Department of Pediatrics, University of Wisconsin, Madison, WI, USA.
Department of Pediatrics, Division of Pediatric Cardiology, University of Wisconsin, Madison, WI, USA.
Cardiol Young. 2022 Jan;32(1):24-30. doi: 10.1017/S1047951121001499. Epub 2021 May 4.
Prior to the recent release of appropriate use criteria for imaging valvulopathies in children, follow-up of valvular lesions, including isolated bicuspid aortic valve, was not standardised. We describe current follow up, treatment, and intervention strategies for isolated bicuspid aortic valve with varying degrees of stenosis, regurgitation, and dilation in children up to 18 years old and compare them with newly released appropriate use criteria.
Online survey was sent to members of the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery and PediHeartNet.
Totally, 106 responses with interpretable data were received. For asymptomatic patients with isolated BAV without stenosis, regurgitation, or dilation follow-up-intervals increased from 7+/-4 months in the newborn period to 28 +/- 14 months at 18 years of age. Respondents recommended more frequent follow-up for younger patients and those with greater disease severity. More than 80% of respondents treat aortic regurgitation or aortic dilation in the setting of bicuspid aortic valve medically. In general, intervention was recommended once stenosis or regurgitation became severe (stenosis of >4 m/s; regurgitation with LV Z score 4) regardless of age, but was not routinely recommended for younger children (newborn - age 6 years) with severe dilation. Exercise was restricted at 38+/-11 mmHg echocardiographic mean gradient.
Current follow-up, treatment, and intervention strategies for isolated bicuspid aortic valve deviate from appropriate use criteria. Differences between the two highlight the need to better delineate the disease course, clarify recommendations for care, and encourage wider adoption of guidelines.
在最近发布儿童瓣膜病影像学适用标准之前，瓣膜病变的随访，包括孤立性二叶式主动脉瓣，并未标准化。我们描述了目前对 18 岁以下不同程度狭窄、反流和扩张的孤立性二叶式主动脉瓣的随访、治疗和干预策略，并将其与新发布的适用标准进行比较。
向美国儿科学会心脏病学和心脏外科学分会以及 PediHeartNet 的成员发送了在线调查。
共收到 106 份具有可解释数据的回复。对于无症状的孤立性 BAV 患者，无狭窄、反流或扩张，随访间隔从新生儿期的 7+/-4 个月增加到 18 岁时的 28 +/- 14 个月。受访者建议对年龄较小的患者和病情较重的患者进行更频繁的随访。超过 80%的受访者对二叶式主动脉瓣伴主动脉瓣反流或主动脉瓣扩张进行药物治疗。一般来说，一旦出现狭窄或反流（狭窄>4m/s；LV Z 评分 4 的反流），无论年龄大小，都会建议进行干预，但对于年龄较小（新生儿-6 岁）且严重扩张的儿童，不会常规建议进行干预。当超声心动图平均梯度为 38+/-11mmHg 时，限制运动。
目前孤立性二叶式主动脉瓣的随访、治疗和干预策略与适用标准不符。两者之间的差异突出表明需要更好地描述疾病过程，阐明护理建议，并鼓励更广泛地采用指南。
