Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands.
Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, Netherlands.
Interact Cardiovasc Thorac Surg. 2021 Apr 19;32(4):506-514. doi: 10.1093/icvts/ivaa304.
Barlow's disease (BD) is characterized by thick, redundant mitral valve (MV) leaflets, which can lead to prolapse and significant mitral regurgitation (MR). MV annular abnormalities are also commonly observed and increasingly recognized as possible primary pathology, with leaflet thickening being secondary to increased stress on the MV apparatus. To provide more insights into this hypothesis, the evolution of MV abnormalities over time in patients with BD was assessed.
A total of 64 patients (54 ± 12 years, 72% male) with BD who underwent MV surgery and had multiple transthoracic echocardiograms (TTE) before surgery were included. In total, 186 TTE were analysed (median time interval 4.2, interquartile range 2.2-6.5 years) including specific MV characteristics.
At baseline, MV leaflet length, thickness, billowing height and annular diameter were larger in patients with BD compared to 59 healthy subjects. Systolic outward motion (curling) of the annulus was observed in 77% and severe mitral annular disjunction (≥5 mm) in 38% of patients with BD. Forty (63%) patients had MR grade I-II and 24 (37%) MR grade III-IV; at baseline, the 2 groups only differed in left atrial volume and in thickness and billowing height of the posterior leaflet, showing comparable MV annular abnormalities and dilatation despite different grades of MR. Over time, MV annulus diameter, leaflet length and billowing height increased significantly along with MR grade.
In patients with BD, MV annulus abnormalities are present at an early stage and precede the development of significant MR, suggesting their substantial role in the pathophysiology of this disease and as an important target for surgical treatment.
巴氏病（Barlow's disease，BD）的特征为二尖瓣（MV）瓣叶增厚、冗余，可导致脱垂和明显的二尖瓣反流（MR）。MV 环异常也很常见，且逐渐被认为可能是原发性病变，瓣叶增厚是 MV 装置承受的压力增加所致。为了更深入地了解这一假说，评估了 BD 患者 MV 异常随时间的演变。
共纳入 64 例（54±12 岁，72%为男性）BD 患者，这些患者接受 MV 手术，并在术前接受了多次经胸超声心动图（TTE）检查。总共分析了 186 次 TTE（中位数时间间隔为 4.2 年，四分位距为 2.2-6.5 年），包括 MV 的具体特征。
在基线时，与 59 名健康对照者相比，BD 患者的 MV 瓣叶长度、厚度、膨出高度和环直径均较大。77%的患者出现 MV 环向外运动（卷曲），38%的患者出现严重的 MV 环分离（≥5 mm）。40 例（63%）患者存在 MR Ⅰ-Ⅱ级，24 例（37%）存在 MR Ⅲ-Ⅳ级；在基线时，两组仅在左心房容积以及后瓣叶的厚度和膨出高度上存在差异，尽管存在不同程度的 MR，但两组 MV 环异常和扩张程度相似。随着时间的推移，MV 环直径、瓣叶长度和膨出高度显著增加，MR 分级也随之增加。
在 BD 患者中，MV 环异常在早期就存在，并先于明显 MR 的发生，这表明其在该疾病的病理生理学中具有重要作用，是手术治疗的重要靶点。
