Measured Oxygen Consumption During Pediatric Cardiac Catheterization is More Accurate than Assumed Oxygen Consumption.

When calculating cardiac index (C.I.) by the Fick method, oxygen consumption (VO) is often unknown, so assumed values are typically used.

Spontaneous rupture of a coronary artery fistula presenting with post-exertional syncope and haemopericardium.

An 8-year-old with a known diagnosis of an asymptomatic coronary artery fistula (CAF) presented with exertional syncope and haemopericardium due to contained rupture of CAF. A transcatheter closure was attempted, but the fistula ruptured again prompting emergency surgical repair. Spontaneous rupture is a catastrophic complication of CAF and warrants a high index of suspicion, timely diagnosis and team management.

Diagnosis and management of the adult patient with a failing Fontan circulation.

Since the original description, the Fontan operation has been widely used for the palliation of children with single ventricle physiology. Although the Fontan operation revolutionized the survival rates of patients with single ventricle physiology, it carries an inevitable risk for long-term morbidity and mortality that impacts clinical outcomes and quality of life. This review will focus on the evaluation and treatment of the patient with the failing Fontan phenotype, with an emphasis on creating an individualized treatment plan.

Surgical and Catheter-Based Reinterventions Are Common in Long-Term Survivors of the Fontan Operation.

Background: There are limited follow-up studies examining surgical and catheter-based reinterventions in long-term survivors of the Fontan operation.

Methods And Results: All 773 patients who underwent Fontan at our institution between 1992 and 2009 were retrospectively reviewed. Current information regarding post-Fontan intervention was available for 70%.

Effect of Fontan-Associated Morbidities on Survival With Intact Fontan Circulation.

Although survival after the Fontan operation has improved, little is known about the burden of major medical morbidities associated with the modern total cavopulmonary connection (TCPC). A total of 773 consecutive patients who underwent a first Fontan operation at our institution between 1992 and 2009 were retrospectively reviewed. All subjects underwent TCPC (53% lateral tunnel, 47% extracardiac conduit).

Long-term survival after the Fontan operation: Twenty years of experience at a single center.

Objective: Existing studies of patients palliated with the Fontan operation are limited by heterogeneous patient populations and incomplete follow-up. This study aimed to describe long-term post-Fontan survival in a modern patient cohort.

Methods: All 773 patients who underwent a first Fontan operation at our institution between 1992 and 2009 were reviewed.

Tetralogy of Fallot: General Principles of Management.

Repaired tetralogy of Fallot (TOF) is one of the most common diagnoses encountered when caring for adults with congenital heart disease. Although long-term survival after childhood TOF repair is excellent, morbidity is common and most patients require reintervention in adulthood. This review provides an overview of key surveillance and management issues for adults with TOF, including residual right ventricular outflow tract disease and timing of pulmonary valve replacement, arrhythmias and risk stratification, left-sided heart disease and heart failure, and pregnancy management.

Cumulative medical radiation exposure throughout staged palliation of single ventricle congenital heart disease.

Patients with single ventricle heart disease undergoing staged palliation are exposed to ionizing radiation from multiple medical sources. We sought to quantify typical radiation burden in this population and identify risk factors for increased exposure. A retrospective review was performed of single ventricle patients surgically palliated at our institution, in which all studies utilizing ionizing radiation occurring from birth through Fontan completion were compiled.

Cumulative exposure to medical sources of ionizing radiation in the first year after pediatric heart transplantation.

Background: Pediatric heart transplant recipients undergo a variety of radiologic tests with the attendant risk of exposure to ionizing radiation. We sought to quantify and describe the cumulative exposure to all forms of medical radiation during the first year after pediatric heart transplantation and identify factors associated with higher exposure.

Methods: Pediatric patients who received a heart transplant between January 2009 and May 2012 with follow-up at our institution were retrospectively reviewed.

Accuracy of conventional oximetry for flow estimation in patients with superior cavopulmonary connection: a comparison with phase-contrast cardiac MRI.

Background: Cardiac catheterization is routinely used as a diagnostic tool in single ventricle patients with superior cavopulmonary connection. This physiology presents inherent challenges in applying the Fick principle to estimate flow. We sought to quantitatively define the error in oximetry-derived flow parameters using phase-contrast cardiac MRI (CMR) as a reference.

Left-sided scimitar vein causing cyanosis after Fontan operation: successful transcatheter device occlusion using magnetic resonance imaging X-ray fusion.

We present the case of a 3-year-old boy with asplenia-type heterotaxy syndrome and functionally single ventricle congenital heart disease who developed cyanosis early after the Fontan operation. Combined cardiac magnetic resonance imaging (MRI) and catheterization identified a large hepatic vein to pulmonary vein connection as the source of right to left shunt. The anatomy was quite unusual, suggesting an underlying diagnosis of mixed total anomalous pulmonary venous connection with left-sided scimitar vein.

Pulmonary histopathology in an experimental model of chronic aspiration is independent of acidity.

Gastroesophageal reflux has become a major health concern in industrialized countries, with drugs aimed at blocking acid production being more frequently prescribed than any other drug. Damage to lung tissue as a result of chronic aspiration of gastric fluid is a primary health risk associated with gastro-esophageal reflux, with such aspiration being suspected in the induction or exacerbation of asthma and other lung diseases. In this study, a rodent model of chronic aspiration was used to characterize the pulmonary histopathology produced by repetitive aspiration events and to investigate the pathologic roles of individual gastric fluid components such as acid and particulate food matter.