News Release

Experts in pediatric heart disease present research at Cardiology 2012 conference

Peer-Reviewed Publication

Children's Hospital of Philadelphia

Pediatric cardiology researchers and clinicians from almost 50 centers from across the U.S. and around the world are gathering at the Cardiology 2012 Conference sponsored by The Children's Hospital of Philadelphia on Feb. 22-26 in Orlando, Fla. The news briefs below summarize 11 research abstracts selected by the conference organizers as featured presentations. The researchers leading these presentations comprise 6 physicians and 5 nurses.

New Early Warning Algorithm Detects Children's Early Cardiac Deterioration Before Inpatient Arrest

Most cardiopulmonary arrests in hospitalized children can be prevented by early recognition and treatment for signs of deterioration. Nurse researchers at Children's Hospital Boston developed and validated the Cardiac Children's Hospital Early Warning Score (C-CHEWS) for pediatric cardiology patients. Used in conjunction with vital signs, the C-CHEWS scores assessments of patient behavior, cardiovascular and respiratory status, and level of family and/or staff concern. The resulting score is used to divide patients into one of three groups: routine care, increased assessment/intervention, or CICU consult/transfer. A retrospective review of patients admitted to the cardiac unit over a 12-month period compared 64 patients who experienced arrest or an unplanned CICU transfer with a comparison group of 248 non-event admissions. When compared with the previously validated Pediatric Early Warning Score (PEWS) tool, the C-CHEWS had greater sensitivity. Overall, C-CHEWS has excellent discrimination for identifying deterioration in children with cardiac disease. M.C. McLellan et al, Children's Hospital Boston; Validation of an Early Warning Scoring Tool for the Identification of Pediatric Cardiac Patients at Risk for Cardiopulmonary Arrest

Patients with Certain Conotruncal Defects Should Be Screened for Chromosome 22q11.2 Deletion Syndrome

A relatively common genetic disorder, chromosome 22q11.2 deletion syndrome (22q11del) can result in multiple congenital anomalies including conotruncal cardiac defects. In this single-center study at The Children's Hospital of Philadelphia, researchers tested 1,613 patients with a variety of conotruncal defects for the presence of the deletion. The study team found the 22q11del in 13 percent of patients with tetralogy of Fallot, in 35 percent of patients with truncus arteriosus, in 5 percent of patients with conoventricular septal defects, and in 56 percent of patients with interrupted aortic arch type B—suggesting that most patients with these particular anomalies should be screened for the deletion during the prenatal or neonatal period. Patients with double outlet right ventricle or transposition of the great arteries should be screened only when an arch anomaly or other syndromic feature is also present. Patients with interrupted aortic arch type A appear to have minimal risk for 22q11del. These findings help to guide cardiac imaging, prenatal and neonatal genetic testing, and counseling in the cardiac population. S. Peyvandi et al, The Children's Hospital of Philadelphia; Refining Screening Strategies for 22q11.2 Deletions in Patients with Conotruncal Defects: Data from 1618 Consecutive Cases

Most Survivors of CHD Who Had ECMO Support Have Normal Neurological Outcomes or Mild Disability at Age Two

Despite established efficacy in children with cardiac disease, reported survival after ECMO support is 40 – 55 percent while the most recent data from Extracorporeal Life Support Organization (ELSO) show a survival of 48%. ECMO survivors are at risk for significant neurologic injury and an interdisciplinary team is crucial to the prevention and follow-up. In this single-center retrospective review the authors evaluated the outcomes of 95 children with cardiac disease who required ECMO support between 2005 and 2010. Sixty-nine patients (73%) survived to hospital discharge. These patients were evaluated by the study team at Children's Hospital of Pittsburgh for neurologic abnormalities using the Pediatric Cerebral Performance Category and the Pediatric Overall Performance Category scales. Evaluation occurred at time of hospital discharge and at a median of almost 2 years follow-up. The indications for ECMO were cardiopulmonary resuscitation refractory to conventional resuscitation (E-CPR), failure to separate from cardiopulmonary bypass, and low cardiac output syndrome. Among the 69 survivors, 64 percent had cardiac surgery before ECMO, 19 percent had single ventricle, 55 percent two ventricles, 22 percent primary myocardial disease and 4 percent primary pulmonary hypertension. At time of hospital discharge 75 percent of the patients had either normal or mild neurologic disability. At the 2 year follow-up 63 patients (66 percent) were still alive and normal to only mild neurologic disability was seen in more than 80 percent of patients. Patients with E-CPR, cerebral infarct or hemorrhage and patients who needed plasma exchange had worse short-term neurologic outcome, whereas worse long-term neurologic outcome was seen only in patients who suffered cerebral infarct. The research team concluded that a sustained interdisciplinary team is vital to improved survival and improved neurologic outcomes after pediatric cardiac ECMO. C. Chrysostomou et al, Children's Hospital of Pittsburgh; Neurologic Outcomes after Extracorporeal Life Support in Children with Cardiac Disease

In Small-Sample Study, Melody Pulmonary Valve Shows Success in Young Children

The Melody Transcatheter Pulmonary Valve has received FDA approval for clinical use, but there is limited experience in implanting this device in young children. A study team from Miami Children's Hospital performed a multicenter retrospective review of all patients weighing less than 30 kg who had this device implanted for conduit dysfunction between Jan. 2008 and May 2011. The researchers analyzed data from 25 patients evaluated for the procedure with median age 8 years and median weight 21.4 kg. Valve implantation was successfully performed in 23 patients, with good hemodynamic results in the majority of patients, as measured by Doppler gradient in the conduit. Immediately following implantation, regurgitation was absent or trivial in 21 patients and mild in 2. Adverse events were common, including pulmonary artery perforation associated with pulmonary hemorrhage (2), contained conduit tear (5) and contained peripheral venous injury (6). These adverse events were self-limiting in all but one patient. The majority of patients had good procedural and early hemodynamic results. More experience and a larger sample size are needed before this procedure can be routinely recommended in young children. D.P. Berman et al, Miami Children's Hospital; Transcatheter Pulmonary Valve Implantation (TPVI) in Young Children with Dysfunctional Right Ventricular Outflow Tract Conduits: Feasibility and Early Follow-up Results

Biomarkers, Found in Blood Test, Identify Newborns at Risk for Death and Brain Injury After Heart Surgery

In newborns undergoing cardiac surgery, diagnosing early brain injury is important for timely intervention, but it is difficult to evaluate sedated, mechanically ventilated infants. This single-center study at Emory University School of Medicine evaluated the sensitivity, specificity and clinical utility of using a blood test to evaluate newborns. The study included 10 neonates undergoing cardiac surgery using cardiopulmonary bypass, with researchers performing preoperative and postoperative brain MRIs, maternal and patient blood samples for biomarkers S-100B and NSE (neuron-specific enolase), and neurodevelopmental testing between the ages of 6 and 10 months. Both S-100B and NSE were useful in identifying newborns at risk for death and abnormal neurodevelopmental outcomes after surgery. Preoperative S-100B also predicted abnormal preoperative brain MRI. The research team concluded that their results justify further study and long-term follow-up of this at-risk population. B.M. Weissman et al, Emory University School of Medicine; Biomarkers Useful in Identifying Newborns at Risk for Death and Abnormal Neurodevelopmental Outcomes Following Cardiac Surgery

Prenatal Laser Photocoagulation Improves Right Ventricle Outflow in Twin-Twin Transfusion Syndrome

Twin-twin transfusion syndrome (TTTS) is associated with abnormal growth of the pulmonary artery (PA) and right ventricular outflow tract obstruction (RVOTO) in the recipient twin. This single-center study at The Children's Hospital of Philadelphia investigated the impact of placental laser photocoagulation (LP) on PA abnormalities and RVOTO. The researchers analyzed fetal echocardiograms of 28 twin pairs with right-sided outflow abnormalities who underwent fetoscopic placental LP in 2009 and 2010. The median gestational age at LP was 20 weeks. Of the 28 recipient twins, 15 fetuses had a PA equal in size to the aorta (group 1), 5 fetuses had a PA smaller than the aorta (group 2), and 8 had frank RVOTO (group 3). Four weeks after LP, all the group 1 and group 2 fetuses had complete resolution of PA abnormalities. In group 3, one patient with "functional" pulmonary atresia had complete resolution one day after LP; five patients with RVOTO at diagnosis did not respond to LP. Overall, placental LP interrupts the pathophysiology of TTTS and improves PA abnormalities, demonstrating the unique plasticity of the human fetal heart. S.L. Wellen et al, The Children's Hospital of Philadelphia; Fetoscopic Placental Laser Photocoagulation Improves Right Ventricular Outflow Tract Obstruction in Twin-Twin Transfusion Syndrome

Home Monitoring Improved Inter-Stage Weight Gain in HLHS Infants Who Had Hybrid Procedure

For infants with hypoplastic left heart syndrome weight gain and home monitoring during the interstage (IS) period have been associated with improved survival. The purpose of this single-center study by nurse-researchers at Nationwide Children's Hospital was to describe IS weight gain and investigate home monitoring in patients undergoing the hybrid procedure. The study team performed retrospective review of 44 patients, of whom 24 received IS home monitoring, from 2006 to 2011. Eight infants died during the IS period. Home monitoring was significantly associated with improved weight gain in these patients, and infants with higher weight gain were more likely to survive the IS period. H. Miller-Tate et al, Nationwide Children's Hospital; Inter-stage Weight Gain for Patients with Hypoplastic Left Heart Syndrome Undergoing the Hybrid Procedure

New Medication Protocol Provides a Non-narcotic Option for Pain Management after Chest Tube Removal in Children

After a literature review revealed the absence of an effective pain management protocol for chest tube removal in children, nurse-researchers at Children's Healthcare of Atlanta designed a double-blind, randomized study to evaluate a new protocol. One treatment group received morphine and Versed, the other group received Toradol and Versed. The study team ended the trial early when it became apparent that pain scores were lower before and after the procedure for children receiving the Toradol and Versed combination. A quality review determined that reported pain scores remained low when a Toradol-Versed protocol was applied to the general pediatric population on the Cardiac Stepdown Unit. A.L. Beckman et al, Children's Healthcare of Atlanta; Pediatric Cardiac Patient Pain Relief Related to Chest Tube Removal: Outcomes of a Practice Change

New Pediatric Protocol Decreases Sternal Wound Infections after Cardiac Surgery

No pediatric guidelines currently address the reduction of sternal wound infections (SWI), a complication of cardiac surgery that increases morbidity, mortality and financial costs. A team of researchers at the University of Texas Health Science Center conducted a two-year prospective quality improvement project aimed at reducing the incidence of SWI. Elements of the protocol included preoperative baths with a skin disinfectant, using single patient use electrodes, attention to timing of preoperative antibiotics, and specialized care of the infant with delayed sternal closure. The study team analyzed 308 children who had sternotomies during the study period and found a reduction in all SWI over the study period; 14 SWI in the first year to 5 SWI in the 2nd year, a 61 percent decrease. Children whose sternal closure was delayed had a significantly higher overall risk of SWI; using a protocol for children with delayed sternal closure produced a lower infection rate, though not statistically significant, from 26.9% in the study's first year to 8.3 percent in the second year. These findings justify a multicenter study of a protocolized approach to SWI prevention in children. C.S. Woodward et al, University of Texas Health Science Center, San Antonio; Sternal Wound Infection Prevention in Pediatric Cardiac Surgery: A Protocolized Approach

ICU Discharge Scores Standardize the Transfer of High-Risk Shunted Newborns

Decisions to transfer neonates with shunted single ventricle physiology out of the ICU currently rely on variable criteria. Premature discharges result in unplanned readmissions and out-of-ICU cardiopulmonary arrests, while deferred transfers unnecessarily prolong the ICU stay. Nurse-researchers at Children's Medical Center Dallas developed an objective scoring system for postoperative single ventricle patients. Factoring into the discharge score were clinical variables such as post-operative echo results, weight, feeding regimen, respiratory support level, anti-platelet therapy, and family readiness. Static variables such as decreased ventricular function and arrhythmia were also taken into account. Based on a retrospective review of patient charts in 2009 and 2010, a discharge score of 10 or less predicted a cardiopulmonary arrest or unplanned readmission. Higher discharge scores predicted extended CICU stays, with the maximum score being 18. The study team proposes that a score of 12 predicts a patient is suitable for transfer from the CICU to the ward, and suggests that the discharge scoring system provides an objective method to help standardize practice for single-ventricle patients. A. Torzone-Jameson et al, Children's Medical Center, Dallas; ICU Discharge of Shunted Single Ventricle Patients: Standardizing a Moving Target via an Objective Discharge Score

Infants with HLHS Who Start Trophic Feeding Before Stage 1 Surgery Have Better Clinical Outcomes

Infants with hypoplastic left heart syndrome (HLHS) are at risk for necrotizing enterocolitis (NEC), the most common life-threatening GI condition in newborns. Multiple studies have investigated a relationship between feeding initiation, gut motility and NEC in premature infants while earlier initiation of trophic feeds has been associated with improved clinical outcomes. However, for infants with HLHS, the effect of trophic feeds (0.5 to 1 ml/hour) prior to stage 1 surgical palliation is unknown. A team of nurse-researchers at Phoenix Children's Hospital evaluated clinical outcomes in 7 infants with HLHS prior to stage 1 surgery. They found that newborns who received initial feeds before their first surgery reached full feeds more quickly after surgery than newborns not on pre-operative feeds. The early feeding group also had shorter length of hospital stay. It is unclear whether early feeding results in a more favorable clinical outcome, or if more stable patients are better able to feed preoperatively. Using a larger sample size in a future study may help to better define this relationship. R.E. Brunelle et al, Phoenix Children's Hospital; Early Initiation of Feeds Is Associated with Improved Clinical Outcomes in Infants with HLHS

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