Pulmonary Venous Gradients in Healthy Controls and Following the Repair of Total Anomalous Pulmonary Venous Return.

The percent of children who can achieve a normal and physiologic pulmonary venous gradient and flow following the repair of Total Anomalous Pulmonary Venous Return (TAPVR) is not known. Pulmonary venous confluence gradients from infants with supra-, infra-, or mixed TAPVR, repaired using a direct anastomotic connection were measured. Data from age, weight, and gender-matched controls established the normal pulmonary venous gradient range (0.

Neurodevelopmental outcomes after neonatal cardiac surgery: Role of cortical isoelectric activity.

Objectives: Neonates with congenital heart disease are at risk for impaired neurodevelopment after cardiac surgery. We hypothesized that intraoperative EEG activity may provide insight into future neurodevelopmental outcomes.

Methods: Neonates requiring surgery had continuous intraoperative EEG and hemodynamic monitoring.

Decreased incidence of supravalvar pulmonary stenosis after arterial switch operation.

Background: Supravalvar pulmonary stenosis (SVPS) is frequently observed after arterial switch. Traditionally the coronary arteries are removed from the neopulmonic root by excising the entire sinus of Valsalva. As a result, reconstruction of the neopulmonic root requires a pericardial patch encompassing two-thirds of the anastomosis between the neopulmonic root and pulmonary artery.

Surgical strategy for aortic coarctation repair resulting in physiologic arm and leg blood pressures.

Objective: The goal of aortic coarctation repair is laminar aortic blood flow resulting in a negative or absent arm:leg blood pressure (BP) gradient. Despite satisfactory relief of coarctation, associated arch hypoplasia can result in residual obstruction and postoperative upper body hypertension.

Intervention: We devised a surgical strategy to create a tension-free anastomosis with a diameter as large as both the adjacent proximal and distal aorta using a radically extended end-to-end anastomosis via sternotomy and/or thoracotomy.

Carpentier edwards porcine valved conduit for right ventricular outflow tract reconstruction.

Background: The optimal conduit for right ventricular outflow tract (RVOT) reconstruction is uncertain, with varying degrees of longevity reported for pericardial, homograft, and xenograft valves utilized in this position.

Methods: A retrospective review of children and adults with congenital heart disease who underwent RVOT reconstruction with the Carpentier Edwards™ (CE) porcine valved conduit was conducted from 2001 to 2009 at the University of Rochester and SUNY Upstate Medical Centers. Clinical data were analyzed for each subject according to conduit size, and all of the Doppler derived transconduit gradients from postoperative echocardiograms were analyzed.

A simple method of measuring thoracic aortic pulse wave velocity in children: methods and normal values.

Background: The objective of this study was to define a simple method of measuring thoracic aortic pulse-wave velocity (PWV) using only an aortic length regression equation based on the subject's height and two pulsed Doppler recordings of the aorta.

Methods: The thoracic aortas of 80 subjects (age range, 0-20 years) were measured retrospectively by direct echocardiographic visualization. A simple linear regression equation for thoracic aortic length on the basis of height was derived.

Left ventricular mass index in children with white coat hypertension.

Objectives: To determine whether children with white coat hypertension (WCH) have evidence of target-organ damage by comparing the left ventricular mass index (LVMI) of subjects with WCH with that of matched normotensive and hypertensive controls.

Study Design: Each subject in the WCH group was matched by body mass index (BMI; +/- 10%), age (+/- 1 year), and sex to a normotensive control and a hypertensive control. Echocardiograms were reviewed to determine the LVMI for each subject.

Young-Simpson syndrome (YSS), a variant of del(1)(p36) syndrome?

The Young-Simpson syndrome (YSS) and 1p36 deletion syndrome are both characterized by facial and heart abnormalities, congenital hypothyroidism, and severe growth and developmental retardation. However, the YSS is characterized by the presence of blepharophimosis and epicanthus inversus, findings not described in monosomy 1p36 patients. We describe a girl with YSS, who presented with the typical facial findings, global retardation, congenital hypothyroidism, and congenital dilated cardiomyopathy.

Effects of childhood primary hypertension on carotid intima media thickness: a matched controlled study.

To determine whether carotid intima media thickness is increased in children with primary hypertension, the current study compared carotid intima media thickness in hypertensive children with that of normotensive control subjects matched closely for body mass index and determined the relationship between carotid intima media thickness and hypertension severity determined by ambulatory blood pressure monitoring. Children with newly diagnosed office hypertension (n=28) had carotid intima media thickness, left ventricular mass index, and ambulatory blood pressure monitoring performed. Carotid intima media thickness was performed in normotensive control subjects (n=28) matched pairwise to hypertensive subjects for age (+/-1 year), gender, and body mass index (+/-10%).