Independent factors associated with outcomes of parachute mitral valve in 84 patients
Independent factors associated with outcomes of parachute mitral valve in 84 patients
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DOI：
10.1161/01.cir.0000129269.98460.c8
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发表时间：
2004-05-18
期刊：
影响因子：
37.8
通讯作者：
McCrindle, BW
中科院分区：
文献类型：
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作者：
Schaverien, MV;Freedom, RM;McCrindle, BW
背景-降落伞二尖瓣(PMV)被定义为二尖瓣索与单个或优势乳头肌的单灶性连接，可能导致瓣膜下阻塞。我们试图确定与结果相关的因素。方法和结果-1977年至2001年间，84例患者(男，，男)，平均年龄3天(出生至5.4岁)，行PMV检查(无房室间隔缺陷)。合并心脏畸形99%包括主动脉缩窄68%，房间隔缺损54%，室间隔缺损46%，主动脉瓣狭窄32%，主动脉瓣下狭窄20%，左心室发育不良19%，复杂畸形14%。非心脏异常的发生率为32%。1年存活率为82%，10年存活率为79%，独立危险因素包括左心室发育不良(P<；0.001)和房间隔缺损(P<；0.003)。在6个月和10岁时，11名患者的二尖瓣手术成功率分别为95%和80%，独立的危险因素包括无主动脉缩窄(P<；0.02)和存在主动脉下狭窄(P<；0.04)。随着时间的推移，PMV的平均梯度没有显著增加，但较高的梯度与二尖瓣上狭窄(P<；0.001)、无房间隔缺损(P<；0.04)、室间隔缺损(P<；0.02)以及随后的二尖瓣关闭不全(P<；0.01)独立相关。二尖瓣阻塞的程度保持稳定，大多数不需要瓣膜切开术。
Background - Parachute mitral valve (PMV) is defined as a unifocal attachment of the mitral valve chordae to a single or dominant papillary muscle and may cause subvalvar obstruction. We sought to determine factors associated with outcomes.Methods and Results - Patients (n = 84; 64% male) who presented between 1977 and 2001 at a median age of 3 days ( range, birth to 5.4 years) were assessed with PMV ( without atrioventricular septal defect). Associated cardiac anomalies in 99% included aortic coarctation in 68%, atrial septal defect in 54%, ventricular septal defect in 46%, aortic valve stenosis in 32%, subaortic stenosis in 20%, and left ventricular hypoplasia in 19%, with complex anomalies in 14%. Noncardiac anomalies were noted in 32%. Survival (n = 18 deaths) was 82% at 1 year and 79% at 10 years, with independent risk factors including left ventricular hypoplasia (P < 0.001) and atrial septal defect (P < 0.003). Freedom from surgical mitral valvotomy (n = 11 patients) was 95% at age 6 months and 80% at 10 years, with independent risk factors including the absence of aortic coarctation (P < 0.02) and the presence of subaortic stenosis (P < 0.04). There was no significant increase in mean gradient of the PMV over time, but higher gradient was independently associated with the presence of supravalvar mitral stenosis (P < 0.001), absence of atrial septal defect (P < 0.04), presence of ventricular septal defect (P < 0.02), and subsequent mitral valvotomy (P < 0.01).Conclusions - Outcomes for patients with PMV are dependent on the spectrum of associated cardiac lesions. The degree of mitral valve obstruction remains stable, and the majority will not require valvotomy.