A Case of Lown-Ganong-Levine Syndrome: Due to an Accessory Pathway of James Fibers or Enhanced Atrioventricular Nodal Conduction (EAVNC)?

BACKGROUND Lown-Ganong-Levine syndrome, includes a short PR interval, normal QRS complex, and paroxysmal tachycardia. The pathophysiology of this syndrome includes an accessory pathway connecting the atria and the atrioventricular (AV) node (James fiber), or between the atria and the His bundle (Brechenmacher fiber). Similar features are seen in enhanced atrioventricular nodal conduction (EAVNC), with the underlying pathophysiology due to a fast pathway to the AV node, and with the diagnosis requiring specific electrophysiologic criteria. CASE REPORT A 17-year-old man presented with a history of recurrent narrow-complex and wide-complex tachycardia on electrocardiogram (ECG). An electrophysiologic study showed an unusually short atrial to His (AH) conduction interval and a normal His to ventricle (HV) interval, without a delta wave. Two stable AH intervals coexisted in the same atrial pacing cycle length. In the recovery curve study, this pathway had a flat conduction curve without an AH increase until the last 60 ms, before reaching the effective refractory period. These ECG changes did not respond to an adenosine challenge. When this pathway became intermittent, there was a paradoxical response to adenosine challenge with conduction via a short AH interval, but without conduction block. Catheter ablation of the AV nodal region resulted in a normalized AH interval, decremental conduction properties, and resulted in a positive response to an adenosine challenge. CONCLUSIONS In this case of Lown-Ganong-Levine syndrome, electrophysiologic studies supported the role of the accessory pathway of James fibers.

Taquicardia ventricular com QRS relativamente estrecho / Relatively narrow QRS ventricular tachycardia

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Lown-Ganong-Levine syndrome in pregnancy.

BACKGROUND: Lown-Ganong-Levine syndrome is characterized by paroxysmal supraventricular tachycardia, a short PR interval, and normal QRS. CASE: A gravida 3, para 2 was diagnosed with Lown-Ganong-Levine syndrome at 16 weeks' gestation after an episode of chest pain and shortness of breath. She was hospitalized and treated with digoxin, and her symptoms subsided. The remainder of her pregnancy was uneventful. CONCLUSION: Lown-Ganong-Levine Syndrome is a rare complication most commonly seen in young to middle-aged women. This syndrome can be treated and controlled medically with a good outcome.

[Atypical symptoms of Fabry's disease: sudden bilateral deafness, lymphoedema and Lown-Ganong-Levine syndrome]. / Nietypowe objawy choroby Fabry'ego--nagla obustronna utrata sluchu, obrzek limfatyczny i zespól Lowna, Ganonga i Levine'a.

A 40-year-old man with Fabry disease, confirmed by decreased leukocyte alpha-galactosidase A activity in 2001, complained of sudden bilateral deafness, as evidenced by clinical history and audiometry. Magnetic resonance of the brain revealed features typical of Fabry disease. Other clinical manifestations of the disease included: angiokeratoma, mild proteinuria with normal renal function, lymphoedema of the lower limbs, pre-excitation syndrome, myocardial hypertrophy.

Lidocaína: su uso como antiarrítmico / Lidocaine: its use as antiarrhythmic drug

La lidocaína es un agente antiarrítmico de rápida acción, perteneciente a la clase 1-B. Su utilidad ha sido demostrada para el tratamiento de arritmia ventricular. Actualmente la lidocaína tiene indicaciones específicas. La dosificación, farmacocinética e interacciones son revisadas. El diagnóstico diferencial de un complejo QRS ancho es analizado. Se comenta su uso en el embarazo.

Lidocaína: su uso como antiarrítmico / Lidocaine: its use as antiarrhythmic drug

La lidocaína es un agente antiarrítmico de rápida acción, perteneciente a la clase 1-B. Su utilidad ha sido demostrada para el tratamiento de arritmia ventricular. Actualmente la lidocaína tiene indicaciones específicas. La dosificación, farmacocinética e interacciones son revisadas. El diagnóstico diferencial de un complejo QRS ancho es analizado. Se comenta su uso en el embarazo. (AU)

Un nuevo algoritmo electrocardiográfico para la localización de las vías accesorias utilizando únicamente la polaridad del complejo QRS / A new electrocardiographic algorithm for the localization of accessory pathways using only the polarity of the QRS complex

Entre abril de 1992 y junio de 1994, se realizó ablación con radiofrecuencia de vías anómalas en 250 pacientes con taquicardias supraventriculares por reentrada auriculoventricular, la cual fue exitosa en 232 pacientes (93 por ciento). De esta población, estudiamos 102 pacientes con preexitación manifiesta en el electrocardiograma basal. Ninguno de ellos tenía cardiopatías congénitas o adquiridas que pudieran afectar la morfología del complejo QRS. Analizamos unicamente la polaridad del complejo QRS en las derivaciones DIII, V1 y V2, y con esto logramos elaborar un algoritmo para localizar la inserción de la vía accesoria en cinco sitios con un 88 por ciento de probabilidad de éxito. Este análisis electrocardiográfico en pacientes con síndrome de Wolff-Parkinson-White facilita el diagnóstico topográfico de la zona de preexitación y mejora los resultados de la ablación transcatéter con radiofrecuencia

Pulmonary developmental anomaly associated with Klippel-Feil syndrome and anomalous atrioventricular conduction.

We report the findings in a patient with Klippel-Feil syndrome and associated agenesis of right upper and middle lobes, hypoplasia of the right lower lobe of the lung, and Lown-Ganong-Levine syndrome. To our knowledge, such an association has not been previously described.

[The inheritance of the ventricular pre-excitation syndrome]. / Nasledovanie sindroma prezhdevremennogo vozbuzhdeniia zheludochkov.

Medico-genetic studies including preparation of the family pedigree, interviews, examinations, electro- and echocardiography were carried out in the families of 75 patients with pre-excitation syndrome (PES). Of these, 35 patients presented with Wolff-Parkinson-White (WPW) syndrome and 40 with Clerc-Lévy-Cristesco (CLC) syndrome. The studies made in possible to define the autosomal-dominant type of the syndrome or PES inheritance and to diagnose for the first time WPW syndrome in 3, PES in 1, CLC syndrome in 32 and CLC phenomenon in 89 persons out of 233 relatives of the first and second degree kinship.

[Disorders of cardiac rhythm in combined ventricular pre-excitation syndrome and primary mitral valve prolapse]. / Narusheniia serdechnogo ritma pri sochetanii sindromov prezhdevremennogo vozbuzhdeniia zheludochkov i pervichnogo prolabirovaniia mitral'nogo klapana.

Primary prolapse of the mitral valve was diagnosed in 27 (11.7%) out of 230 patients with preexcitation syndrome: 10 of them presented with Wolff-Parkinson-White syndrome, 15 with Clerc-Lévy-Critesco syndrome, and two patients with combination of both syndromes. Preexcitation syndrome combined with mitral valve prolapse manifested itself by different disorders of the cardiac rhythm, mainly by the supraventricular forms: by PST in 17, nonparoxysmal tachycardia in 1, atrial fibrillation paroxysms in 7, sinus tachycardia in 2, supraventricular extrasystole in 3, pacemaker migration in the atria in 1 patient. Clerc-Lévy-Critesco syndrome initiated with atrial fibrillation paroxysms in 7 patients with mitral valve prolapse and with the enlargement of the left atrium. The prognostically unfavourable varieties of the preexcitation syndrome and mitral valve prolapse manifested themselves in ventricular tachycardia progressing to ventricular fibrillation, paroxysmal supraventricular tachycardia with a high frequency of ventricular contractions up to 213-230 and atrial tachyarrhythmia up to 250-280 per minute, which were regarded as factor at risk for potential transformation to ventricular tachycardia; as well as in continuously recurrent attacks of arrhythmia in the presence of multiple AV additional tracts or combined with sinoatrial and atrioventricular block.

[Clinical picture of pathological conditions with electrocardiographic features of the LGL syndrome]. / Obraz kliniczny zespolów chorobowych przebiegajacych z elektrokardiograficznymi cechami zespolu LGL.

Three cases of the LGL syndrome are reported. In the first case the syndrome was associated with hyperthyroidism, in the second case ECG abnormalities suggested focal myocardial ischaemia, in the third case pre-excitation coexisted with combined valvular heart disease. Attention is called to frequent occurrence of the pre-exitation syndrome with certain disease entities.