“Brrriing!” My entire world vibrated. I jolted awake in a panic, automatically reaching for my glasses. As the world blinked into existence, I sighed mentally. I was in the call room, and that was the sound of my pager going off. I glanced at it and promptly took off in a run. Midnight emergency C-sections generally did not bode well.
The OR was in a flurry of commotion; the OB was barking orders, and anesthesia was working on the spinal. I made my way to the infant warmer, where the nurse, Dana, and the respiratory therapist, Krista, had already arrived and were setting up. The 2nd-year resident, Cody, was also there, looking eager for action. Dana leaned towards me. “Term, poor prenatal care. She came in with ruptured membranes this morning. Ultrasound shows CDH. Someone said something about the liver being up. Started having recurrent decels in the last hour.”
I swallowed. We’re a level III NICU without ECMO or pediatric surgery. We don’t do CDH repairs here. But our immediate goal was to stabilize. And we had no idea how sick this baby would come out. I rolled my shoulders. “Okay, let’s assign roles. Then let’s set up the bed and intubation supplies.”
I caught the OB resident’s eye. “Please hand the baby directly to our team. No skin‑to‑skin in the DR. If the baby is vigorous, let’s aim for cord clamping for 30-60 sec, but no delays if that is not the case.”
The baby arrived with a strong cry, good tone, but immediate moderate retractions and cyanosis. Classic scaphoid abdomen, diminished left chest air entry, and heart sounds shifted right. Dana seamlessly placed the pulse ox on the right hand and began passing an OG. The monitor beeped – the pulse ox was reading 55% in room air; heart rate 160. I watched the RT reach for the mask.
“No bagging,” I said. “Let’s intubate now.”
This wasn’t about being fancy; it was about avoiding a preventable problem. If I started with mask PPV and blew the stomach and bowel up into an already crowded chest, I could easily push this baby into rapid decompensation, and we couldn’t rescue locally.
We passed a 3.5 ETT, confirmed by color change, bilateral breath sounds, and absence of large gastric insufflation.
| Uncuffed tubes are the norm, but micro cuff tubes may also be used if available, which offer ultra low-pressure sealing and a more cylindrical, gentler cuff. |
The sats climbed into the mid-70s, then low-80s. The baby still worked hard but looked less blue. We had done the one thing this baby absolutely needed from us: secured the airway in a controlled way.
I nodded to Cody to take over bagging while I walked over to where the baby’s father was sitting on a stool beside his wife on the operating room table. “The baby has a hole in his diaphragm, leading to organs from the belly moving up into the chest, which affects how his lungs have developed and makes it hard for them to work properly. We have had to insert a breathing tube to help him. He will need surgery to repair this eventually, but our immediate goal is to bring him to the NICU and get him stabilized”. I winced internally, wishing I had the chance to speak with them prenatally. I wasn’t sure how much was getting through to them in this moment; the dad looked dazed, and the mom was hurling her guts into a vomit bag that the anesthesia resident was holding for her. “I’ll be back to update you once we get your baby settled”.
“His name’s Ash,” the dad finally croaked.
| REMEMBER: CDH means hypoplastic, fragile lungs. The fetus has been affected by not just mechanical compression of lungs but also abnormal streaming of blood flow preferential to RV, reduced pulmonary blood flow due to abnormal vasculature as well as mesenchymal migration defect affecting sidedness. These babies are already prone to a wide range of cardiopulmonary complications and WILL NOT withstand drastic ventilation strategies. |
As we wheeled the baby into the NICU, the admission team was waiting for us. Efficiently, the team began working on securing leads, getting him hooked up to the ventilator, and a PIV was placed in his left hand within the blink of an eye. They began to hook up the OG to low intermittent suction without any prompting. Thank God for NICU nurses.
“Let’s start working on umbilical lines,” I told Cody. The UAC would provide continuous blood pressure and serial gas measurements, while the UVC would let us deliver fluids, inotropes if needed, and later TPN if transport were delayed. “And order a morphine drip,” I added, casting a glance at the squirming baby. His pulmonary pressures would thank us. Minimal handling was the goal once we got things under control.
Cody nodded, typing away at the computer. I turned towards him. “What labs and imaging would you like to get?”
He thought for a moment. “Stat ABG, lactate, CBC, type and screen, coag panel, blood cultures, and a chest x-ray to confirm tube position and show classic findings of left CDH.”
I nodded. “And an echo. We will eventually transfer this baby,” I said, “but we need to document pulmonary pressures, shunt direction, and ventricular function. It will change how quickly a receiving ECMO center moves.”
I resisted the urge to tinker with every vent setting. The priority was to protect the lung as much as possible.
“Okay, so our goals are pH > 7.2, pCO2 45-65, and sats 91-98% but I’m fine with anything >85%. I’d rather not crank the pressures or use too much FiO2. PIP limit of 25. We can wean our FiO2 slowly as tolerated. Keep a close eye on urine output and lactate.” I paused. “And let’s have the oscillator on standby. To ward off the bad spirits.”
| HFOV can be used as a first intention mode of ventilation in the severe cases, particularly those with the liver in the chest. It would not be the wrong option in this case, but if the baby seems stable, there is nothing wrong with starting with the conventional ventilator. You just need to have clear defined criteria for escalation. Other markers of severity include: Observed: Expected Lung/ Head Ratio < 25% for left CDH and < 50% for right CDHObserved: Expected Total Fetal Lung Volume < 35%Intrathoracic liver herniation |
Meanwhile, dextrose ran through the UVC, and we kept the baby NPO. If transport got delayed, we’d talk about TPN—but in those first hours, I cared far more about hemodynamics than calories. Restrictive fluid management is preferred in CDH anyway.
Within the first hour, the numbers started to clarify the picture.
- Right radial ABG: pH 7.22, pCO₂ 58, pO₂ 46 on FiO₂ 0.7; base deficit −5.
- CXR: left‑sided CDH with bowel in the chest, mediastinal shift, ETT at T2–3, no pneumothorax.
- Echo: suprasystemic pulmonary pressures, right‑to‑left shunting at PDA and PFO, RV dysfunction, LV normal.
Our bedside monitor still read: MAP 35–38 with occasional dips, preductal sats 80–88% on 0.7 FiO₂, postductal 70–78%.
| It is important to recognize the different presentations or phenotypic types in these infants with CDH. The PAH/pre-capillary phenotype is widely recognized, but I am now learning that a subset of infants also have LV dysfunction as the predominant feature (post-capillary type). Like most aspects of medicine and neonatology, these classifications are not static but rather dynamic states influenced by transitions, acidosis, the PDA, various interventions, and medications. What is interesting about the post-capillary phenotype is that pulmonary vasodilators can actually worsen this. This makes sense when you think about how LV dysfunction can cause pulmonary congestion and a resultant L > R shunt at the atrial level (there may not be a pre-/post-sat differential). So, unless you want worsening pulmonary edema or onset of hemorrhage, not to mention the PDA is probably also feeding the systemic circulation, be very careful to avoid iNO or other pulmonary vasodilators in these babies! Keeping the duct open and supporting with inotropes is key here. Really highlights the importance of an early echo to assess these hemodynamic variables. In combination with the clinical picture, of course. For the pre-capillary type, think of the PDA as almost a pop-off valve that prevents RV overload. As the PDA starts to close, the RV afterload continues to rise, culminating in RV failure. This is when we would use pulmonary vasodilators in combination with other management strategies for pulmonary hypertension (effective ventilation and alveolar recruitment, sedation, minimizing handling. But this is a delicate balance, as too much blood flow to the lungs is also not good; the iNO needs to be weaned at the first indication of a shunt reversal. Other thoughts are, does the duct need to be kept open (with PGE1), or do you need some kind of inotropic agents to help with the RV dysfunction (low-dose epi vs milrinone)? |
In a quaternary center, this is the moment where people start talking about ECMO. In our NICU, this is the moment when I start dialing the ECMO center’s number.
“We are a non-ECMO, non-surgical NICU. Term infant with prenatally diagnosed left CDH, liver up. We intubated in the delivery room without mask PPV, on SIMV‑VC with TV 4 ml/kg, PEEP 4, FiO₂ 7-%. Preductal sats 80–88%, MAP 35–38. ABG shows pH 7.22, CO₂ 58, PaO₂ 46. UAC and UVC are in; OG on suction. Echo shows suprasystemic PHTN with right-to-left shunt and RV dysfunction, LV normal. We’re concerned this baby may progress to needing ECMO and surgical repair you can provide.”
That narrative matters. It tells them this baby hasn’t already been injured by over‑aggressive ventilation or neglected PHTN.
I also told them what we were already doing and what we could not do: “We can start inhaled nitric oxide if needed, but we cannot cannulate for ECMO. We can titrate inotropes to maintain MAPs, but if the baby needs escalating support beyond that, we cannot rescue.”
That way, the receiving center understands both the baby’s current physiology and the limits of our environment. Even though I can’t offer ECMO, I still think in ECMO thresholds—it helps me decide how urgent the transfer is.
Red flags that make me push for the fastest possible transport:
- Persistent preductal sats < 80–85% despite FiO₂ 100% and optimized, gentle ventilation.
- Worsening acidosis with pH < 7.15–7.20, rising lactate, and high pCO₂ that I can’t fix without high PIPs.
- MAP drifting below the targeted range despite reasonable doses of one or more inotropes with or without oliguria
- Impending ventricular failure
- Oxygenation index > 40
About surgical timing, I don’t pretend to be the decider. Instead, I share the trajectory:
- “Right now, the baby needs FiO₂ 0.7 with sats in the mid‑80s.”
- “If we can get FiO₂ down to 0.6 or less, stabilize the MAP, and see pulmonary pressures on echo moving toward systemic, I imagine you’ll consider repair once you’ve had some time to optimize.”
My role is to hand them a baby who is still a candidate for the decisions they can make.
That’s the skill I’m trying to build at my level with the resources available to me – not how to run ECMO or do the repair, but how to recognize who needs those things, stabilize them wisely, and hand them off in the best possible shape for whatever comes next.
References:
1. Altit G, Lapointe A, Kipfmueller F, Patel N. Cardiac function in congenital diaphragmatic hernia. Semin Pediatr Surg. 2024;33(4):151438. doi:10.1016/j.sempedsurg.2024.151438
2. Gowda SH, Patel N. “Heart of the Matter”: Cardiac Dysfunction in Congenital Diaphragmatic Hernia. Am J Perinatol. 2024;41(S 01):e1709-e1716. doi:10.1055/a-2067-7925
3. Fraga MV, Altit G, Yoder BA, Bhombal S. What is the role of echocardiography in newborns with CDH? Semin Fetal Neonatal Med. 2025;30(3):101657. doi:10.1016/j.siny.2025.101657
4. Moore SS, Keller RL, Altit G. Congenital Diaphragmatic Hernia. Clin Perinatol. 2024;51(1):151-170. doi:10.1016/j.clp.2023.10.001
5. Bhombal S, Patel N. Diagnosis & management of pulmonary hypertension in congenital diaphragmatic hernia. Semin Fetal Neonatal Med. 2022;27(4):101383. doi:10.1016/j.siny.2022.101383
The NICU Fellow Notebook 
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