Hypoplastic Left Heart Syndrome Practice Variation Across 31 Centres From 20 European Countries. An AEPC Imaging Working Group Study
(2025) In European Journal of Pediatrics 184(6). p.1-11- 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... (More)
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. (Table presented.)
(Less)
- author
- Cantinotti, Massimiliano
; Voges, Inga
; di Salvo, Giovanni
; Ortiz-Garrido, Almudena
; Bharucha, Tara
; Grotenhuis, Heynric
; Sabate-Rotes, Anna
; Cavigelli, Anna
; Roest, Arno
; Sendzikaite, Skaiste
; Nolan, Oscar
; Ramcharan, Tristan
; Koubsky, Karel
; Brun, Henrik
; Petropoulos, Andreas C.
; Bellsham-Revell, Hannah
; Kaneva-Nencheva, Anna
; Dinarevic, Senka Mesihovic
; Abumehdi, Mohammad Ryan
; Óskarsson, Gylfi
; Olejnik, Peter
; Doros, Gabriela
; Ojala, Tiina
; Salaets, Thomas
; Sunnegård, Jan
; Bhat, Misha
LU
; Wacker, Julie
; Wåhlander, Håkan
; Lubaua, Inguna
; Herberg, Ulrike
; Miller, Owen
and McMahon, Colin J.
(Less) - publishing date
- 2025-06
- type
- Contribution to journal
- publication status
- published
- keywords
- Congenital heart disease, Hypoplastic left heart syndrome, Imaging, Management, Practice, Variation
- in
- European Journal of Pediatrics
- volume
- 184
- issue
- 6
- article number
- 379
- pages
- 1 - 11
- publisher
- Springer
- external identifiers
-
- scopus:105006924484
- pmid:40448872
- ISSN
- 0340-6199
- DOI
- 10.1007/s00431-025-06175-9
- language
- English
- LU publication?
- no
- additional info
- Publisher Copyright: © The Author(s) 2025.
- id
- a1d08496-14d1-4178-a0b1-be8045d52c1d
- date added to LUP
- 2026-03-04 19:08:24
- date last changed
- 2026-05-14 07:05:33
@article{a1d08496-14d1-4178-a0b1-be8045d52c1d,
abstract = {{<p>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. (Table presented.)</p>}},
author = {{Cantinotti, Massimiliano and Voges, Inga and di Salvo, Giovanni and Ortiz-Garrido, Almudena and Bharucha, Tara and Grotenhuis, Heynric and Sabate-Rotes, Anna and Cavigelli, Anna and Roest, Arno and Sendzikaite, Skaiste and Nolan, Oscar and Ramcharan, Tristan and Koubsky, Karel and Brun, Henrik and Petropoulos, Andreas C. and Bellsham-Revell, Hannah and Kaneva-Nencheva, Anna and Dinarevic, Senka Mesihovic and Abumehdi, Mohammad Ryan and Óskarsson, Gylfi and Olejnik, Peter and Doros, Gabriela and Ojala, Tiina and Salaets, Thomas and Sunnegård, Jan and Bhat, Misha and Wacker, Julie and Wåhlander, Håkan and Lubaua, Inguna and Herberg, Ulrike and Miller, Owen and McMahon, Colin J.}},
issn = {{0340-6199}},
keywords = {{Congenital heart disease; Hypoplastic left heart syndrome; Imaging; Management; Practice; Variation}},
language = {{eng}},
number = {{6}},
pages = {{1--11}},
publisher = {{Springer}},
series = {{European Journal of Pediatrics}},
title = {{Hypoplastic Left Heart Syndrome Practice Variation Across 31 Centres From 20 European Countries. An AEPC Imaging Working Group Study}},
url = {{http://dx.doi.org/10.1007/s00431-025-06175-9}},
doi = {{10.1007/s00431-025-06175-9}},
volume = {{184}},
year = {{2025}},
}