BACKGROUND: 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. CASE SUMMARY: 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. DISCUSSION: 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. Published on behalf of the European Society of Cardiology. All rights reserved.
BACKGROUND: 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. CASE SUMMARY: 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. DISCUSSION: 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. Published on behalf of the European Society of Cardiology. All rights reserved.
Authors: Raul D Santos; Andrea Ruzza; G Kees Hovingh; Albert Wiegman; François Mach; Christopher E Kurtz; Andrew Hamer; Ian Bridges; Andrea Bartuli; Jean Bergeron; Tamás Szamosi; Saikat Santra; Claudia Stefanutti; Olivier S Descamps; Susanne Greber-Platzer; Ilse Luirink; John J P Kastelein; Daniel Gaudet Journal: N Engl J Med Date: 2020-08-29 Impact factor: 91.245
Authors: Francesco Burzotta; Jens Flensted Lassen; Thierry Lefèvre; Adrian P Banning; Yiannis S Chatzizisis; Thomas William Johnson; Miroslaw Ferenc; Sudhir Rathore; Remo Albiero; Manuel Pan; Olivier Darremont; David Hildick-Smith; Alaide Chieffo; Marco Zimarino; Yves Louvard; Goran Stankovic Journal: EuroIntervention Date: 2021-03-19 Impact factor: 6.534