Question: Four years ago, my family doctor and cardiologist told me I may have Marfan syndrome. They tell me my heart is enlarged, but how big is too big? When I took calcium blockers and beta blockers, I had side effects. I also have side effects from diazepam and Lamictal which I take for other problems. Why should I worry over Marfan syndrome when I can’t tell if it is causing problems? If I am wrong, what else could I take that will not have the same side effects?
Dr. Hibberd's Answer:
Marfan syndrome is an inheritable disorder of connective tissue. It is believed to be associated with the inflammatory effects of overproduction of growth factor beta (TGF-B). It is thought to create inflammation, especially in the aorta, heart valves and lung. It weakens these tissues, and causes complications of these tissues that are occasionally life-threatening. You are wrong to ignore preventive treatment.
ARB (angiotensin receptor blocker ) medication has been shown to reduce the abnormal aorta growth often seen in Marfan victims and has been a major breakthrough in extending longevity of Marfan patients. It should not be disregarded.
You need to be alert for changes in your health, especially of your eyes, lungs, heart and spine. Life expectancy for patients with Marfan syndrome has significantly increased over the past 20 years thanks to early intervention and treatment. Marfan is treated by watching for changes and treating them early.
Regular cardiac checkups are especially important to monitor the condition of your heart valves and aorta, as well as to keep blood pressure in check to reduce the chances of aneurisms forming. Medications often used include beta blockers, ACE inhibitors, and ARB's (angiostensin receptor blockers). If unchecked, aneurisms, heart failure and arrhythmias are common and valve and aortic surgery may be urgently needed as a life-saving procedure. Treatment of any obstructive lung changes is also important. The bone, skeletal and eye abnormalities seen in Marfan are generally not life-threatening, but certainly make its victims easy to diagnose.
You need to educate yourself about your condition, as well as to realize that the present longevity seen in Marfan seen today is directly related to interventions. You do not wish to turn back the clock to 30 years ago when many victims died before the age of 30 or 40 due to a lack of preventive treatment!
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