Longitudinal Evaluation of Congenital Cardiovascular Surgical Performance and Skills Retention Using Silicone-Molded Heart Models.

Objective: Hands-on surgical training (HOST) for congenital heart surgery (CHS), utilizing silicone-molded models created from 3D-printing of patients' imaging data, was shown to improve surgical skills. However, the impact of repetition and frequency of repetition in retaining skills has not been previously investigated. We aimed to longitudinally evaluate the outcome for HOST on two example procedures of different technical difficulties with repeated attempts over a 15-week period. Methods: Five CHS trainees were prospectively recruited. Repair of coarctation of the aorta (CoA) and arterial switch operation (ASO) were selected as example procedures of relatively low and high technical difficulty. Procedural time and technical performance (using procedure-specific assessment tools by the participant, a peer-reviewer, and the proctor) were measured. Results: Coarctation repair performance scores improved after the first repetition but remained unchanged at the follow-up session. Likewise, CoA procedural time showed an early reduction but then remained stable (mean [standard deviation]: 29[14] vs 25[15] vs 23[9] min at 0, 1, and 4 weeks). Conversely, ASO performance scores improved during the first repetitions, but decreased after a longer time delay (>9 weeks). Arterial switch operation procedural time showed modest improvements across simulations but significantly reduced from the first to the last attempt: 119[20] versus 106[28] min at 0 and 15 weeks, P = .049. Conclusions: Complex procedures require multiple HOST repetitions, without excessive time delay to maintain long-term skills improvement. Conversely, a single session may be planned for simple procedures to achieve satisfactory medium-term results. Importantly, a consistent reduction in procedural times was recorded, supporting increased surgical efficiency.

Improving Outcomes in Pulmonary Vein Stenosis: Novel Pursuits and Paradigm Shifts.

Pulmonary vein stenosis (PVS) remains a clinical challenge, with progressive restenosis being common. In the past five years, we have seen an exponential increase in both clinical and scientific publication related to PVS. Central to progress in PVS clinical care is the paradigm shift towards collaborative, multidisciplinary care that utilizes a multimodality approach to treatment. This manuscript will discuss recent conceptual gains in PVS treatment and research while highlighting important outstanding questions and barriers.

Case report: Foreign body aspiration requiring extracorporeal membrane oxygenation.

Introduction: Foreign body aspiration is a common cause of respiratory distress in pediatrics, but the diagnosis can be challenging given aspirated objects are mostly radiolucent on chest radiographs and there is often no witnessed choking event. We present a case of a patient who was initially managed as severe status asthmaticus, requiring veno-arterial extracorporeal membrane oxygenation (VA-ECMO) for refractory hypercarbia and hypoxemia, but was later found to have bilateral bronchial foreign body aspiration. This case is unique in its severity of illness, diagnostic dilemma with findings suggesting a more common diagnosis of asthma, and use of ECMO as a bridge to diagnosis and recovery. Patient case: A previously healthy 2-year-old boy presented during peak viral season with a 3-day history of fever, cough, coryza, and increased work of breathing over the prior 24 h. There was no reported history of choking or aspiration. He was diagnosed with asthma and treated with bronchodilator therapy. Physical examination revealed pulsus paradoxus, severe work of breathing with bilateral wheeze, and at times a silent chest. Chest radiographs showed bilateral lung hyperinflation. Following a brief period of stability on maximum bronchodilator therapies and bilevel positive pressure support, the patient had a rapid deterioration requiring endotracheal intubation, with subsequent cannulation to VA-ECMO. A diagnostic flexible bronchoscopy was performed and demonstrated bilateral foreign bodies, peanuts, in the right bronchus intermedius and the left mainstem bronchus. Removal of the foreign bodies was done by rigid bronchoscopy facilitating rapid wean from VA-ECMO and decannulation within 24 h of foreign body removal. Conclusion: Foreign body aspiration should be suspected in all patients presenting with atypical history and physical examination findings, or in patients with suspected common diagnoses who do not progress as expected or deteriorate after a period of stability. Extracorporeal life support can be used as a bridge to diagnosis and recovery in patients with hemodynamic or respiratory instability.

Optimal Surgical Management of Tetralogy of Fallot.

Tetralogy of Fallot with pulmonary stenosis has a diverse clinical spectrum with the degree of right ventricular outflow tract obstruction (RVOTO) and size of the branch pulmonary arteries driving clinical management. Optimal surgical management involves consideration of patient clinical status and degree and location (subvalvar, valvar, and supravalvar) of RVOTO. Timing of repair requires multidisciplinary decision-making and complete surgical repair with relief of RVOTO by either transannular patch or valve sparing repair techniques. The central goals of contemporary surgical management of tetralogy of Fallot incorporate maximizing survival, minimizing reintervention, and preserving right ventricular function across the lifespan.

En contexte de tétralogie de Fallot (TF) avec sténose pulmonaire (SP), le degré d'obstruction à l'éjection du sang par le ventricule droit (OESVD) et le diamètre des branches des artères pulmonaires se caractérisent par divers tableaux cliniques et motivent la prise en charge clinique. Pour une prise en charge chirurgicale optimale, le statut clinique du patient ainsi que le degré et l'emplacement de l'OESVD (avant, après ou au niveau de la valve) doivent être pris en considération. Le moment de l'intervention chirurgicale doit être décidé par une équipe multidisciplinaire, et la technique de greffe en pièce transannulaire ou une approche avec conservation de la valve doit être utilisée pour réaliser une réparation complète. Les objectifs principaux de la prise en charge chirurgicale actuelle de la TF sont de maximiser la survie, de limiter les réinterventions chirurgicales et de préserver la fonction du ventricule droit pour toute la vie du patient.

Innocent until proven guilty? Longstanding atrial ectopy preceding cardiac rhabdomyoma diagnosis in tuberous sclerosis complex: a case report.

BACKGROUND: Cardiac rhabdomyoma are the most common cardiac tumour in childhood and are associated with tuberous sclerosis complex (TSC) up to 96% of infant cases. They classically manifest in the foetal and neonatal period, undergo spontaneous regression in the first years of life and are associated with arrhythmia in part due to interruption of normal conduction pathways by the tumour. CASE SUMMARY: We present a case of a 3-year-old boy with a long-standing history of atrial ectopy who was incidentally found to be in atrial flutter due to a new, rapidly growing cardiac rhabdomyoma impacting ventricular function. The boy was later confirmed with further investigation and TSC1 gene test to have TSC. DISCUSSION: Cardiac Rhabdomyoma does not always present in the infantile period. Any ongoing or new cardiac concern in patient with TSC, even if seemingly minor, should warrant more frequent cardiac evaluation and investigation.

Anterior Translocation of the Right Pulmonary Artery to Relieve Bronchial Compression in Truncus Arteriosus With Interrupted Aortic Arch.

We report an anterior translocation of the right pulmonary artery procedure to relieve severe left bronchial obstruction that was caused by right pulmonary artery stent placement in a 1-year-old patient with truncus arteriosus and interrupted aortic arch. After neonatal repair, the patient re-presented with severe truncal valve regurgitation, right pulmonary artery stenosis, and severe biventricular dysfunction, which was treated with truncal valve repair and right pulmonary artery plasty. The patient suffered from left bronchial compression from right pulmonary artery stent placement, which was successfully treated by the translocation procedure. Bronchial stenosis was successfully relieved by the translocation procedure. Indications, advantages, and disadvantages of this procedure are discussed.

Pulmonary Vein Stenosis: Incremental Knowledge Gains to Improve Outcomes.

Pulmonary vein stenosis remains a considerable clinical challenge, with high mortality still present in children with progressive disease. In this review, we discuss the clinical spectrum of pulmonary vein stenosis and what is known about the etiology and potential modifying and contributing factors in progressive pulmonary vein stenosis.

Primary pulmonary vein stenosis during infancy: state of the art review.

Primary pulmonary vein stenosis (PPVS) is an emerging problem among infants. In contrast to acquired disease, PPVS is the development of stenosis in the absence of preceding intervention. While optimal care approaches remain poorly characterized, over the past decade, understanding of potential pathophysiological mechanisms and development of novel therapeutic strategies are increasing. A multidisciplinary team of health care providers was assembled to review the available evidence and provide a common framework for the diagnosis, management, and treatment of PPVS during infancy. To address knowledge gaps, institutional and multi-institutional approaches must be employed to generate knowledge specific to ex-premature infants with PPVS. Within individual institutions, creation of a team comprised of dedicated health care providers from diverse backgrounds is critical to accelerate clinical learning and provide care for infants with PPVS. Multi-institutional collaborations, such as the PVS Network, provide the infrastructure and statistical power to advance knowledge for this rare disease.

Predicting the Future: Tetralogy of Fallot Will Be Primarily Treated with Catheter Based Intervention Within Two Decades. Surgeon's Perspective.

Interventional cardiology has made extraordinary advances over recent years, but most are still limited to addressing single intracardiac or valvular lesions. This debate considers whether complete interventional repair of more complex congenital defects might become achievable. Tetralogy of Fallot (ToF) is probably the first candidate where complete interventional repair might be achieved-given that various components of the defect have already been successfully addressed-albeit as either a palliative intervention (RVOT stenting) or to address the sequelae of standard surgery (percutaneous PVR). This article considers the challenges that would need to be overcome in terms of the morphology of the condition, the age limitations, and the necessary technological advancements that would be required-while setting these against the benchmark of current surgical outcomes and the parallel progress that is being developed in surgical correction. While complete interventional repair of ToF may still be beyond current techniques, a hybrid approach between surgeons and intentional cardiologists can strive to create a life-long paradigm of care that minimizes the need for surgery and focuses on the maintenance of a healthy right ventricle, such that patients born with ToF can achieve normal life expectancy.

Pulmonary Vein Stenosis in Infants: A Systematic Review, Meta-Analysis, and Meta-Regression.

OBJECTIVE: To quantify outcomes of infants (<1 year of age) diagnosed with pulmonary vein stenosis (PVS). STUDY DESIGN: MEDLINE (PubMed), Scopus, and Web of Science were searched through February 1, 2017, with no language restrictions. Publications including infants diagnosed with primary PVS, defined as the absence of preceding intervention(s), were considered. The study was performed according to Meta-analysis of Observational Studies in Epidemiology guidelines, the Systematic Reviews, and Meta-Analysis checklist, and registered prospectively. The quality of selected reports was critically examined. Data extraction was independently performed by multiple observers with outcomes agreed upon a priori. Data were pooled using an inverse variance heterogeneity model with incidence of mortality the primary outcome of interest. RESULTS: Forty-eight studies of 185 infants were included. Studies were highly diverse with regards to the participants, interventions, and outcomes reported. The median (range) age at diagnosis was 5.0 (0.1-11.6) months. Pooled mortality was 58.5% (95% CI 49.8%-67.0%, I2 = 21.4%). We observed greater mortality incidence among infants with 3 or 4 vein stenoses than in those with 1 or 2 vein stenoses (83.3% vs 36.1%; P < .01). We observed greater mortality among infants with bilateral than unilateral disease (78.7% vs 26.0%; P < .01). CONCLUSIONS: Studies of primary PVS during infancy are highly variable in their methodological quality and estimates of clinical outcomes; therefore, estimates of prognosis remain uncertain. Multicenter, interdisciplinary collaborations, including alignment of key outcome measurements, are needed to answer questions beyond the scope of available data.

Surgical Approaches to Total Anomalous Pulmonary Venous Connection.

Total anomalous pulmonary venous connection (TAPVC) constitutes a spectrum of congenital lesions whereby the pulmonary veins remain connected to systemic venous vessels or aberrantly connect to the right atrium. Definitive management requires surgical intervention and, in patients with obstruction to pulmonary venous flow, urgent operation is required. Use of temporizing catheter-based interventions allow for optimization in hemodynamically unstable neonates. Overall, survival has significantly improved over the past decades through better perioperative management and evolution of surgical approaches to minimize post-repair pulmonary vein stenosis, which persists as a major determinant of long-term outcomes.