Patient Driven EKG Device Performance in Adults with Fontan Palliation.

The aim of this study was to evaluate the accuracy of the KardiaMobile (KM) device in adults with a Fontan palliation, and to assess the KM function as a screening tool for atrial arrhythmias. While patient driven electrocardiogram (EKG) devices are becoming a validated way to evaluate cardiac arrhythmias, their role for patients with congenital heart disease is less clear. Patients with single ventricle Fontan palliation have a high prevalence of atrial arrhythmias and represent a unique cohort that could benefit from early detection of atrial arrhythmias.

Creatinine versus cystatin C to estimate glomerular filtration rate in adults with congenital heart disease: Results of the Boston Adult Congenital Heart Disease Biobank.

Background: Glomerular filtration rate is a key physiologic variable with a central role in clinical decision making and a strong association with prognosis in diverse populations. Reduced estimated glomerular filtration rate (eGFR) is common among adults with congenital heart disease (ACHD).

Methods: We conducted a prospective cohort study of outpatient ACHD ≥18 years old seen in 2012-2017.

Right ventricular performance using myocardial deformation imaging in infants with bronchopulmonary dysplasia.

Objective: Right ventricular (RV) performance among infants with bronchopulmonary dysplasia (BPD) remains poorly understood. We tested the hypothesis that myocardial deformation imaging (MDI) strain and strain rate would allow for differentiation between infants with severe and milder forms of BPD, independent of tissue Doppler imaging (TDI) and superior to conventional echocardiographic measurements.

Study Design: Infants with various severities of BPD (11 with none or mild, 13 with moderate and 10 with severe) underwent conventional echocardiography, TDI and MDI assessments at >36 weeks of corrected gestational age.

Location matters: left heart obstruction in pregnancy.

Objectives: Left heart obstruction in pregnancy is associated with higher rates of morbidity/mortality. The primary aim of this study was to evaluate maternal cardiovascular, obstetric, and fetal/infant events in pregnant women with left heart obstruction.

Study Design: Pregnant women with current or repaired left heart obstruction were retrospectively analyzed (2000-2014): mitral stenosis, left ventricular outflow tract obstruction (subvalvar, valvar, supravalvar), and coarctation of the aorta.

Newer inotropes in pediatric heart failure.

In the cardiac setting in pediatrics, inotropic support is often employed based on the extrapolation from adult studies, the underlying pathophysiology, pharmacodynamics of inotropes, and anecdotal experience. Large pediatric critical care studies involving inotropic support are rare. Levosimendan, a calcium-sensitizing agent with inotropic and lusitropic properties, is a pyridazole dinitrate derivative with linear pharmacokinetics and a relatively short half life, although an active metabolite, namely, OR-1876, has a half life of 70-80 hours accounting for a prolonged effect.

Acute dilated cardiomyopathy in a patient with deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase.

Deficiency of long-chain 3-hydroxyacyl-coenzyme A (CoA) dehydrogenase (LCHADD) is a rare inborn error of metabolism. It is associated with hypertrophic cardiomyopathy and less frequently with dilated cardiomyopathy. The incidence and pathophysiology of cardiac involvement in LCHADD is poorly understood.