Proposed diagnostic criteria for the Brugada syndrome.
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Bundle-Branch Block↗
Biomedical subjects
D Corrado
Publications and source records attributed to D Corrado.
Dispersion of ventricular depolarization-repolarization: a noninvasive marker for risk stratification in arrhythmogenic right ventricular cardiomyopathy.
BACKGROUND: We retrospectively investigated the value of clinical and ECG findings as well as QT-QRS dispersion in predicting the risk of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). METHODS AND RESULTS: Duration and interlead variability of the QT interval and QRS complex were measured manually from standard ECGs in 20 sudden death victims with ARVC diagnosed at autopsy (group I), in 20 living ARVC patients with sustained ventricular tachycardia (group II), in 20 living ARVC patients with </=3 consecutive premature ventricular beats (group III), and in 20 control subjects (group IV). QT and QRS dispersions were greater in group I (77.5+/-10.6 ms for QT and 45.7+/-8.1 ms for QRS) compared with group II (64.5+/-13.9 ms for QT [P=0.001] and 33.5+/-8.7 ms for QRS [P=0.0004]) and in group II compared with group III (48+/-8.9 ms for QT [P<0.0001] and 28+/-5.2 ms for QRS [P<0.0001]) and group IV (33.5+/-4.8 ms for QT [P<0.0001] and 18.5+/-3.6 ms for QRS [P<0.0001]). Negative T wave beyond V(1) and syncope were statistically more frequent in group I (P=0.02 and P=0.007, respectively). On multivariate analysis, QRS dispersion remained an independent predictor of sudden death (P<0.0001), followed by syncope (P=0.09). In assessing risk of sudden death, QRS dispersion >/=40 ms had a sensitivity and specificity of 90% and 77%, respectively; QT dispersion >65 ms, 85% and 75%, respectively; negative T wave beyond V(1), 85% and 42%, respectively; and syncope, 40% and 90%, respectively. CONCLUSIONS: QRS dispersion (>/=40 ms) was the strongest independent predictor of sudden death in ARVC. Syncope, QT dispersion >65 ms, and negative T wave beyond V(1) refined arrhythmic risk stratification in these patients.
Adolescent↗
Right bundle branch block, right precordial st-segment elevation, and sudden death in young people.
BACKGROUND: Patients with the ECG pattern of right bundle branch block and right precordial ST-segment elevation may experience sudden death in the setting of either arrhythmogenic right ventricular cardiomyopathy (ARVC) or a functional electrical disorder such as Brugada syndrome. METHODS AND RESULTS: Among a series of 273 young (</=35 years) victims of cardiovascular sudden death who were prospectively studied from 1979 to 1998 in the Veneto Region of Italy, 12-lead ECG was available in 96 cases. Thirteen (14%; 12 males and 1 female aged 24+/-8 years) had right precordial ST-segment elevation, either isolated (9 cases) or associated with right bundle branch block (4 cases). At autopsy, all patients had ARVC (92%) except one, who had no evidence of structural heart disease. Compared with the 19 young sudden death victims with ARVC and no ST-segment abnormalities from the same series, those with AVRC and right precordial ST-segment elevation included fewer competitive athletes (17% versus 58%; P:=0.03), more often died suddenly at rest or during sleep (83% versus 26%; P:=0.003), and showed serial ECG changes over time (83% versus 0; P:=0.015), polymorphic ventricular tachycardia (33% versus 0; P:=0.016), and predominant fatty replacement of the right ventricular anterior wall (58% versus 21%; P:=0.05), CONCLUSIONS: Right precordial ST-segment elevation was found in 14% of young sudden death victims with available ECG. It mostly reflected underlying ARVC with predominant right ventricular anterior wall involvement and characterized a subgroup of patients who share with Brugada patients the propensity to die from non-exercise-related cardiac arrest and to exhibit dynamic ECG changes and polymorphic ventricular tachycardia.
Adult↗
Ventricular preexcitation in children and young adults: atrial myocarditis as a possible trigger of sudden death.
BACKGROUND: Sudden death (SD) in ventricular preexcitation (VP) syndrome is believed to be the result of atrial fibrillation with rapid ventricular response over the accessory pathway. Previous reports are anecdotal and often lack autopsy validation. METHODS AND RESULTS: Prevalence and clinicopathological features of VP were investigated in a series of 273 SDs in children and young adults (aged <or=35 years). Site of accessory atrioventricular (AV) connection was predicted by 12-lead ECG. Right and left AV ring together with the sinoatrial and AV septal junction were studied in serial histological sections. Ten patients (3.6%; male, mean age 24+/-7 years) had VP: 8 had Wolff-Parkinson-White (WPW) and 2 had Lown-Ganong-Levine (LGL) syndrome. Six patients had previous symptoms, and SD occurred at rest in all but 1. Pathological substrates of LGL consisted of AV-node hypoplasia and right-sided atrio-Hisian tract, respectively. In the 8 WPW patients, 10 total accessory AV pathways consisting of ordinary myocardium were found (7 left lateral, 2 right posterolateral, and 1 septal). These pathways were close to the endocardium (mean distance, 750+/-530 microm) and 310+/-190 microm thick. In 4 WPW patients (50%), isolated acute atrial myocarditis was found, which was polymorphous in 1 and lymphocytic in 3. CONCLUSIONS: VP accounted for 3.6% of SD in young people and was not preceded by warning symptoms in 40%. A left accessory pathway was the most frequent substrate, and its subendocardial location supports the feasibility of catheter ablation. Isolated atrial myocarditis may act as a trigger of paroxysmal atrial fibrillation that leads to SD.
Adult↗
Sudden cardiac death in young people with apparently normal heart.
OBJECTIVE: The aim of the present study was to assess the prevalence of subtle morphologic substrates, clinically unrecognizable, underlying sudden cardiac death (SCD) in young people with apparently normal heart. METHODS: In the time interval 1979--1998, 273 consecutive cases of SCD in young people (< or =35 years) which occurred in the Veneto Region of Italy were prospectively studied. Following exclusion of extracardiac causes of sudden death, the heart was examined according to a detailed morphologic protocol consisting of macroscopic and histologic examination, including study of the specialized conduction system by serial sections. RESULTS: At macroscopic examination, 197 SCD victims (72%) had an overt underlying structural heart disease such as cardiomyopathy in 56, obstructive coronary atherosclerosis in 54, valve disease in 32, non-atherosclerotic coronary artery disease in 28, aortic rupture in 13, postoperative congenital heart disease in five, and other disease in nine. The remaining 76 cases (28%) (50 males and 26 females, aged 4-35 years, mean 23+/-5 years) had a macroscopically normal heart. A total of 28 of them (37%) had experienced one or more of the following prodroma: syncope, palpitations or both in 20, ECG abnormalities in 18 and arrhythmias in ten. In 79% of them, histologic examination disclosed concealed pathologic substrates consisting of focal myocarditis in 27 cases, regional arrhythmogenic right ventricular cardiomyopathy, mostly localized to RV outflow tract, in nine, and conduction system abnormalities in 24 (leading to ventricular preexcitation in 18 and heart block in six). In 16 hearts (6%) there was no evidence of structural heart disease even after histologic study. CONCLUSION: Macroscopic heart features were normal in nearly one-third of young SCD victims. In 79% of them, however, histologic study unmasked concealed pathologic substrates such as focal myocarditis or cardiomyopathy and conduction system diseases. A total of 16 victims (6%) had no evidence of structural heart disease and the mechanism of their SCD remained unexplained.
Adolescent↗
Postmortem diagnosis in sudden cardiac death victims: macroscopic, microscopic and molecular findings.
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Adult↗
Arrhythmogenic right ventricular dysplasia: cardiomyopathy current opinions on diagnostic and therapeutic aspects.
Right Ventricular Dysplasia constitutes a genetic cardiomyopathy characterized by fibrous-adipose substitution of the right and rarely of the left ventricular myocardium. This disorder is associated with ventricular arrhythmias ranging from frequent ventricular ectopic beats, nonsustained and sustained ventricular tachycardia of left bundle branch morphology and sudden death. Therefore, the syndrome has been labelled Arrhythmogenic RVD Cardiomyopathy. Diagnostic criteria, preliminary genetic data, and clinical manifestations are summarized and critical addressed, using data from the literature and from our own experience. The most important aspects of the ECG in this syndrome are reviewed and stressed with particular attention to initial versus advanced clinical subsets. The typical anatomical abnormalities and biopsy or pathology material are presented.
Arrhythmogenic Right Ventricular Dysplasia↗
Right bundle-branch block, ST-segment elevation, and sudden death.
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Bundle-Branch Block↗
Arrhythmogenic right ventricular dysplasia/cardiomyopathy: need for an international registry. Study Group on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy of the Working Groups on Myocardial and Pericardial Disease and Arrhythmias of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the World Heart Federation.
Arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C) is a heart muscle disease characterized by peculiar RV involvement and electrical instability that precipitates ventricular arrhythmias and sudden death. The purpose of the present consensus report of the Study Group on ARVD/C of the Working Groups on Myocardial and Pericardial Disease and Arrhythmias of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the World Heart Federation is to review the considerable progress in our understanding of the etiopathogenesis, morbid anatomy, and clinical presentation of ARVD/C since it first was described in 1977. The present article focuses on important but still unanswered issues, mostly regarding risk stratification, clinical outcome, and management of affected patients. Because ARVD/C is relatively uncommon and any one center may have experience with only a few patients, an international registry is being established to accumulate information and enhance the numbers of patients that can be analyzed and thus answer pending questions. The registry also will facilitate pathological, molecular, and genetics research on the causes and pathogenesis of the ARVD/C. Furthermore, availability of an international database will enhance awareness of this largely unrecognized condition among the medical community. Physicians are encouraged to enroll patients in the International Registry of ARVD/C.
Arrhythmogenic Right Ventricular Dysplasia↗
Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes.
OBJECTIVES: The purpose of this study is to characterize the clinical profile and identify clinical markers that would enable the detection during life of anomalous coronary artery origin from the wrong aortic sinus (with course between the aorta and pulmonary trunk) in young competitive athletes. BACKGROUND: Congenital coronary artery anomalies are not uncommonly associated with sudden death in young athletes, the catastrophic event probably provoked by myocardial ischemia. Such coronary anomalies are rarely identified during life, often because of insufficient clinical suspicion. However, since anomalous coronary artery origin is amenable to surgical treatment, timely clinical identification is crucial. METHODS: Because of the paucity of available data characterizing the clinical profile of wrong sinus coronary artery malformations, we reviewed two large registries comprised of young competitive athletes who died suddenly, assembled consecutively in the U.S. and Italy. RESULTS: We reported 27 sudden deaths in young athletes, identified solely at autopsy and due to either left main coronary artery from the right aortic sinus (n = 23) or right coronary artery from the left sinus (n = 4). Each athlete died either during (n = 25) or immediately after (n = 2) intense exertion on the athletic field. Fifteen athletes (55%) had no clinical cardiovascular manifestations or testing during life. However, in the remaining 12 athletes (45%) aged 16 +/- 7, certain clinical data were available. Premonitory symptoms had occurred in 10, including syncope in four (exertional in three and recurrent in two, 3 to 24 months before death) and chest pain in five (exertional in three, all single episodes, < or =24 months before death). All cardiovascular tests were within normal limits, including 12-lead electrocardiogram (ECG) pattern (in 9/9), stress ECG with maximal exercise (in 6/6) and left ventricular wall motion and cardiac dimensions by two-dimensional echocardiography (in 2/2). CONCLUSIONS: With regard to congenital coronary artery anomalies of wrong aortic sinus origin in young competitive athletes, 1) standard testing with ECG under resting or exercise conditions is unlikely to provide clinical evidence of myocardial ischemia and would not be reliable as screening tests in large athletic populations, 2) premonitory cardiac symptoms not uncommonly occurred shortly before sudden death (typically associated with anomalous left main coronary artery), suggesting that a history of exertional syncope or chest pain requires exclusion of this anomaly. These observations have important implications for the preparticipation screening of competitive athletes.
Adolescent↗
Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy.
OBJECTIVES: We sought to define the clinical picture and natural history of familial arrhythmogenic right ventricular cardiomyopathy (ARVC). BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy is a myocardial disease, often familial, clinically characterized by the impending risk of ventricular arrhythmias and sudden death. METHODS: Thirty-seven ARVC families of northeast Italy were studied. Probands had a histologic diagnosis of ARVC, either at autopsy (19 families) or endomyocardial biopsy (18 families). Protocol of the investigation included basal electrocardiogram (ECG), 24-hour ECG, signal-averaged ECG, stress test and two-dimensional Doppler echocardiography. Invasive evaluation was performed when deemed necessary. RESULTS: Of the 365 subjects, 151 (41%) were affected, 157 (43%) were unaffected, 17 (5%) were healthy carriers, and 40 (11%) were uncertain. Mean age at diagnosis was 31+/-13 years. By echocardiography, 64% had mild, 30% had moderate, and 6% had severe form. Forty percent had ventricular arrhythmias, 49 were treated with antiarrhythmic drugs, and two were treated with implantable cardioverter defibrillators. Sport activity was restricted in all. Of the 28 families who underwent linkage analysis, 6 mapped to chromosome 14q23-q24, 4 to 1q42-q43, and 4 to 2q32.1-q32.3. No linkage with known loci was found in four families and 10 had uninformative results. During a follow-up of 8.5+/-4.6 years, one patient died (0.08 patient/year mortality), and 15 developed an overt form of ARVC. CONCLUSIONS: Arrhythmogenic right ventricular cardiomyopathy is a progressive disease appearing during adolescence and early adulthood. Systematic evaluation of family members leads to early identification of ARVC, characterized by a broad clinical spectrum with a favorable outcome. In the setting of positive family history, even minor ECG and echocardiographic abnormalities are diagnostic.
Adult↗
Hypertrophic cardiomyopathy and sudden death in the young: pathologic evidence of myocardial ischemia.
The mechanism underlying cardiac arrest in patients with hypertrophic cardiomyopathy (HC) is intriguing. In the clinical setting, myocardial ischemia has long been incriminated, particularly in the young. Among 274 cardiovascular sudden deaths in the young (< or = 35 years), 19 (7.0%), 14 males and 5 females, median age 23 years, had HC. Familial occurrence of HC was ascertained in 3 (16%). SD occurred on effort in 6 (31%). Previous syncope occurred in 5 and palpitations in 3. Basal electrocardiogram (ECG) was abnormal in 7 of 8 available cases. Hypertrophy was septal asymmetric in 14. Gross examination showed large isolated or multiple septal scars in 11 (58%); at histomorphometry, the mean percent area of fibrosis of the septal myocardium was 18.6 +/- 6. Four showed a deep intramyocardial course of the left anterior descending coronary artery. At histology, myocardial disarray involved 30 +/- 16% of the septal myocardium; evidence of acute-subacute myocardial necrosis was present in 14 (74%), 1 of them with a regional acute myocardial infarction. By comparing hearts with (n = 11) and without (n = 8) areas of scar-type fibrosis, we found a statistically significant difference in terms of age (25.5 +/- 5.4 v 15.5 +/- 12.4 years, P = .04), septal thickness (25.4 +/- 5.4 v 15.4 +/- 4.9 mm, P < .001), percent increase of septal thickness versus normal value for age and sex (46.2 +/- 15 v 25.2 +/- 13.6%, P < .01) and mean score of small vessel disease (1.7 +/- 0.4 v 1.2 +/- 0.4, P = .04). Linear regression analysis showed a positive correlation of percent area of replacement fibrosis with septal thickness (P = .01) and with mean score of small vessel disease (P < .01). In conclusion, our pathologic findings of ischemic damage, either acute-subacute or in the form of fibrotic scars, support the clinical evidence that ischemia occurs in the natural history of HC and may contribute to life-threatening electrical instability.
Adolescent↗
Clinical diagnosis and management strategies in arrhythmogenic right ventricular cardiomyopathy.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a myocardial disease, often familial, that is characterized by fibro-fatty replacement of the right ventricular (RV) myocardium. The most common clinical manifestations of ARVC consists of ventricular arrhythmias of RV origin, which may lead to sudden death mostly in young people and athletes, electrocardiograph depolarization/repolarization changes mostly localized to right precordial leads, and global and/or regional dysfunction and structural alterations of the RV. The diagnosis of ARVC may be difficult due to several problems with the specificity of the electrocardiograph abnormalities, the different potential etiologies of ventricular arrhythmias with a left bundle branch morphology, the assessment of the RV structure and function, and the interpretation of endomyocardial biopsy findings. Therefore, standardized diagnostic criteria have been proposed by the Study Group on ARVC of the European Society of Cardiology. According to these guidelines, the diagnosis of ARVC is based on the presence of major and minor criteria encompassing electrocardiograph, arrhythmic, morphofunctional, histopathologic, and genetic factors. Because the assessment of sudden death risk in patients with ARVC is still not well established, there are no precise guidelines to determine which are the patients who need to be treated and which is the best management approach. The therapeutic options include beta blockers, antiarrhythmic drugs, catheter ablation, and implantable cardioverter defibrillator. The implantable defibrillator is the most effective safe-guard against arrhythmic sudden death. However, its precise role in changing natural history of ARVC by preventing sudden and nonsudden death needs to be evaluated by a prospective study of a large series of patients. In patients in whom ARVC has progressed to severe RV or biventricular systolic dysfunction with risk of thromboembolic complications, treatment consists of current therapy for heart failure including anticoagulant therapy. In case of refractory congestive heart failure, the patients may become candidates for heart transplantation.
Anti-Arrhythmia Agents↗
Orthodromic tachycardia with atrioventricular dissociation: evidence for a nodoventricular (Mahaim) fiber.
We describe a patient in whom two tachycardias with AV dissociation were inducible by ventricular extrastimulation. The first tachycardia was characterized by a narrow QRS preceded by a His deflection with an HV interval identical to that recorded in sinus rhythm (40 ms). Premature ventricular depolarization delivered when the His bundle was refractory advanced the next His deflection. These findings suggest the presence of a nodoventricular bypass tract involved in an orthodromic tachycardia. The second tachycardia was induced after propafenone infusion and exhibited a wide QRS complex with left bundle branch block morphology; each ventricular complex was consistently associated with a His deflection with a HV interval of -15 ms. The second tachycardia may be considered to represent an antidromic tachycardia through the nodoventricular tract. However, a ventricular tachycardia cannot be excluded.
Atrioventricular Node↗
Arrhythmogenic right ventricular dysplasia/cardiomyopathy: need for an international registry. European Society of Cardiology and the Scientific Council on Cardiomyopathies of the World Heart Federation.
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a heart muscle disease characterized by peculiar right ventricular involvement and electrical instability that precipitates ventricular arrhythmias and sudden death. The purpose of the present consensus report of the Study Group of the European Society of Cardiology and the Scientific Council on Cardiomyopathies of the World Heart Federation is to review the considerable progress in our understanding of the etiopathogenesis, morbid anatomy, and clinical presentation of ARVD/C since its first description in 1977. This article will focus on the important but still unanswered issues, mostly regarding risk stratification, clinical outcome, and management of affected patients. Because ARVD/C is relatively uncommon and any one center may have experience with only a few patients, an international registry is being established to accumulate information and enhance the numbers of patients that can be analyzed to answer the pending questions. The registry also will facilitate pathologic, molecular, and genetics research on the etiology and pathogenesis of the disease. Furthermore, availability of an international database will enhance awareness of this largely unrecognized condition among the medical community. Physicians are encouraged to enroll patients in the International Registry of ARVD/C.
Arrhythmias, Cardiac↗
Arrhythmogenic right ventricular cardiomyopathy: diagnosis, prognosis, and treatment.
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Arrhythmogenic Right Ventricular Dysplasia↗
Is prevention of sudden death in young athletes feasible?
A wide spectrum of cardiovascular abnormalities may cause sudden death in young athletes, and even minor lesions may be life-threatening by jeopardizing the electrical order of the heart during effort. The culprit diseases are clinically covert and difficult to diagnose or suspect. Early identification during systematic preparticipation screening might reduce the risk and incidence of sudden death. In the time interval 1979-1996, 49 young athletes died suddenly in the Veneto Region. The prevalence of sudden death was calculated as 0.75/100,000/year among young nonathletes and 1.6/100,000/year among young athletes (p < 0.01). Among athletes, arrhythmogenic right ventricular cardiomyopathy was the leading cause, followed by atherosclerotic coronary artery disease and congenital coronary anomalies. Hypertrophic cardiomyopathy has been a very uncommon cause of fatal events, due to the identification and disqualification of affected subjects at the preparticipation screening. Only arrhythmogenic right ventricular cardiomyopathy and congenital anomalies of coronary arteries occurred more frequently in athletes than in nonathletes, as to indicate that these diseases are particularly prone to cardiac arrest during effort. The rarity of prodroma in patients affected by coronary artery disease, both acquired and congenital, particularly the absence of chest pain, stresses the difficulty in suspecting these lesions. On the opposite, most of athletes with arrhythmogenic right ventricular cardiomyopathy had something that might have alerted, either in terms of family history or electrical disorders. Our data are strongly in favor of an effective role of the Italian protocol for preparticipation screening in preventing sudden death in young athletes affected by hypertrophic cardiomyopathy. With increased awareness of diagnostic criteria, also athletes with concealed arrhythmogenic right ventricular cardiomyopathy will be identified in the future and sudden death possibly prevented.
Adolescent↗
Sudden death in the young and in the athlete: causes, mechanisms and prevention.
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Adult↗