Marfan syndrome is a genetic disorder that results in abnormal growth of connective tissue, which plays a key role in the development and maintenance of the body's organs. A number of cardiac defects of Marfan syndrome can be serious or life threatening; in addition, serious, non-cardiac complications can also be seen in the lungs, bones, eyes and nervous system.
Two different structures in the heart are most commonly affected by Marfan syndrome. The aorta, the main artery that carries blood from the heart to the body, can become dilated or enlarged, or can develop an aneurysm, in which a weak portion of the vessel wall bulges. In all of these cases, the aorta is at risk for dissection, or tearing, so regular monitoring is critical.
The valves that control blood flow in the heart and from the heart to the blood vessels can also be affected. In these cases, the mitral and aortic valves flap closed incompletely. This can negatively affect normal blood flow and heart rhythm and may require surgical intervention to correct.
Treatment of Marfan Syndrome
Currently there is no cure for Marfan syndrome. When detected early, however, treatments can help delay or alleviate complications. For heart complications associated with Marfan syndrome, early detection allows doctors to monitor the size of the aorta carefully and examine the vessel walls. It also allows them to schedule aortic repair surgery to prevent rupture or tears. After surgery, medications are taken to lower blood pressure and help prevent further complications. This proactive approach also allows doctors to monitor valve function and to determine whether valve repair surgery might be needed.
Our experts at Houston Methodist are committed to caring for patients with Marfan syndrome through regular monitoring and continuous management of serious cardiac complications. Also, because Marfan syndrome can affect a wide range of organs in the body, we work together as a multidisciplinary team of physicians to maximize benefits of cooperation across all health care providers.
The Aortic Network, part of the Houston Methodist DeBakey Heart & Vascular Center, serves the specific needs of patients living with complex aortic conditions. When aortic problems arise, patients know they can rely on Aortic Network doctors and staff for prompt, patient-focused care. Each member of our multidisciplinary team of cardiologists, surgeons, nurse practitioners and other professionals has years of experience in treating conditions specific to the aorta. Our unique combination of unparalleled expertise and advanced technology enables us to offer each patient a treatment program tailored to his or her needs.