Yamauchi Melissa S W, Puchalski Michael D, Weng Hsin Ti, Pinto Nelangi M, Etheridge Susan P, Presson Angela P, Tani Lloyd Y, Minich L LuAnn, Williams Richard V
Department of Pediatrics, Division of Pediatric Cardiology, University of Utah and Primary Children's Hospital, Salt Lake City, Utah, USA.
Department of Pediatrics, University of Utah and Primary Children's Hospital, Salt Lake City, Utah, USA.
Congenit Heart Dis. 2018 May;13(3):432-439. doi: 10.1111/chd.12591. Epub 2018 Feb 21.
Disease progression of an isolated bicuspid aortic valve (BAV) in children is poorly understood and adult management guidelines may not be applicable. Thus, we sought to evaluate disease progression of pediatric isolated BAV and its relationship to current management practices.
Children with a BAV and ≤mild aortic stenosis (AS) and/or aortic regurgitation (AR) at the time of initial evaluation were included in this retrospective cohort study (1/2005-12/2014). Outcomes included change in z-scores for aortic root and ascending aorta diameters, cardiac interventions, adverse outcomes, recommended follow-up interval, and frequency of cardiac imaging studies at each follow up evaluation, as well as AS/AR severity at final evaluation. Outcomes were analyzed using generalized mixed-effect models with subject and provider clustering.
BAV disease progression was evaluated in 294 subjects over 4.1 ± 2.4 (range 0.2-9.5) years. Ascending aorta z-scores increased by 0.1/year (P < .001) but aortic root diameter z-scores were unchanged. AS and/or AR progressed to >mild in 9 (3%), 1 subject underwent cardiac intervention, and none had a major complication. Management was evaluated in 454 subjects (1343 encounters) with 27 different cardiologists. The average recommended follow-up interval was 1.5 ± 0.9 years. Younger age at diagnosis, greater aortic root or ascending aorta z-score at diagnosis, ≥mild AS/AR at follow-up, and earlier diagnosis era were associated with shorter recommended follow-up interval (P < .001 for all). Imaging was obtained at 87% of follow-up encounters and was associated with age at encounter with children ≥12 years most frequently imaged (P < .001). Provider accounted for 14% of variability in recommended follow-up interval and 24% of imaging variability (P < .001 for both).
We found little to no evidence of disease progression in children with an isolated BAV. Given the low risk, close follow-up and frequent cardiac imaging for BAV surveillance may not be warranted for children.
儿童孤立性二叶式主动脉瓣（BAV）的疾病进展情况了解甚少，成人管理指南可能并不适用。因此，我们试图评估儿童孤立性BAV的疾病进展及其与当前管理实践的关系。
本回顾性队列研究纳入了初始评估时患有BAV且主动脉狭窄（AS）和/或主动脉反流（AR）≤轻度的儿童（2005年1月至2014年12月）。结局包括主动脉根部和升主动脉直径的z值变化、心脏干预、不良结局、推荐的随访间隔、每次随访评估时心脏成像研究的频率，以及最终评估时的AS/AR严重程度。使用具有受试者和提供者聚类的广义混合效应模型分析结局。
对294名受试者进行了4.1±2.4（范围0.2 - 9.5）年的BAV疾病进展评估。升主动脉z值每年增加0.1（P <.001），但主动脉根部直径z值无变化。9名（3%）患者的AS和/或AR进展为>轻度，1名患者接受了心脏干预，无患者发生重大并发症。对454名受试者（1343次就诊）进行了管理评估，涉及27位不同的心脏病专家。平均推荐随访间隔为1.5±0.9年。诊断时年龄较小、诊断时主动脉根部或升主动脉z值较高、随访时AS/AR≥轻度以及诊断时代较早与较短的推荐随访间隔相关（所有P <.001）。87%的随访就诊进行了成像检查，且与就诊年龄相关，12岁及以上儿童成像频率最高（P <.001）。提供者占推荐随访间隔变异性的14%，成像变异性的24%（两者P <.001）。
我们发现几乎没有证据表明孤立性BAV儿童存在疾病进展。鉴于风险较低，对于儿童BAV监测，可能无需密切随访和频繁进行心脏成像。
