BACKGROUND: Branch pulmonary artery (PA) stenosis is one of the most common indications for percutaneous interventions in patients with transposition of the great arteries (TGA), tetralogy of Fallot (ToF), and truncus arteriosus (TA). However, the effects of percutaneous branch PA interventions on exercise capacity remains largely unknown. In addition, there is no consensus about the optimal timing of the intervention for asymptomatic patients according to international guidelines. This trial aims to identify the effects of percutaneous interventions for branch PA stenosis on exercise capacity in patients with TGA, ToF, and TA. In addition, it aims to assess the effects on RV function and to define early markers for RV adaptation and RV dysfunction to improve timing of these interventions.
METHODS: This is a randomized multicenter interventional trial. TGA, ToF, and TA patients ≥ 8 years with a class IIa indication for percutaneous branch PA intervention according to international guidelines are eligible to participate. Patients will be randomized into the intervention group or the control group (conservative management for 6 months). All patients will undergo transthoracic echocardiography, cardiac magnetic resonance (CMR) imaging, and cardiopulmonary exercise testing at baseline, 6 months, and 2-4 years follow-up. Quality of life (QoL) questionnaires will be obtained at baseline, 2 weeks post intervention or a similar range for the control group, and 6 months follow-up. The primary outcome is exercise capacity expressed as maximum oxygen uptake (peak VO2 as percentage of predicted). A total of 56 patients (intervention group n = 28, control group n = 28) is required to demonstrate a 14% increase in maximum oxygen uptake (peak VO2 as percentage of predicted) in the interventional group compared to the control group (power 80%, overall type 1 error controlled at 5%). Secondary outcomes include various parameters for RV systolic function, RV functionality, RV remodeling, procedural success, complications, lung perfusion, and QoL.
CONCLUSIONS: This trial will investigate the effects of percutaneous branch PA interventions on exercise capacity in patients with TGA, ToF, and TA and will identify early markers for RV adaptation and RV dysfunction to improve timing of the interventions.
BACKGROUND: ClinicalTrials.gov NCT05809310. Registered on March 15, 2023.

Background and objective Complex congenital heart diseases (CHDs), such as the tetralogy of Fallot (TOF), often warrant reconstruction and augmentation of the right ventricular outflow tract (RVOT). This procedure requires the use of both synthetic and natural materials. However, finding the ideal material for tissue implants can be challenging. Biological materials often face issues such as tissue degeneration, calcium deposition, antigenicity, rejection, shrinkage, and fibrosis. These issues can lead to complications such as stenosis and insufficiency, potentially requiring early reoperations. In light of this, this study aimed to investigate the effectiveness of the Invengenx® bovine patch for RVOT reconstruction and augmentation. Methods This was a retrospective observational study conducted among eight children who underwent TOF correction cardiac surgery. Their demographic and clinical characteristics, intraoperative findings, and postoperative follow-up results at six months were collected from the hospital patient database. Results There were no deaths or complications in this study. We observed a significant reduction in the gradient across the pulmonary valve and the outflow tract at six months post-procedure. The analysis demonstrated that the Invengenx® bovine patch was successful and did not lead to any complications. Conclusions This study demonstrates the safety and efficacy of this engineered bovine pericardial patch (Invengenx®) as a cardiovascular substitute for surgical repair of both simple and more complex congenital cardiac defects.

UNASSIGNED: The main effect of pulmonary stenosis is a rise in right ventricular pressure. This pressure overload leads to multiple changes in the shape, dimensions, and volumes of the right ventricle (RV) that are reversed after the relieve of the valve obstruction. We thought to study the changes in the RV in patients undergoing balloon pulmonary valvuloplasty (BPV) using three-dimensional (3D) echocardiography.
UNASSIGNED: The study included 50 patients with isolated valvular pulmonary stenosis who underwent BPV at our hospital from December 2016 to August 2017; echocardiography was recorded preprocedural and 3 months after the procedural.
UNASSIGNED: The median age of the study group at the time of the procedure was 2.7 years. The indexed RV wall thickness, basal, and mid-right ventricular dimensions decreased significantly after the procedure (P < 0.005), and the longitudinal dimension increased significantly after the procedure (P < 0.005). The end-systolic and the end-diastolic volumes (EDVs) by 3D echocardiography increased insignificantly (P > 0.05), and the right ventricular function increased significantly (P < 0.05), indicating that the changes in the EDVs were more than the changes in the end-systolic volumes.
UNASSIGNED: There are several factors that interplay together and result in reverse remodeling of the RV after BPV including regression in the RV hypertrophy; changes in the interventricular septal morphology, bowing, and mobility; and changes in the ventricular geometry and dimensions, rather than changes in the ventricular volumes.

BACKGROUND: Vector flow mapping (VFM) enables direct visualization of flow pattern and estimation of flow volume. The aim of this study was to determine its accuracy in the quantification of pulmonary regurgitation (PR) in congenital heart patients after repair of right ventricular (RV) outflow obstruction.
METHODS: This study comprised two parts: (1) validation of VFM in the quantification of PR in patients with repaired tetralogy of Fallot by cardiac magnetic resonance and (2) clinical application of VFM to determine PR in patients after biventricular repair of pulmonary atresia and stenosis with intact ventricular septum. PR was quantified by calculation of VFM-derived pulmonary regurgitant ratio (PRVFM), defined as ratio of backward to forward flow volume.
RESULTS: Coefficients of variations for intra- and interobserver variability in the measurements of PRVFM were 7.0% and 10.4%, respectively. Fourteen patients with repaired tetralogy of Fallot aged 31.3 ± 7.3 years were studied. Their PRVFM correlated strongly with cardiac magnetic resonance-derived PR fraction (r = 0.95, P < .001) and RV end-diastolic volume (r = 0.84, P < .001). In the second part, 14 patients with pulmonary atresia with intact ventricular septum aged 25.6 ± 6.0 years, 14 patients with pulmonary stenosis aged 24.2 ± 7.0 years, and 14 healthy control subjects were studied. PRVFM was found to increase across groups of subjects with absent (4.6 ± 3.3%), mild (11.1 ± 7.1%), moderate (29.6 ± 7.8%), and severe (50.1 ± 8.2%) PR as defined semiquantitatively by color flow mapping. Furthermore, PRVFM correlated strongly with the ratio of PR color jet to width of RV outflow (r = 0.92, P < .001).
CONCLUSIONS: VFM is a reproducible technique for accurate quantification of PR in congenital heart patients after repair of RV outflow obstruction.

OBJECTIVE: Repair of tetralogy of Fallot (TOF) with monocusp pulmonary valve reconstruction prevents pulmonary regurgitation (PR) for a variable period. Since postoperative outcome is governed by PR and right ventricular function, we sought to assess the severity of pulmonary regurgitation and right ventricular outflow (RVOT) gradient in the immediate postoperative period and at 1 year and attempted to identify the anatomical substrates responsible for adverse outcomes.
METHODS: The study included 30 patients. Transthoracic echocardiography was performed before surgery, within 5 days of surgery, and 1 year later. Presence and severity of PR, RVOT gradient, and residual branch pulmonary stenosis were assessed. Right ventricular and monocusp valve functions were studied.
RESULTS: Median age was 36.5 months (3-444 months). There were no deaths. Pulmonary regurgitation was mild in 18, moderate in 10, and severe in 2 patients immediately following surgery. At 1 year, 10 patients had severe PR and one had significant RVOT gradient. None of the variables like age, presence of supravalvar pulmonary branch stenosis, main pulmonary artery diameter, or mobility of monocusp valve was found to have any significant association with the progression of PR. McGoon index <1.5 showed a trend toward more PR, while patients with more residual RVOT gradient had lesser regurgitation.
CONCLUSIONS: Repair of TOF with monocusp pulmonary valve reduces immediate postoperative PR. At 1 year, the monocusp valve underwent loss of function in a significant proportion and PR also progressed. This study could not identify any predictors of progression of PR, though patients with McGoon index <1.5 tended to have more PR while those with more outflow gradient had lesser PR.

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