While liver biopsy is the gold standard for assessing liver fibrosis, the optimal scoring system for Fontan-associated liver disease (FALD) is debated. This study was to determine the correlation and agreement between th...While liver biopsy is the gold standard for assessing liver fibrosis, the optimal scoring system for Fontan-associated liver disease (FALD) is debated. This study was to determine the correlation and agreement between the METAVIR score, used for chronic hepatitis, and the Congestive Hepatic Fibrosis Score (CHFS), specifically designed for congestive hepatopathy. We conducted a retrospective review of liver biopsies from 38 Fontan patients. Pathologists independently evaluated each biopsy and assigned a METAVIR fibrosis score and a CHFS. Fontan patients, a mean age of 21 ± 6 years, have undergone the Fontan operation at a mean age of 8 ± 4 years. This established a mean postoperative interval of 13 ± 4 years at the time of study enrollment. When comparing the two scoring systems, the overall weight Kappa of 0.92 suggested perfect agreement between the two methods for assessing the degree of liver fibrosis. However, a subgroup analysis of weight kappa agreement revealed significant differences based on fibrosis severity. In the low-grade fibrosis group, the agreement was substantial agreement (kappa 0.70). In sharp contrast, the high-grade liver fibrosis group (METAVIR F3-F4 and CHFS stages 3-4) demonstrated perfect agreement (kappa 0.90) between the two scoring systems. Overall, METAVIR and CHFS demonstrate strong correlation and agreement. Differences observed in early-stage fibrosis likely arise from their distinct architectural emphases; portal-based in METAVIR versus centrilobular in CHFS.
Given the heart-brain relationship, children with congenital heart conditions (CHC) may experience poor academic outcomes, even if not diagnosed with developmental conditions. Among children without diagnosed development...Given the heart-brain relationship, children with congenital heart conditions (CHC) may experience poor academic outcomes, even if not diagnosed with developmental conditions. Among children without diagnosed developmental conditions, this study evaluates the "healthy and ready to learn" (HRL) metric by CHC status and identifies associated factors. Using 2022-2023 National Survey of Children's Health (NSCH) data, we compared HRL among 3-5-year-olds with no known genetic, intellectual, or developmental disabilities by CHC status using adjusted prevalence ratios (aPRs) and 95% confidence intervals (CIs). HRL was defined using an established algorithm with 5 domains (Early Learning skills, Social-Emotional Development, Self-Regulation, Motor Development, Health) and scored as "needs support," "emerging," or "on track," aligning with developmental expectations. Among children with CHC, we further identified associated characteristics. Of 19,094 included children, 408 (1.9%) had CHC. Compared to children without heart conditions, fewer children with CHC were "on track" for HRL overall (CHC = 62.4%; no HC = 70.4%) and for each HRL domain (CHC range: 64.3%-83.9%; no HC range: 71.9%-92.5%), though most aPR 95% CIs included 1. Among children with CHC, characteristics associated with not being "on track" in some domains were: ≥3 h of screen time on most weekdays, having special healthcare needs, access to fewer neighborhood amenities, experiencing ≥ 1 adverse childhood event, or caregivers with less than a high school education (aPRs range: 0.58-0.79). Less than two-thirds of children with CHC were HRL. Families of preschoolers with CHC may benefit from intervention efforts and resources to improve school readiness.
Children with congenital heart disease (CHD) are at increased baseline cardiovascular (CV) risk due to their structural heart disease and social determinants of health may further increase their CV risk. The Child Opport...Children with congenital heart disease (CHD) are at increased baseline cardiovascular (CV) risk due to their structural heart disease and social determinants of health may further increase their CV risk. The Child Opportunity Index (COI) is a measure of neighborhood conditions and is associated with cardiometabolic risk in children. However, the impact of COI on children with CHD and associated cardiometabolic risk factors has not been well described. Patients aged 13-17 years with echocardiogram data between 2012 and 2019 were reviewed. COI scores were calculated using geolocation data. Patients were classified into state-normed COI groups into Very Low, Low, Moderate, High, and Very High opportunity groups. Prevalence of obesity, hypertension, and left ventricular hypertrophy (LVH) were determined. A total of 768 patients (mean age 15.49 ± 1.46 years, 57% male (437/768) were included. A high proportion of patients were in the Very Low and Low COI groups (37.5%). There were significant differences in obesity prevalence between COI groups (X = 18.52, df = 4, p < 0.001) with the Low group having the highest prevalence (26.4%). There was not a significant difference in hypertension prevalence between groups. There were significant differences in LVH prevalence between groups (X = 11.43, df = 4, p = 0.02) with the highest prevalence in the Low COI group (32.3%). Adolescents with CHD had significant differences in prevalence of obesity and LVH by COI with higher prevalence in the more adverse COI groups. This highlights the importance of considering SDoH when risk stratifying children with CHD.
Primary cardiac tumors are extremely rare in fetal life, with rhabdomyomas being the most frequently encountered. In contrast, fetal cardiac myxomas are exceptionally uncommon, and reports of diagnosis during the first t...Primary cardiac tumors are extremely rare in fetal life, with rhabdomyomas being the most frequently encountered. In contrast, fetal cardiac myxomas are exceptionally uncommon, and reports of diagnosis during the first trimester are exceedingly rare. We report a fetal right atrial myxoma diagnosed at 12 weeks' gestation. Fetal echocardiography revealed a hyperechoic, pedunculated mass originating from the right atrial wall. Serial follow-up demonstrated rapid tumor growth, with increasing pericardial effusion and reversed A-wave flow in the ductus venosus by 14 weeks' gestation, indicating early and severe hemodynamic compromise. After detailed parental counseling regarding the poor prognosis, the pregnancy was electively terminated. Postmortem examination revealed a gelatinous intracardiac mass, and histopathological analysis confirmed the diagnosis of cardiac myxoma, showing stellate cells embedded in a myxoid stroma. To our knowledge, this represents the earliest gestational age at which a fetal cardiac myxoma has been diagnosed and histopathologically confirmed. This case highlights that fetal cardiac myxomas, although histologically benign, may demonstrate rapid progression and lead to significant hemodynamic deterioration even in the first trimester, underscoring the importance of early fetal echocardiographic evaluation and timely parental counseling.
Hypertensive blood pressure (BP) response to exercise is a risk factor for the development of cardiovascular disease. Normative pediatric treadmill-based cardiopulmonary exercise test (CPET) BPs are not well established....Hypertensive blood pressure (BP) response to exercise is a risk factor for the development of cardiovascular disease. Normative pediatric treadmill-based cardiopulmonary exercise test (CPET) BPs are not well established. We aimed to establish reference standards for CPET-derived BP response in children with structurally normal hearts. Retrospective analysis of 2,043 maximal effort Bruce protocol CPETs from patients ages 6-18 years without heart disease. Age- and sex-specific statistical analyses performed to determine BP percentile at peak exercise. A normal systolic blood pressure response (SBP) response to exercise was defined as < 90th percentile, elevated 90th-<95th percentile, and hypertensive ≥ 95th percentile for age- and sex-specific groups. Analysis of variance was performed to analyze differences between BP groups. Maximal exercise SBP percentile data were determined for age and sex specific groups. Peak exercise SBP is similar for males and females during childhood. In adolescence, males have higher peak SBP. Comparison amongst normal, elevated, and hypertensive CPET responses showed that patients with elevated and hypertensive responses to exercise had higher BMI and higher resting SBP. No significant differences in maximal heart rate or aerobic capacity based on peak exercise SBP. This study provides reference standards for BP response to peak exercise in pediatric and adolescent patients with structurally normal hearts using the Bruce protocol. The results help define pediatric BP responses to peak exercise and lay groundwork for future studies on prognostic significance of a pediatric hypertensive response to exercise.
Examine the relationship between the inter-superior vena cava (SVC) distance and pulmonary blood flow (PBF) splitting, post-Fontan outcomes, hemodynamics, Fontan geometry, and pulmonary artery (PA) growth in bilateral bi...Examine the relationship between the inter-superior vena cava (SVC) distance and pulmonary blood flow (PBF) splitting, post-Fontan outcomes, hemodynamics, Fontan geometry, and pulmonary artery (PA) growth in bilateral bidirectional Glenn (BBDG) patients compared to unilateral bidirectional Glenn (BDG) patients. A single center retrospective cohort study comparing demographic, hemodynamic, and post-Fontan outcome variables between BBDG patients and a randomized cohort of BDG patients was conducted. A simple linear regression model was created to evaluate the relationship between Fontan geometry and PBF splitting. Cardiac magnetic resonance images were segmented using Slicer 5.6.2 and center line distance between the right and left SVC was calculated using an in-house code. The relationship between SVC distance and PBF was examined. The Nakata index was compared for BBDG and BDG patients. 42 patients (21 BBDG and 21 BDG) were included. Demographics, post-Fontan complications, and hemodynamics between groups were not different. PBF flow splitting increased as a function of inter-SVC distance. Patients with BBDG experienced a decrease in PA size over time with the mean difference in Nakata index between groups of 128.5 ± 23.73 (95% CI: 75.66, 181.4; p = 0.0003). Patients with BBDG have poor central PA growth compared to BDG patients. Although outcomes and hemodynamics were equivalent between groups, inter-SVC distance impacts PBF. This study provides a foundation on which to direct further prospective, studies of flow efficiencies in patients with BBDG circulations to guide patient-specific reconstruction techniques that maximize pulmonary artery growth potential and Fontan efficiency.
Patent ductus arteriosus (PDA) is common in preterm neonates, especially before 28 weeks' gestation, and is associated with morbidity such as bronchopulmonary dysplasia, hemorrhage, and necrotizing enterocolitis. Beyond...Patent ductus arteriosus (PDA) is common in preterm neonates, especially before 28 weeks' gestation, and is associated with morbidity such as bronchopulmonary dysplasia, hemorrhage, and necrotizing enterocolitis. Beyond immaturity, perinatal factors, postnatal interventions, and altitude, may affect ductal persistence; altitude is proposed as a modifier, though evidence is limited. The objective of the study is to describe the prevalence, the clinical, and echocardiographic characteristics of PDA at 72 h of life in neonates born before 28.6 weeks of gestation in two Colombian cities located at different altitudes. A prospective observational descriptive study with exploratory analysis was conducted from January 2022 to January 2024 in Bucaramanga (959 m above sea level) and Pasto (2,527 m above sea level). Sixty neonates born before 28.6 weeks underwent color Doppler echocardiography at 72 h. Hemodynamically significant PDA was defined using a standardized, center-agreed echocardiographic scoring system criteria adapted from previously described criteria. Patent ductus arteriosus was present in 85% of neonates at 72 h, although only 11.7% (n=7) fulfilled criteria for hemodynamic significance (hs). The frequency of hsPDA did not differ between altitudes. Neonates with hsPDA showed no differences in inotropic support, ventilatory support, or oxygen requirements. No differences were observed in other variables either, such as birth weight, gestational age, surfactant type, or early fluid intake. No differences in early PDA persistence or hemodynamic relevance were observed between altitudes. However, the high prevalence of PDA at 72 h supports the need for early, structured echocardiographic follow-up to identify hemodynamic compromise and guide timely clinical decision-making.
Cardiac involvement is a well-recognized component of Duchenne muscular dystrophy (DMD), typically manifesting later in childhood as progressive cardiomyopathy and arrhythmias. Early cardiac presentations, particularly i...Cardiac involvement is a well-recognized component of Duchenne muscular dystrophy (DMD), typically manifesting later in childhood as progressive cardiomyopathy and arrhythmias. Early cardiac presentations, particularly in the neonatal period, are exceedingly rare. We report a neonate who presented with atrial flutter in the absence of structural heart disease or ventricular dysfunction. The patient underwent successful cardioversion, and initial cardiac evaluation, including echocardiography, was unremarkable. However, markedly elevated creatine kinase levels prompted further investigation. A detailed family history revealed a previously unrecognized history of neuromuscular disease, and subsequent genetic testing confirmed the diagnosis of Duchenne muscular dystrophy. No signs of skeletal muscle weakness or overt cardiac dysfunction were present at the time of diagnosis. Cardiac arrhythmias in DMD are generally associated with advanced myocardial involvement and reduced left ventricular function. In contrast, atrial flutter presenting in the neonatal period, prior to both skeletal muscle symptoms and detectable cardiomyopathy, is highly unusual. Although a definitive causal relationship cannot be established, the temporal association with the underlying genetic diagnosis and the known pathophysiological effects of dystrophin deficiency suggest a potential link, although an incidental relationship cannot be excluded. Such atypical presentations are rarely described and may lead to delayed or missed diagnosis if not recognized. Neonatal atrial arrhythmias, particularly when unexplained, may represent a possible early manifestation of underlying neuromuscular disorders such as DMD. Careful evaluation, including assessment of creatine kinase levels and detailed family history, is essential for early diagnosis. While causality cannot be definitively established, this case suggests a potential association and highlights the importance of considering genetic etiologies in early-life arrhythmias.
Dapagliflozin has been shown to improve outcomes in adult heart failure (HF) but has not been widely evaluated in pediatrics. The present study describes dapagliflozin use and adverse events (AEs) in infants and children...Dapagliflozin has been shown to improve outcomes in adult heart failure (HF) but has not been widely evaluated in pediatrics. The present study describes dapagliflozin use and adverse events (AEs) in infants and children with HF. This descriptive, single-center retrospective review included pediatric patients with HF or recent heart transplant who were started on dapagliflozin and treated for any duration between 9/2020 and 5/2024. Descriptive statistics were used to summarize clinical characteristics, AEs, anthropometrics, and laboratory values during treatment. Of 120 patients (40.8% female, 16.7% <1 year of age), 78 (65.0%) had congenital heart disease, 33 (27.5%) had cardiomyopathy, and 6 (5.0%) were post-heart transplant. Median age at dapagliflozin initiation was 9.5 years (range 0-20). Median dose was 0.16 mg/kg/day (range 0.08-0.38), though this varied by age. The cumulative treatment duration was 25,551 days (median 128, IQR 40-354). The most common AEs were AKI (0.215/100 patient days, 32 patients affected), UTI (0.059/100 patient days, 10 patients affected), and hypoglycemia (0.106/100 patient days, 12 patients affected). The incidence of both AKI and hypoglycemia were higher in infants compared to older children. In 9 patients (7.5%), AEs prompted medication discontinuation. The remainder continued therapy or stopped for unrelated reasons. Notably, weight z-score did not decline and log BNP improved among our cohort over the study period. Dapagliflozin was well tolerated in infants and children with HF when used alongside standard medical therapy. These data call for multicenter, prospective studies to further characterize the efficacy of dapagliflozin in this high-risk population.
Interstage mortality remains high after introduction of right ventricle (RV) to pulmonary artery conduits to stage I palliation for hypoplastic left heart syndrome (HLHS). Prior studies have not shown long-term survival...Interstage mortality remains high after introduction of right ventricle (RV) to pulmonary artery conduits to stage I palliation for hypoplastic left heart syndrome (HLHS). Prior studies have not shown long-term survival benefit with conduits, likely due to multiple factors including regurgitation contributing to decreased RV function. Valved conduits (VC) were introduced to address these limitations. The study aimed to assess differences in RV size and function using echocardiography during the interstage period between patients undergoing stage I palliation with VC versus non-valved conduits (NVC). We hypothesized patients with VC would have smaller increases in RV size and improved function. Twenty-five neonates with HLHS who underwent Stage I palliation with VC were compared to 25 neonates with NVC from 2013 to 2022. Primary outcomes were RV end-diastolic area (EDA) and function measured by fractional area change and global longitudinal strain. There was no difference in EDA increase one month post-operatively between groups, but the VC group had a smaller increase in EDA pre-stage II compared to the NVC group (3.23 v 4.38 cm2, p = 0.01). Function was not significantly different pre-stage II: GLS in VC -16.0% v NVC − 18.7% (p = 0.39) and FAC in VC 41% v NVC 38% (p = 0.26). There was no difference in interstage cardiac events or mortality between VC and NVC groups. Patients with VC at Stage I palliation had smaller interstage increases in ventricular size compared to NVC, but no differences in functional parameters or outcomes. Larger, longitudinal studies are needed to further examine the hemodynamic benefits of VC in the interstage period and beyond.
Fetal dilated cardiomyopathy carries a variable but generally poor prognosis, depending largely upon the etiology. Many cases of fetal cardiomyopathy develop during the second or third trimester. Similarly, fetal cardiac...Fetal dilated cardiomyopathy carries a variable but generally poor prognosis, depending largely upon the etiology. Many cases of fetal cardiomyopathy develop during the second or third trimester. Similarly, fetal cardiac tumors typically present during the second or third trimester, but tumors have not previously been found to cause dilated cardiomyopathy during fetal life. We report a case of an infiltrative fetal cardiac fibroma presenting as a form of right ventricular dilated cardiomyopathy during the second trimester. Fetal cardiomyopathy secondary to an infiltrative cardiac tumor should be considered in the differential of fetal dilated cardiomyopathy.
Monochorionic monoamniotic (MCMA) twin pregnancies are typically delivered at 32–34 weeks of gestation to reduce the risk of intrauterine demise, but this practice may compromise outcomes for a growth-restricted or very–...Monochorionic monoamniotic (MCMA) twin pregnancies are typically delivered at 32–34 weeks of gestation to reduce the risk of intrauterine demise, but this practice may compromise outcomes for a growth-restricted or very–low-birth-weight twin requiring complex cardiac surgery. We conducted a literature review in Cochrane Central Register of Controlled Trials, EMBASE and PubMed from inception to October 26, 2025. We included reports of MCMA twin pregnancies with one fetus with TGA describing perinatal outcomes, cord complications, and cardiac surgery in very–low-birth-weight neonates. Of 388 records identified, 302 abstracts were screened, 54 full texts reviewed, and 8 studies were included. Only five published cases of MCMA with one twin affected by transposition of the great arteries were identified: two in conjoined twins, one with postmortem diagnosis after extreme prematurity, and two operated cases, of whom just one survived. We describe to our knowledge the third non-conjoined MCMA twin pregnancy with TGA and only the second postoperative survivor, delivered at 34 weeks. Systematic reviews have suggested that, in otherwise stable MCMA pregnancies with TGA, prolonging gestation to at least 33 weeks is considered safe, and based on our experience we suggest that prolonging pregnancy to 34 weeks may still be beneficial, but multicenter data is needed to reach this conclusion.
Truncus arteriosus most often has absence of a patent ductus arteriosus unless there is concomitant aortic arch interruption. To date, there are no reports of fetal echocardiography visualizing a patent ductus arteriosus...Truncus arteriosus most often has absence of a patent ductus arteriosus unless there is concomitant aortic arch interruption. To date, there are no reports of fetal echocardiography visualizing a patent ductus arteriosus in truncus arteriosus without arch interruption. Here, we present the case of a fetus prenatally diagnosed with truncus arteriosus type 1 with a patent ductus arteriosus noted on fetal echocardiography. Visualizing this in-utero allowed for proper surgical planning and ligation during truncus arteriosus repair and excellent outcome.
Fontan circulation is characterized by markedly reduced exercise capacity, historically attributed predominantly to impaired preload reserve and cardiac limitations. However, invasive cardiopulmonary exercise testing dem...Fontan circulation is characterized by markedly reduced exercise capacity, historically attributed predominantly to impaired preload reserve and cardiac limitations. However, invasive cardiopulmonary exercise testing demonstrates that a subset of patients exhibit relatively preserved exercise cardiac output with a blunted arteriovenous oxygen difference, implicating impaired peripheral oxygen extraction. We propose that bile acid dysregulation associated with Fontan-related liver disease may contribute to skeletal muscle microvascular and mitochondrial dysfunction, and advocate for integrated physiologic and multi-omics phenotyping to identify actionable therapeutic targets.
Congenital interventional cardiology fellowships are currently unaccredited by the ACGME, leading to significant variability in recruitment and training practices across North America. This study assessed satisfaction am...Congenital interventional cardiology fellowships are currently unaccredited by the ACGME, leading to significant variability in recruitment and training practices across North America. This study assessed satisfaction among recent trainees and identified opportunities for standardization and improvement. A web-based survey was distributed to pediatric and congenital interventional cardiologists, who had completed fellowship within the past 15 years in North America. Respondents provided feedback on their recruitment experiences, satisfaction with the process, and perspectives on training structure. A total of 69 individuals responded. Only 14% were satisfied with the current fellowship application and recruitment system, while 51% expressed dissatisfaction. A majority (55%) felt that a standardized match process would offer greater equity, and 83% favored a universal date for position offers. Nearly all respondents (n = 67; 97%) agreed there should be a formal date and method by which programs announce their intention to accept applications. Additionally, 82% supported incorporating structured feedback or milestones into training. The majority (77%) felt supported during training and 68% agreed that 12 months represented an adequate timeframe, but only 55% felt prepared to perform complex interventional procedures independently at the conclusion of their training year. Most respondents viewed the current non-standardized recruitment process unfavorably. There is a strong consensus favoring a universal timeline and increased structure in training, highlighting the opportunity for reform.
Children with congenital diaphragmatic hernia (CDH) may require cardiac catheterization for evaluation of pulmonary hypertension. The extent of abnormal systemic venous return complicating access to the right heart in th...Children with congenital diaphragmatic hernia (CDH) may require cardiac catheterization for evaluation of pulmonary hypertension. The extent of abnormal systemic venous return complicating access to the right heart in this population has not been well described. We identified children with CDH who underwent cardiac catheterization at the University of California, San Francisco, at less than or equal to 2 years from 2006 to 2025. Non-invasive imaging, catheterization reports, and angiography were reviewed. Nine children with right CDH (three with hepatopulmonary fusion) and twenty-one children with left CDH underwent cardiac catheterization. Initial catheterization occurred at 4 weeks-2 years of age and 1.9-13 kg. The right heart was directly accessed from the inferior vena cava (IVC) via the femoral vein in all patients with left CDH. Patients with right CDH had abnormal venous anatomy precluding direct IVC-right atrial access in several. This was likely secondary to IVC compression at the diaphragm. Fluoroscopy times in the latter group were shorter with superior vena cava access via the internal jugular vein compared to inferior vena cava access via the femoral vein. Abnormal systemic venous anatomy complicating venous access to the right heart should be considered for patients with right CDH, especially those with hepatopulmonary fusion. If not already delineated by non-invasive imaging, initial angiography from the femoral vein can guide the approach to cardiac catheterization.
Del Álamo Peña R, Tolín Hernani MDM, Miranda Cid C
… +6 more, Ballesteros Tejerizo F, Gil Villanueva N, Rincón Rodríguez D, Asenjo Tornel M, Torres Cuenca MI, Sánchez Sánchez C
This study aimed to assess the safety and diagnostic utility of transjugular liver biopsy (TJLB) in children with Fontan-associated liver disease (FALD) and to compare biopsy‑proven liver fibrosis with findings from noni...This study aimed to assess the safety and diagnostic utility of transjugular liver biopsy (TJLB) in children with Fontan-associated liver disease (FALD) and to compare biopsy‑proven liver fibrosis with findings from noninvasive tests. A retrospective, single-center study of children under 18 years, Fontan surgery, underwent TJLB from 2019 to 2025. Data collected included demographics, cardiac anatomy, laboratory tests, ultrasound, Doppler, elastography, hemodynamic measurements, and histological fibrosis staging. A total of 28 biopsies were obtained from 26 patients, with a mean biopsy age of 16 ± 1.5 years and mean time since Fontan of 10.6 ± 1.5 years. Endoscopy revealed esophageal varices in 61% (36% grade II, 25% grade I). Laboratory abnormalities included thrombocytopenia in 39% and elevated ALT in 35% (30.9 ± 17.4 IU/L). Ultrasound showed homogeneous hepatomegaly in 57%, heterogenity in 25%, nodules in 7%, and splenomegaly in 57%. Elastography suggested advanced fibrosis in most patients (63% F4; mean 13.25 ± 4 kPa). However, biopsy demonstrated only 21.4% grade 4 fibrosis, with 64% showing grade 2–3. Sinusoidal dilatation (86%) and perisinusoidal fibrosis (86%) were the predominant findings. Complications were minimal: one mild hepatic hematoma and two minor pneumothoraxes. No significant correlation was found between histological fibrosis and elastography, ultrasound, or laboratory indices. TJLB is a safe, effective diagnostic tool in pediatric FALD and can be performed alongside hemodynamic evaluation by catheterization and endoscopy. In our series, elastography tended to overestimate fibrosis severity compared with biopsy.
BACKGROUND: Pulmonary vein stenosis (PVS) is a rare but serious condition in children, often requiring surgical or catheter-based interventions. The initial optimal treatment strategy remains unclear due to disease compl...BACKGROUND: Pulmonary vein stenosis (PVS) is a rare but serious condition in children, often requiring surgical or catheter-based interventions. The initial optimal treatment strategy remains unclear due to disease complexity, progression, and high rates of recurrence. METHODS: In this retrospective, single-center study, we identified children with primary or secondary PVS from a cardiac catheterization and surgical database between 2015 and 2023. Patients with single ventricle physiology were excluded. Demographics and outcomes were compared between patients who underwent catheter-based intervention only and those who underwent at least one surgical pulmonary vein repair, with or without subsequent catheter-based reintervention. Reintervention following surgical repair was assessed using Kaplan-Meier analysis. RESULTS: Among 56 children with biventricular physiology and PVS (33 males, 59%), 16 (29%) underwent at least one surgical repair at a median age of 9 months (IQR 4-20), while 40 (71%) were managed with catheter-based interventions alone. Surgical repair was more frequently performed in patients with bilateral or complex disease, particularly those without prematurity or with coexisting congenital heart defects requiring open-heart surgery. Over time, catheter-based approaches became increasingly preferred. Overall, 92% of surgical patients required reintervention, most within the first year. Mortality did not significantly differ between groups (p = 0.294). In the surgical group, elevated right ventricular/systemic systolic pressure ratio (HR: 1.25, p = 0.048) and the presence of scimitar syndrome (HR: 8.25, p = 0.011) were associated with increased mortality. CONCLUSIONS: Surgical pulmonary vein repair remains an important option, particularly in cases where catheter-based intervention is not feasible due to anatomical challenges or when multiple pulmonary veins are severely affected. However, recurrent pulmonary vein re-intervention is common, regardless of whether the initial approach was surgical or catheter-based.