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Hypoplastic Left Heart Syndrome Practice Variation Across 31 Centres From 20 European Countries. An AEPC Imaging Working Group Study
M. Cantinotti, I. Voges, G. di Salvo, A. Ortiz-Garrido, T. Bharucha, H. Grotenhuis, A. Sabate-Rotes, A. Cavigelli, A. Roest, S. Sendzikaite, O. Nolan, T. Ramcharan, K. Koubsky, H. Brun, AC. Petropoulos, H. Bellsham-Revell, A. Kaneva-Nencheva, SM....
Jazyk angličtina Země Německo
Typ dokumentu časopisecké články, multicentrická studie
- MeSH
- dítě MeSH
- kojenec MeSH
- lékařská praxe - způsoby provádění * statistika a číselné údaje MeSH
- lidé MeSH
- novorozenec MeSH
- paliativní péče MeSH
- předškolní dítě MeSH
- průzkumy a dotazníky MeSH
- syndrom hypoplazie levého srdce * chirurgie diagnóza terapie MeSH
- Check Tag
- dítě MeSH
- kojenec MeSH
- lidé MeSH
- novorozenec MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- Geografické názvy
- Evropa MeSH
UNLABELLED: Despite significant advances in knowledge and the development of guidelines, the management of hypoplastic left heart syndrome (HLHS) remains highly variable. A structured questionnaire was circulated across European Association of Paediatric & Congenital Cardiology (AEPC) affiliated centres. The aims were to evaluate standards in pre-operative assessment, types of surgery, follow-up and medical practices in children with HLHS. Thirty-one centres from 20 countries completed the survey. Delivery of babies with HLHS occurred in co-located maternity hospitals in 74% of centres; 29% were planned for spontaneous onset of labour, while 54% decided on a case-by-case basis. The preferred initial palliation was a right ventricle-pulmonary artery conduit in 55% of cases, modified Blalock-Thomas Taussig shunt (mBTTS) in 35%, and hybrid in 15% of cases. Timing for Glenn varied from 3 to 6 months of age and preoperative examination varied greatly: 65% performed cardiac catheterization and only 19% performed cardiac magnetic resonance. Stage III palliation was performed at a highly variable interval (2-6 years of age), nearly always employing an extracardiac conduit. Fenestration was routinely performed in 61% and reserved for borderline cases in 39%. All the centers adopted warfarin for the first 3-12 months after Fontan completion, and continued if a fenestration was present, while in non-fenestrated aspirin was left by most centers (e.g. 68%). However, there was a high disparity in the use of heart failure medications (e.g. in interstage I-II 35% use ACE-inhibitors, and only 26% digoxin). Follow-up practice also varied widely with only 60% employing specific protocols. CONCLUSION: This first multi-centre European survey from 31 centres from 20 different European countries highlighted a high practice variation in HLHS management across all the stages of Single Ventricle (Fontan) palliation. Major variations pertained to pre- and post-surgical investigations, surgical strategy for stage I and III, medical treatment regimens, and follow-up programs. WHAT IS KNOWN: • Hypoplastic left heart syndrome (HLHS) remains one of the most complex and challenging congenital cardiac defects to manage. • Investigating the management of children with HLHS across different European centres can facilitate study of the most effective management strategies. WHAT IS NEW: • Significant variation in HLHS management were reported in relation to pre- and post-surgical examinations, surgical strategy at stage I and III, medical treatment regimens, and follow-up programs. • Greater standardisation of imaging and diagnostic evaluation, medical treatment and follow-up surveillance may improve outcomes for these vulnerable patients and warrants further study.
ANUBIH Bosnia and Herzegovina BiH Bosnia Bosnia and Herzegovina
Aziz Aliyev National Postgraduate Training Center Baku Azerbaijan
Cardiología Pediátrica Vall d'Hebron Hospital Campus Barcelona Spain
Cardiology Children's Hospital Zurich Zurich Switzerland
Clinic of Paediatrics Institute of Clinical Medicine Vilnius University Vilnius Lithuania
Department of Paediatric Cardiology National Heart Hospital Sofia Bulgaria
Department of Paediatric Cardiology Oslo University Hospital Oslo Norway
Department of Paediatric Cardiology Royal Brompton Hospital London England
Department Paediatric Cardiology University Hospitals Leuven Leuven Belgium
Department Pediatric Cardiology Birmingham England
Department Pediatric Cardiology Leicester England
Department Pediatric Cardiology Padua Italy
Department Pediatric Cardiology Wilhelmina Children's Hospital UMCU Utrecht The Netherlands
Evelina Children's Hospital London England UK
Faculty of Medicine Comenius University Bratislava Slovakia
Fondazione CNR Regione Toscana G Monasterio Pisa Italy
Pediatric Cardiology University of Helsinki and Helsinki University Hospital Helsinki Finland
Riga Stradins University Clinical University Hospital LV Riga Latvia
Citace poskytuje Crossref.org
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