In June 2025, CHOC, part of Rady Children’s Health, performed its first successful intervention for protein-losing enteropathy (PLE). PLE is a severe lymphatic complication that can occur after the Fontan procedure in children who have been surgically treated for various single-ventricle congenital heart diseases.
This case is an important step for the Heart Institute at CHOC, expanding its scope of specialized pediatric cardiovascular care to include advanced interventional treatment options for children with PLE.
“PLE can be devastating for children living with single-ventricle physiology,” says Dr. Sanjay Sinha, co-director of the Congenital Lymphatic Intervention Program at CHOC and assistant professor of pediatrics at UCI. “The ability to offer this therapy here in Orange County changes what’s possible for our patients and their families.”
About the Heart Institute
The Heart Institute at CHOC brings together experts in pediatric cardiology, cardiothoracic surgery, interventional cardiology, imaging, electrophysiology, and intensive care, all working collaboratively to manage the full spectrum of pediatric cardiac conditions. In 2023, CHOC and UCLA Mattel Children’s Hospital formed an affiliation to further enhance access to world-class pediatric congenital cardiac care for children and their families across Southern California.
Among its specialized programs, the Lymphatic Program is one of the first in the nation to provide comprehensive, multidisciplinary care for children with lymphatic conditions. Led by Dr. Sinha, the program combines pediatric cardiology, pulmonology, gastroenterology, nutrition, and other specialties to evaluate and treat disorders of lymphatic flow. Using advanced imaging modalities, including Dynamic Contrast Enhanced Magnetic Resonance Lymphangiography, and minimally invasive approaches, the team develops individualized treatment plans to correct lymphatic abnormalities and improve outcomes.
Conditions managed by the program include ascites, chylothorax, chylopericardium, plastic bronchitis, thoracic outlet obstruction, and now PLE. Recognized as the most comprehensive pediatric lymphatic program on the West Coast and the second largest in the nation, the Lymphatic Program receives referrals from hospitals and health systems throughout California, Nevada, and Arizona. It operates as part of the Heart Institute’s commitment to providing regional access to advanced care for children with congenital heart disease and associated complications.
“The development of this program represents a significant advance in how we manage children with complex congenital heart disease,” says Dr. Glen Van Arsdell, chief of congenital cardiovascular surgery at UCLA Mattel Children’s Hospital and chief of the UCLA-CHOC Congenital Heart Program. “By integrating surgical, interventional, and imaging expertise, we can address complications that once required transfer to other centers.”
Understanding PLE and its impact
PLE is a rare and life-threatening complication that can develop in patients with single-ventricle circulation after Fontan surgery. The condition develops when lymphatic fluid leaks into the intestinal tract, which can occur from multiple sources, causing a significant loss of serum proteins. The symptoms that patients typically experience include swelling, ascites, and nutritional deficits that can bring on persistent fatigue, recurrent hospitalizations, and impaired growth.
In the past, treatment options for PLE have been limited, and in severe cases, the only definitive therapy was a heart transplant. Over the past decade, lymphatic imaging and interventional approaches have emerged as new tools to identify and treat the lymphatic abnormalities that cause PLE. These procedures can mitigate symptoms, stabilize protein balance, and improve quality of life.
Improving access for families
The availability of PLE intervention at CHOC allows children with post-Fontan complications to remain close to home while receiving complex care that previously required out-of-state travel.
The first patient to undergo the PLE procedure at CHOC was a long-time cardiac patient who years earlier had been transferred to Philadelphia for a similar procedure. In June 2025, the family was able to return to CHOC, where a higher level of expertise and technology is now available with their trusted care team.
“We’re now able to provide advanced lymphatic care right here in Orange County through a fully integrated, multidisciplinary team,” Dr. Sinha says.
This local access reduces logistical and financial barriers for families that come with long-distance travel for medical care and ensures seamless coordination across subspecialty teams within the Heart Institute.
“Every child with complex heart disease needs lifelong, integrated care,” Dr. Van Arsdell says. “This program strengthens that continuum and brings an entirely new dimension of treatment capability to our institute.”
Learn more about the Heart Institute
CHOC and UCLA Health together have been ranked among the top children’s hospitals in the nation for Cardiology & Heart Surgery by U.S. News & World Report.
