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Prominent prolapsing Chiari network: presentation and prognosis in paediatric patients
Cardiology in the Young ( IF 1 ) Pub Date : 2021-09-08 , DOI: 10.1017/s1047951121003668
James J Joyce 1 , Thomas R Joyce 1, 2 , Sunita J Ferns 3
Affiliation  

Chiari network is an infrequently visualized web-like structure in the right atrium that is usually thin and small. Rarely, it can be prominent and elongated with protrusion into the right ventricle during diastole and complications have been reported. Thirty-eight patients (median age 2.5 years) with prolapsing Chiari network were identified and associated cardiac abnormalities documented. Echocardiographic right and left heart parameters were measured and compared to normative data. At presentation, the extent of Chiari network prolapse below the tricuspid annulus was 9.1 ± 3.5 mm (mean ± standard deviation), mean pulmonary valve annulus diameter z-value was reduced (-0.91 ± 0.64), and mean aortic valve z-value was enlarged (+0.97 ± 0.87). Fourteen patients exhibited no other cardiac abnormality. Eight were noted to have atrial septal defects, eight demonstrated supraventricular dysrhythmias, six had mild to moderate tricuspid valve regurgitation, and one patient each had an atrial septal aneurysm, severe pulmonic valve stenosis, small perimembranous ventricular septal defect, bicuspid aortic valve, and mild right upper pulmonary vein stenosis. Fourteen patients (37%) were seen in follow-up from 1 to 8.5 years later (median 3.2 years). During that time, the magnitude of prolapse across the tricuspid valve decreased by up to 7 mm (median 2 mm). Interventions were required in three patients, but many associated cardiac abnormalities resolved spontaneously with growth. Thus, the presence of a prolapsing Chiari network has a substantial risk of associated CHDs. However, the extent of Chiari network prolapse gradually decreases and many related abnormalities resolve during growth.



中文翻译:

突出的脱垂 Chiari 网络:儿科患者的表现和预后

Chiari 网络是右心房中不常见的网状结构,通常又细又小。极少数情况下,它会在舒张期突出并伸长并伸入右心室,据报道有并发症。确定了 38 名患有 Chiari 网络脱垂的患者(中位年龄 2.5 岁)并记录了相关的心脏异常。测量超声心动图左右心脏参数并与标准数据进行比较。就诊时,三尖瓣环下方 Chiari 网络脱垂的程度为 9.1 ± 3.5 mm(平均值 ± 标准差),平均肺动脉瓣环直径 z 值减小(-0.91 ± 0.64),平均主动脉瓣 z 值放大 (+0.97 ± 0.87)。十四名患者没有表现出其他心脏异常。八人有房间隔缺损,8例室上性心律失常,6例轻度至中度三尖瓣关闭不全,1例房间隔瘤、重度肺动脉瓣狭窄、小膜周室间隔缺损、二尖瓣主动脉瓣和轻度右上肺静脉狭窄。14 名患者(37%)在 1 至 8.5 年后(中位 3.2 年)进行了随访。在此期间,三尖瓣脱垂的幅度减少了 7 毫米(中位数 2 毫米)。三名患者需要进行干预,但许多相关的心脏异常随着生长而自发消退。因此,脱垂的 Chiari 网络的存在具有相关 CHD 的重大风险。然而,Chiari 网络脱垂的程度逐渐降低,并且许多相关异常在生长过程中得到解决。

更新日期:2021-09-08
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