OBJECTIVES: We previously described a potential relationship between the presence of migraine and congenital heart defects. It has been hypothesized that a genetic substrate might determine for congenital heart defects and migraine. Therefore, we wanted to study the prevalence of migraine in female patients with coarctation of the aorta (CA) and to determine predictive variables for migraine in this specific population. METHODS: All patients with CA, previously selected for the ZAHARA trial to evaluate pregnancies and outcome, were recruited from the database of congenital heart disease of the University Hospital of Leuven. These patients were contacted by phone and asked to complete a structured headache questionnaire. Based on this questionnaire, a neurologist blinded to the patients' files, diagnosed migraine without or with aura (MA- and MA+, respectively), according to the international headache criteria. Demographic, clinical, and technical data were obtained by reviewing the patients' records. RESULTS: Thirty-four female patients (age 32.2 +/- 6.3 y) were included in the study. The overall prevalence of migraine in female CA patients was 38%, of which 12% were MA- and 26% MA+. No significant relationship between demographic, clinical, and technical variables and migraine could be determined, except for migraine and the number of pregnancies and smoking behaviour (univariate logistic regression; B = 1.2 and 1.9, P = 0.039 and 0.027, respectively). CONCLUSION: In this study, we found a relatively high prevalence of migraine in female adult CA patients. We could also demonstrate a positive relationship between migraine, smoking behaviour, and the number of pregnancies. Therefore, we should pay more attention to these findings in daily practice.