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Risk factors for migraine in Marfan syndrome 

Results show that 44% of Marfan's patients suffer from migraine

Date: 31 Aug 2008
The incidence of migraine with aura is increased with patients with Marfan syndrome, with aortic root surgery found to be an independent risk factor, according to a Dutch abstract.

Recently, Hermans et al (Heart 2007; 3: 361-2) reported that not only the presence of a right-to-left shunt (which occurs with patent foramen ovale) but all congenital heart defects increased the prevalence of migraine with aura. In a separate study, Truong et al (Am J Cardiol 2008; 3: 396-400) showed that 32% of 130 patients with congenital heart disease suffered from migraine with aura, despite not having an intracardiac shunt.

MR angiogram of Marfan patient who had surgical replacement of the aortaIn the current study Jeroen Vis and colleagues, from the Academic Medical Centre in Amsterdam, set about investigating the prevalence of migraine in patients with Marfan syndrome and defining risk factors. 

Marfan patients from the AMC were identified via the CONCOR database, a national registry of congential heart defects in the Netherlands, and invited to join the study, with each patient agreeing to participate and providing contact details of a relative or cquaintance to serve as a control. Altogether 97 patients with Marfan syndrome and 80 controls answered the study questionnaire. Migraine was defined as a recurrent headache, with the attack typically lasting 4-72 hours, and migraine with aura as migraine accompanied by focal neurological symptoms, occurring gradually within 5-20 minutes and lasting for a maximum of 60 minutes.

Results showed that 44% of Marfan's patients suffered from migraine, compared to 28% of controls (p=0.02), and that migraine with aura was diagnosed in 37% of Marfan's patients compared to 10% of controls (p<0.001).

When the authors looked at the association between migraine and specific cardiovascular Marfan features - such as aortic dilatation, aortic dissection, mitral valve prolapse, mitral regurgitation, and mitral valve surgery and aortic root surgery - they found that only aortic root surgery appeared to be an independent risk factor. "One possible explanation is that micro-emboli from aorta graft could be triggering attacks after aortic root surgery," suggests Vis, adding that the next step will be to investigate whether similar patterns of migraine occur in non-Marfan patients after aortic root surgery.


Conclusion One unexpected finding from the study was higher than normal levels of migraine in patients representing the "general population". Familial migraine, the authors suggest, may play a role here, since 16 of the 80 controls were first degree relatives of the Marfan patients.

Authors: Janet Fricker
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