Key result
Multidisciplinary management, including proper follow-up, medical therapy, and timely surgical repair, increases the median cumulative probability of survival to over 72 years in patients with Marfan syndrome.
Early identification and a multidisciplinary approach, including timely surgical repair of aortic complications, can increase the life expectancy of Marfan syndrome patients to an almost normal lifespan.
May support multidisciplinary care in Marfan syndrome; leaves open prospective validation of survival gains.
BACKGROUND: Marfan syndrome (MFS) is the most common inherited disorder of connective tissue affecting multiple organ systems. The most life-threatening and life-shortening complication is aortic dissection. Without surgery, life expectancy of MFS patients is reduced to approximately 32 years. Early identification and appropriate multidisciplinary medical cooperation is essential. CONCLUSION: Proper follow up, therapy and timely surgical repair lead to an almost normal lifespan in affected individuals.
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Petr Šantavý (2013) conducted a review in Marfan syndrome. Multidisciplinary management was evaluated. Multidisciplinary management, including proper follow-up, medical therapy, and timely surgical repair, increases the median cumulative probability of survival to over 72 years in patients with Marfan syndrome.
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