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Abstract：
Hypertrophic Cardiomyopathy (HCM) is an inherited myocardial disease characterised by left ventricular hypertrophy, which carries an increased risk of life-threatening arrhythmias and sudden cardiac death. The age of presentation and the underlying aetiology have a significant impact on the prognosis and quality of life of children with HCM, as childhood-onset HCM is associated with high mortality risk and poor long-term outcomes. Accurate cardiac assessment and identification of the HCM phenotype are therefore crucial to determine the diagnosis, prognostic stratification, and follow-up. Cardiac magnetic resonance (CMR) is a comprehensive evaluation tool capable of providing information on cardiac morphology and function, flow, perfusion, and tissue characterisation. CMR allows to detect subtle abnormalities in the myocardial composition and characterise the heterogeneous phenotypic expression of HCM. In particular, the detection of the degree and extent of myocardial fibrosis, using late-gadolinium enhanced sequences or parametric mapping, is unique for CMR and is of additional value in the clinical assessment and prognostic stratification of paediatric HCM patients. Additionally, childhood HCM can be progressive over time. The rate, timing, and degree of disease progression vary from one patient to the other, so close cardiac monitoring and serial follow-up throughout the life of the diagnosed patients is of paramount importance. In this review, an update of the use of CMR in childhood HCM is provided, focussing on its clinical role in diagnosis, prognosis, and serial follow-up.
摘要：
肥厚型心肌病（HCM）是一种以左心室肥厚为特征的遗传性心肌病，这会增加危及生命的心律失常和心源性猝死的风险。发病年龄和潜在病因对HCM患儿的预后和生活质量有显著影响,儿童期发病的HCM与高死亡风险和不良长期结局相关。因此，准确的心脏评估和HCM表型的鉴定对于确定诊断至关重要。预后分层，和后续行动。心脏磁共振（CMR）是一种能够提供心脏形态和功能信息的综合评估工具，流量,灌注,和组织表征。CMR允许检测心肌组成中的细微异常，并表征HCM的异质性表型表达。特别是,心肌纤维化程度和程度的检测，使用晚钆增强序列或参数映射，对于CMR是独特的，并且在儿科HCM患者的临床评估和预后分层中具有附加价值。此外,儿童HCM可以随着时间的推移而进步。率，疾病进展的时间和程度因患者而异，因此，在确诊患者的整个生命过程中进行密切的心脏监测和连续随访至关重要。在这次审查中,提供了儿童HCM使用CMR的最新情况，专注于其在诊断中的临床作用，预后,和连续随访。
