Ross-Konno operation for patients with Shone complex.

Area of Science:

• Cardiology
• Pediatric Cardiac Surgery
• Congenital Heart Disease

Background:

• Shone complex is a rare congenital heart defect characterized by multiple left-sided obstructive lesions.
• Severe left ventricular outflow tract obstruction and mitral valve obstruction necessitate complex surgical interventions.
• The Ross-Konno operation is a combined aortic and mitral valve procedure, often considered for complex left-sided heart disease.

Observation:

• Two pediatric patients diagnosed with Shone complex presented with severe left ventricular outflow tract obstruction and marginal mitral obstruction.
• Both patients successfully underwent the Ross-Konno operation, with the native mitral valve intentionally preserved.
• Serial echocardiographic follow-up for up to four years was conducted to assess cardiac function.

Findings:

• Postoperative survival was achieved in both pediatric patients.
• Echocardiography revealed a consistent decrease or downward trend in peak mitral diastolic velocity over the follow-up period.
• The preserved native mitral valve demonstrated acceptable function following the cardiac surgery.

Implications:

• The Ross-Konno procedure may be an effective surgical strategy for a specific subset of pediatric patients with Shone complex.
• Preserving the native mitral valve during the Ross-Konno operation appears feasible and beneficial in selected cases.
• This approach offers a potential alternative for managing complex congenital heart disease, warranting further investigation.