Cardiac surgery in patients with marfan syndrome: 20-year single-center experience

Abstract Marfan syndrome is a connective tissue disease whose most fearsome complications are proximal thoracic aorta enlargement and dissection. Nonetheless, cardiac surgery has proven to be able to considerably extend average life expectancy up to approximately 70 years1. The aim of our study was to analyze which procedures had been performed on these patients. From 2000 to 2020 we retrospectively enrolled 89 consecutive patients undergoing a first–time surgical operation at our department. Overall, aortic root surgery represented the most frequent procedure (82 patients, 92.2%), with valve–sparing operation being the preferred one (53 patients, 59.6%). On the other hand, 29 patients (32.6%) underwent Bentall–De Bono procedure. Consequently, we compared these 2 groups of patients. In emergent operation, typically type A aortic dissection, Bentall–De Bono procedure was preferred in almost all cases (p–value 0.031). Few patients required the replacement of part or all of the arch, respectively 3 and 6, with no statistical difference between the two groups. However, while 2 valve–sparing patients required a mitral valve repair, 8 patients who underwent aortic root replacement had also their mitral valve replaced. Considering mean age (average 34.68 years), predictably almost all Bentall–De Bono patients had both their aortic and mitral valve replaced with a mechanical prosthesis (respectively 27 and 7 out of 29 and 8). Although there was no significant difference, replacement of the aortic root with a composite graft required less cross–clamp time, being also different the complexity of the technique. ICU stay was significantly different, since patients who had their aortic valve spared needed intensive assistance for an average of just 1.57 days, while the other group for 6.03 days (p–value 0.037). Similarly, but non significantly, hospital stay differed (13.91 and 18.17 days). Only 2 patients died during hospital recovery, both part of the Bentall–De Bono group (p–value 0.055). During the follow–up period (average 89.39 months), 6 patients died, 2 of them for noncardiac related causes, and 9 (11.3%) required another operation. In conclusion, Marfan syndrome is characterized by a dangerous proximal aorta involvement, which in some cases may be life–threatening if left untreated. Fortunately, surgery has significantly improved life quality and expectancy of these patients, who must be adequately diagnosed and then monitored by their referral centers.