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What is the long term outlook for Marfan syndrome?

What is the long term outlook for Marfan syndrome?

The leading cause of death in Marfan syndrome is heart disease. One in 10 patients may have a high risk of death with this syndrome due to heart problems. Despite the high risk for Marfan related cardiovascular problems, the average life expectancy of those with Marfan syndrome is nearly 70 years.

What are the long term effects of Marfan syndrome?

Over years, this stress or pressure causes the aorta to stretch (dilate) and eventually form an aortic aneurysm. If an aneurysm forms, there is an increased risk of an aortic dissection (tear), which is a life-threatening medical emergency.

What is the long term prognosis for Marfan syndrome?

Survival curves were generated, and data were analyzed. The median cumulative probability of survival was 61 years, significantly increased compared with the median survival of 47 years for patients with Marfan syndrome determined 30 years ago (P < . 0006).

How does Marfan syndrome progress over time?

In most cases, Marfan syndrome progresses as individuals grow older. In some infants, Marfan syndrome may cause severe, rapidly progressive complications during infancy, often quickly affecting multiple organ systems early in life.

What is the quality of life for someone with Marfan syndrome?

In this study of 174 affected adults, the overall quality of life was reported to be adequate, although it was significantly decreased within the spiritual/psychological domain. Approximately 62% agreed that having Marfan syndrome significantly affected their reproductive decision-making.

What is the current status of Marfan syndrome?

Marfan syndrome is rare, happening in about 1 in 5,000 people. Marfan syndrome is caused by a mutation in a gene called FBN1. The mutation limits the body’s ability to make proteins needed to build connective tissue. One in four people with Marfan syndrome develops the condition for unknown reasons.

What happens to the body with Marfan syndrome?

Marfan syndrome most commonly affects the heart, eyes, blood vessels and skeleton. People with Marfan syndrome are usually tall and thin with unusually long arms, legs, fingers and toes. The damage caused by Marfan syndrome can be mild or severe.

What is the prognosis of Marfan syndrome?

The features of Marfan syndrome can become apparent anytime between infancy and adulthood. Depending on the onset and severity of signs and symptoms, Marfan syndrome can be fatal early in life; however, with proper treatment, many affected individuals have normal lifespans.

What is the prognosis for Marfan syndrome?

How do people with Marfan syndrome live?

To protect their hearts, people with Marfan syndrome must limit contact sports and strenuous activity, as well as how much they lift, because lifting can strain the aorta. Most of us shouldn’t lift more than 20 pounds. Women are especially at risk for an enlarged aorta during pregnancy and a torn aorta during labor.