Arrhythmogenic right ventricular dysplasia(ARVD) is an inherited heart muscle disease.Myocyte apoptosis and fibro-fatty scar tissue predisposes patients to malignant ventricular arrhythmias.Patients may present to var...Arrhythmogenic right ventricular dysplasia(ARVD) is an inherited heart muscle disease.Myocyte apoptosis and fibro-fatty scar tissue predisposes patients to malignant ventricular arrhythmias.Patients may present to variety of surgical procedures with diagnosed ARVD.Surgical insult,catecholamine surge and physiological disturbance can be hazardous on the vulnerable myocardium and may result in life-threatening ventricular tachycardia or sudden cardiac death in the perioperative period.Anaesthetists have particular role in perioperative management of this patient population,meticulous perioperative planning,close haemodynamic monitoring and maintenance of physiological stability throughout helps to avoid devastating perioperative loss.展开更多
Background There are few studies on the clinical profile of Chinese patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy （ARVD/C）. The purpose of this study was to describe the clinical characteri...Background There are few studies on the clinical profile of Chinese patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy （ARVD/C）. The purpose of this study was to describe the clinical characteristics of ARVD/C patients from China, particularly to define the features of electrocardiograph and treatment outcomes. Methods Thirty-nine patients hospitalized in Fu Wai Cardiovascular Hospital from 1998 to 2006 were included. The data were obtained from the medical archive and the follow-up records. Results Of these patients 33 were male and 6 female （age at the first presentation was （34.9 ± 9.8） years）. The most common symptoms were palpitation （62%） and syncope （44%）. Right precordial QRSd 〉 110 ms was detected in 69% of the patients, epsilon wave in 59%, and a ratio of QRSd in V1＋V2＋V3/V4＋V5＋V6 ≥ 1.2 in 82%. The most frequent features of electrocardiogram in patients without right bundle-branch block were T-wave inversions and S-wave upstroke in V1-V3 〉55 ms （96% and 90% of 28 patients, respectively）. Radiofrequency catheter ablation （RFCA） for ventricular tachycardia （VT） was successful in 15 （68%） of 22 patients. The recurrence rate of VT was 46% （7/15） during the follow-up of （16.7 ± 11.2） months. Seven patients had cardioverter/defibrillator （ICD） implanted plus drug therapy and 17 patients took antiarrhythmic drugs alone. During the follow-up of （35.6 ± 19.0） months, all patients with ICD implanted received at least one appropriate ICD shock. One patient died of ventricular fibrillation suddenly and one patient underwent heart transplantation for progressive biventricular heart failure during the drug therapy alone. Conclusions This study demonstrated the clinical and ECG features of the 39 ARVD/C Chinese patients. ICD provided life-saving protection by effectively terminating malignant arrhythmias, and the high recurrence of VT was the major problem of RFCA therapy.展开更多
Arrhythmogenic ventricular cardiomyopathy(AVC) isgenerally referred to as arrhythmogenic right ventricu-lar(RV) cardiomyopathy/dysplasia and constitutesan inherited cardiomyopathy.Affected patients maysuccumb to sudde...Arrhythmogenic ventricular cardiomyopathy(AVC) isgenerally referred to as arrhythmogenic right ventricu-lar(RV) cardiomyopathy/dysplasia and constitutesan inherited cardiomyopathy.Affected patients maysuccumb to sudden cardiac death(SCD),ventriculartachyarrhythmias(VTA) and heart failure.Geneticstudies have identified causative mutations in genesencoding proteins of the intercalated disk that lead toreduced myocardial electro-mechanical stability.Theterm arrhythmogenic RV cardiomyopathy is somewhatmisleading as biventricular involvement or isolated leftventricular(LV) involvement may be present and thus abroader term such as AVC should be preferred.The di-agnosis is established on a point score basis accordingto the revised 2010 task force criteria utilizing imagingmodalities,demonstrating fibrous replacement throughbiopsy,electrocardiographic abnormalities,ventricu-lar arrhythmias and a positive family history includingidentification of genetic mutations.Although severarisk factors for SCD such as previous cardiac arrest,syncope,documented VTA,severe RV/LV dysfunctionand young age at manifestation have been identified,risk stratification still needs improvement,especially inasymptomatic family members.Particularly,the roleof genetic testing and environmental factors has to befurther elucidated.Therapeutic interventions include re-striction from physical exercise,beta-blockers,sotalol,amiodarone,implantable cardioverter-defibrillators andcatheter ablation.Life-long follow-up is warranted insymptomatic patients,but also asymptomatic carriersof pathogenic mutations.展开更多
“Arrhythmogenic right ventricular dysplasia” (ARVD), a heart muscle disorder characterized by the presence of fibro-fatty tissue and ventricular electrical vulnerability related to sudden death, was first described ...“Arrhythmogenic right ventricular dysplasia” (ARVD), a heart muscle disorder characterized by the presence of fibro-fatty tissue and ventricular electrical vulnerability related to sudden death, was first described in 1977 by a French team. Since then, other terms such as “arrhythmogenic right ventricular cardiomyopathy” (ARVC), “arrhythmogenic cardiomyopathy” (AC), “left-dominant arrhythmogenic cardiomyopathy” (LDAC), and “arrhythmogenic left ventricular dysplasia” (ALVD) have been introduced. These changes in nomenclature of the same disease entity are based on different explanations of pathomorphologic patterns. The dysplasia theory claims cardiac growth “maldevelopment” whereas the cardiomyopathy has been seen as an atrophy from acquired injury (myocyte death) and repair (fibrofatty replacement). The other area of divergent opinion is with regards to involvement of both ventricles rather than being an isolated right ventricular anomaly that may result in increased likelihood of diagnosing the concealed form manifesting with pre-dominant left ventricular arrhythmias. Multiple line of evidences support common disease path-ways: Presence of fibro-fatty and superimposed myocarditis, desmosome mutations and malfunc-tion. These compelling data regarding the heart growth, and pathological, clinical, phenotype/ genotype correlates have advanced our understanding of arrhythmogenic ventricular dysplasia/ cardiomyopathy and increased the diagnostic accuracy as well as providing an avenue for future development of new mechanism-based therapies.展开更多
Cardiovascular disease is an important contributor to maternal mortality in both developing and developed countries.Systematic search for cardiac disease is usually not performed during pregnancy despite hypertensive ...Cardiovascular disease is an important contributor to maternal mortality in both developing and developed countries.Systematic search for cardiac disease is usually not performed during pregnancy despite hypertensive disease,undiagnosed pulmonary hypertension and cardiomyopathies being recognized as major health problems in these settings.This article reported a 27-year-old female who was normal on clinical examination and basic investigations,and on an antenatal visit was found collapsed in the toilet of her house and was pronounced dead on admission to hospital.She was found to be in the 11th week of pregnancy and had no history of significant illness in the past.Autopsy did not reveal any obvious macroscopic pathology except for a significant amount of epicardial fat infiltrating into myocardium of right ventricle.Detailed histopathological examination of the heart demonstrated fibro-fatty replacement of the heart muscle.The cause of death was arrhythmogenic right ventricular cardiomyopathy/dysplasia(ARVC/D).ARVC/D can cause unexpected sudden death during pregnancy.Therefore,it is recommended that an ECG and echocardiogram be included as screening tests during antenatal follow-up to minimize preventable cardiac deaths like ARVC/D.展开更多
常染色体遗传性疾病——致心律失常性右心室心肌病（arrhythmogenic right ventricular cardiomyopathy,ARVC）主要累及右心室,其部分心肌被脂肪或纤维脂肪替代并且存在右心室心肌电位不稳定.而新近发现一些起源于左心室的室性心律失常...常染色体遗传性疾病——致心律失常性右心室心肌病（arrhythmogenic right ventricular cardiomyopathy,ARVC）主要累及右心室,其部分心肌被脂肪或纤维脂肪替代并且存在右心室心肌电位不稳定.而新近发现一些起源于左心室的室性心律失常而发生猝死的病例,右心室形态正常而病变仅累及左心室,不符合心肌缺血及炎性疾病的改变,可能是与ARVC相似但不同于已认识的ARVC,是一种新的遗传性疾病——致心律失常性左心室心肌病（arrhythmogenic left ventricular cardiomyopathy,ALVC）,本文对此作一综述.展开更多
目的探讨致心律失常性右室心肌病／发育不良（ARVC／D，arrythmogenic right ventricular cardiomyopathy／dysplasia）多参数MRI影像特征及诊断价值。方法收集我院2007年1月至2015年5月间5例ARVC／D的MRI资料，5例患者均符合2010年修订...目的探讨致心律失常性右室心肌病／发育不良（ARVC／D，arrythmogenic right ventricular cardiomyopathy／dysplasia）多参数MRI影像特征及诊断价值。方法收集我院2007年1月至2015年5月间5例ARVC／D的MRI资料，5例患者均符合2010年修订的ARVC诊断标准，MRI检查包括心脏形态、电影及延迟心肌活性成像。结果形态及电影MRI示ARVC／D右室心腔明显扩张及右室流出道扩大，室壁运动减低，3例右室肌壁及外膜缘见不均匀高信号，脂肪抑制后呈低信号，提示心肌脂肪侵润，2例右室心肌未见确切脂肪侵润表现；5例左心室室壁运动及EF值未见正常；延迟MR心肌活性成像（Deiayed enhcaning MRI，DE—MRI）显示右室肌壁变薄、肌壁呈弥漫性高信号，提示心肌纤维化；4例左室肌壁DE-MRI心肌活性未见异常，1例左室侧壁小灶纤维瘢痕，但瘢痕位于肌壁间、且无冠脉供血区分布表现。结论心脏MR形态及电影图像上右室心腔明显扩张和流出道的扩大、室壁运动的减低对ARVC／D诊断有明确的价值，结合DEMRI有助于判断右室心肌纤维化及其程度，但MRI检测的脂肪侵润对ARVC／D诊断的敏感性不高。此外，DE—MRI同时可检测左室肌壁有无纤维瘢痕形成及其范围与部位，有助于ARVC／D预后的判断。展开更多
文摘Arrhythmogenic right ventricular dysplasia(ARVD) is an inherited heart muscle disease.Myocyte apoptosis and fibro-fatty scar tissue predisposes patients to malignant ventricular arrhythmias.Patients may present to variety of surgical procedures with diagnosed ARVD.Surgical insult,catecholamine surge and physiological disturbance can be hazardous on the vulnerable myocardium and may result in life-threatening ventricular tachycardia or sudden cardiac death in the perioperative period.Anaesthetists have particular role in perioperative management of this patient population,meticulous perioperative planning,close haemodynamic monitoring and maintenance of physiological stability throughout helps to avoid devastating perioperative loss.
文摘Background There are few studies on the clinical profile of Chinese patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy （ARVD/C）. The purpose of this study was to describe the clinical characteristics of ARVD/C patients from China, particularly to define the features of electrocardiograph and treatment outcomes. Methods Thirty-nine patients hospitalized in Fu Wai Cardiovascular Hospital from 1998 to 2006 were included. The data were obtained from the medical archive and the follow-up records. Results Of these patients 33 were male and 6 female （age at the first presentation was （34.9 ± 9.8） years）. The most common symptoms were palpitation （62%） and syncope （44%）. Right precordial QRSd 〉 110 ms was detected in 69% of the patients, epsilon wave in 59%, and a ratio of QRSd in V1＋V2＋V3/V4＋V5＋V6 ≥ 1.2 in 82%. The most frequent features of electrocardiogram in patients without right bundle-branch block were T-wave inversions and S-wave upstroke in V1-V3 〉55 ms （96% and 90% of 28 patients, respectively）. Radiofrequency catheter ablation （RFCA） for ventricular tachycardia （VT） was successful in 15 （68%） of 22 patients. The recurrence rate of VT was 46% （7/15） during the follow-up of （16.7 ± 11.2） months. Seven patients had cardioverter/defibrillator （ICD） implanted plus drug therapy and 17 patients took antiarrhythmic drugs alone. During the follow-up of （35.6 ± 19.0） months, all patients with ICD implanted received at least one appropriate ICD shock. One patient died of ventricular fibrillation suddenly and one patient underwent heart transplantation for progressive biventricular heart failure during the drug therapy alone. Conclusions This study demonstrated the clinical and ECG features of the 39 ARVD/C Chinese patients. ICD provided life-saving protection by effectively terminating malignant arrhythmias, and the high recurrence of VT was the major problem of RFCA therapy.
基金Supported by The Georg and Bertha Schwyzer-Winiker Foundation,Zurich,Switzerland
文摘Arrhythmogenic ventricular cardiomyopathy(AVC) isgenerally referred to as arrhythmogenic right ventricu-lar(RV) cardiomyopathy/dysplasia and constitutesan inherited cardiomyopathy.Affected patients maysuccumb to sudden cardiac death(SCD),ventriculartachyarrhythmias(VTA) and heart failure.Geneticstudies have identified causative mutations in genesencoding proteins of the intercalated disk that lead toreduced myocardial electro-mechanical stability.Theterm arrhythmogenic RV cardiomyopathy is somewhatmisleading as biventricular involvement or isolated leftventricular(LV) involvement may be present and thus abroader term such as AVC should be preferred.The di-agnosis is established on a point score basis accordingto the revised 2010 task force criteria utilizing imagingmodalities,demonstrating fibrous replacement throughbiopsy,electrocardiographic abnormalities,ventricu-lar arrhythmias and a positive family history includingidentification of genetic mutations.Although severarisk factors for SCD such as previous cardiac arrest,syncope,documented VTA,severe RV/LV dysfunctionand young age at manifestation have been identified,risk stratification still needs improvement,especially inasymptomatic family members.Particularly,the roleof genetic testing and environmental factors has to befurther elucidated.Therapeutic interventions include re-striction from physical exercise,beta-blockers,sotalol,amiodarone,implantable cardioverter-defibrillators andcatheter ablation.Life-long follow-up is warranted insymptomatic patients,but also asymptomatic carriersof pathogenic mutations.
文摘“Arrhythmogenic right ventricular dysplasia” (ARVD), a heart muscle disorder characterized by the presence of fibro-fatty tissue and ventricular electrical vulnerability related to sudden death, was first described in 1977 by a French team. Since then, other terms such as “arrhythmogenic right ventricular cardiomyopathy” (ARVC), “arrhythmogenic cardiomyopathy” (AC), “left-dominant arrhythmogenic cardiomyopathy” (LDAC), and “arrhythmogenic left ventricular dysplasia” (ALVD) have been introduced. These changes in nomenclature of the same disease entity are based on different explanations of pathomorphologic patterns. The dysplasia theory claims cardiac growth “maldevelopment” whereas the cardiomyopathy has been seen as an atrophy from acquired injury (myocyte death) and repair (fibrofatty replacement). The other area of divergent opinion is with regards to involvement of both ventricles rather than being an isolated right ventricular anomaly that may result in increased likelihood of diagnosing the concealed form manifesting with pre-dominant left ventricular arrhythmias. Multiple line of evidences support common disease path-ways: Presence of fibro-fatty and superimposed myocarditis, desmosome mutations and malfunc-tion. These compelling data regarding the heart growth, and pathological, clinical, phenotype/ genotype correlates have advanced our understanding of arrhythmogenic ventricular dysplasia/ cardiomyopathy and increased the diagnostic accuracy as well as providing an avenue for future development of new mechanism-based therapies.
文摘Cardiovascular disease is an important contributor to maternal mortality in both developing and developed countries.Systematic search for cardiac disease is usually not performed during pregnancy despite hypertensive disease,undiagnosed pulmonary hypertension and cardiomyopathies being recognized as major health problems in these settings.This article reported a 27-year-old female who was normal on clinical examination and basic investigations,and on an antenatal visit was found collapsed in the toilet of her house and was pronounced dead on admission to hospital.She was found to be in the 11th week of pregnancy and had no history of significant illness in the past.Autopsy did not reveal any obvious macroscopic pathology except for a significant amount of epicardial fat infiltrating into myocardium of right ventricle.Detailed histopathological examination of the heart demonstrated fibro-fatty replacement of the heart muscle.The cause of death was arrhythmogenic right ventricular cardiomyopathy/dysplasia(ARVC/D).ARVC/D can cause unexpected sudden death during pregnancy.Therefore,it is recommended that an ECG and echocardiogram be included as screening tests during antenatal follow-up to minimize preventable cardiac deaths like ARVC/D.
文摘常染色体遗传性疾病——致心律失常性右心室心肌病（arrhythmogenic right ventricular cardiomyopathy,ARVC）主要累及右心室,其部分心肌被脂肪或纤维脂肪替代并且存在右心室心肌电位不稳定.而新近发现一些起源于左心室的室性心律失常而发生猝死的病例,右心室形态正常而病变仅累及左心室,不符合心肌缺血及炎性疾病的改变,可能是与ARVC相似但不同于已认识的ARVC,是一种新的遗传性疾病——致心律失常性左心室心肌病（arrhythmogenic left ventricular cardiomyopathy,ALVC）,本文对此作一综述.
