Mejora progresiva de los umbrales de captura auricular y ventricular, detección e impedancias en los cables de estimulación epicárdica en adultos jóvenes que se someten a conversión de Fontan. / Progressive improvement of atrial and ventricular capture thresholds, sensing, and impedances in epicardial pacing leads in young adults undergoing Fontan conversion.

OBJECTIVES: Patients with univentricular hearts who require permanent pacing systems typically require placement of epicardial leads. It is frequently difficult to find a position with good thresholds due to epimyocardial fibrosis or fat. The goal of the study is to assess the progression of capture thresholds (CT), sensing parameters (P waves and R waves), and impedances (imp) of steroid eluting epicardial pacing leads in young adults who underwent Fontan conversion and a pacemaker implant. METHODS: All patients undergoing Fontan conversion in two institutions were retrospectively identified. Demographic data, congenital heart defects, pacing leads used, and pacing parameters were analyzed at implant, at 6 weeks and 12 months after implant. RESULTS: Twenty patients were identified (twelve males); mean age at conversion was 24.9 ± 5.4 years (range 18-35). Epicardial bipolar steroid eluting leads were used. The site of implant both in the atria and the ventricles varied depending on the parameters. At implant, mean atrial and ventricular impedances were 617 ± 171 Ω and 1061 ± 771 Ω, respectively, mean P wave amplitude was 2 ± 0.7 mV, and mean R wave amplitude was 12.5 ± 7.7 mV. Mean CT was 1.7 ± 0.8 V at 0.5 ms for the atrium and 2.2 ± 1.2 V at 0.5 ms for the ventricle. Ventricular CT and impedance showed an improvement within the first 12 months after implant, with four patients having a decrease in threshold of more than 2 V. CONCLUSION: In patients undergoing Fontan conversion, implant ventricular CT and impedances are frequently higher than expected but typically improve during follow-up. Acceptance of higher initial threshold values may be a potential strategy in this patient population.

OBJETIVO: Los pacientes con corazón univentricular que requieren estimulación cardíaca reciben sistemas de estimulación epicárdicos. Debido a la presencia de fibrosis o grasa epi-miocárdica es dificultoso en esta población encontrar sitios con adecuados parámetros de estimulación. El objetivo de este estudio es determinar la progresión de los umbrales de captura, los parámetros de sensado (medición de las ondas P y R) e impedancias (imp) de los catéteres epicárdicos con liberación de esteroides implantados en adultos jóvenes sometidos a cirugía de reconversión de Fontan e implante de marcapasos. MÉTODOS: Los pacientes sometidos a cirugía de reconversión de Fontan en dos instituciones fueron analizados retrospectivamente. Los datos demográficos, el tipo de cardiopatía congénita, de catéteres de estimulación y los parámetros de estimulación fueron analizados al momento del implante, a las 6 semanas y al año. RESULTADOS: Se identificaron 20 pacientes (12 de ellos de sexo masculino); la edad media al momento de la reconversión fue de 24.9 ± 5.4 años (rango 18-35). Se utilizaron catéteres epicárdicos bipolares de fijación pasiva y con liberación de esteroides en todos los casos. El sitio de implante en las aurículas y en los ventrículos fue variable de acuerdo a los parámetros. En el momento del implante las impedancias medias fueron 617 ± 171 W y 1061 ± 771 W respectivamente, la amplitud media de la onda P fue 2 ± 0.7 mV y la media de amplitud de la onda R fue de 12.5 ± 7.7 mV. Las medias de los umbrales de captura fueron 1.7 ± 0.8 V at 0.5 ms para los catéteres auriculares y 2.2 ± 1.2 V at 0.5 ms para los ventriculares. Los umbrales de captura y las impedancias ventriculares mostraron una mejoría en los 12 meses posteriores al implante, y en 4 pacientes esa mejoría en el umbral de captura ventricular fue mayor a 2 V. CONCLUSIONES: En pacientes sometidos a una cirugía de reconversión de Fontan e implante de marcapasos, los umbrales de captura e impedancias ventriculares son más elevados que los esperados, pero mejoran durante el seguimiento. La aceptación de valores más elevados puede potencialmente constituir una alternativa en esta población de pacientes.

Use of a cardioselective beta-blocker for pediatric patients with prolonged QT syndrome.

The data on the efficacy of atenolol for long-QT syndrome (LQTS) are controversial. This study aimed to evaluate the efficacy of atenolol for pediatric patients with LQTS. A retrospective observational study investigating all patients who had LQTS treated with atenolol at two institutions was performed. The study identified 57 patients (23 boys and 34 girls) with a mean QT corrected for heart rate (QTc) of 521 ± 54 ms. The mean age of these patients at diagnosis was 9 ± 6 years. Their clinical manifestations included no symptoms (n = 33, 58%), ventricular tachycardia (n = 10, 18%), syncope (n = 6, 10%), resuscitated sudden cardiac death (n = 4, 7%), atrioventricular block (n = 2, 4%), and bradycardia or presyncope (n = 2, 3%). Of the 57 patients, 13 (22%) had a family history of sudden death. The follow-up period was 5.4 ± 4.5 years. Atenolol at a mean dose of 1.4 ± 0.5 mg/kg/day was administered twice a day for all the patients. The mean maximum heart rate was 132 ± 27 bpm on Holter monitors and 155 ± 16 bpm on exercise treadmill tests, with medication doses titrated up to achieve a maximum heart rate lower than 150 bpm on both tests. During the follow-up period, one patient died (noncompliant with atenolol at the time of death), and the remaining patients had no sudden cardiac death events. Four patients (8%) had recurrent ventricular arrhythmias, three of whom received an implantable cardioverter defibrillator (all symptomatic at the time of diagnosis). For three patients (6%), it was necessary to rotate to a different beta-blocker because of side effects or inadequate heart rate control. Atenolol administered twice daily constitutes a valid and effective alternative for the treatment of pediatric patients with LQTS.

Radiofrequency ablation of nonautomatic focal atrial tachycardia in children with structurally normal hearts.

INTRODUCTION: The majority of children presenting with paroxysmal supraventricular tachycardia (SVT) have either accessory-pathway-mediated tachycardia or AV node reentry tachycardia. The purpose of this study is to report an unusual mechanism of SVT found in children with structurally normal hearts. METHODS AND RESULTS: Records of all patients undergoing an electrophysiology study (EPS) at our institution between 2000 and 2004 were reviewed to identify those with nonautomatic focal atrial tachycardia (NAFAT). Five patients (three males) with an average age of 13.8 years (median 15 years, range 7-18 years) were identified. All presented with paroxysmal palpitations. They all had structurally normal hearts. At EPS, SVT was reproducibly induced with programmed atrial stimulation (single, double, or triple extrastimuli) in all patients. The average cycle length was 276 +/- 9 ms. Adenosine terminated SVT in 2. A 3-D electro-anatomical system mapping was used in all cases. The right atrium (RA) was mapped in all and the left in two. Foci were mapped to the posterior high RA, lateral RA, lower mid RA septum, inferior to the sinus node, and in the right and left posteroseptal areas. Average number of radiofrequency lesions placed was 8.6 +/- 5. The success rate was 80%; there was one late recurrence. No procedural complications were observed. CONCLUSIONS: NAFAT is a rare form of tachycardia that should be considered in the differential diagnosis of children presenting with SVT. It is amenable to mapping and radiofrequency ablation.

Daño reversible del nodo auriculoventricular durante la crioablación de una vía accesoria parahisiana en un niño con preexcitación intermitente

El daño del nodo auriculoventricular (NAV) constituye una de las potenciales complicaciones de la ablación por radiofrecuencia de vías accesorias parahisianas. La crioterapia, con la potencial reversibilidad a temperaturas no extremas, puede constituir una alternativa en la ablación de estas vías. Un niño con preexcitación intermitente con una vía accesoria parahisiana y crisis de taquicardia paroxística supraventricular fue sometido a un estudio electrofisiológico y la crioablación permitió la eliminación de la vía accesoria con preservación de la integridad del NAV.

Reversible Damage of the Atrioventricular Node during Cryoablation of a para-Hissian Pathway in a Child with Intermittent Pre-excitation Syndrome Damage of the atrioventricular node (AVN) is one of the potential complications in radiofrequency ablation of para- Hissian accessory pathways. Cryotherapy, with reversibility potential at non extreme temperatures, may be an alternative in the ablation of these pathways. A child with intermittent pre-excitation syndrome, para-Hissian accessory pathway and a crisis of supraventricular paroxistic tachycardia was subjected to electrophysiological assessment, and cryoablation allowed removing the accessory pathway with preserved AVN integrity.