Deshpande Mrunmayee, Phadke Milind, Khan Abid Talha, Mahajan Ajay U
Department of Cardiology, Lokmanya Tilak Municipal Medical College and General Hospital (LTMGH), Dr Babasaheb Ambedkar Road, Sion (West), Mumbai 400022, India.
Eur Heart J Case Rep. 2021 Jun 16;5(6):ytab175. doi: 10.1093/ehjcr/ytab175. eCollection 2021 Jun.
Familial hypercholesterolaemia (FH) is a primary genetic dyslipidaemia characterized by elevation in serum low-density lipoprotein cholesterol and its deposition in systemic arteries, which causes premature atherosclerosis.
A 10-year-old girl presented with severe symptomatic coronary artery disease. She demonstrated characteristic morphological features of FH. Despite aggressive medical management and lipid-lowering therapy, her symptoms were not relieved and she had dynamic electrocardiogram changes. Coronary angiography showed a distal left main coronary artery lesion along with significant lesions in ostio-proximal and mid-left circumflex artery which were managed by provisional left main coronary artery to left circumflex artery stenting technique, with good immediate- and short-term results and angina relief.
To the best of our knowledge, this is the first reported case of a paediatric patient with FH and acute coronary syndrome treated with percutaneous coronary intervention to left main coronary artery and left circumflex artery using provisional stenting technique. Revascularization strategies for symptomatic coronary artery disease in paediatric patients with FH have multiple unique challenges and remain an unexplored and under-reported subject.
家族性高胆固醇血症（FH）是一种原发性遗传性血脂异常，其特征是血清低密度脂蛋白胆固醇升高并沉积于全身动脉，导致过早发生动脉粥样硬化。
一名10岁女孩因严重的症状性冠状动脉疾病就诊。她表现出FH的典型形态学特征。尽管采取了积极的药物治疗和降脂治疗，但其症状并未缓解，动态心电图有改变。冠状动脉造影显示左冠状动脉主干远端病变以及左回旋支近段和中段的明显病变，采用临时左冠状动脉主干至左回旋支动脉支架置入技术进行处理，即刻和短期效果良好，心绞痛缓解。
据我们所知，这是首例报道的采用临时支架置入技术对左冠状动脉主干和左回旋支动脉进行经皮冠状动脉介入治疗的FH合并急性冠状动脉综合征的儿科患者。FH儿科患者有症状性冠状动脉疾病的血运重建策略面临多个独特挑战，仍是一个未被探索且报道不足的课题。
