Pulmonary Hypertension

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I currently work in a NICU but in two weeks (eek!) am transferring to my hospitals Peds Cardiac ICU. I know I will run into pulmonary hypertension in this unit but was wondering how many patients end up with it about? My NICU seems to breed it :mad: and I lost one consistent a week after she was due to go home, from pulm htn, and my current consistent went from doing cpap trials on her trach to being diagnosed with severe pulm hytn, is now back on ino & the hfov (and for the past 3 weeks has been on milrinone/sildenafil/bosentan prior to becoming this sick). I feel as though my nicu looses a lot of pulmonary hypertension battles and I hate it :crying2: Is it likely to be any better in the CICU? For the record I don't think my unit treats appropriately or pays attention to the early warning signs until it's too late (despite many nursing observation and reports of different symptoms etc) however once diagnosed they are followed by the cardiac ICUs pulm hypertension team.

Also, I understand the general patho of pulmonary hypertension but was wondering what causes pulmonary veins to stenos? (in my unit its the chronic lung, BPD, usually micro preemis, but not always, that end up with pulmonary hypertension).

Thanks!

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Have you had a run of kids with pulmonary vein stenosis? That's a fairly rare cause of PHTN and the atypical presentation can make it difficult to diagnose. It can actually present with things like recurrent pneumonia.

Overall, PVS leading to PHTN has a pretty tough prognosis. I know that there is a center or two trialing the use of Avastin, with or without Gleevec.

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hmm, from what I understand, there have been at least two babies recently (neither being my consistents but ones I have cared for) who were both micro/iugr babies. One is now 7ish months, the other coming up on his 1st birthday. Both have had severe pulmonary hypertension for a long time, both have been in and out of critical states, one trached, one is not. The one not trached recently went on a downhill slide and had a cardiac cath which found 3 of 4 pulmonary veins stenosed...this being different from a previous cath, the almost 1year old has 2 or 4 pulmonary veins stenosed. From my understanding the stenosis have been recent developments in these kiddos, not the primary cause of the pulmonary hypretension...I could be wrong though and like I said, my unit is sometimes slow to diagnose these kids. I do know that the PHTN team pushes to get these kids cathed if they can to rule out PVS in order to give the parents better understanding of prognosis, but in both situations babies were doing well and relapsed severely

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Specializes in Pediatrics, ER.
Have you had a run of kids with pulmonary vein stenosis? That's a fairly rare cause of PHTN and the atypical presentation can make it difficult to diagnose. It can actually present with things like recurrent pneumonia.

Overall, PVS leading to PHTN has a pretty tough prognosis. I know that there is a center or two trialing the use of Avastin, with or without Gleevec.

We've got two babies with PVS, one of them has atresia and almost 90% of volume going through one lung. We use sildenafil, hctz, aldactone, chlorothiazide, and lasix, accompanied by high PC conventional vent. Still doesn't stop the sudden pulmonary hypertensive crises, but does decrease the frequency of them. Occasionally a few hours of paralysis and sedation are needed but overall they're both doing amazing.

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what do you use for paralysis on these kids? in my nicu we use Vec which I saw can cause drops in HR when used with narcs...had a baby months ago who died, presumably from severe PHTN - she was on morphine/versed/vec drips and was continuing to have acute crisis with drops in sats/HR (i remember a time she was fine, satting 99%, her vent heater beeped and she coded), very difficult to ventilate, during her last few days we spent literally hours hand bagging her, she was so stiff, very little chest rise, trialed the hfov but that didnt work (she was also on ino/sildenafil/bosentan/milrinone). Two weeks prior she'd been on .25L NC, getting ready for discharge...she was my primary and to this day her death haunts me...I've wondered if we could have given her too much morphine & vec to cause such low heart rates...but we couldn't get her chest to rise well either, it will always bother me (an xray we got right before she died also showed a small pneumo though the doc thought it was probably insignificant, when her parents were told she would need a chest tube thats when they (her parents) called it, they had been with her 24/7 all her days at the hospital)...I sincerely hate PHTN but have recently found that the unit I am moving too gets all the phtn kids that aren't in nicu so i'm not about to get away from it at all

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Specializes in Pediatrics, ER.

Usually IM fentanyl for initial sedation followed by IV vecuronium and ketamine. As far as I know these patients never received morphine. I have yet to see bradycardia with the crises, it's usually tachycardia to the low 200s or self-resolving SVT. Do you do ECMO in your unit? Was she a candidate? It's only natural as a nurse to let things haunt you but you know she was a very sick baby and that you can only do so much. PPHN is a silent sneaky killer. You can vasodilate and reduce the myocardial workload until the cows come home but not much can break through that severe chest wall rigidity that comes with CLD/BPD. Just know that it gets a little better with kiddos who are a little older and more resilient.

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Specializes in Pediatrics, ER.

By better, I mean they tolerate the low sats and respiratory distress that comes with a crisis better than tiny infants. Many times just paralyzingly and sedating for a few hours is enoug to bring them out of it. This has been my experience.

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Usually IM fentanyl for initial sedation followed by IV vecuronium and ketamine. As far as I know these patients never received morphine. I have yet to see bradycardia with the crises, it's usually tachycardia to the low 200s or self-resolving SVT. Do you do ECMO in your unit? Was she a candidate? It's only natural as a nurse to let things haunt you but you know she was a very sick baby and that you can only do so much. PPHN is a silent sneaky killer. You can vasodilate and reduce the myocardial workload until the cows come home but not much can break through that severe chest wall rigidity that comes with CLD/BPD. Just know that it gets a little better with kiddos who are a little older and more resilient.

we do do ecmo, I doubt her parents would have allowed us to put her through it (she was actually due to go to cath lab the day she died, we all think she would have come back on ecmo), she was not really a CLD/BPB kiddo but she had laryngealmalacia - probably should have had a trach but she compensated for so long, so well that no one really thought of it till too late. She was an ex 35wkr, about 5 mos old, initially at an OSH, then home, came to us with a bad URI, s/p cardiac arrest after botched intubation (also a critical airway), she recovered and was getting ready to go home when the PHTN reared its ugly head. We use morphine & versed as sedation in the NICU, I know they use other things in the PICU/CICU, but not us, we even had a pain team consult on her and they didn't give us any new recs which is weird because they had put a few of our PHTN kids on hydromorphone.

As much as I hate it I am looking forward to going to the cardiac icu and learning other, maybe better ways of management than we use in the nicu.

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Specializes in Pediatrics, ER.

Had she had a cath before? Honestly it sounds like there was some other underlying problem, or her maybe her condition was improperly treated from birth at the OSH. I think you can go round and round with the what ifs, but at the end of the day you did the absolute best you could for her, and it sounds like the parents were realistic about her prognosis. Sometimes all the technology in the world isn't enough for those critically ill kiddos, and even if you pull them through this part, their quality of life ends up being extremely poor as they age. There will always be a handful of patients that stick with you, and you just have to keep them in your heart and memory as motivation to care for the next critically ill baby who tugs at your heartstrings.

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she had never had a cath before, it all kind of snuck up on her as we were preparing for her discharge after a very few critical weeks turned stable enough for her to be in three to ones with the other stable feeder/growers. I think I will just always wonder with her, there were days that docs disregarded my concerns (unusual tachycardia, sweating, fast weight gain & edema, sudden increasing O2 requirements), I will always wonder had I been more persistent, pushed a few more buttons, would it have made a difference for her? It's only been a few months since her death but I continue to find myself questioning every new presenting symptom in a kiddo, could this be the start of PHTN? I will always be on the lookout for it

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