Search

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.ahj.2008.11.020 Byline: Chi-Di Liang (a)(b), Ho-Chang Kuo (a)(c), Kuender D. Yang (a)(c), Chih-Lu Wang (d), Sheung-Fat Ko (b)(e) Abstract: The aim of this study was to investigate the rate, risks factors, and clinical impact of coronary artery fistula (CAF) in Kawasaki disease (KD). Author Affiliation: (a) Department of Pediatrics, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Kaohsiung, Taiwan (b) Chang Gung University College of Medicine, Kaohsiung, Taiwan (c) Graduate Institute of Clinical Medical Science, Chang Gung University, Kaohsiung, Taiwan (d) Department of Pediatrics, Po-Jen Hospital, Kaohsiung, Taiwan (e) Department of Radiology, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Kaohsiung, Taiwan. Chang Gung University College of Medicine, Kaohsiung, Taiwan Article History: Received 17 September 2008; Accepted 26 November 2008

Background/purpose: To investigate mid- to long-term outcomes in children with coronary artery fistula (CAF).Methods: We retrospectively reviewed the medical records of patients seen between September 1996 and August 2011. We enrolled those diagnosed with CAF via echocardiography (Philips SONOS 7500 system and Philips IE33) or angiography. The mean follow time was 42.58 ± 3.4 months (range, 1-166 months). For comparative purposes, participants were grouped as acquired versus congenital, and symptomatic versus asymptomatic. We also measured the size of the coronary artery (CA) in patients with CA dilatation (CAD).Results: Out of 122 CAF patients, spontaneous closure was detected in 37 patients at 21.59 ± 3.45 months after diagnosis. This timeframe did not differ between the acquired and congenital groups (21.64 ± 6.26 months vs. 21.57 ± 4.15 months; p = 0.991). Ninety patients were asymptomatic and remained so; their spontaneous closure rate was 28.89%. Moreover, 24 patients had CAD, including 17 with Kawasaki disease and seven with congenital CAF. The CAs of all congenital-CAF-plus-CAD patients were initially > 5 mm; these patients underwent percutaneous transcatheter intervention, and their CA sizes decreased significantly (6.11 ± 0.79 mm vs. 3.76 ± 0.36 mm; p = 0.002).Conclusion: With the advanced sensitivity of echocardiography, CAF can be detected more easily than ever before. Most patients with small CAFs are asymptomatic and may experience spontaneous closure. Therefore, management of CAF depends on symptoms; if patients are asymptomatic and have small CAFs, intervention may not be necessary, especially in acquired cases. However, if patients present with symptoms or persistent dilatation of the proximal CA, surgical or percutaneous closure should be performed. [ABSTRACT FROM AUTHOR]

Background/Purpose: Intrapulmonary shunting (IPS) due to pulmonary vascular dilatation is frequently observed among patients with end-stage liver disease (ESLD). This study investigated the prevalence and impact of IPS after liver transplantation (LT) in children. Methods: A total of 77 pediatric patients who underwent primary LT were enrolled. All patients had trans-thoracic contrast echocardiography before LT and at least 1 year after transplantation. The patients with IPS and without IPS after LT were designated as group 1 and group 2, respectively. Results: The prevalence of IPS after LT was 6.1%. The patients in group 1 (n =5) were younger (6.4 +/- 2.8 vs. 9.9 +/- 3.6, p =0.036) than in group 2 (n =72). There were no significant differences in gender, weight, hemoglobin level, O2 saturation, or complications between the two groups. Fourteen patients had abnormal liver function tests, two patients in group 1 and 12 patients in group 2 (p =0.22).The overall follow-up period was 6.7 +/- 2.7 years (range, 1.6–13.0). At the latest follow-up, all 5 patients with mild IPS after LT remain asymptomatic with good liver graft function. Conclusion: Among pediatric ESLD patients with preoperative IPS, approximately 6% continue to have mild IPS after LT. Patients with mild IPS after LT remain asymptomatic and have good liver graft function. [ABSTRACT FROM AUTHOR]

Objective: A transpulmonary arterial approach to repair type I ventricular septal defect is the standard. However, the possible adverse effect on the pulmonary valve by this technique has not been investigated. Methods: A retrospective study reviewing type I ventricular septal defect repair from January 1991 to May 2010 was conducted. Of the 142 cases, the ventricular septal defect was repaired via the transpulmonary arterial route in 77 patients (54.2%, PA group) and via the subpulmonic route in 65 patients (45.8%, SP group). All patients received serial annual transthoracic color Doppler echocardiographic evaluation of pulmonary valve function. Results: The age at operation ranged from 1.2 to 272 months (median, 35.0 months; mean, 50.4 months). The mean follow-up period was 96.2 months (range, 2–234 months). Between the PA and SP groups, there was no significant difference in age, body weight, ventricular septal defect size, left to right shunt amount, mean pulmonary arterial pressure, and preoperative pulmonary stenosis or regurgitation. Postoperatively, there was no significant difference in the ejection fraction or incidence of residual ventricular septal defect, right bundle branch block, and pulmonary stenosis. However, the incidence of postoperative pulmonary regurgitation of more than moderate and the total scale of postoperative pulmonary regurgitation were both significantly higher in the PA group (16.9% vs 4.6% and 1.7 ± 0.1 vs 1.4 ± 0.1, P = .031 and .019, respectively). Conclusions: Although the transpulmonary arterial approach for type I ventricular septal defect repair has been advocated for decades, considering the adverse effect on pulmonary valve competency, the subpulmonic approach may be an alternative. [ABSTRACT FROM AUTHOR]

Background/purpose: Pediatric patients with atrial septal defect (ASD) may have failure to thrive. This study aimed to investigate body weight changes in pediatric patients after transcatheter closure of ASD.Methods: From June 2003 to September 2008, we enrolled 60 pediatric patients who underwent transcatheter closure of ASD. Changes in body weight percentile, heart rate, and resolution of right ventricular hypertrophy were compared before and after ASD closure. Patients were divided into two groups according to initial weight percentile: group A, <50(th) percentile (n = 39) and group B, ≥50(th) percentile (n = 21). Echocardiography and routine weight measurements were performed before the procedure and at 3, 6, and 12 months during follow-up. Clinical presentations, laboratory data, and outcomes were measured.Results: Increased body weight percentile (41±4 vs. 48±4, p<0.01), lower heart rate (100±2 beats/min vs. 89±2beats/min, p<0.01), and resolution of right ventricular hypertrophy (59/60 vs. 1/60, p<0.01) were achieved after ASD closure at the 12-month follow-up. Patients in group A were significantly younger (4.6±0.5 years vs. 7.0±0.9 years, p = 0.016), had a higher pulmonary/systemic blood flow ratio (2.2±0.1 vs. 1.8±0.l, p = 0.044), a largerratio of ASD diameter/body surface area (25.0±1.4 vs. 16.4±1.9, p<0.01), and higher percentage of weight gain increase ≥ 5 percentile compared with patients in group B (22/39 vs. 6/21, p = 0.039).Conclusion: Transcatheter closure of ASD positively affects weight gain. An increase of 7 percentile weight was observed at 1 year of follow-up. Patients with a younger age, higher pulmonary/systemic blood flow ratio, and a larger ratio of ASD diameter/body surface area may have better weight gain after ASD closure. [ABSTRACT FROM AUTHOR]

Background and Objective: Atrial septal defect (ASD) is a common congenital heart defect. The course and impact of hemodynamically insignificant ASD in end-stage liver disease (ESLD) patients remains to be elucidated. Our objective is to present our experience in live donor liver transplantation in children with secundum type of ASD and to find out whether ASD has an impact on the outcome of liver transplantation. Patients and Methods: Fourteen recipients (7 male, 7 female) whose median age was 14.2 months (range, 8–28) were included. The diagnosis of secundum type of ASD was confirmed by transthoracic 2-dimensional Doppler echocardiography preoperatively. The mean Child''s score was 9.9, and the mean Pediatric End-stage Liver Disease Score was 14.7. The ASD were classified based on physiologic–hemodynamic (insignificant vs significant) and structural size (small [≤4 mm], medium [5–9 mm], or large [≥10 mm]) parameters. Only 1 patient showed hemodynamically significant ASD based on echocardiography and cardiac catheterization findings. Results: Six small ASD spontaneously closed during the waiting period for transplantation. Four small ASD spontaneously closed posttransplant. The medium- and large-sized ASD persisted or increased in size posttransplantation. There were no perioperative cardiac complications. There were no neurologic complications. All patients are alive with the original grafts. The median follow-up was 49.7 months (range, 19.8–79.4). Conclusion: Hemodynamic insignificant ASD seems to not impact the outcome of liver transplantation in children with ESLD. Further, this series demonstrated that transplantation can be successfully and safely performed in the presence of hemodynamically stable patients with small- to large-sized ASD. [Copyright &y& Elsevier]

Background/purpose: To evaluate the outcome of transcatheter closure of atrial septal defects (ASD) with superior-anterior (SA) rim deficiency using Amplatzer septal occluder (ASO).Methods: Between June 2003 and March 2007, 84 patients with secundum type ASD attempted transcatheter insertion of ASO in our institution. According to the transesophageal echocardiographic findings, patients were divided into two groups: group A, with deficient SA rim (< 3 mm); group B, with sufficient SA rim (> or = 3 mm). There were 43 children and 41 adults (age range, 2.0-79.4 years; mean age, 22.0 +/- 20.2 years). The failure rate, complications and the presence of residual shunt were compared between the two groups.Results: There were 34 patients in group A and 50 patients in group B. Failure of ASO implantation occurred in six patients, three in each group. One patient had two ASOs implanted for two separate ASDs. Therefore, the study cohort consisted of 78 patients with 79 ASO placed. Among 78 patients with successful implantation, five (6.4%) had persistent small residual shunt during follow-up (range, 1-46 months; mean, 21.6 +/- 12.0 months). There was no statistically significant difference between group A and group B in the procedures failure rate (p = 0.682), complications (p = 1.0) and the presence of residual shunt (p = 0.381) during the follow-up period.Conclusion: ASD with deficient SA rim is a common variation. Similar to ASD with sufficient rims, transcatheter closure of secundum type ASD is also effective for ASD with SA rim deficiency. [ABSTRACT FROM AUTHOR]

Background In this study, we investig1ated whether microRNA let-7i regulates dendric cell maturation targeting interleukin-10 (IL-10) via the Janus kinase 1–signal transducer and activator of transcription 3 (JAK1-STAT3) signal pathway subsequently prolongs rat cardiac allograft survival. Methods Quantitative real-time reverse transcriptase polymerase chain reaction, enzyme linked immunosorbent assay, and dual-luciferase assay were performed to verify whether IL-10 was the target of let-7i, and regulatory T cells were assessed by flow cytometry and immunohistochemical study. Western blot was performed to detect JAK1, STAT3, and phosphorylated STAT3 expression. Lewis recipients of Dark Agouti hearts were transfused with phosphate-buffered saline, lipopolysaccharide (LPS)-mature dendritic cells (mDCs), or let-7i–inhibitor-mDCs. Allograft survival times were recorded, and histologic studies were performed. Results Expression of IL-10 messenger RNA level and production of IL-10 were increased in let-7i–inhibitor-mDCs compared with LPS-mDCs. Luciferase activity showed that the translational level of the IL-10 luciferase reporter was decreased by let-7i mimic but increased by let-7i-inhibitor. MicroRNA let-7i inhibitor suppressed DC maturation; however, pretreatment of IL-10 small interfering RNA attenuated the suppression. Expression of JAK1, STAT3, and phosphorylated STAT3 in mDCs were suppressed by let-7i mimic, and pre-treatment of IL-10 small interfering RNA, however, were upregulated by let-7i inhibitor. Lewis recipients transfused with let-7i–inhibitor-mDCs significantly prolonged Dark Agouti cardiac allograft survival. The allografts transfused with let-7i–inhibitor-mDCs showed slight cell infiltration and significantly preserved graft structure. Inhibition of let-7i increased CD4 + CD25 + forkhead box P3 + regulatory T cells and modulated cytokine profiles in vivo and in vitro. Conclusions MicroRNA let-7i regulated DC maturation and function targeting IL-10 through the JAK1-STAT3 pathway. Moreover, transfusion of LPS-induced mDCs transfected with let-7i inhibitor induced prolonged cardiac allograft survival and generated regulatory T cells. [ABSTRACT FROM AUTHOR]