Pulmonary hypoplasia is a condition characterized by small, underdeveloped lungs that can affect not only breathing but also heart function, ability to feed, hearing and overall development. Some children with pulmonary hypoplasia develop a related condition known as pulmonary hypertension, which causes high blood pressure in the arteries of the lungs (the pulmonary arteries). Over time, this pressure causes the pulmonary arteries to narrow, making the right side of the heart work harder as it forces blood through the narrowed arteries.

What causes pulmonary hypoplasia?

Pulmonary hypoplasia occurs secondary to a variety of conditions that limit lung development. The Pulmonary Hypoplasia Program (PHP) provides long-term follow-up care for children who have been diagnosed with pulmonary hypoplasia and related conditions.

The following conditions that limit lung development are typically treated through the PHP:

• Congenital diaphragmatic hernia (CDH)
• Lung lesions, including congenital cystic adenomatoid malformation (CCAM) and bronchopulmonary sequestration (BPS) 
• Giant omphalocele, a type of abdominal wall defect 

Pulmonary hypoplasia treatment

Your child's care and pulmonary hypoplasia treatment plan is based on their specific needs, taking into account each unique diagnosis and treatment your child has received. Ongoing developmental and medical assessment is a standard of care provided to all children who come to CHOP for treatment of pulmonary hypoplasia and related conditions.