二尖瓣环钙化

一种慢性非炎症性退行性病变,其特征是二尖瓣纤维环(主要在后瓣环)发生钙质和脂质沉积。

定义

一种慢性非炎症性退行性病变,其特征是二尖瓣纤维环(主要在后瓣环)发生钙质和脂质沉积,可累及二尖瓣叶基底部,限制其舒张期活动度,并在无交界处融合的情况下引起跨二尖瓣压差升高和/或二尖瓣关闭不全 [V1] [G1-09]。

严重程度标准

  • 单一级别(退行性 / 典型,Ú):
    • 钙化后环厚度 > 4–5 mm,超声心动图可见明显强回声伴后方声影 [G1-09]。
    • 跨二尖瓣平均压差:通常为 2–5 mmHg(在管腔受侵蚀的严重表现中可达 6–8 mmHg)[G1-09] [V1]。
    • 合并二尖瓣关闭不全:通常为轻度(EROA < 0.20 cm²,RV < 30 mL)或中度(EROA 0.20–0.39 cm²)[V1] [V3]。
    • 无二尖瓣交界处融合(与风湿性病因鉴别的关键要点)[V1] [G1-09]。

分层表现

血流动力学

  • 跨二尖瓣平均压差:轻至中度升高(2–5 mmHg),取决于心率和瓣环突入程度 [G1-09]。
  • 左心房压力:静息时正常或轻度升高,心动过速或用力时不成比例地升高 [G1-09] [V1]。

心律

  • 老年人中因左心房扩张及高龄常见正常窦性心律或心房颤动 [V1] [G1-09]。
  • 因钙化扩散至纤维三角传导系统引起房室或室内传导阻滞的风险 [E2] [G1-09]。

ECG

  • 左心房增大、心房颤动或房室 / 室内传导障碍 [E1] [E2]。

心音与杂音

  • 第一心音(S1):正常或轻度低钝(不像风湿性狭窄那样亢进)[G1-10]。
  • 开瓣音(OS):缺如(无交界处融合且瓣叶游离缘活动度保留,阻止了开瓣音的产生)[G1-09] [G1-15]。
  • 舒张期隆隆样杂音:若平均压差升高,心尖部杂音微弱且柔和 [G1-09] [G1-15]。
  • 收缩期杂音:中度二尖瓣关闭不全的心尖部全收缩期杂音,或继发于后瓣环僵硬对合不良所致的反流 [G1-14] [V3]。

动脉脉搏

  • 通常正常,或伴有高龄典型的体循环动脉僵硬体征 [B4]。

颈静脉搏动

  • 正常,除非合并充血性心力衰竭 [B3]。

负荷试验

  • 等长握力试验:增加二尖瓣关闭不全杂音强度 [B1]。
  • Valsalva:减弱收缩期杂音强度 [B1]。

模拟器严重程度分级

模拟器严重程度分级: 单一级别
基线心律: 正常窦性心律

亦称为: 二尖瓣环钙化 · 二尖瓣瓣环钙化 · 二尖瓣环钙化症 · MAC

该组中的其他病变

文献来源

  1. [B1] Bedside diagnosis of systolic murmurs . N Engl J Med (1988) . DOI: 10.1056/NEJM198806163182404 PubMed: 2897627
  2. [B3] The jugular venous pressure revisited . Cleve Clin J Med (2013) . DOI: 10.3949/ccjm.80a.13039 PubMed: 24085809
  3. [B4] The Carotid Pulse . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250154
  4. [E1] AHA/ACCF/HRS recommendations for the standardization and interpretation of the electrocardiogram: part V: electrocardiogram changes associated with cardiac chamber hypertrophy: a scientific statement from the American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; the American College of Cardiology Foundation; and the Heart Rhythm Society: endorsed by the International Society for Computerized Electrocardiology . Circulation (2009) . DOI: 10.1161/CIRCULATIONAHA.108.191097 PubMed: 19228820
  5. [E2] AHA/ACCF/HRS recommendations for the standardization and interpretation of the electrocardiogram: part III: intraventricular conduction disturbances: a scientific statement from the American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; the American College of Cardiology Foundation; and the Heart Rhythm Society: endorsed by the International Society for Computerized Electrocardiology . Circulation (2009) . DOI: 10.1161/CIRCULATIONAHA.108.191095 PubMed: 19228822
  6. [G1-09] Management of Patients With Severe Mitral Annular Calcification: JACC State-of-the-Art Review . J Am Coll Cardiol (2022) . DOI: 10.1016/j.jacc.2022.06.009 PubMed: 35953138
  7. [G1-10] The First Heart Sound . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250175
  8. [G1-14] Systolic Murmurs . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250186
  9. [G1-15] Diastolic Murmurs . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250187
  10. [V1] 2025 ESC/EACTS Guidelines for the management of valvular heart disease . Eur Heart J (2025) . DOI: 10.1093/eurheartj/ehaf194 PubMed: 40878295
  11. [V3] Recommendations for Noninvasive Evaluation of Native Valvular Regurgitation: A Report from the American Society of Echocardiography Developed in Collaboration with the Society for Cardiovascular Magnetic Resonance . J Am Soc Echocardiogr (2017) . DOI: 10.1016/j.echo.2017.01.007 PubMed: 28314623