Early and Long-term Outcomes after Aortic Surgery in Patients with Marfan Syndrome

Background: Cardiovascular manifestations are the most serious complications and determine the prognosis and survival of patients with Marfan syndrome (MFS). The aim of this study is to determine early and long-term outcomes after aortic surgery in patients with MFS.

Methods: Between January 2006 and March 2015, 36 MFS patients (22 male, 61%; 14 female 39%) with a mean age of 37 ± 17 years underwent aortic surgery in our institution. The aortic root was reconstructed, glued and remained untouched in 21 patients (58%), 3 patients (8%) and 4 patients (11%), respectively. 8 patients (22%) underwent the Bentall procedure. Hemiarch replacement was performed in 14 patients (39%) and total arch replacement in 22 patients (61%). Elephant trunk technique was performed in 15 patients (42%) and frozen elephant trunk technique in 3 patients (8%). All patients were treated postoperatively with β-blockers, ACE-inhibitors and statins. Clinical data were prospectively entered into our institutional database. Mean late follow up for late survival and freedom from re-operation/re-intervention was 5 ± 3 years and 81 ± 3% and was 98% complete.

Results: Cardiopulmonary bypass time accounted for 190 ± 71 minutes and the myocardial ischemic time 122 ± 64 minutes. Antegrade cerebral perfusion during mild-moderate systemic hypothermia was performed in all patients. Mean duration of ACP was 48 ± 25 minutes. Unilateral cerebral perfusion was performed in 23 patients (64%), bilateral in 13 patients (36%). Mean core temperature amounted to 28.8°C ± 0.7°C. Mean intensive care unit stay was 4 ± 8 days. We observed new postoperative permanent neurologic deficits in one patient (3%) and transient neurologic deficits in 4 patients (11%). Redo surgery was performed in 7 patients (19%). Two of these patients with redo complete arch replacement died accounting for an overall 30 day mortality of 6% (n = 2). Late survival rate at 6 years was 89%. Freedom from re-operation/re-intervention at late follow-up in patients with hemiarch replacement was 64 ± 5% and only 91 ± 3% in those with total arch replacement (p = 0.04).

Conclusions: Patients with MFS seem to benefit from initial total arch replacement with elephant trunk or frozen elephant trunk techniques. Furthermore we recommend rigorous adjustment of systolic blood pressure below 120 mm Hg and postoperative medical treatment including β-blockers, ACE-inhibitors and statins in MFS patients.