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继发孔型房间隔缺损合并二尖瓣裂缺

[Ostium secundum type of interatrial septal defect associated with cleft mitral valve].

作者信息

Pugliese P, Murzi B, Tommasini G, Eufrate S

出版信息

G Ital Cardiol. 1982;12(4):264-9.

PMID:7152176
Abstract

Eight patients (7 females and one male) ranging in age from 6 to 30 years (mean 11.2 years), with secundum-type atrial septal defect (o.s. ASD) and cleft mitral valve (CVM) were surgically corrected at the Cardiac Surgery Department of Massa Hospital (Massa-Italy) from 1974 to 1981. All patients were studied with non invasive diagnostic techniques and with cardiac catheterization and angiography. All had mitral regurgitation of variable degree. On the ECG, there were P and QRS wave abnormalities but no superior quadrant QRS axis deviation suggestive of endocardial cushion defect. At operation the cleft(s) was found in variable positions: on the anterior leaflet in 4 cases and on the posterior leaflet in four. In two cases there was a double cleft. Correction was accomplished in 5 cases with direct repair of CVM, associated with various types of anuloplasty and in 2 cases anuloplasty only was done, without suturing the cleft. The septal defect was closed by direct suture in one case and with a Dacron patch in the remaining seven. There was one early death in the first 24 hour post-operatively. The seven surviving patients have been followed for a period of time ranging between 2 and 93 months (mean 29 months). No survivor has been re-evaluated by cardiac catheterization and cineangiography. No patient shows clinical or non-invasive evidence of residual mitral regurgitation behind grade I (slight). We conclude that: 1) the CVM associated with o.s. ASD cannot be securely diagnosed preoperatively with the common diagnostic techniques, even when producing severe mitral regurgitation, except perhaps by B-mode echocardiography; 2) the CVM (part of the spectrum of endocardial cushion defect malformations) in no cases was associated with the typical ECG or angiocardiographic abnormalities; 3) the CVM associated with o.s. ASD should always be repaired because, compared to the CVM that occurs with endocardial cushion defects, its site and regurgitation characteristics are variable, and simple ASD closure may not be sufficient.

摘要

1974年至1981年期间,8例年龄在6至30岁(平均11.2岁)的患者(7名女性和1名男性),患有继发孔型房间隔缺损(o.s. ASD)和二尖瓣裂(CVM),在意大利马萨市马萨医院心脏外科接受了手术矫正。所有患者均采用非侵入性诊断技术以及心脏导管检查和血管造影进行研究。所有患者均有不同程度的二尖瓣反流。心电图显示有P波和QRS波异常,但无提示心内膜垫缺损的上象限QRS轴偏移。手术时发现瓣裂位置各异:4例位于前叶,4例位于后叶。2例有双瓣裂。5例通过直接修复CVM并联合各种类型的瓣环成形术完成矫正,2例仅进行了瓣环成形术,未缝合瓣裂。1例房间隔缺损通过直接缝合关闭,其余7例使用涤纶补片关闭。术后24小时内有1例早期死亡。7名存活患者的随访时间为2至93个月(平均29个月)。没有存活患者通过心脏导管检查和电影血管造影进行重新评估。没有患者显示出I级(轻度)以上残余二尖瓣反流的临床或非侵入性证据。我们得出以下结论:1)即使产生严重二尖瓣反流,除了可能通过B型超声心动图外,采用常见诊断技术术前无法可靠诊断与o.s. ASD相关的CVM;2)CVM(心内膜垫缺损畸形谱的一部分)在任何情况下均未与典型的心电图或心血管造影异常相关;3)与o.s. ASD相关的CVM应始终进行修复,因为与心内膜垫缺损时出现的CVM相比

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