Moscatelli Sara, Sabatino Jolanda, Leo Isabella, Borrelli Nunzia, Avesani Martina, Di Salvo Giovanni, Montanaro Claudia, Pergola Valeria, Motta Raffaella, Ielapi Jessica, Di Costanzo Assunta, De Sarro Rosalba, Guglielmi Giulia, Cattapan Irene, Gaudieri Gabriella, Luedke Leonie, Perrone Marco Alfonso
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London WC1N 3JH, UK.
Institute of Cardiovascular Sciences, University College London, London WC1E 6BT, UK.
J Cardiovasc Dev Dis. 2025 Sep 11;12(9):349. doi: 10.3390/jcdd12090349.
Hypoplastic Left Heart Syndrome (HLHS) accounts for 2-3% of congenital heart diseases (CHDs). HLHS is characterized by reduced systemic blood flow due to hypoplastic left ventricle (LV) and underdeveloped left-sided cardiac structures. Without a series of staged interventional treatments, HLHS is often fatal, typically within the first hours or days of life. This manuscript aims to provide a comprehensive overview of the role of echocardiography, cardiovascular magnetic resonance (CMR), and cardiac computed tomography angiography (CCTA) in the optimal management of patients with HLHS. Specifically, it explores the contributions of various non-invasive imaging modalities to the diagnosis, planning of staged palliative interventions, interstage monitoring, and long-term follow-up of HLHS. Furthermore, the advantages and limitations of each imaging technique will be highlighted to aid in clinical decision-making; however, it is important to note that, at present, no universal guidelines exist, and imaging strategies remain largely dependent on individual centre expertise and protocols.
左心发育不全综合征（HLHS）占先天性心脏病（CHD）的2%-3%。HLHS的特征是由于左心室（LV）发育不全和左侧心脏结构发育不良导致体循环血流量减少。如果没有一系列分期介入治疗，HLHS通常是致命的，通常在出生后的最初几小时或几天内死亡。本手稿旨在全面概述超声心动图、心血管磁共振（CMR）和心脏计算机断层血管造影（CCTA）在HLHS患者最佳管理中的作用。具体而言，它探讨了各种非侵入性成像模式对HLHS的诊断、分期姑息性干预计划、阶段间监测和长期随访的贡献。此外，将突出每种成像技术的优点和局限性，以协助临床决策；然而，需要注意的是，目前尚无通用指南，成像策略在很大程度上仍依赖于各个中心的专业知识和方案。
