Clinic for Cardiology, University Hospital, 8091 Zurich, Switzerland.
Clin Cardiol. 2009 Oct;32(10):553-60. doi: 10.1002/clc.20608.
Isolated congenital cleft of the posterior leaflet of the mitral valve is a rare cause of mitral regurgitation (MR). This study describes the clinical, echocardiographic, and intraoperative findings as well as treatment options.
Adults with an isolated cleft of the posterior mitral valve leaflet diagnosed by transthoracic echocardiography were evaluated with respect to clinical, echocardiographic, preoperative and intraoperative findings, and different surgical strategies.
The prevalence of isolated cleft of the posterior mitral valve leaflet in all patients examined was 0.11% (n = 22 out of 19 320 evaluated echocardiograms); male gender was predominant (73%). Dyspnea on exertion was present in almost all patients with at least moderate regurgitation. The predominant localization of the cleft was within segment P2 (59%), followed by a cleft between P1/P2 (18%). An isolated cleft in segment P3 or segment P1 occurred twice in each segment (n = 2; 9%) and between P2/P3 once (n = 1; 5%). Regurgitation was severe in 50% (n = 11), moderate in 9% (n = 2), mild in 27% (n = 6), and only trivial in 14% (n = 3) of the patients. Surgical treatment involved reconstruction with ring annuloplasty in 45% (n = 10) and replacement in 4.5% (n = 1). A total of 11 patients (50%) with mostly mild or trivial mitral regurgitation were treated medically only.
Two-dimensional high-resolution cross-sectional echocardiography allows the distinct diagnosis of a clefted posterior leaflet, whereas clinical presentation, electrocardiogram, chest x-ray, and angiography are failing to identify the correct etiology of MR in patients with isolated posterior leaflet cleft mitral valve (IPLCMV). Patients with moderate to severe MR were treated surgically with excellent outcome.
孤立性先天性二尖瓣后叶裂是二尖瓣反流（MR）的罕见病因。本研究描述了其临床、超声心动图和术中表现以及治疗选择。
经胸超声心动图诊断为孤立性二尖瓣后叶裂的成人，评估其临床、超声心动图、术前和术中发现以及不同的手术策略。
在所有接受检查的患者中，孤立性二尖瓣后叶裂的患病率为 0.11%（22/19320 例经超声心动图检查）；男性占优势（73%）。几乎所有至少存在中度反流的患者均有劳力性呼吸困难。裂的主要定位在 P2 节段（59%），其次是 P1/P2 节段之间（18%）。P3 或 P1 节段的孤立性裂各发生 2 次（n=2；9%），P2/P3 节段之间发生 1 次（n=1；5%）。反流严重程度为 50%（n=11）、中度 9%（n=2）、轻度 27%（n=6）和仅有微量 14%（n=3）。手术治疗包括环瓣成形术重建 45%（n=10）和置换术 4.5%（n=1）。11 名（50%）患者主要存在轻度或微量 MR，仅接受药物治疗。
二维高分辨率切面超声心动图可明确诊断后叶裂，而临床表现、心电图、胸部 X 线和血管造影术无法确定孤立性后叶裂二尖瓣（IPLCMV）患者 MR 的正确病因。中重度 MR 患者采用手术治疗，效果极佳。
