Arrhythmogenic right ventricular dysplasia]

Arrhythmogenic right ventricular dysplasia is a cardiomyopathy with autopsy evidence of fibrous and fatty infiltration of the right ventricular. The disease, which shows familial clustering, causes electrical instability that may place affected subjects at risk of sudden death. It is characterized by ECG changes and right ventricular dysfunction. Death can occur due to ventricular arrhythmia. We describe a 46-year-old female Cypriot who presented with recurrent syncope and palpitation due to ventricular tachycardia with the LBBB pattern; T waves were inverted in V1-V3. Echocardiogram and cardiac catheterization revealed severe right ventricular dysfunction. Her sister had died several years before of a similar syndrome, including ventricular tachycardia. Family members should be screened for right ventricular dysplasia. Those currently asymptomatic should be given prophylactic therapy to prevent sudden death. Treatment is either medical: sotalol, beta-blockers or verapamil; or surgical: ablation of the arrhythmogenic focus in the right ventricle, total disconnection of the right ventricular free wall; or implantation of a cardioverter defibrillator.     

Right precordial leads and sudden death. Relation with arrhythmogenic right ventricular dysplasia

Non-coronary ST-segment elevation during right sided chest pain has been described in subjects with episodes of ventricular fibrillation at rest. This syndrome has been attributed to functional phenomena or to structural myocardial changes. A personal case has features belonging to two categories: ST-segment elevation observed before, during and after episodes of arrhythmia was compared to 11 previously recorded ECG recordings. Right ventricular dysplasia was shown by electrocardiography, electrophysiology and echocardiography. In addition, ST-segment elevation is classified in 3 categories: triangular and dome-shaped are the most commonly observed forms during the arrhythmias: the third form with "saddle"-shaped appearances has not been previously described and would seem to be a minor equivalent observed during intercritical periods. This form is found in 30% of clinically documented cases of arrhythmogenic right ventricular dysplasia.     

Arrhythmogenic marker for the sudden unexplained death syndrome in Thai men

BACKGROUND: Between 1981 and 1988, the Centers for Disease Control and Prevention reported a very high incidence of sudden death among young male Southeast Asians who died unexpectedly during sleep. The pattern of death has long been prevalent in Southeast Asia. We carried out a study to identify the clinical markers for patients at high risk of developing sudden unexplained death syndrome (SUDS) and long-term outcomes. METHODS AND RESULTS: We studied 27 Thai men (mean age, 39.7+/-11 years) referred because they had cardiac arrest due to ventricular fibrillation, usually occurring at night while asleep (n=17), or were suspected to have had symptoms similar to the clinical presentation of SUDS (n=10). We performed cardiac testing, including EPS and cardiac catheterization. The patients were then followed at approximately 3-month intervals; our primary end points were death, ventricular fibrillation, or cardiac arrest. A distinct ECG abnormality divided our patients who had no structural heart disease (except 3 patients with mild left ventricular hypertrophy) into two groups: group 1 (n=16) patients had right bundle-branch block and ST-segment elevation in V1 through V3, and group 2 (n=11) had a normal ECG. Group 1 patients had well-defined electrophysiological abnormalities: group 1 had an abnormally prolonged His-Purkinje conduction time (HV interval, 63+/-11 versus 49+/-6 ms; P=.007). Group 1 had a higher incidence of inducible ventricular fibrillation (93% for group 1 versus 11% for group 2; P=.0002) and a positive signal-averaged ECG (92% for group 1 versus 11% for group 2; P=.002), which was associated with a higher incidence of ventricular fibrillation or death (P=.047). The life-table analysis showed that the group 1 patients had a much greater risk of dying suddenly (P=.05). CONCLUSIONS: Right bundle-branch block and precordial injury pattern in V1 through V3 is common in SUDS patients and represents an arrhythmogenic marker that identifies patients who face an inordinate risk of ventricular fibrillation or sudden death.     

Intramural coronary artery dysplasia of the ventricular septum and sudden death

We report four cases of sudden unexpected death in three males and one female aged 12 to 31 years. Death occurred during exercise in three of four cases, and there was no history of sudden death or previous cardiac history in any patient. At autopsy, there was marked intramural coronary artery dysplasia of the ventricular septum, accompanied in three of the four cases by myocardial fibrosis. The arterial dysplasia was characterized by severe medial thickening with smooth muscle cell disorganization and marked luminal narrowing. There was no evidence of myofiber disarray or asymmetric septal hypertrophy to suggest hypertrophic cardiomyopathy. Other than an ostium secundum type atrial septal defect in one case, there were no associated cardiac or extracardiac lesions found at complete autopsy of these individuals. We conclude that small vessel disease of intramural coronary arteries of the ventricular septum may be an isolated finding leading to sudden cardiac death in young adults.     

Renaissance of beta blocker therapy in myocardial infarct

Beta-adrenergic blocking drugs have been evaluated for the treatment of arrhythmias and for the prevention of sudden cardia death, particularly in post-myocardial infarction patients. Betablockers have been demonstrated to reduce mortality, reinfarction, ventricular fibrillation and cardiac rupture in acute infarction. Therefore, in patients with suspected myocardial infarction and without contraindications, treatment with betablockers should be initiated early and continued for at least 2 years. Side-effects are mild and occur in approximately 10% of patients. Patients who have contraindications for betablockers use early in myocardial infarction should be reevaluated before discharge from the hospital and considered for such therapy. Because betablockers prevent some of the adverse arrhythmogenic mechanisms seen in chronic heart failure, it may be reasonable to expect that these drugs could have a role in preventing sudden cardiac death in these patients. Analysis of some of the betablocker post-infarction trials indicate that betablockers reduced the risk of sudden death in patients with heart failure at baseline. Some studies demonstrated also the symptomatic improvement following therapy with betablockers in patients with heart failure. But the currently available data are too limited to provide conclusive information.     

Therapy of ventricular tachyarrhythmia with implantable cardioverters/defibrillators--mortality and complications using epicardial electrodes

Technical improvements of third generation implantable cardioverter defibrillators (ICD) like antitachycardia pacing modalities lead to an extended use of ICDs, not only in patients with aborted sudden cardiac death, but also in patients with hemodynamically tolerable ventricular tachycardia. In addition, anticipated results of current prospective studies might indicate the prophylactic use of ICDs in patients with high risk for sudden cardiac death, but without documented ventricular tachyarrhythmias. This report reviews mortality and complications associated with the ICDs with epicardial defibrillation leads. Mortality is separated in cardiac death, sudden cardiac, arrhythmogenic "not so sudden" cardiac death, and overall mortality. Pulmonary complications and infections are related to the underlying disease and the surgical procedure. Device- and lead-related complications, high defibrillation thresholds, pacemaker interactions, inappropriate shocks, arrhythmic effects, syncope, and psychosocial problems are reported, respectively.     

Sudden cardiac death: definition, mechanisms and risk factors

Sudden cardiac death is defined as natural death due to cardiac causes, heralded by abrupt loss of consciousness within one hour after the onset of symptoms. The mechanisms are the following: 1. ventricular fibrillation, 2. ventricular tachycardia and flutter with subsequent ventricular fibrillation, 3. torsade de pointe, 4. bradyarrhythmias and asystolic arrest. White the main risk factor is the presence of coronary artery disease, any organic or functional disease of the heart can predispose for sudden cardiac death. To evaluate the risk of sudden cardiac death noninvasive (Holter, echocardiography, exercise test and signal averaged (ECG) and often invasive (electrophysiological study) tests are necessary. The therapy is based on drugs (mainly beta blockers and amiodarone), coronary revascularization, catheter ablation techniques and the implantation of a cardioverter defibrillator; the latter appears to be the most promising approach.     

Right ventricular arrhythmia in the absence of arrhythmogenic dysplasia: MR imaging of myocardial abnormalities

PURPOSE: To evaluate right ventricular abnormalities with magnetic resonance (MR) imaging in patients with arrhythmia but without arrhythmogenic dysplasia. MATERIALS AND METHODS: In 53 patients being evaluated for right ventricular arrhythmia and 15 control subjects, MR imaging was performed to evaluate fixed thinning, fatty replacement, or reduced systolic wall thickening or motion. A diagnosis of idiopathic right ventricular outflow tract tachycardia or indeterminate was assigned for each patient, and the severity of arrhythmia was categorized. RESULTS: Right ventricular abnormalities were revealed in 32 (60%) of the 53 patients: fixed thinning in 27 (84%), fatty replacement in eight (25%), and reduced wall thickening or motion in 31 (97%). Right ventricular abnormalities were found in 35 (76%) of 46 patients with idiopathic right ventricular outflow tract tachycardia and in seven (39%) of 18 patients with indeterminate diagnoses (P = .022). CONCLUSION: Mild right ventricular abnormalities are likely sources for arrhythmias, even in the absence of arrhythmogenic right ventricular dysplasia.     

Left ventricular lesions in arrhythmogenic right ventricular dysplasia and 12-lead electrocardiographic findings

Left ventricular lesions in arrhythmogenic right ventricular dysplasia have not been well described, and the relationship between the left ventricular lesions and the 12-lead electrocardiographic findings has not been analyzed. This study examined whether the presence of left ventricular lesions and the extent of right ventricular lesions due to arrhythmogenic right ventricular dysplasia are predictable by 12-lead electrocardiographic findings. The 12-lead electrocardiograms during sinus rhythm and left and right ventriculography were studied in 29 patients (27 males and 2 females, mean age 42.6 +/- 15.5 years) diagnosed by the current criteria for this disease. After evaluation, patients were divided into two groups: those with normal left ventricles (normal group) and those with left ventricular wall motion abnormalities (abnormal group). Seventeen of the 29 patients (59%) were classified into the abnormal group. Left ventricular wall motion abnormalities were located in the posterolateral (4 patients), apical (1), and posterolateral and apical regions (12). QS patterns of abnormal Q waves in lead I, aVL or V5, V6 rS patterns (R/S ratio < 1) in leads I and V6, and/or R or Rs patterns (R/S ratio > 1) in lead V1 were observed in all patients in the abnormal group, but in none in the normal group. There was a positive correlation between the right ventricular end-diastolic volume index and the number of precordial negative T waves (r = 0.746, p < 0.0001), and the time from onset of the QRS to the terminal portion of the epsilon wave in lead V1 (r = 0.627, p < 0.001). The correlation coefficients showed no significant differences between the groups. A left ventricular lesion associated with arrhythmogenic right ventricular dysplasia was not unusual (59%), and our study suggests that the posterolateral and apical regions are the most frequent sites. The presence of these lesions were predictable by the QRS abnormalities. Moreover, regardless of the presence of such a lesion, the extent of the right ventricular lesion is also predictable by the 12-lead electrocardiographic findings.     

Bilateral stress-fracture of the calcaneal tubercle

Arrhythmogenic right ventricular dysplasia (ARVD) is a heart muscle disease in which muscle tissue has been partially replaced by adipose or fibro-adipose tissue. Morphologic changes in the right ventricle and ventricular arrhythmias are characteristic. Pathomorphological changes should be confirmed by NMR or endomyocardial biopsy. Morphological changes ought to be found by ultrasound methods or angiographic examination. ECG exercise test, Holter monitoring, late potentials, total ventricular activation time and programmed stimulation of right ventricle are used to evaluate the risk of sudden death due to ventricular arrhythmias which is the most important problem. Those methods indicate pharmacologic or invasive therapy (RF ablation, implanted cardioverter-defibrillators), and are used to establish the effectiveness of treatment.     

Bronchodilator response to salbutamol after chronic dosing with salmeterol or placebo

OBJECTIVES: We report the occurrence of cardiac events during long-term follow-up in patients with hypertrophic cardiomyopathy (HCM) after cardioverter-defibrillator implantation. BACKGROUND: The identification of patients at high risk for sudden death and the prevention of recurrence of sudden death in HCM represents a difficult problem. METHODS: We retrospectively analyzed the occurrence of cardiac events during follow-up of 13 patients with HCM who received an implantable cardioverter-defibrillator (ICD) because of aborted sudden death (n = 10) or sustained ventricular tachycardia (n = 3) (group I). Findings were compared with those in 215 patients with an ICD and other structural heart disease or idiopathic ventricular fibrillation (group II). RESULTS: After a mean (+/-SD) follow-up period of 26+/-18 months, 2 of 13 patients in group I received appropriate shocks. The calculated cumulative incidence of shocks was 21% in group I and 66% in group II after 40 months (p < 0.05). We observed a low incidence of recurrence of ventricular tachycardia/fibrillation during follow-up in patients with HCM. No deaths occurred. CONCLUSIONS: Our data suggest that ventricular tachyarrhythmias may not always be the primary mechanism of syncope and sudden death in patients with HCM. The ICD seems to have a less important impact on prognosis in patients with HCM than in patients with other etiologies of aborted sudden death.     

The chronobiology and clinical efficacy of Daflon in the treatment of chronic venous insufficiency

Improvement of methods for diagnosis and treatment of myocardial infarction and its complications permitted the mortality and disability resulting from this condition to be considerably reduced. The aim of the study was to analyse in retrospect clinical and functional disorders in subjects with a history of ventricular fibrillation in the acute phase. Over 3 years, 1096 patients with acute myocardial infarction had been admitted to the Department of Cardioresuscitation. Ventricular Fibrillation was cause of death in 2.1% of cases. Over this time period, resuscitation was a success in 2.9%. The following item was found out to be a factor of unfavourable prognosis during the first two years after development of acute myocardial infarction: it was postinfarction dilatation of the left ventricle having a part in the evolution of cardiac insufficiency and contributing to formation of arrhythmogenic substrate and, to a lesser degree, to ventricular fibrillation in the acute phase.     

Resuscitation in ventricular fibrillation as the first manifestation of Bland-White-Garland syndrome in adulthood

CASE REPORT: This case presents a 31-year-old male patient with anomalous origin of the left coronary artery from the pulmonary trunc. First symptom of the disease was a survived sudden cardiac death. Subsequent angiographic and echocardiographic studies demonstrated the anomalous origin of the left coronary artery from the pulmonary artery. There were no signs of prior myocardial infarction. After reimplantation of the anomalous originating left coronary artery no myocardial ischemia could be detected in the thallium-201 myocardial imaging, which was present before surgical correction. In this case myocardial ischemia was the only potential triggering mechanism responsible for the sudden cardiac death, which was no longer detectable after surgical correction. Therefore no additional pharmacological and nonpharmacological antiarrhythmic treatment was initiated. CONCLUSION: In rare cases the first manifestation of Bland White Garland syndrome in the adult patient could be sudden cardiac death due to ventricular fibrillation.     

Fish, fish oils, arrhythmias and sudden death

Cardiovascular morbidity and mortality is relatively low in individuals with a high consumption of fish oils containing omega-3 fatty acids (eicosapentaenoic acid and docosahexaenoic acid). This has been mainly attributed to the anti atherogenic and anti thrombotic effects of these oils. However, recent evidence suggests that fish and fish oils may also prevent malignant ventricular arrhythmias and sudden cardiac death. Several animal experiments have shown that fish oils can reduce the incidence of ischaemia induced ventricular tachycardia and fibrillation. Observational studies in humans have shown that there is a connection between the intake of omega-3 fatty acids and a lower risk of sudden cardiac death. Some trials suggest that fish oils can prevent ventricular arrhythmias in humans. It is possible that the effect of fish oils on arrhythmias is independent of their anti atherogenic and anti thrombotic activities. There is also some evidence that these oils affect ion fluxes in cardiomyocytes.     

Spectral turbulence versus time-domain analysis of signal-averaged ECG used for the prediction of different arrhythmic events in survivors of acute myocardial infarction

INTRODUCTION: Spectral turbulence analysis of the signal-averaged ECG (SAECG) combines spectral analysis with statistical evaluation of spectrograms of individual parts of the QRS complex. It has been suggested that it may be superior to conventional time-domain analysis of the SAECG. METHODS AND RESULTS: This study compared the power of conventional time-domain (40 to 250 Hz) and spectral turbulence analyses of SAECG for the prediction of cardiac death, ventricular tachycardia, sudden arrhythmic death, and arrhythmic events (ventricular tachycardia or fibrillation, and/or sudden arrhythmic death) after acute myocardial infarction in 603 patients. The population excluded patients with bundle branch block and other conduction abnormalities. During the first 2 years of follow-up, there were 40 cardiac deaths, 21 cases of ventricular tachycardia, 1 sudden arrhythmic deaths, and 29 arrhythmic events. The positive predictive accuracy of spectral turbulence analysis was significantly higher than time-domain analysis for cardiac death at most levels of sensitivity (e.g., 26% vs 20% at 40% sensitivity, P < 0.05). The positive predictive accuracies of the two techniques were not statistically different for the prediction of ventricular tachycardia. For the prediction of sudden arrhythmic death and arrhythmic events, the positive predictive accuracy of spectral turbulence was better than that of time-domain analysis only at the higher levels of sensitivity (9% vs 2%, P < 0.001 for sudden arrhythmic death at 60% sensitivity, and 14% vs 11%, P < 0.05 for arrhythmic events at 60% sensitivity). CONCLUSIONS: Spectral turbulence analysis is essentially equivalent to time-domain analysis for the prediction of arrhythmic events after myocardial infarction. However, it performed significantly better than time-domain analysis for the prediction of cardiac death.     

Prognostic value of arrhythmogenic markers in systemic hypertension

OBJECTIVE: To evaluate the prognostic value of arrhythmogenic markers in hypertensive patients. DESIGN: Two hundred and fourteen hypertensive patients without symptomatic coronary disease, systolic dysfunction, electrolyte disturbances or anti-arrhythmic therapy were included. Recordings were made of 12-lead standard ECGs with calculations of QT interval dispersion, 24 h Holter ECGs (204 patients), echocardiography (187 patients) and signal-averaged ECGs (125 patients). RESULTS: Baseline data: echocardiographic left ventricular hypertrophy was found in 63 patients (33.7%), non-sustained ventricular tachycardia (Lown class IV b) in 33 patients (16.2%), ventricular late potentials in 27 patients (21.6%). Mortality: after a mean follow-up of 42.4 +/- 26.8 months, global mortality was 11.2% (24 patients), cardiac mortality 7.9% (17 patients), sudden death 4.2% (nine patients). Univariate analysis: predictors of global, cardiac and sudden death were age > or = 65 years, ECG strain pattern, Lown class IV b and QT interval dispersion > 80 ms (P < or = 0.01). Left ventricular mass index was closely related to cardiac mortality (P = 0.002). Multivariate analysis: only Lown class IV b was an independent predictor of global (RR 2.6, 95% CI 1.2-6.0) and cardiac mortality (RR 3.5, 95% CI 1.2-9.7). CONCLUSION: In hypertensive patients, non-sustained ventricular tachycardia has a prognostic value.     

MRI semeiology of segmental contraction abnormalities in arrhythmogenic dysplasia of the right ventricle

The diagnosis of localized arrhythmogenic right ventricular dysplasia may be difficult to ascertain. Aside from electrophysiological arguments, visualization of an abnormal right ventricular contraction pattern is of crucial importance for diagnosis. Cine-MR is almost the only examination method which offers detailed informations on the right ventricular contraction pattern. Nine observations of segmental right ventricular contraction abnormalities assessed by cine-MR are described here: dyskinesia of the distal part of the anterior wall (2), of the inferior wall (2), of the right ventricular outflow tract (2); akinesia of the outflow tract (2) and of the inferior wall (1). Morphological abnormalities of the right ventricle are always associated with contraction abnormalities but seem to be less disease specific. Patients should be more readily referred for a cine-MR examination when the diagnosis of localized right ventricular dysplasia is suspected. Cine-MR sequences related to these observations may be reached via Internet at:http:@alsace.u-strasbg.fr/cardio/coeur.htm.     

Sudden unexpected death resulting from an anomalous hypoplastic left coronary artery

We present a case of sudden death in a 24-year-old, healthy white female who was physically active and participated in sports, including soccer. Two weeks prior to her death, an insurance physical examination revealed an abnormal electrocardiogram which demonstrated flipped T waves in the anterior leads. There was no other remarkable medical history. At autopsy, only one coronary ostium was demonstrated and it originated from the right aortic sinus. Approximately 0.8 cm from this right coronary artery (RCA) ostium, a left coronary artery (LCA) branched off the RCA at a 90-degree angle. The LCA had luminal diameter of 0.4 m but the LCA had a luminal diameter of only 0.1 cm. The LCA coursed anterior to the base of the pulmonary artery and down the anterior ventricular septum reflecting the usual course of the left anterior descending (LAD) coronary artery. The LCA and RCA paths appeared to merge or terminate at the anterior left ventricular myocardium which was discolored gray, a process that involved the inner and middle thirds of the myocardium. Based on the autopsy findings, we certified the cause of death as a probable arrhythmia due to myocardial fibrosis and dystrophic calcification resulting from complications of an anomalous hypoplastic left coronary artery. Anomalies of coronary arteries have been classified and studied at autopsy and by clinical angiography. Coronary artery anomalies can be divided into minor and major forms with major anomalies often resulting in cardiac dysfunction that may cause failure and death. Minor anomalies, in general, have no pathophysiological significance and are compatible with life. Minor anomalies include variations in number and location of coronary ostia. A single coronary ostia is exceedingly rare in hearts with no other congenital malformations. The prognostic significance can be unpredictable. A single coronary artery has the potential to be dangerous if obstructed at its main stem, or if it branches at an acute angle. Additionally, hypoplasia of one or more coronary arteries has been found to be associated with sudden death.     

An experimental model of sudden death due to low-energy chest-wall impact (commotio cordis)

BACKGROUND: The syndrome of sudden death due to low-energy trauma to the chest wall (commotio cordis) has been described in young sports participants, but the mechanism is unknown. METHODS: We developed a swine model of commotio cordis in which a low-energy impact to the chest wall was produced by a wooden object the size and weight of a regulation baseball. This projectile was thrust at a velocity of 30 miles per hour and was timed to the cardiac cycle. RESULTS: We first studied 18 young pigs, 6 subjected to multiple chest impacts and 12 to single impacts. Of the 10 impacts occurring within the window from 30 to 15 msec before the peak of the T wave on the electrocardiogram, 9 produced ventricular fibrillation. Ventricular fibrillation was not produced by impacts at any other time during the cardiac cycle. Of the 10 impacts sustained during the QRS complex, 4 resulted in transient complete heart block. We also studied whether the use of safety baseballs, which are softer than standard ones, would reduce the risk of arrhythmia. A total of 48 additional animals sustained up to three impacts during the T-wave window of vulnerability to ventricular fibrillation with a regulation baseball and safety baseballs of three degrees of hardness. We found that the likelihood of ventricular fibrillation was proportional to the hardness of the ball, with the softest balls associated with the lowest risk (two instances of ventricular fibrillation after 26 impacts, as compared with eight instances after 23 impacts with regulation baseballs). CONCLUSIONS: This experimental model of commotio cordis closely resembles the clinical profile of this catastrophic event. Whether ventricular fibrillation occurred depended on the precise timing of the impact. Safety baseballs, as compared with regulation balls, may reduce the risk of commotio cordis.     

Cardiac disease in intracerebral hematomas

INTRODUCTION AND OBJECTIVE: The Central Nervous System (CNS) plays an essential role in the regulation of the cardiac function. There is strong evidence that many CNS lesions, mainly those of hemorrhagic origin, may induce repolarization abnormalities and enlargement of the QT interval (ECG changes) and several types of arrhythmias. In some cases these changes have been related to sudden death. The imbalance between the sympathetic and parasympathetic systems, favoring the former, seems to be the etiopathogenic factor. MATERIAL AND METHODS: We have carried out a study on thirty-two in-patients suffering from non-severe intracerebral hemorrhage, by means of a Holter ECG examination within the first 72 hours and a second record after two months. We have assessed any significative differences on the ECG findings in relation to the location of the hematoma (left or right hemispheres) and the presence of a personal history of arterial hypertension and/or heart disease. RESULTS: One or more ECG changes were present in 69.2% of the patients and 73% showed one or more rhythm abnormalities. There was a higher incidence of supraventricular arrhythmias associated with the right hemisphere hematomas, with an statistical significance for the atrial extrasystolia. No differences were found between the group with a previous history of hypertension and/or heart disease and the one without these conditions. There were two cases of sudden death, both with left hemisphere hematomas, and in one of them the previous rhythm abnormalities were recorded. CONCLUSIONS: This study corroborates the hypothesis that right hemispheric hematomas induce supraventricular arrhythmias more frequently. The possible association between severe ventricular arrhythmias and sudden death with left-hemisferic hematomas should be studied in a higher number of patients. We recommend monitoring every acute case of intracerebral hematoma when possible.