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<p>Despite significant advances in knowledge and the development of guidelines, the management of hypoplastic left heart syndrome (HLHS) remains highly variable.&#xa0;A structured questionnaire was circulated across European Association of Paediatric &amp; 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.&#xa0;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&#xa0;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&#xa0;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&#xa0;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. <i>Conclusion</i>:&#xa0;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.</p><p><Table Float="No" ID="Taba"> <tgroup cols="1"> <colspec align="left" colname="c1" colnum="1" /> <tbody> <row> <entry align="left" colname="c1"> <p><b>What is Known:</b></p> </entry> </row> <row> <entry align="left" colname="c1"> <p>•&#xa0;<i>Hypoplastic left heart syndrome (HLHS) remains one of the most complex and challenging congenital cardiac defects to manage.</i></p> </entry> </row> <row> <entry align="left" colname="c1"> <p>•&#xa0;<i>Investigating the management of children with HLHS across different European centres can facilitate study of the most effective management strategies.</i></p> </entry> </row> <row> <entry align="left" colname="c1"> <p><b>What is New:</b></p> </entry> </row> <row> <entry align="left" colname="c1"> <p>•&#xa0;<i>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.</i></p> </entry> </row> <row> <entry align="left" colname="c1"> <p>•&#xa0;<i>Greater standardisation of imaging and diagnostic evaluation, medical treatment and follow-up surveillance may improve outcomes for these vulnerable patients and warrants further study.</i></p> </entry> </row> </tbody> </tgroup> </Table></p>

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

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年5月31日

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  • Massimiliano Cantinotti,
  • Inga Voges,
  • Giovanni di Salvo,
  • Almudena Ortiz-Garrido,
  • Tara Bharucha,
  • Heynric Grotenhuis,
  • Anna Sabate-Rotes,
  • Anna Cavigelli,
  • Arno Roest,
  • Skaiste Sendzikaite,
  • Oscar Nolan,
  • Tristan Ramcharan,
  • Karel Koubsky,
  • Henrik Brun,
  • Andreas C. Petropoulos,
  • Hannah Bellsham-Revell,
  • Anna Kaneva-Nencheva,
  • Senka Mesihovic Dinarevic,
  • Mohammad Ryan Abumehdi,
  • Gylfi Óskarsson,
  • Peter Olejnik,
  • Gabriela Doros,
  • Tiina Ojala,
  • Thomas Salaets,
  • Jan Sunnegård,
  • Misha Bhat,
  • Julie Wacker,
  • Håkan Wåhlander,
  • Inguna Lubaua,
  • Ulrike Herberg,
  • Owen Miller,
  • Colin J. McMahon

摘要

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.