Spectrum of congenital mitral valve abnormalities associated with solitary undifferentiated papillary muscle in adults.
Spectrum of congenital mitral valve abnormalities associated with solitary undifferentiated papillary muscle in adults.
复制标题
与成年人中孤立的未分化乳头肌相关的先天二尖瓣异常的光谱。
DOI：
10.1016/j.ihj.2015.12.014
复制
发表时间：
2016-09
影响因子：
1.5
通讯作者：
Sethi A
中科院分区：
文献类型：
--
作者：
Mohan JC;Shukla M;Mohan V;Sethi A
先天性异常，其中二尖瓣瓣叶直接附着到乳头肌（PM），伴或不伴短的发育不全的腱索，在成人中很少报告。在之前的研究中，该股骨头下也包括了两个PM伴中间纤维桥的患者。超声心动图能够准确评价瓣叶、腱索和PM的形态和功能。本报告描述了一个系列的6例患者，年龄56-84岁，二尖瓣异常，3年内观察到一个大的孤立性和错误插入的PM。只有那些有一个单一的支柱或桥梁样PM，要么缺席腱索或小欠发达的和弦的患者被纳入分析。在进行的9600次连续超声心动图中，6例患者符合二尖瓣异常伴孤立性大PM的标准。2例患者接受二尖瓣置换术，部分切除PM，其中超声心动图观察结果得到证实。这些患者之前被诊断为肥厚型心肌病（3）和风湿性二尖瓣疾病（3）。三维超声心动图图像的多平面重建在评估这些病例中PM的详细病理解剖结构方面提供了增量价值。在成人患者中，需要高度怀疑才能检测到先天性二尖瓣狭窄/返流伴大孤立PM（类似降落伞二尖瓣），可能伪装成肥厚性心肌病或风湿性二尖瓣疾病。
Congenital anomaly wherein the mitral valve leaflets are directly attached to the papillary muscle(s) (PM) with or without short under-developed chords is rarely reported in adults. Patients with two PMs with an intervening fibrous bridge have also been included under this head in previous studies. Echocardiography enables accurate evaluation of the morphology and function of valve leaflets, chordae tendineae, and PM. This report describes a series of six patients aged 56–84 years who had abnormal mitral valve with a large solitary and anomalously inserted PM seen over a period of 3 years. Only those patients who had a single pillar or bridge-like PM and either absent tendinous chords or small under-developed chords were included in the analysis. Among 9600 consecutive echocardiograms performed, six patients met the criteria of an abnormal mitral valve with solitary large PM. Two patients underwent mitral valve replacement with partial excision of the PM wherein echocardiographic observations were confirmed. The patients were previously followed with the diagnosis of hypertrophic cardiomyopathy (3) and rheumatic mitral valve disease (3). Multi-planar reconstruction of 3D echocardiographic images provided incremental value in assessing the detailed patho-anatomy of PMs in these cases. In adult patients, a high index of suspicion is required to detect congenital mitral stenosis/regurgitation with large solitary PM (resembling a parachute mitral valve) which may masquerade as hypertrophic cardiomyopathy or rheumatic mitral valve disease.