Recruitment into the study was closed in November 2010.

Recruitment for the first phase of the study (which included a 3-year follow-up period) ended in November 2010. For the second phase, in October 2014 the follow-up period was extended to 20 years for enrollees who decided to continue participating in the study.
At this time, recruitment for extended follow-up is complete.

Questions? Contact Dr. Irina Volguina

Study Information for Patients

The Marfan Foundation

1. What is Marfan syndrome?
2. What is the aortic valve and aortic root?
3. What is the value of surgical intervention?
4. What surgical techniques are used to restore aortic valve function?
5. Why conduct this study?
6. What are the goals of this study?
7. How will the study be conducted?
8. Who can participate in the study?
9. What will the study participant experience? Informed Consent Form
10. What study results are expected?
11. Who sponsors the study? 

1. Marfan syndrome is a medical condition and is classified as a heritable disorder of connective tissue.

   Manifestations of the Marfan syndrome appear in many parts of the body, especially in the bones and ligaments (the skeletal system), the eyes (the ocular system), the heart and blood vessels (the cardiovascular system), the lungs (the pulmonary system), and the fibrous membrane covering the brain and spinal cord (the nervous system). For more information, visit the The Marfan Foundation website.

   Excerpted from The Marfan Syndrome, by Reed E. Pyeritz, M.D., Ph.D. and Cheryll Gasner, M.N., C./F.N.P. Fifth Edition, July 1999, Revised 2001.

2. The aortic root is a three cm long part of the aorta adjacent to the left ventricle of the heart. It contains the three cusp aortic valve that prevents backflow of blood into the ventricle after the heart contraction. Aortic root dilatation, which is an increase of the diameter of the aortic root, represents a common clinical feature in patients with Marfan syndrome. Aortic root dilatation contributes to the malfunction of the aortic valve. Marfan patients may need to undergo a surgical operation to correct aortic root dilatation and aortic valve dysfunction.
3. Aortic valve surgical intervention prevents life-threatening complications including malfunction of the aortic valve, aortic dissection, and rupture.
4. While aortic root dilatation is repaired by replacing the dilated aorta with a Dacron graft, two different surgical techniques are used to restore the function of the valve: aortic valve replacement and aortic valve sparing. The AVR procedure uses mechanical or biological valves to replace a patient’s natural valve. Mechanical valves that are used most often require life-long commitment to anticoagulation therapy. The AVS procedure retains a patient’s natural valves and allows the patient to be anticoagulation free.
5. The main reason to conduct the study is to support surgeons and patients with clear indications for choosing the right type of operation. The choice of surgical intervention for each patient is not always easy. Clear indications for choosing the type of operation have not been established yet for the majority of patients, and an accumulation of additional scientific evidence is necessary. Indications for AVR operations exist only for patients who have damaged or inflamed aortic valves and cannot undergo AVS procedures. For the remaining majority of patients who can have both AVR and AVS surgery, the pros and cons of each type of surgery and the outcomes in different categories of patients need further clarification. Choosing the right operative technique will reduce postoperative complications and ensure better survival.
6. The goals the Aortic Valve Operative Outcomes in Marfan Patients study are to evaluate and compare the operative outcomes of aortic valve replacement and aortic valve sparing surgical interventions among different categories of patients with Marfan syndrome. It will assess the prognostic factors that have an impact on operation outcome.
7. Leading surgical centers specializing in aortic root surgery in the United States and worldwide will join their efforts to conduct the three year study.
• Data Coordination Center and Surgical Core
  Dr. Joseph S. Coselli, chief of the Division of Cardiothoracic Surgery of the Michael E. DeBakey Department of Surgery at Baylor College of Medicine, serves as study principal investigator and as head of the Data Coordination Center and Surgical Core.
• Marfan Diagnostic Core
  Dr. Hal Dietz, of Johns Hopkins Hospital, serves as director of the Genetics Core which will verify the Marfan syndrome diagnosis and family history.
  
• Imaging Core
  Dr. Heidi Connoly, Mayo Clinic, Rochester, serves as the principal investigator and director for the Imaging Core which will analyze the imaging information (echocardiograms, CT, MRI).
  
• Surgical Centers
  Twenty five surgical centers will enroll patients into the study, perform the operations and conduct patient follow up for three years.
8. All patients with Marfan syndrome in need of aortic valve operations are encouraged to participate in the study. Three hundred sixteen participants are expected for the prospective component and 150 for the retrospective component.
9. You will find the complete description of study procedures in the Informed Consent Form, sections Procedures and Potential Risks and Discomforts.
10. Expected results A comparison of survival, freedom from complications, and health-related quality of life data will be performed for the AVR and AVS interventions. Special design features and collection of information on different operation types within the framework of one study will allow for the most accurate comparison and assessment available to date. Quality of life estimates will provide a patient-oriented composite evaluation of the operative success. An important component of the study will be the creation of a centralized on-line data registry. The study will establish research infrastructure, present basic results and develop the data registry that will serve as a necessary foundation for future clinical research in this area.
11. The study is sponsored in part by the National Marfan Foundation, Baylor College of Medicine, Stanford University, and other participating institutions.