New surgical approach for scimitar syndrome: Double-decker technique

This condition, with a prevalence of 1–3 per 100,000 births, is usually diagnosed in infants. If left untreated, it can lead to serious complications such as pulmonary hypertension, right heart failure, respiratory failure, heart arrhythmia, and growth disorders.

Key features of scimitar syndrome:

• Anomalous pulmonary venous drainage to the inferior vena cava
• Usual surgical repair involves re-implanting the right pulmonary veins to the left atrium or creating an intra-atrial tunnel
• High risk of postoperative pulmonary vein obstruction leading to severe complications

The traditional surgical methods for Scimitar Syndrome often face issues with postoperative pulmonary vein obstruction, significantly lowering the success rate of re-interventions. The new procedure performed at Kindai University Hospital, however, marked the first successful use of the Double-Decker Technique, originally designed for another type of partial anomalous pulmonary venous return.

This modified technique reconstructs two blood flow pathways using only the patient’s atrial wall without artificial materials, reducing the risk of obstruction. The right pulmonary vein and the inferior vena cava are separated to ensure unobstructed blood flow.

Advantages of the modified double-decker technique:

• Creates wide, separate pathways for the right pulmonary vein and the inferior vena cava
• Reduces the risk of obstruction
• Pathways are expected to grow with the patient's natural growth as they are reconstructed using the atrial wall

The pediatric patient was referred to Kindai University Hospital after developing a fever and was diagnosed with Scimitar Syndrome through cardiac echocardiogram and contrast-enhanced computed tomography. The surgery was performed without complications, and the patient was discharged 10 days later in stable condition.

The advantage of this new technique is that the new blood flow pathway of the inferior vena cava is created outside (on the pulmonary venous pathway). This technique can create wide pathways separately and reduces the risk of obstruction.

The successful application of the Double-Decker Technique on this patient demonstrates its potential as a standard surgical procedure for Scimitar Syndrome, promising improved outcomes for this rare condition. The use of natural tissue over artificial materials also suggests better long-term results and adaptability with patient growth.

MEDICA-tradefair.com; Source: Kindai University