What is CDH?
The diaphragm is a thick muscle that separates the abdominal organs (the intestines, liver, and so on) from the organs in the chest (the lungs and heart). In babies with CDH, the diaphragm develops with a hole, allowing the abdominal organs to migrate into the chest cavity and prevent normal lung growth and development. Despite improvements in ventilator strategies and ECMO (a heart-lung machine), little patients with severe CDH still have a high mortality and survivors have many long-term health problems. It is clear that a novel strategy to enhance lung function and stimulate lung growth will be required to impact these patients. As extraordinary as it sounds, pumping these tiny lungs full of liquid may be the solution.

Putting on the Pressure
“The treatment we are studying is called perfluorocarbon-induced lung growth, or PILG, and it uses a liquid called perflubron that can exchange oxygen and carbon dioxide in the lungs,” explains George B. Mychaliska, M.D., F.A.C.S., director of the fetal diagnosis and treatment center, and assistant professor of surgery and obstetrics and gynecology at U-M. “Basically, this is a liquid you can breathe.”

During PILG, perflubron is pumped into the baby’s lungs until they are full. A constant pressure is then applied to the liquid, which pushes on the inside of the lungs to stimulate growth.

Bench-to-Bedside Research
Patients in this study have left-lung involvement and are on ECMO (a heart-lung bypass machine for infants). “Normal survival rates for these patients is about 50 percent,” says Dr. Mychaliska. “Clearly, this treatment is for patients with severe CDH that hopefully will not only improve survival, but also improve long-term outcomes.”

Although Dr. Mychaliska is the principal investigator for this current trial, PILG is the result of extensive prior research by a co-investigator on the study. “For over 10 years, Dr. Ronald Hirschl, chief of pediatric surgery, has been studying many facets of liquid ventilation, and this current research trial grew out of his experimental and clinical research,” notes Dr. Mychaliska. “PILG is a wonderful example of what we are looking for in research: the ‘bench-to-bedside’ application of new knowledge that directly benefits our little patients.”