Echocardiography evaluation of neonatal vein of Galen aneurysmal malformation.

Background: In neonatal vein of Galen aneurysmal malformation, vein of Galen aneurysmal malformation echocardiography remains the mainstay for early detection and explains various haemodynamic changes occurring due to a large systemic arterio-venous shunt. However, there is limited evidence of echocardiography in risk stratifying neonatal vein of Galen aneurysmal malformation vein of Galen aneurysmal malformation. The objective of this study was to identify echocardiographic parameters that could be associated with major outcomes and guide timing of neuro-intervention.

Vein of Galen Aneurysmal Malformation with Anomalous Right Superior Vena Cava to the Left Atrium Leading to Atypical Clinical and Echocardiographic Findings.

Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular malformation which typically presents in a critically ill newborn with intractable heart failure, severe pulmonary hypertension, and right heart dilation. We report two unusual cases of neonates with VGAM and anomalous connection of right superior vena cava to the left atrium. Both neonates were diagnosed with VGAM in utero and were clinically stable after birth with dilation of the left atrium and left ventricle and no evidence of pulmonary hypertension.

Developmental changes of the fetal and neonatal thyroid gland and functional consequences on the cardiovascular system.

Thyroid hormones play an important role in the development and function of the cardiac myocyte. Dysregulation of the thyroid hormone milieu affects the fetal cardiac cells via complex molecular mechanisms, either by altering gene expression or directly by affecting post-translational processes. This review offers a comprehensive summary of the effects of thyroid hormones on the developing cardiovascular system and its adaptation.

Vein of Galen aneurysmal malformation: rationalizing medical management of neonatal heart failure.

Neonates who present in high output heart failure secondary to vein of Galen aneurysmal malformation can be difficult to manage medically due to the complex physiology that results from the large shunt through the malformation. Though the cardiac function is often normal, right ventricular dilation, severe pulmonary hypertension, and systemic steal can result in inadequate organ perfusion and shock. This report recommends medical management for stabilization of neonates prior to definitive management with endovascular embolization.

Management outcomes of primary pulmonary vein stenosis.

Objectives: Primary pulmonary vein stenosis (PPVS) is increasingly diagnosed in children with no prior pulmonary vein intervention history, and management is challenging. We describe characteristics of patients who underwent surgical repair of PPVS at our center, and examine factors associated with treatment failures.

Methods: A retrospective review of all patients who underwent surgical intervention for PPVS (2002-2016) was completed.

Long-Term Survival of Patients With Coarctation Repaired During Infancy (from the Pediatric Cardiac Care Consortium).

Patients who undergo coarctation repair during infancy have excellent early survival but long-term survival is unknown. We aimed to describe the long-term survival of patients with coarctation repaired during infancy and determine predictors of mortality. We performed a retrospective cohort study using data from the Pediatric Cardiac Care Consortium for patients with coarctation who underwent surgical repair before 12 months of age between 1982 and 2003.

Rapid Cycle Deliberate Practice Versus Reflective Debriefing for Pediatric Septic Shock Training.

Objectives: Rapid cycle deliberate practice is a simulation training method that cycles between deliberate practice and directed feedback to create perfect practice; in contrast to reflective debriefing where learners are asked to reflect on their performance to create change. The aim of this study is to compare the impact of rapid cycle deliberate practice versus reflective debriefing training on resident application and retention of the pediatric sepsis algorithm.

Design: Prospective, randomized-control study.

Reintervention Is Associated With Improved Survival in Pediatric Patients With Pulmonary Vein Stenosis.

Objectives: The aim of this study was to evaluate survival following catheter intervention in pediatric patients with pulmonary vein stenosis (PVS).

Background: Despite aggressive surgical and catheter intervention on PVS in children, recurrence and progression of stenosis can lead to right heart failure and death. Clinicians continue to seek effective treatment options for PVS.

Organ damage in high-risk patients with systemic and incomplete lupus syndromes.

The objective of this study was to characterize organ damage in lupus patients enrolled in Dallas Regional Autoimmune Disease Registry (DRADR). Retrospective chart review was carried out on 99 patients with four or more diagnostic criteria for systemic lupus erythematosus (SLE) and 15 with less than four of these criteria, who were designated as having incomplete lupus erythematosus (ILE). The majority of patients (84 %) were African American or Hispanic/Latino; mean disease duration was 9.