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先天性心脏病患儿肺血流限制器的手术切除:结果揭示了什么。

Surgical removal of pulmonary flow restrictors in children with congenital heart disease: What the outcomes reveal.

作者信息

Haddad Raymond N, Bentham Jamie, Pardo Carlos, Alloush Rasha, Al Soufi Mahmoud, Jaber Osama, Kasem Mohamed, El Rassi Issam

机构信息

Service de cardiologie congénitale et pédiatrique, centre de référence des malformations cardiaques congénitales complexes - M3C-Necker, Hôpital Universitaire Necker-Enfants malades, Assistance Publique-Hôpitaux de Paris, Paris, France.

Department of Congenital Cardiology, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom.

出版信息

JTCVS Open. 2024 Nov 29;23:235-244. doi: 10.1016/j.xjon.2024.11.011. eCollection 2025 Feb.

Abstract

OBJECTIVE

Pulmonary flow restrictors (PFRs) are interesting devices, but their surgical removal outcomes are poorly understood.

METHODS

Retrospective review of clinical data from children with bilateral PFRs who underwent device removal during follow-up surgery.

RESULTS

Thirty-four PFRs were explanted from 17 patients (41.2% boys) at a median of 2 months (interquartile range [IQR], 1.2-5.2 months) postimplantation, with a median patient age of 2.5 months (IQR, 1.6-5.8 months). One patient experienced life-threatening bilateral pulmonary artery (PA) aneurysms 2 months after PFR implantation, necessitating urgent surgery. Two PFRs were found migrated across the left PA's upper lobe branch origin. Twenty-six were removed intact, 1 in 2 fragments, and 7 piecemeal. No thrombus was noted. Neoendothelium was observed on 11 PFRs. Seven PFRs caused endothelial damage, requiring sharp and blunt dissection for removal. Six right and 4 left PA arteriotomies were patched. Hegar dilators, with median sizes of 7 mm (IQR, 6.8-8.3 mm) for right PA and 7 mm (IQR, 7-8 mm) for left PA, confirmed branch patency. At a median follow-up of 14.8 months (IQR, 10.2-18.3 months), echocardiographic maximum velocities in 13 biventricular patients and 2 awaiting future biventricular repair were 1.5 m/second (IQR, 1.4-1.7 m/second) for the left PA and 1.6 m/second (IQR, 1.4-1.7 m/second) for the right PA. One patient with deferred Norwood had normal PAs and well-positioned PFRs on prestage-II catheterization. A patient who underwent stage-II Norwood 3.2 months post-PFR implantation died from sepsis 1 month later, but post-Glenn angiogram revealed no stenosis.

CONCLUSIONS

PFR removal is safe and effective. Complications are manageable, with no PA stenosis observed.

摘要

目的

肺血流限制器(PFRs)是一种有趣的装置,但其手术取出的结果尚不清楚。

方法

回顾性分析随访手术中取出双侧PFRs的儿童的临床资料。

结果

17例患者(41.2%为男孩)共取出34个PFRs,植入后中位时间为2个月(四分位间距[IQR],1.2 - 5.2个月),患者中位年龄为2.5个月(IQR,1.6 - 5.8个月)。1例患者在PFR植入后2个月出现危及生命的双侧肺动脉(PA)动脉瘤,需要紧急手术。发现2个PFRs迁移至左肺动脉上叶分支起始处。26个完整取出,1个分成2块取出,7个碎块取出。未发现血栓。11个PFRs上观察到新生内皮。7个PFRs造成内皮损伤,需要锐性和钝性分离以取出。修补了6个右肺动脉和4个左肺动脉切开处。Hegar扩张器显示,右肺动脉中位尺寸为7mm(IQR,6.8 - 8.3mm),左肺动脉中位尺寸为7mm(IQR,7 - 8mm),确认分支通畅。中位随访14.8个月(IQR,10.2 - 18.3个月)时,13例双心室患者和2例等待未来双心室修复患者的超声心动图显示,左肺动脉最大流速为1.5m/秒(IQR,1.4 - 1.7m/秒),右肺动脉最大流速为1.6m/秒(IQR,1.4 - 1.7m/秒)。1例延期进行诺伍德手术的患者在II期术前导管检查时肺动脉正常且PFRs位置良好。1例在PFR植入后3.2个月进行II期诺伍德手术的患者1个月后死于败血症,但格伦术后血管造影未显示狭窄。

结论

PFR取出安全有效。并发症可控,未观察到肺动脉狭窄。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d1a3/11883757/a47731731472/fx1.jpg

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