Triple coronary bypass in a child with homozygous familial hypercholesterolemia

Area of Science:

• Cardiovascular Surgery
• Pediatric Cardiology
• Medical Genetics

Background:

• Familial homozygous hypercholesterolemia (HoFH) is a rare genetic disorder causing extremely high cholesterol levels.
• Severe coronary atherosclerosis in pediatric patients is uncommon but can lead to critical cardiac events.
• Early diagnosis and aggressive management are crucial for HoFH patients to prevent premature cardiovascular disease.

Observation:

• A 12-year-old female presented with extremely high total cholesterol (1300 mg/dL) and extensive coronary atherosclerosis.
• She had a history of successful out-of-hospital cardiopulmonary resuscitation due to a cardiac event.
• Angiography revealed significant three-vessel disease, including stenosis in the right coronary and left coronary ostia.

Findings:

• The patient underwent a successful triple coronary bypass procedure using arterial and venous grafts.
• Grafts included left internal thoracic artery (LITA) to the left anterior descending artery, radial artery to the obtuse marginal artery, and saphenous vein graft to the right posterior descending artery.
• Post-operative angiography confirmed successful revascularization.

Implications:

• This case highlights the feasibility and success of coronary artery bypass grafting in pediatric patients with severe HoFH and atherosclerosis.
• Intensive lipid-lowering therapy combined with weekly lipid apheresis is essential for long-term management.
• Aggressive surgical and medical interventions can improve outcomes for young patients with this severe genetic lipid disorder.