Left Main Angioplasty for Acute Coronary Syndrome in 7-Year-Old Girl With Familial Hypercholesterolemia

Familial hypercholesterolemia is a genetic disorder caused by mutations in low-density lipoprotein receptor, resulting in markedly elevated low-density lipoprotein cholesterol, premature atherosclerosis, and xanthomas.

We present a case of a 7-year-old girl with homozygous familial hypercholesterolemia, who developed acute coronary syndrome. Coronary angiography revealed critical ostial and mid-shaft left main (LM) stenosis. She underwent LM intravascular ultrasound-guided percutaneous coronary angioplasty (PTCA) with a drug-eluting stent. She also had mild supravalvular aortic stenosis, which warrants ongoing follow-up.

PTCA in pediatric patients is technically challenging. Revascularization strategy can best be determined on a case-to-case basis. This case highlights that with careful planning, LM PTCA can be feasible and life-saving in select pediatric patients.

Pediatric patients with familial hypercholesterolemia require a high index of suspicion for coronary artery disease. Revascularization decision is challenging in pediatric patients due to lack of data, and long-term outcomes remain uncertain.