# What is this?



## Medico (Jul 20, 2013)

For the life of me, I cannot remember what this lady had going on. 

Called for a 33 year old SOB. On scene we find a 33 year old white female sitting on her couch, in obvious emotional distress. AOx4, GCS 15. She states that she has been having shortness of breath for the last four hours. She woke this morning to find her hands and feet blue. They were indeed cyanotic, cool to the touch. Radial pulses present and regular. Pedal pulses absent, +1 pitting edema new onset. Pain described as a pressure 10/10, she requested that we cut her feet off or lance her feet to remove the pressure. Breath sounds clear, equal bilaterally, non labored. 

Pulse: 100 weak and regular.
BP: 70/52
RR: 18, 100% 4LPM, 100% RA. 
ECG: Sinus Tach, negative ectopy.

NKDA
Takes lisinopril.
Hx of heart valve replacement. 
The above MAR and PMH is all we were able to obtain.

Please excuse my brevity, I'm typing this on my phone.

*EDITED*

She also had cyanosis of her nose.


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## VFlutter (Jul 20, 2013)

aust10n said:


> Hx of heart valve replacement.



Which heart valve? Systolic/Diastolic Murmur?

Valve replacement in a 33 year old is concerning for congenital abnormalities or endocarditis. This could be an acute valvular insufficiency or an embolic event.

Any JVD?


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## Medico (Jul 20, 2013)

Chase said:


> Which heart valve? Systolic/Diastolic Murmur?



Unknown. She was a poor historian.


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## VFlutter (Jul 20, 2013)

aust10n said:


> Unknown. She was a poor historian.



Did you listen for a murmur? You can usually figure it out with an assessment.


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## NomadicMedic (Jul 20, 2013)

Acrocyanosis is what the condition is called. Important to know if it has has it happened before. Risk factors for PE or other embolism? How long has she been taking the Lisinopril? Is she urinating as normal? ETCO2 waveform and capnometry? 

*Zebra of the day* TM: renal tubular acidosis secondary to ACE inhibitor.


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## VFlutter (Jul 20, 2013)

I really want to say PDA but I assume it would have been repaired when they replaced the valve. But the surgical repair could have failed.


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## Medico (Jul 20, 2013)

I did not listen. 
I believed it was acrocyanosis too, but is it not acrocyanosis when they are cyanotic else where as well? 

She has been on the lisinopril for 2 years.


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## VFlutter (Jul 20, 2013)

aust10n said:


> I did not listen.
> I believed it was acrocyanosis too, but is it not acrocyanosis when they are cyanotic else where as well?
> 
> She has been on the lisinopril for 2 years.



I do not want to harp on it too much but cardiac auscultation can give you a ton of information but is rarely done, not only in EMS. But I am biased since all I do is listen to hearts all day. 


Acrocyanosis is usually not painful and not accompanied by other symptoms. At least from what I understand.


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## Medico (Jul 20, 2013)

Chase said:


> I do not want to harp on it too much but cardiac auscultation can give you a ton of information but is rarely done, not only in EMS. But I am biased since all I do is listen to hearts all day.
> 
> 
> Acrocyanosis is usually not painful and not accompanied by other symptoms. At least from what I understand.




I'll confirm with those who were on the Medic unit what her cap. was and waveform. Unfortunately, I did not get to run this call to the hospital and did not get to participate with the initiation of all treatments/assessments.

I agree with you. Its a habit I need to get into. One I will promptly begin. 

I understand acrocyanosis the same as you. This is one reason I'm questioning.


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## hogdweeb (Jul 20, 2013)

Chase said:


> I do not want to harp on it too much but cardiac auscultation can give you a ton of information but is rarely done, not only in EMS. But I am biased since all I do is listen to hearts all day.


 Please excuse this... cardiac auscultation. What points do you auscultate? with lung auscultation you auscultate fields of the lungs. heart, not having as much surface area would be harder to auscultate, or would you go for the atrium and ventricle? would there be any benefit for a Basic doing it? Could a basic do it and learn anything about the pt.'s current illness?


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## abckidsmom (Jul 20, 2013)

hogdweeb said:


> Please excuse this... cardiac auscultation. What points do you auscultate? with lung auscultation you auscultate fields of the lungs. heart, not having as much surface area would be harder to auscultate, or would you go for the atrium and ventricle? would there be any benefit for a Basic doing it? Could a basic do it and learn anything about the pt.'s current illness?



Watch this. http://www.youtube.com/watch?v=cE8X1nwZWC4


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## Medico (Jul 20, 2013)

The transporting medic unit did not do capnography. 

She had acute rightsided CHF, stated the MD. However, that does not explain the cyanosis, which he did not know what it was either. 

The pt said multiple time throughout transport that she believed she was going to die. I'll follow up and provide an update.


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## jefftherealmccoy (Jul 21, 2013)

12 lead?


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## MSDeltaFlt (Jul 21, 2013)

Sounds like she threw a clot somewhere important.


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## Medico (Jul 21, 2013)

jefftherealmccoy said:


> 12 lead?



Below is a 12Lead from the call.


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## Amberlamps916 (Jul 21, 2013)

Interesting 12 lead consistent with right-sided cardiac pathology. I second the possibility of a clot.


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## Medico (Jul 21, 2013)

Addrobo said:


> Interesting 12 lead consistent with right-sided cardiac pathology. I second the possibility of a clot.



But what area could a blood clot be to cause isolated cyanosis to only the hands, feet, and nose?


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## MSDeltaFlt (Jul 21, 2013)

aust10n said:


> But what area could a blood clot be to cause isolated cyanosis to only the hands, feet, and nose?



I'm thinking the same clot that caused the sys of 70.


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## tacitblue (Jul 22, 2013)

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

In these patients, long standing left to right shunting from a PDA, ASD, or VSD leads to increased RV volumes and pulmonary artery pressures. Without intervention, intimal scarring and irreversible pulmonary hypertension results, and outflow from the right ventricle is obstructed. Reactive right ventricular hypertrophy begins to develop. Eventually, the septal defect becomes the path of least resistance and the shunt reverses, causing cyanosis, shortness of breath, and signs of right sided failure including edema and hepatic congestion.

Your patient's dyspnea along with cyanosis, edema, and right ventricular hypertrophy on the EKG are strongly suggestive. This is a rare entity and the ED physician would have diagnosed her with right sided failure, but I have a suspicion that a chest xray would show evidence of pulmonary hypertension and an echo would demonstrate a right to left shunt, which is really the only reason for bilateral and central cyanosis.


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## Amberlamps916 (Jul 22, 2013)

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.


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## tacitblue (Jul 22, 2013)

Addrobo said:


> 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.


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## Carlos Danger (Jul 22, 2013)

tacitblue said:


> 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


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## Summit (Jul 22, 2013)

Halothane said:


> 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


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## Carlos Danger (Jul 22, 2013)

Summit said:


> 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


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## Summit (Jul 22, 2013)

Halothane said:


> 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:


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## tacitblue (Jul 22, 2013)

Halothane said:


> 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.


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## Summit (Jul 22, 2013)

^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.


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## Medico (Jul 24, 2013)

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.


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