What is the clinical evidence from this study?

Study design: Cohort. Population: Marfan syndrome with aortic root aneurysm (n=189). Intervention: Aortic valve sparing operations. Primary outcome: Mortality at 20 years (95% CI 14.7-30.8).

What does this research mean for the field?

Aortic valve sparing operations in patients with Marfan syndrome provide stable long-term valve function with low complication rates over two decades, though reimplantation offers better protection against aortic insufficiency and reoperation than root remodeling. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

What question did this study set out to answer?

Evaluate cardiovascular events in patients with Marfan syndrome undergoing valve-preserving aortic root repair.

June 13, 2026

Cardiovascular Events After Aortic Root Repair in Patients With Marfan Syndrome

Key Result

In patients with Marfan syndrome undergoing aortic valve sparing operations, the 20-year mortality rate was 21.5% (95% CI: 14.7-30.8%), with stable valve function but ongoing risk of dissection.

Key Points

Evaluate cardiovascular events in patients with Marfan syndrome undergoing valve-preserving aortic root repair.
Prospective follow-up of patients with Marfan syndrome from 1988 to 2019.
Data collected from clinical assessments and imaging (echocardiography, CT, MRI).
Median follow-up duration of 14 years.
20-year mortality rate was 21.5% (95% CI: 14.7%-30.8%).
Cumulative incidence of moderate/severe aortic insufficiency was 14.5% (95% CI: 9.5%-22.0%).
Remodeling of aortic root linked to higher risk of aortic insufficiency and reoperation compared to valve reimplantation.

Study Design

Type

Cohort (n=189)

Structured PICO

Do aortic valve sparing operations provide stable aortic valve function and low rates of cardiovascular events in patients with Marfan syndrome?

Population

189 patients (mean age 36 years, 67% men) with Marfan syndrome who underwent aortic valve sparing operations, followed prospectively for a median of 14 years.

Exposure

Aortic valve sparing operations (reimplantation of the aortic valve or remodeling of the aortic root)

Comparator

Internal comparison: remodeling of aortic root vs. reimplantation of the aortic valve

Outcome

Occurrence of cardiovascular events (mortality, moderate or severe aortic insufficiency, reoperation on the aortic valve, and new distal aortic dissections) at 20 yearshard clinical

Aortic valve sparing operations in patients with Marfan syndrome provide stable valve function and low reoperation rates over 20 years, though distal aortic dissections remain a significant risk.

Abstract

BACKGROUND The usefulness of aortic valve sparing operations to treat aortic root aneurysm in patients with Marfan syndrome (MS) remains controversial. OBJECTIVES The purpose of this study was to evaluate the occurrence of cardiovascular events in patients with MS who have undergone valve-preserving aortic root replacement. METHODS Patients with MS who had aortic valve sparing operations (reimplantation of the aortic valve or remodeling of the aortic root) from 1988 through 2019 were followed prospectively for a median of 14 years. Pertinent data from clinical, echocardiographic, computed tomography, and magnetic resonance images of the aorta were collected and analyzed. RESULTS There were 189 patients whose mean age was 36 years, and 67% were men. Ten patients presented with acute type A dissection and 29 had mitral regurgitation. There were 52 patients at risk at 20 years. Mortality rate at 20 years was 21.5% (95% CI: 14.7%-30.8%); advancing age and preoperative aortic dissections were associated with increased risk of death by multivariable analysis. At 20 years, the cumulative incidence of moderate or severe aortic insufficiency was 14.5% (95% CI: 9.5%-22.0%), reoperation on the aortic valve was 7.5% (95% CI: 3.9%-14.7%), and new distal aortic dissections was 19.9% (95% CI: 13.9%-28.5%). Remodeling of aortic root was associated with greater risk of developing aortic insufficiency and aortic valve reoperation than reimplantation of the aortic valve. CONCLUSIONS Aortic valve sparing operations provide stable aortic valve function and low rates of valve-related complications during the first 2 decades of follow-up but aortic dissections remain problematic in patients with MS.

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