Background: The angiotensin-converting enzyme inhibitor (ACEI) enalapril is often administered to infants and young children with heart failure (HF) in various dosing regimens and formulations not adapted for their age. Methods: This prospective, two-center, open-label 8-week study evaluated an age-appropriate formulation of orodispersible minitablets (ODMTs) of enalapril (0.25 mg and 1 mg) in children aged 0 to 6 years with HF due to congenital heart disease. An age/weight-based dosing schedule was followed. Measures of echocardiographic parameters, blood pressure, heart rate, modified Ross score, and biochemistry were obtained over the 8-week period. The following two groups were assessed: ACEI-naïve and ACEI-pretreated patients. Results: In total, 53 children (age range of 0.05 to 4.8 years) were enrolled and 29 were ACEI-naïve. The average enalapril dose was 0.098 mg/kg (0.06–0.17 mg/kg) in the naïve group and 0.15 mg/kg (0.07–0.3 mg/kg) in pretreated patients. After 8 weeks, the modified Ross score and left ventricular diastolic dimension (LVD) z-score showed a significant decrease in both groups (p < 0.005). During 8 weeks follow-up, there were no difference in the z-scores for the systolic blood pressure (p = 0.071) or heart rate (p = 0.146). Conclusions: Pediatric patients treated with ODMTs of enalapril for 8 weeks had favorable improvements in LVD and HF symptoms.

Introduction. Balloon valvuloplasty (BVP) is one of the primary therapies for congenital aortic stenosis in children and adolescents. The aim of this interventional procedure is to gain time before possible surgical therapy (aortic valve replacement) until adulthood. Objective. The aim of this study was to evaluate the efficacy, safety and mid-term results of transcatheter BVP in children and adolescent in our Center. Methods. From 2004 to 2011, 50 patients, aged 18 days to 18 years (mean 6.3 years) underwent BVP. Retrospective analysis of the echocardiographic and hemodynamic parameters were performed before and after procedure, especially peak pressure gradient (PG) across the aortic valve, semiquantification of the aortic regurgitation (AR) after the BVP as well as the left ventricle dimensions and functions. Results. The mean peak PG in the whole group decreased from 74.80±27.72 mm Hg to 27.86±13.04 mm Hg (p<0.001) after BVP. In 39 patients (78%), residual PG was lower than 30 mm Hg just after dilation. At the end of follow-up period, 25 patients (50%) had PG above 50 mm Hg, measured by Doppler technique, and four of them underwent re-dilation. Eight patients (16%) had severe AR. During the follow-up period (12-80 months, mean 51 months), six patients (12%) were referred to cardiac surgeons for aortic valve replacement or Ross procedure. Conclusions. This retrospective study analyzes our first experience of BVP as primary therapy of the congenital aortic stenosis. The results confirmed that BVP effectively postponed the need for surgery in children and adolescents toward the adulthood.

Background/Objectives: Hypertrophic cardiomyopathy (HCM) in childhood is associated with a risk of adverse cardiovascular events despite preserved left ventricular (LV) ejection fraction (EF). The aim of this study was to evaluate echocardiographic parameters of longitudinal LV systolic function and determine their relationship with cardiac magnetic resonance (CMR) findings and major adverse cardiovascular events (MACE) in children and adolescents with HCM. Methods: This single-centre prospective observational study enrolled 31 children and adolescents with HCM and preserved LV EF. Echocardiographic assessment included mitral annular plane systolic excursion (MAPSE), tissue Doppler mitral annulus systolic velocity (s′), mitral annular displacement index (MADI), and LV global longitudinal strain (GLS). Investigated CMR parameters encompassed LV mass, maximal wall thickness, and late gadolinium enhancement (LGE). Associations between echocardiographic and CMR findings were analyzed, and the discriminative value of longitudinal function parameters for MACE was assessed. Results: Impaired longitudinal systolic function was frequently detected in our cohort. Lower MAPSE and s′ z-scores were present in 61.3% of patients, reduced MADI in 96.8%, and reduced LV GLS in all subjects. Patients with MACE showed significantly lower MADI (p < 0.001) and worse LV GLS (p = 0.003). An exploratory LV GLS cut-off value of −12.1% showed discrimination for MACE in this cohort, with 75% sensitivity and 95.7% specificity. Echocardiographic parameters significantly correlated with CMR markers of hypertrophy and fibrosis, particularly LV GLS, which demonstrated the strongest associations with LV mass and the presence and extent of LGE. Conclusions: Echocardiographic parameters of longitudinal LV systolic function could contribute to closer clinical surveillance in children and adolescents with HCM. LV GLS may identify subtle myocardial dysfunction and provide exploratory prognostic information; however, its role in risk stratification requires prospective validation in larger pediatric HCM cohorts.

Introduction/Objective. Although cardiac tumors in the pediatric population are seldom findings, their occurrence is constant and with occasional variations. The purpose of this study is to show our experience with surgical options of these masses in two leading national university-level pediatric centers over four decades. Methods. This retrospective study is presenting a total number of 24 surgically treated pediatric patients who have been operated from 1988 to 2020. There were 16 children with primary masses and eight children with metastatic cardiac tumors. Two patients with tumor-like intracardiac masses were not included in the series but had been mentioned as diagnostic challenges. Our patients did not have cardiac transplantation options. Results. The average age of our patients was five and a half years and the most frequent operated tumor was the cardiac myxoma. Four children had neurological symptoms. There were two deaths, one in the primary tumor group and one metastatic patient misdiagnosed as a primary tumor in the early ages of our department. We had two recurrent cases, a girl with Carney complex and an infant with an extremely rare form of cardiac malignancy after a myxoma extraction. One child required a permanent pacemaker insertion. Conclusion. Although rare, the pediatric cardiac tumors can be a source of different life-threatening conditions and lifelong sequelae. Therefore, special considerations should be paid to the diagnostic and surgical modalities of their treatment.

Background/Objectives: We assessed the prevalence of impaired coronary flow veloc-ity reserve (CFVR) and aimed to identify echocardiographic and clinical predictors of coronary microvascular dysfunction in adolescents after neonatal arterial switch op-eration (ASO). Methods: This single-center, cross-sectional study included patients that underwent neonatal ASO for simple D-transposition of the great arteries (D-TGA) during 1998-2013. All patients were evaluated by echocardiography with global left ventricu-lar strain measurement (GLS) and cardiac catheterization, including coronary angi-ography. Coronary flow velocity reserve was assessed by transthoracic Doppler echo-cardiography in the left anterior descending artery (LAD) using adenosine induced hyperemia. Patients were stratified into two groups according to CFVR: group with impaired CFVR (< 2.5) and group with normal CFVR (≥2.5). Spearman correlation was used to assess relationship between CFVR and echocardiographic variables. Binary lo-gistic regression was used to determine independent predictors of impaired CFVR. Results: Out of 48 patients included (median age 16 years, age range 13 to 23 years, 71% male), impaired CFVR was found in 21 patients (44%). These patients had de-creased longitudinal tricuspid annular plane systolic excursion (TAPSE), greater Z-scores for left ventricular end-systolic dimensions and higher mean pulmonary ar-tery pressures (mPAP). CFVR showed modest but significant positive correlations with tricuspid annular plane systolic excursion (TAPSE). Left pulmonary artery branch ste-nosis, reduced TAPSE and mPAP≥ 20 mmHg, were significantly associated with im-paired CFVR, while decreased TAPSE remained independent predictor in multivaria-ble analysis (odds ratio 5.6, 95% confidence interval 1.24-25.26, p=0.025). Conclusions: Impaired CFVR is highly prevalent in adolescents after neonatal ASO. Importantly, impaired CFVR is associated with right ventricular dysfunction.