二孔型二尖瓣(DOMV)是一种少见的先天性心瓣膜病.这种先天畸形多伴有二尖瓣反流,伴二尖瓣狭窄的较为罕见.而对以狭窄为主要表现的先天性二孔型二尖瓣患者作二尖瓣球囊成形术(PBMV),尚未见报道.本文报告一例以狭窄为主要表现的DOMV患者行二尖瓣球囊成形术.并结合文献对二孔型二尖瓣的发生、临床表现、分型及处理作一复习,以及对二尖瓣球囊成形术在这种先天畸形中的适应症及方法作了探讨. Two-hole mitral valve (DOMV) is a rare congenital heart valve disease, which is often accompanied by congenital malformations with mitral regurgitation, mitral stenosis is relatively rare, while the stenosis as the main manifestation Congenital mitral valve mitral valvuloplasty (PBMV) has not been reported yet.This paper reports a case of mitral balloon angioplasty in patients with DOMV with stenosis as the main manifestation, Hollow mitral valve, clinical manifestations, classification and treatment for a review, and the mitral valve balloon angioplasty in this congenital malformations and methods were discussed.