Kathleen E. Carberry

Prospective Trial of a Pediatric Ventricular Assist Device

BACKGROUND Options for mechanical circulatory support as a bridge to heart transplantation in children with severe heart failure are limited. METHODS We conducted a prospective, single-group trial of a ventricular assist device designed specifically for children as a bridge to heart transplantation. Patients 16 years of age or younger were divided into two cohorts according to body-surface area (cohort 1, <0.7 m(2); cohort 2, 0.7 to <1.5 m(2)), with 24 patients in each group. Survival in the two cohorts receiving mechanical support (with data censored at the time of transplantation or weaning from the device owing to recovery) was compared with survival in two propensity-score-matched historical control groups (one for each cohort) undergoing extracorporeal membrane oxygenation (ECMO). RESULTS For participants in cohort 1, the median survival time had not been reached at 174 days, whereas in the matched ECMO group, the median survival was 13 days (P<0.001 by the log-rank test). For participants in cohort 2 and the matched ECMO group, the median survival was 144 days and 10 days, respectively (P<0.001 by the log-rank test). Serious adverse events in cohort 1 and cohort 2 included major bleeding (in 42% and 50% of patients, respectively), infection (in 63% and 50%), and stroke (in 29% and 29%). CONCLUSIONS Our trial showed that survival rates were significantly higher with the ventricular assist device than with ECMO. Serious adverse events, including infection, stroke, and bleeding, occurred in a majority of study participants. (Funded by Berlin Heart and the Food and Drug Administration Office of Orphan Product Development; ClinicalTrials.gov number, NCT00583661.).

Extubation in the Operating Room After Fontan's Procedure: Effect on Practice and Outcomes

BACKGROUND Timely extubation is a well-accepted strategy in the postoperative intensive care unit management of Fontan patients to minimize the deleterious effects of positive-pressure ventilation. In October 2002, this strategy was extended to extubating selective Fontan patients in the operating room (EOR). This retrospective study examines how EOR has affected outcomes and practice in our Fontan population. METHODS Between October 2002 and June 2006, 112 patients underwent primary Fontan procedures; 38 (34%) were EOR and 74 (66%) were non-EOR. These two cohorts were not different (p < 0.05) in age, weight, surgery time, dominant ventricular morphology, hypoplastic left heart syndrome, prior bidirectional Glenn, concomitant procedures, atrioventricular valve regurgitation, and ventricular function. Analysis of variance was used to compare mean pulmonary artery pressure, mean arterial blood pressure, and mean common atrial pressure as a function of time. RESULTS During the first 12 hours postoperatively, mean pulmonary artery pressure and mean common atrial pressure were significantly lower and mean arterial blood pressure was significantly higher in the EOR group than the non-EOR group (p < 0.05). No EOR patient required reintubation. Mean durations of inotropic agents (1.1 versus 2.4 days), chest tubes (5.8 versus 7.2 days), intensive care unit stay (3 versus 4.7 days), and hospital stay (8.6 versus 11.3 days) for EOR patients were shorter than for non-EOR patients (p < 0.05). Intensive care unit and hospital costs for EOR patients were 35% and 31% lower, respectively, than for non-EOR patients (p < 0.05). Kaplan-Meier survival for EOR patients (3 years, 100%) was not different (p = 0.3) than for non-EOR patients (1 and 3 years, 96%). CONCLUSIONS After the Fontan procedure, selective EOR can be performed safely and improves postoperative hemodynamics, decreases hospital resource utilization, and reduces hospital recovery time.

Safety of ketorolac in neonates and infants after cardiac surgery

Background:  Ketorolac is an injectable nonsteroidal anti‐inflammatory drug that is often used as a transitional short‐term analgesic to treat moderate pain and to decrease opioid use. There is a paucity of literature documenting the safety of using ketorolac in neonates and infants after cardiac surgery.

Anomalous Aortic Origin of a Coronary Artery: Toward a Standardized Approach

Anomalous aortic origin of a coronary artery (AAOCA) is a congenital abnormality of the origin or course of a coronary artery that arises from the aorta. It is the second most common cause of sudden cardiac death in young athletes. Its exact prevalence, the pathophysiological mechanisms that cause sudden cardiac death, the actual risk of death for the different types of AAOCA, the optimal way to evaluate these patients, and whether any treatment strategies decrease the risk of sudden cardiac death in patients diagnosed with AAOCA are unknown. This article analyzes what is currently known and unknown about this disease. It also describes the creation of a dedicated multidisciplinary coronary anomalies program and the development of a framework in an initial attempt to standardize the evaluation and management of these patients.

Interstage attrition between bidirectional Glenn and Fontan palliation in children with hypoplastic left heart syndrome.

OBJECTIVE With improving operative mortality for staged palliation of hypoplastic left heart syndrome, interstage death accounts for an increasing proportion of hypoplastic left heart syndrome mortality. We investigated risk factors for death or cardiac transplantation during the interstage period between bidirectional Glenn and Fontan procedures in children with hypoplastic left heart syndrome. METHODS Patients with hypoplastic left heart syndrome who underwent bidirectional Glenn between August 1995 and June 2007 were screened. Standard risk patients, defined by having been discharged after both Norwood and bidirectional Glenn, were included for analysis. Patient demographic, echocardiographic, cardiac catheterization, and operative data were reviewed. Interstage attrition was defined as death or cardiac transplantation more than 30 days after bidirectional Glenn and before the Fontan procedure. Statistical analysis was carried out using the Student t test, Pearson chi-square correlation, and Cox proportional hazard modeling for multivariable analysis. RESULTS Ninety-two patients with hypoplastic left heart syndrome were alive at 30 days after bidirectional Glenn. Of these patients, 8 died and 3 underwent cardiac transplantation at a median of 391 days (range, 59-1175 days) after bidirectional Glenn, yielding an interstage attrition rate of 12%. Removing the 7 patients who are still awaiting Fontan (but all of whom are at least 3.5 years after bidirectional Glenn) adjusts the attrition rate to 13%. Interstage attrition did not correlate with hemodynamic data obtained at cardiac catheterization, aortic arch obstruction, or right ventricular dysfunction. Multivariable analysis demonstrated that the presence of moderate or severe tricuspid valve regurgitation (hazard ratio, 6.02; 95% confidence interval, 1.56-23.24; P < .01) and weight z score (hazard ratio, 0.38; 95% confidence interval, 0.16-0.88; P = .02) were independent preoperative risk factors for interstage attrition. CONCLUSIONS Interstage attrition between bidirectional Glenn and Fontan procedures occurred in 12% of our study population. Moderate or greater tricuspid valve regurgitation and low weight z score at the time of bidirectional Glenn are important risk factors for interstage attrition between the bidirectional Glenn and Fontan procedures in children with hypoplastic left heart syndrome.

Aortic Arch Advancement for Aortic Coarctation and Hypoplastic Aortic Arch in Neonates and Infants

BACKGROUND The optimal treatment for infants with aortic coarctation and hypoplastic aortic arch is controversial. The goal of this study was to report the short-term and mid-term outcomes of aortic arch advancement (AAA) in infants with hypoplastic aortic arch. METHODS All infants who underwent AAA at our institution from 1995 to 2012 were included. AAA consisted of coarctectomy and end-to-side anastomosis of the descending aorta to the distal ascending aorta/proximal arch through a median sternotomy. The cohort was divided into four groups: (1) isolated AAA (n=29, 11%), (2) AAA with closure of ventricular septal defect (n=56, 20%), (3) AAA with other biventricular repairs (n=115, 42%), and (4) AAA as part of single-ventricle palliation (n=75, 27%). RESULTS The cohort included 275 patients: 125 (45%) were female, and the median age was 14 days (interquartile range, 7-34 days). Genetic abnormalities were present in 48 patients (17%). Neurologic adverse events occurred in 3 patients (1%), all in group 4. Left bronchial compression was seen in 2 patients (0.7%); only one required intervention. Vocal cord dysfunction was noted in 36 of 95 patients (38%) on routine laryngoscopy. Only 1 patient had clinical residual dysfunction at the last follow-up visit. Perioperative mortality was 3% (n=8). At a median follow-up time of 6 years, 8 patients (3%) had reinterventions at a median time of 5 months (3-17 months) after repair. CONCLUSIONS AAA is a safe, effective, and durable operation with low rates of adverse events and mid-term reintervention. The advantages include native tissue-to-tissue reconstruction and preserved potential for growth. As such, it is the ideal technique for the management of hypoplastic aortic arch in neonates and infants.

Outcomes after the palliative arterial switch operation in neonates with single-ventricle anatomy.

BACKGROUND Newborns with single-ventricle anatomy, transposition of the great arteries, and systemic outflow obstruction are challenging patients most often managed with a Norwood or Damus-Kaye-Stansel (DKS) procedure. The palliative arterial switch operation (pASO) offers the theoretical advantage of avoiding a systemic-to-pulmonary artery shunt physiology and posterior entrapment of the left pulmonary artery while aligning the single left ventricle with the posterior semilunar valve. Limited outcome data exist for the pASO. The purpose of this study was to examine the clinical course of patients after this operation. METHODS We conducted a retrospective review of all neonates undergoing a pASO at our institution from July 1995 to June 2011. RESULTS Fourteen patients underwent pASO at a median age of 7 days (2-16 days). Primary diagnoses included double-inlet left ventricle (6 patients [43%]), tricuspid atresia (TA) (4 patients [29%]), and other (4 patients [29%]). Concomitant procedures at initial operation included aortic arch reconstruction (13 patients [93%]), pulmonary artery banding (6 patients [43%]), and placement of a systemic-to-pulmonary artery shunt (1 patient [7%]). Median intensive care unit stay and hospital length of stay were 11 days (4-60 days) and 21 days (9-84), respectively. There were no deaths. All patients had advanced to a bidirectional cavopulmonary shunt (BCPS). Four patients required intervention between the pASO and BCPS procedures: systemic-to-pulmonary artery shunt (3 patients [21%]) and pulmonary artery banding revision (1 patient [7%]). Eleven patients had Fontan completion; the remaining 3 patients are candidates for this procedure. At the time of the Fontan operation, 7 (64%) patients required pulmonary artery augmentation. All patients were alive at last follow-up (median, 5 years [3 months-14 years]). At last echocardiographic follow-up (median, 5 years [3 months-10 years]), all patients had normal ventricular function, trivial to mild neoaortic insufficiency, and no left ventricular outflow obstruction. CONCLUSIONS The pASO should be considered in neonates with single ventricle, transposition, and systemic outflow obstruction because it affords a favorable anatomic arrangement for long-term palliation, with excellent survival and preserved ventricular function.

Extracellular matrix remodeling and cell phenotypic changes in dysplastic and hemodynamically altered semilunar human cardiac valves

INTRODUCTION Congenital cardiac valve disease is common, affecting ∼1% of the population, with substantial morbidity and mortality, but suboptimal treatment options. Characterization of the specific matrix and valve cell phenotypic abnormalities in these valves could lend insight into disease pathogenesis and potentially pave the way for novel therapies. METHODS Thirty-five human aortic and pulmonic valves were categorized based on gross and microscopic assessment into control valves (n=21); dysplastic valves, all except one also displaying hemodynamic changes (HEMO/DYSP, n=6); and hemodynamically altered valves (HEMO, n=8). Immunohistochemistry was performed on valve sections and flow cytometry on valvular interstitial cells. RESULTS While both hemodynamically altered aortic and pulmonic valves demonstrated increased collagen turnover and cell activation, prolyl 4-hydroxylase and hyaluronan increased in hemodynamically altered aortic valves but decreased in hemodynamically altered pulmonic valves relative to control valves (P<.001). HEMO/DYSP aortic valves demonstrated decreased collagen and elastic fiber synthesis and turnover compared to both hemodynamically altered aortic valves and control aortic valves (each P<.006). Valvular interstitial cells from both hemodynamically altered and HEMO/DYSP pulmonic valves showed altered cell phenotype compared to control valves (each P<.032), especially increased non-muscle myosin. Furthermore, valvular interstitial cells from hemodynamically altered pulmonic valves and HEMO/DYSP aortic and pulmonic valves each demonstrated greater size and complexity compared to control valves (each P<.05). CONCLUSIONS Dysplastic semilunar valves displayed alterations in collagen and elastic fiber turnover that were distinct from valves similarly exposed to altered hemodynamics as well as to control valves. These results demonstrate that dysplastic valves are not simply valves with gross changes or loss of leaflet layers, but contain complex matrix and cell phenotype changes that, with future study, could potentially be targets for novel nonsurgical treatments.

Mechanical circulatory support for the pediatric patient.

In adult patients, mechanical circulatory support (MCS) devices are part of the standard of care for heart failure. There are several options available that clinicians may use to provide support for their patients and thus the choice of devices can be tailored to their individual needs. Unfortunately, this is not the case for pediatric patients with heart failure, where the options for MCS modalities are far more limited because of size constraints and regulatory hurdles. Furthermore, the pathophysiology of heart failure in children is not identical to that of adults; thus, the device selection and configuration require different considerations. Management of pediatric patients on MCS requires a highly specialized team of pediatric professionals. The nurse caring for a child on MCS is an active participant in all aspects of the patients care and must have an in-depth understanding of the patients underlying physiology, the circulatory physiology of the MCS system, the reason for mechanical support, and the goal of support.

Time-driven activity-based costing to identify opportunities for cost reduction in pediatric appendectomy

PURPOSE As reimbursement programs shift to value-based payment models emphasizing quality and efficient healthcare delivery, there exists a need to better understand process management to unearth true costs of patient care. We sought to identify cost-reduction opportunities in simple appendicitis management by applying a time-driven activity-based costing (TDABC) methodology to this high-volume surgical condition. METHODS Process maps were created using medical record time stamps. Labor capacity cost rates were calculated using national median physician salaries, weighted nurse-patient ratios, and hospital cost data. Consumable costs for supplies, pharmacy, laboratory, and food were derived from the hospital general ledger. RESULTS Time-driven activity-based costing resulted in precise per-minute calculation of personnel costs. Highest costs were in the operating room (