Pulmonary Atresia and Ventricular Septal Defect: Definitions, Nomenclature, and Classification
Published: February 06, 2025.
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Contributors
Christo I. Tchervenkov; Richard Tang; Giles J. Peek; Mark S. Bleiweis; Jeffrey P. Jacobs
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
This manuscript will provide information about pulmonary atresia + ventricular septal defect, including definitions, nomenclature, and classification, based on the 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11). The 2021 IPCCC and ICD-11 provide the following definition for Tetralogy of Fallot with pulmonary atresia: Tetralogy of Fallot with pulmonary atresia is defined as “A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which there is no direct communication between the right ventricle and the pulmonary arterial tree.” The 2021 IPCCC and ICD-11 provide the following definition for Tetralogy of Fallot with pulmonary atresia and systemic-to-pulmonary collateral arteries: Tetralogy of Fallot with pulmonary atresia and systemic-to-pulmonary collateral arteries is defined as “A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which there is no direct communication between the right ventricle and the pulmonary arterial tree and there are collateral blood vessels between the systemic and pulmonary arteries.” “Tetralogy of Fallot + pulmonary atresia” is a specific type of “pulmonary atresia + ventricular septal defect” where the intracardiac malformation is more accurately defined. The presence or absence of major aortopulmonary collateral arteries (MAPCA[s]) does not change these definitions. Therefore, “tetralogy of Fallot + pulmonary atresia + MAPCA(s)” is a specific type of “pulmonary atresia + ventricular septal defect + MAPCA(s)” where the intracardiac malformation is more accurately defined. In the universe of patients with pulmonary atresia + ventricular septal defect, the anatomy and morphology of the pulmonary circulation to a large extent determines the surgical approach and overall outcome, with the intracardiac anatomy playing a secondary role. Based on the characterization of the pulmonary circulation, patients with pulmonary atresia + ventricular septal defect can be classified into three groups: In type A, pulmonary blood flow is provided by native pulmonary arteries (NPAs). In type B, pulmonary blood flow is provided by both NPA and MAPCA(s). In type C, pulmonary blood flow is provided only by MAPCA(s).
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Evaluating Diaphragmatic Paralysis After Pediatric Cardiac Surgery and the Role of Plication in Outcome Improvement: Insights From a Single-Center Experience
Published: February 06, 2025.
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Mohamed M. Elgayar; Mahmoud A. Negm; Emad Gamaleldin Nasr; Hedaia Abdullah; Sayed Hamed; Hatem Honsy
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
IntroductionDiaphragmatic paralysis (DP) is a significant complication following cardiac surgery, particularly impacting neonates and infants. This retrospective study aims to evaluate the incidence of DP, identify risk factors, and assess the impact of diaphragmatic plication as a definitive management strategy.MethodsWe analyzed the clinical records of pediatric patients who underwent cardiac surgery at our center from 2016 to 2023. Cases of DP were identified, potential risk factors examined, and the effectiveness of plication assessed. Patients were categorized into early and late groups based on the timing of DP diagnosis relative to surgery.ResultsOut of 2,331 pediatric cardiac surgery patients (median age 7.2 months), DP was identified in 60 patients (2.6%), with a higher incidence of left-sided paralysis in 37 patients (61.7%). Diaphragmatic paralysis was more frequently associated with specific procedures, such as bidirectional Glenn, arterial switch operations, and Blalock-Taussig-Thomas shunt. Patients with DP had significantly longer intensive care unit (ICU) stays (21 vs 4 days, P < .001) and total hospital stays (38 vs 11 days, P < .001). Following plication, median mechanical ventilation (MV) hours significantly decreased (73-13 h, P < .001), and ICU stays were reduced (15-3 days, P < .001). Early plication was associated with shorter MV time and hospital stays.ConclusionDiaphragmatic paralysis is a notable complication after cardiac surgery. Diaphragmatic plication proves to be an effective intervention, reducing MV duration and hospital stays. These findings underscore the importance of early recognition and intervention for improved postoperative outcomes.
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Reader Comment on: A Low-Cost Workflow to Generate Virtual and Physical Three-Dimensional Models of Cardiac Structures
Published: February 06, 2025.
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Contributors
Amnuay Kleebayoon; Viroj Wiwanitkit
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
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Three-Dimensional Printed Cardiac Models Demonstrating Extensive Cardiac Calcification Assist in Preprocedural Planning
Published: February 06, 2025.
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Ross Foley; Kevin Walsh; Eoin O’Cearbhaill; Colin J. McMahon
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Cardiac calcification is common in adults with congenital heart disease, typically affecting surgical sites and prosthetic material. Understanding the precise location and degree of calcification is important during the decision-making process prior to possible intervention but can be difficult to determine in different imaging modalities. Advances in three-dimensional (3D) modeling and printing have made it possible to distinguish different tissue types and accurately display them in different colors on printed models. We present two adult congenital heart disease patients where advanced 3D printing was used to demonstrate cardiac calcification and aided the preprocedural planning process.
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Neoaortic Dilation and Polyvalvar Dysfunction: The Coexistence of Marfan Syndrome with Hypoplastic Left Heart Syndrome
Published: February 06, 2025.
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Sruti Rao; Rukmini Komarlu; Hani K. Najm; Brittany Hansen; Rene E. Rodriguez; Kenneth G. Zahka
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
We present a unique case of progressive neoaortic root and neoascending aortic dilatation, with poly-valvar regurgitation in a patient with Marfan syndrome and hypoplastic left heart syndrome palliated to Fontan procedure. This progressive dilation and valvar dysfunction necessitated a modified Bentall procedure and tricuspid valve repair. Our case highlights the impact of both a primary and secondary aortopathy complicating the Fontan procedure and the need for mechanical aortic valve replacement.
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Long-Term Coronary Artery Complications Following the Arterial Switch Operation for Transposition of the Great Arteries—A Scoping Review
Published: February 06, 2025.
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Rachel S. Lim; Jeffrey Lefkovits; Samuel Menahem
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Coronary artery complications following the arterial switch operation (ASO) for transposition of the great arteries have become increasingly relevant as those affected are exposed to the comorbidities of later years. A scoping review was undertaken to explore the incidence, clinical features, and management of the long-term coronary complications after the ASO. The selection criteria yielded 73 articles They recorded few long-term coronary artery complications following the ASO, which were difficult to recognize as most affected patients’ symptoms were absent or nonspecific. In patients with suspected coronary artery involvement, coronary angiography or computed tomography provided confirmation, with significant stenosis generally managed by percutaneous trans-catheter interventions.
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Contemporary Practice of Native Coarctation in Children Greater Than One Year of Age: A Society of Thoracic Surgeons Analysis
Published: February 06, 2025.
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Awais Ashfaq; Edo Bedzra; Konrad T. Rajab; Levi N. Bonnell; Ashok Muralidaran; Mark Bleiweis; David L.S. Morales; Robert H. Habib; Jeffrey Jacobs; James A. Quintessenza
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
ObjectiveLimited data exist on surgical repairs for native coarctation of the aorta (Native-CoA) after year-one of life. We sought to establish surgical outcomes benchmark of older Native-CoA repair patients.MethodsAll patients greater than one year of age who underwent off-pump Native-CoA repair were identified from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) between January 1998 and December 2022.ResultsA total of 2,898 patients older than one year (mean age: 6.1 years; mean weight: 23.7 kg) underwent Native-CoA repair through left thoracotomy at 131 STS-CHSD centers. Vascular syndromes including Turners, Williams, and Alagille, were relatively rare at 1.8%. The most common procedures were extended end-to-end (1,309/2898, 45.2%) end-to-end 1,107/2898, (38.2%), and patch aortoplasty ((239/2898, 8.2%). From 1998 to 2018, there was an overall increase in the number of patients (14 vs 156), explained largely by the increased number of hospitals (4 vs 70). However, after 2018, case counts declined by nearly 50%. Operative mortality was rare (n = 3). An average of 1.2 complications per patient (438 total) occurred in 365/2898 (12.6%) patients. The most common complications included chylothorax (101/2898, 3.5%), unplanned 30-day readmission (28/1917, 1.5%), and arrhythmia requiring drug therapy (17/1917, 0.9%). Median length of hospital stay was four days, with hospital stay >6 days in 248 patients (14.8%).ConclusionsFrequency of off-pump Native-CoA repairs (age >1 year) is decreasing despite no notable change in total participating hospitals and case counts. This is likely due to increased percutaneous interventions despite very low documented mortality and morbidity of off-pump surgical repairs.
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Introduction to Proceedings of the World University for Pediatric and Congenital Heart Surgery Webinars About Pulmonary Atresia and Ventricular Septal Defect
Published: February 05, 2025.
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Jeffrey P. Jacobs; James D. St. Louis; Darshan Reddy; Attilio A. Lotto; Osami Honjo; Sivakumar Sivalingam; Robert D. B. Jaquiss; Ignacio Juaneda; Kirsten Finucane; James K. Kirklin; Christo I. Tchervenkov; Carl L. Backer
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
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Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries: The Natural History and Late Presentation
Published: February 05, 2025.
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Darshan Reddy; Robert Kleinloog; Robin Kinsley
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries represents a heterogenous and complex congenital heart defect. Treatment pathways vary widely depending on center expertise. The recommended approach in infancy is to achieve uniform pulmonary artery and capillary bed development, and a biventricular repair when feasible. The natural history of this condition is particularly relevant in low-middle income countries where early intervention is not possible due to poor access to cardiac surgical care. This group of patients may present in late childhood or as adults with symptoms of chronic cyanosis that may be associated with cardiac failure. The surgical approach to this group may range from complete correction to pulmonary artery rehabilitation or unifocalization, or consideration for heart-lung transplantation.
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Outcomes of Atrioventricular Septal Defect Repair: Two-Institutions, 10-Year Experience in Cordoba, Argentina
Published: January 31, 2025.
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Ignacio Juaneda; Lucca Pizzulli; Paula Ferrari; Rodrigo Molinas; Juan Diaz; Irma Azar; Alejandro Allub; Ernesto Juaneda; James St Louis; Alejandro Peirone; Jenkins Kathy
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
BackgroundOutcomes following surgical repair of atrioventricular septal defect have improved over the last decades. Global mortality for repair of this defect is approximately 3.5%. We describe contemporary surgical outcomes from two institutions in Argentina.Material and MethodsA retrospective two-institution review was performed of patients operated on for atrioventricular septal defect (partial, transitional, and complete) at Hospital Privado Universitario de Cordoba and Hospital de Niños Cordoba, from January 2014 to December 2023. We analyzed demographic characteristics, operative data, mortality, and outcomes.ResultsOne hundred and ten patients were operated for atrioventricular septal defect: There were 78 patients with complete atrioventricular septal defect, 10 with partial atrioventricular septal defect, and 22 with transitional atrioventricular septal defect. Mean age and weight at operation were 1.17 year (range 0.04-13 years) and 7.53 kg (2.8-55 kg), respectively. For the complete atrioventricular septal defect subgroup, surgeries with the modified single patch technique had shorter cardiopulmonary bypass times when compared with the two patch technique (95.6 vs 139.7 min, respectively, P < .001). Hospital mortality was 4.5% (5 of 110 patients), all with complete atrioventricular septal defect operated with the double patch technique. Mean follow-up of the entire cohort was 57.4 months (1-118 months). Five patients underwent a reoperation at a mean time from the first operation of 14.4 months (6-24 months).ConclusionsAtrioventricular septal defect surgical repair was successfully achieved in different ages and anatomical subtypes in most patients. Complete atrioventricular septal defect had higher mortality when compared with other subgroups. Our review shows similar outcomes following repair of atrioventricular septal defect compared with an international aggregate in a resource-limited practice.
