Jacksonville Pediatric and Adult Congenital Cardiology, Florida Pediatric Associates, Jacksonville, FL, USA.
Mayne Medical School, The University of Queensland, Brisbane, QLD, Australia.
Cardiol Young. 2022 Jul;32(7):1071-1076. doi: 10.1017/S1047951121003668. Epub 2021 Sep 8.
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.
奇阿利氏网是右心房内一种不常见的网状结构，通常较薄且细小。偶尔，它可能会突出并在舒张期延伸到右心室，并且已经报道了相关并发症。本研究共纳入 38 例奇阿利氏网脱垂患者，记录并分析其伴发的心脏异常。测量并比较了超声心动图的右心和左心参数与正常参考值。在初始表现时，奇阿利氏网脱垂至三尖瓣环下方的程度为 9.1 ± 3.5mm（平均值 ± 标准差），肺动脉瓣环直径 z 值降低（-0.91 ± 0.64），主动脉瓣环 z 值增大（+0.97 ± 0.87）。14 例患者无其他心脏异常。8 例存在房间隔缺损，8 例表现为室上性心律失常，6 例存在轻度至中度三尖瓣反流，1 例分别存在房间隔瘤、重度肺动脉瓣狭窄、小膜周室间隔缺损、二叶主动脉瓣和轻度右上肺静脉狭窄。14 例患者（37%）在 1 至 8.5 年后（中位时间 3.2 年）接受了随访。在此期间，三尖瓣的脱垂程度减少了多达 7mm（中位数为 2mm）。3 例患者需要介入治疗，但许多相关的心脏异常随着生长而自发缓解。因此，奇阿利氏网脱垂存在发生相关 CHD 的重大风险。然而，随着生长，奇阿利氏网脱垂的程度逐渐减小，许多相关异常也会自行缓解。
