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Options for surgical repair in hearts with univentricular atrioventricular connection and subaortic stenosis
H C Cheung1, C Lincoln, R H Anderson
1National Heart and Lung Institute and Hospitals, London, England.
Insights
Surgical enlargement of the ventricular septal defect effectively relieves subaortic obstruction in hearts with a univentricular atrioventricular connection, offering a safe and hemodynamically sound treatment option with low mortality.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Cardiac Physiology
Background:
- Subaortic obstruction is a critical complication in hearts with a univentricular atrioventricular connection.
- Effective surgical management is essential for improving outcomes in these complex congenital heart defects.
Purpose of the Study:
- To evaluate the efficacy and safety of surgical interventions for subaortic obstruction in patients with univentricular atrioventricular connections.
- To compare outcomes between surgical enlargement of the ventricular septal defect and the Damus-Kaye-Stansel procedure.
Main Methods:
- Retrospective review of 13 patients undergoing surgical treatment for subaortic obstruction.
- Procedures included ventricular septal defect enlargement and the Damus-Kaye-Stansel procedure, with some patients also undergoing a modified Fontan procedure.
- Postoperative assessment using cardiac catheterization, angiography, and Doppler flow studies.
Main Results:
- Surgical enlargement of the ventricular septal defect was performed in 9 patients; the Damus-Kaye-Stansel procedure in 4.
- Overall operative mortality was low, with one early death after defect enlargement and two after the Damus-Kaye-Stansel procedure.
- All 10 assessed patients showed satisfactory relief of subaortic obstruction, with good hemodynamic results.
Conclusions:
- Direct surgical enlargement of a restrictive ventricular septal defect is recommended for subaortic stenosis in univentricular hearts with a dominant left ventricle.
- The Damus-Kaye-Stansel procedure is a viable alternative for relieving subaortic stenosis when the atria connect to a dominant right ventricle.
Abstract:
Thirteen patients have undergone surgical treatment because of subaortic obstruction in hearts with a univentricular atrioventricular connection. Nine patients underwent surgical enlargement of the ventricular septal defect and four patients had construction of an aortopulmonary anastomosis and closure of the pulmonary trunk (the Damus-Kaye-Stansel procedure). Two patients undergoing enlargement of the septal defect and two having the Damus-Kaye-Stansel procedure also had a modified Fontan procedure. One patient had complete atrioventricular dissociation after direct enlargement of the ventricular septal defect, which necessitated insertion of an epicardial pacemaker. One patient died within 30 days of the operation after enlargement of the defect and two patients after the Damus-Kaye-Stansel procedure. There was one late death, occurring in a patient who underwent enlargement of the ventricular septal defect. Ten patients have subsequently undergone conventional cardiac catheterization and angiography or transcutaneous Doppler flow studies to assess the relief of the subaortic obstruction. The result has been satisfactory in all. Because of this experience, we now recommend direct surgical enlargement of the restrictive ventricular septal defect for direct relief of subaortic stenosis occurring with a univentricular atrioventricular connection to a dominant left ventricle, inasmuch as it appears to be hemodynamically effective with a low operative mortality and morbidity. The Damus-Kaye-Stansel procedure can also have a role in relieving subaortic stenosis when the atria are connected to a dominant right ventricle.