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努南综合征患儿球囊肺动脉瓣成形术的结果

Results of balloon pulmonary valvoplasty in children with Noonan's syndrome.

作者信息

Holzmann Jonathon, Tibby Shane M, Rosenthal Eric, Qureshi Shakeel, Morgan Gareth, Krasemann Thomas

机构信息

1Department of Paediatric Cardiology,Evelina London Children's Hospital,Westminster Bridge Road,London,UK.

2Department of Paediatric Intensive Care,Evelina London Children's Hospital,Westminster Bridge Road,London,UK.

出版信息

Cardiol Young. 2018 May;28(5):647-652. doi: 10.1017/S1047951117002827. Epub 2018 Jan 10.

DOI:10.1017/S1047951117002827
PMID:29316997
Abstract

UNLABELLED

Pulmonary valve stenosis is common in patients with Noonan's syndrome. The response to balloon valvoplasty varies.We assessed the correlation between re-intervention rate, immediate response, and the progress of the valve gradient over time after intervention.

METHODS

This is a retrospective study conducted from 1995 to 2014.

RESULTS

Of 14 patients identified, seven had re-intervention 28±54 months (range 3-149, median 3.3) after valvoplasty. These patients did not have a significant decrease in gradient after intervention. Their gradient subsequently decreased during follow-up and then became static before increasing years after intervention. In contrast, the gradient of patients not requiring further intervention continually reduced over time. Demographics did not differ between these groups.

CONCLUSION

We could not identify predisposing factors for long-term success of pulmonary valvoplasty in Noonan's patients, but the trajectory of gradients differs significantly between patients needing re-intervention from those who remain free from re-intervention.

摘要

未标注

肺动脉瓣狭窄在努南综合征患者中很常见。球囊瓣膜成形术的反应各不相同。我们评估了再次干预率、即刻反应与干预后瓣膜压差随时间变化之间的相关性。

方法

这是一项1995年至2014年进行的回顾性研究。

结果

在确定的14例患者中,7例在瓣膜成形术后28±54个月(范围3 - 149个月,中位数3.3个月)进行了再次干预。这些患者干预后压差没有显著降低。随后,他们的压差在随访期间下降,然后在干预后数年保持稳定,之后又升高。相比之下,不需要进一步干预的患者的压差随时间持续降低。两组患者的人口统计学特征无差异。

结论

我们无法确定努南综合征患者肺动脉瓣成形术长期成功的 predisposing 因素,但需要再次干预的患者与无需再次干预的患者之间,压差的变化轨迹有显著差异。

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