Every now and then Dr. Cranky picks up a chart from the rack and, even before he walks into a patient’s room, his spidey-sense starts to tingle. This is almost always a bad sign and such was the case last night with a patient we shall refer to as Mr. A.
Your humble servant picked up Mr. A’s chart and quickly perused the nurse’s note before venturing forth. Dr. Cranky learned a long time ago that he should always read the nurse’s note before anything else since it can literally save the patient’s life, as well as his own backside. Although her handwriting was almost as bad as Dr. Cranky’s, the triage nurse had written, “Sudden onset of chest pain while walking in a store. Chest pain resolved. Now with back pain and leg numbness.” A quick glance at the patient’s age revealed he was 52 years old, not much older than Dr. Cranky himself. With just this small amount of information your faithful host started to formulate his differential diagnosis, a list of possible pathological processes which might produce the patient’s aforementioned symptoms. When he looked at his patient’s past medical history, he read the following words: “Marfan’s Syndrome.”
This was most concerning. Marfan’s Syndrome is an inherited disorder in which there is a weakening of connective tissue, the stuff which holds strategic parts of the body together. Patients who have this condition can have some interesting traits, such as the ability to do this:
Unfortunately, one of those strategic parts is the connective tissue which binds the layers of the aorta together. For those of you unfamiliar with basic anatomy, the aorta is the main artery which carries blood from the heart to the rest of the body. It consists of three layers: the intima (or innermost layer), the media (or middle layer) and the adventitia (or outermost layer). Every time the heart beats it generates a pressure wave which moves blood downstream so it can do all the good things blood likes to do. An aortic dissection occurs when a tear takes place in the intima and the blood ,under pressure, peels away this inner lining from the rest of the artery.
This is a very bad thing and can kill in short order. And, people who are afflicted with Marfan’s syndrome have this happen with disturbing frequency.
Dr. Cranky went into the room to speak with his patient. At first glance, Mr. A did not look Marfanoid. Such folks tend to be tall and lanky, have a scooped out appearance of the chest (also known as pectus excavatum), and are blessed with long, thin fingers (arachnodactyly). They usually have a distinctive facial appearance, possess a highly arched palate with the teeth crowded together and wear glasses with thick lenses because of severe myopia. Mr. A did not have any of these features but, as is often the case with emergency medicine, the lack of a “textbook” appearance didn’t mean a thing.
Mr. A told Dr. Cranky he had been walking in a local department store when he suddenly experienced a brief episode of severe left-sided chest pain which lasted a few seconds and then resolved. He said he currently felt fine except for an aching sensation in his buttocks and feet as well as numbness in both legs. He had no other symptoms and claimed an otherwise negative past medical history. He did smoke, which is not a good thing for someone prone to cardiovascular problems, and imbibed alcohol only occasionally. And yes, his father and uncles had a nasty problem with sudden death in their late 30’s.
His vital signs were unremarkable and he had a normal blood pressure. His heart rate was not elevated. A quick physical examination revealed no evidence of a heart murmur which would have alerted Dr. Cranky to the presence of aortic insufficiency, a malfunction of the aortic valve which occurs in Marfan’s because the proximal aorta dilates where it joins up with the heart. His abdomen was soft with no pulsatile masses. In fact, his exam was completely normal except for one disturbing finding: there were no pulses over the femoral arteries at the top of his thighs, the popliteal arteries behind his knees or the posterior tibial and dorsalis pedis arteries in his ankles and feet. Dr. Cranky also noted that his patient’s lower extremities were pale and cool to the touch. Thus Mr. A had four of the Five P’s of Arterial Insufficiency (i.e.Pain, Pallor, Pulselessness, Paresthesia, and Paralysis). Dr. Cranky was quite concerned that this lack of blood flow through the arteries leading to Mr. A’s legs was the direct result of an acute aortic dissection
To cut to the chase, Mr. A was showing all the signs of a patient who, in emergency medicine parlance, was suspiciously close to FTD or Fixin’ to Die. In his storied career Dr. Cranky has seen patients just like this who crashed and burned before his very eyes with no advance warning. Your correspondent would like to report that, just as you might see on a bad episode of ER, he grabbed a scalpel from a nearby tray, opened Mr. A up and replaced his aorta right there in the Emergency Department; and before the commercial break no less! No, I’m afraid that in a situation like this Dr. Cranky is somewhat less dramatic. This is not a manhood issue; it is simply a question of good patient care. You see, Mr. A had chosen to come to a hospital where there was no vascular surgeon available; and a vascular surgeon was exactly the sort of person he needed at a time like this.
At this point, Dr. Cranky had two options as to how he could proceed:
- With the first option, your faithful host would send blood work to the lab, start an IV, obtain a contrasted CT of the chest and abdomen to confirm the diagnosis and then arrange for transfer to BCMC (Big City Miracle Center), where the Pros From Dover do their medical magic. The problem was that it would take at least an hour or two to get all the lab and CT results back, followed by another 45 minutes to get permission for transfer, make sure the EMTALA paperwork was filled out correctly (federal law don’t you know), wait for an EMS unit to arrive and then whisk Mr. A off to those loving arms which would await him at his new home-away-from-home. This is not a good plan for someone with acute FTD Syndrome.
- The second option would be to call BCMC and arrange for immediate transfer based on clinical suspicion alone, which would save a lot of time. We could send off the appropriate laboratory studies, which the good folks at BCMC would have access to on their computers, and fill out the EMTALA paperwork while waiting for transport. Upon EMS arrival, Mr. A could then be on his way within two shakes of a lamb’s tail. During all these events, an airway cart would be moved to the bedside and two large-bore IV’s would be started just in case things took a sudden turn for the worse. Dr. Cranky’s Residency Program Director, The Big C, would have been proud.
Dr. Cranky chose Option 2. It was a no-brainer, really. Sorry to disappoint all you ER fans out there.
To make this long-winded story short, EMS arrived and spirited Mr. A out of the department none the worse for wear. About an hour later Dr. Cranky received a call from Dr. B, the emergency department attending physician at BCMC to report that a CT had demonstrated Mr. A to be the proud owner of a Type 1 Aortic Dissection. This means that Mr. A had separated the lining from the biggest artery in his body all the way from his heart down to his legs. The vascular surgeons were taking him to the OR immediately but, as it usually happens in cases like this, Mr. A was not expected to survive.
Dr. Cranky called BCMC the next day in the hopes of hearing good news, only to discover that Mr. A never made it off the operating table.