Practice A Patient’s Journey

External aortic support for people with Marfan’s syndrome

BMJ 2010; 340 doi: (Published 18 May 2010) Cite this as: BMJ 2010;340:c1692
  1. Camilla Allen, patient1,
  2. John Pepper, professor of cardiothoracic surgery2
  1. 1Thornbury, Bristol
  2. 2Imperial College, Royal Brompton Hospital, London SW3 6NP
  1. Correspondence to: C Allen allen{at}
  • Accepted 15 March 2010

In 2007 Camilla Allen became the ninth person with Marfan’s syndrome to have an external aortic root support wrapped around her dilated aorta. She has since become the first woman with an exostent to become pregnant and give birth.

Stopped in my tracks

In October 2006 I agreed to take part in research related to Marfan’s syndrome being undertaken at St George’s Hospital in London. The research involved detailed measurements of my heart being taken using echocardiograms. I did not give my participation a second thought until a couple of months later when I received a letter from St George’s telling me that my aortic root diameter had expanded to 4.3 cm and that with such a dilated aorta I should not conceive a child due to the risk of fatal aortic dissection. This news was a devastating blow to my husband and me as we had been trying to conceive our second child for the previous nine months.

An appointment with my local cardiology consultant was hastily arranged for January 2007. The measurement was confirmed, and, although it was not particularly worrying in itself, as most patients undertaking root and valve replacement surgery have aortic root diameters nearer the 5.0 cm mark, it showed that mine had increased since my first echocardiogram at the age of 14 and had worsened during my first pregnancy. The consultant considered surgery in the form of the Bentall procedure, a composite aortic root replacement, which is a necessary precaution against a 1 in 10 chance of aortic dissection during pregnancy or labour. Suddenly a 10% chance of my dying brought the situation into sharp focus.

Since first being diagnosed with Marfan’s syndrome as a teenager, I had always considered my diagnosis to be an inconvenience rather than life threatening. The condition is only mild in my family, …

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