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Congenital heart disease
Inheritance of persistent foramen ovale and atrial septal defects and the relation to familial migraine with aura
  1. P T Wilmshurst1,
  2. M J Pearson1,
  3. S Nightingale1,
  4. K P Walsh2,
  5. W L Morrison3
  1. 1Royal Shrewsbury Hospital, Shrewsbury, UK
  2. 2Our Lady’s Hospital for Sick Children, Dublin 12, Ireland
  3. 3The Cardiothoracic Centre, Liverpool, UK
  1. Correspondence to:
    Dr Peter Wilmshurst
    The Royal Shrewsbury Hospital, Shrewsbury SY3 8XQ, UK; peter.wilmshurstrsh.nhs.uk

Abstract

Objective: To determine whether smaller atrial shunts (large persistent foramen ovale (PFO) and small atrial septal defect (ASD)) are inherited and whether this has a role in the inheritance of migraine with aura.

Methods: Contrast echocardiography was used to detect atrial shunts in 71 relatives of 20 probands with a significantly sized atrial shunt (large PFO or ASD). Four families with three generations, 14 families with two generations, and two sibships were studied. The contrast echocardiograms were performed blind to history of migraine. A consultant neurologist, who was blinded to cardiac findings, categorised migraine symptoms in subjects.

Results: The occurrence of atrial shunts was consistent with autosomal dominant inheritance. Usually shunts were large PFOs, but in some cases they were ASDs. There was also evidence that inheritance of more complex congenital heart disease may be related to the inheritance of PFOs. When the proband had migraine with aura and an atrial shunt, 15 of the 21(71.4%) first degree relatives with a significant right to left shunt also had migraine with aura compared with three of 14 (21.4%) without a significant shunt (p < 0.02).

Conclusions: There is dominant inheritance of atrial shunts. This is linked to inheritance of migraine with aura in some families.

  • ASD, atrial septal defect
  • PFO, persistent foramen ovale
  • persistent foramen ovale
  • atrial septal defect
  • migraine
  • migraine aura
  • cyanotic congenital heart disease

https://doi.org/10.1136/hrt.2003.025700

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    Footnotes

    • Conflict of interest: None

    • Contributions of authors: PW conceived the study, collected and analysed the data, and wrote the first draft of the paper. PW and MP performed the contrast echocardiograms. SN did the neurological assessment of patients. KW and WM performed cardiac catheterisation and transcatheter closure of atrial shunts. All authors amended the paper and approved the final version.