[Anesthetic Management of a Patient with Pulmonary Atresia and Intact Ventricular Septum Accompanying Sinusoidal...

Area of Science:

• Cardiology
• Anesthesiology
• Pediatric Surgery

Background:

• Pulmonary atresia with intact ventricular septum (PAIVS) is a complex congenital heart defect.
• PAIVS can present with coronary artery anomalies, including sinusoidal communications from the right ventricle.
• Coronary circulation dependency on right ventricular supply poses unique anesthetic challenges.

Observation:

• A 1-year-old boy with PAIVS, previously treated with balloon atrial septostomy, central shunt, and Glenn procedure, presented for cheiloplasty.
• Preoperative oxygen saturation was 85%, decreasing to 80% with agitation.
• The patient exhibited sinusoidal communications with coronary artery supply originating from the right ventricle.

Findings:

• Anesthetic goals included decreasing pulmonary vascular resistance and increasing pulmonary blood flow to the sinusoidal communications.
• Stable partial arterial oxygen pressure (PaO₂) was maintained using FiO₂ 0.5 and Paco₂ 30-35 mmHg.
• Hemodynamic stability was achieved through fluid transfusion, maintaining urine output, and using an alpha-1 adrenoceptor agonist. Cardiac output was monitored noninvasively using impedance cardiography (Aesculon®).

Implications:

• This case highlights critical anesthetic considerations for PAIVS with sinusoidal communications.
• Effective management strategies are crucial for optimizing pulmonary blood flow and hemodynamic stability in such complex pediatric cardiac cases.
• Noninvasive cardiac output monitoring can aid in managing these high-risk patients.