Prognostic Nutritional Index (PNI) As a Preoperative Screening Tool in Predicting Clinical Outcomes of Postoperative Pediatric Cardiac Surgery Patients
Published: January 29, 2025.
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Satbir Kaur; Himani Pandya; Gargee Bhatt; Deepal Prajapati; Dhruvini Patel; Aparna Sirandas; Jigisha Pujara
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
ObjectiveThe authors sought to evaluate the role of nutritional indices such as Onodera’s prognostic nutrition index (PNI), World Health Organization (WHO)-based anthropometric measurements such as weight for age (w/a), height for age, weight for height, and perioperative serum albumin levels in the determination of postoperative clinical outcomes in pediatric patients who undergo surgery for congenital cardiac defects and surgical complexity (risk-adjusted congenital heart surgery score) and its correlation with postoperative course.Material and MethodsIn this prospective observational study, 108 post-pediatric cardiac surgery patients under the age of 18 months were enrolled between January 2023 and August 2023. Through receiver operating characteristic curve analysis we have found the cutoff value for PNI is ≤66.5 and >66.5. The above mentioned parameters were analyzed for postoperative clinical outcomes such as length of intensive care unit (ICU) stay, length of hospital stay, and duration of mechanical ventilation.ResultsSignificant negative correlation was found between length of ICU stay and hospital stay with lower PNI (P = .019 and <.001, respectively.). Analysis of low versus high PNI groups was suggestive of a remarkable increase in mechanical ventilation time (P = .03), length of ICU stay (0.01), and hospital stay (P ≤ .001) in the low PNI group. Lower WHO-based w/a Z score was found to be significantly associated with low PNI (<66.5), after adjusting for preoperative albumin, postoperative albumin drop, and C-reactive protein (odds ratio = 1.411 per unit 0.28 increment in W/azs, P = .004).ConclusionPreoperative Onoderas PNI is an effective and efficient tool for predicting postoperative clinical morbidity in pediatric patients undergoing congenital heart surgery.
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Single-Stage Midline Unifocalization Is Associated With Excellent Outcomes in Infants of All Ages
Published: January 29, 2025.
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Elisabeth Martin; Michael Ma; Yulin Zhang; Ritu Asija; Jennifer Shek; Chandra Ramamoorthy; Frank L. Hanley; Doff B. McElhinney
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
ObjectiveTetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries are a complex congenital heart defect. For years, our program has recommended early single-stage midline unifocalization at three to six months of age. However, many patients are referred beyond six months. Thus, we sought to evaluate surgical outcomes according to age at repair.MethodsWe performed a retrospective review of patients who underwent unifocalization from age 3 to 12 months. These patients had not undergone prior surgery at our institution or elsewhere and were also not protocoled into early surgery. Patients were divided in the following groups: 3.0 to 4.9 (n = 61), 5.0 to 5.9 (n = 56), 6.0 to 7.9 (n = 56), and 8.0 to 11.9 (n = 47) months. Competing risk regression analyses were performed.ResultsWe included 220 patients from 2001 to 2020. Baseline characteristics were not significantly different among the four groups. Overall, single-stage complete repair with bilateral unifocalization, ventricular septal defect closure, atrial septal defect closure, and right ventricular–pulmonary artery (PA) conduit placement was achieved at the index operation in 174 (79%) patients and did not differ across age groups. Early mortality was 4% (n = 9). At one year, 91% (200/220) of the entire cohort was fully septated. Comparing with group 1, group 4 was significantly less likely to undergo any PA reinterventions [hazard ratio (HR) 0.44, 95% CI 0.21-0.92, P = .028] or surgical PA reinterventions [HR 0.12, 95% CI 0.02-0.95, P = .044] following complete repair.ConclusionsGiven the excellent outcomes across all ages, surgical timing for single-stage unifocalization should be dictated by clinical and anatomic details, with potential advantage in select clinically appropriate older infants who appear to be at a lower probability of PA reinterventions following full septation.
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Short- and Long-Term Outcomes of Patients Who Underwent the Arterial Switch Operation at Siriraj Hospital—Thailand’s Largest National Tertiary Referral Center
Published: January 28, 2025.
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Juthamas Hannarong; Teerapong Tocharoenchok; Somchai Sriyoschati; Punnarerk Thongcharoen; Kriangkrai Tantiwongkosri; Ekarat Nitiyarom; Thaworn Subtaweesin
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
ObjectiveThe aim of this study was to assess the short- and long-term outcomes of patients who underwent the arterial switch operation (ASO) at Siriraj Hospital in Thailand, and to identify postoperative complications and factors that significantly affect patient survival.Materials and MethodsWe retrospectively studied all patients with dextro-transposition of the great arteries and anatomic variants who underwent the ASO from January 1995 to December 2020. Twenty-year overall survival and 15-year freedom from reoperation/reintervention were estimated using the Kaplan-Meier method. Univariate and multivariate Cox regression analyses were used to identify factors independently associated with 30-day mortality.ResultsOf the 171 patients included, there were 116 males (67.84%). The median (minimum, maximum) age at operation was 33.5 (3-4,499) days, and the median (minimum, maximum) bodyweight was 3.4 (2.2-44.9) kg. Most patients (115/171, 67.25%) had an intact ventricular septum, whereas 48/171 (28.07%), had ventricular septal defects. The typical coronary anatomic pattern (1LCx-2R) was found in 120 patients (70.1%). Early mortality was 8.7% ± 4.4%, and there was no late death up to 20 years postoperatively with a median follow-up of five years. Fifteen-year freedom from reoperation or reintervention was 85.7%. Atypical coronary pattern was an independent factor for survival with an adjusted hazard ratio of 5.1 (95% confidence interval: 1.22-21.4; P = .026).ConclusionsThe results of this study revealed excellent short- and long-term outcomes of the ASO at our center. Atypical coronary anatomy was found to be the only factor that independently predicted 30-day mortality.
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Reader Comment on: Surgical Closure of Multiple Muscular Ventricular Septal Defects in Children Using 3D-Printed Models
Published: January 28, 2025.
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Hinpetch Daungsupawong; Viroj Wiwanitkit
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
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Total Anomalous Pulmonary Venous Return, Marfan Syndrome, and Severe Combined Immunodeficiency: A Lethal Combination?
Published: January 28, 2025.
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Spencer J. Hogue; Muhammed Faateh; Awais Ashfaq
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
The authors present an exceptionally rare case of a newborn in whom total anomalous pulmonary venous return (TAPVR) had been diagnosed prenatally and in whom genetic testing after neonatal cardiac repair confirmed a missense variant of the FBN1 gene consistent with Marfan syndrome as well as a PRKDC gene mutation associated with severe combined immunodeficiency. To the authors’ knowledge, this is the first reported case with this unique combination. Neonatal TAPVR repair was undertaken with a good postoperative outcome and survival until the last follow-up.
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Outcomes in Late Presenting Obstructed Infracardiac Total Anomalous Pulmonary Venous Connection—Single Centre Experience
Published: January 27, 2025.
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Kartik Patel; Deepti Kakkar; Himani Pandya; Shyam S Kothari; Megha Sheth; Herin Patel; Trushar Gajjar; Amit Mishra
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
BackgroundSurvival beyond one month of age is rare in children born with obstructed infracardiac total anomalous pulmonary venous connection (TAPVC). There are limited data available on surgical outcomes of the same subset. We conducted this retrospective study to identify risk factors associated with surgical outcomes in this patient population.MethodWe present a retrospective study of patients presenting at our institute with obstructed infracardiac TAPVC surviving beyond one month of age during the period from January 2015 to August 2019 (n = 14). Data reviewed included demographic parameters, preoperative laboratory results, echocardiogram images, and computed tomography if performed. Intraoperative as well as postoperative data including type of surgery, use of deep hypothermic arrest, mortality, and morbidity were noted. Follow-up clinical data were also collected from institutional record or telephonically. We have also collected echocardiographic data on follow up.ResultsThe mean age of our study cohort was 2.14 ± 0.68 months. Out of 14 total patients 3 patients eventually died. Risk factors that were significantly associated with mortality were low weight (odds ratio [OR] = 7.8, P = .005), preoperative bilirubin level (OR = 7.4, P = .007), use of deep hypothermic circulatory arrest (OR = 4.6, P = .031), tricuspid regurgitation grade (OR = 3.8, P = .050), and pulmonary artery hypertension (OR = 10.5, P = .001). Mean follow up for the study cohort was 2189 ± 538 days. One patient has mild flow acceleration across the anastomosis with a gradient of 5/3 mm Hg without any symptoms. The remainder of the patients are alive with excellent clinical as well as echocardiographic outcome.ConclusionThe presentation of infants with obstructed infracardiac TAPVC beyond one month of age is very rare. However, timely intervention can provide acceptable early and excellent late outcomes in this rare patient cohort.
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Legacy and Leadership: Celebrating the Contributions of Six Visionary Presidents of the World Society for Pediatric and Congenital Heart Surgery
Published: January 23, 2025.
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James Kirklin
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Leadership in pediatric and congenital heart surgery requires a distinctive blend of clinical mastery, scientific innovation, and an unwavering commitment to education and mentorship. The individuals who rise to the top of this demanding field exhibit not only surgical prowess but also the ability to foster progress through impactful research, publications, and training the next generation of surgeons. These qualities are exemplified by the six past presidents of the World Society for Pediatric and Congenital Heart Surgery (WSPCHS), whose leadership has been instrumental in advancing both the Society and the broader field of pediatric cardiac care. This article pays tribute to these visionary leaders, highlighting their contributions to the Society and their global impact on congenital heart surgery. Their leadership has profoundly influenced the direction of the WSPCHS, contributing to its success and shaping the future of the specialty
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Surgical Repair of Double Outlet Right Ventricle Infants Guided by Three Dimensional-Computed Tomography Cardiac Modeling and Printing
Published: January 22, 2025.
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Srujan Ganta; Justin R. Ryan; Michael J. Lewis; John J. Nigro
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
BackgroundDouble outlet right ventricle (DORV) is a challenging congenital cardiac lesion to surgically master. We utilize computed tomography–guided-three-dimensional (3D) modeling/printing and novel in-house software to delineate anatomical relationships providing operative insight into the surgical approach. Our intent is to highlight this and showcase our technology.MethodsWe have created a repository of 3D heart reconstructions allowing for review of DORV patients. 3D intraoperative software anatomical manipulation and physical 3D prints were used to gain insight into DORV anatomy with the assistance of an on-site 3D Lab. The software used (Arc 3D Model Viewer) was designed in-house by our 3D Lab, tested and refined through ongoing use by our cardiothoracic surgery team. It allows for the subtraction and addition of anatomical structures and rotation in all axes. Clinicians can pan into the heart and determine specific anatomical boundaries and relationships.ResultsFrom 2010 to 2023, our program operated on 71 patients with DORV and our 3D lab has reconstructed 29 3D-hearts. Reconstructions were analyzed using Arc 3D Model Viewer. 3D reconstructions were viewed in our care conferences and intraoperatively allowing for discussion and determination of the optimal operative approach. Overall survival for DORV patients was 96% (68/71) with two mortalities in patients who did not receive 3D reconstructions.Conclusion3D reconstruction has allowed decision-making to be moved out of the operating room into the preoperative planning phase. 3D reconstruction is now standard for all DORV patients in our surgical service. We hope to demonstrate this technology with our newly developed Arc 3D Model Viewer and summarize our clinical results.
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A Juxta-Arterial and Perimembranous Confluent Ventricular Septal Defect in Transposition of the Great Arteries
Published: January 17, 2025.
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Shinya Yokoyama; Ryohei Fukuba; Rei Tonomura; Kazuhiro Mitani; Hideki Uemura
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Transposition of the great arteries (TGA) with a rare form of ventricular septal defect (VSD) needs careful attention when choosing the optimal strategy for repair. A neonate with TGA and an atypical VSD, which extended from the perimembranous to the juxta-arterial regions, underwent the arterial switch procedure. The large VSD was successfully closed via a trans-tricuspid combined with a trans-aortic valve approach concomitantly with the arterial switch maneuver. Surgeons need to be aware aware that the types of VSD are diverse in TGA.
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Use of Cervical Approach for Insertion of the Impella 5.5 Ventricular Assist Device (Abiomed) in Pediatric Patients: Cohort Study
Published: January 17, 2025.
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Mohammed Absi; Andrew J. Gienapp; Cihangir Buyukgoz; Shyam Sathanandam; Umar Boston
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
BackgroundImpella 5.5 ventricular assist device (VAD) insertion is typically done via the axillary artery or directly through the aorta; however, an axillary artery must be ≥6 mm in diameter, which excludes many pediatric patients who do not meet this criterion. The innominate artery is a larger vessel that can better accommodate the Impella VAD in pediatric patients. Outcomes of this technique were compared with other pediatric patients undergoing the standard axillary artery cannulation.MethodsThe Heart Center at Le Bonheur Children’s Hospital recently adopted the trans-innominate cervical approach for insertion of the Impella 5.5 VAD. We performed a retrospective cohort study comparing these cases to data collected from cases using an axillary approach found in the Advanced Cardiac Therapies Improving Outcomes Network database. Comparisons between preoperative characteristics, postoperative course, adverse events, anticoagulation, and clinical outcomes for these two groups were analyzed.ResultsThirty-nine patients were implanted via the axillary approach; seven patients were implanted via the transinnominate cervical approach at our institution. Patients inserted via the trans-innominate approach had fewer days on the device (median, 8 [range, 3-24]), postimplant intensive care unit days (16 [3-24]), and hospital length of stay (14 [3-28]) compared with axillary insertion patients (12.5 [6.75-29.2], 58.5 [12.5-43.5], and 32 [19.7-83.5], respectively). Trans-innominate patients had fewer adverse events than axillary patients.ConclusionsTrans-innominate cervical approach for insertion of the Impella 5.5 VAD is safe with low rates of adverse events in pediatric patients. As miniaturization of VAD therapy continues to evolve, this technique may provide greater application of these devices for even smaller children.
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Heart Transplantation Outcomes for Fontan Patients When Using Contemporary Strategies: Heart-Liver Transplantation and Ventricular Assist Device Therapy
Published: January 16, 2025.
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Spencer J. Hogue; Jason W. Greenberg; Amir Mehdizadeh-Shrifi; Chet R. Villa; Clifford Chin; Angela Lorts; Alexander R. Opotowsky; David G. Lehenbauer; David L. S. Morales
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
BackgroundWith an increasing number of Fontan patients surviving into adulthood, the burden of end-stage heart failure is increasing. Prior studies have reported suboptimal heart transplantation (HTx) outcomes. Therefore, the authors describe their institutional experience of HTx in patients with Fontan circulation failure, including en-bloc heart-liver transplantation (HLTx) and pretransplant systemic ventricular assist device (SVAD) therapies.MethodsAll consecutive Fontan-palliated patients undergoing HTx or HLTx between 2013 and 2022 at the authors’ institution were included. Pretransplant characteristics and posttransplant outcomes were recorded.ResultsTwenty-six transplant recipients, including 5 (19%) HLTx recipients, were identified. The majority (n = 16, 62%) of patients had a cardiac diagnosis of hypoplastic left heart syndrome. Seven (26%) patients were bridged to transplant on an SVAD; the median duration of support was 104 [IQR 39-543] days. Transplantation occurred at a median of 10.6 [IQR 6.8-15.6] years post-Fontan. Fourteen (54%) patients had ≥1 in-hospital complication and 4 (15%) patients required an in-hospital reoperation. Postdischarge reinterventions included tricuspid valve repair (n = 1, 4%) and retransplantation (n = 1, 4%). Two (8%) in-hospital mortalities [22 days (post-HLTx), 2.9 months (post-HTx] and 4 (15%) postdischarge mortalities [5.4 months, 3.1 years, 5.7 years, 7.1 years (all post-HTx)] occurred. Overall survival was excellent, with in-hospital, one-year, and five-year actuarial survival being 92%, 89%, and 80%, respectively.ConclusionsThe current series demonstrates that excellent posttransplant outcomes are achievable for patients with Fontan circulation failure using a comprehensive approach including SVAD and HLTx therapies. Pretransplant optimization, sometimes including SVAD implantation, and meticulous operative planning are imperative strategies for successful patient outcomes.
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Lightning Can Strike Twice: Management of an Infant With Anomalous Left Coronary Artery from the Pulmonary Artery Complicated by Coccidioidomycosis Infection
Published: January 13, 2025.
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Varshini Methuku; Katie A. Kowalek; Nathan B. Price; Alison Bliss; Kenneth A. Fox; Michael D. Seckeler
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Cardiac disease in young children can be unrecognized until symptoms are unmasked by a precipitating event, such as an infection. We present a case of anomalous left coronary artery from the pulmonary artery causing clinically significant disease in a four-month-old male with concomitant mitral regurgitation and pulmonary coccidioidomycosis who required modification of his surgical management due to the infection. This case highlights how timely diagnosis and perioperative management and recovery can be affected by concurrent infections in patients with congenital heart disease.
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Pulmonary Atresia with Ventricular Septal Defect without Major Aorto-Pulmonary Collateral Arteries: Echocardiography and the Role of Computed Tomography and Magnetic Resonance Imaging
Published: January 13, 2025.
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Sarah Chambers Gurson
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Pulmonary atresia with ventricular septal defect (PA-VSD) is usually diagnosed by transthoracic or fetal echocardiography, with the prenatal diagnosis being feasible and accurate if fetal cardiology services are available. The limitations of transthoracic echocardiography (TTE) in the evaluation of PA-VSD include the complete evaluation of the pulmonary arteries and patent ductus arteriosus, quantitative evaluation of the right ventricle size and function, and delineation of associated cardiac anomalies such as coronary artery anomalies, anomalies of systemic or pulmonary venous return, and complex arch anomalies. Echocardiography also has limitations in evaluating hemodynamics such as flow volumes, shunts, and regurgitant fraction. Despite these limitations, TTE remains the most widely available and the most cost-effective cardiac imaging modality for patients with PA-VSD and its accuracy in evaluating the sources of pulmonary blood flow and in selecting patients for systemic-to-pulmonary artery shunt palliation is well established. Cardiac computed tomography (CT) can answer many of the questions not answered by TTE, including demonstrating the PA anatomy and defining coronary artery, systemic and pulmonary venous, and aortic arch anatomies. The short acquisition time allows for the study to be performed without sedation/anesthesia in most patients. Cardiac CT is also useful in defining postoperative anatomy when there are non-magnetic resonance imaging (MRI) compatible devices or even MRI-compatible devices that cause a significant MRI artifact. Cardiac MRI/magnetic resonance angiography has emerged as an ideal modality to evaluate patients with PA-VSD as it allows for anatomic, functional, and hemodynamic assessment without exposure to ionizing radiation or iodinated contrast material. Both cardiac CT and cardiac MRI can be used to generate 3D imaging of the heart.
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Staged Approach: The Role of Delayed Repair Following Complete Unifocalization of Major Aortopulmonary Collateral Arteries with Ventricular Septal Defect and Pulmonary Atresia
Published: January 13, 2025.
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Mimi X. Deng; Yasmin Zahiri; Osami Honjo; David J. Barron
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
The presentation of pulmonary vasculature in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) is highly variable—as is the number, size and position of the MAPCAs and their relationship with the native pulmonary artery system. The priority in the management of this disease should be attaining timely and complete unifocalization, as opposed to single-stage full repair in every case. The merit of early unifocalization is that it secures the pulmonary vascular bed by (a) avoiding loss of lung segments from progressive stenosis/atresia of MAPCA origins, (b) preventing lung injury from high pressure/flow in areas fed by large, unobstructed MAPCAs, and (c) restoring central continuity of the pulmonary vasculature. Furthermore, there are a small but important group of patients with poorly developed vessels (about 10%-15% of the population) and/or diminutive native pulmonary artery vasculature that require initial shunt procedures to promote rehabilitation and growth of vessels before unifocalization can be attempted. During unifocalization, patients not suitable for single stage repair can be identified by intraoperative flow studies and can be successfully managed with staged strategies that provide time for growth and reinterventions on the pulmonary vasculature. Over 85% of patients can achieve unifocalization. Deferring closure of the VSD to a subsequent procedure is safe and these cases have similar survival to primary repair. Some patients (15%-20%) may never achieve VSD closure with this strategy but can still maintain a good quality of life with a restrictive right ventricular to pulmonary artery conduit and open VSD.
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Milky White Supernatant in the Cardiopulmonary Bypass Circuit in Severe Hypertriglyceridemia During Pediatric Cardiac Surgery: A Case Report
Published: January 03, 2025.
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Reddy Sandhya Poornima; Unnikrishnan KP; Aspari Mahammad Azeez
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
We present a case of severe hypertriglyceridemia (HTG) in a 21-month old female undergoing cardiac surgery for a ventricular septal defect and subaortic membrane excision. During the operation, a milky white supernatant was observed in the cardiopulmonary bypass circuit, prompting immediate lipid profile testing that revealed elevated triglycerides. The management involved maintaining anticoagulation with heparin dose–response testing and ensuring oxygenator function by measuring blood gas analysis, lactate, and arterial line pressure. The child recovered without complications. This case highlights the critical need for prompt identification and management of HTG in pediatric cardiac surgery to mitigate risks and ensure successful outcomes.
