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

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.

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.

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.

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.

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.

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.

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.

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).

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.

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.

Transaortic septal myectomy: techniques and pitfalls.

Hypertrophic cardiomyopathy (HCM) is the most common congenital cardiac disease, affecting up to 1 in 200 individuals. When it causes left ventricular outflow tract (LVOT) obstruction, treatment is indicated to reduce symptoms and the risk of sudden cardiac death. Pharmacologic therapy is the first line treatment, however if it fails, surgical myectomy or percutaneous ablation of the hypertrophic myocardium are the standard therapies to eliminate subaortic obstruction.