A new study published in Journal of Thoracic and Cardiovascular Surgery evaluated the long-term outcomes of patients with Marfan syndrome (MFS) who were undergoing open thoracoabdominal aortic aneurysm (TAAA) repair than those without the condition. Despite their genetic predisposition to connective tissue disorders, MFS patients demonstrated better survival outcomes over time.

This retrospective study analyzed data from 230 consecutive patients who underwent open TAAA repair between 2012 and 2022. Of these, 69 patients had Marfan syndrome. The primary outcome was long-term mortality. The secondary outcomes was early adverse events such as stroke, paraplegia, renal failure, early death, and the need for reoperation.

The MFS group was significantly younger than the non-MFS group (average age 31.9 years vs. 44.8 years). These patients had fewer instances of coronary artery disease (0% vs. 8.1%) and more frequently underwent Crawford extent III repairs (56.5% vs. 34.8%). Use of normothermic iliac perfusion was also more common in MFS patients (91.3% vs. 81.4%).

Early composite adverse event rates were not significantly different between the groups (23.2% for MFS vs. 14.3% for non-MFS), and this finding was confirmed through multiple logistic regression models. However, when it came to long-term survival, MFS patients performed better. Cumulative mortality rates at 1, 5, and 10 years were 4.4%, 8.1%, and 20.9% in the MFS group and 8.7%, 17.2%, and 36.4% in the non-MFS group.

Marfan syndrome is also reported to be a significant protective factor against overall mortality, with hazard ratios below 0.4 in different models (model 1: 0.31; model 2: 0.32; model 3: 0.38).