Ignited by an intricate network of nerves, pain is our personal in-built alarm system that protects us from harm. When it is felt in a vulnerable area, one that has been surgically treated, this will naturally trigger alarm bells. The father of a 15-year-old Marfan boy recently called our Helpline to say his son ‘C’ was experiencing chest pain. C is a successful recipient of the PEARS procedure and mitral valve replacement. He returned impatiently to the (often virtual) classroom and football pitch feeling the benefits of a newly healthy heart. All was well until a fortnight ago when C suddenly felt an intermittent cramping pain in his chest that soared alarmingly over one day. The father wondered if dural ectasia was the catalyst.


Q: As you well know, my son and your patient underwent the PEARS Exstent surgery and mitral valve replacement at the Brompton in August 2019 at the age of 13. All has gone well until a fortnight ago when he felt stabbing, cramping pains in his chest. The pains have become became more persistent and he’s now in our local A&E, seemingly cool yet frightened underneath. (He’s normally unflappable). C is also becoming increasingly breathless, noticeably so during his short walk to electrocardiogram x-ray unit. Could this be dural ectasia? HIs blood pressure is also worryingly raised.


A: No. Dural ectasia causes pain in the lower back, the abdomen, the head and also the thighs. The pain should not reach the chest. It could be pneumothorax which is a collapsed lung and causes both shortness of breath and pain.

However, it could also be muscular pain given that schools are now back. C will be carrying heavy loads of books to and from school and will be exercising more. The pain could easily be attributed to this sudden change in his regime. Has he taken up tennis for example?

Whilst at your local A&E, C should also be given an echo but there must be someone there to analyse the results. Ideally, he would be seen at the Brompton Hospital where he had his aortic surgery, in its specialist cardiac unit


CODA

The father reported that "C was released from our local hospital with a paracetamol dose after an ECG showed nothing of note. They deduced from a blood test that looks for a clotting fibrin there was no dissection. He was not given an echo since there was no one on duty overnight to decipher and translate the results. Dissatisfied with our local hospital’s diagnosis we contacted the liaison nurse at the Brompton who arranged an emergency appointment the next day. C underwent a CT scan, an echogram and an ECG. They diagnosed fluid around his heart which was thought to be the result of a recent infection of unknown origin. This is causing the pain! They sent him home with a prescription for two weeks of ibuprofen.

Interestingly, Brompton Hospital were furious that C had not been referred to their their 24-hour Rapid Access Unit: for Brompton Hospital patients only." However, most UK cardiac surgery centres have a similar post-operative liaison system in place, and patients are given the contact details upon discharge.

C has since returned to school and the football pitch!

(By Dr Anne Child & Victoria Hilton)

The Marfan Trust is registered in England and Wales under charity number 328070 at Guy Scadding Building, Dovehouse Street, London SW3 6LY. Contact us at [email protected] or by phone on + 44 (0)20 7594 1605
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