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You are here: Contents > 2014 > Volume 23 Number 3 May 2014 > AORTIC VALVE DISEASE > Valve-Sparing Aortic Root Replacement in Patients with Marfan Syndrome Enrolled in the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions

Valve-Sparing Aortic Root Replacement in Patients with Marfan Syndrome Enrolled in the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions

Howard K. Song1, Liliana R. Preiss2, Cheryl L. Maslen3, Barbara Kroner2, Richard B. Devereux4, Mary J. Roman4, Kathryn W. Holmes5, H. Eser Tolunay6, Patrice Desvigne-Nickens6, Federico M. Asch7, Rita K. Milewski8, Joseph Bavaria8, Scott A. LeMaire9, for the GenTAC Consortium

Divisions of 1Cardiothoracic Surgery, 3Cardiovascular Medicine and 5Pediatric Cardiology, Oregon Health & Science University, Portland, Oregon; 2Research Triangle Institute, International, Bethesda, Maryland; 4Division of Cardiology, Weill Cornell Medical College, New York, New York; 6National Heart, Lung, and Blood Institute, Bethesda, Maryland; 7Division of Cardiology, MedStar Washington Hospital Center, Washington, DC; 8Division of Cardiothoracic Surgery, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania; 9Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas, USA

Background and aim of the study: The long-term outcomes of aortic valve-sparing (AVS) root replacement in Marfan syndrome (MFS) patients remain uncertain. The study aim was to determine the utilization and outcomes of AVS root replacement in MFS patients enrolled in the Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC).

Methods: At the time of this analysis, 788 patients with MFS were enrolled in the GenTAC Registry, of whom 288 had undergone aortic root replacement. Patients who had undergone AVS procedures were compared to those who had undergone aortic valve replacement (AVR).

Results: AVS root replacement was performed in 43.5% of MFS patients, and the frequency of AVS was increased over the past five years. AVS patients were younger at the time of surgery (31.0 versus 36.3 years, p = 0.006) and more likely to have had elective rather than

emergency surgery compared to AVR patients, in whom aortic valve dysfunction and aortic dissection was the more likely primary indication for surgery. After a mean follow up of 6.2± 3.6 years, none of the 87 AVS patients had required reoperation; in contrast, after a mean follow up of 10.5 ± 7.6 years, 11.5% of AVR patients required aortic root reoperation. Aortic valve function has been durable, with 95.8% of AVS patients having aortic insufficiency that was graded as mild or less.

Conclusion: AVS root replacement is performed commonly among the MFS population, and the durability of the aortic repair and aortic valve function have been excellent to date. These results justify a continued use of the procedure in an elective setting. The GenTAC Registry will be a useful resource to assess the long-term durability of AVS root replacement in the future.

The Journal of Heart Valve Disease 2014;23:292-298


Valve-Sparing Aortic Root Replacement in Patients with Marfan Syndrome Enrolled in the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions

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