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<p>We report on the case of a pediatric patient with homozygous familial hypercholesterolemia (HoFH) who developed significant coronary artery disease (CAD). Percutaneous coronary intervention (PCI) guided by intravascular ultrasound (IVUS) was performed.&#xa0;A 6-year-old female patient was diagnosed with HoFH following the identification of cutaneous xanthomas. Genetic test identified a homozygous mutation in the LDLR gene. Cardiovascular assessment including a coronary CT angiography revealed a severe (70–90%) stenosis of the ostium of the left main coronary artery (LMCA) without significant lesions in other coronary branches. Given the severity of the lesion, PCI was indicated.&#xa0;The patient underwent PCI via the right femoral artery. The LMCA lesion was confirmed (70–90% stenosis, TIMI 3 flow) and a drug-eluting stent was deployed. Intravascular ultrasound (IVUS) played a crucial role in guiding the intervention, ensuring optimal stent expansion and apposition.&#xa0;Post-procedure, dual antiplatelet therapy (aspirin + clopidogrel) was initiated, clopidogrel was stopped after six months. A six-month follow-up CT angiogram demonstrated satisfactory stent positioning, despite metallic artifacts limiting full assessment. Two years after angioplasty, there have been no clinical, electrocardiographic, or echocardiographic signs of myocardial ischemia.&#xa0;This case highlights the feasibility and safety of LMCA stenting in pediatric patients with HoFH. Given the challenges of coronary interventions in pediatric patients, IVUS should be considered a critical adjunct to angiography in complex cases of early-onset coronary artery disease.</p>

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Intravascular Ultrasound-Guided Stenting of the Left Main Coronary Artery in a Pediatric Patient with Homozygous Familial Hypercholesterolemia

  • 期刊论文

  • 发表日期：

    2026年8月1日

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  • Sophie Quennelle,
  • Karine Aouchiche,
  • Thomas Cuisset,
  • Sophie Béliard,
  • Julie Marin,
  • Caroline Ovaert

摘要

We report on the case of a pediatric patient with homozygous familial hypercholesterolemia (HoFH) who developed significant coronary artery disease (CAD). Percutaneous coronary intervention (PCI) guided by intravascular ultrasound (IVUS) was performed. A 6-year-old female patient was diagnosed with HoFH following the identification of cutaneous xanthomas. Genetic test identified a homozygous mutation in the LDLR gene. Cardiovascular assessment including a coronary CT angiography revealed a severe (70–90%) stenosis of the ostium of the left main coronary artery (LMCA) without significant lesions in other coronary branches. Given the severity of the lesion, PCI was indicated. The patient underwent PCI via the right femoral artery. The LMCA lesion was confirmed (70–90% stenosis, TIMI 3 flow) and a drug-eluting stent was deployed. Intravascular ultrasound (IVUS) played a crucial role in guiding the intervention, ensuring optimal stent expansion and apposition. Post-procedure, dual antiplatelet therapy (aspirin + clopidogrel) was initiated, clopidogrel was stopped after six months. A six-month follow-up CT angiogram demonstrated satisfactory stent positioning, despite metallic artifacts limiting full assessment. Two years after angioplasty, there have been no clinical, electrocardiographic, or echocardiographic signs of myocardial ischemia. This case highlights the feasibility and safety of LMCA stenting in pediatric patients with HoFH. Given the challenges of coronary interventions in pediatric patients, IVUS should be considered a critical adjunct to angiography in complex cases of early-onset coronary artery disease.