Chromosome 9p deletion syndrome is a rare autosomal dominant disorder presenting with a broad spectrum of clinical features, including congenital heart defects (CHDs). To date, studies focused on a deep characterization of cardiac phenotype and function associated with this condition are lacking. We conducted a multicentric prospective observational study on a cohort of 10 patients with a molecular diagnosis of 9p deletion syndrome, providing a complete cardiological assessment through conventional echocardiography and tissue Doppler imaging echo modality. As a result, we were able to demonstrate that patients with 9p deletion syndrome without major CHDs may display subclinical cardiac structural changes and left-ventricle systolic and diastolic dysfunction. Albeit needing validation in a larger cohort, our findings support the idea that a complete cardiac assessment should be performed in patients with 9p deletion syndrome and should be integrated in the context of a long-term follow-up.
Background: Right free wall (RFW) accessory pathways (APs) represent a relatively rare form of AP, and transcatheter (TC) ablation of these APs carries high procedural failure rates, both with radiofrequency (RF) and cryoenergy. The aim of this study was to report the outcomes of a minimally invasive approach in non-fluoroscopic 3D TC ablation of RFW APs, comparing cryoenergy and RF. Methods: Between March 2010 and March 2024, 62 consecutive patients with RFW APs underwent transcatheter ablation at our institution with a minimally invasive approach. The ablation results were analyzed and compared. Results: The overall acute success rate was 83.9% [52/62 patients; 25/28 (89.3%) for right lateral (RL) APs, 18/19 (94.7%) for right anterior–lateral (RAL) APs, and 9/15 (60.0%) for right posterior–lateral (RPL) APs, p = 0.014], with very limited fluoroscopy use and no complications. There were no significant differences in the acute success rates between the RF and cryoablation groups (32/37 vs. 20/25, p = 0.506). The median follow-up was 24.8 months (IQR 12.5–49.8), and 16 recurrences (30.8%) were observed (3 in the cryoablation group and 13 in the RF group, p = 0.068). The RAL localization of the AP and age > 12 years were predictors of ablation success in multivariate regression analysis. Conclusions: In children, a minimally invasive 3D TC ablation of RFW APs is a completely safe and quite effective approach, with better results for RAL and RL APs, poorer results for RPL APs, and no significant differences between cryoenergy and RF.
Background: We conducted a systematic review and meta-analysis to compare effectiveness and safety of 9 months of isoniazid (9H) versus shorter rifamycin-containing regimens for treating latent tuberculosis infection (TBI) in children. Methods: We systematically searched MEDLINE, Embase, and Cochrane Central Register of Controlled Trials to June 2025 for randomized, controlled trials (RCTs) and cohort studies that compared regimens that were shorter than 9 months of isoniazid in children aged 1–18 years. Outcomes were development of TB disease, treatment completion, and adverse events. Risk of bias was assessed using RoB 2.0 and the Risk Of Bias In Non-Randomized Studies - of Interventions (ROBINS-I) tool; certainty of evidence was graded using Grading of Recommendations Assessment, Development, and Evaluation (GRADE). Results: Five RCTs and 7 nonrandomized studies that enrolled approximately 2950 children in trials and >25 000 in observational cohorts were included. In pooled analysis of 3 RCTs, shorter rifamycin-containing regimens resulted in little to no difference in development of TB disease compared with 9H (odds ratio [OR], 0.19; 95% confidence interval [CI], .03–1.12; moderate-certainty evidence). Treatment completion was probably higher with shorter regimens (OR, 0.51; 95% CI, .42–0.62; moderate-certainty evidence). Adverse events were similar between groups, but evidence is uncertain (low-certainty evidence). Observational data were consistent with these findings, showing higher completion rates and lower hepatotoxicity with shorter treatments. Conclusions: Shorter rifamycin-containing regimens for pediatric TBI probably increase treatment completion and have similar safety outcomes, with no important difference in development of TB disease compared with the standard regimen. These findings support current guideline recommendations that favor shorter regimens in children.