Lymphatic Failure Often Masquerades as Cardiac Failure in Children and Adults

Jonathan M. Chen, MD

Jonathan M. Chen, MD

A growing body of evidence shows that many conditions being treated as sequelae of congenital cardiac problems such as protein-losing enteropathy (PLE) and plastic bronchitis may in fact be the result of altered lymphatic circulation that leads to these conditions formerly referred to as “mucosal derangements.”

“We have been treating a lot of these disorders completely wrong,” said Jonathan M. Chen, MD, from the Cardiac Center at Children’s Hospital of Philadelphia, who will moderate today’s session on Lymphatic Intervention after Congenital Heart Surgery.

Lymphatic failure also may be a key dysfunction leading to fluid retention in adult heart failure and is implicated in complications associated with some cancers and cancer treatments. Catheter-based and surgical approaches to repair damaged lymph vessels can effectively treat such conditions.

“We’ve been largely focused on managing the symptoms—ascites, airway casts, fluid retention—without focusing on the root cause: lymphatic failure,” Dr. Chen said. “The basis of these disorders is primarily lymphatic dysfunction, not cardiac dysfunction. It is increasingly appearing that the lymphatic circulation is foundational to congenital heart disease and a surprising number of adult conditions we traditionally associate with cardiac morbidity.”

Meet the Experts: Lymphatic Intervention after Congenital Heart Surgery

12:45 p.m. – 1:45 p.m.
Room 225

Yoav Dori, MD, PhD

Yoav Dori, MD, PhD

Pediatric cardiologists often see children with single ventricle circulation who live their entire lives with heart failure, added Yoav Dori, MD, PhD, from the Jill and Mark Fishman Center for Lymphatic Disorders at Children’s Hospital of Philadelphia, who will present at today’s session. 

“Their heart function doesn’t necessarily change much over time, but the lymphatic circulation undergoes changes that lead to accumulation of fluid,” he said. “Adults with heart failure experience similar changes.”

Research from the 1970s found that draining lymphatic fluid could improve the symptoms of heart failure, Dr. Chen said, but the technology of the era could not adequately image the central lymphatic system or reliably measure lymphatic flows. Lymphatic circulation largely was ignored until the 2010s.

“My entry point was having pediatric and adult patients with severe fluid leaks and seeing a few older papers about lymph fluid playing a role in these patients, but there was nothing current to help,” Dr. Dori said. “We developed lymphatic imaging techniques and an understanding that lymphatic circulation is an entire organ system that we had not appreciated. We already are addressing lymphatic failure in the pediatric world and are moving in the same direction in the adult world, where the number of patients is so much greater.”