Surgical strategy for severe aortic hypoplasia and aortic stenosis with ventricular septal defect and normal left ventricle.

At our institution, the strategy for patients with bicuspid aortic valve, aortic valve stenosis (<5 mm), and aortic hypoplasia [hypoplastic aortic arch, coarctation of the aorta (CoA), or interrupted aortic arch (IAA)] with ventricular septal defects (VSDs) as well as normal left ventricular (LV) volume and mitral valve size consists of two parts. The Norwood operation is applied as the first palliation for this group of patients. Second, the decision whether the patients are to undergo the Rastelli operation or a univentricular repair is made depending on the size of the right ventricle after the Norwood operation. This study aimed to examine whether the aforementioned surgical strategy for this group of patients is adequate or not. Seven patients undergoing the Norwood operation as the first palliation for bicuspid aortic valve, aortic valve stenosis (<5 mm), and aortic hypoplasia with VSDs as well as normal LV volume and mitral valve size between February 2005 and March 2010 at Kitasato University Hospital and the Gunma Children's Medical Center were reviewed. Postoperative serum B-type natriuretic peptide (BNP) and central venous pressure (CVP) were measured in the patients undergoing the staged Norwood-Rastelli operation to assess whether the authors' right ventricular end-diastolic volume index (RVEDVI) cutoff (80 % of normal) is adequate. At this writing, all seven patients are alive after a mean follow-up period of 58.8 ± 17.8 months. They all had aortic valve stenosis of <5 mm and a bicuspid aortic valve. Four patients had a diagnosis of CoA with VSD, and three patients had IAA with VSD. Six patients underwent biventricular repair, and one patient had univentricular repair due to the small RVEDVI (74 % of normal). The patients with 80-90 % of normal RVEDVI had higher BNP and higher CVP than those with more than 90 % of normal RVEDVI after the Rastelli operation, whereas the patient undergoing the Fontan operation had a low BNP level. In conclusion, the described strategy for patients with severe aortic hypoplasia and aortic stenosis with VSD as well as normal LV and mitral valve size is reasonable.