Abstract

We report our series of patient treated with TAAA open repair in patients with Marfan syndrome (MFS). In the period 1993 to 2015, 63 of 736 consecutive TAAA open repairs performed in our center were performed in patients with MFS. In this group, mean age was 43 ± 6.2 years, chronic postdissection aneurysms were reported in 51 patients (80.9%), and urgent procedures were performed in nine patients (14.3%). Left heart bypass and cerebrospinal fluid drainage were instituted in 77.7% and 63.5% of the cases, respectively. Four-branched graft was used in 53 cases (84.1%). Total phrenotomy was performed in 55% of the patients. Renal arteries perfusion was performed with 4°C Ringer's lactated solution in 19 patients, with 4°C Custodiol solution in 44. After propensity-score matching based on baseline clinical, anatomical, and intraoperative variables, we were able to match 54 patients with MFS (MFS group) one-to-one with those who undergoing degenerative TAAA repair (degenerative group). Overall operative time, clamping time, and intraoperative bleeding were significantly increased in the MFS group (318 minutes vs 240 minutes [P = .016], 92 minutes vs 62 minutes [P = .03], and 8050 mL vs 4650 mL [P = .07], respectively). The 30-day mortality in MFS group and in degenerative group was 3.7% and 12.9% respectively (P = .01); paraplegia was 5.5% and 11.1%, respectively (P = .048), and respiratory failure was 7.4.% and 16.6%, respectively (P = .02). Freedom from need of hemodialysis or continuous venovenous hemofiltration was significantly increased in the MFS group (1.8% vs 14.8% P = .003) despite longer total renal ischemic time (58.4 ± 17.3 minutes vs 46.1 ± 16.4 minutes; P = .05). Reintervention rate in MFS group and in degenerative group was 3.7% and 14.8%, respectively (P = .09). TAAA Open repair in MFS patients is an effective option with mortality and neurological complications similar to those reported in degenerative TAAA open repair. Perioperative renal function seems to be more preserved in MFS patients. Careful follow-up is mandatory for the higher risk of reintervention in MFS patients.

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