Joseph Mahgerefteh

Use of the Melody™ transcatheter pulmonary valve as a "covered stent" to repair conduit perforation during pulmonary valve implantation

Right ventricular to pulmonary artery conduit tear after balloon dilation is a recognized potential complication, which can, in theory, be treated with a covered stent. We describe use of the Melody™ valve as a so‐called “covered” stent for containment of bleeding from RV‐PA conduit tear.

Relationship of Echocardiographic Z Scores Adjusted for Body Surface Area to Age, Sex, Race, and EthnicityCLINICAL PERSPECTIVE: The Pediatric Heart Network Normal Echocardiogram Database

Background— Published nomograms of pediatric echocardiographic measurements are limited by insufficient sample size to assess the effects of age, sex, race, and ethnicity. Variable methodologies have resulted in a wide range of Z scores for a single measurement. This multicenter study sought to determine Z scores for common measurements adjusted for body surface area (BSA) and stratified by age, sex, race, and ethnicity. Methods and Results— Data collected from healthy nonobese children ⩽18 years of age at 19 centers with a normal echocardiogram included age, sex, race, ethnicity, height, weight, echocardiographic images, and measurements performed at the Core Laboratory. Z score models involved indexed parameters (X/BSA&agr;) that were normally distributed without residual dependence on BSA. The models were tested for the effects of age, sex, race, and ethnicity. Raw measurements from models with and without these effects were compared, and <5% difference was considered clinically insignificant because interobserver variability for echocardiographic measurements are reported as ≥5% difference. Of the 3566 subjects, 90% had measurable images. Appropriate BSA transformations (BSA&agr;) were selected for each measurement. Multivariable regression revealed statistically significant effects by age, sex, race, and ethnicity for all outcomes, but all effects were clinically insignificant based on comparisons of models with and without the effects, resulting in Z scores independent of age, sex, race, and ethnicity for each measurement. Conclusions— Echocardiographic Z scores based on BSA were derived from a large, diverse, and healthy North American population. Age, sex, race, and ethnicity have small effects on the Z scores that are statistically significant but not clinically important.

Late Onset Ictal Asystole in Refractory Epilepsy

Ictal asystole is a cardiac phenomenon associated with epileptic seizures, and may play a role in sudden unexplained death in epilepsy. We present a 17-year-old boy with chronic intractable epilepsy and a vagus nerve stimulator who developed ictal asystole many years after the onset of epilepsy. The asystole was not linked to the vagus nerve stimulator, and ultimately necessitated the placement of a cardiac pacemaker. A cardiac pacemaker and vagus nerve stimulator can be safely used simultaneously after careful testing during placement. The onset of asystolic events many years after the onset of epilepsy suggests that repeated seizures may exert long-term effects on cardiac function.

A new “tool” for transcatheter atrial defect closure: The St. Jude SL2™ sheath

Orienting the left atrial disc of an atrial septal defect (ASD) closure device parallel to the septum is important, particularly in cases of ASD with deficient retroaortic rim. Standard vascular sheaths can make this challenging. We report the use of an electrophysiologic sheath, the SL2™ St. Jude Vascular sheath (St. Jude Medical, St. Paul, MN) for closure of ASD.

Symmetric ambulatory arterial stiffness index in the young

The ambulatory arterial stiffness index (AASI) and the symmetric ambulatory arterial stiffness index (s-AASI) have been shown to correlate to arterial stiffness in adults. This study assesses these indices with anthropometric and blood pressure (BP) measures in children. A total of 102 children at a pediatric hypertension clinic who had ambulatory blood pressure monitoring (ABPM) done from 2009 to 2013 were included (75% males, 7-22yo, 47% hypertensive, 24% prehypertensive, and 34% white-coat hypertensives). AASI is 1 minus the regression slope of diastolic BP values on systolic BP values from a 24-hour ambulatory blood pressure monitoring. s-AASI is the symmetric regression of AASI. Obese patients had a significantly higher AASI. s-AASI correlated with systolic BP variability. In multivariable regression, BP variability independently correlated with AASI and s-AASI. s-AASI is related to systolic dipping.AASI and s-AASI are highly dependent on BP variability in children. Further studies are necessary to assess their utility.

The Last Minute Review

Last minute review tables designed for a quick review before the exam; it helps the learner self-test their ability to the correct or most likely diagnosis and patient management in a very short time. Pediatric board study guide will offer further clarifications and additional information related to the topics on the last minute review section.

Echocardiography as a Marker of Cardiac end Organ Injury at a Young age

Coronary heart disease and stroke are two leading causes of morbidity and mortality. Risk factors have included obesity, metabolic syndrome, hypertension, and diabetes mellitus, and they appear to influence left ventricular mass, geometry, and systolic and diastolic function as well as left atrial size. Echocardiography is a non-invasive tool to assess these effects in children and should be utilized as a preventive as well as a diagnostic modality, as measurements from echocardiography may define an intermediate phenotype between risk and clinical endpoints. Information regarding the pathophysiologic mechanisms of the echocardiographic changes, the risk for future cardiovascular morbidity and mortality, and the effects of intervention can be derived from research.