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多发性室间隔缺损:一种新策略。

Multiple ventricular septal defects: a new strategy.

机构信息

King Fahad Medical City Riyadh, Saudi Arabia.

Alder Hey Children's NHS Foundation Trust Liverpool, UK.

出版信息

Front Pediatr. 2013 Jul 31;1:16. doi: 10.3389/fped.2013.00016. eCollection 2013.

DOI:10.3389/fped.2013.00016
PMID:24400262
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3860893/
Abstract

INTRODUCTION

A multicenter prospective study was conducted to evaluate a new strategy for multiple Ventricular Septal Defects (VSDs).

MATERIALS AND METHODS

From 2004 to 2012 17 consecutive children (3 premature, 14 infants), mean age 3.2 months (9 days-9 months), mean body weight 4.2 kg (3.1-6.1 kg), with multiple VSDs underwent Pulmonary Artery Banding (PAB) with an adjustable FloWatch-PAB(®). Associated cardiac anomalies included patent ductus arteriosus (1), aortic coarctation (2), hypoplastic aortic arch (2), and left isomerism (3). Five patients (5/17 = 29.4%) required pre-operative mechanical ventilation, with a mean duration of 64 days (7-240 days)

RESULTS

There were no early or late deaths during a mean follow-up of 48 months (7-98 months), with either FloWatch removal or last observation as end-points. FloWatch-PAB(®) adjustments were required in all patients: a mean of 4.8 times/patient (2-9) to tighten the PAB, and a mean of 1.1 times/patient (0-3) to release the PAB with the patient's growth. After a mean interval of 29 months (8-69 months) 10/17 (59%) patients underwent re-operation: 7/10 PAB removal, with closure of a remaining unrestrictive VSD in 6 (peri-membranous in 3 patients, mid-muscular in 2, and inlet in 1) and Damus-Kaye-Stansel, bi-directional Glenn, and atrial septectomy in 1; 3/9 patients required only PAB removal. All muscular multiple VSDs had closed in all 10 patients. PA reconstruction was required in 1/10 patient. In 5/7 of the remaining patients with the PAB still in situ, all muscular VSDs had already closed. The only 2 patients with persistent muscular multiple VSDs are the 2 patients with the shortest follow-up.

CONCLUSION

This reproducible new strategy with an adjustable PAB simplifies the management of infants with multiple VSDs and provides the following advantages: (a) good results (0% mortality), delayed surgery with a high incidence (15/17 = 88%) of spontaneous closure of multiple muscular VSDs, and facilitated closure of residual unrestrictive VSD (peri-membranous, mid-muscular, or inlet) at an older age and higher body weight; PAB with FloWatch-PAB(®) and its subsequent removal can potentially be the only procedure required for Swiss cheese multiple VSDs without an associated peri-membranous unrestrictive VSD.

摘要

简介

本研究为多中心前瞻性研究,旨在评估一种治疗多发室间隔缺损(VSD)的新策略。

材料和方法

2004 年至 2012 年,17 名连续患儿(3 例早产儿,14 例婴儿)接受了肺动脉带(PAB)和可调节 FloWatch-PAB(®)的治疗。患儿平均年龄 3.2 个月(9 天至 9 个月),平均体重 4.2kg(3.1-6.1kg),患有多发 VSD。合并心脏畸形包括动脉导管未闭(1 例)、主动脉缩窄(2 例)、主动脉弓发育不良(2 例)和左位心(3 例)。5 例(5/17=29.4%)患儿需要术前机械通气,平均持续时间为 64 天(7-240 天)。

结果

在平均 48 个月(7-98 个月)的随访中,无早期或晚期死亡,以 FloWatch 移除或最后观察为终点。所有患者均需调整 FloWatch-PAB(®):平均 4.8 次/患者(2-9 次)收紧 PAB,平均 1.1 次/患者(0-3 次)释放 PAB 以适应患者生长。平均 29 个月(8-69 个月)后,10/17(59%)名患者接受了再次手术:7/10 例 PAB 移除,其中 6 例(3 例膜周部、2 例肌部和 1 例流入部)关闭残余未限制的 VSD,1 例行 Damus-Kaye-Stansel、双向 Glenn 和房间隔切开术;3/9 例仅需移除 PAB。10 例患儿的所有肌部多发 VSD 均闭合。1/10 例患儿需行肺动脉重建。在仍有 7 例 PAB 的 5 例患儿中,所有肌部 VSD 均已闭合。仅 2 例持续存在肌部多发 VSD 的患儿随访时间最短。

结论

这种可调节肺动脉带的新策略可简化多发 VSD 患儿的治疗,具有以下优点:(a)良好的结果(0%死亡率);延迟手术,多发肌部 VSD 自发性闭合率高(17 例中有 15 例,88%),且更容易在年龄较大、体重较高时闭合残余未限制的 VSD(膜周部、肌部或流入部);带 FloWatch-PAB(®)的肺动脉带及其随后的移除可能是治疗瑞士奶酪型多发 VSD 而不伴膜周部未限制 VSD 的唯一方法。

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