Four-Patch Repair for Supravalvar Aortic Stenosis

Case Presentation: 

This is a case presentation of a 2-month-old female infant who was diagnosed postnatally with a 2/6 ejection murmur. A transesophageal echocardiogram indicated severe supravalvar aortic stenosis at the sinotubular (ST) junction, mild aortic insufficiency, and a patent foramen ovale. Further imaging by the cardiology team illustrated normal coronary arteries and a 68-mmHg gradient from the left ventricular outflow tract (LVOT) to the ascending aorta. Additionally, microarray analysis was completed to rule out Williams syndrome. 

Due to the increased pressure gradient and the severe supravalvar aortic stenosis, the cardiothoracic surgical team utilized a three-patch Brom repair to enlarge the ST junction. However, due to the decreased diameter of the proximal ascending aorta, an additional fourth patch was used for augmentation. 

Surgery: 

The patient was first heparinized and cannulated bicavally, and cardiopulmonary bypass commenced. The ascending aorta was then divided above the commissural posts, and the noncoronary sinus was then incised to allow for inspection of the left coronary ostia and a notably thickened aortic valve, a common problem associated with supravalvar aortic stenosis. 
 
Additional incisions were made to the right of the left coronary artery and left of the right coronary artery to widen the diameter of the aortic root (1). A 10 mm graduated dilator was brought into the surgical field as a template for Cormatrix patch augmentation. Three Cormatrix patches were cut into U-shaped segments and sewn into the incisions along the aortic trunk to increase overall diameter and alleviate aortic stenosis. The 10 mm graduated dilator was then used to check the diameter of the aortic trunk, and saline was injected to ensure aortic valve coaptation with uncompromised leaflet mobility. The enlarged aortic trunk was then partially anastomosed back to the ascending aorta. The ascending aorta was incised, and a fourth Cormatrix patch was cut in a similar U-shaped fashion and anastomosed to the aortic root (2). 
 
Later in the procedure, a patent foramen ovale was closed, but this was deemed not relevant for the presentation of using a modified Brom procedure to alleviate supravalvar aortic stenosis. After administration of protamine and adequate hemostasis, the patient was closed in the standard fashion. 
 
Postoperation: 

A postoperative TEE showed a hyperdynamic left ventricle, a normal right ventricle, mild aortic insufficiency, and a 12 mmHg ST junction gradient. The chest tubes were removed on postoperative day two and the patient was discharged on postoperative day six.  

References

1. Mitchell, M.B. and S.P. Goldberg, Brom Repair for Supravalvar Aortic Stenosis. Operative Techniques in Thoracic and Cardiovascular Surgery, 2011. 16(1): p. 70-84.
2. Hazekamp, M.G., et al., Brom’s three-patch technique for repair of supravalvular aortic stenosis. The Journal of Thoracic and Cardiovascular Surgery, 1999. 118(2): p. 252-258.

Disclaimer

The information and views presented on CTSNet.org represent the views of the authors and contributors of the material and not of CTSNet. Please review our full disclaimer page here.