超声心动图评价致心律失常性右心室心肌病病变程度的临床研究

目的 分析致心律失常性右心室心肌病(ARVC)患者的病变程度与超声心动图表现之间的关系.方法 分析61例已确诊的ARVC患者,根据心脏磁共振(MRI)检查结果,将其按病变侵犯部位分为右心室局部病变组(A组)、右心室弥漫病变组(B组)、累及左心室的双心室病变组(C组),分析比较3组间的超声心动图.结果 心脏MRI结果示,A组患者19例(31%),B组28例(46%),C组14例(23%).共15例(25%)患者超声心动图结果正常(A组13例,B组2例).A组患者中均无右心房增大、右心室室壁变薄、右心室室壁运动减弱.超声心动图示:右心房、心室增大,右心室流出道增宽、右心室室壁变薄及室壁运动减弱均与病变程度呈正相关,而左心参数差异均无统计学意义.结论 早期无症状或局部轻度病变时应用超声心动图检查容易漏诊,应当结合临床表现、心电图并进行心脏MRI检查.依据超声心动图结果中右心参数可以判断病变进展程度,指导治疗方案的选择.     

致心律失常性右心室发育不良的超声心动图特点分析

目的:分析致心律失常性右心室发育不良(ARVC)的超声心动图表现,结合多排螺旋CT、心血管造影等影像学手段,以及心电图等特异性表现,提高超声工作者对ARVC的认识,减少误诊率。方法:分析ARVC的超声心动图特征,并利用二维斑点追踪技术测量右心室壁各节段应变率,与正常组对比分析。结果:超声心动图可表现为右心室增大、右心室流出道增宽、右心室壁运动弥散性减弱以及局部减弱,部分病例可见室壁瘤形成,三尖瓣反流多见等。二维斑点追踪显示ARVC组整体右心室壁应变率减低,以右心室心尖段为著。结论:超声心动图结合患者临床、心电图及其他影像学检查将大大提高ARVC的诊断率,超声心动图可较早发现患者的心脏改变,是评估患者预后及随访观察的重要手段。     

心电图评价致心律失常性右心室心肌病病变程度的临床价值

目的 分析致心律失常性右心室心肌病(ARVC)患者的病变程度与心电图表现之间的关系.方法 分析61例已确诊的ARVC患者,根据心脏核磁共振成像(MRI)检查结果,将其按病变侵犯部位分为右心室局部病变组、右心室弥漫病变组、双心室病变组,分析比较三组的心电图特征.结果 心脏MRI结果显示右心室局部病变组19例(31%),右心室弥漫病变组28例(46%),双心室病变组14例(23%).心电图正常者3例,三组中各1例.伴有Epsilon波的患者24例(39%)、V1～V3导联的QRS波时限≥110 ms的患者21例(34%)、V1～V3导联S波升支≥55 ms的患者17例(28%)、完全右束支传导阻滞的患者10例(16%)、病理性Q波的患者9例(15%),这些指标的发生率均随病变程度的加重而增高(右心室局部病变组＜右心室弥漫病变组＜双心室病变组).Epsilon波、V1～V3导联的QRS波时限≥110 ms、完全性右束支传导阻滞(RBBB)、病理性Q波的发生率在双心室病变组中要高于右心室局部病变组,且两组间比较差异有统计学意义(P＜0.05).V1～V3导联S波升支≥55 ms的发生率在双心室病变组中要高于右心室局部病变组,且两组间比较差异有统计学意义(P＜0.05);在双心室病变组要高于右心室弥漫病变组,且两组间比较差异均有统计学意义(P均＜0.05).一度房室传导阻滞的发生率在双心室病变组中要高于右心室弥漫病变组,且两组间比较差异有统计学意义(P＜0.05).右心室局部病变组患者心电图T波倒置多局限于V1导联,右心室弥漫病变组和双心室病变组T波倒置多数表现于胸前导联V1～V3或超过V3导联的胸前导联、以及下壁导联.结论 心电图正常并不能排除ARVC.ARVC患者T波倒置在12导联心电图上具有很高的发生率,并且T波倒置在胸部导联的延伸与病变程度是相关的,T波倒置的范围可以提示ARVC病变累及的程度.

Abstract：

Objective To analyze the relationship between electrocardiographic (ECG) features and disease severity in patients with the arrhythmogenic right ventricular cardiomyopathy (ARVC). Method The study group consisted of 61 subjects with a definite diagnosis of ARVC on the basis of published guideline criteria and patients were divided into 3 subgroups according to the extent of diseased myocardium defined by cardiac magnetic resonance imaging (MRI): Group A: local involvement (n = 19, 31% ), Group B: diffuse involvement of whole right ventricle ( n = 28, 46% ) and Group C: involvement of both right and left ventricles ( n = 14, 23% ). Results Normal electrocardiogram was shown in 1 patient in each group.Epsilon wave was detected in 24 (39%) patients, QRS duration was prolonged [≥ 110 ms( V1 -V3 )] in 21 (34%) patients, S-wave upstroke was prolonged (≥55 ms) in 17 (28%) patients, complete right branch bundle block was evidenced in 10 ( 16% ) patients and pathologic Q waves was found in 9 ( 15% ) patients. The incidence of above abnormal ECG changes was increased in proportion to the degree of disease severity (group A ＜ group B ＜ group C). Incidence of Epsilon wave and prolonged QRS duration [≥ 110 ms (V1 - V3 )] were significantly higher in Group C than in Group A. Incidence of prolonged S-wave upstroke ( ≥55 ms) was significantly higher in Group C than in Group A and Group B. T-wave inversion in V1 leads was often found in Group A. T-wave inversion in inferior leads ( V1 - V3 leads or beyond V3 ) was often presented in Group B and Group C. Conclusions Normal ECG does not exclude the possibility of diagnosis of ARVC. The extent of T-wave inversion in the precordial leads and incidence of Epsilon wave, prolonged QRS duration [≥ 110 ms (Vt -V3 )] and prolonged S-wave upstroke ( ≥55 ms) were related to degree of disease severity in patients with ARVC.     

右心室壁传导阻滞在致心律失常性右心室心肌病诊断中的地位

目的 探讨右心室壁传导阻滞(parietal block)在致心律失常性右心室心肌病(arrhythmogenic right ventricular dysplasia,ARVC)诊断标准中的适用性.方法 回顾性分析可疑ARVC先证者62例的临床评估资料,项目包括性别、心电图、24 h动态心电图、超声心动图.对比分析...     

超声心动图对致心律失常性右室心肌病的诊断评价

目的分析超声心动图诊断致心律失常右室心肌病（ARVC）的图像特点。方法对5例ARVC患者的临床资料及超声心动图检查结果进行分析。结果5例ARVC患者中4例以室性心律失常发病,1例为家系检查发现。1例猝死、1例心力衰竭、2例晕厥、1例无症状。所有患者均做了心电图、超声心动图检查,部分患者做了心室核素造影和右心室造影。超声心动图表现为：所有患者均右心室扩大,3例右室流出道扩张,1例左室稍大;3例右室壁运动弥漫性减弱,1例局限性减弱,1例右室壁瘤形成,2例室间隔运动异常。5例患者三尖瓣、肺动脉瓣均有反流,反流压差较低,前向流速减低。结论ARVC超声心动图的表现为右心室扩大、右室流出道扩张、右室壁弥漫性、局限性变薄、运动异常。患者以反复发作性室性心动过速、晕厥、猝死为首发症状,在除外其他可引起右室扩大的病变后可提示本病。     

累及左心室的致心律失常性右室心肌病临床表型分析

目的:分析累及左心室的致心律失常性右室心肌病(ARVC)患者的临床特点、心电图及超声心动图指标,探索左心室与ARVC间的关系,以加深对左心室受累临床表型的认识。方法:回顾性搜集郑州大学第一附属医院心内科2011-01-2019-05临床确诊为ARVC的患者,入选超声心动图提示心室结构和(或)功能异常的患者39例,根据超声心动图分为右室孤立病变组21例和左室受累组18例,比较两组的一般临床资料、心电图及超声心动图指标。结果:入选患者中,18例(46.2%)左心室受累。左室受累组发病年龄、确诊年龄均明显小于右室局部病变组。在发病至确诊时长、性别比例、发病诱因、合并症、猝死家族史、症状、临床心力衰竭等一般临床资料和心电图指标(QRS波低电压、Epsilon波、T波倒置)方面,两组差异均无统计学意义。与右室孤立病变组相比,左室受累组左室舒张末期内径(LVEDD)、左室舒张末期容积(LVEDV)、左室收缩末期容积(LVESV)更大,左室射血分数(LVEF)更低,肺动脉收缩压更低。两组RVEDD、右室功能、二尖瓣和三尖瓣反流差异均无统计学意义。结论:左心室受累可能是ARVC的独立临床表型,可发生在疾病的任何阶段,而不是疾病进展至晚期的临床表现。     

超声心动图定量分析妊娠期糖尿病胎儿心脏功能

目的对超声心动图在妊娠期糖尿病胎儿心脏功能评价当中的效果进行探究。方法选取2016年1月—2017年1月间在该院门诊行胎儿心脏超声检查的36例妊娠期糖尿病患者作为观察组,进行超声心动图的胎儿心脏功能分析,选取36名健康孕妇胎儿作为对照组,对胎儿的心脏功能进行评价。对比两组患者的心脏结构以及各项功能指标,对妊娠期糖尿病胎儿心脏功能进行分析。结果在对两组进行超声心动图的检测之后,两组右心室舒张末期内径、左心室厚度、右心室壁的厚度以及右心室收缩末期内径等都差异有统计学意义(P0.05)。而在左心室舒张内径、收缩末期内径以及室间隔舒张末期厚度方面差异无统计学意义(P0.05)。结论对于妊娠期糖尿病胎儿的心脏功能来说,通过超声心动图检测,能够对胎儿的功能以及心脏结构变化有一个清楚的分析,提升妇产科医师对孕妇血糖的检测与控制,避免出现损伤胎儿心脏功能的情况,具有较高的推广价值。     

致心律失常性右心室心肌病累及左心室的临床病理分析

目的:分析9例经病理证实以左心室受累为主的致心律失常性右心室心肌病(ARVC)的临床病理特征,提高对左心室受累致ARVC的认识。方法:回顾49例心脏移植中9例经病理证实为左心室受累为主的ARVC患者的一般特征、猝死和心肌病家族史、临床症状和病程、药物治疗史、心电图、24小时动态心电监测(Holter)、超声心动图、心血池、静态心肌显像、心脏磁共振成像、冠状动脉造影及受体心脏大体和组织学病理特征。结果:9例患者平均年龄13~54(40.4±15.9)岁,女性5例,男性4例。心悸胸闷病史0.33~20年,平均(9.1±8.9)年,晕厥史2例,有电除颤史6例,胺碘酮治疗5例。2例有猝死家族史。ARVC特征性心电图异常包括V1~3/4~6导联QRS间期>1.2(占0%),有ε波(占44.4%),胸前导联T波倒置(占33.3%)。9例24小时动态心电监测中4例有阵发性心房颤动,其中2例合并心房扑动;2例未服胺碘酮者室性早搏>1000次/24h,其余7例(5例服用胺碘酮)室性早搏均<1000次/24h,室性早搏形态平均(3.3±1.1)种/24h。9例术前超声心动图显示平均右心室内径(32.6±6.7)mm,左心房内径(37.1±10.3)mm,左心室舒张末径(57.3±11.9)mm,左心室射血分数0.26±0.09。7例患者术前心脏磁共振成像显示,5例可见异常高强度信号区域,5例可见右心室和右心房扩大,4例可见右心室壁瘤样突出。8例术前用Swan-Gans导管作血流动力学监测:平均右心室压(19.3±8.3)mmHg(1mmHg=0.133kPa),平均肺毛细血管楔压(15.6±7.7)mmHg,平均心脏指数(2.0±0.4)L/(min.m2),平均肺血管阻力(267.8±167.6)dyn.s.cm-5。9例病理特征为右心室腔显著扩大7例,右心室壁整体变薄6例,右心室流出道最薄处<1mm。左心室均有受累,显著扩张8例。左、右心室壁均被不同范围的纤维和脂肪组织替代。结论:虽然ARVC累及双心室者难以与扩张型心肌病鉴别,但是结合临床和影像资料,特别是心脏磁共振成像,可提高ARVC诊断的准确性。     

致心律失常生右心室心肌病

致心律失常性右心室心肌病(ARVC)亦称致心律失常性右心室发育不良,是一种心肌细胞逐渐被脂肪和纤维组织取代,病变以右心室为主,有一定家族倾向的原发性心肌病[1].本病最重要的临床意义在于心脏猝死,在30岁以下的猝死患者中,ARVC可占20%[2].     

超声心动图斑点追踪技术对致心律失常性右室心肌病的诊断及预测价值

目的 研究超声心动图及斑点追踪技术在致心律失常性右室心肌病（ARVC）诊断中的应用价值。 方法 选择2017年1月至2019年6月我院收治的60例ARVC患者作为病例组,选择同时期来我院体检的60例健康人作为对照组。比较两组的基本临床资料,进行心脏彩色超声多普勒及斑点追踪技术检查,观察超声心动图主要表现、右心室侧壁各节段在心肌收缩期的峰值及应变率。病例组根据ARVC病情级别分为3个亚组,II级组29例、III级组21例、IV级组10例,比较3个亚组各节段右心室侧壁各节段心肌收缩期峰值速度及应变率。 结果 病例组右心室侧壁各节段在心肌收缩期的峰值速度及应变率均明显低于对照组（P<0.05）;病例组3个亚组右心室侧壁的基底段、中段及心尖段在心肌收缩期的峰值速度及应变率均为II级组>III级组>IV级组（P<0.05）。不同心肌节段峰值速度、应变率对致心律失常性右室心肌病均有一定对诊断价值,中段心肌应变率对致心律失常性右室心肌病的诊断价值最高,曲线下面积高达0.905（0.612-0.978）。 结论 超声心动图及斑点追踪技术可检测心室结构、心肌收缩功能及运动能力的变化,对ARVC的早期诊断及预测病情程度均具有重要价值。     

Clinical profile of arrhythmogenic right ventricular cardiomyopathy in Chinese patients.

OBJECTIVE: To study the clinical profile of Chinese patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). PATIENTS: Chinese patients who fulfilled the diagnostic criteria of ARVC proposed by the Task Force of the European Society of Cardiology and of the scientific council on cardiomyopathy of the International Society and Federation of Cardiology were recruited for analysis. METHODS: Clinical data of patients with ARVC including age, sex, family history, presenting symptoms, electrocardiograph (ECG), echocardiography, cardiac catheterization, magnetic resonance imaging (MRI), electrophysiology study (EPS) and therapeutic intervention were analyzed. RESULTS: Eleven patients (seven males) were diagnosed with ARVC. Mean age at clinical presentation was 42.6+/-14.8 years. Two patients (18.1%) had positive family history of ARVC or premature sudden cardiac death. The commonest presenting symptoms were palpitation (73%) and dizziness (46%). Spontaneous ventricular tachycardia (VT) was the presenting arrhythmia in 54% and 1 (9%) with ventricular fibrillation and cardiac arrest. Seven patients (64%) had the ECG abnormality as defined by the Task Force. Echocardiography showed right ventricular (RV) dilatation in five patients (46%) and all patients had normal left ventricular function. Nine patients (90%) had RV wall thinning or fibrofatty replacement on MRI examination. Inducible monomorphic VT was detected in four out of nine patients at EPS. All eight patients had normal coronary arteries and left ventriculogram but RV dilatation and global hypokinesia was seen in three patients. Implantable cardioverter defibrillators were implanted in five patients and two of them had shocks delivered during the follow-up period. CONCLUSION: In this study, familial incidence of premature sudden death in patients with ARVC appears to be low and left ventricular involvement in affected individuals is uncommon. MRI is still the best investigation for ARVC.     

Abnormal right ventricular size and ventricular septal motion after atrial septal defect closure: etiology and functional significance.

Postoperative echocardiogram often demonstrate persistent right ventricular dilatation and paradoxic ventricular septal motion after repair of an atrial septal defect. To determine the prevalence, causes and significance of these echocardiographic abnormalities, 31 patients were studied with catheterization and echocardiography before and after repair of an atrial septal defect. Before operation, every patient manifested right ventricular dilatation, and all but one had abnormal septal motion. After operation, right ventricular dilatation was noted in 24 (77%) and abnormal septal motion in 21 (68%) patients despite the absence of residual left to right shunting in 30 (97%). These echocardiographic abnormalities could be correlated with age at operation and length of postoperative follow-up study but did not correlate with the degree of preoperative right ventricular enlargement or with shunt size or right ventricular pressure before or after operation. There was no associated functional deficit as demonstrated by the normal maximal oxygen consumption in all 13 patients who underwent treadmill exercise testing 5 to 38 months after operation; these patients included 9 with persistent right ventricular enlargement and abnormal septal motion.     

Correlation between the Parameters of Signal‐Averaged ECG and Two‐Dimensional Echocardiography in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy

Background: The correlation between parameters of two‐dimensional echocardiography and signal‐averaged ECG (SAECG) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) is not known well. Methods: Thirty‐three patients (13 females, 40.3 ± 14.4 years old) were included in this study. Both the right and left ventricular dimensions and systolic function were assessed with two‐dimensional echocardiography. The SAECG was performed with high‐gain amplification and filtered using bidirectional Butterworth filters between 40 and 250 Hz. We evaluated the correlation between the parameters of the SAECG and two‐dimensional echocardiography. Results: The right ventricular (RV) outflow tract was the most frequently (n = 18, 54%) involved segment. Six (18%) patients had only mildly decreased RV systolic function. All the other patients had normal RV systolic function. Although localized left ventricular wall motion abnormalities were observed in 14 (42%) patients, the left ventricular ejection fraction was normal in most (n = 32, 97%). Late potentials were positive in 22 (63%) patients. There was no significant correlation between parameters of the SAECG and two‐dimensional echocardiography for the entire patient population. Conclusions: The SAECG parameters exhibited no correlation to any of two‐dimensional echocardiography parameters in the patients with ARVC. Fragmented electrical activity may develop with no significant relation to the anatomical changes in the patients with ARVC.     

Clinical evaluation of severe idiopathic tricuspid regurgitation

This study clarified the clinical profile and echocardiographic findings of severe idiopathic tricuspid regurgitation (TR). Among 8,538 consecutive ultrasonic examinations, a total of 63 patients had severe TR, which was depicted by color flow mapping as a regurgitant signal more than 4 cm from the tricuspid valve orifice. Thirteen of the 63 patients had no underlying diseases, and these patients with severe idiopathic TR were the subjects of the present study. All 13 patients were over 66 years of age (mean 77.3 +/- 5.6 years old) and had had episodes of right heart failure which responded effectively to diuretics. All 13 patients had atrial fibrillation. Using two-dimensional echocardiography, thickening (77%), prolapse (69%) and malaligned coaptation (54%) of the tricuspid valves were observed. The tricuspid annular diameters, cross-sectional areas of the right and left atria and the right ventricular end-diastolic dimensions were significantly greater than those of the age-and-gender-matched lone atrial fibrillation group and the normal control group (p less than 0.01). The left ventricular dimension and ejection fraction did not differ from those of the matched lone atrial fibrillation group. Other valvular regurgitations were also detected (AR 77%, MR 100%, PR 69%), but the degrees of regurgitation were minimal. We proposed severe TR with tricuspid annular dilatation, right atrial and right ventricular dilatation observed in the aged as a distinct cardiac disease entity.     

Echocardiographic features of right ventricular infarction

In a series of 75 consecutive patients with transmural acute myocardial infarction (AMI) a right-to-left ventricular filling pressure ratio equal to or greater than 0.65 (RVFP/LVFP greater than or equal to 0.65) was assumed to be indicative of associated right ventricular infarction (RVI). Out of 45 patients with inferoposterior myocardial infarction, 11 (24%) had such hemodynamic evidence of right ventricular infarction (group A). The remaining 34 patients with inferoposterior myocardial infarction (group B) and the 30 patients with anterior myocardial infarction did not. Time-motion and two-dimensional echocardiographic examinations were performed 7-10 days after admission in the 62 patients who survived. Right ventricular wall asynergy was found in six of eight group A patients. In three of these, right ventricular dilatation was also present. No patient in group B with inferior infarction or with anterior infarction showed abnormal right ventricular wall motion. While hemodynamic monitoring seems presently the most specific diagnostic method and it is of invaluable help in the choice of the best pharmacological therapy of right ventricular failure due to RVI, two-dimensional echocardiography is probably highly sensitive and specific for the diagnosis of RVI, by detecting RV wall motion and thickening abnormalities. Due to advantages, such as noninvasivity and repeatibility, two-dimensional echocardiography can be used in the selection of patients who deserve hemodynamic monitoring and in follow-up studies.     

Diagnosis of ischemic right ventricular dysfunction by M-mode echocardiography

The importance of right ventricular infarction depends uponthe presence and severity of the resultant ischemic right ventriculardysfunction. The usefulness of M-mode echocardiography for thediagnosis of ischemic right ventricular dysfunction was investigatedin 63 patients with acute myocardial infarction. Group A included35 patients with ischemic right ventricular dysfunction, andGroup B 28 patients without ischemic right ventricular dysfunction.The diagnosis of ischemic right ventricular dysfunction wasmade when the right atrial pressure was higher than 10 mm Hgand greater than, equal to or not more than 5 mm Hg less thanpulmonary capillary pressure. Right ventricular diastolic dimension was greater in Group Athan in Group B (P<0.01). The upper 95% confidence limitof this parameter was found to be 18 mm/m² in Group B. The rightventricular/left ventricular diastolic dimension ratio was greaterin Group A than in Group B (P <0.01). The upper limit ofthis ratio in Group B was 0.63. When either of these two criteria was present (right ventriculardiastolic dimension 18 mm/m² or right ventricular/left ventriculardiastolic dimension0.63), the diagnosis of ischemic right ventriculardysfunction could be made in 19 of 35 patients (sensitivity54.2%), with no false positives (specificity 100%). The interventricular septum was rapidly displaced upwards atthe beginning of systole and downwards during diastole in eightpatients from Group A, and in no patient from Group B. M-mode echocardiographic indices of right ventricular dilatationmay be of value in the diagnosis of ischemic right ventriculardysfunction, but their sensitivity is low. The discrepancy betweenhemodynamic and M-mode echocardiographic parameters of ischemicright ventricular dysfunction may be attributed to impairmentof right ventricular compliance, with a right ventricular diastoledimension/right atrial pressure ratio greater in Group B thanin Group A (P< 0.01).     

Right ventricular abnormalities in ventricular tachycardia of right ventricular origin: relation to electrophysiological abnormalities.

Patients with right ventricular tachycardia may have adverse electrophysiological abnormalities linked to disturbed right ventricular structure. Seventeen patients who presented with right ventricular tachycardia without coronary artery disease or gross abnormalities of left ventricular function were studied. Patients had the ventricular tachycardia characterised at electrophysiological study and most underwent radionuclide and contrast angiography. At echocardiography specific attention was paid to the right ventricular chamber size. Two groups were identified at echocardiographic study. In group 1, nine patients had normal left ventricular dimensions and relatively normal features at electrophysiological study. Mean right ventricular ejection fraction was 0.45 by krypton-81 measurement. Group 2 comprised eight patients who had dilatation of right ventricular inflow tract, outflow tract, and right ventricular body. This group had more severe features at presentation and at electrophysiological study. In this group all available echocardiographic measurements of right ventricular chamber size were greater than those of group 1 and outside the normal range. Four of the eight patients in group 2 showed regional right ventricular dyskinesia at echocardiography. Mean right ventricular ejection fraction (0.23) in group 2 was significantly lower than in group 1. One patient in group 2 subsequently died. In patients with right ventricular tachycardia, those with less favourable prognostic features at electrophysiological study may have distinct abnormalities of right ventricular structure that can be identified at echocardiographic study. Echocardiography may be of value in the recognition of potentially malignant clinical and electrophysiological features in this group.