Chylothorax, Lymphatic Abnormalities, Lymphatic Disorders, Plastic Bronchitis, Protein Losing Enteropathy
Conditions
Keywords
Lymphatic complications, Multicenter study, Plastic bronchitis, Protein Losing Enteropathy, Chylothorax
Brief summary
This European multicenter observational study aims to evaluate the real-world use, timing, and outcomes of lymphatic interventions in patients with congenital heart disease and/or primary lymphatic disorders. The study will examine the effectiveness of diagnostic imaging and interventional techniques, such as lymphatic embolization, in improving clinical symptoms, biomarkers, and fluid-related complications. The central question is whether early diagnosis and targeted intervention can lead to clinically meaningful improvements and reduced need for reintervention. Data collected retrospectively and prospectively from participating centers will help identify predictors of outcome, assess disease severity, and inform standardized diagnostic and therapeutic pathways across Europe.
Detailed description
This is a prospective, multicenter observational study with retrospective components. The study population includes patients who have undergone or are undergoing lymphatic imaging diagnostics and lymphatic interventions at participating centers across Europe. Patients are included both retrospectively (from medical records) and prospectively as part of their routine clinical care.
Interventions
PROCEDURELymphatic intervention
This intervention was made as part of their clinical treatment.
Sponsors
Rigshospitalet, Denmark
UZ Leuven, Leuven, Belgium
UCL Bruxelles
Lund University Hospital
University Hospital Schleswig-Holstein
University Hospital, Aachen
Deutsches Herzzentrum Muenchen
Universitätsklinikum Erlangen, Erlangen, Bayern, Germany
UZ Gent, Belgium
Hospital for Children and Adolescents, University of Helsinki
Hospital Universitari Vall d'hebron Barcelona, Spain
Children's University Hospital, Ireland
Evelina London Children's Hospital
Guy's and St Thomas' NHS Foundation Trust
Bambin Gesù Hospital IRCCS, Rome, Italy
Study design
Observational model
COHORT
Time perspective
OTHER
Eligibility
Sex/Gender
ALL
Healthy volunteers
No
Inclusion criteria
* Patients of any age with a confirmed central lymphatic disorder, either in the context of CHD or as a primary lymphatic disorder, verified through lymphatic diagnostics (e.g. lymphatic imaging or relevant biomarkers). * Patients who have undergone, or are undergoing, diagnostic lymphatic imaging and/or interventional procedures for their lymphatic disorder. * Patients who are receiving, or have received, conservative (non-interventional) management for their lymphatic disorder.
Exclusion criteria
* Patients with isolated peripheral lymphatic disorders not involving the central lymphatic system. * Patients with acute postoperative iatrogenic chylothorax (\<3-4 weeks duration and not requiring intervention). * Patients without sufficient clinical documentation to confirm diagnosis, treatment, or follow-up. * Patients who decline, or whose legal guardians decline, to provide informed consent (for prospective inclusion).
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Change in Symptoms Following Lymphatic Intervention, assessed by: | From time of treatment to yearly follow-up up to 5 years. | Patient-reported or physician-assessed symptoms (e.g., cough, diarrhea, dyspnea) will be categorized as normalized, improved, unchanged, or worsened compared to baseline. Unit of Measure: Symptom status category (normalized / improved / unchanged / worsened) |
| Change in Objective Biomarkers Following Lymphatic Intervention | From time of treatment to yearly follow-up up to 5 years. | Objective clinical findings (e.g., serum albumin level, lymphocyte count, fluid accumulation) will be categorized as normalized, improved, unchanged, or worsened compared to baseline. Unit of Measure: Biomarker status category (normalized / improved / unchanged / worsened) |
| Composite improvement: | From time of treatment to yearly follow-up up to 5 years. | ≥50% reduction in presenting symptoms and/or ≥20% improvement in key biomarkers (e.g., serum albumin, lymphocyte count etc.). |
Countries
Denmark
Contacts
CONTACTSanam Safi-Rasmussen, MD
STUDY_DIRECTORVibeke E Hjortdal, Professor
Rigshospitalet, Denmark
STUDY_DIRECTORMichael Rahbek Schmidt, Ass. professor, PhD, DMSc
Rigshospitalet, Denmark
Outcome results
None listed