July 1, 2023Open Access
Outcomes of aortic root replacement in patients with Marfan syndrome: the role of valve-sparing and valve-replacing approaches
Key Result
Valve-sparing aortic root replacement in patients with Marfan syndrome was associated with improved late survival but a higher rate of late adverse events (18%) compared to mechanical grafts (4%).
Study Design
Type
Cohort (n=223)
Multicenter
No
Structured PICO
Does valve-sparing aortic root replacement improve survival and reduce reoperation compared to valve-replacing approaches in patients with Marfan syndrome?
P
Population
223 consecutive patients with Marfan syndrome undergoing elective aortic root replacement (ARR) between 1991-2023. Median age 38 [29-52] years.
I
Intervention
Valve-sparing (VS) aortic root replacement (n=62)
C
Comparator
Valve-replacing (VR) approaches, including mechanical composite valve graft (CVG) (n=113) and bioprosthetic root (n=48)
O
Outcome
Operative death, late survival, and reoperationhard clinical
In patients with Marfan syndrome, valve-sparing aortic root replacement is associated with improved late survival compared to valve-replacing approaches, despite a higher risk of late valvular-structural deterioration.
Main Result
Absolute Event Rate: 18% vs 4%
p-value: p=<0.001
Limitations
- Difficulty in evaluating a heterogeneous study population with a complicated history of aortic disease over a long period of time
- Unable to obtain follow-up echocardiographic data on all patients
- Inability to systematically capture late thrombolytic events, late onset of aortic dissection, and late rupture events
- Potential failure to adequately capture all late deaths
Abstract
Background: Marfan syndrome (MFS) is a heritable thoracic aortic disease with pervasive cardiovascular effects, including commonly, a dilated aortic root. Traditionally, the root is replaced using a mechanical composite valve graft (CVG); however, this valve-replacing (VR) approach necessitates a lifelong regimen of anticoagulation with a potential for late bleeding complications. In time, valve-sparing (VS) approaches were developed. Today, several options for aortic root replacement (ARR) exist; each has advantages and disadvantages that helps inform choice. The Aortic Valve Operative Outcomes in Marfan Patients (AVOMP) is a multi-center international registry to analyze clinical outcomes of ARR in MFS patients using either VR or VS techniques to better elucidate choice. We summarize outcomes of AVOMP and present our own experience. Methods: We performed 223 consecutive elective ARR 1991-2023 in patients with MFS; 15 such repairs were included in AVOMP. Repairs included 113 (51%) using a mechanical CVG, 62 (28%) using a VS approach, and 48 (22%) using a bioprosthetic root. Many patients underwent aortic arch repair (30% to 54% by type). Results: The median patient age was 38 29-52 years. In comparing VS and VR groups, patients were similar in age and rates of major comorbidities and symptoms. Patients with VR repair had a more complex aortic history. The rate of redo sternotomy was 24% (n=54). Operative death was uncommon 4% overall (10/223); ranging from 2% to 8% by type, and stroke was rare 1/223 (<1%). Late survival and reoperation differed by operative approach; survival was improved in patients who underwent VS repair. Conclusions: We found that repair in patients with MFS undergoing ARR resulted in low operative risk. Our late results were similar to those of AVOMP in that patients undergoing VS repair tended to experience greater rates of valvular-structural deterioration, although this did not appear to impact survival.