A Randomized Pilot Trial Assessing the Role of Human Fibrinogen Concentrate in Decreasing Cryoprecipitate Use and Blood Loss in Infants Undergoing Cardiopulmonary Bypass.

The objective of this study was to determine whether treatment with human fibrinogen concentrate decreases the need for component blood therapy and blood loss in neonate and infant patients undergoing cardiopulmonary bypass. Pediatric patients (N = 30) undergoing elective cardiac surgery were randomized to receive human fibrinogen concentrate or placebo following cardiopulmonary bypass termination. The primary endpoint was the amount of cryoprecipitate administered. Secondary endpoints included estimated blood loss during the 24 h post-surgery; perioperative blood product transfusion; effects of fibrinogen infusion on global hemostasis, measured by laboratory testing and rotational thromboelastometry; and adverse events. No clinically significant differences were identified in baseline characteristics between groups. A significantly lower volume of cryoprecipitate was administered to the treatment group during the perioperative period [median (interquartile range) 0.0 (0.0-0.0) cc/kg vs 12.0 (8.2-14.3) cc/kg; P < 0.0001] versus placebo. No difference was observed between treatment groups in blood loss, laboratory coagulation tests, use of other blood components, or incidence of adverse events. FIBTEM amplitude of maximum clot firmness values was significantly higher among patients treated with human fibrinogen concentrate versus placebo (P ≤ 0.0001). No significant differences were observed in post-drug HEPTEM, INTEM, and EXTEM results. Human fibrinogen concentrate (70 mg/kg) administered after the termination of cardiopulmonary bypass reduced the need for transfusion with cryoprecipitate in a neonate and infant patient population.ClinicalTrials.gov identifier: NCT02822599.

Safety of bloodless open-heart surgery on cardiopulmonary bypass in selected children: A single center experience with minimal invasive extracorporeal circulation.

INTRODUCTION: Bloodless cardiac surgery refers to open-heart surgery without blood or blood products. The cardiopulmonary bypass (CPB) circuits are primed with crystalloid solely, and there is no intraoperative blood transfusion. METHODS: Our program considers bloodless congenital cardiac surgery with a minimal invasive extracorporeal circulation (MiECC) system for patients above 10 kg of weight. We performed a single-center retrospective cohort study of all consecutive patients undergoing bloodless cardiac surgery for congenital heart defects between January 2016 and December 2018. RESULTS: A total of 164 patients were reviewed (86 male and 78 female) at a median age of 9.6 years (interquartile range (IQR), 4.5-15), a weight of 32 kg (IQR, 16-55), preoperative hemoglobin 13.7 g/dl (IQR, 12.6-14.9), and preoperative hematocrit of 40.4% (IQR, 37.2-44.3). Median CPB time was 81.5 min (IQR, 58-125), and median hematocrit coming off CPB was 26% (IQR, 23-29.7). The congenital heart surgery risk (STAT) category was distributed in STAT 1 for 70, STAT 2 for 80, STAT 3 for 9, and STAT 4 for 5 patients. Most patients (95%) were extubated in the operating room with a low complication rate during the hospital stay (14.6%). Only 6 (4%) patients needed a blood transfusion during the postoperative period, with a higher incidence of complications during the hospital course (p < 0.001). CONCLUSIONS: Bloodless congenital heart surgery with MiECC system is safe in low-surgical-risk patients. Our patients had a low rate of complications and short hospital stays.

Evaluation of Residual Coarctation in Infants with a Single Right Ventricle after Stage I Palliation.

In infants with a single right ventricle (RV), stage I palliation involves aortic reconstruction, systemic-to-pulmonary shunt placement, and atrial septectomy. Many require re-intervention for residual coarctation of the aorta (CoA). Doppler echocardiography can detect residual CoA in most infants, but its ability to predict severity has not been studied. This study compares gradients from Doppler interrogation to those from cardiac catheterization in infants with residual CoA. We performed a retrospective study of infants after stage I palliation from 2000 to 2014. Infants with an echocardiogram and catheterization before the second-stage palliative surgery were included. Infants with an echocardiogram >30 days before catheterization were excluded. Doppler-derived gradients were compared to catheterization-derived gradients. Echocardiographic assessment of tricuspid valve (TV) and RV function were recorded. The cohort included 95 infants, and thirty-three (35%) had CoA. Doppler-derived and catheterization-derived gradients correlated weakly in infants with CoA (r = 0.37, p = 0.036) and without CoA (r = 0.35, p = 0.005). Among infants with CoA, 17/33 had none or trivial tricuspid regurgitation (TR) and normal RV function, and Doppler-derived gradients correlated with catheterization gradients in this group (r = 0.71, p = 0.001). In 16/33 infants with ≥moderate TR or RV dysfunction, gradients did not correlate (r = -0.003, p = 0.992). After a stage I palliation in infants with single RV and CoA, Doppler-derived gradients poorly predicted the severity of CoA. Infants with normal TV or RV function had Doppler-derived gradients more predictive of catheterization-derived gradients. Doppler-derived gradients have limited utility in determining the severity of CoA after a stage I palliation.

Risk Factors for Longer Hospital Stay Following the Fontan Operation.

OBJECTIVES: Children with functional single ventricle undergoing the Fontan operation consume considerable resources. The purpose of this study is to evaluate pre- and intraoperative risk factors for longer hospital stay and to describe the perioperative course at a single institution over a 15-year period. DESIGN: Retrospective cohort study. SETTING: A single pediatric cardiac ICU. PATIENTS: All consecutive patients undergoing a first-time Fontan operation from 2000 to 2014. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Prolonged length of stay was defined as hospital stay greater than 75 percentile at our institution after surgery. Of 218 patients who met inclusion criteria, median length of stay was 10 days (interquartile range, 8-14 d); prolonged length of stay was defined greater than or equal to 15 days. Independent pre- and intraoperative risk factors for prolonged length of stay included higher hemoglobin (odds ratio, 1.29; p = 0.003), higher mean pulmonary artery pressure (odds ratio, 1.14; p = 0.037), and lower aortic saturation (odds ratio, 0.92; p = 0.008) in the entire group. When patients with hepatic vein inclusion (following previous Kawashima) were excluded, higher hemoglobin (odds ratio, 1.24; p = 0.027), lower aortic saturation (odds ratio, 0.92; p = 0.017), and placement of a fenestration (odds ratio, 2.438; p = 0.021) were associated with prolonged length of stay. Fifty-eight patients (26.6%) had major complications defined by Pediatric Cardiac Critical Care Consortium. Placement of a fenestration (odds ratio, 2.297; p = 0.014) and longer aortic cross-clamp time (odds ratio, 1.015; p = 0.003) were independently associated with Pediatric Cardiac Critical Care Consortium major complications. CONCLUSIONS: In this series, 75% of patients had a postoperative length of stay less than or equal to 2 weeks. Preoperative factors suggesting worse hypoxemia/decreased pulmonary blood flow (higher hemoglobin and lower oxygen saturation) and increased pulmonary artery pressure were associated with prolonged length of stay. These findings may help risk stratify this complex patient population, provide more accurate family counseling, and provide preliminary data for changes in preoperative timing of the Fontan and/or changes to postoperative management strategies for those at high risk for increased ICU morbidity.

Comparison of point-of-care activated clotting time systems utilized in a single pediatric institution.

This study compares four different activated clotting time (ACT) point-of-care (POC) testing systems used at our institution for the management of patients undergoing heparin therapy. We evaluated these systems under identical conditions to determine their accuracy, reproducibility, ease of use, and cost. Two separate testing stations containing four ACT systems were used. The testing order was randomized for every sample and performed by two trained individuals. Samples of fresh heparinized whole blood were taken at regular intervals and distributed to each station. Each operator tested 50 samples, totaling 400 ACT tests. The ACT value was significantly affected by the type of machine used at both stations 1 and 2 (p < .001). Compared with all systems, the Medtronic ACT Plus Automated Coagulation Timer System (ACT Plus) resulted in the most consistent ACT values (median = 171, Interquartile Range (IQR): 169-175) and least variability (172.17 +/- 5.24). The Hemochron Signature Elite Whole Blood Microcoagulation System had the most variability (221.10 +/- 14.78) and yielded consistently higher ACT values (median = 220, IQR: 210-229.5) compared with other systems. The ACT values reported by the i-STAT Handheld and Test Cartridge Blood Analysis System (153.30 +/- 7.87) were consistently lower (median = 154, IQR: 147-161) in comparison to the ACT Plus and Medtronic HMS Plus Hemostasis Management System (180.60 +/- 7.60, median = 181, IQR: 175-186). There was no statistical difference in results between the two testing sites (p > .05) or the operators (p > .05). The significant finding of this study was the affect each system has on the ACT value. This investigation demonstrates the variability that exists among different ACT monitoring systems at our institution. The discrepant variation in ACT values that exists with the Hemochron system questions the reliability of its use in the management of patients undergoing heparin therapy.

Hybrid aortic reconstruction for treatment of recurrent aortic obstruction after stage 1 single ventricle palliation: medium term outcomes and results of redilation.

OBJECTIVE: We describe a hybrid approach to the treatment of aortic obstruction after stage 1 palliation (S1P) of hypoplastic left heart syndrome. BACKGROUND: Recurrent aortic obstruction is a common problem after S1P of hypoplastic left heart syndrome. Even mild aortic obstruction is poorly tolerated so early and definitive therapy is desirable. Although stent implantation is an effective treatment for aortic obstruction in older children and adults, technical issues due to small vessels and concerns regarding future potential for expansion have generally precluded the use of stents in this setting. METHODS: Six patients underwent hybrid aortic reconstruction (HAR) in the operating room or catheterization laboratory, with the interventional cardiologist and cardiac surgeon working in cooperation. RESULTS: Patients had a mean weight of 5.8 kg (2.9-7.7) and a mean age of 5.6 months (0.5-12.9) at the time of HAR. Five patients had undergone prior balloon angioplasty at a mean age of 2.8 months (2.1-3.5), and five had moderately depressed single ventricular function prior to HAR. The balloons used had a diameter of 7-10 mm and introducer sheath size ranged from 6 to 10 F. There were no immediate or late procedure related complications. Stent redilation was performed in 5 patients for relief of recurrent obstruction or to keep pace with somatic growth. At a median follow up of 4.8 years (0.2-7.9), there were 3 patients progressing well after Fontan palliation and 3 deaths. CONCLUSIONS: HAR allows for placement of stents that can ultimately reach adult size in small infants who have recurrent aortic obstruction after balloon angioplasty following S1P. Advantages include freedom from delivery sheath constraints when determining stent type/size, facilitation of precise stent position, and avoidance of vascular damage or hemodynamic compromise during the procedure. Longer follow-up and larger experience are required to determine if this therapy will provide a long-term solution to this difficult problem.

The Fontan operation: the pursuit of associated lesions and cumulative trauma.

Our programmatic approach to the Fontan operation has evolved to include using an extracardiac conduit with aggressive presumptive treatment of associated lesions either in the catheterization laboratory or the operating room. Fenestration is used selectively based on hemodynamics, anatomy, and presence of associated lesions. We reviewed our experience to determine the effectiveness and outcome of this strategy and to assess the cumulative trauma to the patients. The records of 137 consecutive patients who underwent Fontan at Miami Children's Hospital from 1995 to 2008 were reviewed. At mean follow up of 5.76 years, freedom from death or transplantation is 94.2% (129/137). Median age at operation was 4.6 years. Longer length of stay correlated with older operative age (P = 0.0056). Pacemakers were implanted in 11.7% (16/137). Additional (not pre-Glenn or pre-Fontan) interventional catheterizations were performed in 51.8% (71/137). Additional operations were done in 10.2% (14/137). No patient has required replacement or revision of the extracardiac conduit. Our current approach to the Fontan operation provides acceptable midterm results. The pursuit of residual lesions results in a significant number of additional interventional catheterizations and operative procedures but might have an important influence on long-term survival after the Fontan procedure.

Transforming patient and family access to medical information: utilisation patterns of a patient-accessible electronic health record.

OBJECTIVE: The purpose of this study was to evaluate the utilisation of a web-based multimedia patient-accessible electronic health record, for patients with congenital cardiac disease. PATIENTS AND METHODS: This was a prospective analysis of patients undergoing congenital cardiac surgery at a single institution from 1 September, 2006 to 1 February, 2009. After meetings with hospital administration, physicians, nurses, and patients, we configured a subset of the cardiac program's web-based clinical electronic health record for patient and family access. The Electronic Health Record continuously measured frequency and time of logins, logins during and between hospitalisations, and page views by type (imaging versus textual data). RESULTS: Of the first 270 patients offered access to the system, 252 became users (93% adoption rate). System uptime was 99.9%, and no security breaches were reported. Users accessed the system more often while the patients were in hospital (67% of total logins) than after discharge (33% of total logins). The maximum number of logins by a family was 440, and the minimum was 1. The average number of logins per family was 25. Imaging data were viewed significantly more frequently than textual data (p 0.001). A total of 12 patients died during the study period and 11 members of their families continued to access their Electronic Health Records after the date of death. CONCLUSIONS: A web-based Patient Accessible Electronic Health Record was designed for patients with congenital cardiac disease. The adoption rate was high, and utilisation patterns suggest that the Electronic Health Record could become a useful tool for health information exchange.

An institutional approach to, and results for, patient with tetralogy with pulmonary atresia and major systemic-to-pulmonary collateral arteries.

BACKGROUND: Tetralogy of Fallot with pulmonary atresia and diminutive or absent intrapericardial pulmonary arteries is a rare congenital abnormality, with high morbidity and mortality. Despite great advances in surgical- and catheter-based therapies, management remains challenging and controversial. We describe the surgical methods and the results from our institution. METHODS: We performed a retrospective study of the medical records of patients included in our institutional database with tetralogy and pulmonary atresia, concentrating on those predominantly managed by our programme over their lifetime. We obtained demographics and records of all catheterisations and operations, and established mortality. We assessed the current state of those surviving in terms of clinical function at their most recent clinical evaluation and right ventricular function by echocardiography. RESULTS: We assessed 38 patients, with 89% follow-up. The mean number of catheterisations for each patients was 5, with a range from 1 to 15. The mean number of operations was 2.2, with a range from 1 to 6. Unifocalisation had been performed in 26 patients, with 12 undergoing procedures to recruit the native pulmonary vasculature. Of the overall cohort, eight patients died. The ventricular septal defect had been closed in all but two patients. Most patients have no or mild exercise intolerance. Right ventricle dysfunction has been a continuing hazard for 15 years. CONCLUSIONS: An individualised approach, using unifocalisation as well as aggressive attempts to recruit the available native pulmonary vasculature, achieves outcomes in the intermediate term superior to the natural history of the lesions, and comparable with those of other studies.

Three-year experience with immediate extubation in pediatric patients after congenital cardiac surgery.

BACKGROUND: In pediatric cardiac anesthesiology, there is increased focus on minimizing morbidity, ensuring optimal functional status, and using health care resources sparingly. One aspect of care that has potential to affect all of the above is postoperative mechanical ventilation. Historically, postoperative ventilation was considered a must for maintaining patient stability. Ironically, it is recognized that mechanical ventilation may increase risk of adverse outcomes in the postoperative period. Hence, many institutions have advocated for immediate extubation or early extubation after many congenital heart surgeries which was first reported decades ago. METHODS: 637 consecutive patient charts were reviewed for pediatric patients undergoing cardiac surgery with cardiopulmonary bypass. Patients were placed into three groups. Those that were extubated in the operating room (OR) at the conclusion of surgery (Immediate Extubation or IE), those that were extubated within six hours of admission to the ICU (Early Extubation or EE) and those that were extubated sometime after six hours (Delayed Extubation or DE). Multiple variables were then recorded to see which factors correlated with successful Immediate or Early Extubation. RESULTS: Overall, 338 patients (53.1%) had IE), 273 (42.8%) had DE while only 26 patients (4.1%) had EE. The median age was 1174 days for the IE patients, 39 days for the DE patients, whereas 194 days for EE patients (p < 0.001). Weight and length were also significantly different in at least one extubation group from the other two (p < 0.001). The median ICU LOS was 3 and 4 days for IE and EE patients respectively, whereas it was 9.5 days for DE patients (p < 0.001). DE group had a significant longer median anesthesia time and cardiopulmonary bypass time than the other two extubation groups (p > 63,826.88 < 0.001). Regional low flow perfusion, deep hypothermia, deep hypothermic circulatory arrest, redo sternotomy, use of other sedatives, furosemide, epinephrine, vasopressin, open chest, cardiopulmonary support, pulmonary edema, syndrome, as well as difficult intubation were significantly associated with delayed extubation (IE, EE or DE). CONCLUSIONS: Immediate and early extubation was significantly associated with several factors, including patient age and size, duration of CPB, use of certain anesthetic drugs, and the amount of blood loss and blood replacement. IE can be successfully accomplished in a majority of pediatric patients undergoing surgery for congenital heart disease, including in a minority of infants.

Continuous incisional infusion of local anesthetic in pediatric patients following open heart surgery.

AIM: To determine the efficacy and safety of a continuous subcutaneous local anesthetic (LA) infusion in pediatric patients following open heart surgery. BACKGROUND: The use of a continuous LA infusion has been shown to be beneficial following adult cardiac surgery. To date there are no studies in the pediatric population. METHODS/MATERIALS: Using a prospective, randomized, and double blind design, we compared LA, either 0.25% levobupivacaine or bupivacaine (Treatment Group) to saline (Placebo Group) delivered subcutaneously via a continuous infusion for 72 h after open heart surgery in 72 patients. Requirements for postoperative analgesics and pain scores were recorded for 72 h and plasma levels of local anesthetic were measured. Secondary outcomes measures included time to first oral intake, time to first bowel movement, time to urinary catheter removal, length of stay, requirements for antiemetics and additional sedation. RESULTS: Total morphine requirements over the first 24 h were less in the Treatment Group than the Placebo Group (0.05 mg x kg(-1) vs 0.2 mg x kg(-1), P = 0.007); this was true for all patient groups except those patients weighing less than 6.3 kg. The number of patients requiring no morphine was greater in the Treatment Group (7/35 vs 1/37, P = 0.02). The Treatment Group also received less midazolam, lorazepam, and ketorolac than the Placebo Group over 72 h due to the reduced clinical need for these agents in patients weighing less than 31 kg. There were no differences in secondary outcomes. CONCLUSIONS: A continuous incisional infusion of LA reduced postoperative analgesic requirement and sedative use in pediatric patients undergoing a median sternotomy incision. Dosed at a maximum rate of 0.4 mg x kg(-1) x h(-1), a continuous incisional infusion of LA is effective and safe for up to 72 h, with plasma levels of local anesthetic well below the toxic threshold.

Frequency and indications for tracheostomy and gastrostomy after congenital heart surgery.

Patients undergoing congenital heart surgery may occasionally require additional surgical procedures in the form of tracheostomy and gastrostomy. These procedures are often performed in an attempt to diminish hospital morbidity and length of stay. We reviewed the Web-based medical records of all patients undergoing congenital heart surgery at Miami Children's Hospital from February 2002 through August 2007. Patients who were deemed preterm and had undergone closure of a patent ductus arteriosis were eliminated. The records of all other patients were queried for the terms gastrostomy, g-tube, Nissan, fundal plication, tracheostomy, or tracheotomy. Patients' medical records in which these terms appeared in any portion were completely reviewed. There were 1660 congenital heart operations performed in the study period. There were 592 operations performed on patients whose age ranged from 1 month to 1 year and 441 neonatal operations. Mortality was 2%. Median postoperative stay was 8 days (range, 1-191 days), 12 days for neonates (range, 3-142 days), and 19 days for neonates undergoing RACHS-1 category 6 operations (range, 4-142 days). Tracheostomies were performed in four patients (0.2%). Gastrostomies were performed on eight patients (0.4%), representing 0.8% of patients <1 year of age, 1.4% of neonates, and 2.4% of patients undergoing RACHS-1 category 6 operations. The rate of patients undergoing either tracheostomy or gastrostomy after congenital heart surgery at our institution was quite low. Avoidance of either of these two procedures was achieved without increased morbidity or length of stay. The rate at which these procedures need to be performed may reflect the magnitude of the patients' lifetime trauma related to their underlying condition and acute and total surgical experiences.

Analysis of regional congenital cardiac surgical outcomes in Florida using the Society of Thoracic Surgeons Congenital Heart Surgery Database.

BACKGROUND: Florida is the fourth largest state in the United States of America. In 2004, 218,045 live babies were born in Florida, accounting for approximately 1744 new cases of congenital heart disease. We review the initial experience of The Society of Thoracic Surgeons Congenital Heart Surgery Database with a regional outcomes report, namely the Society of Thoracic Surgeons Florida Regional Report. METHODS: Eight centres in Florida provide services for congenital cardiac surgery. The Children's Medical Services of Florida provide a framework for quality improvement collaboration between centres. All congenital cardiac surgical centres in Florida have voluntarily agreed to submit data to the Society of Thoracic Surgeons Database. The Society of Thoracic Surgeons and Duke Clinical Research Institute prepared a Florida Regional Report to allow detailed regional analysis of outcomes for congenital cardiac surgery. RESULTS: The report of 2007 from the Society of Thoracic Surgeons Congenital Heart Surgery Database includes details of 61,014 operations performed during the 4 year data harvest window, which extended from 2003 through 2006. Of these operations, 6,385 (10.5%) were performed in Florida. Discharge mortality in the data from Florida overall, and from each Florida site, with 95% confidence intervals, is not different from cumulative data from the entire Society of Thoracic Surgeons Database, both for all patients and for patients stratified by complexity. CONCLUSIONS: A regional consortium of congenital heart surgery centres in Florida under the framework of the Children's Medical Services has allowed for inter-institutional collaboration with the goal of quality improvement. This experience demonstrates, first, that the database maintained by the Society of Thoracic Surgeons can provide the framework for regional analysis of outcomes, and second, that voluntary regional collaborative efforts permit the pooling of data for such analysis.

Smart Graft Monitoring After the Fontan Operation.

The Fontan operation for children with congenital heart disease places them in a physiological state of mildly elevated systemic venous pressure and low-normal cardiac output. Consequently, close follow-up is imperative, yet currently no method is available to obtain Fontan pressures without direct measurements in the cardiac catheterization laboratory while supine and sedated. We hypothesize that by suturing the CardioMEMS sensor device into the Fontan conduit during the standard Fontan operation for a child with single ventricular physiology, clinicians can accurately retrieve Fontan pathway pressure measurements noninvasively during normal physiological states.

The successful use of alternative routes of vascular access for performing pediatric interventional cardiac catheterization.

OBJECTIVE: The objective of this study is to examine the safety/efficacy of alternative routes of vascular access (ARVA) for successful performance of interventions. BACKGROUND: Complex interventional catheterizations may be required in children with limited vascular access, vascular constraints relative to size, and hemodynamic instability. Our approach has been to utilize ARVA in selected cases. METHODS: ARVA pertains to any vessel excluding femoral, jugular/subclavian veins, or umbilical access. A retrospective review performed on patients with an intervention utilizing ARVA between August 1995 and January 2004 was performed. Patients were divided by clinical status: critically ill/emergent (A), elective cases (B). Procedural success was based on previously published criteria. RESULTS: Sixty-four interventions were performed in 50 patients using 54 ARVA. ARVA utilized: radial (1), axillary (2), brachial (2), carotid arteries (25); brachial (2) hepatic (9) veins; and open chest/direct cardiac puncture (13). ARVA provided successful access to target lesions. Interventions included stents (30), valvuloplasty (16), angioplasty (14), and one each of vascular occlusion, septal occlusion, accessory pathway ablation, and septostomy. Group A patients were smaller (P < 0.0002) and younger (P < 0.004) than B. All open chest/direct cardiac and the majority (71%) of carotid arterial approaches were performed in group A. Fifty-six (88%) inteventions were successful with no difference between groups A (88%) and B (86%). There were two complications. Neither resulted in long-term sequelae. CONCLUSIONS: ARVA may provide a strategic advantage that may be safely applied to a variety of interventions regardless of patient size or degree of illness. These techniques may further extend the scope of successful interventions in children.

Stent implantation is effective treatment of vascular stenosis in young infants with congenital heart disease: acute implantation and long-term follow-up results.

BACKGROUND: Stents implantation in infants has been shown to be feasible, however, there are no published reports examining long-term outcomes. Concerns exist regarding creation of fixed obstructions secondary to the stent if expansion to larger diameters over time is not possible. METHODS: A retrospective analysis of the earliest consecutive series of infants who underwent stent placement at our institution between October 1995 and December 1999. RESULTS: Implantation of 33 stents were attempted in 27 infants, median age = 10 (25-24) months, wt = 8.1 (3.4-14.5) kg. Stents used were as follows: 16 large, 13 medium, and 4 coronary. Acute implant success was 94%. There were three nonprocedure-related deaths within 30 days of implantation, 1 patient was lost to follow-up and 1 had acute stent thrombosis. The remaining 22 patients (26 stents) form the long-term follow-up study group. Nineteen stents underwent 33 redilations. Following latest redilation, 67.0 (37-113) months postimplantation, minimal luminal diameter increased from 7.0 +/- 1.8 mm immediately following implantation to 8.7 +/- 2.3 mm (P < 0.001). Seven stents were electively removed/ligated during a planned surgery. All 18 remaining in situ stents are patent without significant obstruction 102 (84-116) months following implantation. There was one late death 51 months after stent implantation. The remaining 21 patients are alive and well. CONCLUSIONS: Stent implantation in infants is safe and effective. Serial redilation is possible to keep pace with somatic growth; however, efforts should be made to implant stents with adult diameter potential in children who will not require further cardiac surgery. Implantation of small- and medium-sized stents can provide effective palliation and should be considered in carefully selected infants who will ultimately require future surgery.

Non-invasive cardiac output monitor validation study in pediatric cardiac surgery patients.

STUDY OBJECTIVE: To validate a non-invasive cardiac output monitor in pediatric cardiac surgery patients. DESIGN: Prospective trial. SETTING: Operating room. PATIENTS: 20 pediatric cardiac surgery patients ASA physical status 3 and 4. INTERVENTIONS: Aesculon noninvasive cardiac output monitor was used and compared to the cardiac output derived from the Fick equation. Oxygen consumption was measured and blood samples were taken from the arterial line and from the superior and inferior vena cava. MEASUREMENTS: Noninvasive cardiac output and cardiac index and Fick cardiac output and cardiac index. Oxygen consumption was measured by the TreyMed metabolic monitor. Blood samples were simultaneously drawn from the arterial line and from the superior and inferior vena cava purse string sites by the surgeon, prior to commencing cardiopulmonary bypass. Another data set was obtained right after termination of cardiopulmonary bypass. RESULTS: There was a direct, significant relationship between Fick CO/CI and NICOM CO/CI measurements. More dispersion is detected when the magnitude of the measure increases, i.e., for older and larger patients. CONCLUSIONS: There is a strong correlation between the cardiac output values derived from the Fick equation and the Aesculon non-invasive cardiac output monitor.

Echocardiographic and Surgical Correlation of Coronary Artery Patterns in Transposition of the Great Arteries.

OBJECTIVE: Determine the accuracy of echocardiography to diagnose coronary anatomy in transposition of the great arteries and to evaluate the effect of accuracy on surgical outcomes and changes in accuracy over time. DESIGN: Retrospective chart review of neonates admitted February 1999 to March 2013 with transposition. Coronary pattern from the preoperative echocardiogram and operative reports were collected and compared with determine diagnostic accuracy. Coronary patterns were further confirmed by intraoperative images taken during surgery. SETTING: Tertiary care children's hospital. PATIENTS: Neonates with transposition of the great arteries and planned arterial switch operation with an echo and operative report or image describing the coronaries. INTERVENTIONS: Not applicable. OUTCOME MEASURES: Accuracy of echocardiography to diagnose coronary anatomy in transposition, and to identify factors related to correct diagnosis. RESULTS: One hundred forty-two patients met inclusion criteria with 122 correctly diagnosed, 16 incorrect, and 4 inconclusive. Accuracy was 86%, with 95% accuracy in patients with typical coronary patterns, 85% with the most common variant (left coronary from the leftward sinus and right and circumflex from the rightward sinus), and 61% with less common patterns. Typical and common variants were more likely to be correct than atypical patterns (P < .001). Cases with ventricular septal defect were more likely to have correctly diagnosed coronaries than with an intact ventricular septum (94% vs. 79%, P = .01). There was no change in accuracy over time (P > .05). There was no difference in duration of cardiopulmonary bypass, cross-clamp times, length of stay, or postoperative stay between the correct and incorrectly diagnosed groups (P > .05). CONCLUSIONS: In our center, accuracy of echocardiographic imaging of the coronary arteries in transposition was 86% without improvement over time, and perioperative outcomes were not affected by diagnostic accuracy. Further invasive imaging may not be necessary to determine the coronary pattern in this lesion.

Interventional catheterization performed in the early postoperative period after congenital heart surgery in children.

OBJECTIVES: The purpose of this study was to examine the safety and efficacy of interventional catheterization performed early after congenital heart surgery. BACKGROUND: Transcatheter interventions performed in the early postoperative period are viewed as high risk. To date, there have been limited published data regarding these procedures. METHODS: All catheterizations performed within six weeks after congenital heart surgery between August 1995 and January 2001 were retrospectively reviewed. A cardiac anesthesiologist, cardiac intensivist, cardiac surgeon, and operating room team were available for all cases. Interventional procedures were performed based on clinical indications, regardless of the time elapsed from surgery. RESULTS: Sixty-two patients, median age four months (2 days to 11 years), weight 4.7 kg (2.3 to 45 kg), underwent 66 catheterizations on median postoperative day 9 (0 to 42 days). Thirty-five cases involved 50 interventional procedures. Nine patients required extracorporeal cardiopulmonary support. Success rates by procedure were: angioplasty, 100%; stent implantation, 87%; vascular/septal occlusion, 100%; and palliative pulmonary valvotomy, 75%. Complications included stent migration (one patient), cerebral vascular injury (one patient), and left pulmonary artery stenosis (one patient). Thirty procedures involved angioplasty or stent implantation, including 26 involving a recently created suture line. Suture disruption or trans-mural vascular tears were not observed. There was no procedural mortality. Thirty-day survival for patients undergoing intervention was 83%. CONCLUSIONS: Transcatheter interventions can be successfully performed in the early postoperative period. These procedures can have a positive impact on patient outcome; however, they should be performed only by a pediatric interventional cardiologist supported by a multi-disciplinary team.

The fenestrated Kawashima operation for single ventricle with interrupted inferior vena cava.

An 8-month-old boy with double outlet right ventricle with hypoplastic left ventricle, heterotaxy, left atrial isomerism, bilateral superior vena cavae without bridging vein, and interruption of the inferior vena cava with azygous continuation to the left superior cava underwent a bilateral bidirectional cavopulmonary anastomosis. A calibrated 3-mm connection between the right pulmonary artery and the common atrium was constructed with the proximal right superior vena cava to allow right to left shunting, analogous to a fenestration in a Fontan operation. We hypothesize that in small young patients undergoing the Kawashima operation a fenestration may improve postoperative hemodynamics.