What is this?

[tacitblue]

From what I've gathered, someone with Eisenmenger's syndrome is highly likely to suffer from both uncontrolled bleeding due to damaged capillaries and high pressure, and random clots due to hyperviscosity and stasis of blood.

Bleeding disorders in these patients is usually from a clotting factor deficiency because of hepatic impairment. Not all of them present with bleeding problems though.

 

[Carlos Danger]

I am gonna disagree with a thromboembolic event and instead suggest she has eisenmenger syndrome.

Shunt reversal is the first thing I thought of, as well. But if that's what it was there's no way she'd be sp02's of 100%. Also, it seems unlikely that she'd have valve surgery and not have the PDA or septal defect fixed at the same time. Maybe the valve surgery was not successful for some reason?

It's impossible to say without more info, but here's my guess:

Exacerbation of R heart failure (which resulted from a valvular problem) --> hypotension --> anxiety --> raynaud's phenomenon

 

[Summit]

Shunt reversal is the first thing I thought of, as well. But if that's what it was there's no way she'd be sp02's of 100%. Also, it seems unlikely that she'd have valve surgery and not have the PDA or septal defect fixed at the same time. Maybe the valve surgery was not successful for some reason?

It's impossible to say without more info, but here's my guess:

Exacerbation of R heart failure (which resulted from a valvular problem) --> hypotension --> anxiety --> raynaud's phenomenon

First paragraph is what I thought after reading the OP before proceeding, except I didn't proceed to your guess (which I also like) because I was willing to disregard the pulse oximetry and look at the physical findings. I know you know that the pulse ox can be a questionable instrument, particularly in the field and without waveform. So, I was thinking that the PDA was missed initially, incompletely surgically closed, or it closed spontaneously, and later spontaneously opened as an adult (I know this is rare, but there are several documented cases). Afterall, we are finding that with increase premie survival there are a lot of people walking around with asymptomatic PDA that gets picked up incidentally on echo.

This one might be from a little off as I'm trying to remember some long late night discussions with exercise physiology researcher, but there are some theories out there that it is quite common and PDA can spontaneously reopen during profound hypoxia.

That said, Occam would place your guess higher on the differential

 

[Carlos Danger]

First paragraph is what I thought after reading the OP before proceeding, except I didn't proceed to your guess (which I also like) because I was willing to disregard the pulse oximetry and look at the physical findings. I know you know that the pulse ox can be a questionable instrument, particularly in the field and without waveform. So, I was thinking that the PDA was missed initially, incompletely surgically closed, or it closed spontaneously, and later spontaneously opened as an adult (I know this is rare, but there are several documented cases). Afterall, we are finding that with increase premie survival there are a lot of people walking around with asymptomatic PDA that gets picked up incidentally on echo.

This one might be from a little off as I'm trying to remember some long late night discussions with exercise physiology researcher, but there are some theories out there that it is quite common and PDA can spontaneously reopen during profound hypoxia.

That said, Occam would place your guess higher on the differential

Believe me, there are reasons why my proposed explanations are usually very simple

 

[Summit]

Believe me, there are reasons why my proposed explanations are usually very simple

Sometimes it is hard not to think zebras when you work at the zoo...:unsure:

 

[tacitblue]

Shunt reversal is the first thing I thought of, as well. But if that's what it was there's no way she'd be sp02's of 100%. Also, it seems unlikely that she'd have valve surgery and not have the PDA or septal defect fixed at the same time. Maybe the valve surgery was not successful for some reason?

It's impossible to say without more info, but here's my guess:

Exacerbation of R heart failure (which resulted from a valvular problem) --> hypotension --> anxiety --> raynaud's phenomenon

According to the OP, she was a poor historian. She may not have even had a valve surgery, maybe it was a different type. Did she forget to mention other heart problems? Admittedly, shunt reversal is rare in the US, but it happens. Also, I don't know if I trust the oximeter in this case. Where was it applied? How was it getting 100% on grossly cyanotic fingers? This patient had evidence of central cyanosis as well. I would at least keep shunt reversal on the differential. Raynuad's, now that's interesting. Lets put that in there as well. Vasopasm along with panic attack could exponentially her symptoms. There would still be the question of edema to deal with.

 

[Summit]

^indeed hence my previous mention of not believing a 100% pulse ox reading on someone with acrocyanosis. I wouldn't believe any peripheral pulse ox with raynaud's, in fact, a reading should be impossible d/t vasospasm. As I said, waveform is key as is correlation with the rest of the picture. I wouldn't expect the common toxicological reasons for false-high pulse ox readings to result in acrocyanosis.

On that note, 1+ edema in a patient with chronic right-sided HF and profound hypotension indicates that the the onset of this exacerbation is very acute because the patient has only suffered long enough to present limited lower extremity edema... or perhaps another person would rate it at 2+ or 3+... the 0-4+ edema scale has poor inter-rater reliability. To me, the very acute onset is a key finding.

Assesment: With the very apparent RA hypertrophy, fairly apparent RV hypertrophy, and hx of valve replacement, the patient probably has a history of pulmonic stenosis or insufficiency prior to the PVR, possibly complicated by PPH or congenital cardiomyopathy. Nobody is disputing the acute exacerbation of chronic right-sided HF this 33 y/o female with a history of valve replacement. Why is this happening?

DDx:
Replacement valve failure (many failure modes: but since this is hyper acute, we'll go with impingement of a leaflet on a mechanical valve)
Eisenmenger's or spontaneous opening/reopening of a L-R shunt
AV regurg or RV hypomotility from an occlusion of a branch of the PIV artery that doesn't present on a standard 12 lead.

Field EMT-IV tx:
Auscultate heart sounds!
Maintain low flow O2 per protocol (although it is probably doing jack because there is no forward flow into the lungs).
IVx1 18 in the AC is perfect, draw for CMP, h/h, coags, trops, run TKO.
Continuous neuro/EKG/SpO2/Cycle cuff q3-5m
Emergent transport high-fowlers to nearest appropriate facility

If ALS: Contact OLMC for Dopamine at 5-10mcg/kg/min if hypotension becomes symptomatic or there will be a prolonged transport with low MAPs (chronic HF patients are far harder to manage with renal complications).

Facility: CXR, Echo, EKG c cardiology consult, run aforementioned labs, lasix, milrinone and possible dopamine, and the rest depends on diagnostic findings whether straight to ICU or somewhere else.

 

[Medico]

Thanks everyone for the response. I always enjoy to read others opinions and the direction of their diagnosis and treatment.

I followed up on the patient today. She was admitted to ICU and died 20 hours later. She was diagnosed with the following; Infected tricuspid valve (which was probably the repaired/replaced valve), septic emboli, and DIC. I had never heard of or seen a patient with the above. This patient was one I'll never forget, and learned a lot from. I especially learned a lesson (although it would not have changed this patients outcome) to begin listening to all of my patients heart tones.