Long-Term Implications of Emergency Versus Elective Proximal Aortic Surgery in Patients With Marfan Syndrome in the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions Consortium Registry

Study Questions:

Does failed surveillance of aortic size and missed opportunities for prophylactic aortic surgery impact outcomes in patients with the Marfan syndrome (MFS)?

Methods:

When the aortic root reaches appropriate size for intervention based on surveillance imaging, elective aortic root surgery in patients with the MFS can be performed with low morbidity and mortality; whereas emergent surgery is associated with higher morbidity and mortality. However, the impact of failed surveillance leading to emergent surgery is not well defined. The GenTAC (Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions) registry was queried for all patients with MFS. Those patients who had undergone proximal aortic replacement procedures, either emergent or elective, were included in the analysis. Procedure information, quality of life (QOL) scores, and imaging data were assessed.

Results:

The GenTAC registry included 635 patients with the Marfan syndrome as of March 2011. Of these, 194 (31%) had undergone proximal aortic surgery. Of these, 147 (76%) were elective for aneurysm or aortic insufficiency, whereas 47 (24%) were emergent in the setting of aortic dissection. Most of the patients were white males in young adulthood or middle age (18-69 years). Patients in the elective group tended to be younger than those in the emergent group at the time of initial surgery (mean age 34.9 vs. 38.0 years, p = NS). Patients who underwent elective surgery were more likely to receive valve-sparing procedures (42% vs. 17% in the emergent group, p = 0.01), and were less likely to undergo multiple aortic procedures (9.9% vs. 26.9% in the emergent group, p = 0.003). Cross-sectional imaging was available in 95 patients at approximately 7 years post-initial surgery. Those in the emergent group were more likely to have evidence of chronic dissection distal to the proximal repair (73% vs. 12% elective group, p < 0.001) and larger aortic dimensions in the more distal aorta. Finally, those in the elective group had higher QOL measures than those in the emergent group at approximately 5-year follow-up.

Conclusions:

In patients with MFS, emergent initial surgery has important long-term consequences including less valve-sparing procedures, multiple subsequent aortic procedures, increased incidence of distal dissection, increased aortic size in the un-operated aorta, and lower QOL scores.

Perspective:

When patients with the MFS undergo emergent surgery for dissection, there is known high upfront morbidity and mortality. This study highlights other important longer-term implications of emergent surgery in the population. However, even in carefully monitored patients with MFS, aortic dissection can occur at aortic dimensions that are smaller than the recommended size for aortic repair. Additionally, the diagnosis of MFS is often not clear at the time of emergent presentation and can impact initial surgical therapy. The GenTAC registry does not currently collect data that can pinpoint when failures in diagnosis and treatment occur. Overall, this study reminds us that increased physician awareness of MFS, and its implications and presentation, may help improve surveillance and optimal initial surgical interventions in this population.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Cardiac Surgery and CHD & Pediatrics, Congenital Heart Disease, CHD & Pediatrics and Interventions, CHD & Pediatrics and Quality Improvement, Interventions and Structural Heart Disease

Keywords: Heart Diseases, Follow-Up Studies, Marfan Syndrome, Emergencies, Cardiac Surgical Procedures, Vascular Surgical Procedures


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