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  • Aortic Valve-Sparing Provides ‘Stable and Durable Function’ in Marfan Syndrome Patients – Long-Term Study

    A new study of patients with Marfan syndrome (MS) suggests that aortic valve-sparing operations, specifically the reimplantation procedure, provide stable and durable aortic valve function.

    Writing in the Sept 12 issue of the Journal of the American College of Cardiology, the Canada-based research team pointed to the procedure’s low rates of valve-related complications during the first 2 decades of follow-up, but also highlight the ongoing challenges in patients with MS.

    “Aortic dissections, however, remain an unresolved problem in these patients, and there is a constant risk of distal aortic dissections, which has been the leading cause of mortality and morbidity after aortic valve sparing operations in our experience,” said the paper’s authors, led by Tirone E. David, MD, from Toronto General Hospital. “Reoperations to manage complications of the aortic dissections are far more common than reoperations on the preserved aortic valve during the first two decades of follow-up.”

    Main study findings

    Results of the study, which were also published Monday online, tracked 189 MS patients, and found 10 of those presented with acute type A dissection, while a total of 29 patients were diagnosed with mitral regurgitation.

    The research team also identified 52 patients at risk at 20 years, with the mortality rate at this period identified as 21.5% (95% confidence interval [CI]: 14.7% to 30.8%).

    Further findings by multivariable analysis revealed that advancing age and preoperative aortic dissections were associated with increased risk of death.

     At 20 years, the cumulative incidence of moderate or severe aortic insufficiency was 14.5% (95% CI: 9.5% to 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%).

    The research team stated that remodeling of aortic root was associated with greater risk of developing aortic insufficiency and aortic valve reoperation than reimplantation of the aortic valve.

    Aortic root repair

    Joseph S. Coselli, MD, and Lauren M. Barron, MD, both from the Baylor College of Medicine, Houston, commented on MS’s role in elastic tissues losing their resilience over time, presenting as aneurysmal disease and dissection of the force-bearing vessels in the cardiovascular system.

    In an accompanying editorial comment, the Coselli and Barron said that while the primary conclusions of this study were centered around valve-related complications, it was likely that these outcomes result from having traversed the peak of the previously described aortic root repair (ARR) learning curve.

    Citing the study’s experience and technical expertise as key in limiting outcomes related to errors in judgement or technique, the commentators then asked what was the best lifetime management of the aorta distal to the root.

    “In porcine aortas, ARR using a straight Dacron graft resulted in higher systolic pressures and a 42% increase in the rate of pressure rise (dp/dt) distal to the prosthesis,” they highlighted. “The importance of limiting the rate of pressure rise is the principal tenant of anti-impulse control in the management of type B aortic dissection with specific attention given to this point in the 2022 Society of Thoracic Surgeons/American Association for Thoracic Surgery guidelines.”

    The editorial went on to comment on the improvement in mortality in MS patients thanks to counselling, medical management, and prophylactic surgery.

    However, MS patients who underwent ARR were a “subset of patients who again present a puzzle,” as the commentators pointed out that image-based flow mapping and animal studies substantiated that altering the root and proximal aorta increased the risk of distal aortic stress.

    “Combining open surgical and endovascular devices may offer an option, but experience and long-term follow-up in hybrid approaches are limited in MFS [Marfan syndrome] patients,” they wrote. “Personalized external aortic root support offers yet another novel option, but thus far, it has only been used in the proximal aorta to prevent growth in an already aneurysmal aorta.”

    Commenting on the lack of a centralized patient cohort of sufficient size to power an analysis of MS patients who underwent ARR, the experts concluded that solving the puzzle of the distal dissection in MS patients “cannot happen without collecting rare pieces of data—it would be easier if the pieces were not all sold separately.”

    Study methodology

    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.

    Echocardiographic follow-up was for a median of 11.9 years (interquartile range: 7.2-18.7 years), which was shorter because of the difficulties in obtaining elective cardiac imaging during the COVID-19 pandemic.

    These patients totaled 189, whose mean age was 36 years, and 67% were men. Of the patient population, 164 had reimplantation of the aortic valve and 25 had remodeling of the aortic root.

    Pertinent data from clinical, echocardiographic, computed tomography, and magnetic resonance images of the aorta were collected and analyzed.


    David TE, Park J, Tatangelo M, et al. Cardiovascular Events After Aortic Root Repair in Patients With Marfan Syndrome. J Am Coll Cardiol. 2023;82:1068–1076.

    Coselli JS, Barron LM. Lifetime Aortic Management in Marfan Syndrome: Puzzle Pieces and Rare Diseases. J Am Coll Cardiol. 2023;82:1077–1079

    Image Credit: Николай Зотов –

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