Criley J M, Siegel R J
Postgrad Med J. 1986 Jun;62(728):515-29. doi: 10.1136/pgmj.62.728.515.
There has been a longstanding controversy about the significance of intracavitary pressure gradients in hypertrophic cardiomyopathy (HCM). It has been generally assumed that the gradient is the result of an 'obstruction' that impedes left ventricular outflow and which can be relieved by operative intervention. In the first decade after the discovery of HCM (1957-66), the site of 'obstruction' was thought to be a muscular sphincter or contraction ring in the submitral region of the left ventricle, and operations designed to emulate pyloromyectomy (for hypertrophic pyloric stenosis) were developed. Following a challenge to the existence of the 'contraction ring' and an alternative non-obstructive explanation of the pressure gradient, the site of 'obstruction' was translocated to a point of apposition between the anterior mitral leaflet and the interventricular septum, a result of systolic anterior motion (SAM) of the mitral valve. Despite the translocation of the site and mechanism of 'obstruction', the operation for 'relief of obstruction' has not changed significantly. The newer site of 'obstruction' has been challenged on the grounds that the ventricle is not demonstrably impeded in its emptying; when a gradient is provoked, the ventricle empties more rapidly and more completely than it does without a gradient. In addition to a non-obstructive explanation of the gradient, other phenomena thought to be indicative of 'obstruction' can be explained by rapid and complete emptying of the ventricle (cavitary obliteration). Since the morbidity and mortality of symptomatic HCM patients without pressure gradients may exceed that of patients with pressure gradients, it is suggested that 'obstruction' may be unimportant in the pathophysiology of HCM and attention should be focused on abnormal diastolic function and life threatening arrhythmias.
关于肥厚型心肌病(HCM)心腔内压力梯度的意义,长期以来一直存在争议。人们普遍认为,这种压力梯度是由阻碍左心室流出的“梗阻”所致,可通过手术干预予以缓解。在发现HCM后的第一个十年(1957 - 1966年),“梗阻”部位被认为是左心室二尖瓣下区域的肌肉括约肌或收缩环,并开发了旨在模仿幽门肌切开术(用于肥厚性幽门狭窄)的手术。在对“收缩环”的存在提出质疑以及对压力梯度提出另一种非梗阻性解释之后,“梗阻”部位转移到二尖瓣前叶与室间隔的贴合点,这是二尖瓣收缩期前向运动(SAM)的结果。尽管“梗阻”部位和机制发生了转移,但“解除梗阻”的手术并未有显著改变。新的“梗阻”部位受到了挑战,理由是心室排空并未明显受阻;当诱发压力梯度时,心室比无压力梯度时排空得更快、更完全。除了对压力梯度的非梗阻性解释外,其他被认为是“梗阻”指征的现象也可以用心室的快速完全排空(心腔闭塞)来解释。由于无症状性HCM患者的发病率和死亡率可能超过有压力梯度患者,因此有人提出“梗阻”在HCM的病理生理学中可能并不重要,应将注意力集中在舒张功能异常和危及生命的心律失常上。