Isolated right Superior Vena Cava drainage into the left atrium in the absence of other cardiac anomalies is an extremely rare condition. The vein of Galen aneurysmal malformation is a congenital vascular malformation. It comprises 1% of all pediatric congenital anomalies. The association vein of Galen aneurysmal malformation, with congenital heart disease has been described. We describe a 16-months old toddler presenting at 7-months of age with respiratory distress and cyanosis. CT brain showed Vein of Galen aneurysmal malformations. Echocardiography showed partial anomalous systemic venous drainage in the form of right superior vena cava drained into left atrium. Four sessions of Endovascular embolization were performed. Surgical repair of partial anomalous systemic venous drainage was done successfully. The superior vena cava in our case overrides the atrial septum promoting direct drainage of venous return into the LA, thus causing dilated left ventricle instead of dilatation of right ventricle which is the usual presentation of VAGMs.

Background: Surgical repair of total anomalous venous drainage (TAPVD) is lifesaving. The operative mortality is reported between 4 and 35%. Anatomical type, obstructed presentation, associated single ventricle, and heterotaxy syndromes are thought to influence short- and long-term outcomes. The effect of simple versus sutureless repair for primary surgery is unclear. This study reports the outcomes of the surgical repair and the effect of these variables in a contemporary setting. Results: Between 2011 and 2019, all patients undergoing surgical repair for TAPVD were included. Operative mortality, length of hospital stay, and long-term survival were reported. The effect of anatomical type, surgical technique, obstruction, and associated lesions was assessed. Pearson’s test, Wilcoxson’s test, and generalized linear regression with Poisson distribution were used. Forty-nine patients from two centers underwent TAPVD repair. The operative mortality was 4%. Postoperative pulmonary vein stenosis occurred in three patients, and reoperation was done in one patient. Survival free from reoperation was 98%, 98%, and 90% at 1, 24, and 60 months in the absence of pulmonary vein stenosis. However, all three patients who developed pulmonary vein stenosis died, at 0.5, 2.7, and 6.3 months of follow-up, respectively. We were unable to detect a significant effect of anatomical type, preoperative obstruction, associated single ventricle, or heterodoxy syndrome on operative mortality or long-term freedom from death or reoperation. Patients who presented with obstruction and infracardiac or supracardiac TAPVD had longer hospital stay. Conclusion: TAPVD repair outcomes are excellent except for patients who develop postoperative pulmonary venous stenosis. Anatomical type, obstructed presentation, associated single ventricle, or heterotaxy are not significant predictors of survival.

Background: Factors affecting the outcomes after Fontan palliation are still controversial. Thus, this study aimed to compare hospital and long-term outcomes after Fontan palliation in patients with left-dominant, right-dominant, and co-dominant ventricles. Moreover, the study sought to identify factors associated with long-term surgical reintervention, catheter-based interventions, and morality. This retrospective cohort study was conducted in a single tertiary referral center between 2010 and 2024 and included all patients who had Fontan palliation (n = 247). The patients were grouped according to the dominant ventricle into three groups: left-dominant (n = 105), right-dominant (n = 108), and codominant (n = 36). Results: No significant differences were found in demographic variables among groups. Heterotaxy was more common in codominant patients who exhibited higher preoperative saturation levels. Early postoperative complications were more frequent in right-dominant patients, with low cardiac output significantly lower in left-dominant patients. Codominant patients experienced more arrhythmias and complete heart block, while operative mortality rates were similar across groups. During a median follow-up of 80 months, the freedom from surgical reintervention at 5, 10, and 15 years was highest in the left-dominant group (97%) compared to right dominance (93%, 78%) and codominance (97%, 87%). Mortality was significantly higher in the right-dominant and codominant groups, with survival rates at 5, 10, and 15 years being 97% for left dominance, 92% for right dominance, and 91% for codominance. Factors influencing long-term mortality were older age, right dominance, and elevated pulmonary artery pressure. Conclusions: Patients with left dominance exhibited superior survival rates and fewer complications compared to those with right dominance and codominance. The findings emphasize the importance of anatomical considerations in risk stratification and clinical decision-making. Young age at the time of palliation could improve the outcomes of the Fontan procedure.

Background: Right ventricular outflow tract (RVOT) reconstruction is frequently performed for pediatric patients with pulmonary valve anomalies, yet optimal techniques remain debated. The equine pericardium offers a promising substrate for pulmonary valve reconstruction but has been understudied in pulmonary valve reconstruction. This study evaluated a novel technique using the equine pericardium to create a cylinder bicuspid pulmonary valve for RVOT reconstruction. Methods: In this retrospective cohort study, 17 pediatric patients (median age: 10 months; 53% male) underwent RVOT reconstruction with equine pericardium between 2023 and 2024. The valve was fashioned from a patch of equine pericardium into a cylinder to create a functionally bicuspid valve. The height of the cylinder ranged from 1.5–2 cm. The diameter was measured around a Hegar dilator corresponding to a valve size z-score of +3. The outcomes included the degree of postoperative pulmonary regurgitation, RVOT pressure gradients, postoperative complications, and reinterventions. Results: Postoperatively, the median peak RVOT pressure gradient decreased significantly from 70 mmHg (IQR: 65–90) to 25 mmHg (IQR: 20–40; p < 0.001). Mild pulmonary regurgitation persisted in one patient (5.9%). Five patients had mild right ventricular dysfunction (29.41%). At a median 3-month follow-up (IQR: 1–8), 17.7% (n = 3) underwent cardiac catheterization. There was no postoperative mortality. Conclusions: Cylinder bicuspid pulmonary valve reconstruction using the equine pericardium effectively reduces RVOT obstruction while maintaining pulmonary valve competence and demonstrates acceptable short-term safety. Having a competent pulmonary valve after repairing the hypoplastic pulmonary valve annulus is very promising; however, the small cohort and limited follow-up preclude definitive conclusions about long-term durability. Larger prospective studies with longer follow-up periods are needed to validate this technique for RVOT reconstruction.