Pulmonary atresia with ventricular septal defect and pulmonary sequestration

Area of Science:

• Pediatric Cardiology
• Congenital Heart Surgery
• Thoracic Surgery

Background:

• Neonatal complex congenital heart disease presents significant surgical challenges.
• Pulmonary sequestration requires careful management alongside cardiac anomalies.
• Systemic-to-pulmonary shunts are critical for palliation in certain heart defects.

Observation:

• A 5-day-old infant presented with pulmonary atresia, ventricular septal defect, patent ductus arteriosus, and right lower lobe pulmonary sequestration.
• The patient also had a retroesophageal innominate vein, adding anatomical complexity.
• The surgical team opted for a novel approach to manage the pulmonary sequestration concurrently.

Findings:

• A systemic-to-pulmonary shunt was successfully performed.
• Arteries supplying the sequestrated lung were ligated via median sternotomy during cardiopulmonary bypass.
• This approach successfully avoided the need for thoracotomy.

Implications:

• This technique offers a less invasive option for managing pulmonary sequestration in neonates with complex congenital heart disease.
• Avoiding thoracotomy minimizes risks of lung infection and reduces overall patient morbidity.
• The successful outcome highlights the feasibility of combined cardiac and thoracic procedures through a single sternotomy incision.