Cases reported "Pre-Excitation Syndromes"

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1/8. Pre-excitation syndrome secondary to cardiac rhabdomyomas in tuberous sclerosis.

    Rhabdomyomas are not uncommon in infants with tuberous sclerosis. We describe a neonate who presented with hydrops fetalis arising from a tachyarrhythmia during fetal life related to rhabdomyomas. After reversion of the arrhythmia, pre-excitation was noted on an interval electrocardiogram. Following regression of the tumours, the delta wave disappeared with no further arrhythmias noted.
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2/8. The prominant T wave: electrocardiographic differential diagnosis.

    The prominent T wave is an abnormal T-wave morphology encountered in the earliest phase of ST-segment elevation acute myocardial infarction (AMI). Prominent T waves, however, are associated with other diagnoses, including hyperkalemia, early repolarization, and left ventricular hypertrophy (LVH). This article focuses on the electrocardiographic differential diagnosis of the prominent T wave with the presentation of 4 illustrative cases. We also recommend that the designation hyperacute should refer exclusively to the prominent T waves of ST-segment elevation AMI.
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3/8. Electrocardiographic T-wave inversion: differential diagnosis in the chest pain patient.

    Inverted T waves produced by myocardial ischemia are classically narrow and symmetric. T-wave inversion (TWI) associated with an acute coronary syndrome (ACS) is morphologically characterized by an isoelectric ST segment that is usually bowed upward (ie, concave) and followed by a sharp symmetric downstroke. The terms coronary T wave and coved T wave have been used to describe these ischemic TWIs. Prominent, deeply inverted, and widely splayed T waves are more characteristic of non-ACS conditions such as juvenile T-wave patterns, left ventricular hypertrophy, acute myocarditis, wolff-parkinson-white syndrome, acute pulmonary embolism, cerebrovascular accident, bundle branch block, and later stages of pericarditis.
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4/8. Intermittent noninfarction Q waves: a finding suggestive of latent preexcitation.

    OBJECTIVE: To describe 3 patients who presented with chest pain and intermittent Q waves on the electrocardiogram (ECG) and were subsequently found to have latent preexcitation. patients AND methods: During a span of 8 years, 3 patients were evaluated because of atypical chest pain and pathologic Q waves in the inferior leads; in all 3 patients, the Q waves were intermittent. No patient had a history of arrhythmia or had Wolff-Parkinson-White pattern on the ECG. Diagnostic and therapeutic interventions for suspected myocardial infarction included cardiac catheterization in 2 patients, intravenous thrombolytic therapy in 1 patient, and heparin in 2 patients. Ischemic heart disease was excluded in all. patients underwent pharmacological testing and/or electrophysiologic study for suspected preexcitation. RESULTS: Despite the absence of ECG markers of preexcitation, the presence of a latent accessory atrioventricular connection was confirmed in each patient by pharmacological or electrophysiologic studies. CONCLUSION: In patients who present with intermittent noninfarction Q waves, the most likely diagnosis is latent preexcitation. Clinicians need to be educated about this clinical diagnosis and encouraged to pursue confirmatory testing. Such patients should be informed about the nature and importance of their electrocardiographic abnormality.
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5/8. Complete AV block developing in a patient with manifest ventricular preexcitation.

    This case report describes a 37-year-old man with manifest atriofascicular and fasciculoventricular connections who developed complete atrioventricular block. After resumption of 1:1 atrioventricular conduction, an electrophysiological study was conducted. During sinus rhythm, manifest ventricular preexcitation was observed and the AH and HV intervals were 80 msec and 50 msec, respectively. No change in AH interval during constant atrial pacing was observed by bolus injection of 20 mg of adenosine triphosphate. No changes in the morphology of the delta wave or HV interval were observed by changing the atrial pacing site. This patient developed HV block by atrial extrastimulus (S1S1: 600 msec, S1S2
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6/8. Supraventricular tachycardia in a patient with lown-ganong-levine syndrome associated with apical hypertrophic cardiomyopathy.

    Electrophysiologic study of a 55-year-old patient with lown-ganong-levine syndrome associated with apical hypertrophic cardiomyopathy is reported. The patient had a history of recurrent attacks of tachyarrhythmia and his electrocardiogram showed a short P-R interval (0.10 sec) with narrow QRS complex and left ventricular hypertrophy with giant negative T waves. His cineangiogram showed severe apical hypertrophy. An electrophysiologic study was performed. The results of programmed atrial pacing show the existence of the dual A-V nodal pathways. The A-H interval at rapid atrial pacing increased maximally by 103 msec. Atrial stimulation could depolarize parts of the atrium without altering the supraventricular tachycardia. These findings suggested that preferential rapidly conducting A-V nodal and intranodal reentry are the responsible mechanisms in this reciprocating tachycardia. We conclude that the short P-R interval was due to intranodal reentry through the dual A-V nodal pathways. To our knowledge, a case of lown-ganong-levine syndrome with apical hypertrophic cardiomyopathy has not been previously described in the literature.
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7/8. ventricular fibrillation resulting from synchronized internal atrial defibrillation in a patient with ventricular preexcitation.

    This case describes ventricular proarrhythmia as a result of a synchronized internal atrial defibrillation shock in a 29-year-old man with Ebstein's anomaly referred for radiofrequency ablation of a right posterior accessory pathway. During the electrophysiologic study, atrial fibrillation was induced and 3/3 msec shocks of various strengths were delivered between two decapolar defibrillation catheters in the coronary sinus and right atrial appendage. A 2.0-J biphasic shock synchronized to an R wave after a short-long-short ventricular cycle length pattern with a preshock coupling interval of 245 msec induced ventricular fibrillation, which was externally defibrillated with 200 J. This observation has implications for the development of implantable atrial defibrillators.
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8/8. Benign early repolarization: electrocardiographic manifestations and differentiation from other ST segment elevation syndromes.

    Early repolarization, also known as benign early repolarization (BER) or normal variant, is noted in approximately 1% of the population and in up to 48% of patients seen in the emergency department with chest pain. BER represents a benign variant of the normal electrocardiogram and is one of several syndromes producing electrocardiographic ST segment elevation. BER electrocardiographically includes diffuse or widespread ST segment elevation, upward concavity of the initial portion of the ST segment, notching or slurring of the terminal QRS complex, and concordant T waves of large amplitude. This article focuses on BER and includes a discussion of the electrocardiographic tools useful in making this diagnosis and in distinguishing BER from other ST segment elevation syndromes.
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