首页 临床医学 心血管病学 超声心动图 第二阶段 Valves

# Learning Obiectives

  • Anatomy of the PV 解剖
  • Echocardiographic views to assess PV 超声切面
  • Pathologies of the PV 病理学
  • Assessment of severity of PV lesions 严重程度评估

# Pulmonary Valve (PV)

  • Semilunar valve: separates RVOT from main PA
    半月瓣：分隔右室流出道与肺动脉
  • Anterior and superior to the aortic valve
    位于主动脉瓣前上
  • Trileaflet; but a "thinner' structure than the AV as it operates against a lower pressure
    三叶瓣；肺动脉瓣的瓣膜厚度比主瓣要薄，因为它对抗的压力要比主瓣小；有时候超声短轴切面没法看清楚三个瓣
  • Most disorders are congenital
    大多数疾病是先天性的

  • The relative positions of the aortic, mitral, pulmonary, and tricuspid valves are shown in the diagram of the heart at the center of the figure.
    主动脉瓣、二尖瓣、肺瓣和三尖瓣的相对位置如图中心的心脏图所示。
  • The aortic valve has three cusps: the left coronary cusp (LCC), the right coronary cusp (RCC), and the non-coronary cusp (NCC).
  • The mitral valve has an alphanumeric nomenclature that numbers from the anterior to the posterior, with respect to the heart, and attaches an A or a P in front of the anterior or posterior leaflets, respectively (A1-A3, P1-P3).
  • The pulmonary valve has three cusps: the anterior cusp (AC), the left cusp (LC), and the right cusp (RC).
  • The tricuspid valve has three leaflets named the anterior (A), septal (S), and posterior (P).

# Pulmonary Stenosis (PS)

  • Prevalence 8/10,000 live births
    发病率
  • Site of stenosis 狭窄部位
    • Valvular 瓣膜
    • Sub valvular: (RVOT obstruction)
      瓣下（右室流出道狭窄）
    • Supra valvular
      瓣上
  • Severity of stenosis 狭窄的严重程度
  • Aetiology of stenosis 狭窄的病因（风湿性或其他）
  • Ancillary findings: i.e RV hypertrophy
    辅助发现：即右室肥厚

Table 1 Common genetic syndromes associated with pulmonary stenosis [1]
与肺动脉狭窄相关的常见遗传综合征

Syndrome
综合征

Genetic defect
遗传缺陷

Cardiac features
心脏特征

Non-cardiac features
非心脏特征

Noonan
努南综合征

PTPN11, SOS1, aberrant RAS-MAPK-signalling, heterogeneous trait

Dysplastic pulmonary valve stenosis, supravalvular pulmonary stenosis, hypertrophic cardiomyopathy
发育不良的肺动脉瓣狭窄、瓣上狭窄、肥厚型心肌病

Short stature, hypertelorism, downward eye slant, low set ears
身材矮小，高度近视，眼睛向下倾斜，耳朵低垂

Williams-Beuren
威廉综合征

7Q11.23 deletions, autosomal dominant trait
常染色体显性遗传

Supravalvular aortic or pulmonary stenosis
主动脉瓣上或肺动脉瓣狭窄

Elfin face, short stature, impaired cognition and development, endocrine disorders, genitourinary abnormalities
瘦脸、身材矮小、认知和发育受损、内分泌紊乱、泌尿生殖系统异常

Leopard
Noonan 综合征伴多发性痣

PTPN11, RAF-1, autosomal dominant trait
常染色体显性遗传

Electrocardiographic abnormalities, supravalvular or valvular pulmonary stenosis
心电图异常、瓣上或瓣膜肺动脉狭窄

Lentigines, ocular hypertelorism, abnormal genitalia, retardation of growth, deafness
痣、高度近视、生殖器异常、生长迟缓、耳聋

DiGeorge (velocardiofacial)
室间隔畸形

22011 deletion, autosomal dominant trait
常染色体显性遗传

Conotruncal defects such as tetralogy of Fallot, interrupted aortic arch, truncus arteriosus, vascular rings and ASD/VSD
圆锥干畸形（流出道、大血管先天畸形），如法洛四联症、主动脉弓中断、动脉干、血管环及房间隔 / 室间隔缺损

Hypertelorism, low set and posteriorly rotated ears, palatal abnormalities, micrognathia. Developmental delay, hypoplastic thymus, hypocalcaemia, variety of immunological abnormalities
耳廓过长、低位及后倾、腭部异常、小颌下垂。发育迟缓、胸腺发育不良、低钙血症、各种免疫异常

Allagile

JAG-1, NOTCH-2, dominant trait
显性遗传

Peripheral pulmonary stenosis
外周性肺动脉狭窄

Facial dysmorphias (triangular face, wide nasal bridge, deep set eyes), intrahepatic cholestasis, butterfly vertebrae
面部变形 (三角脸、鼻梁宽、眼睛深陷)、肝内胆汁淤积症、蝴蝶椎体

Keutel

MGP mutations, autosomal recessive trait
常染色体隐性遗传

Multiple peripheral pulmonary stenosis
多发性周围性肺狭窄

Abnormal cartilage calcifications, brachytelephalangy. subnormal IQ, hearing loss
软骨异常钙化，短指畸形。智商不正常，听力丧失

Congenital rubella
先天性风疹

-

Peripheral pulmonary stenosis, open ductus Botalli
外周性肺动脉狭窄、动脉导管未闭

Congenital cataract/glaucoma, deafness, pigmentary retinopathy
先天性白内障 / 青光眼、耳聋、色素视网膜病变

ASD, atrial septal defect; VSD, ventricular septal defect.

# Valvular Pulmonary Stenosis (PS) 瓣膜性肺动脉狭窄

  • Frequently asymptomatic systolic murmur
    频繁无症状收缩期杂音
    第二心音由主动脉瓣、肺动脉瓣关闭产生，如果存在狭窄，瓣膜关闭时间会延长，临床上表现为第二心音延长
  • Mild PS seldom progresses; seldom symptomatic (ie no reduction in exercise tolerance)
    轻度 PS 很少进展；很少有症状 (即运动耐力没有降低)
  • Moderate PS progressive; associated with RV hypertrophy
    中度进行性 PS；与右室肥厚相关
    如果实际操作中测得 PS 为轻度，应在多个切面放置 CW 或 PW，以反复确认跨瓣压差，避免低估狭窄程度
  • Severe PS: exercise intolerance, dyspnoea, lightheadedness, chest pain (RV angina)
    重度 PS，有临床症状：运动不耐受、腹泻、头晕、胸痛（右心室心绞痛）
    右室提供了左室的血供，如果因为肺动脉瓣狭窄导致左室前负荷降低，充盈的延迟或减少，会导致血压降低导致头昏；如果有右室肥厚，血液必须供应更大量的肌肉，因此可能会出现心肌缺血（ischemia）而出现心绞痛症状

  • Almost always congenital in origin; isolated valvular PS ~ 8-10% of all congenital heart defects
    几乎都是先天性的；单纯瓣膜 PS 占所有先天性心脏病的 8-10%
  • Often associated with other complex congenital HD: eg ToF
    常与其他复杂的先天性心脏病有关：例如法洛四联症
  • Acquired PS: commonest is carcinoid (PS and PR), very rarely rheumatic
    获得性 PS：最常见的是类癌 (肺动脉狭窄和反流)，很少是风湿性的
  • Congenital PS: dysplastic leaflets: ~ 20%
    先天性 PS：瓣叶发育异常约占 20%

# SUB Valvular 瓣膜下

  • Congenital: associated with VSD / ToF
    先天性：与室间隔缺损 / 法洛四联症相关
  • Acquired: due to severe RVH, prior surgery, associated with hypertrophic/infiltrative cardiomyopathy
    获得性：由于严重的右室肥厚，既往手术，与肥厚性 / 浸润性心肌病相关
  • Often PV is normal
    通常情况下，肺瓣本身是正常的

# SUPRA Valvular 瓣膜上

  • main pulmonary arteries(MPA), bifurcation, branch vessels
    大肺动脉、分叉、分支血管
  • Associated with Noonan's syndrome, Williams syndrome
    与努南综合征、威廉姆斯综合征相关


# Pulmonary Regurgitation (PR) 肺动脉瓣反流

  • Presents to some degree in 40-78% of individuals with normal pulmonary valves
    40%-78% 的正常肺动脉瓣患者可有不同程度的反流表现
  • Acquired PR due to dilatation of the PA: most commonly due to PHTN
    由于 PA 扩张而获得的 PR：最常见的原因是 PHTN（肺动脉高压）
  • Severe PR usually Occurs with other cardiac abnormalities
    严重的 PR 通常与其他心脏异常一起发生

# AETIOLOGY 病因学

  • Congenital abnormalities: bicuspid/quadricuspid valve
    先天性畸形：二尖瓣 / 四尖瓣
  • Pulmonary valve hypoplasia 肺动脉瓣发育不全
  • Post TOF repair 法洛四联症修复后
  • Prolapse: rare 脱垂：罕见
  • Bacteria endocarditis 细菌心内膜炎
  • Carcinoid syndrome 类癌综合征
  • Post valvotomy 瓣膜切开术后

# Echocardiographic evaluation 超声心动图评估

  • TTE, TOE, 3DE views (standard and modiffied)
    标准和改良的切面

    • Limited visualisation; most difficult valve to evaluate
      有限的可视化；视野受限，是最难评估的瓣膜
    • Anomalies of the leaflet: no of cusps, thickening, doming, calcification
      瓣叶形态异常：无尖、增厚、隆起、钙化
      评估包括狭窄和反流（定性和定量）
    • Abnormal structure: hypoplasia, dysplasia
      结构异常：发育不全、发育异常

  • Evaluation of the PV should be performed in conjunction
    应联合进行

    • RVOT 右室流出道
    • RV 右室
    • Pulmonary branches 肺动脉分支
  • 狭窄时可能有 RVOT 扩张，右室肥厚
  • 肺动脉主干或者分支有没有扩张，来评估狭窄的位置是瓣上还是瓣膜本身还是瓣下


  • 左侧 9 点钟位置 —— 右房
  • 上方 12 点钟位置 —— 右室
  • 两者之间是三尖瓣
  • 右下方 3 点钟位置 —— 肺动脉瓣、肺动脉主干，下方是两个肺动脉分支

# TTE





  • PLAX, PSAX, Subcostal views
    左侧胸骨旁（最常用）长轴、短轴，剑突下
  • PSAX, standard view for assessment of the PV
    胸骨旁短轴，是 PV 评估的标准视图
  • Transducer position at base of heart where PA bifurcates
    换能器放在心底部 PA 分叉处
  • 患者左侧卧位，手放在头部后方
  • 探头从胸骨旁长轴切面开始，顺时针 90° 旋转得到主动脉瓣水平短轴切面
  • 向上向右肩倾斜，得到改良版的肺动脉瓣水平切面


  • Subcostal views with anterior angulation
    剑突下切面伴探头前倾，成像角度不一样但内容与胸骨旁差不多
    • Transducer on abdomen just below xyphoid process
      腹部剑突正下方的换能器
    • Notch at 6 o'clock
      切口指向 6 点钟位置
    • Tilt the ultrasound probe leftward
      向左倾斜超声探头

# Pulmonary Valve (PV)

Qualitative to identify mechanism
定性，识别机制

Quantitative
定量

  • Description of number of cusps 描述瓣叶数量
  • Prolapse 脱垂
  • Dysplasia 发育不良
  • Absent of PV (atresia or agenesis)
    无肺动脉瓣（闭锁或发育不全）
  • Annulus and RVOT diameters 瓣环和右室流出道内径
  • Calculation of PV area 瓣膜钙化
  • CW Doppler
  • Systolic and diastolic VTI
    收缩和舒张期速度时间积分
  • PW Doppler

# Pulmonary Stenosis (PS)

  • 2D Echo (anatomy and structure) 解剖与结构
  • PLAX
  • PSAX: level of the AV
  • Thickened leaflets, doming, calcification
    描述瓣叶，增厚、穹隆样改变、钙化
  • Variable degree of commissural fusion
    不同程度的交界区融合
  • Post stenotic dilatation of MPA
    狭窄后，肺动脉主干的继发扩张
  • RV size is usually normal: as with RVH (>5mm) and RV hypoplasia
    右室大小通常是正常的：例如伴右室肥厚 (>5 mm) 和右室发育不良

居中的是主动脉瓣，大概 1-2 点钟方向是肺动脉瓣。PV 看起来有点增厚、钙化，不像正常时的那么薄，有点穹隆样改变，活动有点僵硬，尤其是上方的瓣膜活动度欠佳。右方的肺动脉呈现瓣膜狭窄后继发的扩张（正常应该是小于主动脉部分的尺寸）。
看到这样的 2D 图像时，就应该要放上彩色血流、CW、PW 进行分析。


Echocardiographic measurements of right ventricular dimensions. (A) Parasternal long axis view. (B) Parasternal short axis view. (C) Apical four chamber view. Ao, aorta; LA, left atrium; LV, left ventricle; PA, pulmonary artery; RA, right atrium; RV, right ventricle; RVIT, right ventricular inflow tract; RVLAX, right ventricular long axis; RVOT, right ventricular outflow tract.
超声心动图测量右室内径。(A) 胸骨旁长轴观。(B) 胸骨旁短轴切面。(C) 心尖四腔切面。AO，主动脉；LA，左房；LV，左室；PA，肺动脉；RA，右房；RV，右室；RVIT，右室流入道；RVLAX，右室长轴；RVOT，右室流出道。

  • Colour Flow Doppler 彩色血流
    • Turbulence/aliasing 有无湍流 / 混叠现象
    • Site of turbulence helps placement of Doppler cursor 湍流位置及方向有助于放置多普勒取样线，进行最大血流速度的描绘
    • Associated regurgitation 伴反流

在 2D 下如果见到了肺动脉瓣增厚或穹隆样改变，可以加上彩色血流。彩色血流图可以看到湍流，在放置 CW 或者 PW 时，可以使取样线对齐湍流的方向，以获得最大速度来确定狭窄的程度
舒张期也可以观察有没有肺动脉瓣反流，若存在，也需要评估和分析

  • Doppler PW and CW
    • PW Doppler
      • locate the site of peak velocity
        定位峰值速度的位置
    • CW Doppler
      • Peak velocity/VTI
        峰值速度 / 速度时间积分
      • Mean gradient
        平均压差
      • Peak gradient (4V2)
        峰值压差
    • 3-5 cycles to adjust for respiratory changes
      3-5 个周期以适应呼吸变化


完整超声评估一般开始于胸骨旁长轴，进行成像，观察室间隔、左室内腔、下侧壁或使用 2D M 型进行测量，然后是彩色多普勒，观察各瓣膜有没有湍流或反流
左室长轴结束后，切到右室流入道流出道观察。右室流出道包括右室流出道、肺动脉瓣、肺动脉主干的观察。
再到胸骨旁短轴切面，成像主动脉瓣水平以及改良版看肺动脉瓣，加持彩色多普勒，若存在异常可以 CW 或 PW 进行定量分析
继续进行心尖四腔五腔三腔等系统的完整评估

MildModerateSevere
CWPeak Doppler velocity (m/s) 最大速度＜33-4＞4
Peak Doppler gradient (mmHg) 最大跨瓣压差＜3636- 64＞64
Mean Doppler gradient (mmHg) 平均跨瓣压差＞ 40
  • Aways check tricuspid regurgitation gradient to rule out overestimation of pulmonary stenosis gradient
    通常也要评估三尖瓣反流压差，以避免高估肺动脉狭窄程度

# Supravalvular PS

  • Often associated with syndromes 通常伴有综合征的出现
  • Can have stenosis at multiple sites ie MPA + branched PA
    狭窄可以出现在多个部位（例如主干 + 分支或更远端）
  • PV gradient should comprise most of RVSP if single for stenosis
    如果狭窄是单一的，跨肺动脉瓣压差应该包括大部分右室收缩压


  • CW 应该尽量取样到合适的部位以获得最大血流速度，以更好的估算压差

# Pulmonary Regurgitation (PR)

  • Diastolic jet in the RVOT 右室流出道中的舒张期血流
  • Jet length >10 mm 射流长度 > 10 毫米
  • Physiological vs pathological PR: duration of flow ie holodiastolic
    生理与病理 PR: 血流持续时间，即全舒张期
  • Jet width: wider jets worse； evaluate at jet origin "vena contracta" [2]
    射流宽度：较宽的射流更糟；在反流起始处评估缩流颈宽度
  • Severe PR: jet width vs RVOT width > 55-65%
    严重 PR: 射流宽度 vs RVOT 宽度 > 55-65%
  • Detection of flow reversal in pulmonary arteries
    肺动脉血流逆转的检测


  • CW Doppler: jet intensity (qualitative)
    连续波多普勒：反流束强度 (定性)
  • Rapid flow deceleration (short PHT < 100ms)
    血流快速减速 (短 PHT<100ms)

  • (A) left, complete lack of valve coaptation and right, the measurement of the vena contracta width(VC);
  • (B) colour-coded M-mode depicting the time-dependency of flow signal during the heart cycle;
  • (C) continuous Doppler recording of PR showing a rapid flow deceleration during the diastole (red arrow) and increased systolic flow velocity (not related to concomitant pulmonary stenosis).
    PR 的连续多普勒记录显示舒张期血流迅速减速 (红色箭头)，收缩期血流速度增加 (与合并的肺动脉狭窄无关)。

  • CW Doppler of pulmonic flow. Calculation of pulmonic regurgitation index (PR index = A/B) is shown, an index of PR severity, quantitating early termination of diastolic regurgitant flow.
    肺动脉血流的连续波多普勒。计算了肺动脉反流指数 (PR 指数 = A/B，A = 反流时长，B = 反流开始到肺动脉瓣再次打开)，作为 PR 严重程度的指标，量化了舒张期反流束的早期终止。
Pulmonary regurgitation
MildModerateSevere
Pulmonic valve 肺动脉瓣形态NormalNormal or abnormalAbnormal and may not be visible

Right ventricle (Size) 右室大小

Normal1
(RVD1basal < 41mm)

Normal or dilated

Dilated2
(RVD1basal > 41mm)

RegJetlength 反流束长度

Regurgitant jet length (Nyquist limit 50-70cm/s)

Thin with a narrow origin 稀薄、起源窄
(usually < 10 mm in length)

Intermediate
介于两者之间

Broad origin; variable depth of penetration
起源宽、穿透深度多变

RatioRegJet/PV 反流束宽度与瓣环径比值

Ratio regurgitant jet width / pulmonary valve annulus

> 70%3

RegJetdensity 反流束密度（CW)

Regurgitant jet density (CW doppler)

Soft
软

Dense
密集

Dense; early termination of diastolic flow
致密；舒张期血流早期终止

DTRegJet 反流束减速时间

Deceleration time of pulmonary regurgitant jet

Short4 短
(<260ms)

PHTRegJet 反流束压差减半时间

Pressure half time of pulmonary regurgitant jet

< 100ms5

PR index6 肺动脉瓣反流指数

Pulmonory regurgitation index

< 0,77

< 0,77

PAreversal flow 肺动脉逆向血流

Reversal flow in the branch pulmonary artery

Yes

PV VTI / LVOT VTI7 肺瓣 VTI 与左室流出道 VTI 之比

Pulmonic systolic VTI compered to LVOT VTI

Slightly increased

Intermediate

Greatly increased

RF8 肺动脉瓣反流分数

Regurgitant fraction of pulmonary valve

< 20%

20-40%

> 40%

  1. Unless there are other reasons for RV enlargement.
    除非有其他原因导致 RV 扩大。
  2. Exception: acute PR. 排除急性反流
  3. Identifies a CMR-derived PR fraction > 40%.
    确定 CMR 所测得的 PR 分数 > 40%。
  4. Steep deceleration is not specific for severe PR.
    陡峭减速不是严重 PR 所特有的
  5. Not reliable in the presence of high RV end diastolic pressure.
    在存在高 RV 舒张末期压力的情况下不可靠
  6. Defined as the duration of the PR signal divided by the total duration of diastole, with this cutoff identifying a CMR-derived PR fraction > 25%.
    定义为 PR 信号的持续时间除以舒张期的总持续时间，该分界值识别 CMR 所测得 PR 分数 > 25%。
  7. Cutoff values for regurgitant volume and fraction are not well validated.
    反流体积和分数的分界值没有得到很好的验证
  8. RF data primarily derived from CMR with limited application with echocardiography.
    反流分数的数据主要来自 CMR，超声心动图的应用有限。

Other techniques of PR quantification

  • Very limited data; small sample size and not validated
    数据非常有限；样本量小，未经验证
    • PW Doppler: $$ \text{difference between reverse and forward VTI} = \% \text{regurgitant flow} $$
      PW 多普勒：反向与正向 VTI 的差值 = 反流血流的百分比
    • PV forward VTI vs AV forward VTI < 1 normally
      正常情况下，PV 正向 VTI 与 AV 正向 VTI 之比 < 1
  • Not validated; no ref ranges
    未验证；没有参考范围

    • %RF=VTI of PRVTI of forward flow\% \text{RF} = \frac{\text{VTI of PR}}{\text{VTI of forward flow}}

    • EROA (mm2)
    • PISA method

  1. Cuypers, J. A. A. E., Witsenburg, M., van der Linde, D. & Roos-Hesselink, J. W. Pulmonary stenosis: update on diagnosis and therapeutic options. Heart 99, 339–347 (2013). ↩︎

  2. Lancellotti, P. et al. Recommendations for the echocardiographic assessment of native valvular regurgitation: an executive summary from the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging 14, 611–644 (2013). ↩︎