The Impact of High Burden Idiopathic Premature Ventricular Contractions in Pediatric versus Adult Populations. A Retrospective Cohort Study
Idiopathic premature ventricular contractions (PVCs) can occur in up to 40% of children with structurally normal hearts. The study aims to assess the impact of high-burden idiopathic PVCs on children in comparison with adults in terms of symptoms, distribution of origin, management strategies, and safety considerations. This is a single-center retrospective cohort study that included patients with frequent PVCs (> 10% burden in Holter monitoring) and categorized by age into adult group (≥ 18 years) and pediatric group. Data encompassed demographic profiles, clinical symptoms, echocardiographic findings, electrocardiographic analyses, and one-year follow-up management. A total of 224 PVC cases were collected during a 2-year study (120 children and 104 adults). Syncope occurred significantly more in children than adults (15.8% vs. 0.9%, p = 0.003). In children, the right ventricular outflow tract (RVOT) free wall was the predominant site (48 patients; 40%), while the septal RVOT was most common in adults (35 patients; 33.7%). Pediatric cases exhibited significantly higher PVCs originating from the outflow tract (92.5% vs. 69.2%; p = 0.001). Nevertheless, there were no significant differences between groups regarding the safety, acute, and one-year outcomes of RF ablation. Age disparities were evident in syncope incidence and PVC origin, with no variance in PVC-induced myopathy prevalence or the safety and outcomes of RF catheter ablation between children and adults.
3D Modeling of Self-Expandable Valves for PPVI in Distinct RVOT Morphologies
Tetralogy of Fallot often requires transannular patch repair, leading to pulmonary insufficiency. Percutaneous pulmonary valve implantation (PPVI) with self-expandable valves offers a promising alternative, especially for enlarged right ventricular Queryoutflow tracts (RVOT). Five RVOT types identified in patients with Tetralogy of Fallot reflect anatomical variations due to disease and prior surgeries. This study assesses the Pulsta THV® valve's in vitro hemodynamic performance across these RVOT morphologies using 3D-printed models. Five RVOT morphologies were recreated as 3D models from patient-specific imaging data. The Pulsta THV® valves, available in 28, 30, and 32 mm sizes, were evaluated using the ViVitro Pulse Duplicator System at three cardiac outputs (2, 3.5, and 5 L/min). Hemodynamic performance was assessed by measuring regurgitation rates and pressure gradients in the left and right pulmonary arteries. The Pulsta THV® performed optimally in RVOT Types 1 and 2, demonstrating lower regurgitation rates and pressure gradients, particularly with larger valve sizes. Conversely, RVOT Types 3 and 5 showed increased pressure gradients and hemodynamic variability, indicating less favorable outcomes. The results highlighted the critical role of precise anatomical compatibility, with larger valve sizes proving more effective in enlarged RVOT geometry. Valve sizes tailored to specific RVOT morphologies can enhance PPVI outcomes. Types 1 and 2 are ideal for PPVI, while Types 3 and 5 present challenges due to hemodynamic variability. This study supports 3D modeling and in vitro testing for pre-procedural planning to reduce complications, with future research exploring dynamic imaging and materials mimicking tissue properties.
Revealing the Rare: Prenatal Diagnosis of Inferior Vena Cava Aneurysm
This case report highlights the prenatal diagnosis of a rare saccular inferior vena cava (IVC) aneurysm. A 25-year-old gravida 4 para 1 patient underwent detailed ultrasonography at 20 weeks of gestation, revealing a 15 × 10 × 14 mm infrarenal IVC aneurysm. Genetic analysis identified a de novo 2p16.3 deletion of uncertain significance. Serial imaging showed progressive aneurysm dilation and mild cardiomegaly. Postnatal CT angiography confirmed the aneurysm, and anticoagulant therapy was initiated. This report underscores the importance of detailed prenatal imaging and genetic evaluation in the identification and management of rare vascular anomalies, contributing to the understanding of their clinical implications.
Rebuttal to Gut Microbiome in Children with Congenital Heart Disease After Cardiopulmonary Bypass Surgery (GuMiBear Study)
Virtual Reality Curriculum Increases Pediatric Clerkship Students' Knowledge of Congenital Heart Disease
This study aimed to assess the impact of a virtual reality (VR) educational curriculum on the knowledge of congenital heart disease (CHD) among medical students during their pediatric clerkship. A multi-center study was conducted involving medical students from two large academic medical centers. Participants engaged in the Stanford Virtual Heart (SVH) educational program during their pediatric clerkship from July 2020 to September 2021. The study employed a stratified approach for group assignment based on timing of enrollment, with Center 1 stratifying by the first or second half of the academic year and Center 2 by month-long rotations. A validated 27-question assessment tool focused on six common CHDs was administered, with the intervention group completing the SVH before the assessment and the control group completing the SVH after the assessment. Additionally, a qualitative survey gauging the participants' experience was conducted. Results were compared between the intervention and control groups. A total of 373 medical students (179 intervention and 194 control) participated in the study. The intervention group exhibited significantly better performance on the assessment compared to the control group across both medical centers (P value < .0001). More than 92% of participants rated the VR experience as 'easy' or 'very easy' to use. Over 99% of participants recommended integrating the program into the pediatric clerkship curriculum. The SVH curriculum was seamlessly integrated into two medical school pediatric clerkships, resulting in a significant improvement in CHD understanding. The overwhelmingly positive feedback supports the incorporation of VR-based educational tools into medical education.
Changes in Non-invasive Myocardial Stroke Work Related to Variation in Pacing Sites and Heart Rates in Adolescents
Non-invasive assessment of myocardial work is a newly described technique to assess myocardial energetics. This has not been previously studied to assess the effects of right ventricular pacing at different sites or at different heart rates in children. We aimed to study the effects of right ventricular apical, septal, and His bundle pacing on myocardial work along with the effects of increasing heart rate. This was a prospective pilot study performed on six patients with structurally normal hearts and function following an electrophysiology study. Global work index and global work efficiency was highest during His pacing and lowest during right ventricular apical pacing. The global constructive work, index, and efficiency were progressively worse with increasing heart rates. In this prospective pilot study, we demonstrated that myocardial work indices differ depending on myocardial activation pattern and at different heart rates in pediatric patients. Myocardial performance as assessed by myocardial work efficiency is worse when pacing is performed at the right ventricular apex when compared to His and right ventricular septal pacing. Myocardial performance can be affected by higher heart rates, following a work-frequency relationship.
Transcatheter Closure of Ventricular Septal Defects after Upfront Transvenous Antegrade Cannulation from the Right Ventricle
Transcatheter closure (TCC) of certain ventricular septal defect (VSD) subtypes typically requires arteriovenous loop (AVL) formation or retrograde transarterial deployment. Upfront transvenous cannulation from the right ventricle avoids arterial access and loop-related complications. We retrospectively reviewed data of patients who underwent TCC for perimembranous, intraconal, and post-surgical residual VSDs at our institution (January 2019-December 2023). Patients receiving upfront transvenous VSD cannulation and closure were compared to those undergoing antegrade closure after AVL formation. Upfront retrograde closure cases were excluded. In a cohort of 163 patients, upfront transvenous cannulation was performed in 116 (71%) and AVL formation in 31 (19%). Upfront transvenous cannulation use increased from 25.6% in 2019 to 93% in 2023. For the upfront group, the median patient age was 55 months (IQR, 17.8-120.8), median weight was 15.8 kg (IQR, 9.6-29.8), indexed VSD size was 9.2 mm/m (IQR, 5.5-14.8), and 68.9% had pulmonary arterial hypertension. Cannulation was successful in 83.6% (97/116) of cases, with a switch to retrograde transarterial cannulation in 16.4% (19/116). No deaths or pacemaker implantations occurred. Compared to AVL group, upfront group had significantly shorter procedural times (p = 0.002) and lower radiation exposure (p < 0.001). Smaller patient weight (OR: 0.97, 95%CI: 0.95-0.99), larger indexed VSD size to patient weight (OR: 36.5, 95%CI: 2.49-533.9) and to BSA (OR: 1.23, 95%CI: 1.06-1.4) were significant independent predictors for successful transvenous cannulation. Upfront transvenous VSD cannulation simplifies transvenous TCC by eliminating the need for arterial access and AVL formation, particularly successful in small patients with large defects.
Rebuttal to Letter to the Editor: Congenital Heart Defects in Patients with Anorectal Malformations: A Retrospective Cohort Study of 281 Patients
Differentiating Kawasaki Disease and Multisystem Inflammatory Syndrome in Children Using Blood Composite Scores: Insights into Clinical Outcomes and Predictive Indices
The study sought to assess the clinical utility of complete blood count-derived composite scores, suggesting their potential as markers of inflammation and disease severity in Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) with Kawasaki-like features. This retrospective study analyzed data from 71 KD and 73 MIS-C patients and 70 healthy controls. The KD group showed a higher rate of coronary involvement (26.7% vs. 10.9%), while the MIS-C group had a higher intensive care unit (ICU) admission rate (34.2% vs. 2.8%). Platelet counts, lymphocyte counts, mean platelet volume (MPV), MPV/Lymphocyte (MPVLR), and MPV/Platelet (MPVPR) ratios demonstrated the highest specificities in distinguishing MIS-C than KD (84.5%, 83.1%, 91.1%, 88.7%, and 88.7%, respectively). Monocyte counts, MPV, and MPVPR demonstrated the highest specificities to predictive ICU admission in the MIS-C group (83.3%, 89.6%, and 89.6%, respectively). Lymphocyte counts, platelet/lymphocyte ratio (PLR), neutrophil/lymphocyte ratio (NLR), MPVLR, and Systemic Immune-Inflammation Index (SII) parameters were found to have high negative predictive values for predicting KD patients without coronary artery lesions (CALs) (85.7%, 86.1%, 87.1%, 87.1%, and 85.7%, respectively)., Systemic Inflammation Response Index (SIRI), MPVPR, and CRP were independently predictive of ICU admission in the MIS-C group, and lymphocyte count and IVIG resistance were also identified as significant predictors of CALs in the KD group. NLR, MPVLR, MPVPR, and NPR indices effectively differentiate MIS-C from KD and predict ICU admission in MIS-C. NLR, PLR, MPVLR, and SII are valuable in excluding CALs in KD with high negative predictive values. In addition, SIRI and MPVLR were independent predictors of ICU admission in MIS-C, and lymphocyte count was identified as an independent predictor of CALs in KD.
Epicardial Pacemaker Lead Related Cardiac Strangulation: The Importance of Early Recognition
Lead strangulation is a dangerous complication of epicardial pacemaker insertion. This complication has been increasingly highlighted lately. Our institution has recently identified four cases over the past five years. This study's aim was to 1) identify risk factors for strangulation and 2) prospectively screen existing epicardial pacemaker patients for unrecognized strangulation or features that would prompt closer review. Patients known to the pacemaker clinic with epicardial pacemakers inserted from 2005 to 2023 were included. Electronic health records were used to locate all subjects and gather data. Risk factors were identified using Firth's penalized method of logistic regression. Forty-five patients were included, of which four (8.8%) had evidence of strangulation. Posterior-anterior (PA) chest radiographs all demonstrated characteristic looping patterns of the pacing leads, with confirmation on CT angiography. All affected patients underwent revision surgery. Implantation at a weight of less than 6.5 kg was associated with a significantly increased incidence of strangulation (OR 25, P 0.044). Other factors including lead length, presence of structural cardiac disease, and dual-chamber insertion were not statistically significant. No patients who were prospectively screened were found to have strangulation. Children undergoing insertion of a pacemaker early in infancy are at particularly high risk of strangulation and should be closely monitored following surgery. Regular chest radiography (every three years) to screen for this complication is advised. Larger multi-center studies to pool data for this relatively rare complication may help identify other risk factors for strangulation.
Pleural Drainage, Clinical Characteristics, and Management Strategies in the Perioperative Fontan Patient: A Multicenter Report
Prolonged pleural drainage and chylothorax are common in postoperative Fontan patients and are associated with increased morbidity and mortality. Multiple medical and interventional treatment strategies exist and vary between centers. This is a retrospective multicenter observational cohort study of pediatric patients who underwent Fontan operation at 8 pediatric cardiac surgical institutions from 1/1/2019 to 12/31/2021. Data were obtained from institutional records and collected from the Pediatric Cardiac Critical Care Consortium (PC). 185 patients underwent Fontan operation with median age of 3.8 years [IQR 3.2-4.5]. Chest tube drainage for > 14 days occurred in 40 patients (22%). Chylothorax occurred in 33 patients (18%, incidence 9.1-26.2% across centers). Compared to non-chylothorax patients, those with chylothorax had lower preoperative ventricular end diastolic pressures (8 vs. 9 mm Hg, p = 0.019), greater chest tube utilization (13 vs. 7 days, p = < 0.001), ICU LOS (7 vs. 4 days, p = 0.001), hospital LOS (12 vs. 9 days, p < 0.001), and more weight loss (- 2.7% vs. 0.8%, p = 0.019). Using a receiver-operating characteristic curve, chest tube output > 18.8 mL/kg/day on POD 2 predicted chylothorax with an AUC of 0.73. Common chylothorax treatments were diet modification (n = 15, 45%) and sildenafil (n = 14, 52%). Interventional procedures were used in six chylothorax patients (18%). Postoperative chylothorax in Fontan patients was associated with increased chest tube utilization, postoperative interventions, greater weight loss, and longer ICU and hospital LOS. Center level variation suggests outcomes and resource utilization could be improved with further studies and establishment of best practices.
Pulmonary Atresia with Intact Ventricular Septum, an Evolving Strategy in the Era of PDA Stenting: Single Center Experience
Repair or palliation of pulmonary atresia with intact ventricular septum (PA/IVS) often falls into one of 4 categories: cardiac transplant, 2-ventricular circulation, 1.5 ventricle circulation, or single ventricle circulation. The optimal management strategy has been an area of much debate. We sought to review the management strategy of patients with PA/IVS at our institution to better understand what metrics can be used to guide management and initial interventions. The study aims to examine the outcomes of a single-center approach to managing patients with PA/IVS. Our cohort included 29 patients; one patient underwent a planned transplant at ten days of life (3.4%), 12 underwent repair via a two-ventricle circulation (41.4%), 7 underwent repair with 1.5 ventricle circulation (24.1%), and 7 underwent repair with single ventricle circulation (24.1%). Survival was achieved in 93.1% with two patients (6.9%) expiring. The TV annulus z-score was significantly different between the three groups, with the 1 V group having the smallest median TV annulus z-score at - 4.04 (IQR - 4.60- - 3.60) and the 2 V group having the largest median TV z-score at - 1.4 (IQR - 2.24- - 0.12). Six patients underwent late right ventricular decompression. We present a post-hoc algorithm to help guide treatment strategies for patients with PA/IVS.
Incidence and Prognosis of Surgical Heart Block in Patients with L-transposition of the Great Arteries
L-transposition of the great arteries (L-TGA) represents a spectrum of congenital heart defects (CHD) associated with atrioventricular block (AVB). However, the incidence and prognosis of postoperative AVB among patients with variants of L-TGA is uncertain. Assess the incidence and risk factors for postoperative AVB requiring permanent pacemaker (PPM) implantation for pediatric patients with L-TGA undergoing cardiac surgery. Single-center retrospective analysis of patients with L-TGA who underwent cardiac surgery from 2000 to 2022. Patients with postoperative AVB and those requiring PPM implantation were compared to those who did not receive a PPM. 161 cardiac surgeries were performed in 75 patients with L-TGA. Postoperative AVB occurred in 8 cases (5%) with 5 cases (3%) requiring PPM. Univariate analysis identified risk factors for PPM which included ventricular septal defect (VSD) intervention [odds ratio (OR) 58.4, p < 0.01], pre-operative non-single-ventricle physiology (OR 13.3, p = 0.02), and longer pre-operative PR interval Z-score (OR 3.3, p < 0.01). Excluding biventricular conversions, no surgery (n = 116) in patients with univentricular circulation required a PPM in the postoperative period due to AVB. Multivariate analysis did not identify any statistically significant risk factors for AVB requiring PPM. The overall risk of postoperative AVB in L-TGA was 5% despite the anatomic vulnerability of the conduction system. However, L-TGA patients appear less likely to recover AV conduction than all comers to CHD surgery (38% L-TGA vs 60% overall, p < 0.01) and may be considered for earlier PPM placement.
Impact of Lesion Severity in Fetal Cardiac Counseling
Approximately 1% of all live births in the USA are affected by congenital heart disease (CHD), the leading cause of congenital defect-related illness and infant death. Although technological innovations have improved CHD diagnosis in utero, variation among fetal cardiac counseling practices persists. Our study aims to evaluate physician counseling content based on cardiac defect complexity. We conducted an anonymous survey of providers who perform fetal cardiac counseling. Participants stated their counseling practices for 21 CHD lesions, choosing from postnatal surgical intervention, termination of pregnancy, and/or postnatal palliative comfort care. CHD lesions were divided by severity into three groups. Chi-square tests were performed to assess whether counseling content varied based on provider response to the statement "some life is always better than no life at all." There were 138 respondents, with postnatal surgical intervention counseling frequency consistent among all lesions. Inclusion of termination and palliative/comfort care in counseling increased in frequency as disease severity increased, with the former being counseled more frequently. For tetralogy of Fallot (p = 0.02), interrupted aortic arch (p = 0.03), hypoplastic left heart syndrome (p = 0.03), and pulmonary atresia with intact ventricular septum (p = 0.03), those providers who disagreed with the statement that "some life is always better than no life at all" were more likely to counsel termination of pregnancy and/or palliative care compared to their counterparts. Our study found that the interventions and options included in fetal cardiac counseling vary based on complexity. Moreover, physicians' personal beliefs tended to influence counseling practices in some moderate or severe defects.
Prenatal Diagnosis of Congenital Heart Disease in Liveborn Infants in the New England Region
Prenatal diagnosis of congenital heart disease requiring early cardiac catheterization or surgical intervention enables optimal delivery planning for appropriate postnatal cardiovascular intervention and care. This allows for improved morbidity and mortality. Prior national data reported prenatal diagnosis rates of 32% for congenital heart disease requiring intervention in infants in the first 6 months of life in the New England region. With improved technology, access to care and changes to the obstetrical ultrasound guidelines for mid trimester fetal study, it is expected that diagnostic rates should improve. The New England Congenital Cardiology Association (NECCA) conducted a quality improvement study to determine the rates of prenatal detection in the current era with the hypothesis that there has been improvement in detection rates in this region. Ten of fourteen medical centers delivering pediatric cardiology care in New England contributed prenatal diagnosis data for 286 infants born at the participating centers during a one year period. The overall prenatal detection rate was 68%. Detection rates ranged from 39 to 90%. When fetal echocardiogram was performed at a pediatric cardiology center, the detection rate was 95% with only 7 moderate (7/195; 3.6%) and 3 severe (3/195; 1.5%) diagnostic discrepancies. Prenatal diagnostic rate and accuracy are high among pediatric cardiology centers in the New England region, and much improved over historical data. To improve fetal detection of congenital heart disease further, future work is needed to better determine the etiology of missed prenatal diagnoses and efforts should be focused on increasing appropriate referrals to pediatric cardiology centers for fetal evaluation.
Cardiovascular Performance in Neonates with Hypoxic-Ischemic Encephalopathy Under Therapeutic Hypothermia: Evaluation by Conventional and Advanced Echocardiographic Techniques
This study aimed to evaluate the hemodynamic and ventricular performance of neonates with hypoxic-ischemic encephalopathy (HIE) undergoing therapeutic hypothermia using conventional and advanced echocardiographic techniques. This observational, prospective study included 22 neonates with HIE matched with 22 healthy neonates. Echocardiographic studies were performed 24 h after achieving target temperature during hypothermia and 24 h after rewarming. Evaluated echocardiographic parameters included ejection fraction (EF), shortening fraction (SF), right ventricular fractional area change, biventricular Tei index, right ventricular s' wave velocity, tricuspid annular plane systolic excursion, biventricular stroke volume and cardiac output, left ventricular (LV) and right ventricular (RV) global longitudinal strain (GLS), LV circumferential and radial strain, LV twist, and LV torsion. LV EF and SF did not change significantly between the hypothermia and rewarming periods (EF:73 ± 7% vs. 74 ± 5%, p = 0.21; SF:39 ± 6% vs. 41 ± 5%, p = 0.26); however, both were higher after rewarming compared to the control group (EF:70 ± 5%, p = 0.003; SF:36 ± 4%, p = 0.002). There were no significant differences in LV GLS, circumferential and radial strain, twist, and torsion between the HIE and control groups. Pulmonary artery systolic pressure (PASP) and RV GLS were worse in the study group compared to the control group (PASP: hypothermia 45 ± 24 mmHg, p = 0.01; rewarming 53 ± 34 mmHg, p = 0.01; control group 29 ± 11 mmHg; RV GLS: hypothermia 18 ± 5%, p = 0.02; rewarming: 18 ± 4%, p = 0.01; control group 21 ± 2%). Therapeutic hypothermia appears to have no detrimental impact on LV systolic function. RV GLS was the only parameter that demonstrated impaired RV systolic function during therapeutic hypothermia, likely due to elevated PASP.
Management Strategies for Truncus Arteriosus: A Comparative Analysis of Staged vs. Primary Repair
We reviewed the outcomes of truncus arteriosus repair (primary vs. staged repair incorporating bilateral pulmonary artery banding), focusing on survival, reintervention, and functional data. We analyzed 39 patients who underwent a first intervention for truncus arteriosus (staged, n = 19; primary, n = 20) between 1992 and 2022. The median follow-up period was 8.0 (2.2-13.2) years. Survival, freedom from reoperation, and freedom from catheter intervention were estimated using the Kaplan-Meier method. High-risk patients were defined as those with a weight ≤ 2.5 kg, ≥ moderate truncal valve regurgitation, interrupted aortic arch, or preoperative shock. In the staged group, patients with a median weight of 2.6 kg had a median intensive care unit stay of 5 days and no hospital mortality after bilateral pulmonary artery banding. At repair, the staged group had a larger conduit for the right ventricular outflow tract (14 vs. 12 mm; P = .008). Catheter intervention on the branch pulmonary artery was required in 67% of patients in the staged group, but right ventricular end-diastolic pressure at follow-up was comparable between the groups (P = .541). Survival rates were higher among high-risk patients in the staged group (87.5% vs. 21.4% at 15 years; P = .004) but were comparable between groups for standard-risk patients (P = 1.000). Bilateral pulmonary artery banding was a safe, effective procedure. Reintervention for branch pulmonary artery was common but did not affect functional outcomes. Staged repair may play a pivotal role regarding survival in high-risk patients, and risk stratification is vital.
The Role of Fetal Echocardiography in Predicting Postnatal Pulmonary Venous Obstruction in Neonates with TAPVR: A Single Center Experience
Total anomalous pulmonary venous return (TAPVR) is a high risk and rare cardiac malformation with a low prenatal detection rate and predicting obstruction in these cases is difficult. We sought to investigate fetal echocardiographic parameters associated with postnatal pulmonary venous obstruction (PPVO). We performed a retrospective review of 26 patients with TAPVR who had a fetal echocardiogram from 2010 to 2021. Blinded readers analyzed the fetal Doppler features of the pulmonary veins (PV) and vertical veins (VV) at final fetal assessment. Of the 26 patients, 17 (65%) were prenatally diagnosed and overall 10 (38.5%) developed PPVO. All patients had additional associated congenital heart disease. Compared to patients without PPVO, patients with PPVO had a lower VV variability index (VI) (0.51 (0.17-0.79) vs 1.43 (0.54-2.58); p = 0.003), a higher likelihood of having an abnormal VV or PV Doppler pattern (p = 0.042, p = 0.015), a higher VV minimum velocity (0.435 m/s (0.17-1.05) vs 0.14 m/s (- 0.16 to 0.35) p = 0.001) and a higher VV mean velocity (0.641 m/s (0.31-1.15) vs 0.321 m/s (0.19-0.5); p = 0.009). A fetal VV VI of ≤ 0.8 was associated with PPVO (100% sensitivity, 86% specificity). Our data show that some fetal PV and VV Doppler indices are significantly different between those that develop PPVO and those who do not, with the most significant difference seen in the VV variability index and VV minimum velocity. Prenatal predictors of PPVO have the potential to influence prenatal counseling, perinatal care, surgical timing, and outcomes associated with this critical diagnosis.
Factors Associated with Prolonged Mechanical Ventilation in Late Repair of Tetralogy of Fallot
Research establishing factors associated with duration of mechanical ventilation after Tetralogy of Fallot repair, is mainly based on population presenting at early infancy. There are fewer reports regarding repair after infancy, during childhood and preadolescence. To compare two groups of late TOF repair based on post-operative invasive mechanical ventilation duration and explore associations with pre-operative clinical markers of severity of right ventricular outflow tract obstruction. A single-center retrospective cohort study based on medical charts review of patients older than 12 months who underwent primary complete TOF repair between 2017 and 2023. Patients younger than 12 months or those who had distinct anatomical variants were excluded. Pre-operative characteristics reflecting the severity of right ventricular outflow tract obstruction were collected, including baseline oxygen saturation, the occurrence of hypercyanotic spells, maximum right ventricular outflow tract pressure gradient. Perioperative variables included repair type, cardiopulmonary bypass and aortic cross-clamp duration, and rates of residual lesions. 280 patients from 27 countries were screened; 181 remained eligible and were significantly older than previously reported. 129 (71%) were extubated early post-operatively, while 52 (29%) had a longer ventilation course. In a multivariate analysis, only baseline oxygen saturation and repair type maintained a significant association with length of mechanical ventilation. In distinctly older patients undergoing TOF repair, those with lower base line saturation undergoing a non-valve preserving repair are more likely to have a longer course of mechanical ventilation. Other clinical markers reflecting the severity of right ventricular outflow tract obstruction were not independently associated with mechanical ventilation duration.
Evaluating the Sapien® XT Valve in Native Right Ventricular Outflow Tracts After Tetralogy of Fallot Repair: Mid- and Long-Term Results
Although the long-term outcomes of the surgical grafts are well defined and reported, the data regarding the mid-and long-term results of the balloon-expandable percutaneous valves in the native right ventricular outflow tract (RVOT) is limited. We retrospectively evaluated 42 patients who underwent PPVI (Sapien® XT valve) to native RVOT due to severe pulmonary regurgitation (PR) and/or moderate to severe pulmonary stenosis (PS) between August 2015 and November 2020. The median patient age at the time of PPVI was 13.4 years (6.1-36.5 years). The median body weight of the patients was 42 kg (15-110 kg). The rate of patients who were followed up without the need for percutaneous or surgical intervention was 97.4% at the end of year 1, 89.3% at the end of year 3, and 85.8% at the end of year 5. At the end of year 6, the proportion of patients requiring no procedure remained constant, with year 5 at 85.8%, but decreased to 70.2% at the end of year 7. Although the early results are very encouraging, it is seen that PPVI in patients with RVOT in the long term brings some problems. The most important of these is tricuspid valve problems, which were not considered before the procedure. Patients requiring reintervention due to pulmonary regurgitation show similar characteristics to surgical valves' long-term results.
Correction: Response to the Letter Titled "Same Entity, Different Hemodynamics: Residual VSD After TOF Repair and Isolated VSD Closure" From Dr. Safak ALPAT