Survivor Story of Cliff Grover

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

My main reasons for writing this story are not only to tell others about my experience but to try and help others avoid in the first place the problems I had, and to let them benefit from my experience. So here goes.

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. I don't know what the basis of their assessment was but I suspect it was against the standard European/US Guidelines figure 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, plus periodic CT checks against echo.

From 2006 to 2016 my blood pressure was slightly high at 140/90 but it was resistant to most drugs and/or I suffered unacceptable side effects. But in 2017 it started to increase further and I was waiting for a referral to a hypertension clinic as soon as I got back from our holiday (!).

I had previously had very little explanation of what the risk of my enlarged aorta was. I had heard the term “Annulo-Aortic Ectasia”, but from memory (which may be faulty) no-one had ever said “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 is not to treat until 140/90 is exceeded. I did, however, put an emergency card in my wallet and I had an ICE app on my phone which both mentioned the enlarged aorta.

In Dec 2017 my wife and I traveled 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 was possibly 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 sight which, strangely and for some unfathomable reason, didn’t worry me.

Paramedics arrived in 14 minutes and took me to hospital. My sight returned in the ambulance, though blurry. 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 sight 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 you see 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 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 I had not in fact had an MI, in spite of the presentation and findings. At that point I was sent for CT.

As I entered the CT room, I recognized the white doughnut of the machine and for the first time suddenly recalled my aorta, and told the nurse. My type A AD was quickly found 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 the 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 (so, up to 4:30 a.m. on Christmas day) provided me with a new mechanical aortic valve Bentall graft, ascending and arch grafts and a Frozen Elephant’s Trunk (“FET”), plus a CABG and some innovative plumbing around my “aberrant” coronary artery configuration. The Surgeon identified a tear underneath the arch below the left subclavian, and I surmise (supported by my reading of the surgeon’s sketch) that a dissection flap and/or the hematoma had possibly caused my loss of sight, perhaps until a false lumen established. The sketch I have also shows an intramural hematoma extending back to the coronary ostia, probably blocking them or at least severely reducing blood flow and this is what had given the symptoms of a classic MI. I subsequently discovered this does occur in a minority of AD cases. The FET was necessary due to friable tissue in the descending aorta which was refusing to suture.

The extensive operation was in part made possible by the surgeon's use of a special technique to ensure blood kept flowing to my brain and provide “cerebral perfusion”. 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, as they say, 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 op (never in the end done). Plus all the time the craziest dreams I had ever had - but they’re not dreams; you forget dreams. I then had 2 weeks on the Cardiac ward and 2 months at my daughter's for follow-up etc. before being allowed to fly home.

I’m writing this at +30 months and the intervening period life has been tough with a constant search for the right combination of drugs, including for (post-operative) Atrial Fibrillation of which I had a prior history and an ablation in 2002, but which was occasionally still bothersome. I’m just at the point where we have carefully withdrawn the statin and the beta blocker, both of which were of limited benefit in my case but which I tolerated badly. An 8-week cardiac rehab class - lasting 5 months - helped greatly and I am now able to walk up to around 3 or 4 miles and have even been back on my mountain bikes round local lanes and tracks (gently!). I’m at long last feeling a lot more confident about the future although sleep is still a problem, which may be an aspect of the PTSD I suffered in the first 18 months. I was just getting to grips with all this as the COVID-19 lockdown hit, but this was no more limiting to me than had been my previous 2 years since dissection. “Welcome to my world, World!”

I learned some lessons and formed opinions which I feel can be useful for anyone diagnosed early of any aortic disease, to help minimize the risk or outcome of a serious event. I in no way mean these lessons I learned to 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 and would dearly love that no-one else ever finds themselves in the same place, but predicting and preventing an aortic event is still a science in relative infancy. 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, they need to be seen and monitored by a specialist aortic team, of which there are not many. Hospitals which carry out sufficient numbers of aortic repairs are very specialized and few in number and if you can get under their wing that is best, plus you can also 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 takes 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: I strongly feel the guideline 5.5 cm figure must not be used in the manner of “below 5.5 cm is safe”. This guideline was developed in 2002 on the limited data available, but in 2007 a major study published that nearly 60% of actual dissections occur below 5.5 cm. The 5.5 cm was a population based statistical assessment, not an indicator of exactly who will dissect amongst a risk group. 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.

• Blood pressure must be well controlled. As an engineer I can calculate that an aorta of 5.5 cm diameter with a BP of 120 mm Hg is under the same degree of wall stress as the case of a BP of 160 mm Hg (my eventual BP), but at only 4.1 cm. This is a simplistic view, but demonstrates the quantitative effect BP can have. Get your BP to 120 or lower; aortic disease is a different and more direct risk to those from general hypertension and the reasons behind the UK (and elsewhere) 140/90 guidance.

• 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 a way in which the message cannot be missed – always wear a medical alert bracelet. My phone ICE/wallet card warning, my own knowledge, and my wife'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. Think Aorta.

I can’t leave this story without thanking everyone else involved - 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.