Search Results (1,344)

Background: Pediatric pulmonary vein stenosis (PVS) is a progressive and recurrent condition associated with high morbidity and mortality. Long-term outcomes of individual pulmonary veins remain poorly described. Methods: All pediatric (<18 years) PVS cases diagnosed between September 2005 and January 2020 with a minimum follow-up of 5 years were included. Vein-specific outcomes were assessed using competing-risk survival analysis adjusted for patient death and vein atresia. Veins with recurrent disease maintaining patency for ≥3 years were analyzed longitudinally to characterize reintervention burden as a surrogate for disease trajectory. Results: A total of 244 affected veins in 107 patients were identified. The cumulative incidence of vein loss progressively increased during the first 3 years, followed by plateauing thereafter. Overall, 147 of 244 veins (60%) were lost within 3 years of diagnosis, including 75 veins lost secondary to patient death. Vein attrition was greatest during the first year following diagnosis. Ninety-seven veins remained patent for ≥3 years, including 73 with recurrent disease. Among these veins, the median reintervention rate decreased significantly from 2 (IQR: 1–4) during the first 3 years to 1 per year (IQR: 1–3) thereafter (p < 0.01) while maintaining continued patency. Conclusions: In pediatric PVS, the first 3 years after diagnosis represent a critical period of active disease with high rates of vein attrition. Veins maintaining patency beyond this period demonstrate a reduced reintervention rate, suggesting stabilization of disease progression. Aggressive surveillance and intervention during this therapeutic window may improve long-term vein and patient outcomes. Full article

Background: Although most patients with univentricular physiology undergo staged palliation during childhood, a small subset reaches adolescence or adulthood without complete cavopulmonary connection. The role of late total cavopulmonary connection (TCPC) in these patients remains poorly defined. This review summarizes the available evidence regarding TCPC surgery in adolescents and adults and discusses contemporary patient selection. Methods: A structured narrative review of PubMed/MEDLINE was conducted from database inception to December 2024. Studies reporting primary Fontan or TCPC completion in adolescents and adults were included, whereas studies exclusively addressing Fontan conversion were excluded. The review was conducted according to SANRA recommendations. Results: Sixteen studies reporting outcomes after late TCPC completion were identified. Most adult candidates presented with cyanosis, reduced functional capacity, previous palliation, and dominant left ventricular morphology. Surgical techniques evolved from atriopulmonary connections to lateral tunnel and extracardiac conduit TCPC. Early postoperative mortality ranged from 0% to 20%, with low cardiac output syndrome representing the leading cause of death. Reported survival at 5, 10, and 15 years ranged from 76 to 95%, 71–81%, and 66–79%, respectively. Preserved ventricular function, low pulmonary vascular resistance, and absence of significant atrioventricular valve regurgitation were consistently associated with better outcomes. Conclusions: Late TCPC surgery in carefully selected adults is feasible and may improve oxygenation and functional status. Contemporary decision-making should integrate anatomical, hemodynamic, functional, rhythm, and end-organ assessment while considering alternative strategies such as transplantation or conservative management. Full article

Background: Necrotizing enterocolitis (NEC) is a severe gastrointestinal complication that may develop in term neonates with critical congenital heart disease (CHD), despite the absence of prematurity. We aimed to evaluate the incidence of NEC and identify clinical factors associated with NEC development in term neonates with critical CHD. Methods: This retrospective cohort study included term neonates (≥37 weeks’ gestation) with critical CHD admitted to a tertiary pediatric cardiac intensive care unit between 1 January 2022, and 1 January 2026. NEC was defined as stage IIA or higher according to modified Bell’s criteria. Demographic, clinical, perioperative, and nutritional variables were analyzed. Multivariable logistic regression analysis was performed to identify factors independently associated with NEC development. Results: A total of 780 term neonates with critical CHD were included. NEC developed in 18 infants (2.3%). The median age at NEC diagnosis was 7 days (IQR 5–10), and four patients (22%) required surgical intervention. Seven NEC cases occurred preoperatively, whereas 11 developed after cardiac surgery; among postoperative cases, 64% occurred within the first 72 h after surgery. Birth weight < 2.5 kg (aOR 4.1, 95% CI 1.2–13.4; p = 0.021), gestational age < 38 weeks (aOR 3.9, 95% CI 1.3–12.0; p = 0.018), functional single-ventricle physiology (aOR 5.8, 95% CI 1.6–20.7; p = 0.007), and mechanical ventilation dependency (aOR 5.1, 95% CI 1.7–15.2; p = 0.004) were independently associated with NEC development. Mortality was significantly higher among infants with NEC compared with those without NEC (50% vs. 7.7%, p < 0.001). Conclusions: NEC remains an important complication associated with substantial mortality in term neonates with critical CHD, despite its relatively low incidence. Early-term birth, low birth weight, functional single-ventricle physiology, and dependence on mechanical ventilation were independently associated with increased NEC risk in this vulnerable population. Full article

Introduction: Congenital heart disease remains the most common congenital anomaly and a leading cause of infant morbidity and mortality. Although the three-vessel and trachea view has substantially improved prenatal assessment of the great arteries and aortic arch anatomy, detailed evaluation of the distal arch, aortic isthmus, and arch convergence may remain challenging in routine fetal echocardiography. Methods: A structured literature review of PubMed-indexed studies was conducted to examine the anatomical basis, clinical applications, diagnostic performance, and technological developments related to the three-vessel and trachea views. The reviewed evidence served as the scientific basis for the development of the proposed Three-Y and Trachea concept. Results and Discussion: The three-vessel and trachea view provides a standardized transverse assessment of the great vessels and contributes substantially to the prenatal detection of conotruncal and aortic arch anomalies. Building on this established approach, the proposed Three-Y and Trachea concept emphasizes the Y-shaped convergence of the ductal arch and transverse aortic arch at the level of the aortic isthmus, while preserving the trachea as a key anatomical landmark. This additional anatomical perspective may facilitate assessment of arch continuity, aortic isthmus morphology, and flow relationships within the upper mediastinum while highlighting the anatomical and hemodynamic significance of the aortic isthmus within the fetal circulation. Conclusions: The Three-Y and Trachea view represents a conceptual extension of the conventional three-vessel and trachea view that may offer a more comprehensive framework for anatomical and functional evaluation of the fetal aortic arch and aortic isthmus. Further prospective studies are required to assess its feasibility, reproducibility, and potential clinical value in prenatal cardiac screening. Full article

Background/Objectives: Despite advances in transcatheter therapy, surgery remains the gold standard treatment for sinus venosus defect (SVD). However, data on long-term outcomes following surgical repair remain scarce. This multicenter study evaluates the long-term outcomes of surgically treated SVD patients with comparison to surgically and percutaneously treated secundum atrial septal defect (ASD). Methods: Clinical, surgical, imaging and invasive data were retrospectively reviewed from eight centers in Europe. Results: A total of 209 patients were included, of whom 80 were surgically treated SVD, 57 surgically treated secundum ASD and 72 percutaneously treated secundum ASD. Operation for SVD mainly occurred in adulthood, with a median age of 28.5 years (2–68 years). During follow-up, the observed reoperation rate was higher in the SVD cohort than ASD cohorts (p = 0.033), despite shorter follow-up period in the SVD cohort. Reoperations, in the SVD cohort, occurred at a median of 28 years (1–50 years) after index surgery. The need for pacemaker implantation was similar between groups (p = 0.301). The prevalence of late atrial fibrillation did not differ between groups (p = 0.588). Surgically treated SVD tended to have a higher prevalence of atrial flutter and atrial tachycardia, with a significantly higher rate of electrophysiological studies than percutaneously treated secundum ASD (p = 0.013), similar to surgically treated secundum ASD (p = 0.405). Conclusions: Long-term follow-up of surgically treated SVD is essential to monitor for reintervention and arrhythmic complications. Full article

Background: In congenital heart disease patients with single-ventricle physiology, accurate anatomical and hemodynamic evaluation prior to the Fontan completion procedure is critical for surgical planning and long-term outcomes. Although conventional catheter angiography (CCA) remains the reference standard for hemodynamic evaluation, cardiac computed tomography angiography (CTA) has emerged as an increasingly valuable tool for anatomical assessment in the preoperative evaluation of Fontan candidates. Objective: To compare the anatomical and hemodynamic information provided by CTA and CCA before Fontan completion and to evaluate their complementary roles in the preoperative assessment of pediatric patients with single-ventricle physiology. Methods: This single-center, retrospective study included 32 patients with single-ventricular physiology who underwent staged palliation and Fontan completion. All patients underwent TTE and CCA. Twenty-three patients also underwent cardiac CTA with three-dimensional reconstruction. Anatomical and hemodynamic findings obtained from CTA and CCA were compared, with particular focus on extracardiac anatomy, collateral vessels, and findings relevant to surgical planning. Results: Cardiac CTA showed concordant detection of clinically significant anatomical findings identified by CCA, while providing a more comprehensive assessment of extracardiac anatomy. While CCA remained superior for hemodynamic assessment, CTA provided more comprehensive evaluation of extracardiac anatomy. In this cohort, catheterization findings did not alter the planned surgical strategy (0%, 95% CI 0–10.9%). Conclusions: CTA may provide valuable anatomical information for preoperative assessment and surgical planning; however, larger prospective multicenter studies are required before less invasive pre-Fontan evaluation strategies can be considered. Full article

Background: The purpose of the study is the assessment of mortality from cardiovascular diseases (CVDs) and their dose–response relationships and the calculation of the number of years of life lost (YLL) in Kazakhstani residents living in territories around the Semipalatinsk nuclear test site. Materials and Methods: The study is based on the State Scientific Automated Medical Registry (SSAMR) database. The study included 3482 residents of the Abay and Beskaragai districts exposed to radiation and 1886 residents of the Kokpekty district (control group). The median equivalent radiation dose for the exposed group was 864.0 mSv, compared to 64.4 mSv in the control group. The study period was from 1949 to 2024. Results: Mortality rates in the exposed group exceeded those of the comparison group throughout the study. The relative risk (RR) of mortality was 1.41 for all CVDs, 2.0 for stroke, 7.88 for chronic cerebrovascular disease (CCVD), and 2.39 for congenital heart disease (CHD). Age-standardized mortality rates were higher in the radiation-exposed population, with the highest excess risk recorded in 1960–1964 (RR = 5.31; 95% CI 4.32–6.53). The number of YLL from acute myocardial infarction (AMI) was 6097.0 in the exposed group versus 5893.0 in the comparison group, 3857.5 from hemorrhagic stroke versus 1996.9, and 2696.6 from CHD versus 957.7. An increase in radiation dose by 1 cSv was associated with an 8.5% increase in the odds of death from CVDs (OR = 1.085; 95% CI 1.075–1.094; p < 0.001). Radiation dose demonstrated good predictive ability for mortality from cardiovascular diseases (AUC = 0.700). Conclusions: The results indicate an increased risk of CVD mortality among residents of radiation-contaminated areas of Kazakhstan throughout the study period. Full article

Ventricular tachycardia and sudden cardiac death remain the principal late causes of mortality in repaired tetralogy of Fallot. Clinical practice is evolving from a “reactive” paradigm centered on defibrillator therapy and post-event ablation toward a “proactive” paradigm targeting slowly conducting anatomical isthmuses before clinical arrhythmias become manifest. Monomorphic ventricular tachycardia in this population typically occurs due to a discrete, anatomically defined set of slowly conducting isthmuses bounded by surgical patches or incisions and valve annuli. Substrate-targeted catheter and surgical ablation are technically feasible, safe, and associated with high arrhythmia-free survival when complete bidirectional block is achieved. The current indication for “proactive” ablation is for substrate evaluation before transcatheter pulmonary valve replacement, after which endocardial access to the dominant isthmus may be permanently obscured. Pulmonary valve replacement alone does not abolish the arrhythmogenic substrate, thus providing the rationale for combining valve intervention with proactive ablation. This narrative review discusses substrate biology, risk stratification, comparative outcomes of reactive and proactive ablation strategies, and the role of pulmonary valve replacement. It also proposes an operational pathway integrating both approaches within shared decision-making. The ongoing CATAPULT-TOF study and subsequent multicenter work will determine the populations in which proactive substrate evaluation should become routine. Full article

Background: Trisomy 21 (T21) is strongly associated with congenital heart disease, particularly atrioventricular septal defect (AVSD), ventricular septal defect (VSD), atrial septal defect (ASD) and selected conotruncal and arch anomalies. First- and second-trimester ultrasound, Doppler and fetal cardiac MRI enable increasingly early and detailed characterization of these lesions, while advances in molecular cardiogenesis have linked specific phenotypes to dosage-sensitive genes on chromosome 21. Methods: This narrative review synthesizes contemporary evidence on structural and functional cardiovascular anomalies in T21 fetuses in the first and second trimester, integrating fetal echocardiography, Doppler assessment and fetal cardiac MRI with embryologic and molecular insights, and summarizing trimester-specific detectability and pathophysiologic links to candidate genes in the Down syndrome-critical region. Approximately one quarter to one third of T21 fetuses have major congenital heart disease on high-quality prenatal echocardiography, with AVSD representing about half of all lesions and VSD, tetralogy of Fallot (TOF), arch anomalies, venous return abnormalities and functional markers (increased nuchal translucency, tricuspid regurgitation, ductus venosus abnormalities) comprising the remainder. Results: First-trimester detection relies on functional markers and early four-chamber and outflow-tract views, whereas second-trimester studies refine anatomic definition and hemodynamics, with MRI reserved for complex cases. Overexpression of genes such as DSCAM, COL6A1/COL6A2, DYRK1A and RCAN1 perturbs endocardial cushion, conotruncal and vascular development. Conclusions: Early, protocol-driven cardiac imaging in T21 supports timely diagnosis, risk stratification and multidisciplinary counselling, and links fetal imaging phenotypes with chromosome 21 gene dosage to advance personalized management and future genotype–phenotype research. Full article

Background/Objectives: While the 2024 international expert consensus acknowledges that operator-managed sedation (OMS) may provide equal safety to anesthesiologist-directed care, it emphasizes clinical judgment over numerical risk scores alone, yet supporting institutional data remain limited. We evaluated the safety outcomes and risk factors for adverse events (AEs) during OMS at a single tertiary center. Methods: We retrospectively reviewed 342 cardiac catheterization procedures in pediatric patients under six years of age performed under OMS between 2020 and 2022. Results: A total of 342 cardiac catheterization procedures were performed in 307 patients, of which 30.4% were diagnostic. The overall incidence of AEs was 7.0%, and all events were successfully managed with complete recovery. In the univariate analysis, neonatal age (OR = 13.5, p < 0.001), body weight ≤ 10 kg (OR = 3.04, p = 0.01), and genetic abnormalities (OR = 3.92, p = 0.023) were identified as significant risk factors. Neonatal age and genetic abnormalities remained significant in the multivariate analysis. The incidence of AEs increased with higher Catheterization Risk Score for Pediatrics (CRISP) scores, showing a significant linear trend (p < 0.001), whereas no sedation-related AEs occurred in patients with scores below 2. Conversion to GA occurred only in patients with CRISP scores above 5. Conclusions: OMS can be performed safely across a wide CRISP score range in experienced centers. Neonatal age and genetic abnormalities were associated with increased AE risk; however, case selection should be guided by each patient’s overall clinical picture rather than any single risk factor alone. Full article

Background/Objectives: Adults with single-ventricle physiology (SVP) represent a growing population with complex cardiovascular conditions and an increasing need for noncardiac surgical and interventional procedures. However, perioperative outcomes in this group remain poorly characterized. This study aimed to provide a descriptive characteristic of perioperative management, complications and mortality in adults with SVP undergoing noncardiac surgical and interventional procedures. Methods: We conducted a retrospective cohort study including all adult patients (≥18 years) with SVP who underwent noncardiac surgical and interventional procedures requiring anesthesia or sedation at a tertiary university hospital between 1 January 1995 and 30 November 2023. Demographic data, comorbidities, type of procedure and anesthetic technique were collected. Complications were defined as intraoperative or postoperative adverse events requiring intervention or associated with hemodynamic, respiratory, or cardiovascular instability. Primary outcomes were perioperative complications and all-cause mortality at 24 h, 30 days, and one year, with mortality reported at the patient level. Results: A total of 114 procedures were performed in 67 patients (mean age 32.3 ± 10.8 years). Most procedures were elective (78.9%) and minimally invasive, frequently performed under sedation, with or without local anesthesia (67.5%). Common comorbidities included arrhythmias (46.3%), liver disease (49.3%), and heart failure (17.9%). The overall complication rate was 6.1% (2.6% intraoperative, 3.5% postoperative). Mortality was 1.5% in 24 h, 2.9% in 30 days and 5.9% at one year. Most clinically relevant adverse events occurred in patients with earlier-stage palliation, advanced functional limitation or multiple comorbidities. Conclusions: Perioperative outcomes in adults with SVP undergoing noncardiac surgical and interventional procedures were acceptable when procedures were elective and managed in specialized settings. Risk remains heterogeneous and appears to be influenced by physiological status and stage of palliation. Full article

Background: The prognostic value of electrocardiography (ECG)- and continuous rhythm monitoring (CRM)-derived markers for predicting atrial fibrillation (AF) onset and progression remains unclear in patients with congenital heart disease (CHD). Methods: We retrospectively analyzed 573 CHD patients who underwent 24 h Holter monitoring between 2003 and 2015. Baseline ECG and CRM parameters were assessed. Cox regression identified predictors of new-onset AF and AF progression, and interaction analyses explored effect modification by left atrial (LA) dilatation. Results: During 13 ± 5 years of follow-up, AF occurred in 107 patients (18.7%), of whom 32 (29.9%) progressed to persistent/permanent AF (PeAF). Patients with AF more frequently had prolonged PR and QTc intervals and higher atrial ectopy (AE) and ventricular ectopy burdens. Independent predictors of new-onset AF were older age, LA dilatation, higher AE burden, atrial tachycardia, and pacemaker implantation. AF progression was independently associated with older age, LA dilatation, higher AE burden, and prolonged PR interval. AE burden showed a stronger association with AF risk in patients without LA dilatation. Conclusions: In CHD patients, baseline ECG PR-intervals and CRM-derived AE burden independently predict AF onset and/or progression. These noninvasive markers may improve risk stratification and support earlier personalized rhythm management. Full article

Pediatric congenital right heart obstructive lesions encompass a spectrum of diseases that obstruct blood flow from the right atrium to the pulmonary artery. Right ventricular inflow obstructions include tricuspid valve abnormalities, such as Ebstein anomaly, tricuspid valve dysplasia, and tricuspid atresia. Right ventricular outflow obstructions include pulmonary valve stenosis, pulmonary atresia, and tetralogy of Fallot. Cardiac computed tomography angiography (CCTA) is a valuable tool for the diagnosis, treatment planning, and follow-up of these lesions. In this pictorial review, we highlight the diagnostic utility of CCTA in congenital right heart obstructive lesions, emphasizing its role in preoperative planning. Full article

Background: It is rare in clinical practice to encounter a common arterial trunk when the ventricular septum is intact. In this setting, other clinical diagnoses, such as hypoplastic left heart syndrome with aortic atresia, may be mistaken for a common arterial trunk. Data for this combination is largely limited to case reports and small case series. We have conducted a systematic review of reported cases, performing cluster analyses to provide an objective grouping of the cases. Methods: A systematic review of the literature was performed to identify cases of a common arterial trunk with an intact ventricular septum. Cases for which individual data were available were included in the final analyses. Cluster analysis using K-means clustering was conducted to provide an objective grouping of the hearts based on morphologic findings. Results: K-means clustering identified three distinct groups among hearts with a common arterial trunk with intact ventricular septum. The commitment of the common ventriculo-arterial junction to the left, right, or both ventricles was the defining feature of each group. Hearts with a common trunk committed to one of the ventricles demonstrated significant hypoplasia or atresia of structures related to the other ventricle. Conclusions: Distinct patterns can be identified when a common arterial trunk is found with an intact ventricular septum. They depend on the ventricle or ventricles, which support the common ventriculo-arterial junction. Full article

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly with exceptional survival into adulthood. We present a 66-year-old woman with chest and back pain in whom ALCAPA was diagnosed using coronary computed tomography angiography (CCTA) with curved planar reformation and cinematic volume rendering technique (cVRT). Photorealistic three-dimensional reconstruction provided complementary three-dimensional visualization that may facilitate anatomic understanding and communication of the anomalous origin. Conservative management was adopted given the patient’s age and well-developed collateral circulation. This case underscores the value of advanced CCTA visualization in diagnosing rare coronary anomalies in elderly patients. Full article