James C Nielsen

• MD
• Associate Chief, Pediatric Cardiology at New York University

The right ventricle of subjects after infundibulotomy (n = 20) and of subjects free of right ventricular disease (n = 19) was partitioned into the infundibular and sinus segments to examine the relative size and function of each. Excellent agreement was found between observers for measuring regional volume and ejection fraction. Although the surgic...

The measurement error for right ventricular (RV) size and function assessed by cardiac MRI (CMRI) in congenital heart disease has not been fully characterized. As CMRI parameters are being increasingly utilized to make clinical decisions, defining error in the clinical setting is critical. This investigation examines the repeatability of CMRI for R...

Background and objective Although the right ventricle to pulmonary artery (RV-PA) conduit in stage 1 Norwood operation results in improved interstage survival, the long term effects of the ventriculotomy used in the traditional technique (TT) remain a concern. The ‘periscopic technique’ (PT) of RV-PA conduit placement has been described as an alter...

Background: Moxifloxacin is a priority recommended drug for rifampin-resistant tuberculosis (RR-TB) treatment, but there is limited pediatric pharmacokinetic and safety data, especially in young children. We characterize moxifloxacin population pharmacokinetics, QT-interval prolongation and evaluate optimal dosing in children with RR-TB. Methods:...

An established echocardiographic (echo) standard for assessing the newborn right ventricle (RV) for hypertrophy has not been thoroughly developed. This is partially due to the RV’s complex architecture, which makes quantification of RV mass by echo difficult. Here, we retrospectively evaluate the thickness of the inferior RV wall (iRVWT) by echo in...

Clofazimine is recommended for the treatment of rifampicin-resistant tuberculosis (RR-TB), but there is currently no verified dosing guideline for its use in children. There is only limited safety and no pharmacokinetic (PK) data available for children. We aimed to characterize clofazimine PK and its relationship with QT-interval prolongation in ch...

Rifampicin-resistant tuberculosis (RR-TB) involves treatment with many drugs that can prolong the QT interval; this risk may increase when multiple QT-prolonging drugs are used together. We assessed QT interval prolongation in children with RR-TB receiving one or more QT-prolonging drugs. Data were obtained from two prospective observational studie...

Background: Antiretroviral options for neonates (< 28 days of life) should be expanded. We evaluated the pharmacokinetics, safety, and acceptability of the '4-in-1' fixed-dose paediatric granule formulation of abacavir/lamivudine/lopinavir/ritonavir (30/15/40/10 mg) in neonates. Methods: The PETITE study is an ongoing phase I/II, open-label, sin...

This chapter focuses on congenital structural abnormalities of the mitral valve. Mitral valve prolapse (MVP) occurs when there is systolic motion of a leaflet segment beyond the annular plane. Historically, the definition of MVP has varied considerably resulting in some diagnostic confusion. The pathophysiology associated with either abnormal MV de...

Intrapericardial teratomas are rare, predominantly benign tumors that warrant surgical resection in the neonatal period because of their potential detrimental effects on the cardiorespiratory system. Surgical resection can be a challenge when the tumor encases and obscures a coronary artery. Adherence to certain operative principles is necessary to...

Diagnosis of anomalous origin of the right subclavian artery (AORSA) from the right pulmonary artery (RPA) is usually made using CT, MRI, or invasive angiography. We report a patient diagnosed using transthoracic echocardiography (TTE). A newborn girl prenatally known to have d‐TGA presented with cyanosis sparing the right hemithorax and arm. Oxyge...

Quantification of left ventricular (LV) mass by echocardiography has not been validated against the gold standard of cardiac magnetic resonance imaging (CMR) in the pediatric population. The purpose of this study was to compare LV mass by two-dimensional and conventional M-mode echocardiography versus CMR in children. Consecutive CMR studies were p...

Right atrial (RA) size is a prognostic indicator for heart failure and cardiovascular death in adults. Data regarding use of RA area (RAA) by two-dimensional echocardiography as a surrogate for RA size and allometric modeling to define appropriate indexing of the RAA are lacking. Our objective was to validate RAA as a reliable measure of RA size an...

3D imaging datasets from cardiac computed tomography (CT), magnetic resonance imaging (CMR), or echocardiography can be used to create 3D virtual models for 3D printing. Although CT imaging is not considered first line to diagnose congenital heart disease, it has significant advantages when a 3D printed cardiac model is needed. Advantages over ultr...

Assessment of left ventricular dilation plays an important role in the management of left ventricular volume overload lesions. Various echocardiographic methods exist, such as the 5/6 area-length and biplane Simpsons, but their agreement with cardiac magnetic resonance imaging in patients with chronic left ventricular volume overload from a young a...

Objective: To compare chest X-ray with echocardiogram (ECHO) in the localization of an umbilical venous catheter (UVC) tip in very low birth weight infants (VLBW). Secondary objectives determined the association between techniques for tip placement by the vertebral body level on X-ray, as well as the length of the thoracic inferior vena cava-right...

The method of cardiac magnetic resonance (CMR) three-dimensional (3D) image acquisition and post-processing which should be used to create optimal virtual models for 3D printing has not been studied systematically. Patients (n = 19) who had undergone CMR including both 3D balanced steady-state free precession (bSSFP) imaging and contrast-enhanced m...

Cardiac magnetic resonance (CMR) assesses myocardial involvement in myocarditis (MYO). Current techniques are qualitative, subjective, and prone to interpretation error. Feature tracking (FT) analyzes myocardial strain using CMR and has not been examined in MYO. We hypothesize that regional left ventricular (LV) strain is abnormal in MYO. Regional...

As the population of adults with congenital heart disease continues to grow, so does the number of these patients with heart failure. Ventricular assist devices are underutilized in adults with congenital heart disease due to their complex anatomic arrangements and physiology. Advanced imaging techniques that may increase the utilization of mechani...

Cardiovascular magnetic resonance (CMR) imaging in adults is considered the gold standard for assessment of left ventricular mass (LVM) and left ventricular hypertrophy (LVH). The authors aimed to evaluate agreement of LVM measurements and LVH determination between echocardiography (ECHO) and CMR imaging in children with hypertension (HTN) confirme...

Anomalies of the mitral valve may involve one or more of its components: the mitral annulus, valve leaflets, chordae tendinae, or papillary muscles. Mitral valve anomalies are commonly associated with additional cardiovascular anomalies. This chapter focuses on isolated congenital mitral valve anomalies - mitral valve prolapse, cleft mitral valve,...

Open cardiovascular magnetic resonance (CMR) scanners offer the potential for imaging patients with claustrophobia or large body size, but at a lower 1.0 Tesla magnetic field. This study aimed to evaluate the efficacy of open CMR for evaluation of pediatric and congenital heart disease. This retrospective, cross-sectional study included all patient...

Our goal was to construct three-dimensional (3D) virtual models to allow simultaneous visualization of the ventricles, ventricular septal defect (VSD) and great arteries in patients with complex intracardiac anatomy to aid in surgical planning. We also sought to correlate measurements from the source cardiac magnetic resonance (CMR) image dataset a...

The presence of myocardial late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) imaging in concert with electrocardiography and elevated biomarkers helps support the diagnosis of acute myocarditis. Two-dimensional echocardiography is limited to global and qualitative regional function assessment and may not contribute to the diagno...

This study aimed to determine whether quantification of subpulmonary stenosis (SPS) in tetralogy of Fallot (TOF) in the second-trimester fetus can predict postnatal clinical outcome measured by pulmonary valve size and/or timing or type of intervention. The study retrospectively identified fetuses with TOF from 1998 to 2010 diagnosed at 26 weeks ge...

Background: Decreased right ventricular (RV) ejection fraction (EF) increases morbidity in repaired tetralogy of Fallot (rToF). Cardiac magnetic resonance (CMR) is the accepted reference standard for RV EF measurement. There are no established color Doppler tissue imaging (cDTI) values that identify decreased RV EF in rToF. We sought to assess acc...

Thoracic aortic disease is a known cause of aortic dilatation and poses significant risk of aortic dissection and rupture. Serial assessment of aortic root dimensions is traditionally performed using echocardiography, which is limited with older age and following surgery, due to poor acoustic windows. Although diastolic measurements are utilized as...

Purpose: Currently the diagnosis of vascular rings relies on multiple imaging studies. Neither clear guidelines nor strong supporting evidence exist to the safest, most cost effective approach for accurate diagnosis. This is a retrospective review of the diagnostic testing performed prior to the definitive diagnosis of vascular rings. Specifically,...

Background: Left atrial volume (LAV) increase is an indicator of diastolic dysfunction and a surrogate marker of significant left to right shunts. Normalization of LAV is currently performed by indexing to body surface area(1) (BSA(1)). The indexed LAV thus derived does not account for the nonlinear relationship of physiologic variables to BSA and...

Accurate assessment of the proximal thoracic vasculature in infants and children with congenital heart disease (CHD) is vital for deciding the appropriate surgical or interventional procedure and predicting outcomes. This information usually is obtained by transthoracic echocardiography (TTE). Contrast-enhanced magnetic resonance angiography (CE-MR...

Introduction: The prevalence of right ventricular dysfunction in idiopathic dilated cardiomyopathy is incompletely studied in children. Furthermore, right ventricular function may signal worse outcomes. We evaluated recently published right ventricular function echocardiographic indices in identifying dysfunction in children with idiopathic dilate...

Background Accurate assessment of the proximal thoracic vasculature in infants with congenital heart disease (CHD) is vital in deciding the appropriate surgical or interventional procedure and predicting outcomes. In most infants this information is obtained by transthoracic echocardiography (TTE). When the image quality is limited by TTE, Contrast...

Imaging of the coronary arteries is an important part of the evaluation of children with congenital heart disease and isolated congenital coronary artery anomalies. Echocardiography remains the main imaging modality and is complemented by MRI and CT angiography in the older or difficult-to-image child. We review echocardiography, MRI, and CT angiog...

A published formula containing minimal aortic cross-sectional area and the flow deceleration pattern in the descending aorta obtained by cardiovascular magnetic resonance predicts significant coarctation of the aorta (CoA). However, the existing formula is complicated to use in clinical practice and has not been externally validated. Consequently,...

The hemodynamics of isolated large ventricular septal defect with an intact atrial septum results in greater mitral valve (MV) annular dilation and an increased incidence of mitral regurgitation (MR). MR will resolve in most patients after surgical VSD closure alone without MV repair.

Restrictive cardiomyopathy (RCM) is characterized by irreversible diastolic dysfunction with preserved systolic function. The aim of this study was to investigate the presence of impaired ventricular contractility even in the presence of normal ejection fraction (EF) in children with RCM. Longitudinal Doppler tissue velocities were obtained from ap...

Objectives To create a cardiovascular magnetic resonance (CMR) based tree algorithm for predicting coarctation (CoA) transcatheter systolic pressure gradient ≥ 20mmHg. Background A published algorithm containing minimal aortic crosssection area and the flow deceleration pattern in the descending aorta measurements obtained by CMR can predict the pr...

Background Respiratory navigated, ECG-gated, 3D-isotropic steady state free precession (3D-SSFP) is increasingly utilized in pediatric aortopathy. This is particularly true in adolescents as acoustic windows become limited. The agreement between echocardiography (ECHO) and 3D-SSFP obtained measurements cou ld be influence by imaging plane, timing (...

Left ventricular (LV) end-diastolic volume (LVEDV) can be estimated by the formula (5/6) x area x length, or the "bullet" method. The aim of this study was to determine the range of normal LVEDV values in infants and young children (aged 0-3 years) by the subxiphoid bullet method. Echocardiograms from 100 normal subjects aged ≤ 3 years were retrosp...

We report a case of a 68-year-old woman with a basal postmyocardial infarction ventricular septal defect that recurred 4 months after initial surgical repair. Successful transcatheter closure using an Amplatzer Muscular VSD Occluder was facilitated by direct percutaneous left ventricular access.

Left ventricular end-diastolic volume (LVEDV) calculation is important in decision making in children with congenital heart disease (CHD). This study examined the accuracy of LVEDV calculation by the formula: 5/6 x area x length ("bullet" method), by comparing echocardiographic (ECHO) LVEDV against cardiac magnetic resonance (CMR). ECHO and CMR dat...

Introduction Definitions Incidence Etiology Morphology and classification Pathophysiology Imaging References

The incidence of congenital coronary anomalies is mainly derived from autopsy series and pre-participation exams in athletes. Limited data exist regarding the spectrum of coronary anomalies that can be detected in asymptomatic patients. We sought to describe echocardiographically detected congenital coronary artery anomalies in asymptomatic childre...

This study evaluated the quality of the visualization of extracardiac thoracic vessels by magnetic resonance angiography (MRA) in young infants with congenital heart disease. Echocardiography is often sufficient in evaluating CHD in young infants. Cardiac catheterization is needed in some instances to evaluate extracardiac thoracic vessels. Extraca...

The physiology of isolated partially anomalous pulmonary venous connection of a single pulmonary vein has yet to be fully characterized. This study assessed the magnitude of the left-to-right shunt and right ventricular (RV) dilation from a single anomalous pulmonary vein using cardiac magnetic resonance imaging. Subjects with >1 anomalous pulmonar...

Survivors of tetralogy of Fallot (TOF) repair constitute a large and growing population of patients. Although postsurgical outcome is generally favorable, as these patients move into adulthood, late morbidity is becoming more prevalent and the notion that TOF has been "definitively repaired" is increasingly being challenged. Recent evidence suggest...

We sought to explore the relationship of color Doppler tissue imaging-derived systolic indices of tricuspid valve annular motion and magnetic resonance imaging-derived right ventricular (RV) ejection fraction in patients with congenital heart disease. Patients with congenital heart disease who underwent echocardiography and magnetic resonance imagi...

MRI is increasingly used for anatomic assessment of aortic coarctation (CoA), but its ability to predict the transcatheter pressure gradient, considered the reference standard for hemodynamic severity, has not been studied in detail. This study evaluated the ability of MRI to distinguish between mild versus moderate and severe CoA as determined by...

LEARNING OBJECTIVES To recognize the MRI appearance of Tetralogy of Fallot.To learn the common complications and long-term outcome after surgery for Tetralogy of Fallot.To understand how cardiac MRI can assist in evaluating both anatomic and functional assessment.To familiarize the radiologist with technical aspects of cardiac imaging. ABSTRACT Te...

A five-month old male with a single ventricle palliated with a bidirectional cavopulmonary anastomosis developed severe respiratory insufficiency from respiratory syncytial virus (RSV) pneumonitis. He was successfully rescued with extra-corporeal membrane oxygenation (ECMO) therapy and recovered with minimal morbidity.

To the Editor: We read with interest the article by Araoz et al1 and its description and beautiful three-dimensional (3D) images of a rare criss-cross heart. The 3D gadolinium-enhanced magnetic resonance angiography (MRA) images clearly demonstrate the complex anatomy, in particular the relationship between the crossed atrioventricular pathways. B...

To assess the reliability of ventricular septal position in predicting elevated right ventricular pressure (RVP) in patients with ostium primum atrial septal defects (ASD 1). Echocardiograms of 4 groups were retrospectively analyzed: Patients with ASD 1 and low RVP, patients with ASD 1 and high RVP, and 2 age-matched control groups: one with isolat...

The femoral vein is an important site for central venous access in newborns and infants. The objectives of this study are to determine whether age or weight can be used clinically to predict the size of the femoral vein in newborns and infants, and to compare the size of the vein in each individual in both the supine and reverse Trendelenburg posit...

Introduction: Central venous access in newborns and young infants is a challenge. Data about the size of the femoral vein in these groups is lacking. This report describes the size of the femoral vein in relation to age. weight, and position in 24 euvolemic subjects. Methods: We measured the femoral vein diameter in patients from the pediatric ward...