OBJECTIVES: Congenital supravalvular aortic stenosis (SVAS) is a rare arteriopathy associated with the Williams-Beuren syndrome (WBS) and other elastin gene deletions. Our objective was to review the mid-term outcomes of SVAS repair with extended aortoplasty. METHODS: Congenital SVAS repairs from 2001 to 2010 were retrospectively reviewed. The follow-up records, reintervention and reoperation data and most recent echocardiograms were obtained. RESULTS: From 2001 to 2010, 21 patients (15 males) underwent surgical repair of SVAS by extended aortoplasty with autologous pretreated pericardium, which is a modification of the Doty technique. The mean age was 3.1 ± 4.2 years. WBS was diagnosed in 14 of the patients. There was no early mortality, but one late death was observed. At the latest follow-up (mean follow-up, 4.3 ± 2.9 years; range, 1-108 months), echocardiograms revealed a peak Doppler gradient across the aortic outflow tract of 15 ± 8 mmHg. The majority of the patients had minimal to mild aortic insufficiency. No reoperation or reintervention was required. CONCLUSIONS: Extended aortoplasty provides excellent mid-term relief of SVAS and, in addition, reshapes the aortic root geometry to a much more favourable anatomical configuration. It can be performed without any increase in operative risks. The mid-term results are excellent.
OBJECTIVES:Congenital supravalvular aortic stenosis (SVAS) is a rare arteriopathy associated with the Williams-Beuren syndrome (WBS) and other elastin gene deletions. Our objective was to review the mid-term outcomes of SVAS repair with extended aortoplasty. METHODS: Congenital SVAS repairs from 2001 to 2010 were retrospectively reviewed. The follow-up records, reintervention and reoperation data and most recent echocardiograms were obtained. RESULTS: From 2001 to 2010, 21 patients (15 males) underwent surgical repair of SVAS by extended aortoplasty with autologous pretreated pericardium, which is a modification of the Doty technique. The mean age was 3.1 ± 4.2 years. WBS was diagnosed in 14 of the patients. There was no early mortality, but one late death was observed. At the latest follow-up (mean follow-up, 4.3 ± 2.9 years; range, 1-108 months), echocardiograms revealed a peak Doppler gradient across the aortic outflow tract of 15 ± 8 mmHg. The majority of the patients had minimal to mild aortic insufficiency. No reoperation or reintervention was required. CONCLUSIONS: Extended aortoplasty provides excellent mid-term relief of SVAS and, in addition, reshapes the aortic root geometry to a much more favourable anatomical configuration. It can be performed without any increase in operative risks. The mid-term results are excellent.
Authors: Minoo N Kavarana; Meghan Riley; Vikram Sood; Richard G Ohye; Eric J Devaney; Edward L Bove; Jennifer C Hirsch Journal: Ann Thorac Surg Date: 2012-03-03 Impact factor: 4.330
Authors: Daniel J Scott; David N Campbell; David R Clarke; Steven P Goldberg; Daniel R Karlin; Max B Mitchell Journal: Ann Thorac Surg Date: 2009-05 Impact factor: 4.330
Authors: Rosa Roemers; Jolanda Kluin; Frederiek de Heer; Sara Arrigoni; Regina Bökenkamp; Joost van Melle; Tjark Ebels; Mark Hazekamp Journal: World J Pediatr Congenit Heart Surg Date: 2018-03