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): Thierry Carrel Friedrich S. Eckstein Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Carrel, T. Articles by Eckstein, F. S. Search for Related Content PubMed PubMed Citation Articles by Carrel, T. Articles by Eckstein, F. S. Related Collections Great vessels Valve disease

Eur J Cardiothorac Surg 2004;25:671-675
© 2004 Elsevier Science NL

Reoperations and late adverse outcome in Marfan patients following cardiovascular surgery

Clinic for Cardiovascular Surgery, University Hospital, CH-3010 Berne, Switzerland

Received 29 October 2003; received in revised form 15 January 2004; accepted 21 January 2004.

^* Corresponding author. Tel.: +41-31-632-2375; fax: +41-31-632-4443
e-mail: thierry.carrel{at}insel.ch

	Abstract

Top Abstract 1. Introduction 2. Patients and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

 
Objectives: Anulo-aortic ectasia represents the most common cardiovascular manifestation requiring surgery in Marfan patients. Aim of this report was to analyze the type of presentation and the incidence of cardiovascular lesions and the clinical follow-up after initial surgery, mainly aortic root repair or replacement. Methods: Between 1/1990 and 6/2003 a total of 71 patients (mean age 29±17 years, 8–65 years) received first surgical treatment at our institution. 69 received root repair or replacement. 22 patients presented with acute aortic dissection (31%), out of them, 3 pregnant females and 1 just after delivery. Composite graft replacement was performed in the majority of patients (61/71, 85%). Aortic valve sparing root repair was performed in 7 patients, supra-coronary graft with refixation of the aortic valve in 2 and replacement of the descending aorta in 1 patient. All patients underwent close clinical and imaging follow-up in a specialized outpatient consultation. Results: During a mean follow-up interval of 5.2±1.8 years, 14 patients (20.5%) underwent a total of 27 aortic reoperations. Seven patients had one and 6 patients had up to 4 reoperations; 13/14 patients had chronic aortic dissection. There was no hospital mortality and no major cardiac or neurologic morbidity following reoperation. During follow-up, 2 patients suffered from acute type B dissection following aortic root surgery and 3 patients surprisingly died: 2 from a rupture of a normal-sized descending aorta and one from intracranial hemorrhage. These 5 patients had had uncomplicated primary aortic root operation. Conclusions: The incidence of reoperations is significantly higher in patients who presented initially with acute type A aortic dissection than in those with dilatation only. In addition, this survey demonstrates that unexpected fatal outcome may appear in the remaining native aorta following uncomplicated elective aortic root surgery, even if the aorta is normal-sized. A close follow-up of all Marfan patients is necessary to detect asymptomatic changes requiring surgery because complex elective redo-operations can be performed with a very low perioperative risk.

Key Words: Marfan syndrome • Aortic root • Reoperations • Outcome

	1. Introduction

Top Abstract 1. Introduction 2. Patients and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

 
Dilated aortic root represents the most common cardiovascular manifestation requiring surgery in Marfan patients. Elective repair or replacement of the aortic root is the treatment of choice for these patients. Following successful aortic root surgery, a substantial number of patients may present various additional cardiovascular pathologies, e.g. aneurysms in the residual native aorta and less frequently mitral valve regurgitation, dilatative cardiomyopathy and more rarely coronary artery disease that requires operative treatment.

Residual or recurrent disease after initial repair, expanding aneurysm in a previously dissected but not resected aortic segment, pseudoaneurysms at anastomotic suture lines as well as ongoing degeneration of the aortic valve leaflets following a valve-sparing root repair represent potential reasons leading to reoperation at the level of the thoracic aorta [1–6]. There is a considerable amount of literature dealing with aortic surgery, but few reports have addressed the special problems of redo-surgery, especially in the context of Marfan syndrome [7–12].

Aim of this report was to analyze the type of presentation and the incidence of cardiovascular lesions and the clinical follow-up after successful initial surgery, mainly aortic root repair or replacement.

	2. Patients and methods

Top Abstract 1. Introduction 2. Patients and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

 
Between 1/1990 and 6/2003 a total 71 patients with Marfan syndrome (mean age 29±17 years, 8–65 years) underwent primary surgery at our institution. 22 patients (31%) presented with acute aortic dissection (21 type A and 1 type B). Three female patients were pregnant at the time of type A acute aortic dissection and one suffered acute type B dissection immediately after delivery. All patients with aortic dissection were operated on an emergency basis [13]. The remaining 49 patients received elective surgery.

Table 1 summarizes the main patients' demographic factors. The diagnosis of Marfan syndrome was established using the Gent nosology and a mutation analysis in occasional cases.

	3. Results

Top Abstract 1. Introduction 2. Patients and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

 
Fourteen patients underwent a total of 27 reoperations. There were 7 male and 7 female patients with a mean age of 37±16 years. Three redo-procedures were performed on the proximal aorta and 22 on the downstream aorta. One patient received mitral valve replacement and one cardiac transplantation. Mean interval between initial surgery and first redo was 5.2±1.8 years but decreased to 2.8±1.1 years when subsequent surgery following first redo was required. Seven patients had one and six patients had up to 4 reoperations; 13/14 patients who required a redo-procedure had chronic aortic dissection. The majority of these (12/14) could be performed on an elective basis. Table 2 presents some details of redo-operations. Interesting findings are demonstrated in Figs. 1 and 2 .

View larger version (140K): [in this window] [in a new window]   Fig. 1. CT scan of a patient who presented a rupture of the aortic patch (black arrow) aimed to reattach the visceral arteries following thoracoabdominal aortic repair. At redo-surgery, separate reimplantation of each visceral artery was performed.

View larger version (90K): [in this window] [in a new window]   Fig. 2. Different morphologies of the aortic arch which required reoperation following replacement of the aortic root with a composite graft. (A) Persistent dissection with expansion of the aortic arch (7 cm) and (B) occlusion of the false lumen with compression of the true lumen leading to a pseudocoarctation with intractable hypertension in the upper extremities.

3.1. Late adverse outcome
During follow-up interval, 2 patients suffered from acute type B dissection 3 and 5 years following uneventful aortic root repair. The intimal tear was classically located below the left subclavian artery and was not considered to have a relationship with the previous surgery. Both patients were treated conservatively. Surprisingly, 2 younger patients (13 and 14 years old) died from rupture of a normal-sized, non-dissected descending aorta, 2 and 3 years following uncomplicated aortic root operation. Both had underwent minor surgery some days before rupture (one had inguinal hernia repair, the other partial excision and laser of a facial naevus flammeus). Arterial hypertension was not present in any of these two patients. A 49-year-old patient died from intracranial hemorrhage 18 months following uncomplicated aortic root replacement with a mechanical composite-graft. At that time he was on warfarin but INR value was in the therapeutic range.

	4. Discussion

Top Abstract 1. Introduction 2. Patients and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

 
Recent reports have demonstrated an incidence of ongoing pathology of the aorta and/or appearance of late surgical complications requiring redo-surgery between 7 and 25%, depending on the type of prior operation, the nature of the disease and the extent of follow-up [1–6,13,14]. In the specific context of Marfan patients, the same incidence varies between 5 and 35% [9–12,15,16].

In the most historical and largest series, Crawford reported a series of more than 700 patients operated on the ascending aorta and aortic arch. Of this, 150 patients had previous cardiac or aortic operation (21%), the causes of reoperations being progression of residual aneurysm, aortic dissection, graft stenosis or infection, false aneurysm, valvular insufficiency and coronary artery disease [4]. Kouchoukos reported the results with 172 aortic root replacement, including 16% of patients who had undergone previous aortic operations. In contrast to the findings of this series dealing with Marfan patients, Kouchoukos observed a rather long interval between first and second operation [17].

It is expected that patients with Marfan syndrome will require multiple interventions on the remaining native aorta. In a long-term observational study, Finkbohner found that the most common pattern of aneurysm repair was proximal ascending aneurysm repair followed by descending aortic surgery. The following variables predicted patients requiring second surgery: acute or chronic dissection at the time of initial surgery, hypertension and a history of smoking. Patients who received prophylactic aortic root repair were less likely to require subsequent aortic surgery [15].

Kazui recently reported on a series of 14 Marfan patients who required late reoperation at a mean interval of 8 years following composite graft replacement of the aortic root. Main problems were aneurysm at the site of coronary artery reimplantation and suture line aneurysms following repair of aortic dissection using the inclusion technique. They concluded that the open coronary artery button technique was the most appropriate way to reattach the coronary arteries to avoid late complications [8]. Similarly to our findings, acute type A aortic dissection was a frequent initial event in patients requiring reoperation. Distal aortic lesions (e.g. at the level of the aortic arch and/or descending aorta) were also frequently observed. To diminish some of the complications encountered, Kazui recommends a separate reattachment of the supra-aortic branches to exclude the maximum of pathologic aortic tissue. We fully agree with this recommendation in Marfan patients but not as a general rule when complete aortic arch repair is required in atherosclerotic aneurysms and aortic dissection.

Pseudoaneurysm at anastomotic suture lines is a rare but serious complication which can occur at any suture line following graft to aorta anastomoses or reattachment of coronary, supra-aortic, intercostal and visceral arteries and branches. This complication was well known following the inclusion technique during composite graft repair [17] and has decreased since open repair has been established. The mechanism of pseudoaneurysm includes most probably excessive tension on the suture line and persistent oozing or bleeding in the perigraft space when the aorta is wrapped around the graft. In the present series, both patients who developed pseudoaneurysms could be treated successfully with endovascular interventional techniques. Long-term observation will show if this less invasive option provides a durable repair.

Aortic valve sparing root repair has provided excellent clinical outcome and relatively few valve-related complications [16,18–20]. The function of the reconstructed aortic root remains unchanged in the majority of patients during early and mid-term follow-up. Reoperation rate is around 4% during a follow-up of approximatively 3 years [21]. In Yacoub's series, results were slightly better in the absence of dissection [18,19]. In the largest series on aortic root replacement reported by Gott, 30 day mortality was 1.5% for elective, 2.7% for urgent repair and 11.7 for emergency surgery [22]: composite graft was performed in the majority of patients in this series.

One of the most interesting question of this study is: can the necessity of redo-surgery be influenced by initial surgical behaviour? We could not eliminate the incidence of redo although we have adopted the following rules: (1) most complete eradication of the pathological tissue during initial surgery, especially at the level of the aortic root, (2) extensive use of separate reattachment of the aortic branches during aortic arch and of the visceral arteries during abdominal aortic replacement [12], (3) open distal anastomosis and hemi-arch repair [23] as minimal requisite during repair of acute aortic dissection, (4) extensive use of the conventional and retrograde elephant trunk to facilitate subsequent operations [24], (5) restrictive use of the graft inclusion technique and (6) aggressive treatment of concomitant pathology, e.g. mitral valve regurgitation during initial.

	5. Conclusions

Top Abstract 1. Introduction 2. Patients and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

 
Results obtained after elective primary surgery in Marfan patients are comparable to those obtained in non-Marfan patients; in the last 100 elective composite graft procedures, mortality was 0% compared to 3.2% (2/61) in Marfan patients. The necessity for reoperations is significantly higher in those patients who presented initially with acute type A aortic dissection. Unexpected fatal outcome may happen in the downstream aorta following uncomplicated elective aortic root surgery despite normal sized aorta. This type of events have not been described before and may be addressed very carefully. Marfan patients who require non-cardiac surgery associated with postoperative pains seem to be at an unforeseenable risk. A close follow-up of all Marfan patients is necessary to detect all potential asymptomatic changes requiring surgery. Complex redo-operations can be performed with a low perioperative risk.

	Footnotes

 
Presented at the joint 17th Annual Meeting of the European Association for Cardio-thoracic Surgery and the 11th Annual Meeting of the European Society of Thoracic Surgeons, Vienna, Austria, October 12–15, 2003.

	Appendix A. Conference discussion

Top Abstract 1. Introduction 2. Patients and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

 
Mr J. Pepper (London, UK): It is a very wide experience you have. I am interested in the individual reattachment of the head vessels. If you have problems that extend into the bifurcation, for example, of the right carotid and the right subclavian, do you prefer to approach that surgically or with a combination of stent and surgery, assuming this is an elective situation?

Dr Eckstein: We would perform a conventional operation with replacement of the diseased vessel. We don't have any experience in endovascular stenting in Marfan patients, so I can't comment on that.

Do you have some recommendation or do you have an experience with stents?

Mr Pepper: A very small experience. When you replace more and more of the aorta with Dacron, the impedance, of course, is enormous. So the energy transfer from the left ventricle goes to the branch vessels off the aorta. So one is likely to have continuing problems. And as it gets more peripheral, we are beginning to use stenting, but we have a very limited experience.

	References

Top Abstract 1. Introduction 2. Patients and methods 3. Results 4. Discussion 5. Conclusions Appendix A. Conference... References

 

1. Kouchoukos N.T., Dougenis D. Surgery of the thoracic aorta. N Engl J Med 1997;336:1876-1888.[Free Full Text]
2. Westaby S., Saito S., Katsumata T. Reoperation after type A dissection repair. J Card Surg 2002;17:28-34.
3. Carrel T., Pasic M., Jenni R., Tkebuchava T., Turina M. Reoperations after operation on the thoracic aorta: etiology, surgical techniques and prevention. Ann Thorac Surg 1993;56:259-269.[Abstract]
4. Crawford E.S., Crawford J.L., Safi H.J., Coselli J.S. Redo operations for recurrent aneurysmal disease of the ascending aorta and the transverse aortic arch. Ann Thorac Surg 1985;40:439-455.[Abstract]
5. Bachet J., Brizard C.H., Goudot B., Dreyfus G., Teodori G., Brodaty D., Dubois C., Delentdecker P., Guilmet D. Repeated surgery for recurrent dissection of the aorta. Eur J Cardiothorac Surg 1990;4:238-244.[Abstract]
6. Schepens M.A., Dossche K.M., Morshuis W.J. Reoperartions on the ascending aorta and aortic root: pitfalls and results in 134 patients. Ann Thorac Surg 1999;68:1676-1680.[Abstract/Free Full Text]
7. Pasic M., von Segesser L., Carrel T., Laske A., Bauer E., Turina M. Surgical treatment of cardiovascular complications in Marfan syndrome: a 27 years experience. Eur J Cardiothorac Surg 1992;6:149-155.[Abstract]
8. Kazui T, Yamashita K, Terada H, Washiyama N, Suzuki T, Ohkura K, Suzuki K. Late reoperations for proximal aortic and arch complications after previous composite graft replacement in Marfan patients. Ann Thorac Surg 2003;76:1203–7.
9. Akashi H., Aoyaga S., Fukunaga S., Tayama K. Long-term results of aortic root replacement for Marfan syndrome. Kyobu Geka 2002;55:628-632.[Medline]
10. Shiiya N., Matsuzaki K., Maruyama R., Kunihara T., Murashita T., Aoki H., Yasuda K. Surgical results for aortic involvement in Marfan syndrome. Kyobu Geka 2002;55:644-649.[Medline]
11. Yamazaki F., Shimamoto M., Fujita S., Nakai M., Aoyama A., Chen F., Nakata T., Yamada T. Surgical treatment of Marfan syndrome: analysis of the patients who required multiple surgical interventions. Kyobu Geka 2002;55:675-678.[Medline]
12. Carrel T., Signer C. Separate revascularization of the visceral arteries in thoraco-abdominal aortic repair. Ann Thorac Surg 1999;68:575-576.[Abstract/Free Full Text]
13. Immer F., Bansi A., Immer-Bansi A., McDougall J., Zehr K., Schaff H.V., Carrel T. Aortic dissection in pregnancy: analysis of risk factors and outcome. Ann Thorac Surg 2003;76:309-314.[Abstract/Free Full Text]
14. Haverich A., Miller D.C., Scott W.C., Mitchell R.E., Oyer P.E., Stinson E.B., Jamieson S.W., Baldwin J.C., Shumway N.E. Acute and chronic aortic dissection—determinants of the long-term outcome for operative survivors. Circulation 1985;72(Suppl 2):22-34.
15. Finkbohner R., Johnston D., Crawford E.S., Coselli J., Milewicz D.M. Marfan syndrome. Long-term survival and complications after aortic aneurysm repair. Circulation 1995;91:728-733.[Abstract/Free Full Text]
16. Tambeur L., David T.E., Unger M., Armstrong S., Ivanov S., Webb G. Results of surgery for aortic root aneurysm in patients with the Marfan syndrome. Eur J Cardiothorac Surg 2000;17:415-419.[Abstract/Free Full Text]
17. Kouchoukos N.T., Wareing T.H., Murphy S.F., Perillo J.B. Sixteen year experience with aortic root replacement: results of 172 operations. Ann Thorac Surg 1991;214:308-320.
18. Birks E.J., Webb C., Radley-Smith R., Yacoub M.H. Early and long-term results of a valve-sparing operation for Marfan syndrome. Circulation 1999;100(Suppl II):II-29-II-35.
19. Yacoub M.H., Gehle P., Chadrasekaran V., Birks E.J., Child A., Radley-Smith R. Late results of a valve-preserving operation in patients with aneurysms of the ascending aorta and root. J Thorac Cardiovasc Surg 1998;115:1080-1090.[Abstract/Free Full Text]
20. David T.E., Armstrong S., Ivanov J., Feindel C.M., Omran A., Webb G. Results of aortic valve-sparing operations. J Thorac Cardiovasc Surg 2001;122:39-46.[Abstract/Free Full Text]
21. Kallenbach K., Karck M., Leyh R., Hagl C., Walles T., Harringer W., Haverich A. Valve-sparing aortic root reconstruction in patients with significant aortic insufficiency. Ann Thorac Surg 2002;74:S1765-S1768.[Abstract/Free Full Text]
22. Gott V.L., Greene P.S., Alejo D.E., Cameron D.E., Naftel D.C., Miller D.C., Gillinov M., Laschinger J.C., Pyeritz R.E. Replacement of the aortic root in patients with Marfan's syndrome. N Engl J Med 1999;340:1307-1313.[Abstract/Free Full Text]
23. Nguyen T., Müller M., Kipfer B., Althaus U., Carrel T. Different techniques of distal aortic repair in acute type A dissection: impact on late aortic morphology and reoperation. Eur J Cardiothorac Surg 1999;15:496-501.
24. Carrel T., Berdat P., Eckstein F., Kipfer B., Schmidli J. The reversed and bidirectional elephant trunk technique for complex thoracic aortic aneurysms. J Thorac Cardiovasc Surg 2001;122:587-591.[Abstract/Free Full Text]

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]

				S. Goland and U. Elkayam Cardiovascular Problems in Pregnant Women With Marfan Syndrome Circulation, February 3, 2009; 119(4): 619 - 623. [Full Text] [PDF]

				E. Girdauskas, T. Kuntze, M. A. Borger, V. Falk, and F. W. Mohr Distal Aortic Reinterventions After Root Surgery in Marfan Patients Ann. Thorac. Surg., December 1, 2008; 86(6): 1815 - 1819. [Abstract] [Full Text] [PDF]

				M. P. Siegenthaler, R. Celik, J. Haberstroh, P. Bajona, H. Goebel, K. Brehm, W. Euringer, and F. Beyersdorf Thoracic endovascular stent grafting inhibits aortic growth: an experimental study. Eur. J. Cardiothorac. Surg., July 1, 2008; 34(1): 17 - 24. [Abstract] [Full Text] [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]

				M. Scharfschwerdt, H.-H. Sievers, J. Greggersen, T. Hanke, and M. Misfeld Prosthetic Replacement of the Ascending Aorta Increases Wall Tension in the Residual Aorta Ann. Thorac. Surg., March 1, 2007; 83(3): 954 - 957. [Abstract] [Full Text] [PDF]

				J. Bachet, F. Larrazet, B. Goudot, G. Dreyfus, T. Folliguet, F. Laborde, and D. Guilmet When Should the Aortic Arch Be Replaced in Marfan Patients? Ann. Thorac. Surg., February 1, 2007; 83(2): S774 - S779. [Abstract] [Full Text] [PDF]

				P M Engelfriet, E Boersma, J G P Tijssen, B J Bouma, and B J M Mulder Beyond the root: dilatation of the distal aorta in Marfan's syndrome Heart, September 1, 2006; 92(9): 1238 - 1243. [Abstract] [Full Text] [PDF]

				S. A. LeMaire, S. A. Carter, I. V. Volguina, A. T. Laux, D. M. Milewicz, G. W. Borsato, C. K. Cheung, J. Bozinovski, J. M. Markesino, W. K. Vaughn, et al. Spectrum of Aortic Operations in 300 Patients With Confirmed or Suspected Marfan Syndrome Ann. Thorac. Surg., June 1, 2006; 81(6): 2063 - 2078. [Abstract] [Full Text] [PDF]

				F. F. Immer, E. Krahenbuhl, U. Hagen, M. Stalder, P. A. Berdat, F. S. Eckstein, J. Schmidli, and T. P. Carrel Large Area of the False Lumen Favors Secondary Dilatation of the Aorta After Acute Type A Aortic Dissection Circulation, August 30, 2005; 112(9_suppl): I-249 - I-252. [Abstract] [Full Text] [PDF]

				F. F. Immer, U. Hagen, P. A. Berdat, F. S. Eckstein, and T. P. Carrel Risk factors for secondary dilatation of the aorta after acute type A aortic dissection Eur. J. Cardiothorac. Surg., April 1, 2005; 27(4): 654 - 657. [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): Thierry Carrel Friedrich S. Eckstein Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Carrel, T. Articles by Eckstein, F. S. Search for Related Content PubMed PubMed Citation Articles by Carrel, T. Articles by Eckstein, F. S. Related Collections Great vessels Valve disease