Collins-Nakai R L, Rosenthal A, Castaneda A R, Bernhard W F, Nadas A S
Circulation. 1977 Dec;56(6):1039-47. doi: 10.1161/01.cir.56.6.1039.
The clinical course of 38 patients with congenital mitral stenosis (MS) is reviewed. Associated cardiac defects were present in 28 patients, including tetralogy of Fallot in five. In all but one of the eight patients with supravalvar mitral ring (SVR), there were concomitant abnormalities of the mitral valve. Delay in the diagnosis of MS was common. Serial cardiac catheterizations and pulmonary pathologic examination indicated that pulmonary vascular obstructive disease develops during childhood. Mitral valve surgery was performed in 19 of 38 patients: valvotomy alone in eight, excision of SVR in five (two ofwhom also had valvotomy) and mitral valve replacemtnt in seven. Additional non-mitral cardiac surgery was performed in 18 patients. Overall surgical mortality was 49%; mortality for surgery on the mitral valve was 26%. Only patients having mitral valve replacement or with isolated SVR which was then resected became asymptomatic and had normal hemodynamics on postoperative catheterization.
回顾了38例先天性二尖瓣狭窄（MS）患者的临床病程。28例患者存在相关心脏缺陷，其中5例为法洛四联症。在8例患有瓣上二尖瓣环（SVR）的患者中，除1例之外，其余患者均伴有二尖瓣异常。MS诊断延迟很常见。系列心脏导管检查和肺部病理检查表明，儿童期会发生肺血管阻塞性疾病。38例患者中有19例接受了二尖瓣手术：单纯瓣膜切开术8例，SVR切除术5例（其中2例还接受了瓣膜切开术），二尖瓣置换术7例。18例患者还接受了非二尖瓣心脏手术。总体手术死亡率为49%；二尖瓣手术死亡率为26%。只有接受二尖瓣置换术的患者或切除孤立性SVR的患者术后无症状，术后导管检查显示血流动力学正常。
