Assessment of Diastolic Function in Single-Ventricle Patients After the Fontan Procedure.

Background: Patients with functional single ventricles after the Fontan procedure have abnormal cardiac mechanics. The aims of this study were to determine factors that influence diastolic function and to describe associations of diastolic function with current clinical status.

Methods: Echocardiograms were obtained as part of the Pediatric Heart Network Fontan Cross-Sectional Study.

A systematic method for using 3D echocardiography to evaluate tricuspid valve insufficiency in hypoplastic left heart syndrome.

With surgical palliation of hypoplastic left heart syndrome (HLHS), the tricuspid valve (TV) becomes the systemic atrioventricular valve and moderate/severe TV insufficiency (TVI), an adverse risk factor for survival to Fontan, has been reported in up to 35% of patients prior to stage I palliation. Precise echocardiographic identification of the mechanism of TVI cannot be determined by two-dimensional echocardiography. Three-dimensional echocardiography (3DE) can provide significant insight into the mechanisms of TVI.

Screening for aortic aneurysm after treatment of coarctation.

Isolated coarctation of the aorta (CoA) occurs in 6-8 % of patients with congenital heart disease. After successful relief of obstruction, patients remain at risk for aortic aneurysm formation at the site of the repair. We sought to determine the diagnostic utility of echocardiography compared with advanced arch imaging (AAI) in diagnosing aortic aneurysms in pediatric patients after CoA repair.

Safety and imaging quality of MRI in pediatric and adult congenital heart disease patients with pacemakers.

Background: Magnetic resonance imaging (MRI) is a standard of care in evaluating many disease processes. Given concerns about device damage or movement, programming changes, lead heating, inappropriate pacing, and image artifact, MRI is contraindicated in pacemaker patients. Despite this, studies have demonstrated safety and efficacy of MRI in adults with acquired heart disease and endocardial pacing leads.

Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy?

Background: Progressive cardiomyopathy is a common cause of death in Duchenne muscular dystrophy (DMD), presumably secondary to fibrosis of the myocardium. The posterobasal and left lateral free wall of the left ventricle (LV) are initial sites of myocardial fibrosis pathologically. The purposes of this study were to assess whether cardiac magnetic resonance imaging (CMRI), utilizing late gadolinium enhancement (LGE), could identify fibrosis in selective areas of the myocardium, and to assess the relationship of the presence and extent of fibrosis to LV function.

Anomalous right coronary artery from the pulmonary artery: noninvasive diagnosis and serial evaluation.

Isolated anomalous origin of the right coronary artery from the main pulmonary artery (ARCAPA) is a rare congenital cardiac malformation. We reviewed the current literature and found only 31 patients with ARCAPA. We report the first case that was diagnosed and followed on a noninvasive basis with cardiovascular magnetic resonance after surgical re-implantation.

Usefulness of real-time navigator magnetic resonance imaging for evaluating coronary artery origins in pediatric patients.

Navigator coronary magnetic resonance imaging (MRI) was evaluated in assessing coronary artery origins in a pediatric and adolescent population. Sixty-five consecutive infants, children, or adolescents (age range 11 days to 21 years) were referred for MRI evaluations to assess coronary artery origins. Coronary artery origins were unambiguously delineated in 62 of 65 patients.

Myocardial performance index with sevoflurane-pancuronium versus fentanyl-midazolam-pancuronium in infants with a functional single ventricle.

Background: Patients with congenital heart disease characterized by a functional single ventricle make up an increasing number of patients presenting for cardiac or noncardiac surgery. Conventional echocardiographic methods to measure left ventricular function, i.e.

Anatomic reconstruction for recurrent aortic obstruction in infants and children.

Background: Patients undergoing operative repair of aortic obstruction are at a lifelong risk of recurrent obstruction, and there is controversy regarding the optimal surgical technique. We have used an alternative strategy for recurrent aortic obstruction, typically involving anatomic reconstruction by means of a median sternotomy, and describe our techniques and results.

Methods: Twenty-one patients presented with recurrent aortic arch obstruction.

The Warden procedure for partially anomalous pulmonary venous connection to the superior caval vein.

Purpose: When there is partially anomalous pulmonary venous connection to the superior caval vein, intracardiac repair alone can result in obstruction. Although the Warden procedure involving translocation of the superior caval vein is commonly performed as an alternative to atriocavoplasty, follow-up of a larger number of patients in the modern era is lacking. We report and discuss the experience of a single institution with the Warden procedure for correction of partially anomalous pulmonary venous connection to the superior caval vein.