Mohan Jagdish C, Shukla Madhu, Mohan Vishwas, Sethi Arvind
Fortis Institute of Cardiac Sciences, Fortis Hospital, Shalimar Bagh, New Delhi 110088, India.
Fortis Institute of Cardiac Sciences, Fortis Hospital, Shalimar Bagh, New Delhi 110088, India.
Indian Heart J. 2016 Sep-Oct;68(5):639-645. doi: 10.1016/j.ihj.2015.12.014. Epub 2016 Jan 11.
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.
二尖瓣叶直接附着于乳头肌且伴有或不伴有短的发育不全腱索的先天性异常在成人中鲜有报道。在以往研究中，有中间纤维桥的双乳头肌患者也被纳入此类。
超声心动图能够准确评估瓣膜叶、腱索和乳头肌的形态及功能。本报告描述了6例年龄在56 - 84岁之间的患者，在3年期间发现他们患有二尖瓣异常，伴有一个大的孤立且异常附着的乳头肌。分析仅纳入那些有单个柱状或桥状乳头肌且腱索缺失或短小发育不全的患者。
在连续进行的9600次超声心动图检查中，6例患者符合二尖瓣异常伴孤立大乳头肌的标准。2例患者接受了二尖瓣置换术并部分切除乳头肌，术中超声心动图观察结果得到证实。这些患者之前分别被诊断为肥厚型心肌病（3例）和风湿性二尖瓣疾病（3例）。三维超声心动图图像的多平面重建在评估这些病例中乳头肌的详细病理解剖方面具有额外价值。
在成年患者中，对于可能伪装为肥厚型心肌病或风湿性二尖瓣疾病的伴有大的孤立乳头肌（类似降落伞样二尖瓣）的先天性二尖瓣狭窄/反流，需要高度怀疑指数才能检测出来。
