Complete sternal cleft with pectus excavatum (PE) is a very rare congenital midline defect of the sternum. Most of the published reports describe this entity in isolation without associated intracardiac defects. We report a case of an 11-year-old child with a complete cleft sternum with PE and tetralogy of Fallot (TOF), who presented with cyanotic spells. The child underwent total correction for TOF, followed by chest wall reconstruction in the same setting.

Background:The optimal management of neonates with tetralogy of Fallot (TOF) and unfavorable anatomy remains debated. Early primary repair carries risks, while palliative options such as right ventricular outflow tract (RVOT) stenting have emerged as effective alternatives to conventional shunts. However, outcomes of corrective surgery following RVOT stenting remain underexplored. Patients and Methods:A retrospective study was done on 11 patients with TOF who underwent RVOT stenting followed by surgical repair between April 2021 and March 2025, from a total of 619 TOF surgeries. Data on demographics, intraoperative findings, pulmonary artery (PA) growth, and postoperative outcomes, including 30-day mortality, were collected. PA growth was assessed via z-scores pre- and poststenting. The correlation between time from stenting to surgery and operative duration was evaluated. Results:The median age and weight at surgery were 31 months and 10.2 kg, respectively. Complete stent excision was possible in 91% of patients. Transannular patch repair was required in 90.9% of cases. Prestent PA z-scores improved significantly by the time of surgery (left PA: -1.92 to -0.11; right PA: -2.95 to -0.46). Maximum PA growth occurred within 5 months poststenting. An increased interval between stenting and surgery correlated with prolonged cardiopulmonary bypass and aortic cross-clamp times. No in-hospital mortality or 30-day mortality occurred; the median ventilation was 12.7 h, and the mean intensive care unit stay was 2.1 days. Conclusions:Corrective surgery after RVOT stenting in TOF is safe and feasible, with favorable early outcomes. Early repair - preferably within 5 months of RVOT stenting - facilitates optimal PA growth and minimizes surgical complexity. Larger, prospective studies are warranted to standardize timing and assess long-term outcomes.

BACKGROUND:Fenestration in the Fontan procedure was introduced to improve early postoperative outcomes by reducing systemic venous pressure and augmenting preload to the systemic ventricle. It is believed to decrease pleural drainage, shorten hospital stay, and reduce complications. However, fenestration may result in systemic desaturation and carries a potential risk of paradoxical thromboembolism. The benefits of routine fenestration remain controversial. This study aimed to evaluate whether adding a fenestration influences early postoperative outcomes. PATIENTS AND METHODS:Between March 2024 and November 2025, 52 patients underwent Fontan completion at our institution. Forty patients met the inclusion criteria and were prospectively divided into two equal groups: non-fenestrated (Group 1, n = 20) and fenestrated (Group 2, n = 20). Twelve patients were excluded due to a primary Fontan procedure, elevated pulmonary vascular resistance, increased transpulmonary gradient, or significant branch pulmonary artery distortion. Demographic and perioperative parameters, including ventilation duration, inotrope duration, chest drain duration and volume, ICU stay, and total hospital stay, were analysed. RESULTS:Baseline demographic and intraoperative variables were comparable between groups. Duration of mechanical ventilation, ICU stay, total chest drainage, and cardiopulmonary bypass time were similar. Chest drain duration was shorter in the fenestrated group (8.1 ± 4.2 vs. 10.2 ± 5.3 days), but this difference was not statistically significant (p = 0.173). Discharge oxygen saturation was significantly lower in the fenestrated group (88.9 ± 5.6% vs. 93.3 ± 3.5%; p = 0.0072). CONCLUSION:In the present cohort, fenestration was not associated with a statistically significant improvement in most early perioperative outcomes. Larger studies are needed to define its role.

Neonatal cardiac surgery, performed within the first 28 d of life, remains amongst the most complex areas of pediatric heart care, particularly in low- and middle-income countries (LMICs). In India, the burden of congenital heart disease (CHD) is substantial, with more than 200,000 affected newborns annually, nearly one-fifth requiring urgent intervention. The most common neonatal pathologies include, transposition of the great arteries, total anomalous pulmonary venous connection, arch anomalies, pulmonary atresia, truncus arteriosus, and single-ventricle variants. This narrative review gathers evidence regarding outcomes of neonatal cardiac surgery from published Indian series, a multicentric survey of 12 centres, and early registry data from Indian Association of Cardiovascular Thoracic Surgeons (IACTS). Published reports demonstrate in-hospital mortality ranging from 4.5

Quality improvement (QI) in congenital heart surgery depends mainly on the availability of reliable, organized, and usable data. While high-income countries benefit from mature national registries and well-established benchmarking systems, low- and middle-income countries (LMICs), which carry the largest global burden of congenital heart disease, continue to face significant challenges in data collection. This narrative review examines the current evolving data landscape for congenital heart surgery in LMICs, explores the relationship between existence of data systems and QI, identifies persistent barriers to data collection, and proposes context-appropriate strategies for sustainable progress. Evidence from published outcome studies, national and regional registry reports, and some major international databases demonstrates encouraging growth in progressive maturation of data systems across LMICs. Importantly, successful models illustrate that even incremental data collection, when focused on high-impact indicators such as mortality, major morbidity, and hospital length of stay, can generate meaningful improvements when linked with local clinical leadership, simple audit mechanisms, and collaborative learning networks. Although comprehensive, high-fidelity data collection may be unrealistic in many resource-constrained environments, the pursuit of perfection should not impede progress. Stepwise, local data strategies, supported by international collaboration, capacity building, and context-sensitive implementation, offer a realistic and transformative pathway toward safer, more equitable congenital cardiac care in LMICs.

Late-presenting transposition of the great arteries (TGAs) with an intact ventricular septum is characterized by left ventricular (LV) regression, making primary arterial switch operation (ASO) unsafe. Ductal stenting offers a less invasive alternative compared to conventional LV retraining using pulmonary artery banding and Blalock-Taussig-Thomas shunt. We analyzed outcomes of ASO following ductal stenting for LV retraining in six consecutive TGA children (median weight 3.3 kg). Median age at stenting and at surgery was 2 months and 2.5 months, respectively. The median interval from stenting to surgery was 33 days (8-238 days). LV mass increased significantly from 31.5 ± 3.8 to 60.6 ± 9.4 g/m2 (P = 0.0021), and LV posterior wall thickness increased from 3.4 ± 0.4 to 4.4 ± 0.3 mm (P = 0.0048). All patients underwent successful ASO with ductal stent removal and no mortality. Ductal stenting appears to be a safe, effective strategy for LV retraining, and enabling late ASO.

Background: India’s thoracic organ transplant program has undergone significant development, positioning the country as a leading player in the South Asian region. Despite development of infrastructural support and surgical skillsets, the transplant numbers are limited compared to the total population due to low donation and geographical challenges for organ transport. West Bengal, a state in Eastern India, initiated its heart transplant program relatively late in 2018, but has since witnessed notable growth. Methods: Data were collected from seven participating centers in West Bengal in an anonymized manner, including hospital records, outpatient follow-up data, and readmissions. Results: The study included 38 heart transplants conducted in West Bengal from May 2018 to October 2023. Most patients were male (73.6%), with idiopathic dilated cardiomyopathy being the most common pathology (52.6%). The inhospital mortality rate was 13.1%, primarily due to low cardiac output syndrome likely due to primary graft dysfunction (PGD). Follow-up data revealed infections to be a major contributor in mortality and morbidity rates. The average number of transplants per year varied, with 2019 seeing the highest number (nine cases). Conclusion: Despite its delayed initiation, West Bengal’s heart transplant program has experienced significant growth, with seven accredited centers now offering services. This development marks a milestone in the region’s health care, providing hope to patients with end-stage heart failure. Challenges such as postoperative PGD and infections persist but are expected to be addressed with continued efforts, gaining of further experience and infrastructure development.

Recent advancements in pediatric cardiac surgery have significantly influenced the management of congenital heart disease (CHD) patients in intensive care units (ICUs). These developments include earlier surgical interventions, increasing complexity of procedures, and the rising prevalence of surgeries in neonates, premature infants, and low-birth-weight babies. Complex repairs, nonsternotomy approaches, such as minimally invasive surgeries and heart failure management with mechanical circulatory support, present unique challenges in postoperative care, including respiratory support, infection prevention, and management of low-cardiac output state (LCOS) and multiorgan dysfunction. In resource-limited settings, delayed presentations and fiscal constraints further complicate ICU management. Innovations such as protocol-based treatment, integration of artificial intelligence, and multidisciplinary collaboration are revolutionizing care strategies. This narrative review highlights the trends in pediatric cardiac surgery, their direct impact on ICU practices, and future directions aimed at improving survival rates, reducing complications, and enhancing the quality of care for CHD patients.

Background:Tetralogy of Fallot (TOF) with absent pulmonary valve (PV) accounts for 3% to 6% of TOF cases. It is physiologically distinct due to tracheobronchial compression caused by dilatation of the pulmonary arteries. We reviewed the surgical outcomes of TOF with absent PV at our center using various strategies. Methods: A retrospective analysis was conducted on all patients diagnosed with TOF with absent PV who underwent surgery from May 2018 to December 2024. Data on demographics, surgical techniques, in-hospital mortality, ventilation duration, intensive care unit (ICU) and hospital stay, and early follow-up outcomes were collected. The surgical technique was individualized to the patient based on the severity of the clinical presentation, age, and radiologic evidence of tracheobronchial compression. Results:Twenty-eight patients were included (median age: 9 months; median weight: 5 kg); six (21.4%) were neonates. Branch pulmonary artery reduction was performed in 75% (21/28) of cases. Surgical approaches included valveless transannular patch (5/28, 17.8%), 0.1 mm polytetrafluoroethylene (PTFE) bicuspid valve with transannular patch (12/28, 42.8%), PTFE bicuspid valve with LeCompte's maneuver (6/28, 21.4%), and valved conduit repair (5/28, 17.8%). One patient (3.6%) experienced in-hospital mortality. Median mechanical ventilation time was 11 h (range: 4-82 h) with neonates requiring prolonged ventilation (>72 h, median 74.5 h). Median ICU stay was 24 h, and median hospital stay was six days. One patient (3.5%) required reintervention during follow-up. Conclusion:Tailoring surgical therapy to each patient's clinical presentation and anatomy yields reasonable outcomes in TOF with absent PV. Early presentation and severe airway compression needs an aggressive surgical strategy. Neonates demonstrated a need for prolonged ventilatory support.

Extracorporeal cardiopulmonary resuscitation (E-CPR) employs rapid venoarterial extracorporeal membrane oxygenation (VA-ECMO) during cardiopulmonary resuscitation (CPR) to restore perfusion. This case involves a 3.5-month-old girl with anomalous left coronary artery from the pulmonary artery (ALCAPA), severe left ventricular dysfunction, and moderate mitral regurgitation. After surgical correction, she experienced cardiac arrest post-extubation. Conventional CPR failed, and E-CPR was initiated after 70 min. Hemodynamic stability and end-organ function were maintained during 72 h and 45 min on ECMO. Post-decannulation, she showed significant recovery, normal neurological status, and improved cardiac function. At 6 months post discharge, her left ventricular function was normalized.

A rare but historically significant variant of transposition of the great arteries (TGA) was first identified in 1971. A posterior and rightward-positioned aorta, an underdeveloped subaortic conus, and a well-developed subpulmonary conus characterize this entity. The relationship of the great vessels mimics that of normally related great arteries, with the pulmonary artery positioned anteriorly and the aorta located posteriorly. This report describes a patient with such a variant of TGA, associated with a ventricular septal defect, who underwent a successful arterial switch operation. Despite the unusual positioning of the great arteries, coronary artery transfer and pulmonary artery anastomosis were effectively performed. Although published literature advises against performing LeCompte’s maneuver in such anatomy, we performed the maneuver in this case with anterior and posterior looping coronaries to protect the proximal coronary arteries from excessive stretch.

Over the past decade, remarkable advancements in congenital cardiac surgery have transformed patient outcomes through innovations in imaging, surgical techniques, patient care, and technology. Enhanced diagnostic modalities, including 3D modeling and virtual reality (VR), have facilitated precise preoperative planning, particularly for complex heart defects. The incorporation of artificial intelligence (AI) and big data analytics has enabled predictive modeling, improving surgical decision-making and outcomes. Significant progress has also occurred in fetal interventions, minimally invasive procedures, and hybrid techniques, reducing surgical risks and accelerating recovery. Advancements in surgical strategies, such as arterial switch operations, ventricular septal defect (VSD) closures, and Fontan procedures, have markedly improved survival and reduced complications. Concurrently, pediatric heart transplantation and mechanical circulatory support (MCS) have seen substantial improvements, including the adoption of ventricular assist devices (VADs) tailored for children, better immunosuppression protocols, and refined postoperative management. Looking ahead, the integration of augmented reality (AR), real-time physiological tracking systems, and minimally invasive interventions holds promise for even greater enhancements in patient care, precision, and personalized treatment, significantly improving long-term survival and quality of life for children with congenital heart defects.

The readiness of essential equipment like airway trolleys is critical in pediatric cardiac intensive care units (PCICUs). Kaizen, a Lean management principle, has been increasingly applied in healthcare to enhance efficiency and patient care. This study investigates the transformative impact of Kaizen principles on airway trolley preparation in a 10-bedded PCICU. A 12-month prospective observational study was conducted between January 1, 2024 and December 31, 2024. Kaizen was introduced in the system on July 1, 2024. Pre-and post-Kaizen data were compared to evaluate its impact on response times, preparation efficiency, error reduction, resource utilization, and nursing satisfaction. We demonstrated remarkable reduction in response times during pediatric cardiac emergencies, from 4.82 to 2.14 min, post-Kaizen (p < .001). Time spent on airway trolley preparation decreased significantly from 12.5 to 7.3 min (p < .001). The frequency of errors in preparation decreased significantly from 4.2 to 1.1 errors per month. Waste reduction was achieved through streamlined processes, with nurses reporting a 30% reduction in preparation time. Nursing staff expressed heightened confidence and preparedness during high-stress situations. The application of Kaizen principles significantly optimized airway trolley preparation processes, highlighting their potential for broader healthcare applications.

Extracorporeal membrane oxygenation (ECMO) has revolutionized the management of severe cardiopulmonary failure in neonates, children, and adults. Initially developed as an extension of cardiopulmonary bypass, ECMO has evolved with advancements in oxygenators, miniaturized circuits, and dual-lumen venous cannulas, improving survival rates. This article reviews the history, indications, circuit components, management strategies, and outcomes of pediatric ECMO. The Extracorporeal Life Support Organization registry data highlight a steady increase in pediatric ECMO use, with survival rates improving to 50%–72% based on etiology. Veno-arterial ECMO is commonly used for cardiac failure and extracorporeal cardiopulmonary resuscitation (E-CPR), whereas veno-venous ECMO is preferred for isolated respiratory failure. Despite its benefits, ECMO carries risks such as bleeding, thrombosis, infection, and neurological sequelae, necessitating meticulous anticoagulation management. Recent advances in anticoagulation strategies, left heart decompression techniques, and sedation protocols have enhanced patient outcomes. The growing role of E-CPR in pediatric cardiac arrests underscores ECMOs life-saving potential. However, long-term neurodevelopmental outcomes remain an area of ongoing research. This review provides a comprehensive perspective on ECMO’s current applications, challenges, and future directions in pediatric critical care.

IntroductionSelective antegrade cerebral perfusion (SACP), combined with continuous cardiopulmonary bypass (CPB), has become a widely accepted alternative to deep hypothermic circulatory arrest (DHCA). This technique ensures a continuous supply of oxygenated blood to the brain during aortic arch surgery, minimizing the risk of ischemic injury and improving neurological outcomes.MethodsRetrospective data of all 36 patients undergoing aortic arch repair with selective antegrade cerebral perfusion between September 2020 to June 2024 was collected from hospital medical records. In addition to demographic details, intraoperative details, cardiopulmonary bypass (CPB) time, aortic cross clamp (ACC) time, SACP time, additional procedures if any were recorded. In-hospital surgical outcomes including mortality and morbidity data were analysed.ResultsBetween 2002 and 2024, 36 patients underwent aortic arch repair using selective antegrade cerebral perfusion. Median age was 1 month. The in-hospital mortality was 5.5%. (2/36). Stroke occurred in 5.5% (2/36), seizures in 11.1%, (4/36) and acute kidney injury in 16.6%. (6/36).Overall outcomes were favourable, with most neurological complications resolving fully and with no residual deficits.ConclusionSACP is a safe neuroprotective strategy for aortic arch surgery in congenital heart disease. Optimal flow rates and neuromonitoring minimize neurological complications. Larger studies are required to definitively establish SACP's superiority over DHCA in these complex procedures.

We report a unique intraoperative finding of an additional double left atrial appendage (LAA) during an arterial switch operation with ventricular septal defect closure in a 4-month-old girl. Immediately after the procedure, a prolapsing mass within the left atrium (LA) on the transesophageal echocardiogram raised concerns of a possible thrombus. The LAA was clearly visible with a pressure monitoring line which was put intraoperatively. To investigate further, cardiopulmonary bypass was resumed, and the heart was arrested and explored. There was an appendage-like structure, separate from the one that had the pressure monitoring line, which was inverted inside. It was pulled out from outside clearly establishing a double LAA. This report illustrates an example of a diagnostic dilemma caused by a double atrial appendage which was invaginated into LA masquerading as a mass or thrombus.

The Fontan procedure is crucial for managing univentricular heart conditions but can lead to prolonged pleural effusions, affecting recovery and hospital stays. This study evaluated the effectiveness of vasopressin in reducing pleural effusion and improving recovery outcomes following Fontan procedure. This comparative observational study reviewed patient records from October 2021 to September 2023. Two cohorts were included of 23 patients each: patients who received postoperative vasopressin from October 2022 to September 2023 (VP group) and a historical control group from the previous year (NVP group), excluding those who underwent fenestrated Fontan. Vasopressin was administered postoperatively to the VP group to mitigate pleural effusion. Primary outcomes were the volume and duration of chest tube drainage. Secondary outcomes included hospital and intensive care unit (ICU) stay durations and fluid balance metrics. There were no significant differences in the primary or secondary outcomes between the vasopressin group and the control group. The median total drain outputs in the VP and NVP groups were 69.4 ml/kg and 53.9 ml/kg, respectively (p = 0.96). The median duration of chest tube stay was 5.5 days for the VP group and 6 days for the NVP group (p = 0.74). Hospital stay duration (p = 0.74) and ICU stay duration (p = 0.82) showed no significant difference. Vasopressin does not significantly impact chest tube drainage volume or duration, nor does it reduce hospital stays in Fontan patients, suggesting a limited role in managing postoperative pleural effusions. Further research is needed to explore its benefits for specific patient subgroups and acute hemodynamic instabilities postoperatively.

The surgical management of d-transposition of the great arteries (dTGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction (LVOTO) poses intricate challenges, demanding tailored surgical interventions. This case report elucidates a patient involving a 9-year-old child with dTGA, VSD, and LVOTO with a dysplastic pulmonary valve with adequate annulus who underwent neo-aortic valve replacement with a mechanical prosthesis, arterial switch, and VSD closure. The recovery of the patient was uneventful and follow-up echocardiogram and clinical evaluation at 18 months have remained satisfactory. We describe the clinical details of this unusual approach for management of dTGA, VSD, and LVOTO.

In low- and middle-income countries (LMICs), the delayed detection and presentation of congenital heart defects (CHDs) present significant hurdles, including challenges in assessing operability, unique surgical complexities during operations, and difficulties in post-operative care. Factors contributing to late detection include delayed diagnosis and referrals, limited healthcare resources, geographic barriers to specialized care, and lack of awareness leading to inappropriate medical advice. This narrative review discusses these challenges and how late presentation affects outcomes of CHDs in LMICs. A thorough assessment of operability tailored to each specific CHD, effective intraoperative strategies, and comprehensive post-operative care are crucial for achieving favourable outcomes. Current literature indicates that surgery for late-detected CHDs in LMICs can yield satisfactory results. Proper selection and management of this patient subgroup can mitigate the impact of late detection on outcomes.

Background and Aims:The pediatric cardiac intensive care unit (PCICU) frequently uses noninvasive ventilation (NIV). There are several reasons for its use, including prophylactic use right after the patient has been extubated. It is also used when patients are experiencing acute respiratory failure due to either cardiac or noncardiac reasons but are still able to maintain their airways. The objective of this study was to understand the spectrum of use of NIV following congenital cardiac surgery and analyze the outcome. Methods and Results:A retrospective observational study was conducted in a 14-bed PCICU, reviewing data from August 2019 to August 2022. Among 1750 congenital cardiac surgeries, 523 patients (29.9%) received NIV. The median age of the population was 2.5 months. Factors such as higher Risk-Adjusted Classification for Congenital Heart Surgery-1 category, longer intraoperative cardiopulmonary bypass time, and aortic cross-clamp time were associated with increased NIV use. Preoperative ventilator needs, infections, genetic syndromes, diaphragmatic paralysis, high vasoactive inotrope score (VIS) in the first 24 h, neonatal age, and weight <5 kg were independently associated with increased NIV need. The NIV group had a longer intensive care unit (ICU) stay compared to non-NIV patients. The success rate of NIV was 84%, with 440 successful cases and 83 failures. The mortality rate in the success and failure groups was not significantly different (5.27% vs. 6.0%). Conclusions:NIV is widely used in PCICU, but it is associated with longer ICU stays. It proves beneficial after congenital cardiac surgery, especially for patients with specific risk factors. However, NIV may not directly impact mortality rates, suggesting that other factors contribute to patient survival.