OBJECTIVES:To assess international practice patterns for prostaglandin E1 (PGE1) initiation and dosing in neonates with dextro-transposition of the great arteries (d-TGA) and to evaluate whether these practices differ by clinical specialty. METHODS:We conducted an international, cross-sectional, web-based survey of neonatologists and pediatric cardiac intensivists involved in stabilizing neonates with d-TGA. The survey assessed strategies for routine versus selective PGE1 initiation, oxygen saturation thresholds, starting dose, and maximum dose policies. The primary outcome was self-reported routine PGE1 initiation. Categorical variables were compared using χ2 tests, and multivariable logistic regression analysis identified independent predictors of routine initiation, adjusting for specialty, region, experience, cardiology availability, institutional protocols, and sex. RESULTS:A total of 526 clinicians responded (282 neonatologists and 244 intensivists; 65.8% response rate). Neonatologists more frequently reported routine PGE1 initiation than cardiac intensivists (84.0% vs 58.6%, P < .001). Starting dose selection differed by specialty (P < .001), whereas oxygen saturation thresholds did not (P = .436). In adjusted analysis, cardiac intensivists were less likely than neonatologists to report routine initiation (adjusted OR 0.15, 95% CI 0.05-0.45). CONCLUSIONS:Substantial specialty-based variation exists in PGE1 initiation and dosing for neonatal d-TGA stabilization. Because both routine and selective strategies have plausible physiologic rationale, this heterogeneity should be interpreted as hypothesis-generating, identifying the interspecialty divide as a priority target for prospective, outcome-linked comparative-effectiveness research that should precede any consensus recommendation. Limitations include the survey design, collinearity between specialty and care setting, and lack of patient-level outcome data.
IntroductionExtracorporeal membrane oxygenation (ECMO) is becoming more prevalent post-Norwood procedure, and its use has been linked to reduced survival, with wide variability between centres. The reasons for this variability, particularly the roles of anatomy, surgical technique and ECMO indication, remain poorly understood. This study examines associated factors with survival to discharge in patients undergoing post-Norwood ECMO.MethodsWe systematically searched PubMed, EMBASE, Cochrane Central and Web of Science for relevant variables. The analysed variables included operative parameters, ECMO variables and postoperative complications during ECMO support post-Norwood procedure. Statistical analyses were performed using RevMan 5.4 to calculate pooled risk ratios, odds ratios (OR) or mean differences (MD) with 95% confidence intervals (CIs) using random-effects models.ResultsA total of 8 studies were included (1614 neonates; 41% post-Norwood ECMO survivors). Cardiopulmonary bypass and ECMO durations were significantly longer in the non-survivor group (MD = -24.33, CI = -39.19 to -9.47, p = .001) and (MD = -42.76, CI = -51.65 to -33.87, p < .00001),respectively. ECMO indications and cannulation technique were similar between the groups. Important complications such as bleeding (OR = 0.67, p = .003), neurological injury (OR = 0.55, p < .0001), arrhythmia (OR = 0.45, p < .00001) and cardiac tamponade (OR = 0.49, p = .03) were all significantly greater in the cohort of neonates that did not survive to discharge.ConclusionFor infants requiring ECMO support following the Norwood procedure, longer cardiopulmonary bypass times and longer ECMO duration were observed among neonates who did not survive to discharge. ECMO indications showed no clear association with survival. There was a significantly higher incidence of bleeding, neurological injury, and arrhythmia in the non-survivor cohort.
Objective While many studies view patients with hypoplastic left heart syndrome (HLHS) through the lens of financial burden, an overall sense of the medical burden of these patients has rarely been reported. We sought to introduce a granular analysis of the medical burden of patients with HLHS to capture a view of their journey to adulthood. Methods Children with HLHS (≤18 years) born from 2004 to 2008 with 18 years of follow-up were identified in the Pediatric Health Information System (PHIS). Children who did not have 18 years of complete follow-up were excluded. Results By 1 year of age, a child with HLHS had a total median number of visits of 4 (3-6), length of stay (LOS) of 52 days (d) (29-87), 2 (1-3) cardiac catheterizations, 55 (42-74) unique drugs, and 4% cardiac transplant prevalence. By 5 years of age, a child with HLHS had a total number of visits of 10 (6-18), LOS of 73d (46-119), 3 (2-6) cardiac catheterizations, 89 (70-132) unique drugs, and a 10% cardiac transplant prevalence. By 10 years of age, a child with HLHS had a total number of visits of 18 (8-35), LOS of 85d (54-147), 5 (3-8) cardiac catheterizations, 120 (97-178) unique drugs, and an11% cardiac transplant prevalence. By 18 years of age, an adult with HLHS had a total number of visits of 44 (17-80), LOS of 154d (83-264), 9 (5-12) cardiac catheterizations, 139 (127-220) unique drugs, and a 19% cardiac transplant prevalence. Conclusion The present report offers detailed insight into the medical burden of patients with HLHS and can serve as a reference to inform patients and families of the journey.
BackgroundIn ventricular septal defect (VSD) closure, accurately defining the margins of the VSD is essential for success. Transatrial tricuspid valve detachment (TVD) aims at improving visualization, which is often impaired by the tricuspid valve apparatus. However, this may affect operative time, atrioventricular (AV) conduction, and tricuspid valve function.MethodsAll pediatric patients who underwent transatrial isolated VSD closure from 2011 to 2020 were studied. Based on the use of TVD, postoperative outcomes, including tricuspid valve function, incidence of residual VSD and postoperative heart block, were compared between the two groups.ResultsOf 268 patients, 88 (32.8%) underwent VSD closure with TVD and 180 (67.2%) without TVD. Preoperative characteristics were similar, including median age and weight (13.5 vs 12.5 months and 7 vs 6.9 kg; P = .98 and P = .60). The VSDs were predominantly perimembranous (83% [73/88] vs 88.3% [159/180]; P = .17), and of similar size (7.8 vs 7.5 mm; P = .32). There were no significant differences in the degree of postoperative tricuspid regurgitation (TR) (>mild 7.1% [6/84] vs 5% [9/180]; P = .38), small residual VSD (11.9% [10/84] vs [35/180] 19.6%; P = .12), or incidence of transient heart block (2.3% [2/84] vs 4.4% [8/179]; P = .38). At the median follow-up time of 38.3 months, there were no reoperations for TR nor residual VSD. Only one patient from the non-TVD group needed a reoperation for a permanent pacemaker. The cardiopulmonary bypass and cross-clamp times were longer in the TVD group (70 ± 25 vs 61 ± 19 min; P = .002) and (50 ± 18 vs 40 ± 15 min; P < .001).ConclusionsThe tricuspid valve detachment technique for VSD closure is associated with comparable postoperative tricuspid valve function and AV conduction when compared with nondetachment techniques. It is a safe and effective approach for challenging-to-assess transatrial VSD closures.
Window ductus (WD) is a very rare and anatomically distinct variant of patent ductus arteriosus, also known as a type B patent ductus arteriosus. We report the case of a neonate who was born with a rare triad of congenital heart defects including a large WD, multiple ventricular septal defects (VSD), and an atrial septal defect (ASD). The child had significant left-to-right shunting and pulmonary hypertension, and successfully underwent complete surgical repair. At 11 years of follow-up, the patient is doing well with good flow in both the aortic arch and the pulmonary arteries. To the best of our knowledge, this is the first case of WD diagnosis associated with multiple VSDs and an ASD that has been described in the literature.
BackgroundA subset of children with congenital heart disease (CHD) travel internationally for definitive cardiac surgery, yet outcome data for selectively referred international patients undergoing complex congenital cardiac procedures remain limited. We describe results for international pediatric cardiac patients operated on within a structured referral model at a US tertiary congenital cardiac center.MethodsFrom September 2020 to July 2025, 1465 patients underwent cardiac surgery at our institution. Sixty-six consecutive international patients underwent 117 procedures, coordinated through Global Services. Demographics, diagnoses, operative variables, and outcomes were analyzed.ResultsMedian age was 53 months (interquartile range [IQR] 12-140) and weight 15.6 kg (IQR 8.5-39.5); 62% (41/66) were male. Referring countries were the United Arab Emirates (37/66, 56%), Kuwait (35%, 23/66), and Qatar (9%, 6/66). Prior cardiac surgery occurred in 74% (49/66), and 58% (38/66) had ≥2 sternotomies; 25% (17/66) had single-ventricle physiology. Society of Thoracic Surgeons - European Association for Cardio-Thoracic Surgery Congenital Heart Surgery category 3 to 5 procedures accounted for 48% (32/66) of the cases. Median cardiopulmonary bypass time was 174 min (IQR 120-255) and cross-clamp time 83 min (IQR 33-137). There was no hospital mortality. Early reoperation occurred in 7.5% (5/66) (3% for bleeding). Median intensive care unit and hospital stay were 5 (IQR 3-12) and 11 days (IQR 7-30). Major complications included extracorporeal membrane oxygenation, stroke, and dialysis in 1 patient each (1.5% each) and vocal cord injury in 6/66 (9%). Over a mean follow up of 312 days, 2 late deaths occurred (6 months and 1 year).ConclusionsInternational patients with CHD can achieve excellent early outcomes despite high complexity when managed at experienced centers with structured referral coordination and multidisciplinary infrastructure.
Congenital heart disease (CHD) is the leading cause of pediatric heart disease in Africa. In Uganda, approximately 16,000 infants are born annually with CHD, yet only a small proportion access timely surgery due to financial constraints and a shortage of pediatric cardiology and surgical expertise. Amid prolonged surgical waiting lists and ongoing efforts to expand local capacity, we introduced a morale-strengthening strategy focused on celebrating incremental surgical milestones to enhance motivation, ownership, and continuous quality improvement. The Brave Hearts Children's Initiative was launched on December 7, 2023, at the Uganda Heart Institute under the theme “Little Hearts, Big Courage.” Initially conceived as a children's Christmas event, it evolved into a structured platform for advocacy, family support, and fundraising. The initiative highlights annual achievements in pediatric cardiac surgery and brings together families, clinicians, and key stakeholders, including government, academic institutions, and international partners. A key system change involved shifting surgical listing and patient selection from the surgical team to the cardiology team, improving coordination and reducing delays. This restructuring, alongside increased government and partner support, was associated with improved surgical output. Following the second Brave Hearts Children's event, surgical volume rose to 8 to 12 open-heart and approximately 8 closed-heart procedures per month. The waiting list is now systematically managed, team engagement has improved, and families report better psychosocial support. Celebrating milestones beyond statistics can strengthen team cohesion, build stakeholder trust, and support sustainable growth of pediatric cardiac services in resource-limited settings.
ObjectivesThe objective of this study was to evaluate the postoperative outcome of minimally invasive surgical repair of partial atrioventricular septal defects (AVSD), focusing on surgical safety and postoperative left atrioventricular (AV) valve function.MethodsWe retrospectively reviewed the clinical data of 32 consecutive patients who underwent minimally invasive right vertical infra-axillary thoracotomy approach for repair of partial AVSD at our institution between January 2020 and December 2023. Left AV valve function was evaluated by echocardiography, and Fisher's exact test was used to compare the number of patients who had moderate left AV valve regurgitation pre- and 1-year postoperatively.ResultsThe median age at repair was 2.4 years, and the median body weight was 12 kg. The mean follow up after surgical repair was 2.8 years, with no mortality or surgical reintervention. There was no conversion to median sternotomy, hemorrhage, pneumothorax, or diaphragm paralysis. However, complete heart block developed in 1 patient (1.5 years old, 9 kg). Thirty patients underwent isolated left AV Valve cleft closure, and 4 patients underwent additional left AV valve annuloplasty. Preoperative echocardiogram showed moderate or more left AV valve regurgitation in 19/32 patients (60%), which improved significantly after the surgical repair. Postoperative echocardiograms showed mild or less left AVvalve regurgitation in 31/32 patients (97%) (P < .01). However, 1 patient (4 months old, 5.5 kg) had moderate left AV valve regurgitation from a residual cleft.ConclusionsRight vertical infra-axillary thoracotomy can be safely performed in patients with partial AVSD, and the short-term outcome of the left AV valve function was satisfactory.
An 11-year-old patient with partial anomalous venous connection, atrial septal defect (ASD), and persistent left superior vena cava underwent a closed-chest, totally thoracoscopic robotic repair using the Warden technique. The patient was extubated in the operating room and stayed in the intensive care unit for 24 h. He was discharged home on day 5 without complications. At 1-year follow-up, the right ventricular size had improved, and tricuspid regurgitation was reduced. He remains asymptomatic and tolerates physical activity adequately. Minimally invasive robotic surgery for pediatric and congenital heart pathologies appears to be safe and feasible, potentially offering a swifter return to daily and even sports activities.
ObjectivesNeonates and infants with conotruncal heart defects often require placement of valved conduits to establish continuity between the right ventricle (RV) and pulmonary arteries (PA). The objective of this study was to assess the function and reintervention profile of RV-PA conduits implanted in patients who are less than 1 year of age.MethodsNinety-one infants underwent placement of an RV-PA conduit at a single institution between 2012 and 2023. Patient records and imaging were reviewed for demographics, operative characteristics, and postoperative outcomes.ResultsThe median age and weight at the time of index RV-PA conduit were 15 (IQR 6-174) days and 3.6 (IQR 3.0-6.5) kg. The median conduit diameter was 10 (IQR 9-12) mm, and the most common conduit was a pulmonary homograft (45%, 41/91). There were no mortalities at the index operation. Median follow-up was 5.8 (IQR 2.8-9) years.All patients in the cohort have undergone at least 1 conduit replacement at a median interval of 27.2 (IQR 10.1-53.2) months, with 24% of those patients (22/91) being less than 1 year of age at first replacement. There were 7 deaths following the first reoperation over a median follow-up of 14.2 (0.6-59.5) months, and 86% (6/7) of those who died were less than 1 year of age at the time of conduit replacement.ConclusionsThese results benchmark contemporary outcomes of right ventricle to pulmonary artery conduit durability in neonates and infants. There is significant morbidity and mortality associated with reoperation for conduit replacement at less than 1 year of age.
BackgroundMinimally invasive cardiac surgery aims to reduce surgical trauma while maintaining excellent outcomes. Evidence comparing minimally invasive right thoracotomy with conventional median sternotomy for septal defect closure remains limited in large contemporary cohorts. We compared perioperative and short-term outcomes between these approaches for atrial septal defect (ASD) and ventricular septal defect (VSD) repair.MethodsWe retrospectively analyzed 527 consecutive patients undergoing surgical ASD or VSD closure between January 2024 and October 2025 at the National Heart Institute, Kuala Lumpur. Patients were stratified by surgical approach: minimally invasive right thoracotomy (MIS, n = 81) and median sternotomy (MS, n = 446). Propensity score matching (1:1) was performed using logistic regression incorporating age, body surface area, defect type, and relevant comorbidities, yielding 79 well-balanced matched pairs (standardized mean difference <0.1). Primary endpoints included perioperative mortality, major morbidity, intensive care unit (ICU) length of stay, and duration of mechanical ventilation.ResultsAfter propensity score matching, baseline characteristics and operative times were comparable between groups. No perioperative mortality occurred in either group. MIS was associated with significantly shorter ICU stay (median 2.0 vs 3.0 days, P < .001) and reduced duration of mechanical ventilation (7.5 vs 11.4 h, P = .007). Total hospital length of stay did not differ significantly (8.2 vs 8.8 days, P = .367). Complication profiles differed: thoracotomy patients experienced higher rates of diaphragm paralysis (6.3% [5/79] vs 2.5% [2/79]) and pneumothorax (10.1% [8/79]vs 6.2% [5/79]), whereas sternotomy patients had higher incidences of pleural effusion (1.3% [1/79] vs 0%), chylothorax (1.3% [1/79] vs 0%), pericardial effusion (5.1% [4/79] vs 0%), and ventilation >48 h duration (5.1% [4/79] vs 0%). Reintervention was required in 1 of 79 thoracotomy patients (1.3%) for ASD closure revision. At median follow-up of 0.1 to 0.3 years, 100% of patients were alive with no significant difference in reintervention-free survival (log-rank P = .249).ConclusionsMinimally invasive right thoracotomy for ASD and VSD closure shortens ICU stay and ventilatory support without compromising operative or short-term outcomes compared with median sternotomy. However, the higher observed rates of phrenic nerve injury and pneumothorax in the thoracotomy cohort represent clinically important safety considerations that warrant transparent informed consent and meticulous surgical technique. In carefully selected patients at experienced centres, this approach remains a sound and reproducible alternative to conventional sternotomy.
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) arising from the nonfacing sinus presents a significant technical challenge because direct reimplantation may not be feasible due to insufficient coronary length. We describe a dual aortic-pulmonary flap technique enabling anterior tension-free reimplantation.A 6-month-old infant with mildly reduced left ventricular systolic function secondary to ALCAPA originating from the anterior nonfacing pulmonary sinus underwent surgical repair. Following maximal coronary mobilization, a large anterior pulmonary artery flap and a posterior-based aortic flap were constructed and sutured together to create a tubular native arterial extension, permitting tension-free anterior implantation. The anterior pulmonary valve commissure was temporarily detached and subsequently resuspended.Following reimplantation, left ventricular ejection fraction improved from 54% preoperatively to 62% at discharge and mitral regurgitation regressed from moderate to trivial at 1-year follow up. Good coronary artery flow was demonstrated on echocardiogram and the patient remains asymptomatic at 1-year follow up.The dual-flap technique provides additional coronary length using exclusively native arterial tissue and facilitates anterior tension-free reimplantation in selected cases of nonfacing sinus ALCAPA. Further follow up is required to determine long-term durability.
The ductal malformation known as a "Window Ductus" or "type B ductus" is a very rare congenital anomaly. The unique anatomy results in a diagnostic and surgical dilemma. It can easily be mistaken for a "typical" patent ductus arteriosus and approached via left thoracotomy. This can potentially result in a surgical misadventure. We report a patient diagnosed with a Window Ductus at 2 months of age with recurrent upper respiratory tract infections and complete left lung collapse. Repair was performed through a median sternotomy on cardiopulmonary bypass. The Window Ductus was clamped only on the aortic side and divided, the aortic cuff was closed primarily, and the resultant opening in the pulmonary artery was patched with autologous pericardium. The patient had a successful repair and complete reexpansion of the left lung over time. Advanced cross-sectional imaging (CT scan) provides the best views for planning the surgical approach for a Window Ductus. Median sternotomy with cardiopulmonary bypass allows a safe and reproducible repair.
BackgroundTetralogy of Fallot with absent pulmonary valve syndrome (TOF-APVS) presents significant surgical challenges in infants due to airway compression from dilated branch pulmonary arteries. Surgery typically involves closing the ventricular septal defect (VSD) and reducing the size of the pulmonary arteries to relieve airway compression. A competent pulmonary valve is especially beneficial in these cases. We describe creating an Ozaki pulmonary valve using native pericardium for this condition.Material and MethodsFour infants (median age 4.8 months [IQR 4.3-5.4], median weight 3.5 kg [IQR 2.0-5.0]) presented with respiratory distress and were diagnosed with TOF-APVS. The presence of bronchial compression was confirmed with computed tomography of the thorax. All patients underwent total intracardiac repair, including closure of the VSD, the Lecompte maneuver, reduction of the enlarged pulmonary arteries, and bicuspid pulmonary valve reconstruction using glutaraldehyde-treated pericardium with the Ozaki technique. Postoperative transesophageal echocardiogram confirmed well-functioning pulmonary valves with minimal pressure gradients. Each patient recovered uneventfully, was discharged, and is doing well on follow-up.ConclusionThe Ozaki technique facilitates precise and symmetric reconstruction of the bicuspid pulmonary valve in cases of TOF-APVS, demonstrating encouraging early results for infants, particularly in resource-limited environments.
We present the unique combination of a criss-cross heart and common arterial trunk, our management strategy, and challenges of the treatment course. The child was born weighing 2.4 kg as one of twins at the 36th week of gestation. Bilateral pulmonary artery (PA) banding was performed initially as symptoms of congestive heart failure manifested. The decision to proceed with a 2-stage approach was made due to the low body weight (<2.5 kg) and distorted anatomy, suspected to affect the position of a right ventricle to pulmonary artery (RV-PA) conduit. During the interstage period, the patient developed moderate to severe truncal valve insufficiency. At the age of 8 months, complete repair was performed that included truncal valve repair. The ventricular septal defect was closed, and a 14 mm RV-PA conduit was implanted. Pericardial patch augmentation of the right ventricular outflow tract and both PA branches was performed in order to insure unobstructed pulmonary blood flow. The patient developed severe truncal valve regurgitation on postoperative day 3. A 21 mm mechanical valve was implanted utilizing several maneuvers to fit the over sized valve with the intention of decreasing the reoperation burden. To the best of our knowledge, only one published case report of this rare combination of congenital heart defects could be identified in the literature, and we believe this is the first case that was surgically repaired.
We present a 6-year-old girl with neoaortic route stenosis and neoaortic valve regurgitation following a Yasui operation. Despite progressive neoaortic route obstruction, systemic output was maintained through the native aortic valve, which grew sufficiently over time to allow an elective Yasui takedown. The Damus-Kaye-Stansel anastomosis was disconnected, the aortic arch was patched, and the Yasui baffle was removed, restoring neoaortic valve leaflet mobility. The right ventricle to pulmonary artery conduit was removed and the right ventricular outflow tract was reconstructed using the native pulmonary trunk after closing a diminished ventricular septal defect. The postoperative course was uneventful, without recurrent aortic stenosis or pulmonary regurgitation.
AimThe present study was done to compare the hemodynamic effects of 3 drugs-milrinone, levosimendan, and dobutamine in patients undergoing bidirectional cavopulmonary connection (BCPC) surgery.Materials and MethodsIn this prospective observational study, a total of 75 patients between 3 months and 2 years of age who underwent BCPC were included. They were divided into milrinone (M), levosimendan (L), and dobutamine (D) groups. The primary aim was to compare the hemodynamic effects of 3 drugs in this subset of patients.ResultsThe mean age at surgery was 6.4 ± 2.6 months, with no significant differences in gender or weight among the groups. Perioperatively, heart rate and femoral venous pressure were significantly higher in the dobutamine group. Significant differences were noted in SpO2, Glenn pressures, Glenn pressure to mean arterial pressure (MAP) ratios, and near infrared spectroscopy values, with milrinone showing the most favorable outcomes, followed by levosimendan and dobutamine. Postoperative E/e' values were significantly lower in all groups (P < .001), with milrinone (4.7 ± 0.5) performing better than levosimendan (4.9 ± 0.9) and dobutamine (6.5 ± 1.2). Vasoactive inotropic scores decreased postoperatively in all groups but were significantly higher in the levosimendan group. No significant differences were observed in the duration of mechanical ventilation or intensive care unit stay. Notably, there were no mortalities during the study period.ConclusionsMilrinone offers superior hemodynamic benefits over levosimendan and dobutamine in patients undergoing BCPC using cardiopulmonary bypass. Its ability to significantly reduce Glenn pressure, lower the Glenn pressure to MAP ratio, and improve both systolic and diastolic functions makes it the preferred inotropic agent in this subset of patients.
BackgroundMinimally invasive approaches are increasingly used in congenital cardiac surgery; however, their application remains challenging, particularly in pediatric patients. We report clinical outcomes and technical refinements for minimally invasive congenital cardiac surgery.MethodsThis prospective observational single-center study included 31 patients who underwent minimally invasive congenital cardiac surgery for atrial septal defects, ventricular septal defects, partial atrioventricular septal defects, and other selected lesions using cardiopulmonary bypass between January 2024 and January 2025. Patient selection was based on lesion complexity and anatomical accessibility. Data included demographic characteristics, echocardiographic findings, surgical approach, cannulation strategy, operative times, and postoperative outcomes.ResultsThe study population consisted of 23 females and 8 males, with a median age of 145 months (range: 8-672) and a median weight of 32.5 kg (range: 9-89). Femoral arterial cannulation was performed in 27 patients, with combined femoral and internal jugular venous cannulation used when bicaval drainage was required. Mean aortic cross-clamp time was 51.2 min (range: 20-139). Extubation within 3 h was achieved in 93.5% (29/31) of patients. Mean intensive care unit stay and hospital stay were 2.06 days (range: 1-5) and 6.29 days (range: 4-15), respectively. There was no operative mortality and no conversion to median sternotomy. One of 31 patients (3.2%) developed postoperative atrioventricular block requiring permanent pacemaker implantation. Follow-up of at least 12 months shows satisfactory surgical results.ConclusionsMinimally invasive congenital cardiac surgery is feasible and safe in selected patients. These approaches should be considered as part of a modern congenital cardiac surgery program.
