George T. Nicholson

Caloric intake during the perioperative period and growth failure in infants with congenital heart disease.

Infants with congenital heart disease have impaired weight gain during the first several months of life. Efforts have focused on improving weight gain and nutritional status during the first months of life. Close examination of the data suggests that the immediate postoperative period is problematic. Etiology of this early growth failure should be identified to develop effective interventions. This is a retrospective study of neonates who underwent modified systemic–to–pulmonary artery shunt, including Norwood palliation, at Children’s Healthcare of Atlanta between January 2009 and July 2011. We analyzed growth from time of surgical intervention to hospital discharge. Measures of calculated weight-for-age Z-score (WAZ score) were performed using the World Health Organization’s Anthro Software (version 3.2.2, January 2011; WHO, Geneva, Switzerland). Seventy-three patients were identified. Eight patients did not meet inclusion criteria. Complete data were collected on the remaining 65 patients. Median caloric intake patients received was 50.4 [interquartile range (IQR) 41.6 to 63.6] calories/kg/day while exclusively on parental nutrition. At hospital discharge, the median WAZ score was −2.0 (IQR −2.7 to −1.2) representing an overall median WAZ score decrease of −1.3 (IQR −1.7 to −0.7) from time of shunt palliation to hospital discharge. Despite studies showing poor weight gain in infants with congenital heart disease after neonatal palliation, this study reports the impact of hospital-based nutritional practices on weight gain in infants during the immediate postoperative period. Our data demonstrate that actual caloric intake during the cardiac intensive care unit stay is substantially below what is recommended.

Transcatheter interventions across fresh suture lines in infants and children: an 8-year experience.

The purpose of this study was to examine the outcomes of catheter dilation interventions in general upon surgical anastomotic sites in the immediate postoperative period.

Late outcomes in children with Shone's complex: a single-centre, 20-year experience.

OBJECTIVE Shones syndrome is a complex consisting of mitral valve stenosis in addition to left ventricle outflow obstruction. There are a few studies evaluating the long-term outcomes in this population. We sought to determine the long-term outcomes in our paediatric population with Shones syndrome and the factors associated with left heart growth. METHODS All patients diagnosed with Shones syndrome with biventricular circulation treated between 1978 and 2010 were reviewed. Baseline echocardiograms and data from catheterisations were also reviewed. Number of interventions (surgical+transcatheter), incidence of mitral valve replacement, and incidence of heart transplantation were tracked. Survival of the population and left heart structural growth were also reviewed. RESULTS A total of 121 patients with Shones syndrome presented at a median age of 28 days (0-17.3 years) and were followed-up for 7.2 years (0.01-35.5 years). These patients underwent 258 interventions during the study period, and the presence of coarctation was associated with repeat left heart interventions. The 10-year, transplant-free survival was 86%. Presence of pulmonary hypertension was associated with mortality. Left heart structural growth was seen for mitral and aortic valve annuli and left ventricular end-diastolic dimension over time. CONCLUSIONS Shones syndrome patients undergo a number of left heart interventions. Coarctation of the aorta is associated with an increased likelihood for repeat interventions. Survival appears to be more favourable than expected. Significant left heart growth will occur in the population. Pulmonary hypertension is associated with an increased risk of mortality.

Direct physician reporting is associated with reductions in radiation exposure in pediatric cardiac catheterizations

The objective is to quantify radiation dose in children undergoing cardiac catheterization and determine the impact of increased reporting transparency on total radiation exposure. Background: Cardiac catheterization (cath) can result in significant radiation exposure in children. There has been growing interest in quantifying and reducing radiation exposure in pediatric cath procedures. Our center underwent a slight change in practice recently that resulted in direct physician reporting of radiation dose following every case.

Transfusion-associated Babesiosis in a 7-month-old Infant after Bidirectional Glenn Procedure

We describe a case of transfusion-associated babesiosis following bidirectional Glenn procedure. This unique case highlights the importance of including babesiosis and other typically vector-borne infections in the differential diagnosis for patients with recent blood transfusions and fever without obvious source.

Outcomes After Decompression of the Right Ventricle in Infants With Pulmonary Atresia With Intact Ventricular Septum Are Associated With Degree of Tricuspid Regurgitation: Results From the Congenital Catheterization Research Collaborative

Background— Outcomes after right ventricle (RV) decompression in infants with pulmonary atresia with intact ventricular septum vary widely. Descriptions of outcomes are limited to small single-center studies. Methods and Results— Neonates undergoing RV decompression for pulmonary atresia with intact ventricular septum were included from 4 pediatric centers. Primary end point was reintervention post-RV decompression; secondary end points included circulation type at latest follow-up. Ninety-nine patients (71 with pulmonary atresia with intact ventricular septum and 28 with virtual atresia) underwent RV decompression at median 3 (25th–75th, 2–5) days of age. Seventy-one patients (72%) underwent at least 1 reintervention after decompression. Median duration of follow-up was 3 years (range, 1–10). Freedom from reintervention was 51% at 1 month and 23% at 3 years. In multivariable analysis, reintervention was associated with virtual atresia (hazard ratio [HR], 0.51; 95% confidence interval [CI], 0.28–091; P=0.027), smaller RV length (HR, 0.94; 95% CI, 0.89–0.99; P=0.027), and ⩽mild tricuspid regurgitation (TR; HR, 3.58; 95% CI, 2.04–6.30; P<0.001). Patients undergoing surgical shunt or ductal stent were less likely to have virtual atresia (HR, 0.36; 95% CI, 0.15–0.85; P=0.02) and more likely to have higher RV end-diastolic pressure (HR, 1.07; 95% CI, 1.00–1.15; P=0.057) and ⩽mild TR (HR, 3.50; 95% CI, 1.75–7.0; P<0.001). Number of reinterventions was associated with ⩽mild TR (rate ratio, 1.87; 95% CI, 1.23–2.87; P=0.0037). Multivariable analysis indicated that <2-ventricle circulation status was associated with ⩽mild TR (odds ratio, 18.6; 95% CI, 5.3–65.2; P<0.001) and lower RV area (odds ratio, 0.81; 95% CI, 0.72–0.91; P<0.001). Conclusions— Patients with pulmonary atresia with intact ventricular septum deemed suitable for RV decompression have a high reintervention burden although most achieve 2-ventricle circulation. TR ⩽mild at baseline is strongly associated with reintervention and <2-ventricle circulation at medium-term follow-up. Degree of baseline TR may be an important marker of long-term outcomes in this population.

Tissue Plasminogen Activator Use in Children: Bleeding Complications and Thrombus Resolution.

OBJECTIVE To review our institutional experience with tissue plasminogen activator (tPA) to determine outcomes related to bleeding complications and thrombus resolution. STUDY DESIGN We performed a retrospective review of all patients who received systemic tPA for thrombolysis. Data points included location of thrombus, initial and maximum tPA dose, and duration of tPA. The primary endpoint was bleeding complication. RESULTS Between 2005 and 2014, 46 patients received systemic tPA for thrombolysis: 17 (37%) were patients with a primary cardiac diagnosis, there were 17 (37%) hematology/oncology patients, and 12 (26%) patients with noncardiac, nonhematology/oncology diagnoses. The indication for tPA was central venous thrombus (n = 23), pulmonary artery thrombus (n = 9), and cardiac or aortic thrombus (n = 14). Bleeding complications occurred in 15 patients (33%). Median initial tPA dose in the bleeding complication group was 0.10 mg/kg/h vs 0.03 mg/kg/h in the group without bleeding complication group (P = .01). Cardiac patients experienced more bleeding complications (P = .01). Multivariate analysis indicated that dose of tPA (P = .01) and diagnostic category (P < .01) were associated with bleeding complication. Complete thrombus resolution occurred in 21 patients, partial in 10 patients, and no resolution in 15 patients. Complete resolution of thrombus was not associated with diagnosis, thrombus location, tPA dose, or duration. CONCLUSIONS Cardiac patients appear to be at highest risk of bleeding complication; bleeding complications were associated with higher doses of tPA, and cardiac patients were the cohort who received the highest doses of tPA. Higher tPA doses are associated with increased risk of bleeding complication but are not associated with successful thrombus resolution.

Cardiac Catheterization in the Early Post-Operative Period After Congenital Cardiac Surgery

OBJECTIVES This study sought to demonstrate that early cardiac catheterization, whether used solely as a diagnostic modality or for the use of transcatheter interventional techniques, can be used effectively and with an acceptable risk in the post-operative period. BACKGROUND Cardiac catheterization offers important treatment for patients with congenital heart disease. Early post-operative cardiac catheterization is often necessary to diagnose and treat residual anatomic defects. Experience with interventional catheterization to address post-operative concerns is limited. METHODS This was a retrospective cohort study. The medical and catheterization data of pediatric patients who underwent a cardiac catheterization ≤30 days after congenital heart surgery between November 2004 and July 2013 were reviewed. Patients who underwent right heart catheterization and endomyocardial biopsy after heart transplantation were excluded. RESULTS A total of 219 catheterizations (91 interventional procedures, 128 noninterventional catheterizations) were performed on 193 patients. Sixty-five interventions (71.43%) were dilations, either balloon angioplasty or stent implantation. There was no difference in survival to hospital discharge between those who underwent an interventional versus noninterventional catheterization (p = 0.93). One-year post-operative survival was comparable between those who underwent an intervention (66%) versus diagnostic (71%) catheterization (p = 0.58). There was no difference in the incidence of major or minor complications between the interventional and diagnostic catheterization cohorts (p = 0.21). CONCLUSIONS Cardiac catheterization, including transcatheter interventions, can be performed safely in the immediate post-operative period after congenital heart surgery.

Is there a benefit to postoperative fluid restriction following infant surgery

OBJECTIVE Fluid restriction is often employed immediately following cardiac surgery in children. The goal of this approach is to achieve an early negative fluid balance, which theoretically should lead to less interstitial edema and earlier extubation. The purpose of this study was to determine whether time to negative fluid balance in infants after undergoing systemic-to-pulmonary artery shunt palliation impacts duration of mechanical ventilation and hospital length of stay. DESIGN This is a retrospective study of neonates who underwent a modified systemic-to-pulmonary artery shunt at a single institution. SETTING University hospital pediatric cardiac intensive care unit (CICU). PATIENTS Neonates who underwent a modified systemic-to-pulmonary artery shunt between January 1, 2009 and June 1, 2011. OUTCOME MEASURES Information collected included time to negative fluid balance (in hours), CICU and hospital length of stay (in days), and the number of patients who had delayed sternal closure and/or underwent cardiopulmonary bypass. RESULTS Data were available for 65 subjects. Median fluid administration in the 24 hours postoperatively was 43.9 cc/kg/day (interquartile range: 32.9-61.0). Mean time to negative fluid balance was 25.0 ± 12.8 hours. Time to negative fluid balance was not associated with time to extubation, CICU and hospital length of stay, or change in weight-for-age z-score at intensive care unit discharge. CONCLUSION Time to negative fluid balance is not associated with duration of mechanical ventilation, CICU, and hospital length of stay in patients after undergoing systemic-to-pulmonary artery shunt palliation. The utility of a restricted fluid strategy immediately following infant heart surgery is questionable.

Pulmonary Hypertension in Kawasaki Disease

This report describes the case of two pediatric patients who demonstrated echocardiographic evidence of pulmonary hypertension (PH) during the acute phase of Kawasaki disease. The etiology of PH development in this setting is currently unknown, but the authors hypothesize that pulmonary vasculitis may play a significant role. Fortunately, the PH appeared to be self-limited and resolved in both cases with routine treatment of Kawasaki disease.