Objectives: Conflicting results have been reported on late aortic growth and complication rates of the descending thoracic aorta in patients with Marfan syndrome (MFS) after proximal aortic surgery.
Methods: Of 198 Marfan patients followed up regularly, 121 (43% David-I, 7% David-II, 11% supracoronary replacement, 52% mechanical conduit, 8% arch replacement) were analysed after proximal aortic surgery retrospectively. 97% had MFS1, 3% MFS2 (Loeys-Dietz-Syndrome); 56% were male and the mean age was 35 ± 13 years. 65% were initially operated on for root/ascending aortic aneurysm and 35% for aortic dissections. Using automated computed tomography angiography and magnetic resonance angiography cross-sectional analyses, the mean diameters of the distal arch, mid-descending and distal supradiaphragmatic descending thoracic aorta were measured at early and late follow-up (mean 6.3 years for aneurysms and 4.7 years for dissections). The mean duration of clinical follow-up was 7.6 years and the cumulative clinical follow-up comprised 894 patient-years.
Results: At 20 years, overall freedom from distal aortic complications and/or reintervention was 76% (51-86%) for aneurysms and 52% (28-71%) for dissections (P = 0.03). In non-dissected aortas, distal aortic growth was significant, but minimal: arches grew from 25.2 ± 0.6 to 26.3 ± 0.8 mm (P = 0.01), mid-descending aortas from 22.2 ± 0.5 to 24.9 ± 1.2 mm (P = 0.05) and distal descending aortas from 22.1 ± 0.7 to 24.2 ± 1.4 (P = 0.02, 0.58 mm/year ± 0.5 mm). Dissected distal aortas increased by a mean of 0.3 ± 0.5 mm/year. Dissection (P < 0.001), urgent procedure (P = 0.02) and hypertension (0.052) were associated with larger distal aortic diameters at late follow-up and more significant aortic growth over time.
Conclusions: Late distal complication rates are low for patients initially presenting with aneurysms. The risk of late distal reoperation is dictated by the initial pathology and by the presence of an initial dissection and not by faster distal aortic growth. Strategies to completely restore a non-dissected anatomy might improve late surgical outcome in Marfan's syndrome.
Keywords: Aortic aneurysm; Aortic dissection; Aortic root surgery; Marfan syndrome; Prosthetic aortic replacement; Thoracic aortic stent-grafting.