Sasaki Takashi, Asou Toshihide, Shirakawa Makoto, Takahashi Ken-Ichiro, Kunugi Shinobu, Nitta Takashi
Department of Cardiovascular Surgery, Nippon Medical School Hospital, 1-1-5, Sendagi, Bunkyo-ku, Tokyo, 113-8603, Japan.
Department of Cardiovascular Surgery, Kanagawa Children's Hospital, Yokohama, Japan.
Gen Thorac Cardiovasc Surg. 2020 Apr;68(4):380-384. doi: 10.1007/s11748-019-01084-9. Epub 2019 Feb 19.
A 4-year-old boy with Noonan syndrome accompanied by hypertrophic obstructive cardiomyopathy presented with refractory heart failure owing to severe left ventricular outflow obstruction and mitral regurgitation. He underwent mitral valve replacement and trans-mitral myectomy. The pathology of the resected myocardium was consistent with hypertrophic cardiomyopathy, and the mitral valve leaflets were severely degenerated. Compared with a control patient who is a non-syndromic 4-year-old boy with hypertrophic obstructive cardiomyopathy, mitral pathology was much more severe in Noonan syndrome. Regarding trans-mitral myectomy, residual pressure gradient at the mid-ventricular level made us think about more aggressive muscle resection with various approaches.
一名患有努南综合征并伴有肥厚性梗阻性心肌病的4岁男孩，因严重的左心室流出道梗阻和二尖瓣反流出现难治性心力衰竭。他接受了二尖瓣置换术和经二尖瓣心肌切除术。切除心肌的病理与肥厚性心肌病一致，二尖瓣叶严重退变。与一名患有肥厚性梗阻性心肌病的非综合征4岁男孩对照患者相比，努南综合征患者的二尖瓣病变要严重得多。关于经二尖瓣心肌切除术，心室中部水平的残余压力梯度让我们考虑采用各种方法进行更积极的肌肉切除。
