997;63:28-36.[Abstract/Free Full Text]
6. Kouchoukos N.T., Daily B.B., Rokkas C.K., et al. Hypothermic bypass and circulatory arrest for operations on the descending thoracic and thoracoabdominal aorta. Ann Thorac Surg 1995;60:67-77.[Abstract/Free Full Text]
7. Beighton P., de Paepe A., Danks D., et al. International nosology of heritable disorders of connective tissue, Berlin, 1986. Am J Med Genet 1988;29:581-594.[Medline]
8. Kitamura M., Hashimoto A., Akimoto T., et al. Operation for type A aortic dissection: introduction of retrograde cerebral perfusion. Ann Thorac Surg 1995;59:1195-1199.[Abstract/Free Full Text]
9. Kitamura M., Hashimoto A., Tagusari O., et al. Operation for type B aortic dissection: introduction of left heart bypass. Ann Thorac Surg 1995;59:1200-1203.[Abstract/Free Full Text]
10. Borst H.G., Walterbusch G., Schaps D. Extensive aortic replacement using "elephant trunk" prosthesis. Thorac Cardiovasc Surg 1983;31:37-40.[Medline]
11. Coselli J.S., LeMaire S.A., Buket S. Marfan syndrome: the variability and outcome of operative management. J Vasc Surg 1995;21:432-443.[Medline]
12. Coselli J.S., LeMaire S.A. Current status of thoracoabdominal aortic aneurysm repair in Marfan syndrome. J Card Surg 1997;12:167-172.[Medline]
13. Svensson L.G., Crawford J.L., Hess K.R., et al. Experience with 1509 patients undergoing thoracoabdominal aortic operations. J Vasc Surg 1993;17:357-370.[Medline]
14. Coselli J.S. Thoracoabdominal aortic aneurysms: experience with 372 patients. J Card Surg 1994;9:638-647.[Medline]
15. Svensson L.G., Crawford E.S., Coselli J.S., et al. Impact of cardiovascular operation on survival in the Marfan patient. Circulation 1989;80(Suppl):233-242.

This article has been cited by other articles:

				L. Botta, V. Russo, C. La Palombara, M. Rosati, R. Di Bartolomeo, and R. Fattori Stent graft repair of descending aortic dissection in patients with Marfan syndrome: An effective alternative to open reoperation? J. Thorac. Cardiovasc. Surg., November 1, 2009; 138(5): 1108 - 1114. [Abstract] [Full Text] [PDF]

				D. G. Cooper, S. Markur, S. R. Walsh, C. Cousins, P. D. Hayes, and J. R. Boyle Hybrid Endovascular Repair of an Aneurysmal Chronic Type B Dissection in a Patient with Marfan Syndrome With an Aberrant Right Subclavian Artery Vascular and Endovascular Surgery, June 1, 2009; 43(3): 271 - 276. [Abstract] [PDF]

				M. S. Kalkat, I. Rahman, K. Kotidis, B. Davies, and R. S. Bonser Presentation and outcome of Marfan's syndrome patients with dissection and thoraco-abdominal aortic aneurysm Eur. J. Cardiothorac. Surg., August 1, 2007; 32(2): 250 - 254. [Abstract] [Full Text] [PDF]

				K. Kirali, V. Erentug, M. B Rabus, A. Izgi, N. U Bozbuga, K. Erkanli, E. Akinci, and C. Yakut Extensive Aortic Surgery in Marfan Syndrome: 16-Year Experience Asian Cardiovasc Thorac Ann, December 1, 2003; 11(4): 337 - 341. [Abstract] [Full Text] [PDF]

This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Shigeyuki Aomi Akimasa Hashimoto Hitoshi Koyanagi Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Niinami, H. Articles by Koyanagi, H. Search for Related Content PubMed PubMed Citation Articles by Niinami, H. Articles by Koyanagi, H.