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强直性肌营养不良中的二尖瓣脱垂。

Mitral valve prolapse in myotonic dystrophy.

作者信息

Streib E W, Meyers D G, Sun S F

出版信息

Muscle Nerve. 1985 Oct;8(8):650-3. doi: 10.1002/mus.880080804.

DOI:10.1002/mus.880080804
PMID:4058472
Abstract

Forty-three patients with myotonic dystrophy (MyD) and 43 age- and sex-matched controls were prospectively evaluated for mitral valve prolapse (MVP) using both cardiac auscultation and echocardiography (ECHO). Prevalence of MVP was significantly increased in MyD patients when using either abnormal auscultation plus abnormal ECHO (23.3%, P less than 0.02) or abnormal ECHO alone (30%, P less than 0.01) as diagnostic criteria for MVP. Frequency of MVP was even more increased in patients with a thin, flat chest compared with patients with normal physical appearance (P less than 0.007). These findings are in support of the concept that the increased frequency of MVP in neuromuscular disorders is likely due to geometrical changes of the heart caused by thorax deformities, rather than structural changes of the mitral valve. For this reason, MVP in MyD probably belongs to the entity of so-called benign, secondary MVP. This is further supported by the absence of symptoms of "systemic" complications of MVP in our patient population.

摘要

对43例强直性肌营养不良(MyD)患者以及43例年龄和性别匹配的对照者,采用心脏听诊和超声心动图(ECHO)对二尖瓣脱垂(MVP)进行前瞻性评估。当使用异常听诊加异常ECHO(23.3%,P<0.02)或单独使用异常ECHO(30%,P<0.01)作为MVP的诊断标准时,MyD患者中MVP的患病率显著增加。与体型正常的患者相比,胸部瘦平的患者中MVP的发生率更高(P<0.007)。这些发现支持了这样一种观点,即神经肌肉疾病中MVP发生率增加可能是由于胸廓畸形导致心脏几何形状改变,而非二尖瓣结构变化。因此,MyD中的MVP可能属于所谓的良性继发性MVP。我们的患者群体中没有MVP“全身性”并发症的症状,这进一步支持了这一观点。

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Mitral valve prolapse in myotonic dystrophy.强直性肌营养不良中的二尖瓣脱垂。
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引用本文的文献

1
Left ventricular diastolic function in congenital myotonic dystrophy.先天性强直性肌营养不良患者的左心室舒张功能
Arch Dis Child. 1999 Mar;80(3):267-70. doi: 10.1136/adc.80.3.267.
2
Electrocardiographic findings in myotonic dystrophy.强直性肌营养不良的心电图表现
Br Heart J. 1988 Jan;59(1):47-52. doi: 10.1136/hrt.59.1.47.
3
Cardiac involvement in congenital myotonic dystrophy.先天性肌强直性营养不良的心脏受累情况。
Br Heart J. 1990 Feb;63(2):119-21. doi: 10.1136/hrt.63.2.119.
4
Intestinal pseudo-obstruction in myotonic dystrophy.强直性肌营养不良中的肠道假性梗阻。
J Med Genet. 1992 Nov;29(11):791-3. doi: 10.1136/jmg.29.11.791.