Department of Congenital Heart Diseases, Institute of Cardiology, Warsaw, Poland.
Kardiol Pol. 2011;69(2):139-43.
Double-orifice mitral valve (DOMV) is a rare congenital malformation characterised by the presence of two orifices in the left atrio-ventricular valve area, each having an independent chordal attachment to the papillary muscle.
To establish the echocardiographic features and incidence of DOMV in patients undergoing an echo study in a tertiary referral centre.
We carried out a retrospective review of 215,193 echocardiographic studies in 79,919 patients performed between 1993 and 2006. Transthoracic echocardiographic examinations (TTE) of nine patients with DOMV (six female, mean age 37.9 years, range 8-59) were analysed.
In six patients, the complete bridge type of DOMV was recognised, two patients had a duplicate mitral valve, and one had the hole type. In all cases, mild to moderate mitral regurgitation was present. Moderate to severe mitral stenosis was diagnosed in five cases and was associated with the complete bridge type. In four patients, ostium primum atrial septal defect was present. The TTE was sufficient to define the type, anatomy and associated lesions in seven cases. In two patients, TEE was essential to establish the diagnosis.
Transthoracic echocardiographic examination is a reliable, and in most cases sufficient, means of diagnosing DOMV and determining its type. The DOMV as a cause of symptomatic mitral valve disease is seen in middle-aged/elderly patients. The incidence of double-orifice mitral valve in the adult tertiary referral echocardiographic laboratory is estimated at 0.01%.
双孔二尖瓣（DOMV）是一种罕见的先天性畸形，其特征为左房室瓣区域存在两个瓣口，每个瓣口均有独立的腱索附着于乳头肌。
确定在三级转诊中心进行超声心动图检查的患者中 DOMV 的超声心动图特征和发生率。
我们对 1993 年至 2006 年间在 79919 例患者中进行的 215193 次超声心动图研究进行了回顾性分析。分析了 9 例 DOMV 患者（6 例女性，平均年龄 37.9 岁，范围 8-59 岁）的经胸超声心动图检查（TTE）结果。
在 6 例患者中，识别出完全桥型 DOMV，2 例患者存在重复二尖瓣，1 例患者存在孔型。所有患者均存在轻至中度二尖瓣反流。5 例患者诊断为中度至重度二尖瓣狭窄，与完全桥型相关。4 例患者存在原发孔房间隔缺损。TTE 足以在 7 例患者中确定类型、解剖结构和相关病变。在 2 例患者中，TEE 对确定诊断至关重要。
经胸超声心动图检查是诊断 DOMV 及其类型的可靠且在大多数情况下足够的方法。DOMV 作为有症状二尖瓣疾病的病因，见于中老年患者。成人三级转诊超声心动图实验室中双孔二尖瓣的发生率估计为 0.01%。
