Survivor Story of Cliff Grover

Cliff Grover’s Story and how to give yourself the best chance

My reasons for writing this story are to tell others about my experience, help others avoid the problems I had, and let people benefit from my experience. I hope it will be of help.

My enlarged aorta was first identified on echo when being examined for A-Fib at about age 40, 23 years before I dissected. It grew from 3.7 cm in 1996 to 4.5 cm by 2016 (slow - 0.04 mm per year) and the doctors were unconcerned. The basis of their assessment was probably the standard European/US Guidelines figure at the time of 5.5 cm ascending diameter before the risk of dissection becomes greater than the risk of operating. My monitoring interval was reduced in 2016 to every 8 months, with periodic CT checks against echo.

From 2006 to 2016 my blood pressure was slightly high at 140/90 but we could not get it down. In 2017 it started to increase further and I was waiting for referral to a hypertension clinic when we got back from our holiday(!)

To this point, no-one had clearly explained what the risk of my enlarged aorta was. One doctor used the term annulo-aortic ectasia, but from memory no-one had ever explained “aneurysm” or “dissection” to me, nor was the importance of getting one’s BP seriously under control (120 mm Hg) stressed - the UK guidance for hypertension (based on non-aortic risks) is not to treat until 140/90 is exceeded. However, I did put an emergency card in my wallet and had an ICE app on my phone which both mentioned the enlarged aorta.

In Dec 2017 my wife and I travelled from Scotland where we live to New Zealand to visit our daughter. I was 63. On 22nd Dec I dissected when I took a small jump to reach something whilst working outside my daughter’s house. I felt a sudden crushing pain in my chest and felt this could be the heart attack pain people describe, of an elephant standing on your chest. I made it indoors, called out “chest pain” and collapsed on the floor. I also lost my vision.

Paramedics arrived in 14 minutes and took me to hospital. My vision returned in the ambulance, though blurry, and I could hear the medics radioing in my case saying they thought it was a heart attack, but they weren't sure. Presumably the loss of vision did not tally, and I have since learned that an MI does not normally cause either such sudden ‘thunderclap’ pain, or a collapse - MI’s are not as dramatic as often depicted on TV.

On reaching hospital my ECG showed ST elevation and bloods were taken. I was sent to the Cath Lab for angiogram. A narrowed coronary artery was identified, this was stented and I was put on Ticegralor and aspirin anti-platelets. On return to the ward I did begin to feel better, and remember texting friends back home that I had had a heart attack. “You could have had that at home” was one reply.

With ongoing pain relief I forget the next 24 hours or so but my pain did not stop and the hospital also checked and rechecked my bloods which indicated this was not primarily an MI, in spite of the earlier presentation and findings. I was sent for CT.

As I was wheeled in to the CT room I recognized the white doughnut of the machine and for the first time suddenly recalled my aorta, and told the nurse. They quickly found my type A dissection and I was returned to the ward and prepared for surgery. The surgeon explained to my family that the anti-platelets I had received for the stent and ‘MI’ were going to make things difficult but that they would manage these with blood products - and that not operating was not an option.

I went to surgery at 14:30 on 24th Dec. My dissected aorta was measured at just below 5 cm which, as they grow slightly at the point of dissection, may have still been around only 4.5 cm pre-dissection. A 14-hour operation (through to 4:30 a.m. on Christmas day) provided me with a new mechanical aortic valve Bentall graft, ascending and arch grafts, a Frozen Elephant’s Trunk (FET), plus a CABG and some new plumbing around my aberrant coronary artery configuration. My surgeon identified a tear and a nearby PAU (penetrating atherosclerotic ulcer) underneath the arch below the left subclavian, plus a contained rupture(!) The surgeon’s sketch I have also shows an intramural hematoma extending back to the coronary ostia, probably blocking them or at least severely reducing blood flow to give the symptoms of a classic MI. I subsequently discovered this does occur in a minority of AD cases. From the surgeon’s sketch it seems to me that a dissection flap and/or the hematoma had possibly caused my loss of vision, perhaps until a false lumen established. The FET was necessary due to friable tissue in my descending aorta which was refusing to suture - 7 hours into the surgery.

The extensive operation was in part made possible by my surgeon using a special technique he had jointly developed to ensure blood kept flowing to my brain. This allowed him a much longer time to deal with the challenges of deferred diagnosis, anti-platelets and friable tissue, and in the end he was able to fully repair me and said I should not need another operation. I was incredibly lucky to be in these hands.

I had an eventful 6-week ICU stay with pneumonia, heart pauses, bloated guts, a tracheotomy, every drug they put me on created a different problem, a cardiac arrest, severe problems withdrawing sedation, ICU delirium and a plan for a pacemaker when I was well enough for another procedure (never in the end done). Plus all the time I was having the craziest dreams - but they’re not dreams, you forget dreams. After a further 2 weeks on the Cardiac ward and 2 months at my daughter's I was allowed to fly home.

I’m updating this story at 5-1/2 years. These years have been tough with a constant search for the right combination of drugs, including for post-operative atrial fibrillation where I already had a prior history of AF and an ablation in 2002. A number of medication adjustments partly helped with my overall weakness, but nothing has returned me anywhere near my original fitness. I took an 8-week cardiac rehab class - lasting 5 months - which initially helped and for a while I was able to walk 3 or 4 miles and get back on my mountain bike for gentle rides. However, things started to deteriorate again. COVID came along when I was 2 years out from the op, and delayed further investigation into my debilitation. Once lockdowns were lifted I eventually got seen but though the first tentative diagnoses were of PICS (post-intensive-care-syndrome), MRI scans showed nothing apparently wrong and I am now waiting for re-review. We did find I had an extraordinarily high Lp(a) figure. This is a cholesterol-like substance not normally tested for which has some associations with aortic problems. My figure was 205, where above 30 is normally the trigger for concern, so this may have contributed to my PAU and perhaps dissection.

I learned a few lessons which can be useful for anyone incidentally diagnosed of aortic disease, to help minimize the risk or outcome of a serious event. These lessons don’t infer that I am critical of any of the care I had before - quite the reverse - I just feel that the best armoury if you discover you have aortic disease before it strikes unannounced is knowledge, so work together with your doctors. Bear in mind I am not a doctor but I believe the following is all correct:

• I dissected below the 5.5 cm guideline (now reduced to 5.0 cm for surgery at high-volume centres) and would dearly love that no-one else ever finds themselves in the same place, but predicting and preventing an aortic event is still an imprecise science, with a lot of uncertainty. In this situation, I strongly feel it is up to the individual to educate themselves and with the help of their doctors map out a path which truly minimizes the risk of an emergency.

• When a person has an enlarged aorta (perhaps found incidentally like mine), or has other risk markers such as a connective tissue disorder (Marfan etc.), a bicuspid aortic valve, unusual vasculature, or a family history of aneurysm or dissection, they need to be seen and monitored by a specialist aortic team. There is a limited number of specialist aortic hospitals but if you can get under their wing that is best, plus you can establish your emergency pathway to them if the worst comes to the worst. Non-specialists will largely depend on national guidelines on aortic disease management and these are necessarily based only on well-established science. But inevitably this means the guidelines may not be as up to date as a specialist will be in what is a fast-moving area of medicine; it can take a long time to get updates to guidelines into general use.

• Whilst genetic testing is as yet relatively limited (around 30 known genes at present, and most dissectees do not have a known abnormality), I was not tested at all prior to my dissection. I did not know it was advisable and practice has moved on from when I was first diagnosed. If you are found to have an aortic risk, you really need to know next if you have a genetic susceptibility.

• Genes and diameter work together. Firstly, I strongly feel the guideline 5.5/5.0 cm figures must not be used in the manner of ‘below 5.5/5.0 cm is safe’. The 5.5 cm figure was developed in the late 1990’s on the data available, but in 2007 a major study found that nearly 60% of actual dissections occur below 5.5 cm, creating an apparent paradox. The original 5.5 cm was a statistical assessment of a particular cohort with already quite large aneurysms, not an indicator of exactly who will dissect amongst a much larger group with somewhat smaller aneurysms. Genes are a major part of this equation and the intervention diameter has slowly crept down over the years, to as low as 4.0 cm for certain cases. Best practice now is firstly to know any genetic pre-disposition - if you don’t know you have a particular gene, how do your doctors know what diameter you should be monitored against?

• The balance of risk between early elective intervention and risking a dissection has changed over time, with surgical techniques constantly being improved. The risk of elective operation is reducing all the time, and this has in part contributed to the reduction to 5.0 cm for high-volume centres.

• Blood pressure must be well controlled. An aorta at the 5.5 cm criterion and a BP of 120 mm Hg is under the same degree of stress as a case of BP of 160 mm Hg, but at only 4.1 cm. This is a simplistic view, but demonstrates the quantitative effect BP can have. Aortic disease is a different and more direct risk to normal hypertensive risks behind the UK (and elsewhere) 140/90 guidance. A 2022 study showed dissection risk increases above 110/75.

• Know that if the worst happens, AD is a rare and difficult diagnosis and if you have any prior indication of aortic disease, help the ED team to help you. The team that saw me and initially went down the route of MI is one of the most experienced in the world, with a top aortic centre within the hospital, and yet it took them time to overcome AD's tendency to masquerade and my own comparatively rare presentation of an already rare condition. If only I had managed to tell of my aortic risk straight away.

• So, know your numbers, know your risk, and if the worst happens you must be able to communicate that. You need to ensure the message cannot be missed – always wear a medical alert bracelet. My phone ICE/wallet card warning, my own knowledge, and my family’s knowledge were never communicated due to circumstances - everyone was too worried and I was ‘out of it’ with pain. And if you are getting strange other symptoms or pain elsewhere from your chest/back, tell them; often these are peripheral indicators of dissection and not a separate problem.

~Good luck, but remember - you can make your own luck. #ThinkAorta.

*I’d like to finish by thanking everyone involved in my case - firstly my wife who had a horrendous and worrisome time whilst I was away with the fairies on morphine, my daughter and her husband who took us into their home for as long as needed, my son who flew out to NZ with his father in a precarious position, and then of course my surgeon and his team, the ICU and ward personnel there. A big thanks also to all other family and friends who supported me. Lastly the entire medical team looking after me back at home and the various support groups I have joined and who have helped me through the difficult days. Thank you all.