Q: This is regarding Marafan's syndrome. A patient with aortic root dilatation had undergone Bentall's procedure. What is the average life expectancy after this procedure? In what ways can genetic counseling help him avoid having children with Marfan's syndrome?

A:Quality of life and life expectancy after Bentall's procedure is good and close to normal. However, future health of the patient depends upon: 1. Careful and meticulous anticoagulation and its regulation that is regular use of blood thinning medicine such as Acitrom and monitoring of its action by regular prothrombin time estimation. 2. Keeping blood pressure absolutely normal or on the lower side of normal that is 130/80 mmHg or less. 3. Condition of the rest of the aorta that is if the other parts of the aorta are also diseased then long term outcome is not good. 4. For all the above reasons, regular and intense medical follow up is mandatory. The prevention of occurence of Marfan's syndrome in off spring of diseased individuals depends upon identification of the gene responsible. This can be done by amniocentesis (that is removal of amniotic fluid from mother’s uterus) in early pregnancy. If the culprit gene is identified, then that fetus should be aborted. I hope I have answered your queries but if you any other, please feel free to contact us and it shall be our pleasure to answer them.