Congenital cardiac anomalies and white matter injury.

Cardiac abnormalities are the most common birth defects. Derangement of circulatory flow affects many vital organs; without proper supply of oxygenated blood, the brain is particularly vulnerable. Although surgical interventions have greatly reduced mortality rates, patients often suffer an array of neurological deficits throughout life.

Personal glimpses into the evolution of truncus arteriosus repair.

Truncus arteriosus (common arterial trunk) is an uncommon but complex congenital heart anomaly. Until the early 1970s, typically, patients died between the age of a few weeks to six months. Congestive heart failure owing to large pulmonary blood flow and truncal valve regurgitation was the major cause of death until innovative surgical techniques were discovered.

Splitting blood and blood product packaging reduces donor exposure for patients undergoing cardiopulmonary bypass.

Background: Cardiopulmonary bypass for congenital heart surgery requires packed red cells (PRBC) and fresh frozen plasma (FFP) to be available, both for priming of the circuit as well as to replace blood loss. This study examines the hypothesis that splitting one unit of packed red blood cells and one unit of fresh frozen plasma into two half units reduces blood product exposure and wastage in the Operating Room.

Methods: Beginning August 2013, the blood bank at Children's National Medical Center began splitting one unit of packed red blood cells (PRBC) and one unit of fresh frozen plasma (FFP) for patients undergoing cardiopulmonary bypass (CPB).

Choice of Peripheral Venoarterial Extra-Corporeal Membrane Oxygenation Cannulation Site in Patients Above 15 kilograms.

Background: Extracorporeal membrane oxygenation (ECMO) is a life-saving measure for pediatric patients with cardiopulmonary failure. The option of cannulating neck vessels versus those of the groin exists for patients over 15 kg; however, each carries the risk for complications. We present a single-center experience comparing the risks and benefits of these alternate peripheral ECMO cannulation sites.

Magnesium Lowers the Incidence of Postoperative Junctional Ectopic Tachycardia in Congenital Heart Surgical Patients: Is There a Relationship to Surgical Procedure Complexity?

Magnesium sulfate was given to pediatric cardiac surgical patients during cardiopulmonary bypass period in an attempt to reduce the occurrence of postoperative junctional ectopic tachycardia (PO JET). We reviewed our data to evaluate the effect of magnesium on the occurrence of JET and assess a possible relationship between PO JET and procedure complexity. A total of 1088 congenital heart surgeries (CHS), performed from 2005 to 2010, were reviewed.

A contemporary, single-institutional experience of surgical versus expectant management of congenital heart disease in trisomy 13 and 18 patients.

The objective of this study was to examine a large institutional experience of patients with trisomy 13 and trisomy 18 in the setting of comorbid congenital heart disease and present the outcomes of surgical versus expectant management. It is a retrospective single-institution cohort study. Institutional review board approved this study.

Congenital aneurysm of the aortomitral intervalvular fibrosa.

A 5-year-old boy was found to have a congenital left ventricular outflow tract (LVOT) aneurysm of the intervalvular fibrosa, LVOT obstruction after repair of a perimembranous ventricular septal defect, and aortic coarctation. The patient underwent successful plication of the aneurysm, resection of the fibrous subaortic stenosis, and septal myectomy.

Surgical cardiac denervation therapy for treatment of congenital ion channelopathies in pediatric patients: a contemporary, single institutional experience.

Background: Congenital ion channel disorders, including congenital long QT syndrome (LQTS), cause significant morbidity in pediatric patients. When medication therapy does not control symptoms or arrhythmias, more invasive treatment strategies may be necessary. This study examines our institution's clinical experience with surgical cardiac denervation therapy for management of these arrhythmogenic disorders in children.

Single center experience on dosing and adverse events of recombinant factor seven use for bleeding after congenital heart surgery.

There are limited data on the relationship between the administered dose of recombinant factor seven (rFVIIa) and the development of adverse clinical outcomes after congenital heart surgery. This single institution case series reports on dosing, adverse events, and blood product usage after the administration of rFVIIa in the congenital heart surgery patient population. A retrospective review identified 16 consecutive pediatric patients at an academic, free-standing, children's hospital who received rFVIIa to curtail bleeding following congenital heart surgery between April 2004 and June 2012.

Genotypes and antibiotic resistance of bovine Campylobacter and their contribution to human campylobacteriosis.

Campylobacter jejuni and Campylobacter coli are the most important bacterial causes of human gastroenteritis. Chicken has been recognized as a major source for human infection, whereas cattle might also contribute to a lesser extent. However, there is a paucity of information available regarding Campylobacter in Swiss cattle and their role for human campylobacteriosis.

Early primary repair of tetralogy of Fallot does not lead to increased postoperative resource utilization.

Background: Although early primary repair of tetralogy of Fallot has gained wider acceptance, there is some speculation that repair at a younger age may be associated with increased morbidity and resource utilization.

Methods: A retrospective review of all consecutive patients undergoing tetralogy of Fallot repair between September 2004 and December 2011 was performed. Primary end points were hospital charges, and surrogates of postoperative hospital resource utilization, including ventilation time, intensive care unit (ICU) stay, and hospital stay.

Twenty-four hour in-hospital congenital cardiac surgical coverage improves perioperative ECMO support outcomes.

Background: Extracorporeal membrane oxygenation (ECMO) support is often required in the management of perioperative congenital heart surgery (CHS) patients. However, 24-hour in-hospital congenital cardiac surgical coverage (24-CCSC) is not available at all institutions. The purpose of this study is to evaluate the effect of 24-CCSC on perioperative ECMO outcomes in CHS patients.

Incorporating three-dimensional printing into a simulation-based congenital heart disease and critical care training curriculum for resident physicians.

Objective: Although simulation-based education is now commonly utilized in medicine, its use in the instruction of congenital heart disease remains limited. The objective of this study is to evaluate whether heart models created with three-dimensional printing technology can be effectively incorporated into a simulation-based congenital heart disease and critical care training curriculum for pediatric resident physicians.

Design: Utilizing heart models created with a three-dimensional printer, pediatric residents participated in a 60-minute simulation seminar with three consecutive components: (1) didactic instruction on ventricular septal defect anatomy; (2) didactic/simulation-based instruction on echocardiographic imaging of ventricular septal defects and anatomical teaching/operative simulation of ventricular septal defect repair; (3) simulation-based instruction on postoperative critical care management of ventricular septal defects.

The importance of patient-specific preoperative factors: an analysis of the society of thoracic surgeons congenital heart surgery database.

Background: The most common forms of risk adjustment for pediatric and congenital heart surgery used today are based mainly on the estimated risk of mortality of the primary procedure of the operation. The goals of this analysis were to assess the association of patient-specific preoperative factors with mortality and to determine which of these preoperative factors to include in future pediatric and congenital cardiac surgical risk models.

Methods: All index cardiac operations in The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) during 2010 through 2012 were eligible for inclusion.

Genotypes and antibiotic resistances of Campylobacter jejuni isolates from cattle and pigeons in dairy farms.

Campylobacter jejuni is the most common food-borne zoonotic pathogen causing human gastroenteritis worldwide and has assumed more importance in Italy following the increased consumption of raw milk. Our objectives were to get an overview of genotypes and antibiotic resistances in C. jejuni isolated from milk, cattle feces, and pigeons in dairy herds of Northern Italy.

Utilizing Three-Dimensional Printing Technology to Assess the Feasibility of High-Fidelity Synthetic Ventricular Septal Defect Models for Simulation in Medical Education.

Background: The current educational approach for teaching congenital heart disease (CHD) anatomy to students involves instructional tools and techniques that have significant limitations. This study sought to assess the feasibility of utilizing present-day three-dimensional (3D) printing technology to create high-fidelity synthetic heart models with ventricular septal defect (VSD) lesions and applying these models to a novel, simulation-based educational curriculum for premedical and medical students.

Methods: Archived, de-identified magnetic resonance images of five common VSD subtypes were obtained.

Outcomes of tracheostomy following congenital heart surgery: a contemporary experience.

Introduction: Following congenital heart surgery, pediatric patients may experience persistent respiratory failure that requires tracheostomy placement. Currently, definitive knowledge of the optimal timing for tracheostomy placement in this patient population is lacking.

Methods: An 8-year retrospective review of 17 pediatric patients who underwent congenital heart surgery and subsequently required tracheostomy placement was performed.

Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association.

Background: The goal of this statement is to review available literature and to put forth a scientific statement on the current practice of fetal cardiac medicine, including the diagnosis and management of fetal cardiovascular disease.

Methods And Results: A writing group appointed by the American Heart Association reviewed the available literature pertaining to topics relevant to fetal cardiac medicine, including the diagnosis of congenital heart disease and arrhythmias, assessment of cardiac function and the cardiovascular system, and available treatment options. The American College of Cardiology/American Heart Association classification of recommendations and level of evidence for practice guidelines were applied to the current practice of fetal cardiac medicine.