MioAtlas
G2: 右心系统瓣膜

肺动脉瓣狭窄（穹隆样）

先天性肺动脉瓣狭窄，特征为瓣交界处融合以及薄且柔软瓣叶的收缩期穹隆样（doming）膨出。

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定义

因瓣叶增厚、交界融合或瓣叶发育不良导致肺动脉瓣水平先天性右心室流出道梗阻，引起收缩期穹隆样开放（doming），在肺动脉内产生高速湍流射流并对右心室施接收缩期压力负荷过重 [C4] [C5] [G1-01] [G23-04]。

严重程度标准

  • 轻度（L）：跨肺动脉瓣峰值流速（Vmax）< 3.0 m/s；跨瓣峰值压差 < 36 mmHg [C5] [G1-01]。
  • 中度（M）：Vmax 3.0–4.0 m/s；峰值压差 36–64 mmHg [C5] [G1-01]。
  • 重度（G）：Vmax > 4.0 m/s；峰值压差 > 64 mmHg（或平均压差 > 35–40 mmHg）[C5] [G1-01] [C4]。

分层表现

血流动力学

  • 跨肺动脉瓣峰值血流流速增快：L < 3.0 m/s，M 3.0–4.0 m/s，G > 4.0 m/s [C5] [G1-01]。
  • RV-PA收缩期压力阶差显著升高，在重度病变中右心室收缩压可等于或超过体循环动脉压 [C5] [G1-01]。
  • 肺动脉远端压力正常或偏低 [C5]。

心律

  • 正常窦性心律 [C5]。

ECG

  • 重度向心性右心室肥厚：V1导联高R波（> 7 mm），V1导联R/S比值 > 1，右胸前导联V1–V3出现ST段压低及不对称性T波倒置（右室收缩期负荷过重图形）[E1] [C5]。
  • QRS电轴右偏（电轴右偏 > +90° 至 +110°）及右心房扩大的尖峰P波（肺型P波）[E1]。

心音与杂音

  • 收缩期喷射性杂音：在胸骨左缘第 2 肋间（肺动脉瓣区）闻及最响并向左锁骨及肩胛间区传导的粗糙收缩中晚期喷射性杂音（菱形渐强-渐弱型）。随着严重程度增加，杂音持续时间延长且达峰后移 [G1-14] [G23-04]。
  • 肺动脉瓣喷射喀喇音：紧随S1之后在肺动脉瓣区闻及的高频瓣膜开启喀喇音，其独特声学特性表现为在吸气时减弱或消失（吸气使右室舒张末期压升高，在心室收缩前被动顶开瓣叶）[G1-16] [G23-04] [G23-05]。
  • 第二心音（S2）：S2宽分裂，因右心室射血时间延长及瓣叶僵硬导致显著延迟且柔和低钝的肺动脉瓣成分（P2）（P2低钝）[G1-11] [G23-04]。
  • 右心第四心音（S4）：在重度病变中常见，由右心房对僵硬肥厚的右心室有力收缩所致 [G1-13]。

动脉脉搏

  • 动脉脉搏正常，或极重度狭窄伴右心前向输出减少时呈小脉（细脉）[B4]。

颈静脉搏动

  • 在重度病变中巨大“a”波极为显著，反映右心房对抗顺应性减低的肥厚RV进行有力收缩 [B3]。

负荷试验

  • 深吸气：使肺动脉瓣喷射喀喇音减弱或消失（以此与主动脉喀喇音鉴别的特征性表现）[G23-04] [G23-05]。
  • Valsalva动作：用力相期间杂音强度减弱，用力解除后迅速恢复（在 1–2 次搏动内）[B1]。

模拟器严重程度分级

模拟器严重程度分级: 轻度, 中度, 重度
基线心律: 正常窦性心律

亦称为： 肺动脉瓣狭窄 · 先天性肺动脉瓣狭窄 · 瓣膜性肺动脉瓣狭窄 · 肺动脉狭窄 · PS

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. [C4] 2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines . Circulation (2025) . DOI: 10.1161/CIR.0000000000001402 PubMed: 41411375
  5. [C5] 2020 ESC Guidelines for the management of adult congenital heart disease . Eur Heart J (2020) . DOI: 10.1093/eurheartj/ehaa554 PubMed: 32860028
  6. [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
  7. [G1-01] Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice . J Am Soc Echocardiogr (2009) . DOI: 10.1016/j.echo.2008.11.029 PubMed: 19130998
  8. [G1-11] The Second Heart Sound . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250183
  9. [G1-13] The Fourth Heart Sound . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250185
  10. [G1-14] Systolic Murmurs . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250186
  11. [G1-16] Ejection Clicks . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250188
  12. [G23-04] Auscultatory and phonocardiographic signs of pulmonary stenosis . Br Heart J (1957) . DOI: 10.1136/hrt.19.3.303 PubMed: 13446314
  13. [G23-05] Recommendations for evaluation of prosthetic valves with echocardiography and doppler ultrasound: a report From the American Society of Echocardiography's Guidelines and Standards Committee and the Task Force on Prosthetic Valves, developed in conjunction with the American College of Cardiology Cardiovascular Imaging Committee, Cardiac Imaging Committee of the American Heart Association, the European Association of Echocardiography, a registered branch of the European Society of Cardiology, the Japanese Society of Echocardiography and the Canadian Society of Echocardiography, endorsed by the American College of Cardiology Foundation, American Heart Association, European Association of Echocardiography, a registered branch of the European Society of Cardiology, the Japanese Society of Echocardiography, and Canadian Society of Echocardiography . J Am Soc Echocardiogr (2009) . DOI: 10.1016/j.echo.2009.07.013 PubMed: 19733789