2018 年 2 巻 1 号 p. 49-54
Background: The clinical outcomes of pediatric patients with bicuspid aortic valve (BAV) are unclear. The aim of this study was to explore the short and mid-term outcomes and the risk of the progression of valve dysfunction.
Methods: We evaluated the fusion type of cusps and the valve dysfunction of 34 of 80 patients (6 of 30 screened infants and 28 of 50 outpatients) with BAV by echocardiography, from January 2009 to May 2016.
Results: Among 34 BAV patients without any complications, right- noncoronary cusp (R-N) fusion was the most common (62%) finding, followed by left-noncoronary cusp (L-N) fusion (32%). The progression of aortic regurgitation (AR) was observed in 6 patients (R-N, n=3; L-R, n=3). In contrast, AS progressed in one patient and improved in 6 patients. However, AR remained mild in most cases (76%) and progressed to a moderate or severe state in a few patients (19%) who were 8–20 years of age.
Conclusions: With the exception of cases involving infants with severe AS, AS remained unchanged, while AR mildly progressed in a pediatric population with BAV. It appears that the progression to a moderate or severe degree of AR typically occurs at 8 to 20 years of age.
