[Reoperation for diffuse supravalvular aortic stenosis with Williams syndrome--extended patch aortoplasty and extra-anatomic bypass from the ascending aorta to the descending aorta in a median sternotomy].
A case of diffuse supravalvular aortic stenosis (SVAS) with Williams syndrome is reported. In this case of severe diffuse SVAS, we performed the diamond-patch aortoplasty in a child. However he has been suffering from residual SVAS. At 9-years old, the myocardial injury was noted by myocardial scintigraphy. Preoperative cardiac catheterization and angiography revealed the hypoplastic ascending aorta and arch with a pressure gradient of 89 mmHg at the distal site from the left subclavian artery. Through only a median stenotomy, an extended patch aortoplasty between the valsalva sinus and distal arch was performed and an extraanatomic bypass from the ascending aorta to the descending aorta was employed using a 10 mm tube graft. We realize this technique is available because this method can relieve the left ventriculus of the pressure load and operate via only median sternotomy.
Kumada
Y
Y
First Department of Surgery, Gifu University, Japan.
Yasuda
H
H
Sasaki
E
E
Murakawa
S
S
Mori
Y
Y
Hirose
H
H
D001011:Aorta / Q000601:surgery*; D001013:Aorta, Thoracic / Q000601:surgery*; D001024:Aortic Valve Stenosis / Q000601:surgery*; D019917:Blood Vessel Prosthesis Implantation; D002648:Child; D006801:Humans; D008297:Male; D008722:Methods; D012086:Reoperation; D013249:Sternum / Q000601:surgery; D018980:Williams Syndrome / Q000601:surgery*