Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy.

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 (≤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.

/pdf/sudden-cardiac-death-in-young-people-with-apparently-normal-1x5wpyy6zu.pdf

Sudden cardiac death in young people with apparently normal heart

https://www.onlinejacc.org/content/accj/40/8/1445.full.pdf

Prospective evaluation of relatives for familial arrhythmogenic right ventricular cardiomyopathy/dysplasia reveals a need to broaden diagnostic criteria

Introduction… 316

General Evaluation… 316

Syncope in the Patient With a Normal Evaluation… 318

Syncope in the Patient With Coronary Artery Disease… 319

Nonischemic Dilated Cardiomyopathy… 320

Syncope in Other Forms of Structural Heart Disease… 320

Syncope Resulting From Inherited Cardiac Ion Channel Abnormalities… 321

Evaluation of the Pediatric Patient With Syncope… 321

Special Considerations in the Elderly… 323

Neurological Evaluation… 323

Conclusions… 324

Syncope, a transient loss of consciousness, is a common clinical problem. The most common causes of syncope are cardiovascular in origin and are associated with a high rate of mortality in patients with underlying heart disease, transient myocardial ischemia, and other less common cardiac abnormalities.1

The primary purpose of the evaluation of the patient with syncope is to determine whether the patient is at increased risk for death. This involves identifying patients with underlying heart disease, myocardial ischemia, Wolff-Parkinson-White syndrome, and potentially life-threatening genetic diseases such as long-QT syndrome (LQTS), Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia. If these diagnoses can be excluded, the goal then becomes identification of the cause of syncope in an attempt to improve the quality of the patient’s life and to prevent injury to the patient or others. The purpose of this statement is to summarize the data that direct the evaluation of the patient with syncope (Figure 1). 



Figure 1. Flow chart for the diagnostic approach to the patient with syncope.



In the general population, the most common cause of syncope is neurocardiogenic, followed by primary arrhythmias. Other names for neurocardiogenic syncope include neurally mediated, vasodepressor, and vasovagal syncope. The causes of syncope are highly age dependent.2 Pediatric and young patients are most likely to have neurocardiogenic syncope, conversion reactions (psychiatric causes), and primary arrhythmic causes such as the LQTS and Wolff-Parkinson-White syndrome. In middle age, neurocardiogenic syncope …

AHA/ACCF Scientific Statement on the Evaluation of Syncope: From the American Heart Association Councils on Clinical Cardiology, Cardiovascular Nursing, Cardiovascular Disease in the Young, and Stroke, and the Quality of Care and Outcomes Research Interdisciplinary Working Group; and the American College of Cardiology Foundation In Collaboration With the Heart Rhythm Society

Arrhythmogenic right ventricular cardiomyopathy

Arrhythmogenic right ventricular dysplasia is responsible for ventricular tachycardia affecting an apparently healthy heart. It can sometimes lead to sudden death, which may be the presenting symptomatology of the disease. It results from fibro-adipose infiltration of the free wall of the right ventricle, and sometimes of the septum, possibly secondary to myocarditis. The prognosis depends upon the quality of the left ventricle. If it is healthy, the only risk is that of arrhythmia. Treatment using anti-arrhythmic drugs is most often effective and, with proper management, the prognosis is good and the risk of sudden death eliminated. If the left ventricle is abnormal, there is the risk that dysplasia associated with arrhythmia will progress to right then congestive cardiac failure in the context of a dilated idiopathic cardiomyopathy with ventricular tachycardias originating on the right side. Arrhythmogenic right ventricular dysplasia is a notable cause of sudden death in athletes. Routine screening of such individuals is justified, as is that of those with high risk occupations (locomotive and vehicle drivers, etc.).

Right ventricular dysplasia

Le comportement de stimulateurs cardiaques recents a ete surveille lors de leur exposition a des champs magnetiques 50 Hz et 60 Hz (frequence des reseaux de distribution electrique), et 20 kHZ a 50 kHz (frequences de fonctionnement des plaques de cuisson a Induction). Soixante patients, implantes en 1998 et 1999 avec des stimulateurs cardiaques double chambre de 9 constructeurs differents, ont ete Inclus dans l'etude. L'ECG etait enregistre en continu par telemetrie pendant toute la duree de l'exposition : passage de face, de profil et station dans le systeme d'exposition 50/60 Hz, utilisation d'une plaque de cuisson a induction a faible et forte puissances, sur 1 ou 2 foyers. Les tests ont ete realises avec 2 reglages du stimulateur. Le premier est le reglage standard du stimulateur, dit « medicalement correct », choisi par le medecin pour chaque patient. Ensuite, les stimulateurs etalent reprogrammes en mode unipolaire avec une sensibilite auriculaire et ventriculaire la plus elevee possible, sans induire d'inhibition du stimulateur par les contractions musculaires lors des mouvements du patient. Entre chaque serie d'exposition (50/60 Hz ou plaque a induction) la programmation du stimulateur etait controlee. A la fin des tests, le stimulateur etait reprogramme dans son reglage standard. L'ECG etait controle par un cardiologue qui ne savait pas s'il existait ou non un champ magnetique. Aucun stimulateur n'a ete influence par la presence du champ magnetique avec le reglage standard « medicalement correct ». En reglage unipolaire a sensibilite maximale, une perturbation du stimulateur a ete observee dans 10 cas: des modes de reversion transitoire prevus par le constructeur lors de la detection d'interferences ont ete observes dans 6 cas; une acceleration transitoire due a la detection d'interferences par l'oreillette a ete observee dans 3 cas; une onde T a ete detectee par la sonde ventriculaire dans 1 cas. Aucune inhibition ni deprogrammation n'a ete observee. Un seul appareil (Biotronik) est sorti d'un programme special (recherche d'hysteresis) pendant les tests avec la plaque d'induction, tout en gardant les valeurs de son programme de base, sans que cet evenement ait pu etre repete. Conclusion: les stimulateurs cardiaques actuels n'ont pas de risques d'interferences notables avec les champs magnetiques 50 Hz et 60 Hz et les frequences de fonctionnement des plaques de cuisson a induction. Ils ne sont pas sensibles avec un reglage medicalement correct. Les reglages avec une sensibilite elevee, inhabituelle, entrainent seulement un mode de reversion ou une acceleration du rythme ventriculaire, de facon transitoire.

Effets des champs magnétiques de 50 et 60 Hz et de 20 à 50 kHz sur le fonctionnement des cardiostimulateurs implantés

UNLABELLED The effect of 50 Hz and 60 Hz (frequencies of current distribution) and 20 kHz to 50 kHz (frequencies of induction cooktop) magnetic interference on implanted pacemakers have been assessed with the present generation of device technology. Sixty patients implanted in 1998 and 1999 with dual chamber pacemakers from 9 different manufacturers were monitored with telemetry while passing through, and standing between a system of two coils. They generated a 50 Hz or a 60 Hz magnetic field at 50 microT. Then, patients used a cooktop at different power. The recordings were made with the standard setting of "medically correct" sensing parameters chosen for the patients. Then pacemakers were reprogrammed to the unipolar mode, with the highest atrial (A) and ventricular (V) sensitivity that did not induce muscular inhibition while moving. Between each exposure (50 Hz, 60 Hz or 20 kHz to 50 kHz), the pacemaker programmation was controlled. At the end of the tests, pacemakers will be reprogrammed with the standard setting. The medical observer being blind to the existence or not of the magnetic field. No pacemaker was influenced by the vicinity of the magnetic field at medically correct settings. At unipolar high sensitivity, no inhibition nor reprogramming was observed. Transient reversion to interference mode was observed in 6 cases, 3 transient acceleration due to atrial detection of the interference, and one T wave detection by the ventricular lead. All were observed with the 60 Hz, and only 3 with the 50 Hz magnetic field. One device (Biotronik) shifted out of its special program (hysteresis research) during the tests with the induction cooktop, but it maintained its standard program, and the event could not be repeated despite further testing. CONCLUSION Actual pacemakers do not present any electromagnetic interference with 50 Hz and 60 Hz or induction cooktop frequency working. They are insensitive with medically correct settings. Unusual high sensitivity leads only to noise reversion mode, or transient ventricular tracking.

Effects of 50 to 60 Hz and of 20 to 50 kHz magnetic fields on the operation of implanted cardiac pacemakers

The authors propose a classification of the outcome of arrhythmogenic right ventricular dysplasia with reference to 4 selected cases with a follow-up period of over 9 years. In type I, the left ventricular ejection fraction is normal (EF > 50%) and the risk, exclusively arrhythmic, can be controlled by appropriate antiarrhythmic therapy. This is the commonest form of arrhythmogenic right ventricular dysplasia with different varieties according to the degree of dilatation of the right ventricle. In type II, there is a variable degree of left ventricular involvement (30 < EF < 50%) either by extension of a comparable disease process as observed in the right ventricle or by an isolated or superimposed phenomenon of myocarditis. This form is stable and may remain stable for many years providing the arrhythmias are correctly treated. In type III, progressive degradation of the myocardium is observed over a period of about 10 years with a clinical presentation comparable to that of certain arrhythmogenic dilated cardiomyopathies which are often hereditary. In this case, the patients have an arrhythmic risk associated with that of cardiac failure which becomes progressively irreversible. The histology shows interstitial fibrosis with biventricular lymphocytic infiltration suggesting an autoimmune phenomenon. Therefore, the classification of cases of arrhythmogenic right ventricular dysplasia depends on the potential evolutivity of the lesions. When the patient is seen in the early stages of the disease, the prognosis should be garded, especially in a hereditary form.

Outcome of arrhythmogenic right ventricular dysplasia. Apropos of 4 cases

La mise en place d'un stimulateur cardiaque est devenue un acte courant. De nouvelles indications sont en cours d'evaluation. Il faut cependant rappeler qu'il s'agit d'un acte chirurgical avec implantation d'une prothese avec de possibles complications. Cet acte doit donc rester un acte reflechi. Il existe des complications precoces qui surviennent dans les 6 semaines suivant l'implantation. On sous-estime leur incidence (jusqu'a 7 %) et leur gravite. Il existe des complications tardives. Certaines sont responsables d'un dysfonctionnement de la prothese dont le risque est proportionnel a la dependance du patient pour sa stimulation cardiaque definitive. Mais la migration d'une sonde ou la fracture d'une sonde Accufix expose a un risque bien plus important. Les complications veineuses sont oubliees, car generalement asymptomatiques. Un syndrome cave superieur est cependant une complication grave de la stimulation cardiaque. Deux etudes recentes (MOST et DAVID) rappellent l'effet deletere sur l'hemodynamique d'une stimulation ventriculaire droite (VD) inutile. Cette stimulation VD peut avoir un effet pro-arythmique a l'etage ventriculaire pouvant etre responsable de morts subites, mais egalement auriculaire meme si le synchronisme auriculo-ventriculaire est conserve. Les complications infectieuses sont egalement sous-estimees, en partie en raison de la difficulte du diagnostic. Elles mettent en jeu le pronostic vital et imposent l'extraction du materiel implante. En conclusion : il faut savoir que si un patient ne tire pas benefice de son materiel implante, ce meme materiel peut etre deletere. Les stimulateurs doivent etre regles, en particulier, pour eviter les stimulations ventriculaires droites inappropriees.

Stimulateurs cardiaques, défibrillateurs implantables et IRM

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