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The study reports a girl with pyridoxine-dependent epilepsy. The girl was admitted at the age of 2 years because of intermittent convulsions for 1.5 years and psychomotor retardation. She had a history of "hypoxia" in the neonatal period. At the age of 5 months recurrent epileptic seizures occurred. The child was resistant to antiepileptic drugs, and had many more seizures when she got cold or fever. She also had a lot of convulsive status epilepticus. No discharges were found during several video-EEG monitorings. Cerebral MRI examinations showed normal results. So Dravet syndrome was clinically suspected. ALDH7N1 gene mutation analysis revealed two heterozygote mutations, and pyridoxine-dependent epilepsy was thus confirmed. Seizures were generally controlled after pyridoxine supplementation.
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Preescolar , Femenino , Humanos , Aldehído Deshidrogenasa , Genética , Epilepsia , Mutación , Trastornos Psicomotores , ConvulsionesRESUMEN
<p><b>BACKGROUND</b>Transcatheter closure of congenital coronary artery fistulas (CCAFs) is an alternative therapy to surgery; however, data regarding transcatheter closure for CCAF with a giant coronary artery aneurysm (CAA) in pediatric patients are still limited due to the rarity of the disease. We aimed to evaluate the efficacy and safety of transcatheter closure for CCAF with a giant CAA in a pediatric population at a single center.</p><p><b>METHODS</b>Medical records of pediatric patients (<18 years old) who underwent transcatheter closure of CCAF with a giant CAA between April 2007 and September 2016 at Guangdong Cardiovascular Institute (Guangdong, China) were reviewed.</p><p><b>RESULTS</b>Twelve patients (median age, 6.1 years; range, 1.9-11.0 years) underwent successful transcatheter closure procedures. One patient underwent closure at both the entry and exit points of the CAA, three patients underwent closure at the exit point of the CAA, and eight patients underwent closure at the entry point of the CAA. After a mean follow-up of 7.2 years (range, 0.5-9.8 years), one patient (with closure at the exit point of the CAA) underwent transcatheter re-intervention because of a significant residual shunt. She eventually underwent a surgical procedure due to aneurysm dilation after the second intervention. One patient experienced thrombus formation within the CAA after the procedure. Among those with closure at the entry point of the CAA, a mild-to-moderate residual shunt was detected in three patients.</p><p><b>CONCLUSIONS</b>Transcatheter closure appears to be a safe and effective alternative therapy for CCAF with a giant CAA in the pediatric population. Closure at the entry point of the CAA, and closure at both the entry and exit points when feasible, may reduce the risk of postinterventional complications.</p>
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The genus Xestospongia is one of the most widespread genera of sponges, containing abundant secondary metatolites with novel structures and potent bioactivities. The main structure types of secondary metatolites found in this genus are alkaloids, quinines, terpens, steroids, lipids, polyketones, etc. These metatolites exhibit a variety of bioactivities, such as cytotoxic, antibacterial and antiviral activities. This paper reviews the progress in the chemistry and pharmacological activities of the second metabolities from sponges of Xestospongia, especially for recent five years, with the aim for further research.
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Animales , Metabolismo Secundario , Xestospongia , QuímicaRESUMEN
<p><b>OBJECTIVE</b>To evaluate the adrenocortical function in children with severe and critical enterovirus 71 infection by using a high-dose (250 µg) adrenocorticotropic hormone (ACTH) stimulation test. And to at provide experimental basis for glucocorticoid in the treatment of hand-foot-and-mouth disease (HFMD).</p><p><b>METHOD</b>This was a prospective multi-center study which was carried out in PICUs of Beijing Children's Hospital, Zhengzhou Children's Hospital, Kaifeng Children's Hospital and Linyi People's Hospital in Shandong province. Children with severe and critical hand-foot-mouth disease admitted to PICUs of the four hospitals from June 2009 to April 2010 were enrolled in this study, and EV71 virus nucleic acid test and high-dose (250 µg) ACTH stimulation started at the same time. EV71 virus nucleic acid positive 51 cases were eventually enrolled in the study. Cortisol test was performed at baseline (T0) and after high-dose (250 µg) ACTH stimulation at 30 minutes (T30), 60 minutes (T60) in the first 6 hours after admission, but before glucocorticoid was given. The adrenocortical function was evaluated according to ΔTmax [ΔTmax=(T30, T60 maximum)-T0]. Diagnostic criteria of adrenal insufficiency (AI) is increment (ΔTmax)≤9 µg/dl.</p><p><b>RESULT</b>The incidence of AI in 51 cases was 52.94% (27/51). The incidence of AI in severe group was 44.74% (17/38), which was significantly higher in critical group 76.92% (10/13), P<0.05. Of the cases with a pediatric critical illness score (PCIS)≤70, 81.82% (9/11) had adrenal insufficiency, and it was 28.57% (4/14) when PCIS≥90. The incidence of AI was 75% (6/8) and 48.84% (21/43) in death and survivor group respectively, but there were no significant difference between the two groups (P>0.05). Baseline (T0) cortisol in death group was higher than survivor group (P<0.05).</p><p><b>CONCLUSION</b>AI may occur in children with enterovirus 71 infection. The critical enterovirus 71 infection had a high incidence of AI. AI may affect the prognosis of patients with severe and critical enterovirus 71 infection. Exogenous glucocorticoids administration may be considered when AI is identified or highly suspected. The timing, dosage and regimen of glucocorticoid are still unclear. Further animal experiments and clinical trials are needed.</p>
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Preescolar , Femenino , Humanos , Lactante , Masculino , Insuficiencia Suprarrenal , Quimioterapia , Hormona Adrenocorticotrópica , Usos Terapéuticos , Enterovirus Humano A , Virulencia , Enfermedad de Boca, Mano y Pie , Quimioterapia , Virología , Pronóstico , Estudios ProspectivosRESUMEN
<p><b>OBJECTIVE</b>To assess the safety and efficacy of transcatheter intervention for critical pulmonary stenosis (CPS) and pulmonary atresia with intact ventricular septum (PA/IVS) in neonates.</p><p><b>METHOD</b>From June 2006 to September 2011, 27 neonates including CPS in 19 patients and PA/IVS in 8 patients underwent transcatheter intervention. All patients had membranous stenosis or atresia without severe Ebstein's anomaly and severe right ventricle and pulmonary valve hypoplasia, without right ventricle-dependent coronary circulation in PA/IVS. The mean age was (16.8 ± 9.9) d. The mean weight was (3.3 ± 0.5) kg. Two of them were premature neonates, the weight was 2.3 kg and 2.5 kg, respectively. The procedural success, early outcome, complication rates, midterm results and pulmonary regurgitation were retrospectively studied.</p><p><b>RESULT</b>Twenty-six patients were successfully treated with transcatheter intervention. Right ventricular pressure fell from (112.0 ± 21.0) mm Hg (1 mm Hg = 0.133 kPa) to (50.4 ± 15.9) mm Hg (P < 0.001). The ratio of right ventricular pressure and aortic pressure fell from 1.7 ± 0.1 to 0.7 ± 0.3 (P < 0.001). One patient died early of PA/IVS. Complication occurred in 5 patients. Hemopericardium occurred in 3 patients, tachyarrhythmia in 2 patients. Five patients needed prolonged prostaglandin E(1) infusion for 3 to 14 days because of desaturation after the procedure. No patient needed surgery in neonatal period. At a mean follow-up of (33.5 ± 18.3) months (from 6 months to 5 years), 21 patients had no further transcatheter or surgical intervention. Four patients with CPS had moderate to severe residual pulmonary stenosis after the procedure, 3 of them underwent a second balloon dilation at 3 months of follow-up, the other one was waiting for the second balloon dilation. One patient with PA/IVS was waiting for a bidirectional Glenn procedure because of chronic right ventricular failure. Mild pulmonary regurgitation occurred in 18 patients (69.2%), and moderate pulmonary regurgitation in 8 patients (30.8%).</p><p><b>CONCLUSION</b>Transcatheter intervention for CPS and PA/IVS in neonates is safe and effective. It can avoid neonatal surgery. Some patients may require repeat balloon valvuloplasty in infant period. In most patients surgical or transcatheter intervention could be avoided and mild pulmonary regurgitation was the common finding in midterm follow-up.</p>
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Femenino , Humanos , Recién Nacido , Masculino , Cateterismo , Métodos , Estudios de Seguimiento , Cardiopatías Congénitas , Cirugía General , Atresia Pulmonar , Cirugía General , Válvula Pulmonar , Cirugía General , Estenosis de la Válvula Pulmonar , Cirugía General , Reoperación , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
<p><b>OBJECTIVE</b>To investigate the effects of sIL-5Rα and sIL-13Rα2 on VCAM-1 and IFN-γ in allergic rhinitis rats.</p><p><b>METHODS</b>A total of 50 Wistar rats were randomly divided into 5 groups: the normal group (group A), the allergic rhinitis model group (group B), the sIL-5Rα treatment group (group C), the sIL-13Rα2 treatment group (group D), the combination of sIL-5Rα and sIL-13Rα2 treatment group (group E or the combined treatment group). Rats in the latter 4 groups were sensitized with ovalbumin (OVA) and Al(OH)(3), and challenged with OVA to establish allergic rhinitis models, while rats in the normal group were treated with saline. Rats in the sIL-5Rα treatment group, the sIL-13Rα2 treatment group and the combined treatment group were absorbed on day 31 to day 38 once daily once nasal cavity with sIL-5Rα(100 µg), sIL-13Rα2 (100 µg) and the combination of sIL-5Rα (100 µg) and sIL-13Rα2 (100 µg) 30 min before challenged, while rats in the allergic rhinitis model group received PBS(50 µl). Then the levels of VCAM-1 and IFN-γ in serum and nasal lavage fluid (NLF) were measured by ELISA.</p><p><b>RESULTS</b>Compared with the normal group, the levels of VCAM-1 in the allergic rhinitis model group were higher, while IFN-γ were lower (all P < 0.01). Compared with the allergic rhinitis model group, the sIL-5Rα treatment group, the sIL-13Rα2 treatment group and the combined treatment group could effectively reduced serum and NLF VCAM-1 level [group E: (283.5 ± 5.7) µg/L, (101.8 ± 4.8) µg/L; group C: (311.5 ± 12.6) µg/L, (133.9 ± 5.8) µg/L; group D: (304.7 ± 6.6) µg/L, (128.5 ± 7.7) µg/L], and increased IFN-γ level [group E: (874.7 ± 9.6) pg/ml, (349.2 ± 12.1) pg/ml; group C: (600.2 ± 16.1) pg/ml, (195.5 ± 16.1) pg/ml; group D: (577.9 ± 9.6) pg/ml, (196.7 ± 9.9) pg/ml ]; compared with single treatment, the combined treatment group also had significant differences(P < 0.01).</p><p><b>CONCLUSIONS</b>Combined treatment with sIL-5Rα and sIL-13Rα2 to treat the allergic rhinitis rats can significantly reduce VCAM-1 levels in serum and NLF, and increase IFN-γ levels, thus, to achieve the purpose of mitigation and treatment of allergic rhinitis.</p>
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Animales , Masculino , Ratas , Interferón gamma , Sangre , Ratas Wistar , Receptores de Interleucina-13 , Usos Terapéuticos , Receptores de Interleucina-5 , Usos Terapéuticos , Rinitis Alérgica , Rinitis Alérgica Perenne , Metabolismo , Terapéutica , Molécula 1 de Adhesión Celular Vascular , SangreRESUMEN
<p><b>OBJECTIVE</b>To report the results of transcatheter interventional therapy (TIT) of congenital heart disease (CHD) register from 23 medical centers in China.</p><p><b>METHOD</b>In this retrospective multicenter registry study, clinical data from 5808 patients who underwent TIT between January 2008 to December 2010 in 23 Chinese medical centers in 14 cities were analyzed.</p><p><b>RESULTS</b>Procedure was successful in 5720 cases (98.5%), success rate was 99.5% for PDA, 98.8% for ASD, 97.4% for VSD and 98.5% for pulmonary stenosis (PS). Multivariate regression analysis showed that PDA size and procedure time, age and procedure time, distance from VSD to AV were significantly associated with the procedure success rate of PDA, ASD and VSD closure, respectively. Early complications occurred in 306 cases (5.3%), 36 cases (0.6%) experienced major complications including device embolization in 7 cases, serious aorta regurgitation in 5 cases, serious tricuspid regurgitation in 4 cases, tricuspid stenosis in 2 cases, heart block (HB) in 13 cases (2 in ASD and 11 in VSD), cardiac tamponade in 2 cases (1 ASD and 1 PS) and hemolysis in 3 cases. Procedure time and PDA size, ASD size, device size, age and PS degree were risk factors related to the occurrence of the early complications for PDA, ASD and VSD closure and PBPV respectively. The median follow-up time was 15 months (range 1-36 months). The complete closure rate during follow up was 100% for ASD, PDA and VSD and the pressure gradient in PS decreased to normal range in all PS patients. Late complications occurred in 15 cases (0.2%), of which 3 cases needed surgery intervention and permanent pacemaker was implanted in 1 patient. There was no death during procedure and at follow-up period.</p><p><b>CONCLUSIONS</b>TIT of CHD offers encouraging results in China. Follow up is warranted to monitor the occurrence of serious complications, especially late complications.</p>
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Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Persona de Mediana Edad , Adulto Joven , Cateterismo Cardíaco , China , Epidemiología , Conducto Arterioso Permeable , Cirugía General , Cardiopatías Congénitas , Epidemiología , Cirugía General , Defectos del Tabique Interatrial , Cirugía General , Defectos del Tabique Interventricular , Cirugía General , Estenosis de la Válvula Pulmonar , Cirugía General , Sistema de Registros , Estudios RetrospectivosRESUMEN
<p><b>OBJECTIVE</b>To retrospectively summarize the effect of non-medical therapies for pediatric patients with hypertrophic obstructive cardiomyopathy (HOCM).</p><p><b>METHODS</b>From Nov. 2008 to Jun. 2010, 4 children with drug-refractory HOCM were admitted to our hospital. Their ages were 14, 7, 9 and 6 years old, respectively. Their body weights were 38, 17, 21.5 and 17 kg, respectively. Before operation, the pressure gradients over left ventricular outflow tract (LVOTG) were 60, 147, 58 and 114 mm Hg (1 mm Hg = 0.133 kPa), respectively. And mitral regurgitation (MR) areas were 2.2, 7.3 cm(2) and 2.9 cm(2), respectively, except that it was trivial in one case. Percutaneous transluminal septal myocardial ablation (PTSMA) was performed in case 1 and 2. Septal myectomy (SM) was performed in case 3 and 4. Follow-up was first performed right after operation or before discharge, then 1 month, 3 months, 6 months, and 12 months after operation, and then once a year. The follow-up period was 1 - 18 (9.3 ± 8.1) months.</p><p><b>RESULTS</b>All patients experienced relieved symptoms. Three of them had their NYHA functional class improved except case 2. Echocardiography revealed that LVOTGs right after operations were 38, 79, 20 and 0 mm Hg, respectively, suggesting significant improvement of left ventricular outflow tract obstruction (LVOTO) in all patients. During follow-up, case 2 suffered from recurrence of LVOTO, while the other 3 cases showed sustained relief. In the last follow-up, the LVOTGs of the four patients were 19, 168, 16 and 0 mm Hg, respectively. Echocardiography also revealed that MRs of all patients were significantly reduced, even in case 2 whose LVOTG rebounded, with no recurrence during follow-up. Severe complications were absent, such as ventricular septum perforation, cardiac tamponade, ventricular tachycardia or ventricular fibrillation. No one suffered from complete heart block. Transient complete right bundle branch block (CRBBB) was observed in case 1 after PTSMA and converted to intraventricular block after 1 month. Complete left bundle branch block (CLBBB) was present in both case 3 and 4, who received SM. In case 4, it converted to intraventricular block after 1 month while in case 3 CLBBB persisted.</p><p><b>CONCLUSIONS</b>The initial experience showed that PTSMA and SM were safe and effective for drug-refractory symptomatic HOCM children, with satisfactory short-term results. Further studies are needed to evaluate the long-term results and complications.</p>
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Adolescente , Niño , Femenino , Humanos , Masculino , Procedimientos Quirúrgicos Cardíacos , Cardiomiopatía Hipertrófica , Cirugía General , Terapéutica , Ablación por Catéter , Estudios de SeguimientoRESUMEN
<p><b>OBJECTIVE</b>To evaluate the efficacy of interventional therapy for complex congenital heart defects patients with un-repaired or postoperative residual lesions.</p><p><b>METHODS</b>Between March 1998 and April 2009, 42 patients (12 females), mean age 6 years (5 months to 30 years) received interventional therapy, 17 cases underwent occlusion of major aorto-pulmonary collateral arteries (MAPCAs), 15 underwent device closure of residual ventricular or atrial septal shunting, 12 underwent balloon angioplasty (n = 10) and stenting (n = 2) for stenosis of the anastomosis of vessels or branched pulmonary arteries.</p><p><b>RESULTS</b>Twenty-three MAPCAs were performed in 17 patients without residual shunting. One patient died of multiple organ failure after intervention therapy and the remaining patients discharged without complication, successful device closure was performed in 15 patients and there was minimal residual shunting in 1 patient. There were no severe arrhythmias such as complete atrio-ventricular block during and post procedure. Exercise capacities were significantly improved in 12 patients underwent balloon angioplasty or stenting. Pressure gradients were significantly decreased and there was no aneurysmal or thromboembolic formation post procedure.</p><p><b>CONCLUSIONS</b>Interventional therapy is a safe and effective therapy option for treating complex congenital heart defects patients with un-repaired or postoperative residual lesions.</p>
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Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Adulto Joven , Cateterismo Cardíaco , Cardiopatías Congénitas , Cirugía General , Terapéutica , Periodo PosoperatorioRESUMEN
<p><b>OBJECTIVE</b>To summarize the experience in diagnosis and treatment of arrhythmogenic right ventricular cardiomyopathy (ARVC) in children.</p><p><b>METHODS</b>A total of 14 children (7 females and 7 males) with ARVC were involved. The cases underwent electrocardiography, echocardiography, cardiac CT or MRI examinations. All cases were treated with sotalol or amiodarone in combination with propranolol. In 2 cases with drug-refractory ventricular extrasystoles, catheter ablation treatment was performed. In 6 cases with obvious impaired ventricular function, additional pharmacological therapy including vasodilators, diuretics, and digitalis were given.</p><p><b>RESULTS</b>Ventricular extrasystoles occurred in all 14 cases and ventricular tachycardia in 8 cases. Ten cases showed Epsilon wave on electrocardiography. All 14 cases had enlarged right ventricle and reduced right ventricular ejection fraction. CT or MRI examination showed right ventricular dilatation and a thinned wall of right ventricle in 10 cases. Ventricular extrasystoles or tachycardia disappeared in 7 cases and was reduced in 4 cases after treatment. The two children receiving catheter ablation treatment did not present ventricular extrasystoles or tachycardia in a 3-month follow-up. The heart function was improved in the 6 children with obvious impaired ventricular function after pharmacological therapy.</p><p><b>CONCLUSIONS</b>The clinical manifestations are diverse in children with ARVC. A definite diagnosis of ARVC should be based on a combination of electrocardiography and echocardiography examinations. Pharmacological therapy is effective partially. Catheter ablation treatment appears to be a promising option in patients with drug-refractory ventricular extrasystoles.</p>
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Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Displasia Ventricular Derecha Arritmogénica , Diagnóstico , Quimioterapia , Ecocardiografía , Electrocardiografía , Tomografía Computarizada por Rayos XRESUMEN
<p><b>OBJECTIVE</b>To simplify the methods of transcatheter mapping and ablation in the pediatric patients with left posterior fascicular tachycardia.</p><p><b>METHOD</b>While in sinus rhythm, the fascicular potential can be mapped at the posterior septal region (1 - 2 cm below inferior margin of orifice of coronary sinus vein), which display a biphasic wave before ventricular wave, and exist equipotential lines between them. When the fascicular potential occurs 20 ms later than the bundle of His' potential, radiofrequency was applied. Before applying radiofrequency, catheter position must be observed using double angle viewing (LAO 45°RAO 30°), and it should be made sure that the catheter is not at His' bundle. If the electrocardiogram displays left posterior fascicular block, the correct region is identified and ablation can continue for 60 s. Electrocardiogram monitoring should continue for 24 - 48 hours after operation, and notice abnormal repolarization after termination of ventricular tachycardia. Aspirin [2 - 3 mg/(kg·d)] was used for 3 months, and antiarrhythmic drug was discontinued. Surface electrocardiogram, chest X-ray and ultrasound cardiography were rechecked 1 d after operation. Follow-up was made at 1 month and 3 months post-discharge. Recheck was made half-yearly or follow-up was done by phone from then on.</p><p><b>RESULT</b>Fifteen pediatric patients were ablated successfully, and their electrocardiograms all displayed left posterior fascicular block after ablation. None of the patients had recurrences during the 3 to 12 months follow-up period. In one case, the electrocardiogram did not change after applying radiofrequency ablation and the ventricular tachycardia remained; however, on second attempt after remapping, the electrocardiogram did change. The radiofrequency lasted for 90 seconds and ablation was successful. This case had no recurrences at 6 months follow-up.</p><p><b>CONCLUSION</b>Transcatheter ablation of the fascicular potential in pediatric patients with left posterior fascicular tachycardia can simplify mapping, reduce operative difficulty and produce a distinct endpoint for ablation.</p>
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Adolescente , Niño , Femenino , Humanos , Masculino , Bloqueo de Rama , Cirugía General , Ablación por Catéter , Métodos , Electrocardiografía , Taquicardia Ventricular , Cirugía GeneralRESUMEN
<p><b>OBJECTIVE</b>To explore safety, indications and advantages of mapping and ablation of arrhythmia in children guided by Carto and Ensite system.</p><p><b>METHODS</b>Guided by Carto system, radiofrequency catheter ablation (RFCA) was performed on 8 pediatric patients with tachycardia whose mean age was (6.2 + or - 1.7) years, mean weight was (18.0 + or - 2.0) kg. Guided by Ensite system, RFCA was performed on 10 pediatric patients with arrhythmia, 8 of them were ablated guided by Ensite Array system: 6 cases with premature ventricular contractions (PVCs), 2 cases with right atrial tachycardia, their mean age was (11.3 + or - 1.2) years, and mean weight (40.0 + or - 5.0) kg. The other two cases with W-P-W syndrome were ablated guided by Ensite Navx system.</p><p><b>RESULT</b>Guided by Carto system, 8 cases were successfully mapped and ablated: 6 cases had incision atrial tachycardia, 1 case had left atrial tachycardia and 1 case had right atrial tachycardia. In 1 case with incision atrial tachycardia the condition recurred after 3 months, and was ablated again successfully. Guided by Ensite Array system, 6 cases with PVCs (in 2 originating from the right ventricular inflow tract and in 4 originating from the right ventricular outflow tract) and 2 cases with right atrial tachycardia were successfully mapped and ablated, PVCs of the first 6 cases were reduced from (32 333 + or - 4509) 24 h to (0-4)/24 h after ablation. In 1 case with automatic atrial tachycardia, mapping could not be done by Ensite Array system, because P wave could not be identified from T wave. Single bolus of adenosine 20 mg was given within 30 s to let ventricles stop for 2 s (cardio-ventricular pacing standby) until T wave vanished, mapping and ablation were operated again successfully, but another atrial tachycardia occurred 1 day later. Guided by Ensite Navx system, 2 cases with W-P-W syndrome were successfully ablated, operation under X-rays lasted for 8 and 10 min. In none of the 9 patients the disease recurred after follow-up for 6 months.</p><p><b>CONCLUSION</b>Carto system is suitable for mapping and ablation in pediatric patients with continuous tachycardia, especially with incision atrial tachycardia; Ensite Array system fits children older than 10 years with right heart discontinuous arrhythmia; and Ensite NavX system can set up model and display endocardial anatomic structure quickly. Compared with two-dimensional mapping system, the three-dimensional mapping system (Carto and Ensite) can display the origin of arrhythmia and activation sequence clearly, decrease difficulty of operation efficiently and diminish operation time under X-ray.</p>
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Niño , Preescolar , Humanos , Arritmias Cardíacas , Cirugía General , Ablación por Catéter , Métodos , Técnicas Electrofisiológicas Cardíacas , Métodos , Imagenología Tridimensional , Resultado del TratamientoRESUMEN
<p><b>OBJECTIVE</b>To assess the feasibility of transcatheter intervention for critical pulmonary arterial valve stenosis and atresia with intact ventricular septum in neonates.</p><p><b>METHODS</b>From June 2006 to January 2008, 13 neonates (9 with critical pulmonary arterial valve stenosis, 4 with pulmonary arterial valve atresia) underwent transcatheter intervention(one of them was premature neonate). Ten of the patients were boys and 3 were girls. The mean age was (17.4 +/- 10.7) days. The mean weight was (3.4 +/- 0.8) kg. All the patients received prostaglandin E(1) infusion to dilate the ductus arteriosus before and during the procedure. Valvuloplasty was performed using a low-profile balloon (2.5 - 4.0 mm in diameter), and then a balloon approximately 1.0-1.2 times the annulus diameter was applied. In patients with pulmonary atresia, the valve was perforated with a radiofrequency wire, and then valvuloplasty was performed.</p><p><b>RESULTS</b>Twelve patients (92%) were successfully treated with transcatheter intervention. Right ventricular systolic pressure decreased by 50% [from (120.1 +/- 17.0) mm Hg (1 mm Hg = 0.133 kPa) to (58.8 +/- 7.7) mm Hg, P < 0.001], and the ratio of the right ventricular systolic pressure to systemic systolic pressure decreased by 44% [from (1.6 +/- 0.2) to (0.9 +/- 0.1), P < 0.001]. The right ventricular angiography showed that the pulmonary valves were opened. The ratio of the balloons to annulus were (1.1 +/- 0.1) (the maximal balloon size range from 6 to 12 mm). The procedure lasted for a mean of (127.5 +/- 32.6) minutes. The duration of fluoroscopy was (25.2 +/- 7.2) minutes. Complications occurred in 4 patients (30%). Hemopericardium occurred in 2 patients, hypoxia in 1 patient and supraventricular tachycardia in 1 patient. One (8%) patient died. The follow-up periods were (7.2 +/- 6.4) months. Three patients had moderate to severe residual pulmonary stenosis. One of them underwent a second balloon dilation at 3 months of follow-up. The other two were waiting for the second dilation. Nine patients had mild to moderate residual pulmonary stenosis. All patients had mild pulmonary regurgitation. Ductus arteriosus was naturally closured in 11 patients, and nearly closured in 1 patient.</p><p><b>CONCLUSIONS</b>Transcatheter intervention for critical pulmonary stenosis and atresia with intact ventricular septum in neonates is safe and effective. Some patients may require repeat balloon valvuloplasty.</p>
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Femenino , Humanos , Recién Nacido , Masculino , Cateterismo , Enfermedad Crítica , Estudios de Factibilidad , Atresia Pulmonar , Terapéutica , Estenosis de la Válvula Pulmonar , Terapéutica , Resultado del TratamientoRESUMEN
<p><b>OBJECTIVE</b>To observe the effects of transcatheter closure method for treating congenital coronary artery fistula (CAF) in children.</p><p><b>METHODS</b>Twenty-three children with CAF received transcatheter closure. Under anesthesia, heart catheterization and selective coronary angiography were performed to show the CAF size and relationship with normal coronary artery. CAF with the narrowest inner diameter < 3 mm (n = 16) were occluded with coil device, and CAF with narrowest inner diameter > 3 mm (n = 7) were closed with Amplatzer duct or VSD occluder.</p><p><b>RESULTS</b>Transcatheter closure was successfully performed in 21 cases and failed in 2 cases (CAF is too tortuous in one case and right CAF outlet near the right coronary artery main stem in another case) and CAF were closed by surgery in these 2 patients. No residual shunt or other complications were observed during the 3 months to 3 years follow up.</p><p><b>CONCLUSION</b>Transcatheter closure was an effective and mini-traumatic method for CAF treatment in children.</p>
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Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Fístula Arterio-Arterial , Terapéutica , Cateterismo Cardíaco , Anomalías de los Vasos Coronarios , TerapéuticaRESUMEN
<p><b>OBJECTIVE</b>To evaluate the early complications during and after transcatheter closure of perimembranous ventricular septal defects (PMVSDs) in children.</p><p><b>METHODS</b>A total of 223 patients received transcatheter closure of PMVSDs from March 2002 to December 2005 in our hospital were included in this retrospective study.</p><p><b>RESULTS</b>The overall complications rate was 26.9% (60/223). Major complications occurred in 9 patients (4.0%) including III degrees atrioventricular block (AVB) in 2 (0.9%), hemolysis in 3 (1.3%) and surgical interventions in 4 patients (1.8%) because of device malposition (1), mild aortic regurgitation (2) and device embolization (1) and all 4 patients recovered without further complications. The 2 patients with III degrees AVB were completely recovered to normal sinus rhythm after 7 days treatment with temporary pacemaker and corticosteroid. Hemolysis in 3 patients disappeared after corticosteroid treatment. Minor complications occurred in 51 patients (22.8%) including bundle branch block (BBB) in 37 (16.6%), first-degree AVB in 2 (0.9%), second-degree AVB in 1 (0.4%), new-onset mild aortic regurgitation in 5 (2.2%) and new-onset mild to moderate tricuspid regurgitation in 6 patients (2.6%). Except for right bundle branch blocks, other BBBs were treated with albumin and corticosteroid and completely recovered. No treatment was applied for new-onset valve regurgitations. There was no death in all 223 patients.</p><p><b>CONCLUSIONS</b>Early complications post PMVSDs in children are mostly minor with good prognosis and the prognosis for major complications post PMVSDs is good after proper treatment.</p>
Asunto(s)
Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Cateterismo Cardíaco , Estudios de Seguimiento , Defectos del Tabique Interventricular , Terapéutica , Complicaciones Posoperatorias , Pronóstico , Estudios RetrospectivosRESUMEN
<p><b>OBJECTIVE</b>To discuss the treatment of arrhythmia occurs in the process of transcatheter closure of perimembranous ventricular septal defects (VSD) in pediatric patients.</p><p><b>METHODS</b>182 cases (mean age: 6.2 +/- 3.3 years) with membranous VSD underwent transcatheter occlusion procedure. Two different devices were used: the Amplatzer membranous VSD occluder in 81 patients and the domestic-made device in 101 patients. Electrocardiogram of all patients was recorded before and during closure and at one day after the procedure, and Holter monitoring was performed one week after the procedure.</p><p><b>RESULTS</b>Perioperation arrhythmia occurred in thirty-one patients (17%). Second- or third-degree atrioventricular bundle (AVB) was noted during the procedure in four patients. Normal AV conduction recovered spontaneously before the catheters were withdrawn in three cases and another patient underwent surgical repair. In the other twenty-seven patients, arrhythmia was first documented between one day and one week after the procedure. Third-degree AVB was found in three (1.6%) children after the procedure and underwent the temporary pacemaker (TPM) was implanted, two of them recovered to normal sinus rhythm within one week, another patient underwent elective surgery to remove the occluder and repair the defect. Other arrhythmias were: left bundle-branch block (n = 3), right bundle-branch block (n = 12), second-degree AVB (n = 2), sinus tachycardia (n = 6).</p><p><b>CONCLUSIONS</b>In properly selected cases of perimembranous VSD, the transcatheter closure is safe and effective by using appropriate devices. During and after the procedure, closure of VSD can be associated with some kinds of arrhythmia, such as A-V block, more intensive observation and follow-up were therefore needed.</p>
Asunto(s)
Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Arritmias Cardíacas , Terapéutica , Cateterismo Cardíaco , Métodos , Defectos del Tabique Interventricular , Cirugía GeneralRESUMEN
<p><b>OBJECTIVE</b>To document the five-year follow-up results of transcatheter Amplatzer duct occluder (ADO) occlusion of patent ductus arteriosus (PDA) in children and to assess the safety and effectiveness of this method for PDA closure.</p><p><b>METHODS</b>A retrospective cohort study was completed in patients with PDA underwent transcatheter closure by the ADO in Guangdong Cardiovascular Institute from April 1998 to December 2003.</p><p><b>RESULTS</b>Transcatheter closure of PDA with ADO was attempted in 250 children patients. The median age was 5.3 years and median weight was 15.1 kg. The PDAs were from 1.8 mm to 11.0 mm (median 4.2 mm) in the narrowest diameter, of which 52 were larger than 5 mm (20.0%). All PDAs were occluded with 4 approximately 14 mm ADO through 6F (n = 205) or 7F (n = 45) sheaths. Devices were successfully implanted in 245 patients (98.0%). The Qp/Qs decreased from 1.90 +/- 0.60 to 1.03 +/- 0.21 (P < 0.05). All shapes of the PDA could be closed. Late complication occurred in five patients, including hemolysis in three patients and the los of the pulse of femoral artery in two patients. Follow-up after device implantation was accomplished in 205 patients (82.0%). The incidence of residual shunt at follow-up periods of 1 d, 1 m, 6 m, 12 m, 24 m, 36 m, 48m and 60 m after device occlusion was 9.2%, 2.8%, 1.2%, 0.8%, 0, 0, 0 and 0, respectively. Five patients (2.0%) required re-intervention to treat residual shunt with or without hemolysis. Event-free rates were 98.0% at one year and five years.</p><p><b>CONCLUSION</b>This long-term follow-up result confirmed the safety and effectiveness of ADO closure of PDAs in children.</p>
Asunto(s)
Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Oclusión con Balón , Métodos , Cateterismo Cardíaco , Conducto Arterioso Permeable , Diagnóstico por Imagen , Terapéutica , Ecocardiografía Doppler en Color , Embolización Terapéutica , Métodos , Estudios de Seguimiento , Hemólisis , Radiografía Intervencional , Estudios Retrospectivos , Factores de Tiempo , Resultado del TratamientoRESUMEN
<p><b>OBJECTIVE</b>To study the clinical technology of transcatheter closure of secundum atrial septal defects (ASD) with Amplatzer device in younger and lower body weight children.</p><p><b>METHODS</b>The transcatheter closure of ASD using Amplatzer septal occluder (ASO) was performed in 165 children under 5 years of age (75 boys and 90 girls) with secundum ASD from Aug 1998 to May 2004. The age of the cases ranged from 2 to 5 (mean 3.7 +/- 1.1) years. The body weight ranged from 9 to 18 (mean 12.6 +/- 2.3) kg. The ratio of pulmonary circulation quantity to the systemic circulation quantity (Qp/Qs) was 3.2 +/- 1.9. All the patients underwent clinical examination, X-ray, electrocardiography (ECG) and echocardiography (Echo) for diagnosis of secundum ASD. The transthoracic echocardiography (TTE) was used to detect and measure the defect of the patients and even trans-esophageal echocardiography (TEE) had to be used when it was necessary. With Echo and X-ray guidance, the measuring balloon was used in the body and outside the body to determine the balloon-stretch diameters of ASD, and proper occluders were selected accordingly for the patients for interventional treatment of ASD.</p><p><b>RESULTS</b>The devices were implanted successfully in 163 (98.8%) cases. One failure occurred in a case in whom the device moved into the left atrium after release, and the other failure was that the position of the device was uncertain because of temporary unavailability of a special transducer for TEE. Surgical operations were performed for these two cases. The stretch diameter of ASD was from (8 - 30) mm, (mean 18.3 +/- 5.1) mm. The size of device was selected according to the stretch diameter of ASD. The diameter of the occluders selected was from (8 - 30), (mean 18.6 +/- 5) mm in this series. The occlusion procedure was monitored by fluoroscopy and TTE and in 5 cases (3%) by TEE. The diameter of right ventricle was improved within 2 days after occlusion from (mean 16.4 +/- 4.9) mm to (mean 12.6 +/- 3.8) mm, (p < 0.01). One hundred and forty seven cases belonged to the simple secundum ASD(89%). Thirteen cases who were complicated with other cardiac deformity were treated successfully with different interventional procedure. Six cases had multiple openings and three of these cases had tumour-like changes of the atrial septum which were closed completely just by one occluder. In only one case small quantity of residual shunt remains. No other severe complication was found in this group. About 100 cases (60%) had large ASD, so the procedure was more difficult in those cases.</p><p><b>CONCLUSION</b>The clinical effectiveness of treatment of ASD in children under 5 years of age with Amplatzer occluders was satisfactory and therefore this therapeutic procedure is feasible for this age group of patients. Nevertheless, we do not recommend to use the technique for infants and children under 2 years of age. Strict selection of indications and proper size of occluder and good cardiologic and surgical settings are among the basic factors for successful interventional occlusion of ASD in young children.</p>
Asunto(s)
Preescolar , Femenino , Humanos , Masculino , Ecocardiografía , Defectos del Tabique Interatrial , Diagnóstico por Imagen , Cirugía General , Dispositivo Oclusor SeptalRESUMEN
<p><b>OBJECTIVE</b>The aim of this study was to develop a new perimembranous VSD occluder and to evaluate it.</p><p><b>METHODS</b>The shape of VSD occluder was designed as fabric frame "I" shape that comprised two types: symmetric and asymmetric. The safety, efficacy, feasibility and complication were tested in 22 animal models and in 58 VSD patients in clinical trial. The device were compared with Amplatzer occluder in this study.</p><p><b>RESULTS</b>The new perimembranous VSD occluder was passed the national material test. In animal study, artificial VSD were all occluded by using the new devices with no complication in follow up except one pig expresented wound infection. In clinical trial, all 58 VSD cases were healing with the new device. One patient suffered with atria-ventricular block 5 days after procedure and was free from AV block with medicine therapy. Compared with Amplatzer perimembranous VSD occluder, the new devices had lower frequency of residual shunt.</p><p><b>CONCLUSION</b>The new perimembranous VSD occluder is a safe and effective perimembranous VSD interventional apparatus, and the effect of the new occluders seems not worse than that of the Amplatzer ones.</p>
Asunto(s)
Adolescente , Adulto , Animales , Niño , Preescolar , Femenino , Humanos , Masculino , Adulto Joven , Oclusión con Balón , Métodos , Cateterismo Cardíaco , Métodos , Diseño de Equipo , Defectos del Tabique Interventricular , Cirugía General , Implantación de Prótesis , Porcinos , Resultado del TratamientoRESUMEN
<p><b>OBJECTIVE</b>In children with congenital heart diseases who have undergone surgical interventions, postoperative arrhythmias frequently complicate the clinical course. "Incisional" atrial tachycardia or flutter is one of the most common forms of postoperative arrhythmias in these patients and can lead to significant morbidity and even mortality. The aim of this study was to investigate how to use antiarrhythmic drugs and the CARTO system to treat these cases.</p><p><b>METHODS</b>There were 12 patients with "incisional" atrial tachycardia or flutter complicating surgery for congenital heart diseases in this study (3 patients with correction of tetrology of Fallot, 3 with atrial septal defect repair, 2 with ventricular septal defect repair, 1 with switch, 1 with repair of Ebstein's anomaly, 1 with total anomalous pulmonary venous drainage, and 1 with atrial septal closure with the Amplatzer septal occlusion). Patients whose body weight was less than 10 kg or those who did not wish to accept ablation were treated with antiarrhythmic drugs, including digitoxin, propranolol, metoprolol and cordarone. CARTO system was used to map 6 patients whose body weight was more than 10 kg and who agreed with accepting ablation for atrial tachycardia and flutter. Radio-frequency ablation was performed in these 6 cases including two cases of "incisional" atrial tachycardia and 4 of atrial flutter.</p><p><b>RESULTS</b>(1) The antiarrhythmic drug was successful in 6 patients with "incisional" atrial tachycardia. (2) Six patients including 2 children with "incisional" atrial tachycardia and 4 children with atrial flutter were successfully ablated. But one case of "incisional" atrial tachycardia relapsed after 3 months of ablation. This case, however, was successfully ablated again later. No further relapse was observed during the 2 - 24 months of follow-up.</p><p><b>CONCLUSION</b>Ablation of "incisional" atrial tachycardia and flutter is the first choice to treat the patients whose body weight is more than 10 kg and those who agree with accepting ablation by CARTO system. Drug therapy of "incisional" atrial tachycardia and flutter is palliative and it is the only selection to treat the patients whose body weight is less than 10 kg or those who do not wish to accept ablation procedure.</p>