Operations for subaortic stenosis in univentricular hearts

Area of Science:

• Pediatric Cardiac Surgery
• Congenital Heart Disease
• Cardiovascular Surgery

Background:

• Subaortic stenosis (SAS) in univentricular hearts presents complex management challenges, particularly with aortic arch obstruction.
• Optimal surgical timing and techniques for SAS in this population remain controversial.
• High pulmonary blood flow and associated anomalies necessitate tailored surgical approaches.

Purpose of the Study:

• To analyze surgical outcomes for infants and children with univentricular hearts and subaortic stenosis.
• To evaluate different surgical strategies for relieving SAS in complex congenital heart disease.
• To assess the impact of interventions on subsequent Fontan completion and survival.

Main Methods:

• Retrospective analysis of surgical results in four groups of patients with univentricular hearts and SAS.
• Surgical interventions included Damus-Kaye-Stansel connection, aortopulmonary window, arterial switch, and subaortic resection.
• Patients underwent procedures either concurrently with or staged before Fontan completion.

Main Results:

• Group 1 (infants with native SAS) had significant operative mortality (3/11) with mixed outcomes after initial repairs.
• Group 2 (acquired SAS post-pulmonary artery banding) showed 11% Fontan mortality after staged or concurrent SAS relief.
• Groups 3 and 4 (SAS diagnosed at Fontan evaluation or later) demonstrated successful Fontan completion with SAS relief.

Conclusions:

• Surgical relief of subaortic stenosis in univentricular hearts can be achieved through various methods, including Damus-Kaye-Stansel connection and subaortic resection.
• Outcomes vary based on initial presentation, age, and surgical strategy, with higher mortality in neonates with complex arch anomalies.
• Staged or concurrent management of SAS is feasible and associated with acceptable Fontan completion rates and survival.