Cases reported "Pre-Excitation Syndromes"

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1/17. Fifteen-year follow-up of a heterozygous Fabry's disease patient associated with pre-excitation syndrome.

    A 47-year-old woman with heterozygous Fabry's disease with pre-excitation syndrome has been followed up for 15 years. diagnosis was confirmed by the typical electron microscopic feature of the endomyocardial specimen and a decreased plasma alpha-galactosidase activity. As the disease progressed, the interventricular septum thickened from 11 to 17 mm as measured by echocardiography, while the AH interval was prolonged from 80 to 140 msec. In Fabry's disease, the PR interval has been reported to be variable from short PR to AV block. Therefore, this case may be helpful to understand the time course in the AV conduction abnormalities with the progression of Fabry's disease.
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2/17. Concealed accessory pathway manifesting clinically only after pacemaker implantation.

    A 66-year-old man with coronary artery disease and persistent left superior vena cava received a DDDR pacemaker for symptomatic 2:1 heart block. There was no previous history of tachyarrhythmias. Endless loop tachycardia and repetitive nonreentrant ventriculoatrial synchrony occurred afterwards and were triggered by a late coupled atrial premature beat. ECGs suggested a concealed left posterior accessory pathway that was confirmed during electrophysiological study. Effective palliation was achieved with extension of the PVARP and enabling noncompetitive atrial pacing operation.
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3/17. syncope and preexcitation: a case of a Kent and fasciculoventricular Mahaim fibers.

    A case involving an 8-year-old girl with syncope and preexcitation on a surface electrocardiogram (ECG) that was suggestive of wolff-parkinson-white syndrome is presented. An intracardiac electrophysiologic study revealed a posteroseptal bidirectionally conducting Kent fiber. Radiofrequency ablation of the Kent fiber was successful, but the patient had a residual short His-ventricular (HV) interval and a new preexcitation pattern. Atrial extra stimuli and ventricular pacing revealed a fixed, preexcited QRS. Nodal block and loss of preexcitation was provoked with adenosine. The surface QRS and electrophysiologic features are consistent with a left septal fasciculoventricular Mahaim fiber.
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4/17. 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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5/17. Congenital complete atrioventricular block and preexcitation syndrome: a well-matched partner.

    A patient who had been previously diagnosed with congenital complete atrioventricular block (CCAVB) twenty years ago developed atrioventricular (AV) conduction through an accessory pathway (AP). With enhanced sympathetic tone (exercise, isoproterenol), 1:1 conduction down the AP occurred. An electrophysiologic study confirmed a suprahissian AV block and the presence of an AP. The AP was located on the left side and posterior. The absence of retrograde conduction through the AP and also a long conduction time were demonstrated.
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6/17. 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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7/17. Demonstration of accessory pathway interaction by computerized mapping in preexcitation syndrome.

    While interaction between the normal conduction system and an accessory pathway (AP) has been reported, interaction between two APs has not been well documented. With the assistance of computerized mapping techniques, we recently operated on a patient with two APs. One of these two APs had slow anterograde conduction velocity and was concealed during sinus rhythm and atrial pacing. Intraoperative computerized mapping studies revealed that the interaction between the APs was responsible for the anterograde conduction block of the slow AP during sinus rhythm and atrial pacing. This is the first direct demonstration of inhibition of conduction of one AP by the other AP in a patient with preexcitation syndrome.
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8/17. Coexistence of kent accessory pathway, enhanced AV node conduction, and various conduction disturbances in a young athlete with tricuspid valve dysplasia.

    An asymptomatic 19-year-old top-level athlete had electrocardiographic evidence of intermittent cardiac preexcitation and intermittent left bundle branch block. The electrophysiologic study demonstrated the presence of a direct accessory pathway and enhanced atrioventricular node conduction that resulted in infrahisian and intraventricular conduction disturbances. The echocardiogram disclosed tricuspid valve dysplasia.
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9/17. Intermittent preexcitation: marked enhancement of anterograde conduction in the atrioventricular accessory pathway with isoproterenol.

    A 29-year-old man presented with intermittent preexcitation and exertional syncope. Electrophysiological evaluation in the control state demonstrated a single, left posterior free-wall atrioventricular accessory pathway. The anterograde and retrograde effective refractory periods and block cycle lengths were long in the control state. No tachycardias were induced during programmed electrical stimulation. After intravenous administration of isoproterenol, anterograde conduction of the accessory pathway was markedly enhanced (block cycle length shortened 45% to less than 240 ms) and rapid antidromic reciprocating tachycardia (CL = 250 ms) associated with syncope was observed. Following successful surgical dissection of the accessory pathway the patient has been without tachycardia or exertional symptoms. We conclude that, under the influence of exercise or emotion, patients with intermittent preexcitation may be at risk for serious arrhythmias.
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10/17. Narrow QRS complex tachycardia with atrioventricular dissociation.

    We describe the case of a 22-year-old man who had frequent episodes of narrow QRS complex tachycardia with atrioventricular dissociation. The ECG during sinus rhythm showed normal PR and QRS intervals, but it showed a left bundle branch block configuration during atrial pacing or after injection of verapamil. An electrophysiological study demonstrated that the patient had nodoventricular Mahaim fibers. The narrow QRS complex tachycardia was explained by a circuit involving antegrade conduction via the atrioventricular nodo-His axis and retrograde conduction via the nodoventricular bypass tract.
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