Ann Thorac Surg 2006;82:546-547
© 2006 The Society of Thoracic Surgeons
Original article: Cardiovascular
Invited commentary
Lars Svensson, MD, PhD
Marfan and Connective Tissue Disorder Clinic, The Cleveland Clinic Foundation, 9500 Euclid Ave, F25, Cleveland, OH 44195
(Email: svenssl{at}ccf.org).
Although the overall results are excellent for those patients who have Marfan syndrome with a 99.3% survival rate after surgery, and an 82% 10-year survival rate in our experience, the question of how to best treat that subset of Marfan patients with chronic aortic dissection remains a quandary. Indeed, the 5-year survival of patients who present with both Marfan syndrome and chronic aortic dissection is a sobering 50%, mainly as a result of ruptures, re-dissection, complications of surgery and reoperations as documented in separate studies by Gott and colleagues [1], Smith and colleagues [2] and Svensson and colleagues [3]. For elective cases of the Marfan chronic aortic dissection subset, we recommend doing a two-stage elephant trunk procedure. This approach occurs most often for reoperations in patients who have had previous acute dissection repairs. The other strategies include the clam shell incision for patients with aneurysms limited to the chest (ie, usually proximal descending aorta with a leak or rupture); combined mediastinal and left thoracotomy incision repairs for patients who need the aorta repaired to the celiac artery (ie, for more extensive aneurysms of the descending aorta with leak or rupture of a large aneurysm); a thoracoabdominal incision with right subclavian arterial inflow with hypothermic arrest in symptomatic patients who require repair from just above the sinotubular ridge, or after previous composite valve graft insertion, to the aortic bifurcation; and the rather uncommon approach of a combined mediastinal and thoracoabdominal incision for symptomatic patients with extensive large aneurysms (ie, typically those who have both cardiac disease and thoracoabdominal components that are symptomatic and requiring immediate repair). It should be noted that for chronic dissection we avoid doing endovascular stenting procedures, particularly because Marfan patients are young.
In their article, Hu and colleagues [4] advocate "total" or "subtotal" replacement as the primary procedure for these patients because of the high mortality rates associated with staged procedures. Although we have reported our 30-day survival rate for first stage procedures as 98%, a number of patients did not survive to have the second stage procedure [5], as also documented by Safi and colleagues [6] and Schepens and colleagues [7]. Having been alerted to this problem and applying modifications of our operative technique, such as avoiding prophylactic elephant trunk procedures (ie, only about 6% required a second-stage procedure) and earlier use of stenting after surgery, we were gratified in our most recent study that 85% of our patients are alive 42 months after surgery. In comparison, our operative mortality rate for the combined mediastinal and type I or II thoracoabdominal aneurysm repairs has been 16% with a considerably reduced long-term survival.
Although Hu and colleagues [4] describe excellent results during a 20-month period while performing this combined approach on 13 patients, described by us [8] and Massimo and colleagues [9], it should be noted that, as in the case of the Massimo and colleagues' article [9], "total and subtotal" does not always seem to mean that the entire aorta or even the entire thoracic aorta was replaced during one operation, which is somewhat misleading. Indeed, 4 patients were replaced from the ascending aorta to the diaphragm (ie, the abdominal aorta), and 4 patients had previously undergone Bentall procedures, and if the descending aorta was the symptomatic section, which is not reported in the article, then the patients may have well been candidates for less risky operations. Another caveat is that Hu and colleagues [4] advocate leaving an en-bloc Carrel patch behind for the aortic arch vessels or visceral arteries anastomoses in Marfan patients with aortic dissection, although this is well documented to substantially increase the risk that a large number of these patients will require reoperation for patch ruptures or dilatations. Thus we prefer to do the elephant trunk stage procedure with branch grafts to the greater arch vessels and branch grafts to the visceral arteries in patients with Marfan syndrome and chronic aortic dissection. Therefore we only advocate replacing the entire aorta in a single operation in emergency or urgent surgery patients who have both cardiac and extensive thoracoabdominal pathology. Of note, in a selected subset of patients who had severe comorbid disease, we used endovascular stenting techniques for the second-stage elephant trunk procedure.
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References
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- Gott VL, Cameron DE, Alejo DE, et al. Aortic root replacement in 271 Marfan patientsa 24-year experience. Ann Thorac Surg 2002;73:438-443.[Abstract/Free Full Text]
- Smith JA, Fann JI, Miller DC, et al. Surgical management of aortic dissection in patients with the Marfan syndrome Circulation 1994;90:II235-II242.[Medline]
- Svensson LG, Crawford ES, Coselli JS, Safi HJ, Hess KR. Impact of cardiovascular operation on survival in the Marfan patient Circulation 1989;80:I233-I242.[Medline]
- Hu X-P, Chang Q, Zhu J-M, Yu C-T, Liu Z-G, Sun L-Z. One-stage total or subtotal aortic replacement Ann Thorac Surg 2006;82:542-547.[Abstract/Free Full Text]
- Svensson LG, Kim KH, Blackstone EH, et al. Elephant trunk procedurenewer indications and uses. Ann Thorac Surg 2004;78:109-116.[Abstract/Free Full Text]
- Safi HJ, Miller 3rd CC, Estrera AL, et al. Staged repair of extensive aortic aneurysmslong-term experience with the elephant trunk technique. Ann Surg 2004;240:677-684.[Medline]
- Schepens MA, Dossche KM, Morshuis WJ, van den Barselaar PJ, Heijmen RH, Vermeulen FE. The elephant trunk techniqueoperative results in 100 consecutive patients. Eur J Cardiothorac Surg 2002;21:276-281.[Abstract/Free Full Text]
- Svensson LG, Shahian DM, Davis FG, et al. Replacement of entire aorta from aortic valve to bifurcation during one operation Ann Thorac Surg 1994;58:1164-1166.[Abstract/Free Full Text]
- Massimo CG, Presenti LF, Favi PP, Crisci C, Cruz Guadron EA. Simultaneous total aortic replacement from valve to bifurcationexperience with 21 cases. Ann Thorac Surg 1993;56:1110-1116.[Abstract/Free Full Text]
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