From the Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, Rochester, MN.
Circ Cardiovasc Imaging. 2015 May;8(5). doi: 10.1161/CIRCIMAGING.114.002989.
Fibro-elastic deficiency (FED) and diffuse myxomatous degeneration (DMD) are phenotypes of degenerative mitral valve disease defined morphologically. Whether physiological differences in annular and valvular dynamics exist between these phenotypes remains unknown.
We performed triple quantitation of cardiac remodeling and of mitral regurgitation severity and of annular and valvular dimensions by real-time 3-dimensional-transesophageal-echocardiography. Forty-nine patients with degenerative mitral valve disease classified as FED (n=31) and DMD (n=18) by surgical observation showed no difference in age (65±10 versus 59±13; P=0.5), body surface area (2.0±0.2 versus 2.0±0.2 m(2); P=0.5), left ventricular and atrial dimensions (all P>0.55), and mitral regurgitation regurgitant orifice (P=0.62). On average, annular dimensions were larger in DMD versus FED, but height was similar resulting in lower saddle shape. Dynamically, annular DMD versus FED display poorer contraction and saddle-shape accentuation in early systole and abnormal enlargement, particularly intercommissural, in late-systole (all P<0.05). Valvular dynamics showed stable valvular area in systole in FED versus considerable systolic increased area in DMD (P<0.001). Prolapse height and volume increased little throughout systole in FED versus marked increase in DMD (P<0.001).
Our novel observations show that FED and DMD, although both labeled myxomatous, display considerable physiological phenotypic differences. In DMD, the annular increased size and profoundly abnormal dynamics demonstrate DMD-specific annular degeneration compared with the enlarged but relatively normal FED annulus. DMD does not incur more severe mitral regurgitation, despite larger prolapse and valve redundancy, underscoring potential compensatory role of tissue redundancy of DMD (or aggravating role of tissue paucity of FED) on mitral regurgitation severity.
纤维弹性缺失(FED)和弥漫性黏液样变性(DMD)是通过形态学定义的退行性二尖瓣疾病的表型。这两种表型之间是否存在环形和瓣叶动力学的生理差异尚不清楚。
我们通过实时三维经食管超声心动图对心脏重构和二尖瓣反流严重程度以及环形和瓣叶尺寸进行三重定量。49 例退行性二尖瓣疾病患者根据手术观察分为 FED(n=31)和 DMD(n=18)。FED 和 DMD 患者的年龄(65±10 岁 vs. 59±13 岁;P=0.5)、体表面积(2.0±0.2 m2 vs. 2.0±0.2 m2;P=0.5)、左心室和心房大小(均 P>0.55)和二尖瓣反流瓣口(P=0.62)无差异。平均而言,DMD 的环形尺寸大于 FED,但高度相似,导致马鞍形状较低。在动力学方面,DMD 与 FED 相比,环形收缩和早期收缩马鞍形状加重较差,晚期收缩(均 P<0.05)时跨隔交界异常增大。瓣叶动力学显示 FED 在收缩期瓣叶面积稳定,而 DMD 在收缩期瓣叶面积显著增加(P<0.001)。FED 在收缩期瓣叶突度和体积增加较小,而 DMD 则明显增加(P<0.001)。
我们的新观察结果表明,尽管都被标记为黏液样,但 FED 和 DMD 表现出相当大的生理表型差异。在 DMD 中,环形增大和明显异常的动力学显示与扩大但相对正常的 FED 环相比,DMD 具有特定的环形退化。尽管 DMD 瓣叶脱垂和冗余较大,但并未导致更严重的二尖瓣反流,这强调了 DMD 组织冗余(或 FED 组织不足的加重作用)对二尖瓣反流严重程度的潜在代偿作用。