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Nitric Oxide in CDH

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I have a a 30 day of neonate with CDH. never been  extubated. Got him down to 50% and in 0.5-1ppm of Nitric Oxide. Have tried weaning him slowly of the NO on multiple occasions. Always go into 90%  TO 100% Fio2. Already on maximum doses of Sildenafil. Not oedematous on PCAC 20/5 with good CO2 clearance. I cannot get the final bit of NO off.


Any strategies from the forum would be greatly appreciated.

PS Operated not paralysed synchronising well good drive

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What about his ECHO finding still have severe PHTN or improving from last ECHO

If he is improving wait and see continue your weaning trials

X ray chest is there is improvement in the hypoplastic side take care of sildenafil as some times causing lung collapse

Just be patient

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We have also had some success in this difficult scenario with nebulised prostacycline as an adjunct therapy to sildenafil. Has to be done two hourly...but has done the trick!

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Good work @spartacus007, would you be kind enough to share with us the X-ray finding before surgery?
Was the hypoplastic lung apical? or hilar or you couldn't identify it?

Would you share the current BP and ECHO results?

Failure or difficult weaning of inhaled NO for treating pulmonary hypertension in hypoplastic lungs in CDH and preterms is not uncommon . Concerning your presented case, In our NICU settings we would prefer using HFO using MAPs 2~3 higher than your MAP on conventional (Go slowly on the increase in MAP increase by 1). We would prefer the HFO not to injure the hyoplastic lung and to recruit the lung volume to improve oxygenation and ventilation.  Continue to increase your MAP to lower the FIO2 to below or =30%, but be careful not to over inflate the lungs which would  be seen by rebound increase of FIO2 needs during your gradual increase in MAP or by X-ray seeing the size of the heart and level of the diaphragm.

In addition,  in our NICUs setting for PPHN due to CDH we would start Dopamine (1st increase SVR ) and according to the ECHO findings on TR and direction of flow through the PDA we would start NO  at 20 mpp (2nd) if TR is still persist. BPs, follow up ECHO heart to see the effect on TR + FIO2 needs would be our guides. If still PH persist, we would add Milrinone (3rd).

Sildenafil is used in our setting to wean off NO or if the above 3 medications were not controlling the PH.

In your current setting, still FIO2 is 50% , Still needs to go down on the FIO2 before withdrawing your NO.

I would work on recurring the lungs first before weaning NO totally ( as presented above).

Hoping our settings could help you with your patient.

Good luck.




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Thanks guys we are already on Noradrenaline infusion and minimal ventilation with good CO2 clearance. Have you got a dose for inhaled epoprostenol



Also on Milrinone

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@spartacus007 the epoprostenol dose (in this paper: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3342750/) is:


The epoprostenol was diluted for continuous nebulization at a rate of 8 ml/hr, and a dose of 50 ng/kg/min, or intermittently as 50 ng/kg diluted in 3 ml of diluent.

We have used the intermittent dose of 50 ng/kg (typically in term born infants on CPAP, with PPHN, and FiO2  ~0.9-1.0)

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On 10/28/2017 at 8:31 AM, Stefan Johansson said:

How are things going @spartacus007? Any progress? 


Guys we struggled with everything so I got a CT angigram as lungs were wet. Confirmed a infracardiac TAPVC with scimitar syndrome. Inoperable hence we have moved to palliative care.

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