A deadly case…can you figure it out before the autopsy?

Hi everybody, welcome back on EMDutch. As promised I am working on “diuretics in AHF” right now. To be honest, it doesn’t seem to be the cornerstone of treatment in my mind, but that will be discussed in my next post. Because these CAT’s (“critical approved topics”) take a lot of time I thought it would nice to present a case for you today!
Every doctor in his career will encounter patients he or she will never forget. Sometimes because of their appearance, attitude or disease, but in this case it was the outcome……DEAD! And off course we often see people die in the ED. And off course you kind of get used to it. And off course it’s part of the job. But when an otherwise healthy men, 50 years of age, dies a couple of hours after you have seen him it tends to stick! Getting interested??? Just read the case below. Hopefully you will learn something from it and otherwise you can always e-mail/tweet/post me what a bad job I did! Please don’t scroll to the end, but test yourself and try to find what I missed, while reading the case!

So this 50 year old men was seen by one of my colleagues in the ED with acute onset of chest pain without radiation. He has hypertension in his medical history and had no prior episode of chest pain. He was hemodynamically stable. His ECG showed minimal ST-elevation in V2-V3 and an inverted T-wave in III and aVF. On the chest X-ray a slightly widened mediastinum was found which was explained by the technique of the photo. The cardiac enzymes were negative and he was admitted for chest pain with low molecule heparin and ASA.
The ergometric stress test showed no ischemic response and ultrasound of the heart showed a slighty widened aorta ascendens (44mm) and a bicuspid aorta valve. He developed abdominal pain with fever and an elevated WBC and CRP and was transferred to internal medicine. The abdominal ultrasound performed showed no pathology.
On the 3rd day he got a second episode of severe chest pain, which was almost gone when I came to take a look at the patient. I was in my first year and did my rotations in cardiology. Like with every chest pain patient in the house I was called to take a look at the patient. He looked fine and was hemodynamically stable. There were new changes on the EKG this time (see below). And because I was in Emergency Medicine I knew Amal Mattu (really, who doesn’t???)….and when you know him, you know EKG’s…and in this case pericarditis. The EKG showed ST-elevations in multiple leads, so I asked myself the 3 questions to differentiate between pericarditis and MI:
1.) Is there any ST-depression (beyond aVR & V1)  No (we can debate about lead III & aVF, but it was exactly the same as in the 1st EKG.)
2.) Is there ST-elevation covex upwards or horizontal  No
3.) ST-elevation III>II  No
THIS MEANS NO MI, IT MEANS PERICARDITIS!

SR, 65/min, 1st degree AV-block, concave ST elevation I, aVL, V2-V5, inverted T wave III, aVF and aVR

He got NSAID’s next to the antibiotics he already was given and his symptoms disappeared. But….In the 5th night the patient suddenly died.
So what did I miss? I had no idea! Was it a MI after all, was it a PE….or was it something else??? Well, it was something else! Autopsy showed an aortic dissection type A with massive hemopericardium.

So where did I go wrong and what were the clues I missed? In retrospect there were 3 (and if you more, please let me know!):
1.) 1 diagnosis is just 1 diagnosis, but maybe not the whole diagnosis. Pericarditis may present as a sudden sharp pain, but other causes like MI, PE, GERD and dissection has to be in your mind. It wasn’t in mine. I stopped at the 1st diagnosis! Boy, did I learn my lesson!
2.) In 1987 Saner He, et al (1) already describes 5 cases in which pericarditis was the 1st sign of aortic dissection (type A). The hypothesis is that slow pentration of blood into the pericardial space causes inflammation. It’s striking that 2 of these cases had a bicuspid aortic valve, just like our patient. Even before 1987 Edwards (in 1978) already describes the relation between dissection and bicuspid valve (2) In my defence….at the time I had no knowledge of this and it’s sad to figure this out when it’s too late for the patient.

3.) This patient had chest- and abdominal pain. So he had pain above and below the diaphragma. Like somebody recently told me (I know…too little too late!)….think dissection ! One article showed that in dissection 67% has chest pain, 84% had sudden onset of any pain and 23% had abdominal pain (and 64% has a history of hypertension !!!) (3)

In conclusion:
An aortic dissection is a difficult diagnosis to make. That’s why it is important to know it can initially present as a pericarditis. There is often a ‘therapeutic window of treatment’, which means you have time on your side to get to the correct diagnosis in time.
This case doesn’t justify a CT with every patient presenting in the ED with a pericarditis, but in retrospect our patients had some signs which could have led to the correct diagnosis. He had a widened mediastinum and a bicuspid aortic valve which is a known risk factor for aortic dissection. Futhermore he has chest- and abdominal pain !

Ok….hopefully you have enjoyed reading this case and remember….aortic dissection is ‘the great masquerader’ and you will miss some in your carreer, but maybe this can help you to find more aortic dissections in your carreer! Good luck!

Until next time !

Egon Zwets

References:
1.)
Chest. 1987 Jan;91(1):71-4.
Aortic dissection presenting as pericarditis.
Saner HE, Gobel FL, Nicoloff DM, Edwards JE.

2.)
Circulation. 1978 May;57(5):1022-5.
Dissecting aortic aneurysm associated with congenital bicuspid aortic valve.
Edwards WD, Leaf DS, Edwards JE.

3.)
JAMA. 2002 May 1;287(17):2262-72.
Does this patient have an acute thoracic aortic dissection?
Klompas M.