Cardiac arrest under anesthesia in a pediatric patient with Williams syndrome: a case report

Area of Science:

• Cardiology
• Pediatric Anesthesiology
• Medical Genetics

Background:

• Williams syndrome is a genetic disorder associated with cardiovascular abnormalities, including supravalvular aortic stenosis.
• Patients with Williams syndrome may present with complex medical histories requiring careful anesthetic management.
• Hypercalcemia is a common comorbidity in Williams syndrome, potentially impacting cardiovascular function.

Observation:

• A 3-year-old boy with Williams syndrome, hypercalcemia, and mild supravalvular aortic stenosis suffered serial cardiac arrests during anesthesia induction.
• The initial arrests presented as profound bradycardia, hypotension, and pulseless electrical activity with significant ST wave changes.
• The patient experienced recurrent cardiac arrests during a subsequent cardiac catheterization, necessitating extracorporeal membrane oxygenation and emergency cardiac surgery.

Findings:

• The case highlights the critical risks associated with anesthesia and invasive cardiac procedures in pediatric patients with Williams syndrome.
• Significant intraoperative instability and recurrent cardiac arrests underscore the complex cardiovascular challenges in this patient population.
• The need for emergency extracorporeal membrane oxygenation and cardiac surgery indicates severe, acute cardiovascular compromise.

Implications:

• Thorough preoperative cardiac assessment, including echocardiography and electrocardiography, is vital for patients with Williams syndrome.
• Cardiac catheterization, while potentially diagnostic, carries an elevated risk in this population and requires meticulous monitoring.
• This case emphasizes the importance of multidisciplinary care and preparedness for critical events in managing pediatric patients with complex congenital conditions.