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

Children's Heart Centre Faculty of Medicine Charles University Prague and Motol University Hospital 5 Úvalu 84 Prague Czech Republic

Clinic of Paediatrics Institute of Clinical Medicine Vilnius University Vilnius Lithuania

Department for Congenital Cardiology and Pediatric Cardiology University Hospital Schleswig Holstein Campus Kiel Kiel Germany

Department of Paediatric Cardiology Children's Hospital Reykjavik Landspitali University Hospital Reykjavik Iceland

Department of Paediatric Cardiology Hospital Materno Infantil of Malaga University of Malaga Málaga Spain

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 of Paediatric Cardiology University Hospital Southampton NHS Foundation Trust Southampton England

Department of Pediatric Cardiology Children's Heart Center Skåne University Hospital in Lund 221 85 Lund SE Sweden

Department of Pediatrics Division of Pediatric Cardiology Willem Alexander Children's Hospital Leiden University Medical Centre Leiden Netherlands

Department Paediatric Cardiology Children's Health Ireland at Crumlin University School of Medicine University College Dublin Ireland

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 Unit Department of Woman Child and Adolescent Medicine Children University Hospital of Geneva Geneva Switzerland

Pediatric Cardiology University of Helsinki and Helsinki University Hospital Helsinki Finland

Pediatric Heart Centre The Queen Silvia Children´s Hospital University Hospital University of Gothenburg Gothenburg Sweden

Riga Stradins University Clinical University Hospital LV Riga Latvia

University Hospital RWTH Aachen Aachen Germany

Victor Babes UMF Timisoara 3rd Pediatric Clinic Louis Turcanu Emergency Hospital for Children Timisoara Romania

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