The circle of life!

[VentMedic]

The differential physical assessment will have to be done very carefully. This is something that just has to be stressed.

As I said earlier, there are many disease processes that mimic CHD.

PPHN is not limited to diaphragmatic hernia or mec aspiration but can happen to almost anything associated with a cardiorespiratory compromise at birth or before.

We may have to use 100% O2 for PPHN and as low as 14% for cyanotic heart disease. We want a high SpO2 in PPHN in hopes to decrease the PVR and low SpO2 of 70 - 80% to keep the PVR from decreasing and keep the PDA from closing. Keeping an adequate HCT will be necessary to maintain O2 carrying capacity.

Nitrogen and CO2 may be two of the gases uses instead of O2 to mix on the gas blender. One will also need the ability to buffer the body as you monitor the acid base. However, each of the buffering agents have their own set of problems.

And, if you recognize an infant in distress with a possible cardiac disorder, just because a NICU is level 3 certified does not necessarily mean it can handle cardiac babies for emergency interventional procedures as in a pedi cath lab. For some of the cyanotic hearts, an atrial balloon septoplasty may have to be done immediately to increase mixing. If you have a choice of NICUs, choose carefully.

The Norwood procedure has been very successful and many of these babies are able to live into adult life before they need their heart transplant. There are many now of adult age, some near 50 y/o so don't be surprised to see them in your ambulance. I always stress to EMTs to read the charts and get a history. Not all hx of CHF is just CHF nor is the sternal scar from a CABG.

[chbare]

Good job Streethealer, looks like we nailed the diagnosis.

Ventmedic, I understand, I simply threw out a couple of common causes of PPHN.

I hope you enjoyed this scenario and I hope you were able to learn about other conditions and the physiology of some of these problems. One more consideration, some of these conditions will have long lasting effects and problems into adulthood. The patient who inspired this scenario is actually a young adult.

Take care,

chbare.

[Mateo_1387]

Thanks for the scenario chbare. I appreciate the chance to have a challenging review of congenital heart defects.

I have two questions. Can you explain the finding of an enlarged liver? Is it due to a backup of blood in the venous system? Also, with the picture provided by streethealer, it shows space where the left ventricle should be as a large muscle mass. If the picture is accurate, why would it show RAD? I would think the large left ventricle would show LAD.

Thanks

Mateo

[Eydawn]

I saw that picture as the right ventricle extending (where we can't see it) into the space where the left usually should be, with the left only being the open part shown...

But maybe I'm wrong. Visual arts has never been my strong suit.

Wendy

CO EMT-B

[chbare]

I am glad you enjoyed the scenario.

Essentially, yes, the right ventricle is simply not designed to function physiologically like the left ventricle. Back up of blood can occur, hence the enlarged liver. This is not seen with every case of hypoplastic left heart syndrome. In addition, you can have a left to right shunt of blood from the left atrium. This shunt; however, can be restricted by the size of the foramen ovale. If you have significant resistance through the ovale combined with the resistance met when blood tries to push through the mitral valve, you can easily appreciate how pulmonary venous back flow can occur and ultimately, severe CHF like signs.

As far as RAD: remember the ECG measures the wave of depolarization. In hypoplastic left heart syndrome you may or may not have a significant amount of tissue in the area of the left ventricle; however, you still have a highly underdeveloped chamber with limited contractile action. The right ventricle is doing all of the work, hence, this is where more of the electrical activity will occur. Thus leading to RAD and RVH. I hope that makes sense.

Hopefully this image will help you understand. You can clearly see the underdeveloped left ventricle compared to the large right ventricle. Subsequently, ultrasound is a valuable tool in diagnosing hypoplastic left heart syndrome. Note: Shamelessly taken from emedicine.

Take care,

chbare.

[chbare]

After some discussion via PM, I want to emphasize that this scenario is not designed to make anybody an expert on CHD's. Again, the situation was unrealistic and simply inserted as a way to push things foreword a day. In addition, CHD's are complex disorders and the diagnosis of an infant in distress involves a complex team approach. Obviously, this was not the case with this scenario. Again, my scenarios are simply about learning.

Take care,

chbare.

[Mateo_1387]

Thanks. The picture clears it up. I can see the enlarged right ventricle on the picture you provided. With the right ventricular wall that size, I can "see" the RAD. The other picture is a bit misleading.