Rachel's aortic dissection patient story

Rachel recounts her personal story which started with a diagnosis as a young child.

‘I knew I had Marfan Syndrome from around the age of 7 or 8 years old, but I was diagnosed even before this following genetic testing and various other tests- unfortunately I found - I was a spontaneous mutation. For as long as I can remember I have needed regular heart check-ups although my heart hadn't really changed much over the years - I just had a leaky but stable mitral valve and high blood pressure but my aorta measurements were typically stable.

I had been under the care of my local hospital for my heart from a young age until around age 20 when I began going to Manchester Royal Infirmary for my annual heart reviews. Again, everything remained stable for the majority of my twenties. However, after close monitoring, my aortic root measurements began slowly creeping up, so the team kept a close eye on me.

Unfortunately, that all changed when in March 2022 I suddenly experienced regular sharp shooting pains one morning, going through my chest to my back which progressively got worse. 

With how severe the pain felt, I knew something was seriously wrong which sent sheer panic through me. It got so bad I couldn't move or speak and was struggling to catch my breath. I felt even more fearful because, at the time, I was home alone.

I managed to ring my parents to tell them what was happening so they could return home.  We rang for an ambulance but was told it was a 4-5 hour wait.  I knew I couldn’t wait that long – I felt like I was having a cardiac arrest or dying. We rushed to hospital, and I informed them I had Marfan Syndrome. After several tests, I was eventually told I was having a TYPE B dissection.

What followed was a 3-week hospital stay and now my Type B is medically managed. For this monitoring and my on-going care, I was transferred to Liverpool Heart and Chest Hospital. I have experienced a lot of severe pain in my life, but that pain was off the scale.

I thought I was dying.

This all came as a massive shock to me as my team at Manchester had only recently talked about keeping a close eye on the aortic root and that my other measurements were stable. Another reason this was a huge shock to me, was because I had never been told anything about the severity of aortic dissections and how they could be fatal. Nor had I ever been told that I was at an increased risk due to having Marfan Syndrome. Looking back now, I can't believe I wasn’t made aware of this life-threatening risk.

Fast forward nine months and I needed to have a valve sparing aortic root and arch replacement via the frozen elephant trunk surgery. This was unfortunately an emergency operation. I have been under Liverpool Heart and Chest Hospital since I dissected and now return for checks for my heart and aorta review every 6 months. 

The team at LHCH led by Miss Harrington have been excellent throughout. It took me a long time to recover from both traumatic events which was prolonged due to Marfan Syndrome which delays healing. This was also complicated by my severe scoliosis, another factor in delaying my recovery. I am now doing as well as can be expected but the physical and mental impact has certainly left its toll.

I have been through a lot of severe traumas in my life, and I’ve have found it hard to find people who can understand and relate to everything that you have been through. This is why am so grateful to be a part of the aortic dissection buddy group, as people understand the physical and mental impact that this imprints on your life.

I am scared for the future as I know I will need more surgery but, I know I am strong and resilient enough to cope with what the future has in store for me.'