A Pulmonary Right-to-left Shunt in Patients with Hereditary Hemorrhagic Telangiectasia is Associated with an Increased Prevalence of Migraine

Introduction: Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal-dominant vascular dysplasia with a high prevalence of pulmonary arteriovenous malformation (PAVM). Recent studies report an increased prevalence of migraine in patients with a cardiac right-to-left shunt. The aim of our study was to evaluate whether there is also an increased prevalence of migraine in patients with a pulmonary right-to-left shunt (PAVM).

Methods: All patients with HHT referred to our hospital till April 2004 with or without PAVM and with or without migraine were included in the study.

Results: In total, 538 HHT patients (41.6% men; mean age +/- SD, 39.3 +/- 18.6 years) could be included. PAVM was present in 208 patients (38.7%; mean age, 39.3 +/- 17.6 years). Significantly more women were present in the PAVM subgroup compared to the non-PAVM subgroup, 65.4% vs 53.9% (p = 0.009). Migraine occurred in 88 patients with HHT, a prevalence of 16.4%. The prevalence of migraine in women with HHT was significantly higher compared to men, 19.4% vs 12.1%, respectively (p = 0.03) The prevalence of migraine in patients with PAVM was 21.2%, which was significantly higher then in patients without PAVM, 13.3% (p = 0.02). The occurrence of PAVM in the patients with migraine is significantly higher than in those without migraine, 50.0% vs 36.4%, respectively (p = 0.02).

Conclusion: This study showed a higher prevalence of PAVM in patients with migraine and HHT. The right-to-left shunt due to the PAVM might play a causal role in the pathogenesis of migraine in patients with HHT. This needs to be determined in further studies.