Journal of Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2433-1783 Print ISSN: 2433-2720
Japanese Society of Pediatric Cardiology and Cardiac Surgery
Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Journal of Pediatric Cardiology and Cardiac Surgery 2(1): 49-54 (2018)
doi:10.24509/jpccs.180106

Original ArticleOriginal Article

The Outcomes of Bicuspid Aortic Valve and Valve Dysfunction from Infancy to Early Adulthood

Sayaka Mii1，Tameo Hatano1，Daichi Fukumi1，Yasuaki Kishimoto1，Masanobu Ikoma2，Satoru Kawai3，Yoshie Fukasawa4Sayaka Mii1, Tameo Hatano1, Daichi Fukumi1, Yasuaki Kishimoto1, Masanobu Ikoma2, Satoru Kawai3, Yoshie Fukasawa4

1 Department of Pediatric Cardiology, Japanese Red Cross Nagoya Daiichi Hospital ◇ Aichi, Japan

2 Department of Pediatrics, Toyota Kosei Hospital ◇ Aichi, Japan

3 Department of Cardiology, Aichi Children’s health and Medical Center ◇ Aichi, Japan

4 Department of Pediatrics, Nagoya University Hospital ◇ Aichi, Japan

受付日：2017年1月17日Received: January 17, 2017
受理日：2017年12月1日Accepted: December 1, 2017
発行日：2018年3月31日Published: March 31, 2018
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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.

Key words: bicuspid aortic valve; progressive valve dysfunction; progressive aortic dilatation

© 2018 特定非営利活動法人日本小児循環器学会© 2018 Japanese Society of Pediatric Cardiology and Cardiac Surgery

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