Baessato Francesca, Romeo Cristina, Rabbat Mark G, Pontone Gianluca, Meierhofer Christian
Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technical University of Munich, 80636 Munich, Germany.
Department of Cardiology, Regional Hospital S. Maurizio, 39100 Bolzano, Italy.
Diagnostics (Basel). 2022 Apr 19;12(5):1022. doi: 10.3390/diagnostics12051022.
Cardiomyopathies (CMPs) are a heterogeneous group of diseases that involve the myocardium and result in systolic or diastolic impairment of the cardiac muscle, potentially leading to heart failure, malignant arrhythmias, or sudden cardiac death. Occurrence in pediatric age is rare but has been associated with worse outcomes. Non-invasive cardiac imaging techniques, integrated with clinical, genetic, and electrocardiographic data, have shown a pivotal role in the clinical work-up of such diseases by defining structural alterations and assessing potential complications. Above all modalities, cardiovascular magnetic resonance (CMR) has emerged as a powerful tool complementary to echocardiography to confirm diagnosis, provide prognostic information and guide therapeutic strategies secondary to its high spatial and temporal resolution, lack of ionizing radiation, and good reproducibility. Moreover, CMR can provide in vivo tissue characterization of the myocardial tissue aiding the identification of structural pathologic changes such as replacement or diffuse fibrosis, which are predictors of worse outcomes. Large prospective randomized studies are needed for further validation of CMR in the context of childhood CMPs. This review aims to highlight the role of advanced imaging with CMR in CMPs with particular reference to the dilated, hypertrophic and non-compacted phenotypes, which are more commonly seen in children.
心肌病（CMPs）是一组异质性疾病，累及心肌，导致心肌收缩或舒张功能受损，可能引发心力衰竭、恶性心律失常或心源性猝死。儿科患者中发病率较低，但预后较差。非侵入性心脏成像技术与临床、基因和心电图数据相结合，通过明确结构改变和评估潜在并发症，在这些疾病的临床检查中发挥了关键作用。在所有检查方式中，心血管磁共振成像（CMR）已成为一种强大的工具，可作为超声心动图的补充，因其具有高空间和时间分辨率、无电离辐射且重复性好，有助于确诊、提供预后信息并指导治疗策略。此外，CMR能够对心肌组织进行活体组织特征分析，有助于识别诸如替代性或弥漫性纤维化等结构病理变化，这些变化是预后较差的预测指标。在儿童CMPs的背景下，还需要大型前瞻性随机研究来进一步验证CMR的作用。本综述旨在强调CMR等先进成像技术在CMPs中的作用，特别提及扩张型、肥厚型和未致密化型表型，这些在儿童中更为常见。
