Infant cardiac magnetic resonance imaging using oscillatory ventilation: safe and effective.

Cardiac magnetic resonance imaging (CMR) for infants and young children typically requires sedation. General anesthesia with controlled ventilation can eliminate motion artifact with breath-holds during imaging to limit respiratory artifact, but these may lead to atelectasis or other complications. High-frequency oscillatory ventilation (HFOV) provides ventilation with near-constant mean airway pressure and minimal movement of chest wall and diaphragm, thus obviating the need for breath-holding.

Fontan fenestration closure and event-free survival.

Objectives: The purpose of the present study was to evaluate the association of open and closed Fontan fenestration status with event-free survival.

Methods: All patients who underwent a fenestrated Fontan procedure at our institution from January 1994 through June 2007 were reviewed. Patient information was obtained from the medical records.

Dual-axis rotational coronary angiography: a new technique for detecting graft coronary vasculopathy in pediatric heart transplant recipients.

Annual surveillance coronary angiograpyhy to screen for graft coronary vasculopathy is routine practice after orthotopic heart transplantation. Traditionally, this is performed with direct coronary angiography using static single-plane or biplane angiography. Recently, technological advances have made it possible to perform dual-axis rotational coronary angiography (RA).

Type 2 Abernethy malformation presenting as a portal vein-coronary sinus fistula.

We report the case of a child with Abernethy malformation with an anomalous connection between the portal vein and the coronary sinus. After 30 months of close follow-up, the patient developed hepatoblastoma, a previously documented complication of the Abernethy malformation. This case reports a unique variant of Abernethy malformation and documents the first reported case of hepatoblastoma in a patient with type 2 Abernethy malformation.

Automated external defibrillators and secondary prevention of sudden cardiac death among children and adolescents.

Sudden cardiac death is devastating at any age, but it is especially so among children and adolescents. This report discusses the outcomes for patients with out-of hospital cardiac arrest (OHCA) and describes public access defibrillation programs in general and those directed at children and adolescents. In addition, the relatively new concept of cardiopulmonary resuscitation (CPR)–automated external defibrillator (AED) programs directed at schools is discussed.

Near infrared spectroscopy describes physiologic payback associated with excess postexercise oxygen consumption in healthy controls and children with complex congenital heart disease.

Exercise creates a physiologic burden with recovery from such effort crucial to adaptation. Excess postexercise oxygen consumption (EPOC) refers to the body's increased metabolic need after work. This investigation was designed to determine the role of near infrared spectroscopy (NIRS) in the description of exercise recovery in healthy controls (NL) and children with congenital heart disease (CHD).

Implementation of a routine developmental follow-up program for children with congenital heart disease: early results.

Objective: To describe the implementation of a routine developmental follow-up program for children with congenital heart disease, summarize the developmental outcomes of the first clinic visits of the referred patients, and determine what factors predict variability in early developmental outcomes.

Design: Infants with congenital heart disease who had cardiac surgery within the first 30 days of life, had a cyanotic lesion (with or without surgery) or were believed to be at risk for developmental delay due to comorbid conditions or perioperative complications such as seizures or stroke were referred to the program as part of standard clinical care. Patients were evaluated using the Bayley Scales of Infant and Toddler Development-III.

Aortopulmonary window.

Aortopulmonary window is a rare defect caused by failure of fusion of the two opposing conotruncal ridges that are responsible for separating the truncus arteriosus into the aorta and pulmonary artery. Aortopulmonary window may occur as an isolated lesion or it can be associated with other cardiac abnormalities in one third to one half of cases. The most common associated lesions are arch abnormalities, specifically interrupted aortic arch and coarctation of the aorta.

Surgery and critical care for anomalous coronary artery from the pulmonary artery.

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital cardiac malformation that accounts for 0.25-0.50% of children with congenital cardiac disease and can cause myocardial dysfunction in young infants.

Corrected QT Interval in Children With Brain Death.

Prolongation of the QT interval is a well-documented finding in adults with severe brain injury. However, QT prolongation has not been well documented in the pediatric population with brain injury. Our objective was to determine the range of QT intervals in children with the diagnosis of brain death, hypothesizing that the QT interval corrected for heart rate (QTc) is longer in this population than in a normal population.

Perioperative monitoring in high-risk infants after stage 1 palliation of univentricular congenital heart disease.

Objective: Survival of high-risk patients with univentricular heart disease after Norwood palliation is reduced. We hypothesized that early goal-directed monitoring with venous oximetry and near-infrared spectroscopy would offset their increased vulnerability and improve survival.

Methods: A prospective database of patients undergoing stage 1 palliation was used to assess differences in outcomes across risk groups in the setting of a comprehensive, goal-directed monitoring program.

Successful congenital heart surgery for a toddler with idiopathic infantile arterial calcification.

We present the first case report of successful cardiac surgery in a child with idiopathic infantile arterial calcification (IIAC), a disease that is generally lethal within the first 6 months of life. This 27-month-old Hispanic American boy with IIAC successfully underwent cardiothoracic surgery for severe pulmonary valve (PV) stenosis after unsuccessful balloon valvotomy in the cardiac catheterization laboratory.

Hypersensitivity reactions to aprotinin re-exposure in paediatric surgery.

Objective: Hypersensitivity to aprotinin is low (1-3%) but more likely with re-exposure. The manufacturer issued a black box warning which lists aprotinin re-exposure within 1 year of prior exposure as a contraindication. We investigated the temporal relationship between re-exposure interval and hypersensitivity in children.

Fontan palliation in the modern era: factors impacting mortality and morbidity.

Background: Advances in management of the Fontan patient include interval superior cavopulmonary shunt, total cavopulmonary connection, either lateral tunnel or extracardiac conduit, and the use of a fenestration. Coincident with these improvements, Fontan palliation has been applied to a wider ranger of anatomic subgroups.

Methods: A cross-sectional analysis of 256 consecutive patients undergoing a total cavopulmonary connection Fontan after superior cavopulmonary shunt between January 1, 1994, and June 30, 2007 were studied.

Automated external defibrillators utility, rational, and controversies.

Automated external defibrillators (AEDs) and their role in resuscitation has been documented in the adult population with the reported beneficial outcomes documented in multiple studies. Data for children are lacking because of the relatively small incidence of cardiac arrest in the pediatric population as well as the absence of randomized studies. There have been a few studies that have reviewed sudden cardiac arrest in the school setting as well the use and efficacy of AEDs.

Near-infrared spectroscopy in neonates before palliation of hypoplastic left heart syndrome.

Background: Neonates with hypoplastic left heart syndrome have circulatory vulnerability that results in shock and high risk of mortality without intervention. High arterial saturation (SaO(2)) is often used as a proxy for inadequate systemic oxygen delivery and triggers the use of invasive therapies to restore circulatory balance. We hypothesized that preoperative use of near-infrared spectroscopy (NIRS) would reduce the need for invasive therapies, including controlled ventilation and inspired gas manipulation.

Causes of recurrent focal neurologic events after transcatheter closure of patent foramen ovale with the CardioSEAL septal occluder.

Transcatheter patent foramen ovale (PFO) closure has been undertaken to eliminate paradoxical emboli as a cause for recurrent strokes/transient ischemic attacks (TIAs). We report the results of investigations to determine causes of all significant focal neurologic events (FNEs) after PFO closure reported to our center. Records of 216 consecutive patients who underwent PFO closure were reviewed.

Assessment of regional right ventricular velocities, strain, and displacement in normal children using velocity vector imaging.

Background: Velocity vector imaging (VVI) is a novel technique to assess regional myocardial mechanics. We evaluated the utility of VVI in the assessment of right ventricular (RV) function in normal children.

Methods: RV images (apical 4-chamber view, high frame rate capture) from 30 normal children (mean 8.

Mixed venous oxygen saturation monitoring after stage 1 palliation for hypoplastic left heart syndrome.

Background: Staged palliation for hypoplastic left heart syndrome has been marked by high early mortality due to the limited cardiac output of the postischemic single right ventricle combined with the inefficiency and volatility of parallel circulation.

Methods: Since July 1996, we have performed stage 1 palliation (S1P) in 178 patients. Within this group is a consecutive cohort of 116 patients with true hypoplastic left heart syndrome that underwent S1P with a modified Blalock-Taussig shunt.