Lymphatic Disorder Management in Pediatric Patients With Congenital Heart Disease in European Pediatric Cardiology Centers: Current Status, Disparities, and Future Considerations

Abstract

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.

Keywords: MRI; congenital heart disease; imaging modalities; lymphatic disorders; lymphatic interventions.

Figure 1. Congenital cardiac interventions and surgery in 31 participating centers in Europe.

Figure 2. Lymphatic interventions performed per center.

Figure 3. Lymphatic disorders and their occurrence.

Chylothorax is presented as average cases per year, with a median of 8 (IQR, 3 ‐8) patients per center per year. Plastic bronchitis and protein‐losing enteropathy, presented as total cases across all centers over an undefined number of years due to their low incidence, were found to have medians of 2 (IQR, 0 ‐ 4) and 5 (IQR, 1 ‐ 10), respectively. IQR indicates interquartile range.

Figure 4. Lymphatic disorders and their treatments across the 31 European centers.

Other surgical treatments for chylothorax indicates 3 thoracic duct ligation, 2 pleurodesis, and 1 pleura adhesion; for the remaining 6 centers, this was not specified. Other treatments for plastic bronchitis: plasminogen‐activator inhalation, alteplase when available, and finally surgical Hraska during the Fontan procedure. TPN indicates total parenteral nutrition.

Figure 5. Noncontrast MRL.

Multiplanar reconstructions from heavily T2‐weighted noncontrast MRL show a dilated and tortuous thoracic duct (A, B). The thoracic duct is also well seen on 3‐dimensional SSFP images (C, D). MRL indicates magnetic resonance lymphangiography; and SSFP, steady‐state free precession.

Figure 6. DCMRL.

A, Intranodal DCMRL demonstrating mediastinal and bilateral pulmonary lymphatic perfusion syndrome more on the right (arrow). B, In a patient with PB, blue dye injection into the TD demonstrated dilated peribronchial lymphatic networks. C, Blue dye injection into the liver demonstrating leak into the duodenal lumen. D, Intrahepatic DCMRL demonstrating leak into the duodenal lumen characteristics of PLE (arrow). Reproduced from Dori et al under the terms and conditions of the Creative Commons Attribution (CC‐BY) license (https://creativecommons.org/licenses/by/4.0/). DCMRL indicates dynamic contrast magnetic resonance lymphangiography; PB, plastic bronchitis; PLE, protein‐losing enteropathy; and TD, thoracic duct.