Hot Seat #4 Denouement: RUQ Pain and Complex Cardiac History

The case
by Mel Tavarez
It is 4am on your overnight shift on the main campus, and a 17 y/o F with a complex cardiac history including HLHS s/p Norwood and extra-cardiac Fontan presents with 1 day of RUQ pain. She states the pain was initially intermittent and generalized, but after 3-4 hours became constant and localized to the RUQ; she describes it as pleuritic. She traveled to Florida 2 wks ago and has had non-bloody, loose stools. Remainder of ROS including fever, SOB, CP, N/V, dysuria, hematuria, vaginal discharge, anorexia, calf pain/swelling and sick contacts is negative.

Of note, she has a history of DVT 2 years prior after a hip fracture related to MVC. Her only medication is aspirin and there is a family history of gallstones.

Her triage VS include temperature 37.8C, HR 88, RR 20, BP 119/83, Sats 99% on RA in triage. When you see her, she is splinting and her sats are 94% and she says it hurts under her rib cage on the right side when she breathes. She has a III/VI holosystolic murmur and clear BS. She has localized RUQ TTP without guarding, but you do note a Murphy’s sign. Otherwise, her exam is normal. Specifically, there is no leg swelling or tenderness. 

Update: She has an initial chest x-ray that shows no lung pathology.

You plan to work the patient up with initial labs and imaging. 

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The Teaching Attending Hot Seat Response
by Jo Cohen 
Well, there are a lot of leading questions towards PE here in the case presentation. To Sabah’s point about the confusing nature of complex congenital heart disease repair, I try to simplify it in my mind. This patient was born with single ventricle physiology and therefor that one single ventricle needs to be working on systemic circulation. Therefore, I can presume that this extra-cardiac Fontan , without even being able to visualize where in the body this conduit is, must involve some sort of passive pulmonary circulation, in which case this patient has a low flow system that predisposes her to thrombus formation and therefore PE. The fact that this patient has had DVTs in the past certainly doesn’t make me feel better.

The patient’s initial presentation, including normal trial VS and denial of SOB or CP, does not sound like a PE. Although she describes her pain as pleuritic, it localizes to the RUQ of the abdomen, not to the chest. However, you mention that on evaluation she is actually hypoxic and splinting. I think a screening D-dimer is warranted based on this, but I would not jump to a Chest CT yet. My understanding is that the D-dimer has a good negative predictive, but there are a lot of false positives. In this case, given the low pretest probability based on the history, if the patient had a negative D-dimer, I would forgo the CT scan. If the D-dimer was elevated, I would err on the side of caution and get the CT.

Having worked out in my mind how I would handle her workup of the thing most likely to kill her, I’d turn my attention to other likely things on my differential diagnosis. Cholelithiasis or cholecystitis would be unusual unless she was obese, but we can rule this out pretty quickly with the bedside ultrasound. I would check her LFTs as Dewesh mentioned and her T bili on the comprehensive panal will also help reassure us that she doesn’t have GB disease. If I remember correctly hyperbilirubinemia can also give you a false positive D-dimer so I would keep this in mind if her Tbili is elevated, her D-dimer is elevated and her CT chest was negative. Her LFTs will help us evaluate liver pathology. I would also take a sexual history. Hey, she’s a teenager. She could have Fitz Hugh Curtis or maybe she’s having a panic attack because she’s actually pregnant and by the way, pregnancy causes an elevated D-dimer too!
Basically, I would put her on some oxygen for comfort, give her some pain medication and order a CBC, CMP and D-dimer and go from there….

Your Polled Responses 

Would you include a D-Dimer in your initial orders?

Would you place this patient in ETU status and ultrasound in the morning?

Would you do a spiral CT now?

If you decided to send a d-dimer, the result is 1832 ng/mL; it is now 6am, would you do a spiral CT at this point? 

The Denouement

by Mel Tavarez

The patient’s HEADSS assessment was positive for SA with 1 male partner, no vaginal discharge or dysuria. Our initial plan was to send a CMP, CBC, UA, urine HCG and d-dimer given the patient’s past medical history and the pleuritic nature of her pain. We obtained a CXR after the d-dimer result which was negative on our read. Her CBC had a normal WBC with a left shift; electrolytes were normal; total bilirubin was slightly elevated at 1.2 with normal ALT, AST, and alkaline phosphatase. Her urine HCG was negative and her UA showed pyuria without nitrites. At the time of signout at 7am, the plan was to go ahead with an ultrasound since she was HDS and reassess need for further PE workup if the ultrasound was non-diagnostic.

Her ultrasound showed a thickened gallbladder without cholelithiasis or ductal dilatation and a sonographic Murphy sign. Her urine GC/CT were negative. She was admitted to GI service and received meropenem with a presumptive diagnosis of acalculous cholecystitis. Her PO intake improved and she was discharged within the next 24hrs on PO ciprofloxacin to have an elective cholecystectomy as an outpatient.