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.

Aortic coarctation surgery in pediatric patients requires the placement of two arterial cannulas to monitor pressure gradients and surgical correction adequacy. Near-infrared spectroscopy (NIRS) monitoring provides insight into regional blood flow. This study aimed to investigate the correlation between NIRS values and invasive arterial pressures, exploring NIRS monitoring as a potential substitute for arterial cannulation. In a cohort of 21 consecutive pediatric patients undergoing aortic coarctation surgery, recordings of NIRS and invasive arterial pressure values were evaluated at various time intervals. Pearson correlation evaluated the relationship between NIRS values and invasively measured arterial pressures. Moderate to strong correlations were observed between the mean arterial pressure (MAP) of the upper and lower arteries and cerebral (rSO₂-C) and somatic (rSO₂-S) NIRS values 5 min after cross-clamp placement (r = 0.621, p = 0.003; r = 0.757, p < 0.001). Strong correlations were found 15 min after cross-clamp placement (r = 0.828, p = 0.002; r = 0.783, p = 0.004). Before transfer to the ICU, a strong correlation existed between the upper artery MAP and rSO₂-C (r = 0.730, p < 0.001), but there was no correlation between the lower artery MAP and rSO₂-S. These findings are promising, but further studies are required to validate it as a reliable substitute for invasive pressure monitoring in this patient population.

Partial anomalous pulmonary venous return (PAPVR) with dual drainage is a very rare congenital heart anomaly. We report the case of a 6-year-old boy with PAPVR in whom both upper pulmonary veins (PVs) drain anomalously into the systemic venous circulation, while maintaining preserved intrapulmonary collateral venous connections with the remaining pulmonary veins draining into the left atrium. Careful balloon occlusion testing of the anomalous PVs was performed, simultaneously with measurements of pulmonary pressures and control angiography, proving the absence of venous congestion in the upper lung fields during the pulmonary venous phase. Transcatheter occlusion using vascular plugs was safely and successfully performed.

Partial anomalous pulmonary venous return (PAPVR) with dual drainage is a very rare congenital heart anomaly. We report the case of a 6-year-old boy with PAPVR in whom the both upper pulmonary veins (PVs) drain anomalously into the systemic venous circulation, while having preserved intrapulmonary collateral venous connection with remaining pulmonary veins draining into the left atrium. Careful balloon occlusion testing of the anomalous PVs was performed, simultaneously with measurements of pulmonary pressures and control angiography proving the absence of venous congestion in the upper lung fields during the pulmonary venous phase. Transcatheter occlusion using vascular plugs was safely and successfully performed.

The association of a major aortopulmonary collateral artery (MAPCA) with simple transposition of the great arteries (TGA) is uncommon. Such high-flow lesions in the postoperative period following arterial switch operation (ASO) may lead to pulmonary hypertension, pulmonary hemorrhage, heart failure (HF), failure to thrive and prolonged mechanical ventilation. We report a neonate who developed pulmonary overcirculation and HF in the early postoperative period due to a hemodynamically significant MAPCA. Although the association of MAPCA with simple TGA is infrequent, such lesions should be considered in cases of unexplained cardiovascular compromise following ASO. Following transcatheter occlusion of the MAPCA with a vascular coil, rapid hemodynamic stabilization and subsequent extubation of the patient were achieved.