Nagata S, Park Y D, Ohmori F, Beppu S, Kawazoe K, Fujita T, Sakakibara H, Nimura Y
Department of Medicine, National Cardiovascular Center, Suita.
J Cardiol. 1988 Jun;18(2):363-72.
The left ventricular outflow pressure gradient in hypertrophic cardiomyopathy results from systolic anterior motion of the mitral valve (SAM). This abnormal orientation of the valve was previously proposed to be caused by inappropriately hypertrophied papillary muscles which protrude to the interventricular septum (IVS). Septal myotomy can alter the orientation of the papillary muscles and resolve the pressure gradient, without myectomy. Recently, we have experienced two instructive cases to prove our previously advocated hypothesis. Case 1: This 54-year-old man complained of effort dyspnea, and his echocardiogram disclosed marked SAM, and a thickened IVS (28 mm) and left ventricular posterior wall (16 mm). The intraventricular pressure gradient was 134 mmHg, and there was mitral regurgitation of grade 2/4. A longitudinal incision via the aorta on the anterior portion of the IVS, toward the base of the anterolateral papillary muscle, resolved the pressure gradient and mitral regurgitation, and two-dimensional echocardiography demonstrated that the SAM resolved at the lateral aspect of the valve, but it remained on the medial side. Case 2: This 57-year-old man complained of dyspnea during effort. He had marked SAM. The intraventricular pressure gradient was 65 mmHg, and there was grade 3/4 mitral regurgitation. Longitudinal incisions on the anterior, medial and posterior parts of the IVS abolished the SAM and reduced mitral regurgitation to grade 1/4. In both cases, during systole, the anterolateral papillary muscle protruded into the left ventricular ontflow tract, causing SAM. After surgery, the direction of the muscle axis moved toward the mitral orifice during systole, resulting in alleviation of SAM on the same side of the location of septotomy. This further confirmed our concept that disoriented papillary muscles play essential roles in causing SAM. If the Venturi forces previously stressed by other investigators cause SAM, the latter should resolve on both the medial and lateral aspects, even by septotomy. Thus, the Venturi theory seems untenable.
肥厚型心肌病的左心室流出道压力梯度源于二尖瓣收缩期前向运动(SAM)。此前认为这种瓣膜的异常取向是由过度肥厚的乳头肌向室间隔(IVS)突出所致。室间隔肌切开术可改变乳头肌的取向并消除压力梯度,而无需心肌切除术。最近,我们遇到了两个有启发性的病例来证明我们之前主张的假设。病例1:这名54岁男性主诉劳力性呼吸困难,其超声心动图显示明显的SAM,室间隔增厚(28mm)和左心室后壁增厚(16mm)。心室内压力梯度为134mmHg,存在2/4级二尖瓣反流。经主动脉在室间隔前部朝向外侧乳头肌基部做纵向切口,消除了压力梯度和二尖瓣反流,二维超声心动图显示SAM在瓣膜外侧消失,但在内侧仍存在。病例2:这名57岁男性主诉劳力时呼吸困难。他有明显的SAM。心室内压力梯度为65mmHg,存在3/4级二尖瓣反流。在室间隔的前、中、后部做纵向切口消除了SAM,并将二尖瓣反流减少至1/4级。在这两个病例中,收缩期外侧乳头肌均突入左心室流出道,导致SAM。手术后,收缩期肌轴方向朝二尖瓣口移动,导致在肌切开术部位同侧的SAM减轻。这进一步证实了我们的观点,即乳头肌方向异常在导致SAM中起重要作用。如果其他研究者之前强调的文丘里力导致SAM,那么即使通过肌切开术,SAM也应在内侧和外侧均消失。因此,文丘里理论似乎站不住脚。