What is the life expectancy of a patient with Marfan syndrome?
Life Expectancy and Causes of DeathMost people with Marfan syndrome have a normal lifespan. However, because of the risk of complications, people with Marfan syndrome should have regular check-ups with a doctor who is familiar with the condition.
About 90 percent of people who die of Marfan syndrome die of aortic complications. Other causes of death in Marfan syndrome include mitral valve prolapse (MVP) and its complications (such as congestive heart failure and stroke); spontaneous pneumothorax (collapsed lung); and complications of any surgical procedures.
Aortic Complications
Aortic complications include:
Aortic dissection or aneurysm. About 75 percent of people with Marfan syndrome develop aortic dissection or aneurysm. In an aortic dissection, a layer of the aortic wall tears, causing the wall to separate and bleed. If an aortic aneurysm bursts, it can be fatal.
Aortic regurgitation. Aortic regurgitation occurs when the aortic valve does not close completely, causing blood to leak back into the heart.
Acute aortic syndrome. This rare but serious condition occurs when both aortic dissection and aortic regurgitation are present.
MVP
Mitral valve prolapse (MVP) occurs when the mitral valve does not close completely, causing blood to leak back into the heart. About 50 percent of people with Marfan syndrome develop MVP. MVP can lead to congestive heart failure and stroke.
Spontaneous Pneumothorax
Spontaneous pneumothorax occurs when air leaks into the space between the lung and the chest wall, causing the lung to collapse. About 25 percent of people with Marfan syndrome develop spontaneous pneumothorax. Spontaneous pneumothorax can be a serious complication, especially if it occurs repeatedly.
Complications of Surgery
People with Marfan syndrome are at an increased risk of complications from any surgical procedures, including heart surgery. These complications may include:
Bleeding
Infection
Formation of blood clots
Organ damage
Death