
pmid: 39508150
pmc: PMC11681416
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.
Cardiac & Cardiovascular Systems, Cardiac Care Facilities, PROTEIN-LOSING ENTEROPATHY, Cardiopatia congènita, CHILDREN, Heart Defects, Congenital/therapy, Fontan Procedure, FONTAN, Lymphatic Diseases/therapy, DISEASES::Congenital, Hereditary, and Neonatal Diseases and Abnormalities::Congenital Abnormalities::Cardiovascular Abnormalities::Heart Defects, Congenital, Medicine and Health Sciences, lymphatic interventions, Practice Patterns, Physicians', Child, PLASTIC BRONCHITIS, 1102 Cardiorespiratory Medicine and Haematology, Original Research, Sistema limfàtic - Tractament, OUTCOMES, HEALTH CARE::Health Care Facilities, Manpower, and Services::Health Facilities::Hospitals::Hospitals, Special::Cardiac Care Facilities, Lymphatic interventions, Lymphography, EMBOLIZATION, ENFERMEDADES::enfermedades y anomalías neonatales congénitas y hereditarias::anomalías congénitas::anomalías cardiovasculares::cardiopatías congénitas, congenital heart disease, General medicine, internal medicine and other clinical medicine, Hospitals, Europe, Otros calificadores::Otros calificadores::/terapia, Child, Preschool, Healthcare Disparities/trends, SURVIVAL, 3201 Cardiovascular medicine and haematology, Cardiology and Cardiovascular Medicine, Life Sciences & Biomedicine, Infants, ATENCIÓN DE SALUD::instalaciones, servicios y personal de asistencia sanitaria::centros sanitarios::hospitales::hospitales especializados::instalaciones cardiológicas, Mri, MRI, Heart Defects, Congenital, Child, preschool, NAMED GROUPS::Persons::Age Groups::Child, Imaging modalities, Other subheadings::Other subheadings::/therapy, Enquestes, DENOMINACIONES DE GRUPOS::personas::Grupos de Edad::niño, Cardiac Care Facilities/organization & administration, Europe/epidemiology, TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::técnicas de investigación::métodos epidemiológicos::recopilación de datos::encuestas y cuestionarios, DISEASES::Hemic and Lymphatic Diseases::Lymphatic Diseases, Diseases of the circulatory (Cardiovascular) system, Humans, Healthcare Disparities, Lymphatic Diseases, Congenital heart disease, imaging modalities, Science & Technology, ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Investigative Techniques::Epidemiologic Methods::Data Collection::Surveys and Questionnaires, CHYLOTHORAX, Fontan Procedure/adverse effects, lymphatic disorders, Practice Patterns, Physicians'/trends, RC666-701, ENFERMEDADES::enfermedades hematológicas y linfáticas::enfermedades linfáticas, Health Care Surveys, Cardiovascular System & Cardiology, Lymphatic disorders, CARDIAC-SURGERY
Cardiac & Cardiovascular Systems, Cardiac Care Facilities, PROTEIN-LOSING ENTEROPATHY, Cardiopatia congènita, CHILDREN, Heart Defects, Congenital/therapy, Fontan Procedure, FONTAN, Lymphatic Diseases/therapy, DISEASES::Congenital, Hereditary, and Neonatal Diseases and Abnormalities::Congenital Abnormalities::Cardiovascular Abnormalities::Heart Defects, Congenital, Medicine and Health Sciences, lymphatic interventions, Practice Patterns, Physicians', Child, PLASTIC BRONCHITIS, 1102 Cardiorespiratory Medicine and Haematology, Original Research, Sistema limfàtic - Tractament, OUTCOMES, HEALTH CARE::Health Care Facilities, Manpower, and Services::Health Facilities::Hospitals::Hospitals, Special::Cardiac Care Facilities, Lymphatic interventions, Lymphography, EMBOLIZATION, ENFERMEDADES::enfermedades y anomalías neonatales congénitas y hereditarias::anomalías congénitas::anomalías cardiovasculares::cardiopatías congénitas, congenital heart disease, General medicine, internal medicine and other clinical medicine, Hospitals, Europe, Otros calificadores::Otros calificadores::/terapia, Child, Preschool, Healthcare Disparities/trends, SURVIVAL, 3201 Cardiovascular medicine and haematology, Cardiology and Cardiovascular Medicine, Life Sciences & Biomedicine, Infants, ATENCIÓN DE SALUD::instalaciones, servicios y personal de asistencia sanitaria::centros sanitarios::hospitales::hospitales especializados::instalaciones cardiológicas, Mri, MRI, Heart Defects, Congenital, Child, preschool, NAMED GROUPS::Persons::Age Groups::Child, Imaging modalities, Other subheadings::Other subheadings::/therapy, Enquestes, DENOMINACIONES DE GRUPOS::personas::Grupos de Edad::niño, Cardiac Care Facilities/organization & administration, Europe/epidemiology, TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::técnicas de investigación::métodos epidemiológicos::recopilación de datos::encuestas y cuestionarios, DISEASES::Hemic and Lymphatic Diseases::Lymphatic Diseases, Diseases of the circulatory (Cardiovascular) system, Humans, Healthcare Disparities, Lymphatic Diseases, Congenital heart disease, imaging modalities, Science & Technology, ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Investigative Techniques::Epidemiologic Methods::Data Collection::Surveys and Questionnaires, CHYLOTHORAX, Fontan Procedure/adverse effects, lymphatic disorders, Practice Patterns, Physicians'/trends, RC666-701, ENFERMEDADES::enfermedades hematológicas y linfáticas::enfermedades linfáticas, Health Care Surveys, Cardiovascular System & Cardiology, Lymphatic disorders, CARDIAC-SURGERY
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