A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was: ‘In [patients undergoing Fontan palliation] does [fenestration] affect [early and late postoperative outcomes]?’ Altogether 509 papers were found using the reported search, of which 11 papers represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. Current data suggest that the use of fenestration has advantages in the immediate postoperative course, with fewer complications such as pleural effusions, shorter hospitalization and decreased early Fontan failure, but comparable long-term outcomes to a non-fenestrated approach. Fenestration should be used in high-risk patients or based on the haemodynamic parameters measured before weaning from cardiopulmonary bypass. Routine use may potentially lead to additional late fenestration closure procedures in some patients, without improving long-term outcomes.

Sex differences have been described in adults with hypertrophic cardiomyopathy (HCM), but it is unknown if similar differences exist in childhood-onset disease. This study aimed to investigate the influence of biological sex on the clinical characteristics and outcomes of children with HCM. An international retrospective cohort of patients diagnosed with nonsyndromic HCM ≤16 years was formed. Sex differences in baseline characteristics and clinical outcomes were investigated. Primary outcome was all-cause mortality or cardiac transplantation. Secondary outcomes include major arrhythmic cardiac event and heart failure event. Of 1,433 patients diagnosed at a median age of 11 years (IQR: 6-14), 471 (33.0%) were female. Although there were no sex differences in phenotype in preadolescent patients (<12 years), adolescent female patients were more likely to have heart failure symptoms (n = 53 [31.9%] vs n = 86 [22.5%]; P = 0.019). Adolescent female patients had larger left atrial size (1.4 z-score [±2.3] vs 2.1 z-score [±2.5]; P = 0.0056) but there was no difference in degree of hypertrophy or proportion with obstructive disease. Over a median follow-up of 5.3 years (IQR: 2.9, 8.0) annual incidence of all-cause mortality or cardiac transplantation, major arrhythmic cardiac event or heart failure events did not vary by sex. Young female patients with HCM are more likely to experience heart failure symptoms and have echocardiographic features of diastolic impairment. Despite differences in phenotype, outcomes during childhood and young adulthood are not different. Further studies are required to explore the underlying mechanisms for these observed differences.

Hypertrophic cardiomyopathy (HCM) is a heterogeneous genetic disorder, most often caused by sarcomeric gene mutations, with a small proportion due to variants in non-sarcomeric loci. Phospholamban (PLN) is a phosphoprotein associated with the cardiac sarcoplasmic reticulum, a major determinant of cardiac contractility and relaxation. We conducted a retrospective study to determine the prevalence, phenotypical spectrum and clinical course of patients carrying the PLN p.Leu39* variant. A cohort including 11 PLN patients was identified among all patients with HCM (9/189, 4.8%) and DCM (2/62, 3.2%) who underwent genetic testing from two tertiary centers and five more were detected through cascade screening. Complete phenotyping was performed. PLN p.Leu39* variant-driven cardiomyopathy presented mostly as hypertrophic, with frequent progression to end-stage dilated HCM. We proceeded to compare these results to a similar analysis of a control cohort consisting of age-matched individuals that inherited pathogenic or likely pathogenic variants in common sarcomeric genes (MYBPC3/MYH7). Overall, the clinical characteristics and examination findings of patients carrying PLN p.Leu39* were not different from patients with cardiomyopathy related to sarcomeric mutations except for the presence of pathological Q waves and the incidence of non-sustained ventricular arrhythmias, which were higher in PLN patients than in those with MYBPC3/MYH7-related diseases.

Background: Kawasaki disease (KD) is an acute inflammatory vasculitis with a particularly high incidence of coronary artery complications and constitutes a significant cause of acquired heart disease in children and young adults. Methods: We conducted a retrospective analysis of consecutive patients aged 0–18 years hospitalized at the “Prof. Dr. Matei Balş” National Institute of Infectious Diseases in Bucharest with Kawasaki disease over a period of 6 years (2018–2023). Results: A total of 25 children were discharged from hospital with this diagnosis during the analyzed period. The mean age was 2.9 years, and 56% were boys. Fever ≥5 days was present in all cases, and the most frequent additional sign was the presence of oral changes. Patients were treated according to in-effect guidelines with intravenous immunoglobulin (IVIG) (100%) and acetylsalicylic acid (68%). Only two cases were considered IVIG resistant and received a second IVIG infusion. Only mild cardiovascular changes were noted in echocardiography: mild coronary artery dilatation (21.7% of cases), mild valvular regurgitation, and small pericardial effusion. Infants displayed less inflammation and higher percentages of leukocytosis, developed an increase in platelet count sooner, received IVIG faster, and had longer hospital stays. Outcomes were generally favorable, and 92% of children were discharged, while the two remaining patients were transferred to other centers. No deaths were recorded. Conclusions: To our knowledge, this is the largest contemporary Romanian cohort of Kawasaki disease published to date, outlining the local diagnostic process, therapeutic strategies, and early outcomes of Kawasaki disease.

Background: Herpes zoster (HZ) is uncommon in children and is typically considered a mild disease. However, hospitalized cases may be associated with complications. Data on herpes zoster in children in Eastern Europe are limited. This study aimed to describe the clinical features, complications, and factors associated with severity in hospitalized pediatric HZ. Methods: We conducted a retrospective cohort study including children (<18 years) hospitalized with HZ between January 2015 and December 2025, at a tertiary infectious diseases center in Bucharest, Romania. Demographic, clinical, laboratory, and outcome data were extracted from medical records. Univariable and multivariable analyses were performed to identify factors associated with complications and prolonged hospital stay. Results: Among 612 pediatric HZ cases, 92 (15.0%) required hospitalization. The median age was 8.8 years (IQR 4.8–13.2), and 52.2% were male. Overall, 43.5% developed complications, most commonly bacterial superinfection (30.4%), followed by neurological and ophthalmological involvement. Herpes zoster sine herpete was identified in 4.3% of cases and was associated with central nervous system involvement. Headache (OR = 4.34, p = 0.025) and lymphopenia (OR = 3.01, p = 0.020) were independently associated with complications. Patients with complications had longer hospital stays (median of 6 vs. 4 days, p = 0.002). In multivariable analysis, complications, immunocompromised status, and chronic conditions were associated with prolonged hospitalization. Conclusions: Herpes zoster in children is generally mild and has a favorable prognosis; however, hospitalized cases are often associated with complications, especially bacterial superinfections and neurological involvement. These findings derive from a selected population and highlight the role of clinical and host-related factors in shaping outcomes in hospitalized pediatric herpes zoster.