Results after thoracic aortic reoperations in Marfan syndrome.

BACKGROUND: Reoperations on the aortic root and distal thoracic aorta late after initial root surgery in patients with Marfan syndrome might carry high periprocedural risks and be associated with adverse early and midterm clinical outcome. METHODS: Overall clinical follow-up was 13.0±7.6 years and 3.7±3 years after secondary aortic procedures. Cumulative follow-up was a total of 148 patient-years. Kaplan-Meier, log-rank, and multiple logistic regression calculations were performed to identify risk factors for mortality. RESULTS: Of 122 patients with Marfan syndrome who underwent aortic root surgery from 1998 to 2013, 40 (21 men; age, 33±12 years) underwent subsequent open thoracic aortic or endovascular secondary procedures between 1998 and 2013. Initial aortic root procedures were performed for aneurysmal disease (n=16, 40%) or acute or subacute Stanford type A dissection (n=24, 60%). Secondary interventions were performed on the aortic arch or descending thoracic or thoracoabdominal aorta (n=18, 45%; n=8 stent grafts) and aortic valve, root, or ascending aorta (n=22, 60%) 9.3±6.7 years (range, 0.06 to 24 years) after initial root surgery. Survival at 5 and 10 years after secondary aortic surgery was 80% (range, 65% to 90%) and 66% (range, 35% to 85%), respectively (n=13 and n=5 remaining at risk at 5 and 10 years). No difference between stent graft and conventional procedures was detected (p=0.756). Actuarial freedom from stroke was 89% at 5 and 10 years (range, 69% to 96%; stent graft versus no stent graft log-rank p=0.47). Four patients had tertiary aortic procedures. The presence of a chronic dissection or root or valve disease was not associated with adverse outcomes after secondary procedures. The only strong predictor of mortality after reoperations was acute dissection at the time of the initial treatment. CONCLUSIONS: Aortic reoperations in patients with Marfan syndrome can be performed with acceptable midterm outcomes. Performed as a bailout procedure, stent grafting was not associated with increased mortality. The only strong predictor of mortality after reoperations is acute dissection at the time of the initial treatment.

Late complications and distal growth rates of Marfan aortas after proximal aortic repair.

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.