Pillai Vivek Velayudhan, Karunakaran Jayakumar
Sree Chitra Tirunal Institute for Medical Sciences and Technology, Department of Cardiovascular and Thoracic Surgery, Trivandrum, Kerala, India.
Braz J Cardiovasc Surg. 2017 Jul-Aug;32(4):338-340. doi: 10.21470/1678-9741-2016-0034.
Double orifice left atrioventricular valve (DOLAVV) or double orifice mitral valve (DOMV) is a rare congenital cardiac anomaly manifesting either as an isolated lesion (mitral stenosis or mitral insufficiency) or in association with other congenital cardiac defects. Signs of mitral valve disease are usually present along with the symptoms of associated coexistent congenital heart diseases. Mitral insufficiency due to annular dilatation is seen when DOLAVV is associated with endocardial cushion defects. Surgical intervention like mitral valve repair or replacement is required in 50% of patients and yields good results. We report a case of a 56-year-old lady who successfully underwent surgical correction of DOLAVV with partial atrioventricular canal defect.
双孔型左房室瓣(DOLAVV)或双孔型二尖瓣(DOMV)是一种罕见的先天性心脏异常,表现为孤立性病变(二尖瓣狭窄或二尖瓣关闭不全)或与其他先天性心脏缺陷并存。二尖瓣疾病的体征通常与相关先天性心脏病的症状同时出现。当DOLAVV与心内膜垫缺损相关时,可出现因瓣环扩张导致的二尖瓣关闭不全。50%的患者需要进行二尖瓣修复或置换等手术干预,且效果良好。我们报告一例56岁女性患者,成功接受了伴有部分房室通道缺损的DOLAVV手术矫正。