What are the surgical implications of a Rastelli Type A congenital heart defect involving an Atrioventricular Canal (AVC) defect?

From the Guidelines

The surgical implications of a Rastelli Type A congenital heart defect involving an Atrioventricular Canal (AVC) defect typically involve a single-patch repair technique, as recommended by the 2018 AHA/ACC guideline for the management of adults with congenital heart disease 1. The surgical procedure includes:

• Closure of the primum atrial septal defect (ASD)
• Repair of the cleft mitral valve
• Closure of the inlet ventricular septal defect (VSD) A single pericardial patch is used to close both the ASD and VSD simultaneously. The cleft in the anterior leaflet of the mitral valve is sutured to prevent regurgitation. This repair is usually performed in infancy, ideally between 3-6 months of age, to prevent the development of pulmonary hypertension and heart failure. The surgery is done via median sternotomy under cardiopulmonary bypass. Post-operative care involves close monitoring for residual defects, arrhythmias, and valve function. Long-term follow-up is essential to assess for potential complications such as left ventricular outflow tract obstruction or mitral valve regurgitation. The single-patch technique is preferred for Rastelli type A AVC because it allows for a more physiological repair, maintaining the normal anatomy of the left ventricular outflow tract and minimizing the risk of subaortic stenosis. This approach also tends to result in better long-term outcomes compared to two-patch techniques in this specific anatomical variant, as supported by the guidelines 1. Key considerations in the surgical management of Rastelli Type A AVC defect include:
• The importance of early repair to prevent long-term complications
• The need for careful intraoperative and post-operative monitoring to minimize the risk of residual defects and other complications
• The potential benefits of the single-patch technique in maintaining normal cardiac anatomy and function, as highlighted in the 2018 AHA/ACC guideline 1.

From the Research

Surgical Implications of Rastelli Type A Congenital Heart Defect Involving Atrioventricular Canal (AVC) Defect

The surgical implications of a Rastelli Type A congenital heart defect involving an Atrioventricular Canal (AVC) defect are complex and depend on various factors, including the morphology of the valve and symptoms.

• The Rastelli one-patch procedure is a safe and reproducible technique for repairing complete atrioventricular canal defects, with excellent long-term results 2.
• The procedure involves closing the cleft in the mitral valve, either completely or partially, and repairing any associated anomalies, such as pulmonary obstruction or coarctation 2.
• The timing and surgical technique of the repair depend on the morphology of the valve and symptoms, with some patients requiring early repair and others benefiting from delayed repair 3.
• Novel techniques, such as left atrioventricular valvuloplasty, have been developed to address specific challenges, such as parachute left atrioventricular valve and absent left mural leaflet 4.
• The Rastelli procedure has been used to repair various types of atrioventricular canal defects, including those with straddling atrioventricular valve, with successful outcomes reported in several studies 2, 5.

Key Considerations

• The Rastelli procedure is a complex operation that requires careful planning and execution to achieve optimal results 2.
• The procedure can be performed in patients with various types of atrioventricular canal defects, including those with associated anomalies 2, 5.
• The long-term results of the Rastelli procedure are excellent, with high survival rates and low rates of reoperation for mitral regurgitation 2.
• Novel techniques, such as left atrioventricular valvuloplasty, offer new options for repairing complex atrioventricular canal defects 4.