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CV Outcomes After Aortic Root Repair in Marfan Syndrome
Quick Takes
- In a cohort of 189 Marfan syndrome patients undergoing valve-sparing aortic root repair operations at a high-volume aortic center, the cumulative incidence of aortic valve reoperation at 20 years was <10%.
- New distal aortic dissections were common, with a 20-year incidence of ~20%.
Study Questions:
What are the long-term clinical outcomes of valve-sparing aortic root repair operations in patients with Marfan syndrome?
Methods:
This prospective observational study followed Marfan syndrome patients who underwent valve-sparing aortic root operations for aortic root aneurysm or aortic dissection at Toronto General Hospital from 1988–2020. Medical therapy on discharge included beta-blockers and/or renin-angiotensin-aldosterone system inhibitors (more recently, both). Postoperatively, patients were followed with echocardiograms every 2-3 years and cross-sectional imaging of the thoracic and abdominal aorta every 3-5 years. Outcomes of interest included postoperative mortality, postoperative moderate or severe aortic regurgitation (AR), new distal aortic dissection, and reoperation on the aortic valve.
Results:
The analysis included 189 patients (mean age 36.1 years, 67% male). Sixteen patients (8%) underwent surgery for aortic dissection, 10 patients (5%) had bicuspid aortic valves, and the median preoperative aortic root diameter was 52 mm. The David operation (reimplantation of the aortic valve inside a graft) was performed in 164 patients (86.7%), and the Yacoub operation (remodeling of the aortic root with excision of the aortic sinuses and tailoring of a graft to reshape the root) was performed in 25 (13.2%). The median clinical follow-up period was 14 years. A total of 36 deaths occurred following the index hospitalization. Cumulative mortality was 3.2% at 5 years, 6.7% at 10 years, and 21.5% at 20 years.
Following the David operation, cumulative incidence of moderate or severe AR was 5.1% at 5 years and 8.5% at 20 years; following the Yacoub operation, cumulative incidence was 7.8% at 5 years, 20.0% at 10 years, and 35.4% at 20 years (adjusted hazard ratio [aHR] for development of significant AR with Yacoub operation, 5.47; p = 0.001). A total of 10 patients underwent aortic valve reoperation (cumulative incidence 7.5% at 20 years). The Yacoub operation was associated with increased risk of valve reoperation relative to the David operation (aHR, 6.09; p = 0.018).
New distal aortic dissections occurred in 32 patients (cumulative incidence 5.3% at 5 years and 19.9% at 20 years), and 22 patients underwent surgery or endovascular repair for dissection complications. The type of aortic root repair that was initially performed was not associated with dissection risk.
Conclusions:
Valve-sparing aortic root repair, particularly the David operation, is associated with favorable long-term valvular outcomes in Marfan syndrome patients. However, these patients remain at high risk for distal aortic dissections.
Perspective:
The outcomes described in this study reflect upon care provided in a high-volume aortic center with experienced surgeons and infrastructure for close long-term follow-up. Valve-sparing operations can offer freedom from long-term warfarin therapy that is required with mechanical aortic prostheses, an advantage that is especially relevant in the relatively young Marfan syndrome population. Although a few patients in this cohort had bicuspid aortic valves, the findings cannot be readily extrapolated to patients undergoing valve-sparing root repair for bicuspid aortopathy, as such patients are at greater risk of progressive aortic stenosis.
Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Valvular Heart Disease, Vascular Medicine, Aortic Surgery, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Interventions, Interventions and Structural Heart Disease, Interventions and Vascular Medicine
Keywords: Aortic Aneurysm, Cardiac Surgical Procedures, Heart Defects, Congenital, Heart Valve Diseases, Marfan Syndrome
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