Ashraf Harahsheh

Identified mortality risk factors associated with presentation, initial hospitalisation, and interstage period for the Norwood operation in a multi-centre registry: a report from the National Pediatric Cardiology-Quality Improvement Collaborative

INTRODUCTION Despite improvements in care following Stage 1 palliation, interstage mortality remains substantial. The National Pediatric Cardiology-Quality Improvement Collaborative captures clinical process and outcome data on infants discharged into the interstage period after Stage 1. We sought to identify risk factors for interstage mortality using these data. MATERIALS AND METHODS Patients who reached Stage 2 palliation or died in the interstage were included. The analysis was considered exploratory and hypothesis generating. Kaplan-Meier survival analysis was used to screen for univariate predictors, and Cox multiple regression modelling was used to identify potential independent risk factors. RESULTS Data on 247 patients who met the criteria between June, 2008 and June, 2011 were collected from 33 surgical centres. There were 23 interstage mortalities (9%). The identified independent risk factors of interstage mortality with associated relative risk were: hypoplastic left heart syndrome with aortic stenosis and mitral atresia (relative risk = 13), anti-seizure medications at discharge (relative risk = 12.5), earlier gestational age (relative risk = 11.1), nasogastric or nasojejunal feeding (relative risk = 5.5), unscheduled readmissions (relative risk = 5.3), hypoplastic left heart syndrome with aortic atresia and mitral stenosis (relative risk = 5.2), fewer clinic visits with primary cardiologist identified (relative risk = 3.1), and fewer post-operative vasoactive medications (relative risk = 2.2). CONCLUSION Interstage mortality remains substantial, and there are multiple potential risk factors. Future efforts should focus on further exploration of each risk factor, with potential integration of the factors into surveillance schemes and clinical practice strategies.

Pediatric Chest Pain-Low-Probability Referral: A Multi-Institutional Analysis From Standardized Clinical Assessment and Management Plans (SCAMPs®), the Pediatric Health Information Systems Database, and the National Ambulatory Medical Care Survey.

We conducted a study to assess test characteristics of red-flag criteria for identifying cardiac disease causing chest pain and technical charges of low-probability referrals. Accuracy of red-flag criteria was ascertained through study of chest pain Standardized Clinical Assessment and Management Plans (SCAMPs®) data. Patients were divided into 2 groups: Group1 (concerning clinical elements) and Group2 (without). We compared incidence of cardiac disease causing chest pain between these 2 groups. Technical charges of Group 2 were analyzed using the Pediatric Health Information System database. Potential savings for the US population was estimated using National Ambulatory Medical Care Survey data. Fifty-two percent of subjects formed Group 1. Cardiac disease causing chest pain was identified in 8/1656 (0.48%). No heart disease was identified in patients in Group 2 (P = .03). Applying red-flags in determining need for referral identified patients with cardiac disease causing chest pain with 100% sensitivity. Median technical charges for Group 2, over a 4-year period, were US2014

Recurrent Cardiology Evaluation for Innocent Heart Murmur: Echocardiogram Utilization.

775 559. Eliminating cardiac testing of low-probability referrals would save US2014

ST segment depression after Norwood/systemic-pulmonary artery shunt

3 775 182 in technical charges annually. Red-flag criteria were an effective screen for children with chest pain. Eliminating cardiac testing in children without red-flags for referral has significant technical charge savings.

High-risk single ventricle palliation in children with Down syndrome: single institution experience.

We conducted a retrospective study to identify electrocardiogram (ECG) and echocardiogram utilization among patients presenting for a follow-up cardiology evaluation with innocent heart murmur between 2012 and 2014. The 2014 echocardiogram Appropriate Use Criteria was applied. We observed high rates of ordering ECGs and echocardiograms on follow-up visits (79% and 36%); only 1 patient had an appropriate indication for echocardiogram while the rest had rarely appropriate indication. Having had an ECG done did not affect echocardiogram ordering behavior. Older patient age was the only factor associated with a higher likelihood for ordering echocardiograms on follow-up visit (odds ratio = 1.016, P = .021). In this small sample study, we noticed high rates of test utilization and low-probability utilization of echocardiogram in the recurrent evaluation of children with innocent heart murmur. A larger, multicenter prospective study to investigate patterns and drivers of test utilization in children with innocent heart murmur presenting for a follow-up cardiology visit is needed.

Beyond the Standard Lipid Profile: What is Known about Apolipoproteins, Lp(a), and Lipoprotein Particle Distributions in Children

A three-month-old girl with double inlet left ventricle (S;D;D), hypoplastic outlet ventricle, restrictive bulboventricular foramen, d-transposition of great arteries and interrupted aortic arch underwent Norwood stage 1 operation, systemic to pulmonary artery (PA) shunt and atrial septectomy, and developed ectopic atrial tachycardia that responded to digoxin therapy. She developed fussiness lasting two hours, not associated with cyanosis, and not relieved by feeding. Her pulse oximetry was 88% while breathing room air, and she had no differential blood pressure gradient. Apart from single second heart sound and a grade IV/VI ejection systolic murmur, the examination was normal. Her electrocardiogram and echocardiogram were unchanged. A Holter monitor revealed ST segment depression and T wave inversion upon increasing the heart rate beyond 140 beats/minute (Figs. 1 and 2). A cardiac catheterization showed patent systemic to PA shunt, patent native aorta, and no evidence of coarctation of aorta. After undergoing the second stage operation, her diastolic runoff and symptoms of fussiness disappeared and a surveillance Holter monitor showed better-looking ST segments and T waves even at higher heart rates (Figs. 3 and 4). Three months later, the patient is doing well and is awaiting Fontan completion. Figure 1 Absence of ST segment depression with a heart rate <140 beats/minute in a patient with Norwood/systemic to pulmonary artery shunt with diastolic runoff flow. Figure 2 ST segment depression and T wave inversion with a heart rate >140 beats/minute in a patient with Norwood/systemic to pulmonary artery shunt with diastolic runoff flow. Figure 3 Absence of ST depression with a heart rate <140 beats/minute after stage II palliation with no diastolic runoff flow. Figure 4 Absence of ST depression with a heart rate >140 beats/minute after stage II palliation with no diastolic runoff flow. Despite many advances in the field of pediatric cardiology, the interstage mortality rate remains high [1]. The use of the systemic to PA shunt in the Norwood operation has been complicated by wide pulse pressure, diastolic runoff flow [2,3], uneven distribution of pulmonary blood flow [4], and increased early mortality [5]. Providers need to keep a high index of suspicion for the adverse effects of the diastolic runoff that accompanies the presence of systemic to PA shunts.

The Impact of a Designated Cardiology Team Involving Telemedicine Home Monitoring on the Care of Children with Single-Ventricle Physiology After Norwood Palliation

BACKGROUND Of the children with Down syndrome 40-50% have cardiac defects and the majority of these cardiac defects are amenable to biventricular repair. The outcome of single ventricle palliation is improving; nonetheless, there are limited data on Down syndrome patients with associated high-risk factors undergoing single ventricle palliation. Our aim was to study the outcomes of children with Down syndrome and high-risk factors on the single ventricle palliation pathway. METHODS A retrospective study on all patients with Down syndrome on the single ventricle palliation pathway from 2005 until 2011 was conducted. Operative, clinical, echocardiographic, haemodynamic data, and follow-up data were reviewed. RESULTS A total of 310 patients underwent at least one single ventricle surgical intervention. Of those, eight patients had Down syndrome, five of which had associated risk factors - low birth weight, high pulmonary vascular resistance, pulmonary vein stenosis, significant atrioventricular valve regurgitation, and extracardiac anomalies. Mortality in the high-risk group was 80% (4/5), compared with 33% (1/3) in the non-high-risk patients. Overall, after a median follow-up period of 138 days (8-576 days), only 37.5% (3/8) of patients were alive. CONCLUSION Despite many improvements in the care of single ventricle patients, the fate of those with Down syndrome and associated high-risk factors remains poor. Further multicentre longer-term studies are needed to validate and quantify the cumulative effects of negative prognostic factors in this complex group of patients.

An Innovative Pilot Curriculum Training Pediatric Residents in Referral and Communication Skills on a Cardiology Rotation

Apolioproteins associate with lipid and phospholipid to help render them soluble within lipoproteins, determining receptor interactions and directing diverse metabolic and vascular functions. Apolipoprotein AI and apolipoprotein B (apoB) may be better predictors of atherosclerotic risk than traditional cholesterol measures. Because small, dense LDL particles each carry one signature apoB molecule, apoB levels are accurate predictors of the actual burden of LDL. Though less often considered in large CVD risk-reduction trials, the apoliprotein Cs and Es involved in triglyceride metabolism have earned increased attention with the rise in obesity and the highly atherogenic metabolic syndrome; through regulatory interactions that direct lipid metabolism, they directly influence the lipoprotein particle distribution. Finally when a distinct and highly variable apolipoprotein (a) joins apoB on LDL, an atherothrombotic lipoprotein (a) is formed that is cleared less efficiently and can further augment the LDL particle burden. These important members of the proteome are reviewed.