Hypertrophic cardiomyopathy with mid-ventricular obstruction complicated with apical left ventricular aneurysm and ventricular tachycardia: a case report

A 69-year-old man was admitted with palpitations and syncope. His medical history included hypertension and left ventricular hypertrophy. Arterial pulsation was not palpable on admission. Electrocardiography revealed ventricular tachycardia, and cardioversion restored normal sinus rhythm. An electrophysiological study reproducibly induced polymorphic ventricular tachycardia, so a cardioverter defibrillator was implanted. Echocardiography revealed mid-ventricular obstruction and an apical aneurysm, and Doppler color flow imaging showed a diastolic paradoxic jet from the apex toward the base. Coronary angiography showed no stenosis of the extramural coronary arteries. Ventricular tachycardia on admission showed a right bundle branch block pattern and a superior axis deviation, so the arrhythmia was thought to originate from the apical aneurysm. Apical aneurysm can result from elevated intraventricular pressure or relative myocardial ischemia. This is a rare case of hypertrophic cardiomyopathy with mid-ventricular obstruction complicated with apical aneurysm and polymorphic ventricular tachycardia.     

Electro-vectorcardiographic study of ventricular extrasystole in arrhythmogenic dysplasia of the right ventricle]

The morphology of ventricular extrasystole (VES) in 46 cases of arrhythmogenic dysplasia of the right ventricle (ADRV) was correlated with the point of origin located by intracavitary mapping. The cases concerned 41 of left bundle-branch block (LBB) with various axes on the frontal plane (FP), 4 of right bundle-branch block (RBB), and 5 of atypical morphology (frontal plane shifted inferiorly and increased R from V1 to V6; on the horizontal plane, clockwise rotation of the loop oriented anteriorly and leftward). There is a good correlation with the site of origin: VESs which were LBB in appearance originated in the right ventricle (apex, septum, infundibulum); VESs which were RBB in appearance originated in the apex of the left ventricle, while the atypical VESs started in the upper posterior septum. A study of morphology may therefore also give an indication of the location of the disease.     

36例致心律失常性右室心肌病的心电图特征和临床观察

目的探讨致心律失常性右室心肌病(ARVC)的心电图特征和临床表现。方法回顾分析符合欧洲心脏病协会ARVC诊断标准的36例患者的心电图参数、临床表现、超声心动图、腔内电生理检查等临床资料。结果36例中男26例、女10例,年龄37±13岁;33例表现为心悸、胸闷,11例同时伴有晕厥,2例有家族性猝死史。心电图研究发现10例(28%)出现Epsilon波,29例(81%)右胸(V1～V3)导联QRS波时限≥110ms;在29例无右束支传导阻滞的患者中,右胸导联分别有16例(55%)出现T波倒置、18例(62%)出现S波升支时间≥55ms;17例(47%)QRSd1/QRSd2(V1～V3导联与V4～V6导联QRS波时间平均值之比)≥1.2;24例(67%)出现室壁阻滞;27例(75%)记录到持续性或非持续性室性心动过速。29例超声心动图表现为严重的右室受累。25例行腔内电生理检查,20例诱发出右室起源的室性心动过速,即刻射频消融成功11例。结论ARVC好发于青年男性,是引起晕厥、室性心律失常和室壁运动异常的重要原因,Epsilon波、右胸导联QRS波时限≥110ms与T波倒置、右室起源的室性心律失常为其特征性的心电图改变,QRSd1/QRSd2≥1.2、室壁阻滞、右胸导联S波升支时间≥55ms有助于该病的诊断,经导管射频消融治疗室性心动过速成功率低。     

Risk stratification of sudden cardiac death and malignant ventricular arrhythmias in right ventricular dysplasia-cardiomyopathy

Arrhythmogenic right ventricular dysplasia-cardiomyopathy is in most cases a benign cause of ventricular arrhythmias in young patients. The major reason of mortality is sudden arrhythmic death with an annual rate of 2-3% as the first manifestation of the disease in most cases. Little is known about risk factors of sudden arrhythmic death so far. The purpose of the retrospective study was to classify risk factors from invasive and non-invasive examinations. METHODS: In a cohort of 121 consecutive patients sampled from 1986 to 1998 the value of right ventricular dilatation, left ventricular involvement analysed by angiocardiography or echocardiography and standard ECG parameters such as precordial T wave inversions, right precordial ST elevation, precordial QRS dispersion, left precordial JT interval prolongation and complete right bundle branch block were determined. The whole cohort of patients were divided into two groups with high arrhythmic risk (aborted or non-aborted sudden death, recurrent ventricular tachycardia despite medical treatment, recurrent syncopes) and low risk (frequent ventricular premature beats, non sustained ventricular tachycardia, uneventful course under medical therapy). RESULTS: From angiocardiography or echocardiography in a quantitative approach right ventricular dilatation (p<0.0001) and additional left ventricular abnormalities (p<0.0001) could be identified as major risk factors. From an ECG point of view increased precordial QRS dispersion > or =50 ms (p<0.01) with complete right bundle branch block and right ventricular dilatation in most cases and precordial T wave inversions beyond V3 (p<0.0001) and the phenomenon of left precordial JT interval prolongation (JT dispersion > or =30 ms) in cases of additional left ventricular abnormalities represented non-invasive predictors of recurrent arrhythmic events. Right precordial ST segment elevation could be excluded as risk factor of sudden arrhythmic death. CONCLUSIONS: Right ventricular dilatation with ECG depolarisation abnormalities and additional left ventricular involvement with striking ECG repolarisation abnormalities could be identified as strong risk factors of recurrent arrhythmic events in ARVD with unfavorable prognosis.     

An adult case of arrhythmogenic right ventricular dysplasia

A forty-year-old man was admitted to our hospital because of dizziness, palpitations and an oppressive feeling in the precordium. Physical examination was normal. A chest roentgenogram revealed mild cardiomegaly and the electrocardiogram showed ventricular tachycardia of a left bundle branch block configuration which was terminated by the intravenous injection of procainamide. During sinus rhythm the electrocardiogram showed incomplete right bundle branch block, PQ prolongation and inverted T waves in leads V1 through V3. Two-dimensional echocardiography revealed only moderate right ventricular dilatation. Right ventricular angiography showed severe right ventricular dilatation and hypokinesis of the right ventricular apex and pulmonary artery infundibulum. From these characteristics signs we concluded that this adult patient had arrhythmogenic right ventricular dysplasia (ARVD), suggesting that this condition is not confined to children but may occur in adults as well.     

Ventricular tachycardia of left bundle branch block configuration in patients with isolated right ventricular dilatation. Clinical and electrophysiological features.

Electrophysiological studies showed ventricular tachycardia in five patients (four male, one female) with isolated right ventricular dilatation. All had been asymptomatic before the onset of palpitation which had developed in adolescence or early adult life. Tachycardia had been associated with syncope in four patients, and three had been resuscitated from ventricular fibrillation before investigation. The electrocardiogram during ventricular tachycardia showed a left bundle branch block pattern, and endocardial mapping at electrophysiological study confirmed the right ventricular origin. The presenting tachycardia could be induced in all patients by programmed stimulation, and in three patients ventricular tachycardia of differing configuration could be induced, but the right ventricular origin and left bundle branch block pattern were maintained. In two patients ventricular tachycardia degenerated into ventricular fibrillation. Cineangiography, cross sectional echocardiography, and multigated radionuclide angiography confirmed the dilated abnormal right ventricle while indicating that left ventricular function was normal. On resting electrocardiograms T wave inversion over the right precordial leads was the sole abnormality. There were no signs of right heart failure and exercise tolerance was normal. Four patients have received maintenance treatment with antiarrhythmic drugs, and one had undergone operative mapping and ablative surgery. Thus ventricular tachycardia complicating right ventricular dilatation may be associated with serious symptoms and ventricular electrical instability; and in adults it may be suspected on clinical grounds by inverted T waves in the right precordial leads.     

Ventricular tachycardia of left bundle branch block configuration in patients with isolated right ventricular dilatation. Clinical and electrophysiological features

Electrophysiological studies showed ventricular tachycardia in five patients (four male, one female) with isolated right ventricular dilatation. All had been asymptomatic before the onset of palpitation which had developed in adolescence or early adult life. Tachycardia had been associated with syncope in four patients, and three had been resuscitated from ventricular fibrillation before investigation. The electrocardiogram during ventricular tachycardia showed a left bundle branch block pattern, and endocardial mapping at electrophysiological study confirmed the right ventricular origin. The presenting tachycardia could be induced in all patients by programmed stimulation, and in three patients ventricular tachycardia of differing configuration could be induced, but the right ventricular origin and left bundle branch block pattern were maintained. In two patients ventricular tachycardia degenerated into ventricular fibrillation. Cineangiography, cross sectional echocardiography, and multigated radionuclide angiography confirmed the dilated abnormal right ventricle while indicating that left ventricular function was normal. On resting electrocardiograms T wave inversion over the right precordial leads was the sole abnormality. There were no signs of right heart failure and exercise tolerance was normal. Four patients have received maintenance treatment with antiarrhythmic drugs, and one had undergone operative mapping and ablative surgery. Thus ventricular tachycardia complicating right ventricular dilatation may be associated with serious symptoms and ventricular electrical instability; and in adults it may be suspected on clinical grounds by inverted T waves in the right precordial leads.     

A case of anomalous origin of right coronary artery from the left sinus of Valsalva with ventricular aneurysm ventricular tachycardia and paroxysmal A-V block

A 51-year old man was admitted to our hospital, because of syncope. During admission, he had three episodes of syncopal attack. During the episodes, monitor ECG showed two times of ventricular tachycardia and one of paroxysmal A-V block. The left ventriculogram showed dilatation of left ventricle with posterobasal aneurysm, anterobasal and apical hypokinesis. The left coronary artery was normal. The right coronary artery originated from the left sinus of Valsalva and passed through between aortic root and pulmonary trunks. There was no atherosclerotic lesions in both coronary arteries. Non-sustained ventricular tachycardia was induced by triple premature stimulations. The inverse relation between the coupling interval of premature stimulation and the echo interval was recognized. Lidocaine (50 mg IV), Flecainide (300 mg/day), Mexiletine (450 mg/day), and Aprindine (60 mg/day) prevented ventricular tachycardia. Rapid atrial pacing induced paroxysmal A-V block. Permanent pacemaker was implanted because of syncope due to paroxysmal A-V block and ventricular tachycardia was prevented by Aprindine. Recently, the case with anomalous origin of the coronary artery increased by the popularity of coronary angiography. But, this case considered to be rare because of complication with ventricular aneurysm and lethal arrhythmia (ventricular tachycardia and paroxysmal A-V block).     

Is there an overlap between Brugada syndrome and arrhythmogenic right ventricular cardiomyopathy/dysplasia?

The Brugada syndrome is a congenital syndrome displaying an autosomal dominant mode of transmission in patients with a structurally normal heart. The disease has been linked to mutations in SCN5A , a gene located on the short arm of chromosome 3 (p21-24) that encodes for the alpha subunit of the sodium channel. The syndrome is characterized by a dynamic ST-segment elevation (accentuated J wave) in leads V 1 to V 3 of the ECG followed by negative T wave. Right bundle-branch block of varying degrees is observed in some patients. The syndrome is associated with syncope and a relatively high incidence of sudden cardiac death secondary to the development of polymorphic ventricular tachycardia that may degenerate into ventricular fibrillation. An acquired form of the Brugada syndrome is also recognized, caused by a wide variety of drugs and conditions that alter the balance of currents active during the early phases of the action potential. Among patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia, there is a subpopulation with a clinical and electrocardiographic pattern similar to that of the Brugada syndrome. These cases of arrhythmogenic right ventricular cardiomyopathy/dysplasia are thought to represent an early or concealed form of the disease. This review examines the overlap between these 2 syndromes.     

Familial dilated cardiomyopathy complicated by left ventricular aneurysm

Two siblings presented with symptoms of left ventricular dysfunction and ventricular arrhythmias. Echocardiography and left ventriculography revealed dilatation, diffuse hypokinesis and apical aneurysm of the left ventricle in both cases. Myocardial infarction was unlikely by history and examinations. We diagnosed them as cases of familial dilated cardiomyopathy complicated by left ventricular aneurysm.     

Right ventricular dysplasia: a clinical and pathological study of two families with left ventricular involvement.

BACKGROUND--Right ventricular dysplasia is a heart muscle disease of unknown cause that is often familial and is anatomically characterised by adipose or fibroadipose infiltration of the right ventricular myocardium. It is generally regarded as a selective disorder of the right ventricle. AIM--To investigate the prevalence and characteristics of left ventricular involvement in two families in which at least one member had right ventricular dysplasia confirmed at necropsy. METHODS AND RESULTS--Eight patients were found to be affected by right ventricular dysplasia. In three of them this was confirmed at necropsy. Echocardiography or angiography or both showed left ventricular involvement in seven. This ranged from localised wall motion abnormalities to moderate or severe left ventricular dysfunction. The disease was progressive in four cases. At necropsy the left ventricular myocardium showed predominant fibrosis and degenerative changes of the myocardial cells. There were areas of myocardial thinning with fatty infiltration at the apex in two patients. CONCLUSIONS--Familial right ventricular dysplasia can be a progressive disorder that affects the left ventricle. Advanced disease may be clinically confused with dilated cardiomyopathy.     

2 cases of arrhythmogenic dysplasia of the right ventricle of familial occurrence

Cases of familial arrhythmogenic right ventricular dysplasia (ARVD) have been reported by many authors, and a genetic mechanism of transmission has been hypothesized. Both autosomal dominant and autosomal recessive mechanism of inheritance were suggest. We present a father and a daughter affected by arrhythmogenic right ventricular dysplasia, belonging to a family with many cases of sudden death. Both of them presented with an episode of ventricular tachycardia with left bundle branch block. The clinical diagnosis was made according to electrocardiographic, echocardiographic, angionuclear and hemodynamic criteria of ARVD. The familia analysis suggest an autosomal dominant mechanism of transmission.     

Exercise-induced bidirectional ventricular tachycardia with alternating right and left bundle branch block-type patterns--a case report

Exercise-induced ventricular tachycardia in young adults may occur with various structural heart diseases or with structurally normal heart. The structural heart diseases reported to cause exercise-induced ventricular tachycardia in this patient population include arrhythmogenic right ventricular dysplasia, hypertrophic cardiomyopathy, dilated cardiomyopathy, myocarditis, congenital heart disease, and myocardial ischemia. The conditions well identified to cause exercise-induced ventricular tachycardia with structurally normal heart are congenital long-QT syndrome and familial polymorphic ventricular tachycardia. Exercise-induced ventricular tachycardia may display polymorphic, monomorphic, or bidirectional morphologies. A case of exercise-induced catecholamine-sensitive bidirectional ventricular tachycardia with alternating right and left bundle branch block patterns is reported in a young boy in the absence of structural heart disease, the conditions causing bidirectional ventricular tachycardia, and family history of such an event or sudden cardiac death. The bidirectional tachycardia typically displays right bundle branch block in right precordial leads with alternating polarity of the QRS-complex in frontal plane leads but in this case the bidirectional morphology of tachycardia was caused by alternating right and left bundle branch block-type patterns. The conditions causing bidirectional ventricular tachycardia are digoxin toxicity, ischemic heart disease, hypokalemia, myocarditis, and familial polymorphic ventricular tachycardia syndrome but the exact cause in this patient remained obscure, and the possibility of an underlying electrical or ion channel disease of the heart could not be ruled out.     

Pregnancy and delivery in patient after Fontan's operation due to common ventricle of left ventricular morphology

A 53-year-old male presented to our emergency department with a sudden onset of ventricular tachycardia with left bundle branch block and right axis deviation. After the tachyarrhythmia converting to sinus rhythm, the ECG displayed sinus rhythm with a typical epsilon wave in leads V1 and V2. Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) was suspected. The epsilon wave is the most specific hallmark for the diagnosis of ARVD/C, although it is insensitive. For the management of these patients, antiarrhythmic medications appear to be effective. However, implantable cardioverter defibrillators are the only reliable treatment to prevent sudden cardiac death.     

国人Brugada综合征特点探讨(附三例报道)

目的：探讨国人Brugada综合征的特点。方法 报道3例Brugada综合征，并复习国内文献报道的20例，分析其特点。结果 (1)共23例Brugada综合征中男20例，女3例，发病年龄为9—73岁，平均40岁。(2)临床表现为晕厥14例，其中4例死亡，单纯阵发性心悸、胸闷3例，无症状6例；18个家系中有5个家系有明确或可疑家族史。(3)所有病例心电图Vl—V3导联ST段呈尖峰状和／或马鞍状抬高，大多ST段抬高程度多变，同时15例伴典型右束支传导阻滞，11例发生室速或室颤，其他还可见阵发性房颤、早博、房室传导阻滞、窦房传导阻滞等心律失常。结论：(1)国人Brugada综合征临床和心电图特点与国外文献报道相似。(2)诊断Brugada综合征主要应根据Vl—V3导联ST段尖峰状和／或马鞍状抬高的特征性改变，晕厥及典型右束支传导阻滞并非必需。(3)Vl—V3导联段抬高幅度多变是Brugada综合征的一个重要特点，ST段抬高程度可能与晕厥发作有关。     

Anomalous origin of the right coronary artery from the left coronary sinus: case report and literature review

A 41-year-old man with no relevant cardiac history presented for evaluation of episodic syncope. Electrocardiography showed sinus rhythm with right bundle-branch block and Q waves in leads II, III, and aVF. Subsequent exercise treadmill testing and technetium 99m sestamibi study revealed a reversible posterior defect. Coronary angiography later showed a right coronary artery that arose from the left coronary sinus--an anomaly that has been associated with angina, myocardial infarction, and sudden cardiac death. After being informed of the potential risks and options associated with this anomaly, the patient chose long-term beta-blocker medical therapy. At follow-up, this treatment appeared to have resolved his episodic syncope.     

Significance of asymmetrically inverted T wave

Two consecutive series of patients with a T wave asymmetry ratio of 2.0 or greater have been studied. Patients with bundle-branch block or who were on digoxin or a similar drug were excluded. In 50 of the 69 patients, the heart was examined either by echocardiography or by direct inspection. Sixty-one of the 69 patients had diseases commonly associated with left (or right) ventricular hypertrophy and/or dilatation. The remaining eight patients had clinically pure ischaemic heart disease. Of the 50 hearts examined by echocardiography or direct inspection (including six with pure ischaemic heart disease), 49 were found to have abnormal thickness of the left (or right) ventricle, or increased end-diastolic left ventricular diameter, or a combination of hypertrophy and dilatation. In 12 of the 47 patients with left ventricular hypertrophy or dilatation, the electrocardiogram did not satisfy the Sokolow and Lyon voltage criterion of left ventricular hypertrophy.     

Arrhythmogenic right ventricular dysplasia presenting with ventricular tachycardia in a father and son

A father and his son presented with ventricular tachycardia of left bundle-branch block configuration, two years apart. The patients had no clinical signs of right or left ventricular dysfunction. The diagnosis of arrhythmogenic right ventricular dysplasia was based on right ventricular angiographic and radionuclide findings. Microscopic sections obtained at surgery for the ventricular tachycardia in the father revealed abnormal infiltration of fat and focal fibrosis in the right ventricular myocardium, confirming the diagnosis. The importance of thorough evaluation of right and left ventricular function and structure in patients with ventricular tachycardia of right ventricular origin is emphasized. Currently available diagnostic techniques and management are presented.     

Left bundle-branch block with right axis deviation—a unique aberrancy during supraventricular tachycardia

A tachycardia with left bundle-branch block morphology and right axis deviation points to the diagnosis of ventricular tachycardia. Conversely, any supraventricular tachycardia with left bundle-branch block is typically associated with a normal or leftward QRS axis. We present the case of a 34-year-old man showing atrioventricular nodal reentrant tachycardia with left bundle-branch block/right axis deviation as an exception to this rule.     

Significance of asymmetrically inverted T wave.

Two consecutive series of patients with a T wave asymmetry ratio of 2.0 or greater have been studied. Patients with bundle-branch block or who were on digoxin or a similar drug were excluded. In 50 of the 69 patients, the heart was examined either by echocardiography or by direct inspection. Sixty-one of the 69 patients had diseases commonly associated with left (or right) ventricular hypertrophy and/or dilatation. The remaining eight patients had clinically pure ischaemic heart disease. Of the 50 hearts examined by echocardiography or direct inspection (including six with pure ischaemic heart disease), 49 were found to have abnormal thickness of the left (or right) ventricle, or increased end-diastolic left ventricular diameter, or a combination of hypertrophy and dilatation. In 12 of the 47 patients with left ventricular hypertrophy or dilatation, the electrocardiogram did not satisfy the Sokolow and Lyon voltage criterion of left ventricular hypertrophy.