Coronary artery patch augmentation for congenital left coronary ostial stenosis in Williams syndrome

Area of Science:

• Cardiology
• Pediatric Surgery
• Medical Genetics

Background:

• Left coronary ostial stenosis is a rare but serious condition, particularly in infants with Williams syndrome, often leading to sudden cardiac death.
• Effective long-term surgical revascularization strategies for coronary ostial stenosis in young children remain largely undefined.
• Williams syndrome is a genetic disorder associated with various cardiovascular anomalies, including coronary artery abnormalities.

Observation:

• An 18-month-old boy with Williams syndrome presented with severe cardiogenic shock secondary to left coronary ostial stenosis.
• The patient's critical condition necessitated immediate and effective surgical intervention.
• Standard surgical approaches for this condition in infants are not well-established.

Findings:

• Patch augmentation of the left coronary ostium was successfully performed using glutaraldehyde-treated autologous pericardium.
• The surgical technique provided immediate relief from the stenosis and improved hemodynamic stability.
• Post-operative follow-up demonstrated no adverse events, myocardial ischemia, or restenosis.

Implications:

• This case highlights the efficacy of autologous pericardial patch augmentation for treating left coronary ostial stenosis in infants with Williams syndrome.
• This surgical approach represents a potentially reliable method for long-term revascularization in this vulnerable pediatric population.
• Successful surgical management may prevent sudden cardiac death and improve long-term outcomes for children with Williams syndrome and coronary ostial stenosis.