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[Direct aortic reimplantation for BWG syndrome including mitral valve repair]
Insights
Direct aortic reimplantation is ideal for Bland-Garland-White (BWG) syndrome, even with coronary artery anomalies. Surgical techniques successfully treated two boys with BWG syndrome, improving cardiac function and mitral valve function.
Area of Science:
- Cardiovascular Surgery
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Bland-Garland-White (BWG) syndrome, characterized by anomalous origin of the coronary artery from the pulmonary artery, presents surgical challenges.
- Direct aortic reimplantation is the preferred treatment, but achieving adequate coronary artery length without tension can be difficult.
- Mitral valve regurgitation is a significant negative prognostic factor in BWG syndrome.
Observation:
- A 6-year-old boy presented with a left coronary artery originating from the pulmonary trunk, distant from the aorta.
- A 3-year-old boy had BWG syndrome with severe mitral valve regurgitation.
- Surgical intervention involved direct aortic reimplantation and, in one case, simultaneous mitral valve repair.
Findings:
- Successful direct aortic reimplantation was achieved in the 6-year-old by creating a large ostial flange and mobilizing the coronary artery.
- Simultaneous direct aortic reimplantation and mitral valve repair (anterior commissuroplasty) were performed in the 3-year-old.
- Postoperative data indicated improved cardiac performance in both patients.
Implications:
- This approach demonstrates the feasibility of direct aortic reimplantation for BWG syndrome, even with challenging coronary artery anatomy.
- Simultaneous mitral valve repair offers a potential strategy to improve outcomes in BWG syndrome patients with mitral regurgitation.
- The findings suggest the possibility of restoring papillary muscle function through valve repair, avoiding the need for valve replacement.
Abstract:
Direct aortic reimplantation is the ideal treatment for the BWG syndrome, but is often impossible to obtain a sufficient length of the coronary artery without undue tension on the vessel. We experienced a 6-year-old boy whose left coronary artery was originated from pulmonary trunk widely distant from aorta. Direct reimplantation was successfully performed taking care of excising the ostium of the anomalous vessel as a largest flange and mobilizing the coronary artery from the adjacent tissue sufficiently. Mitral valve regurgitation is also a poor prognosis factor of BWG syndrome. We performed direct reimplantation and mitral valve repair (anterior commissuroplasty) simultaneously to a 3-year-old boy of BWG syndrome with severe mitral valve regurgitation. Postoperative data showed improvement of cardiac performance. Possibility of recovering the papillary muscle function by valve repair without replacement was suggested.