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Lymphatic Disorder Management in Pediatric Patients With Congenital Heart Disease in European Pediatric Cardiology
Sanam Safi1, Vibeke E Hjortdal1, Marc Gewillig2
1Department of Cardiothoracic Surgery University Hospital of Copenhagen, Rigshospitalet Copenhagen Denmark.
Insights
Lymphatic disorders like chylothorax are common after Fontan circulation. While treatments vary across Europe, lymphatic interventions are increasing, highlighting the need for standardized care.
Area of Science:
- Pediatric Cardiology
- Lymphatic Medicine
- Congenital Heart Disease Management
Background:
- Lymphatic disorders (protein-losing enteropathy, plastic bronchitis, chylothorax) are significant complications of Fontan circulation.
- These complications increase the risk of early mortality in affected children.
- European centers are increasingly adopting lymphatic procedures to manage these conditions.
Purpose of the Study:
- To survey European pediatric cardiology centers regarding lymphatic disorders.
- To map the prevalence of these disorders and the treatments offered.
- To understand the current landscape of lymphatic interventions in Europe.
Main Methods:
- A survey with 37 questions was sent to 49 European pediatric cardiology centers.
- Thirty-one centers (63% response rate) participated.
- Data collected included disorder prevalence, diagnostic methods, and intervention rates.
Main Results:
- Chylothorax was the most frequent lymphatic disorder, followed by protein-losing enteropathy and plastic bronchitis.
- Noncontrast magnetic resonance lymphangiography was the most common diagnostic tool (52%).
- Eleven centers (35%) performed lymphatic interventions, with a median of 3 procedures annually per center.
Conclusions:
- The study confirms the rarity and variability in treatment approaches for lymphatic disorders in Europe.
- There is a growing adoption of lymphatic interventions, with at least 11 centers offering them.
- Centralization of services and evidence-based diagnostics/interventions are crucial for improving patient outcomes.
Background:
Lymphatic disorders such as protein-losing enteropathy, plastic bronchitis, and chylothorax are important complications of the Fontan circulation and ultimately result in an increased risk of early death. Several European centers are now performing lymphatic procedures. The aim of this study is to map the extent of these lymphatic disorders and treatments provided across European pediatric cardiology centers.
Methods And Results:
A survey was circulated to 49 European pediatric cardiology centers consisting of 37 questions including a mix of binary, categorical, and continuous variables. Thirty-one centers (63%) participated in the study, performing a median of 250 (interquartile range, 178 - 313) cardiopulmonary bypass procedures per year. Chylothorax emerged as the most prevalent lymphatic disorder followed by protein-losing enteropathy and plastic bronchitis. The most common diagnostic investigation method was noncontrast magnetic resonance lymphangiography (52%). Eleven centers (35%) conducted lymphatic interventions with a median of 3 (interquartile range, 1 - 4) procedures per year and 12 (interquartile range, 5 - 15) interventions in total per center.
Conclusions:
This study confirms the rarity of and variation in treatment approaches for lymphatic disorders across Europe. With at least 11 centers offering lymphatic interventions, the adoption of these procedures is on the rise in Europe. To improve the quality of care and treatment outcomes for these complex patients, it is crucial to consider evidence-based lymphatic diagnostics, interventional lymphatic procedures, and the centralization of services in Europe.
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