Magnetic resonance imaging of the right ventricle in pediatric pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) causes changes in the right ventricle (RV), affecting RV size and function, ultimately leading to death. These changes have been evaluated by cardiac MRI (CMR) in adults with PAH, but not in children. Using CMR in pediatric patients with PAH, we examined how RV size and function (1) compare to normal data, (2) change over time, and (3) compare to similar studies in the adult population.

Interstudy variability in cardiac magnetic resonance imaging measurements of ventricular volume, mass, and ejection fraction in repaired tetralogy of Fallot: a prospective observational study.

Purpose: To assess the interstudy variability of cardiac magnetic resonance imaging (CMR) parameters of ventricular size and function in repaired tetralogy of Fallot (TOF).

Materials And Methods: Patients with TOF (n = 30, median age 23.5 years, 43% male) were enrolled prospectively.

Right atrial hemangioma in the newborn: Utility of fetal imaging.

We present a rare primary right atrial tumor diagnosed in-utero with fetal echocardiography, and further characterized as a congenital hemangioma with magnetic resonance imaging. Surgical resection was done six days after birth. This case illustrates the complementary roles of evolving advanced imaging techniques for fetuses and infants with congenital heart disease that allows for surgery early in the neonatal period.

Hypertrophic cardiomyopathy in a neonate associated with nemaline myopathy.

Nemaline myopathy is a congenital nonprogressive skeletal muscle disorder with a characteristic rod body formation in the skeletal muscle fibers. Cardiac involvement in nemaline myopathy is rare, although both dilated and hypertrophic cardiomyopathy have been reported. We describe an infant diagnosed with hypertrophic cardiomyopathy and hypotonia on the first day of life.

Congenital arteriovenous malformation between the subclavian artery and superior vena cava presenting in neonatal heart failure.

Congenital arteriovenous malformations are a well-described cause of neonatal heart failure. Fistulous connections are typically intrahepatic or intracranial. We present a case of a neonate with an intrathoracic arteriovenous malformation between the subclavian artery and superior vena cava resulting in florid neonatal heart failure.

Oxygen supplementation is helpful for the echocardiographic detection of anomalous left coronary artery from the pulmonary artery.

Background: The echocardiographic diagnosis of anomalous left coronary artery from the pulmonary artery (ALCAPA) can be challenging. The aim of this study was to assess the hypothesis that diagnosis can be enhanced by using supplemental oxygen, which decreases pulmonary vascular resistance and increases retrograde flow from the coronary artery into the pulmonary artery.

Methods: Demographic, echocardiographic, and cardiac catheterization data were reviewed in patients presenting with ALCAPA from 1999 to 2007.

Long-term outcomes with ambrisentan monotherapy in pulmonary arterial hypertension.

Background: This study evaluated long-term outcomes in patients with pulmonary arterial hypertension (PAH) undergoing treatment with ambrisentan monotherapy, a selective oral endothelin-1 receptor antagonist.

Methods And Results: Patients who participated in the Ambrisentan in Pulmonary Arterial Hypertension: A Phase 3, Randomized, Double-Blind, Placebo-Controlled Multicenter Efficacy Study (ARIES-1) clinical trial and extension phase at our institution were included. Cardiac catheterization, 6-minute walk distance (6MWD), and cardiac magnetic resonance (MRI) data were retrospectively reviewed.

Patent ductus arteriosus in micropreemies and full-term infants: the relative merits of surgical ligation versus indomethacin treatment.

Background/purpose: Treatment of ductus arteriosus is controversial. The merits and timing of ductus ligation versus indomethacin treatment were compared.

Methods: Study parameters for infants with PDA (June 1988 through March 2001) included age, weight, physical findings, echocardiograph, success/complications of treatment, and length of stay (LOS).

Laparoscopic appendectomy: An unnecessary and expensive procedure in children?

Background/purpose: Laparoscopic Appendectomy (LA) is a safe procedure in adults resulting in shorter hospitalization and sooner return to activity. The relative merits of LA and open appendectomy (OA) are evaluated in this first prospective and randomized study in children.

Methods: A total of 129 children with appendicitis were included.