IV access, O2, NS bolus, D51/2NS maintenance, cont pulse ox, cbc, chem8, ua, lfts, lactate, ammonia, blood cx, abg, urine cx, esr, cxr, ekg, abx, LP would be clinical judgement for me but from everything described, not sure I'd go there quite yet but certainly would be very near on my "next to do" list of diagnostic tests. If he gets abx in the ED, still have window of 2-3 hours to get LP without abx affecting results. If I had time I would do a bedside cardiac UTZ as long as pt was stable.
I'm not really comfortable playing the guessing game with a 2 week old and am not a neonatologist or pediatrician. What concerns me at this point from everything described, are primarily airway concerns, hypoxemia, and hypotension, possible sepsis. The kid is retracting, and visibly with some element of respiratory distress, tachycardia, tachypnea, element of hypoxemia, etc.. already meets SIRS criteria. If I end up leaning towards sepsis pathway, then the kid gets an LP.
The important matter though is that the kid is coming in, not going home, and he's going to the PICU/NICU very shortly and may or may not require intubation soon if his respiratory rate does not decrease or he manifests further signs of respiratory distress. I'm assuming he doesn't have a PDA or we would have heard that on auscultation but a closing one is certainly on differential. Don't know what to make of the decreased BP in LE's vs UE's other than possible coarctation? (Would also explain pre and post coarct/ductal oxygen saturation. Either way, he's coming in and needs an ICU bed, not my job to sit on this kid playing Sherlock Holmes in the ED for an extended period of time waiting for some form of "recognizable badness" to happen. Soon as I get all this stuff going, ICU is getting immediately consulted. It's a 2 week old, and I don't have time to play Humphrey Bogart in the room while waiting to see if this kid crumps.
Emergent concerns: Hypoxemia, Respiratory distress, hypotension, possible sepsis. --> ICU and they can figure out the rest, and to hell with it, I'd prob do the LP too. I'm taking no chances with this kid and truth is I don't know what's going on and with a normal night (lots of other kids rolling in) I don't have as much time to sit down and think as I would probably like. Obviously history is vital, but from everything that has been described, nothing is jumping out at me and smacking me on the head with a big "AHA!" stamp. My 2 cents.
I think your thinking-as the emergency room doc-is spot on. Yours is not to figure it all out. That said, if you're a two hour ride from The Big Children's Hospital and weather isn't going to allow the copter to fly, you still may have ongoing stabilization to accomplish. I think the general DDx has been laid out pretty well. I'd argue, still, that having a basic cardiac DDx and idea of what may be or may not be seen is actually helpful to the ED doc in this case.
Great thought on repositioning. Makes no difference in this case, however.
No one else at home is sick. The infant has had 4-6 wet diapers a day and has had mustard looking BMs (not white), several a day. UOP has only been 2 in the past 12 hours or so, which mom says is less than she usually goes, but he's also not feeding as well. She seems fussy and then stops feeding early. Mom hasn't noticed any sweating.
Here's the rest of the exam:
General: Ill-appearing, alert, fussy with a weak cry
HEENT: Dry lips, nasal congestion, PERRL
Chest: Tachypneic, intermittent grunting, CTAB with good air
entry, + subcostal retractions
CV: tachycardic, no murmur or gallop appreciated
Abd. Soft, ND, + BS, Liver edge ↓ 2 cm
Ext: cool, delayed CR of 3 sec, radial pulses 1+, pedal pulses not
readily palpable
Skin: Mottled
The nurse moved the probe to the right hand and sats look a little better at 97%. Four extremity BPs were also obtained:
RUE- 95/57
RLE- 75/43
LUE- 90/65
LLE- 79/53
People have mentioned fluids and oxygen. Any hesitation or concern about giving either one? Antibioitics? Would you attempt an LP first?
Ditto. Plus, coarctation can be associated with other cardiac abnormalities such as transposition of the great vessels, VSD, hypoplastic left heart, etc. I'd also be on the lookout for sepsis tipping the kid over given a <30 day presentation.
Interestingly, plain vanilla transposition (+/- intact septum) doesn't have a coarctation usually. More commonly associated with pulmonary stenosis. When associated with certain forms of double outlet right ventricle (particularly the Taussig-Bing defect) CoA is much more common. This is the type of peds cardiology esoterica that the ED doc does not need to know (but it's fun for me
🙂).
These are all good thoughts. You can't always sort out what's going on, but you've hit the big ones: sepsis, cardiac and metabolic. Sometimes I think we can get focused on sepsis and forget the others.
Keep in mind when you see these kids that pre/post ductal sats and 4 extremity blood pressures are easy to get and can give you a lot of information. And you're never wrong for sending an ammonia level.
Here are some labs:
Na 137, K 5.1m, Cl 101, HCO3 24, BUN12, Cr <0.2, Glu94
i Ca++ 1.11
WBC 7.3, H/H 9/27, plt 277
55 segs
4 bands
31 lymph
3 mono
pH-7.203
PCO2-58.2
PaO2-19
HCO3- 23
Base excess- (-)5
Lactate 9.3
NH3 normal (don't have the value on hand, sorry)
EKG is normal (don't have a pic but it is)
Okay, okay, here's your chest x ray (it's not specifically this patient's x ray, but it looks the same). Can't get one by you guys.
😀
Oh yeah, the BNP (as someone above mentioned ordering) was greater than 70,000.
Question for JRad if he's still following: how useful is a BNP? I send them to help convince cards that it's really the heart or to make myself feel better if the number is low, but I don't have great data to support doing that.
Now, with a heart this size, are we giving fluids or holding back? Oxygen? Prostins? What heart lesions cause something like this at two weeks of age? Are we dealing with failure or cardiogenic shock? Both?
Hmm, still think coarctation with possible PDA though not sure how I wouldn't have heard that on careful auscultation. Any chance on that ABG yet? I'm hoping that was a VBG you just gave. I'd also like a lactate from the ABG. After seeing the CXR, obviously cardiomyopathy/CHD, etc.. is slapping me, thinking more cardiogenic shock at this point instead of sepsis. There's a R->L shunt, just trying to figure out where it's coming from, gotta be distal to L subclavian since your pressures are equal in uppers along with SaO2 (might move the probe over to LUE just to make sure it's the same as RUE since I'm neurotic like that).
...I'd give PGE-1 since I'm still worried about coarctation and have no idea what kind of defect we're dealing with, could there be some element of transverse arch hypoplasia?, if so need that ductus open. Can I wake up the peds cardiologist yet? Pretty please?
OK here are my thoughts:
Basic physiologies:
Left sided obstructive lesions
-Cardiogenic shock
-Can have right to left shunt (ex. HLHS)
-Can have differential cyanosis (usually LE sats<UE sats)
-Doesn't have to have differential cyanosis (critical AS)
-Can have left to right shunt (ex. HLHS, Interrupted arch which almost always has a VSD)
--Since L-->R shunt can coexist, O2 is NOT indicated as it promotes overcirculation (PVR should be low in the 2 week old and blood goes to the path of least resistance). You can and will make this child worse. I don't think there is a place for a hyperoxia challenge of any type in this age group. Tolerate sats, say >70% without giving supplemental O2.
-Progressive LV dysfunction caused by obstruction-->CHF (manifests as tachypnea, tachycardia, poor feeding, hepatomegaly, possibly "cardiac asthma" [wheezes and crackles], gallops).
-If this kid is a coarct, why no murmur? What if his EF is 20%? Gradients across obstruction can be low in the setting of poor cardiac output.
Transposition physiology (separate circulations)
-Most typically present early and present really blue-all over, usually. A two week old with a RUE sat of 97% doesn't have transposition.
R-->L shunts
-Tet-varying degrees of cyanosis (pink vs. blue). They all have a murmur. They shouldn't have differential cyanosis. If clinical hx is c/w a Tet spell, they can be acidotic. Go down Tet spell pathway which does include O2. This kid does not have the story or exam findings of a kid in a tet spell.
-TAPVR-the TAPVR presenting at two weeks will have potentially unimpressive cyanosis (80s to even low 90s) and wet appearing lungs on CXR. Feeds may be crapping out, but they don't usually present with acute decompensation and acidosis. Obstructed veins present (sick) early, not at 2 weeks in the ED. The murmur of TAPVR is a pulmonary flow murmur and split S2 (from ASD)
-Truncus is usually a lesion of overcirculation with generally mild cyanosis. Except when there is interrupted arch, which is common with truncus. This little factoid matters not a whit to you because your basic thought/worry will be for a left sided obstructive lesion and you will treat accordingly and the dx can come later.
-There are single ventricle lesions with R-->L shunts without left sided obstruction (ex. tricuspid atresia with normally related great vessels). They can also have large L-->R shunts. If this is the case, their presentation will usually be mild cyanosis, poor feeding and CHF, but not cardiogenic shock. Again, O2 is not indicated.
Large L--R shunts (large VSDs, AV canals, AP windows).
-CHF sans cardiogenic shock unless associated with other lesions.
-No O2.
One take home point: O2 is almost uncommonly indicated in the resuscitation in this age group. If you want to pick a number, let them go down to 70% before giving Os and don't shoot for the moon on sats.
As far as fluids: if you have hypoperfusion, they are indicated, BUT this baby also has hepatomegaly. Give gingerly: 10ml/kg and check liver again. You may not want to give a second. This baby isn't hypotensive, in fact, he's hypertensive. But with a heart like that on CXR, pressors may be near in his future.
As far as the BNP thing, I don't know. We don't use them much other than for trending some of the heart failure patients. The article about the use in the ED was interesting, but I want to know that they matched severity of illness and acidosis (i.e. the septic kids with low BNPs had similar pHs to the cardiac kid with the high BNPs).
Now specific to this case:
Recheck the labs and gas. They do not make any sense for the patient who is in front of you. Did they get mixed up with someone else?
In general: How many ED docs here are comfortable with their staff's ability to check BPs (nevermind 3-4 point BPs) in a baby accurately? Do most of your EDs routinely check BPs in children <3yo? BPs are going to be dubious. If you have time (by patient severity and patient load) try checking Doppler BPs, but I wouldn't work too hard for that. The sats are probably more reliable, and I'm much more concerned with the pulses (before the CXR and [maybe] BNP which have now pretty much pointed your way pretty nicely)
And back to the case: yeah, go ahaid. Start the Prostin.
