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PubMed This is a summary of 22 peer-reviewed journal articles Updated
Neonatology · Congenital Diaphragmatic Hernia (CDH) with Pulmonary Hypertension

How is Pulmonary Hypertension Treated in CDH Babies?

At a Glance

To treat pulmonary hypertension in babies with CDH, NICU teams use pulmonary vasodilators like inhaled nitric oxide (iNO), sildenafil, and milrinone. These medications relax tight blood vessels in the lungs, improving oxygen levels and helping the heart pump blood more effectively.

Seeing your newborn hooked up to continuous gas breathing tubes and intravenous (IV) lines is overwhelming and can be frightening. The medical team uses these tools to deliver specific medications—most commonly inhaled nitric oxide (iNO), sildenafil, or milrinone—to relax the blood vessels in your baby’s lungs, improving blood flow and oxygenation.

When a baby is born with a congenital diaphragmatic hernia (CDH), the blood vessels in their lungs are often smaller, fewer in number, and abnormally thick. This creates high pressure in the lungs, known as pulmonary hypertension, making it hard for the heart to pump blood through them to get oxygen [1][2]. To help lower this pressure, the neonatal intensive care unit (NICU) team uses medications called pulmonary vasodilators to signal the tight muscles around the lung’s blood vessels to relax.

Quick Reference: Common Pulmonary Vasodilators

Medication How it is given Primary Target Expected Timeline
Inhaled Nitric Oxide (iNO) Gas in the breathing tube Lung blood vessels Acute NICU stay (short-term)
Sildenafil IV line or feeding tube Lung blood vessels NICU stay to long-term (sometimes at home)
Milrinone IV line Lung blood vessels & heart muscle Acute NICU stay (short-term)

Inhaled Nitric Oxide (iNO)

Often the first medication used, inhaled nitric oxide is a gas given continuously through your baby’s breathing tube [3]. Because it is inhaled directly into the lungs, it primarily affects the blood vessels there without lowering the baby’s overall body blood pressure [3].

Nitric oxide works by stimulating the production of a chemical messenger in the blood vessel walls that tells the tight muscles to relax. While it can rapidly improve oxygen levels in many newborns, CDH lungs are unique. Some studies suggest iNO might not be as universally effective for babies with CDH as it is for other types of newborn pulmonary hypertension, so doctors will watch closely to see if your baby is responding to it [4][5]. Because it can occasionally cause the blood to carry oxygen less effectively (a condition called methemoglobinemia), the care team will monitor your baby’s blood work carefully while they are on this gas [6].

Sildenafil

You may know sildenafil by its brand name, Revatio or Viagra. In the NICU, it is a highly effective medication for pulmonary hypertension [7]. If the “relaxation messenger” created by iNO is a light switch turning on blood vessel relaxation, sildenafil works by blocking the enzyme (PDE5) that naturally tries to turn that switch off [8].

By keeping these relaxation signals active, sildenafil helps keep the lung’s blood vessels open. It is frequently used if iNO alone isn’t enough, or it may be started to help wean your baby off iNO or other supports [9][10]. While iNO is only given in the hospital, sildenafil can be given through an intravenous (IV) line and later through a feeding tube or by mouth [11]. Because of this, some babies with CDH will go home on sildenafil to manage their lung pressures long-term. The doctors will monitor your baby to ensure the medication doesn’t cause their overall body blood pressure to drop [12].

Milrinone

Managing CDH isn’t just about the lungs; the heart also has to work extremely hard to pump blood against the high lung pressure. Milrinone is a unique medication because it does two important things at once: it relaxes the blood vessels in the lungs, and it helps the heart muscle pump more effectively [13][14].

It does this by blocking a different enzyme (PDE3) in both the blood vessels and the heart [15]. Because milrinone can sometimes relax blood vessels throughout the whole body (not just the lungs), it can occasionally cause a drop in the baby’s overall blood pressure [16]. The care team will monitor your baby’s blood pressure continuously and may use other medications alongside milrinone to keep their circulation stable [17].

How the Team Chooses and Next Steps

Because every baby with CDH is different, there is no “one size fits all” approach [18]. The doctors will constantly monitor your baby using echocardiograms (ultrasounds of the heart) to look at how blood is flowing and how well the heart is pumping. Based on those pictures and your baby’s oxygen levels, they may use one of these medications or a combination of them to give your baby the best possible support [19][20].

The primary goal of these medications is to stabilize your baby and manage their pulmonary hypertension [21]. However, if these therapies are not enough to safely lower the pressure and provide adequate oxygenation, your medical team will discuss advanced life support options, such as ECMO (Extracorporeal Membrane Oxygenation). ECMO is a machine that temporarily takes over the work of the heart and lungs to give your baby’s body time to rest and respond to treatment, and these medications are often used in the effort to either avoid the need for ECMO or to successfully transition off of it [21][22].

Common questions in this guide

Why do babies with CDH need pulmonary hypertension medications?
Babies with CDH often have smaller, underdeveloped lungs with abnormally thick blood vessels. This creates high pressure in the lungs, requiring specialized medications to relax the blood vessels and help the heart pump oxygen-rich blood.
What does inhaled nitric oxide (iNO) do for my baby?
Inhaled nitric oxide is a gas given continuously through a baby's breathing tube. It directly targets the lungs to relax tight blood vessels and rapidly improve oxygen levels, usually without lowering the baby's overall body blood pressure.
How does milrinone help babies with CDH?
Milrinone is a unique medication that serves a dual purpose. It relaxes the high-pressure blood vessels in the lungs while simultaneously helping the heart muscle pump more effectively, which is critical when the heart is working extremely hard.
Will my baby need to take sildenafil at home?
Yes, while inhaled nitric oxide is only given in the hospital, sildenafil can be administered by mouth or through a feeding tube. Because of this flexibility, some babies with CDH will go home on sildenafil to manage their lung pressures long-term.
How do these medications relate to ECMO?
ECMO is a life-support machine that temporarily takes over the work of the heart and lungs. Doctors use medications like sildenafil and milrinone to stabilize the baby's lung pressure, often in an effort to either prevent the need for ECMO or to successfully transition the baby off the machine.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What did today's echocardiogram show about my baby's lung pressure and heart function?
  2. 2.Is my baby responding well to the inhaled nitric oxide (iNO), or are you considering transitioning to another medication?
  3. 3.What side effects or blood pressure changes are you monitoring for while my baby is on milrinone or sildenafil?
  4. 4.How do these medication adjustments fit into the timeline for potentially avoiding or weaning off ECMO?
  5. 5.Do you anticipate my baby might need to go home on sildenafil, and if so, how will we manage that dosing?

Questions For You

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Related questions

Why Do CDH Survivors Need Multidisciplinary Clinics?Why Does CDH Cause Severe GERD and Feeding Issues?Will My Baby Outgrow Their CDH Patch? Risks & MonitoringCan CDH Surgery Be Done While on ECMO?Left vs. Right-Sided CDH: What Are the Differences?What is the FETO Procedure Timeline for CDH?What is Gentle Ventilation for CDH?How Does Congenital Diaphragmatic Hernia Affect Lungs?CDH Liver Up vs Liver Down: What Does It Mean?Will My CDH Child Have Asthma or Need Oxygen?What Are the Prenatal Genetic Testing Options for CDH?Does CDH Affect Your Child's Chest & Spine Growth?What Causes CDH? Did I Cause My Baby's Hernia?What Happens in the Delivery Room with a CDH Baby?What to Expect When Your Baby is on ECMO for CDHWhy is CDH Surgery Delayed? The Role of Stabilization

References

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This page provides educational information about medications used for babies with CDH. Always consult your neonatologist or NICU care team for medical advice regarding your child's specific treatment plan and monitor alarms.

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