Atypical presentation of acute aortic dissection in a young competitive rower

Abstract

A 27-year-old man (who is also the lead author) presented with dull pain deep to the suprasternal notch, following a period of intense exercise (rowing). He was initially sent home with no diagnosis, but 24 hours later returned to a different Accident & Emergency (A&E), due to continued discomfort and an increasingly altered mental state, and was diagnosed with an extensive type A aortic dissection extending from the aortic root to the iliac bifurcation of the aorta, with an ~8 cm aneurysm on the ascending aorta and a diseased aortic valve. Following emergency surgery to replace the aortic valve and the aorta from the aortic root to the middle of the aortic arch (hemiarch), the patient recovered well.

Background

Aortic dissection (AD) is a rare but life-threatening condition in which a tear occurs in the wall of the aorta (typically the intima layer) and allows blood to flow between the layers. It was first documented in 1760 by Frank Nichols in his autopsy report of King George II, who is reported to have suffered a fatal AD while straining on the commode. The illness is considered interesting as it has a very high mortality rate (33% within 24 hours and 50% within 48 hours if undiagnosed)¹ and a varied presentation, which requires a high degree of clinical suspicion, to diagnose within the short time frame typically available.

This case is considered particularly interesting as:

• The patient had none of the typical risk factors (hypertension, cocaine use, marfanoid appearance, bicuspid valve,² etc.) and was much younger than most cases of AD. However, he had been rowing just prior to onset.

• The presenting complaint was ‘dull pain at the bottom of my throat’, which is very different to the classical symptoms of ‘tearing pain between the shoulder blades’, or even syncope. This led to his being sent home from Accident & Emergency (A&E), where he stayed for almost 20 hours. It is classically said that acute type A AD carries a mortality rate of 1%–2% per hour.³

• The diagnosis may have been made earlier by checking blood pressure (BP) in both arms, or checking for radio-radial delay at an earlier stage (cannot say this definitively because it is not clear if differential pulse was already present at initial presentation).

Case presentation

A 27-year-old man (who is also the lead author) presented to A&E with low-level dull pain deep to the suprasternal notch (reported by the patient as the bottom of his throat), which intermittently radiated to the left shoulder, breathlessness, physical weakness and an altered mental state, all of which developed acutely, during moderate exercise, half an hour after rowing at high intensity for 2 hours. The patient was athletic, smoked only very occasionally (1–2 cigarettes/month), and had no known genetic conditions or relevant family history.

Investigations

Following an ECG, Full Blood Count (FBC) and BP measurement conducted on one arm only, which all showed no irregularities, he was discharged with no known diagnosis. The following day, he returned to a different A&E, with the same symptoms and a ‘feeling of fullness’ in his left arm. After some time, BP measurements were recorded bilaterally and found to be 190/74 mm Hg in the left arm and 74/34 mm Hg in the right arm. Radio-radial delay was also present. A CT scan showed a dissecting aneurysm (see image) with the dissection extending from the root of the aorta to the iliac bifurcation of the aorta. The aneurysm was located on the aortic root and had a diameter of ~8 cm.

Differential diagnosis

Initial differentials included anaphylaxis (he was sweeping a dusty area when the symptoms appeared) and an allergic reaction to the influenza jab (which the patient had received 2 days earlier). Cardiovascular illness was initially discounted due to a normal ECG and FBC, the location of the pain and absence of risk factors.

However, once radio-radial delay and the difference between L and R arm BP readings were detected, the patient was sent for a CT scan (figure 1), which confirmed the suspected diagnosis of type A AD.

Figure 1.

CT scan of patient showing dissecting aneurysm on the ascending aorta and continuing dissection in the descending aorta.

Treatment

Emergency open-heart surgery was successfully performed, replacing the aorta from the aortic root to the middle of the aortic arch (hemiarch) with a Dacron graft and replacement of the aortic valve with a prosthetic mechanical valve. During surgery, it was noted that the patient did not have a bicuspid aortic valve.

Outcome and follow-up

The patient recovered well, although suffered an episode of atrial flutter 1 month postsurgery, which was treated with an ablation procedure. He still has remnant dissection of the descending aorta, with abdominal branches being perfused off both the true and the false lumen, which are both patent. He takes warfarin (target International Normalised Ratio (INR): 1.5–2.5), bisoprolol (2.5 mg), doxazosin (2 mg), aspirin (75 mg) and ranitidine (150 mg b.d.). His BP is well controlled (approximately 115/70 at rest) as is his heart rate (resting HR: 55–60).

Because of the residual dissection causing weakness of the aorta, and the possibility of some undiagnosed connective tissue disorder, it is important that the patient avoids any activity that could cause spikes in his BP or place additional strain on the arterial walls. This is true of all patients who survive an acute AD; however, very little is known about postoperative exercise limits, and given that moderate exercise is important to maintain good heart health and limit future risks, clinicians have to find a balance when providing advice. Recommendations for activities of 3–5 Metabolic Equivalent of Tasks (METS) for at least 30 min on most days and avoidance of any lifting, which leads to straining, have been made⁴; however, a survey of clinicians found great variability on the BP and HR limits that they recommend to their patients.⁵

Discussion

There are numerous case reports detailing dissections related to weightlifting.^6–8 Often, these cases are reported in individuals without marfanoid appearance (presumably because those with a known connective tissue disorder are advised of the risks). Given that the movement when rowing resembles a dead lift, it is unsurprising that rowing could carry the same risks; however, no other documented cases could be found.

The diagnosis of the condition still remains overlooked on initial presentation, and even with modern imaging techniques, the rate of missed diagnosis ranges from 15% to 30%.^9–11 The actual level may be higher than this, as many cases are only discovered on postmortem. This case represents a good example of an initial misdiagnosis that could possibly have been avoided had bilateral pulses, radio-radial delay and BP differential been assessed on initial presentation. Bilateral pulses are a particularly important bedside test as pulse deficits are present in approximately 1/3 of patients with AD and are strongly correlated with mortality (2.7 times higher when pulse deficits are present).¹²

Patient’s perspective.

When my symptoms first appeared I found breathing quite difficult for 5–10 min and was gasping for air, however, after that feeling passed I didn’t actually feel particularly unwell. I didn’t feel much pain, more of a discomfort, which felt like it was in my throat. I did however, feel that something was wrong, but perhaps in my desire to not panic and to not be seen to panic by my peers (I had just started at medical school and probably wanted to avoid developing a reputation as someone who made a fuss), I didn’t initially make much effort to impress this point on the medical staff who saw me. I’m sure patients frequently say things like ‘as soon as it happened I knew something was wrong’ and retrospectively claim to have known what was happening, and I imagine these claims are (sometimes probably correctly) treated with a degree of scepticism by medical staff. However, having gone back over WhatsApp messages that I sent to friends during the period after I’d been sent home from the first A&E department, I can see that I did suspect that there was something more serious occurring.

When I returned the following day to a different A&E department, my mental state was still slightly altered and I felt similar to how I had the previous day (although quite tired as I hadn’t really slept). I remember feeling like the doctor I saw didn’t believe me when I said that it felt like my blood pressure was higher in one arm than the other, and, when again an FBC and CXR didn’t show any abnormalities, he was keen for me to go home. I had to press reasonably hard for this not to happen but, after my BP had been measured in both arms, I was seen by a consultant who very quickly checked for radio-radial delay and sent me for a CT scan. I don’t remember too much after this, but I do recall seeing the surgeon and being told that my chances of surviving the operation were about 80%. I’m not sure I really had time (or the mental clarity) to properly absorb the implications of this, but I did appreciate the honesty and clarity with which he laid this out.

After the operation I felt very well cared for in the hospital and actually rather enjoyed my time there. I think in my head, I was imagining that I would swiftly make a full recovery and that everything would soon be back to normal. I remember explaining to a nurse, 6 days after my operation, that I had to go as I had an exam that I needed to sit. I was still quite convinced that I would soon return to my studies, but over the next 2–3 weeks it became clear that my cognitive abilities weren’t returning quite as quickly as I’d hoped and when I had atrial flutter and required an ablation, it was clear that I wouldn’t be able to return to my studies that year. That was quite hard for me to accept, but more importantly, it gradually became clear to me over the next few months (I think I had probably been told this all along but was just reluctant to accept it), that I would never be able to return to what I considered a normal level of exercise. Prior to the surgery, sport and exercise had played a huge part in my life and formed an integral part of how I perceived myself. I used to cycle to university every day, cycle out to Surrey at the weekends, train with the rowing team most days and row on the Thames at the weekend, I also used to go cycling for nearly all my holidays. Now I struggle to walk 1 mile without needing a prolonged rest afterwards. Losing that has had a huge impact on me and required a lot of gradual adjustments in how I see myself and also how I cope with stress, build friendships and gain pleasure in life. While I feel like I’ve had fantastic care at almost every stage, one thing that I think has been consistently underappreciated is the impact that this has had on me. Perhaps clinicians are used to seeing this sort of condition in much older patients, so aren’t surprised or overly concerned when their physical capacity is drastically reduced afterwards, but I can’t really over-state the effect it’s had on me.

Having said all of that, I feel extremely fortunate to be alive and very grateful to everyone who’s treated me along the way. Plus I quite like my scar.

Learning points.

• Aortic dissection (AD) may not present with high level pain (indeed, approximately 5% of cases present without pain¹³) and the pain may occur sufficiently high up for cardiovascular causes to be incorrectly discounted.

• AD is uncommon, but can often be fatal and should not be easily discounted, even when the patient lacks classical risk factors and symptoms. Pulse deficits are an important test as they help identify dissections and predict mortality.

• Intense exercise, particularly that which is known to cause dramatic spikes in blood pressure (eg, weightlifting, rowing and sprinting), is a risk factor for AD. This also means that patients who survive an AD should be advised to avoid these activities.