Editorial Comment: Total aortic arch replacement in Marfan patients: caution or boldness?

Abstract

In their article titled ‘Should aortic arch replacement be performed during initial surgery for aortic root aneurysm in patients with Marfan syndrome?’ Schoenhoff et al. raise again a very important question which, after several decades of aortic surgery in this particular group of patients, still remains unresolved. They have operated on 94 patients who underwent 148 procedures. About two-thirds of the patients were first operated on for elective aortic root replacement and one-third, for acute type A dissection. As could be expected from this surgical group that has a very large experience with aortic surgery, the reported results are outstanding and among the best published in this matter [1]. By analysing their experience and comparing the rate of primary and secondary total aortic arch replacement in those various groups, the authors came to the conclusion that replacing such an aortic segment during elective surgery is not necessary. Conversely extending the aortic replacement to the arch during surgery of acute type A dissection may dramatically reduce the rate of further reoperations and, in particular, the number of secondary arch replacements. This confirms what many groups dealing with Marfan patients have intuitively considered for years. It also confirms our own work published a few years ago and in which we came to the same conclusion. Indeed, in our experience, the transverse arch was replaced only once in 44 patients (2.2%) operated on either electively for aortic root aneurysm (25 cases) or in emergency for acute type A dissection (19 cases). Four (16%) secondary arch replacements were required after elective complete aortic root replacement. These were mandated at a second and a third reoperation in 1 patient by the presence of a false aneurysm, and in 1 patient by the occurrence and evolution, 9 years after initial elective surgery, of an acute type B dissection initially treated conservatively. In the remaining patients, the arch replacement was the surgeon’s choice as the patients needed a reoperation of the ascending aorta and aortic root. In contrast, a subsequent arch replacement was required in 14 of the 19 patients (73%) operated initially for acute type A dissection because of the further evolution of a persistent false lumen in the aortic arch and distal aorta [2]. Nevertheless, despite this sort of intuitive knowledge about what should be done, very few groups (if any) have yet published any experience of systematic arch replacement in Marfan patients suffering from acute type A dissection. Therefore, the article by the Bern group is quite important. It is indeed a critical step forward to the transformation of this silent but, so far, unproductive agreement into an open one and towards a sort of recommendation to the whole surgical community when performing surgery in Marfan patients. However, a few points in the present article may be questioned. The authors state that ‘there was no patient that suffered from persistent neurological impairment after primary or secondary Total Arch Replacement in our institution’. This could leave the reader with the feeling that such procedures encompass a very low neurological risk. Yet, there were neurological complications in 6 (4%) of all the procedures, either primary or secondary, with or without TAR. This is, indeed, quite low a rate. However, since Marfan patients operated on electively for aortic root replacement very seldom experience any neurological complication, we may suppose that most of the reported neurological complications occurred in patients operated on for acute type A dissection or during a reoperation. Then, the rate of neurological impairment would be much higher and closer to what we had observed in our own experience. Another interesting and somewhat intriguing point is that, whether or not the patient underwent a total arch replacement, the rates of late reoperations on the distal thoracic and thoracoabdominal aorta were not significantly different. Therefore, one might wonder ‘why then complicate an already difficult emergency operation with Total Arch Replacement?’ It seems that this was the conclusion of the authors as they state ‘In (Marfan) patients with acute type A dissection, the need for reintervention is precipitated by the dissection itself and not by limitating the procedure to a hemiarch replacement in the emergency setting’. This is a cautious statement, and possibly a too-cautious one. First, because the number of patients at risk was too low to reach any statistical significance that would have been reached with a larger cohort as the trend seems to indicate, and secondly, because in their and our experiences, 33 and 73% patients, respectively, required a secondary total arch replacement. Having replaced the arch during the first emergency