Hypoplastic Left Heart Syndrome Practice Variation Across 31 Centres From 20 European Countries. An AEPC Imaging Working Group Study

Abstract

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.

Keywords: Congenital heart disease; Hypoplastic left heart syndrome; Imaging; Management; Practice; Variation.

Fig. 1

Examination performed before Norwood operation at different centres

Fig. 2

Preferred initial palliation procedure at different centres

Fig. 3

Follow-up interval after Stage 1 Norwood palliation at different centres

Fig. 4

Pre-operative examination before Glenn at different centres

Fig. 5

Pre-operative examination before total cavo-pulmonary connection (TCPC) at different centres