Pulmonary hypoplasia care
Our goal is to help children with pulmonary hypoplasia breathe easier through coordinated care, fewer appointments, and a treatment plan that grows with your child.
Breathing room for fragile lungs
Pulmonary hypoplasia means a baby’s lungs haven’t developed fully, making it hard for them to get the oxygen they need. At Primary Children’s Hospital, our Pulmonary Hypoplasia Program brings together experts from pulmonology, cardiology, and surgery to care for these complex conditions..
Conditions we treat
Some of the conditions we treat may include:
Congenital diaphragm conditions
Congenital diaphragmatic hernia
Recurrent or complex CDH
Giant omphaloceles
Airway & lung conditions
Congenital pulmonary airway malformations
Airway and lung malformations
Lung underdevelopment concerns
Feeding & growth concerns
Pulmonary hypertension
Reflux and aspiration
Breathing, feeding, and growth issues
Treatments and services
Our Pulmonary Hypoplasia Program provides coordinated specialty care. Treatments may include:
Advanced imaging and testing tailored to your child’s needs, including echocardiograms for pulmonary hypertension, chest CT or MRI, bronchoscopy, pulmonary function tests (PFTs), and hearing tests, often completed the same day as your visit.
Expert surgical management for conditions such as congenital diaphragmatic hernia (CDH) repair (including patch or redo repairs), CPAM resection (thoracoscopic when appropriate), and abdominal wall reconstruction for omphaloceles.
Comprehensive treatment for pulmonary hypertension with pulmonary vasodilators, as well as inhaled therapies, oxygen, and ventilatory support (including home ventilation). Our inpatient team provides specialized support, including extracorporeal membrane oxygenation (ECMO) when needed.
Guidance and care for NG or G-tubes, reflux management, and access to speech and feeding therapy.
Ongoing monitoring of growth, school readiness, and activity guidance, with clear transition pathways from NICU to home and caregiver education every step of the way.
Our locations
Frequently asked questions
Get answers to some common questions about our pulmonary hypoplasia care
Yes. Many babies with pulmonary hypoplasia survive with specialized care and ongoing monitoring. Outcomes depend on how underdeveloped the lungs are and whether other conditions, such as congenital diaphragmatic hernia (CDH) or pulmonary hypertension, are also present.
Pulmonary hypoplasia can happen when the lungs do not fully develop before birth. Causes may include congenital diaphragmatic hernia (CDH), giant omphaloceles, low amniotic fluid levels, airway or lung malformations, and certain genetic or developmental conditions.
Pulmonary hypoplasia is commonly associated with congenital diaphragmatic hernia (CDH). Babies with pulmonary hypoplasia may also experience pulmonary hypertension, feeding difficulties, reflux, or other airway and lung conditions.
Not always. Some babies with congenital pulmonary airway malformations (CPAM) need surgery to prevent breathing problems, infections, or future complications, while others may be monitored closely over time. Your child’s care team will recommend the best treatment plan based on symptoms and imaging results.
Symptoms can vary depending on severity but may include fast breathing, low oxygen levels, noisy breathing, feeding difficulties, poor growth, frequent respiratory infections, or ongoing need for oxygen support.
Children with pulmonary hypoplasia may be cared for by specialists in pulmonology, cardiology, pediatric surgery, gastroenterology, nutrition, speech and feeding therapy, and neonatology working together in one coordinated program.
The outlook for children with pulmonary hypoplasia varies widely because the condition can range from mild to life-threatening. Survival depends on factors such as how underdeveloped the lungs are, whether one or both lungs are affected, and whether other congenital conditions are present.
In the most severe cases, especially when pulmonary hypoplasia is associated with conditions like congenital diaphragmatic hernia (CDH) or prolonged low amniotic fluid during pregnancy, the risk of death is significantly higher. However, many children with mild or moderate pulmonary hypoplasia survive and go on to live active lives with specialized medical care. Advances in neonatal intensive care, respiratory support, and pediatric surgery have greatly improved outcomes over the past several decades.
Pulmonary hypoplasia is often linked to other conditions that affect fetal growth and lung development. Some of the most common include:
- Congenital diaphragmatic hernia (CDH), where abdominal organs move into the chest and limit lung growth.
- Congenital pulmonary airway malformation (CPAM) and other congenital lung malformations.
- Giant omphalocele, a birth defect where abdominal organs develop outside the body.
- Kidney and urinary tract conditions, such as bilateral renal agenesis or severe urinary tract obstruction, can lead to low amniotic fluid (oligohydramnios).
- Skeletal dysplasias, including forms of dwarfism that restrict normal chest growth.
- Genetic syndromes and chromosomal conditions that affect multiple organs and fetal development.
Because pulmonary hypoplasia is frequently associated with other complex conditions, children often benefit from care by a multidisciplinary team that includes neonatologists, pulmonologists, pediatric surgeons, cardiologists, and genetic specialists.
