Nucleated Red Blood Cell Counts Differentiate Cardiac from Respiratory Causes of Cyanosis at Birth.

Tissue hypoxia increases erythropoietin production and release of immature erythrocytes that can be measured using nucleated red blood cell counts (nRBC). We hypothesized that hypoxia due to congenital heart disease (CHD) is chronic and is better tolerated than hypoxia due to respiratory disease (RD), which is an acute stress in newborns leading to higher nRBC. This study assesses the utility of nRBC as a marker to differentiate hypoxia due to CHD vs RD in term neonates.

Child Health Needs and the Pediatric Cardiology Workforce: 2020-2040.

This article evaluates the pediatric cardiology (PC) workforce and forecasts its future supply. Produced as part of a supplement in Pediatrics, this effort represents a collaboration among the American Board of Pediatrics Foundation, the University of North Carolina at Chapel Hill's Carolina Health Workforce Research Center, the Strategic Modeling and Analysis Ltd., and members of the pediatric subspecialty community.

Blood Pressure Response to Treadmill Cardiopulmonary Exercise Test in Children with Normal Cardiac Anatomy and Function.

Objectives: To describe blood pressure (BP) responses during cardiopulmonary exercise testing (CPET) and propose criteria for abnormal BP responses in children with normal hearts.

Study Design: Treadmill CPET data of children <18 years with normal hearts were retrospectively analyzed to evaluate BP responses to exercise at the Children's Hospital of Michigan over an 8-year period (2011-2018).

Results: Our cohort included 1085 children (boys, 59%) aged 7-17 years.

Optimal criteria for transcatheter closure of Fontan fenestration: a single-center experience with a review of literature.

Fenestration closure is considered to remove the persistent right-to-left shunt after the Fontan operation. However, the criteria for effective transcatheter closure of fenestration to avoid both acute and chronic Fontan failure have not been clarified. The objective of this study was to describe the hemodynamic data with test occlusion of the Fontan fenestration for patients who underwent a closure and those who did not, along with subsequent development of Fontan-associated diseases (FAD) at follow-up.

Cardiac catheterization for hemoptysis in a Children's Hospital Cardiac Catheterization Laboratory: A 15 year experience.

Objectives: The aim of this study was to evaluate the diagnostic utility of cardiac catheterization and the efficacy of transcatheter intervention in patients with hemoptysis.

Background: Cardiac catheterization may play a role in identifying the etiologies of hemoptysis with the potential for transcatheter intervention.

Methods: This was a retrospective study of all the patients who were brought to the pediatric cardiac catheterization laboratory for the indication of hemoptysis over a 15-year period (2006-2020).

Reducing radiation exposure by lowering frame rate in children undergoing cardiac catheterization: A quality improvement study.

Introduction: Reduction of radiation dosage in the pediatric cardiac catheterization laboratory (PCL) is important to reduce the risk of its stochastic effect in children with congenital heart disease. Lowering the frame rate would reduce radiation dosage possibly at the expense of image quality, potentially resulting in higher fluoroscopic time and procedural complication rate.

Methods: The data were retrospectively analyzed in three eras: era 1 (n = 234), cineangiography 30 frames/sec (f/s) and fluoroscopy 15 pulse/sec (p/s); era 2 (n = 381), cineangiography 30 f/s and fluoroscopy 6 p/s; and era 3 (n = 328), cineangiography 15 f/s and fluoroscopy 6 p/s.

Noninvasive Echocardiographic Measures of Pulmonary Vascular Resistance in Children and Young Adults with Cardiomyopathy.

Background: Patients with cardiomyopathy (CM) are at increased risk for pulmonary hypertension (PH). Data are lacking on the use of noninvasive PH measures by echocardiography in patients with CM. The aim of this study was to evaluate the correlation between Doppler-derived echocardiographic indices and catheterization-based measurement of pulmonary vascular resistance (PVR) in children and young adults with CM.

NT-pro BNP-A marker for worsening respiratory status and mortality in infants and young children with pulmonary hypertension.

Aim: To evaluate predictors of morbidity and mortality in pediatric patients with pulmonary hypertension (PH), laboratory and echocardiographic measures of PH were analyzed.

Methods: A retrospective review of all infants and children < 2 years of age with PH from January 2011 to August 2016 was conducted. Correlations were determined using Spearman's rank correlation coefficients.

Large pseudoaneurysm following right ventricle to pulmonary artery homograft placement in an infant.

Pseudoaneurysm (PSA) is a known but rare complication of the right ventricle to pulmonary artery (RV-PA) conduits. The patient's clinical presentation can be variable ranging from asymptomatic to potential rupture. We describe an unusual case of a massive PSA in an infant who underwent RV-PA pulmonary homograft placement after relief of right ventricular outflow tract obstruction.

Treatment of Refractory Infantile Hemangiomas and Pulmonary Hypertension With Sirolimus in a Pediatric Patient.

Infantile hemangioma is a benign vascular neoplasm that spontaneously involutes over time. Management, when needed, consists of medications, laser treatment and surgical excision. We describe a 3-year-old girl who presented shortly after birth with diffuse cutaneous hemangiomas, hepatosplenomegaly with liver lesions, anemia, and acute heart failure.

Reversal of severe mitral regurgitation by device closure of a large patent ductus arteriosus in a premature infant.

We report a critically ill premature infant with severe mitral valve regurgitation associated with pulmonary hypertension and a severely dilated left atrium from a large patent ductus arteriosus. The mitral valve regurgitation improved significantly with normalisation of left atrial size 4 weeks after percutaneous closure of the patent ductus arteriosus. This case highlights the potential reversibility of severe mitral valve regurgitation with treatment of an underlying cardiac shunt.

The Long Way to a Successful Medical Therapy of Heart Failure with Beta-blockers in Children with Heart Disease.

Heart failure remains the main cause of death in children with heart disease. In USA and Europe hospital mortality of children with heart failure is about 7% of children, nearly twice as high as in adults. In this review a group of authors report about their experience with beta-blockers in childhood heart failure.

Severity of mitral regurgitation predicts risk of death or cardiac transplantation in children with idiopathic dilated cardiomyopathy.

Clinical outcomes among children with idiopathic dilated cardiomyopathy (IDC) are diverse, which makes the decision as to when a patient should be listed for a cardiac transplantation challenging. This study aimed to determine echocardiographic and clinical variables that can help clinicians identify those at highest risk for death or cardiac transplantation. The study was a single-center, retrospective chart review of children with IDC.

Pulmonary vein stenosis with Down syndrome: a rare and frequently fatal cause of pulmonary hypertension in infants and children.

Down syndrome (DS) patients are prone to pulmonary hypertension (PHTN) due to various cardiopulmonary causes. However, the association of DS with pulmonary vein stenosis (PVS) is not adequately described. We illustrate three cases from our center and an additional 13 cases from an extensive review of the literature of DS patients with PHTN and PVS.