Lymphatic Disorder Management in Pediatric Patients With Congenital Heart Disease in European Pediatric Cardiology Centers: Current Status, Disparities, and Future Considerations
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 st...
Saved in:
| Main Authors: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2024-11-01
|
| Series: | Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease |
| Subjects: | |
| Online Access: | https://www.ahajournals.org/doi/10.1161/JAHA.124.036597 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1849221151373393920 |
|---|---|
| author | Sanam Safi Vibeke E. Hjortdal Marc Gewillig Owen Miller Mohammad Ryan Abumehdi Massimiliano Cantinotti Heynric Grotenhuis Francesca Raimondi Almudena Ortiz Garrido Arno Roest Jan Sunnegårdh Thomas Saleats Henrik Brun Helen Pärna Oscar Nolan Anna Sabaté Rotés Antigoni Deri Daniel De Wolf Ulrike Herberg Petru Liuba Thomas Möller Gylfi Óskarsson Micol Rebonato Willem A. Helbing Gabriela Doros Iolanda Muntean Tiina Ojala Inguna Lubaua Misha Bhat Julie Wacker Beatrice Bonello Tristan Ramcharan Gerald Greil Skaiste Sendzikaite Damien Bonnet Jan Marek Inga Voges Colin J. McMahon |
| author_facet | Sanam Safi Vibeke E. Hjortdal Marc Gewillig Owen Miller Mohammad Ryan Abumehdi Massimiliano Cantinotti Heynric Grotenhuis Francesca Raimondi Almudena Ortiz Garrido Arno Roest Jan Sunnegårdh Thomas Saleats Henrik Brun Helen Pärna Oscar Nolan Anna Sabaté Rotés Antigoni Deri Daniel De Wolf Ulrike Herberg Petru Liuba Thomas Möller Gylfi Óskarsson Micol Rebonato Willem A. Helbing Gabriela Doros Iolanda Muntean Tiina Ojala Inguna Lubaua Misha Bhat Julie Wacker Beatrice Bonello Tristan Ramcharan Gerald Greil Skaiste Sendzikaite Damien Bonnet Jan Marek Inga Voges Colin J. McMahon |
| author_sort | Sanam Safi |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-d1917b434a534ea382e20edc6477ea5c |
| institution | Kabale University |
| issn | 2047-9980 |
| language | English |
| publishDate | 2024-11-01 |
| publisher | Wiley |
| record_format | Article |
| series | Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease |
| spelling | doaj-art-d1917b434a534ea382e20edc6477ea5c2024-11-19T12:31:39ZengWileyJournal of the American Heart Association: Cardiovascular and Cerebrovascular Disease2047-99802024-11-01132210.1161/JAHA.124.036597Lymphatic Disorder Management in Pediatric Patients With Congenital Heart Disease in European Pediatric Cardiology Centers: Current Status, Disparities, and Future ConsiderationsSanam Safi0Vibeke E. Hjortdal1Marc Gewillig2Owen Miller3Mohammad Ryan Abumehdi4Massimiliano Cantinotti5Heynric Grotenhuis6Francesca Raimondi7Almudena Ortiz Garrido8Arno Roest9Jan Sunnegårdh10Thomas Saleats11Henrik Brun12Helen Pärna13Oscar Nolan14Anna Sabaté Rotés15Antigoni Deri16Daniel De Wolf17Ulrike Herberg18Petru Liuba19Thomas Möller20Gylfi Óskarsson21Micol Rebonato22Willem A. Helbing23Gabriela Doros24Iolanda Muntean25Tiina Ojala26Inguna Lubaua27Misha Bhat28Julie Wacker29Beatrice Bonello30Tristan Ramcharan31Gerald Greil32Skaiste Sendzikaite33Damien Bonnet34Jan Marek35Inga Voges36Colin J. McMahon37Department of Cardiothoracic Surgery University Hospital of Copenhagen, Rigshospitalet Copenhagen DenmarkDepartment of Cardiothoracic Surgery University Hospital of Copenhagen, Rigshospitalet Copenhagen DenmarkDepartment of Pediatric Cardiology University Hospitals Leuven Leuven BelgiumDepartment Paediatric Cardiology Evelina London Children’s Hospital London UKDepartment Pediatric Cardiology Royal Brompton Hospital London UKFondazione CNR‐Regione Toscana G. Monasterio (FTGM) National Research Institute (CNR) Pisa ItalyDepartment Pediatric Cardiology Wilhelmina Children’s Hospital/UMCU Utrecht The NetherlandsDivision of Pediatric Cardiology Meyer University Hospital, Florence University Florence ItalyDepartment Pediatric Cardiology Hospital Materno Infantil Malaga AL SpainDepartment of Pediatrics, Division of Pediatric Cardiology Willem‐Alexander Children’s Hospital, Leiden University Medical Center Leiden NetherlandsChildren’s Heart Center The Queen Silvia Children’s Hospital Sahlgrenska University Hospital Göteborg SwedenDepartment Pediatric Cardiology University Hospitals Leuven Leuven BelgiumDepartment of Pediatric Cardiology Oslo University Hospital Oslo NorwayDepartment of Pediatric Cardiology Tallinn Children’s Hospital Tallinn EstoniaDepartment of Pediatric Cardiology University Hospitals Leicester Leicester UKServicio de Cardiología Pediátrica Hospital Universitario Vall d’Hebron, Universidad Aut’onoma de Barcelona Barcelona SpainDepartment Pediatric Cardiology Leeds University Leeds UKPediatric Cardiology Ghent University Hospital (UZ Gent) Ghent BelgiumDepartment or Pediatric Cardiology and Congenital Heart Disease University of Aachen Aachen GermanyDepartment of Cardiology Pediatric Heart Center, Skåne University Hospital Lund, Lund University Lund SwedenDepartment of Pediatric Cardiology Oslo University Hospital Oslo NorwayUniversity of Iceland Reykjavik IcelandMedical and Surgical Department of Pediatric Cardiology Ospedale Pediatrico Bambino Gesù, IRCCS Rome ItalyDepartment of Pediatrics, Division of Pediatric Cardiology Erasmus MC‐Sophia Children’s Hospital Rotterdam The NetherlandsVictor Babes UMF, IIIrd Pediatric Clinic Louis Turcanu Emergency Children Hospital Timisoara RomaniaClinic of Paediatric Cardiology Institute for Cardiovascular Diseases and Transplantation, UMFST “George Emil Palade” Timisoara RomaniaDepartment Pediatric Cardiology Children’s Hospital, Helsinki University Hospital Helsinki FinlandDepartment of Pediatric Cardiology Riga Stradins University Riga LatviaDepartment of Cardiology Pediatric Heart Center, Skåne University Hospital Lund, Lund University Lund SwedenPediatric Cardiology Unit, Department of Woman, Child and Adolescent Medicine Children University Hospital of Geneva Geneva SwitzerlandDepartment Pediatric Cardiology Great Ormond Street NHS Trust London UKBirmingham Children’s Hospital Birmingham UKDepartment of Pediatrics, Division of Pediatric Cardiology UT Southwestern Dallas TX USAClinic of Children’s Diseases, Institute of Clinical Medicine, Faculty of Medicine Vilnius University Vilnius LithuaniaM3C‐Necker Enfants Malades, AP‐HP Paris FranceDepartment Pediatric Cardiology Great Ormond Street NHS Trust London UKDepartment for Congenital Cardiology and Pediatric Cardiology University Hospital Schleswig‐Holstein Campus Kiel GermanyDepartment Pediatric Cardiology Children’s Health Ireland at Crumlin Dublin IrelandBackground 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.https://www.ahajournals.org/doi/10.1161/JAHA.124.036597congenital heart diseaseimaging modalitieslymphatic disorderslymphatic interventionsMRI |
| spellingShingle | Sanam Safi Vibeke E. Hjortdal Marc Gewillig Owen Miller Mohammad Ryan Abumehdi Massimiliano Cantinotti Heynric Grotenhuis Francesca Raimondi Almudena Ortiz Garrido Arno Roest Jan Sunnegårdh Thomas Saleats Henrik Brun Helen Pärna Oscar Nolan Anna Sabaté Rotés Antigoni Deri Daniel De Wolf Ulrike Herberg Petru Liuba Thomas Möller Gylfi Óskarsson Micol Rebonato Willem A. Helbing Gabriela Doros Iolanda Muntean Tiina Ojala Inguna Lubaua Misha Bhat Julie Wacker Beatrice Bonello Tristan Ramcharan Gerald Greil Skaiste Sendzikaite Damien Bonnet Jan Marek Inga Voges Colin J. McMahon Lymphatic Disorder Management in Pediatric Patients With Congenital Heart Disease in European Pediatric Cardiology Centers: Current Status, Disparities, and Future Considerations Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease congenital heart disease imaging modalities lymphatic disorders lymphatic interventions MRI |
| title | Lymphatic Disorder Management in Pediatric Patients With Congenital Heart Disease in European Pediatric Cardiology Centers: Current Status, Disparities, and Future Considerations |
| title_full | Lymphatic Disorder Management in Pediatric Patients With Congenital Heart Disease in European Pediatric Cardiology Centers: Current Status, Disparities, and Future Considerations |
| title_fullStr | Lymphatic Disorder Management in Pediatric Patients With Congenital Heart Disease in European Pediatric Cardiology Centers: Current Status, Disparities, and Future Considerations |
| title_full_unstemmed | Lymphatic Disorder Management in Pediatric Patients With Congenital Heart Disease in European Pediatric Cardiology Centers: Current Status, Disparities, and Future Considerations |
| title_short | Lymphatic Disorder Management in Pediatric Patients With Congenital Heart Disease in European Pediatric Cardiology Centers: Current Status, Disparities, and Future Considerations |
| title_sort | lymphatic disorder management in pediatric patients with congenital heart disease in european pediatric cardiology centers current status disparities and future considerations |
| topic | congenital heart disease imaging modalities lymphatic disorders lymphatic interventions MRI |
| url | https://www.ahajournals.org/doi/10.1161/JAHA.124.036597 |
| work_keys_str_mv | AT sanamsafi lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT vibekeehjortdal lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT marcgewillig lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT owenmiller lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT mohammadryanabumehdi lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT massimilianocantinotti lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT heynricgrotenhuis lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT francescaraimondi lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT almudenaortizgarrido lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT arnoroest lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT jansunnegardh lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT thomassaleats lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT henrikbrun lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT helenparna lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT oscarnolan lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT annasabaterotes lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT antigonideri lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT danieldewolf lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT ulrikeherberg lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT petruliuba lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT thomasmoller lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT gylfioskarsson lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT micolrebonato lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT willemahelbing lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT gabrieladoros lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT iolandamuntean lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT tiinaojala lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT ingunalubaua lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT mishabhat lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT juliewacker lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT beatricebonello lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT tristanramcharan lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT geraldgreil lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT skaistesendzikaite lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT damienbonnet lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT janmarek lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT ingavoges lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations AT colinjmcmahon lymphaticdisordermanagementinpediatricpatientswithcongenitalheartdiseaseineuropeanpediatriccardiologycenterscurrentstatusdisparitiesandfutureconsiderations |