Splanchnic Near-Infrared Spectroscopy and Risk of Necrotizing Enterocolitis After Neonatal Heart Surgery

Infants with critical congenital heart disease, especially patients with a single-ventricle (SV) physiology, are at increased risk for the development of necrotizing enterocolitis (NEC). Decreased splanchnic oxygen delivery may contribute to the development of NEC and may be detected by regional oximetry (rSO₂) via splanchnic near-infrared spectroscopy (NIRS). This prospective study enrolled 64 neonates undergoing biventricular (BV) repair or SV palliation for CHD and monitored postoperative splanchnic rSO₂ before and during initiation of enteral feedings to determine whether changes in rSO₂ are associated with risk of NEC. Suspected or proven NEC was observed in 32 % (11/34) of the SV subjects and 0 % (0/30) of the BV subjects (p = 0.001). Compared with the BV subjects, the SV palliated subjects had significantly lower splanchnic rSO₂ before and during initiation of enteral feedings, but the groups showed no difference after correction for lower pulse oximetry (SpO₂) in the SV group. The clinical parameters were similar among the SV subjects with and without NEC except for cardiopulmonary bypass times, which were longer for the patients who experienced NEC (126 vs 85 min; p = 0.03). No difference was observed in splanchnic rSO₂ or in the SpO₂–rSO₂ difference between the SV subjects with and without NEC. Compared with the patients who had suspected or no NEC, the subjects with proven NEC had a lower average splanchnic rSO₂ (32.6 vs 47.0 %; p = 0.05), more time with rSO₂ less than 30 % (48.8 vs 6.7 %; p = 0.04) at one-fourth-volume feeds, and more time with SpO₂–rSO₂ exceeding 50 % (33.3 vs 0 %; p = 0.03) before feeds were initiated. These data suggest that splanchnic NIRS may be a useful tool for assessing risk of NEC, especially in patients with an SV physiology.     

Late Onset of Heart Block After Open Heart Surgery for Congenital Heart Disease

Late onset of complete heart block is a potentially dangerous complication after open heart surgery for congenital heart disease. The characteristics of patients with late-onset heart block have not been well described. A retrospective review of a pacemaker database was done to identify patients who presented with new onset heart block between 1988 and 2006, after they had been discharged from the hospital after open heart surgery with normal AV conduction. Fifteen patients were identified. The age at the time of the last surgery before the onset of heart block was 2.0 ± 3.2 years (range: 3 days to 10 years). Nine had a ventricular septal defect repair, four had an atrioventricular canal, and two other patients had other types of heart defect. The last EKG available for analysis before the onset of heart block had been obtained 5.1 ± 6.5 years (range: 7 days to 16 years) after surgery. The symptoms at the time of presentation were variable. Four patients presented with fatigue or exercise intolerance, two with syncope, two with congestive heart failure, and one with irritability, and the remaining six patients were diagnosed during routine follow-up. The time between open heart surgery and placement of a permanent pacemaker was 6.8 ± 7.3 years (range: 2 months to 19 years). There were seven patients in whom the onset of heart block was more than 6 years after surgery. Late onset of complete heart block after open heart surgery could be dangerous when presenting without warning. These data would support the notion that patients should be followed for life after repair of congenital heart defects, with special attention to the conduction system, particularly after repair of septal defects.     

Cerebral Near-Infrared Spectroscopy Correlates to Vital Parameters During Cardiopulmonary Bypass Surgery in Children

Near-infrared spectroscopy (NIRS) can monitor changes in cerebral regional oxygen saturation (rSO₂) and tissue hemoglobin content (HbT). The relation between cerebral NIRS readings and vital parameters has not been analyzed before at a fine temporal scale. This study analyzed this relation during cardiopulmonary bypass (CPB) surgery in 10 children (0–9 years, 1,770 min of data records) by using a novel random-coefficient model. The analysis indicated that a small number of patients is sufficient for obtaining significant results with this model. Changes of vital parameters explained 84.7 % of rSO₂ changes and 90.7 % of HbT changes. Cerebral rSO₂ correlated positively with perfusion pressure and inversely with body temperature (P < 0.05). Cerebral HbT correlated positively with perfusion pressure, central venous pressure, and temperature and inversely with arterial oxygen saturation (P < 0.05). During hypothermic circulatory arrest, the half-life of the exponential rSO₂ decay correlated to the rSO₂ reserve (P = 0.016). In conclusion, NIRS readings of cerebral hemoglobin content and tissue oxygen saturation correlate well to vital parameters during CPB surgery in children. NIRS may therefore become a monitoring device for the neuroprotective optimization of those vital parameters.     

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.     

Academic Proficiency in Children After Early Congenital Heart Disease Surgery

Children with early surgery for congenital heart disease (CHD) are known to have impaired neurodevelopment; their performance on school-age achievement tests and their need for special education remains largely unexplored. The study aimed to determine predictors of academic achievement at school age and placement in special education services among early CHD surgery survivors. Children with CHD surgery at <1 year of age from January 1, 1998 to December 31, 2003, at the Arkansas Children’s Hospital were identified. Out-of-state births and infants with known genetic and/or neurologic conditions were excluded. Infants were matched to an Arkansas Department of Education database containing standardized assessments at early school age and special-education codes. Predictors for achieving proficiency in literacy and mathematics and the receipt of special education were determined. Two hundred fifty-six children who attended Arkansas public schools and who had surgery as infants were included; 77.7 % had either school-age achievement-test scores or special-education codes of mental retardation or multiple disabilities. Scores on achievement tests for these children were 7–13 % lower than those of Arkansas students (p < 0.01). They had an eightfold increase in receipt of special education due to multiple disabilities [odds ratio (OR) 10.66, 95 % confidence interval (CI) 4.23–22.35] or mental retardation (OR 4.96, 95 % CI 2.6–8.64). Surgery after the neonatal period was associated with decreased literacy proficiency, and cardiopulmonary bypass during the first surgery was associated with decreased mathematics proficiency. Children who had early CHD surgery were less proficient on standardized school assessments, and many received special education. This is concerning because achievement-test scores at school age are “real-world” predictors of long-term outcomes.     

Prehospital Discharge Car Safety Seat Testing of Infants After Congenital Heart Surgery

Background This study aimed to expand the American Academy of Pediatrics (AAP) car safety seat testing recommendation to include high-risk infants after cardiac surgery. Methods Car safety seat testing (≤4 days before discharge), performed according to AAP guidelines, was retrospectively reviewed for 66 postoperative infants. Failure was defined as apnea, bradycardia, or oxygen desaturation. Results The average birth weight of the study infants was 3.1 ± 0.5 kg. Two patients were born at less than 37 weeks gestation. Surgical procedures included modified Blalock–Taussig shunt technique (n = 15), arterial switch operation (n = 12), Norwood Sano modification (n = 11), coarctation repair (n = 8), repair of tetralogy of Fallot (n = 6), repair of truncus arteriosus (n = 4), repair of total anomalous pulmonary venous return (n = 3), pacemaker placement (n = 2), repair of interrupted aortic arch and ventriculoseptal defect (VSD) (n = 1), repair of coarctation and VSD (n = 1), orthotopic heart transplant (n = 1), repair of VSD (n = 1), and patent ductus arteriosus ligation (n = 1). The average age at discharge was 28 ± 21 days. Four patients (6%) failed car safety seat testing because of a fall in oxygen saturation. One of the four patients passed on retesting after parental education, whereas three of the four (75%) were discharged home in a supine car safety seat. There was no relationship between the type of surgery and car safety seat test failure. Conclusion It may be beneficial to extend the AAP recommendations for car safety seat testing to include high-risk infants after cardiac surgery.     

Junctional Ectopic Tachycardia After Congenital Heart Surgery in the Current Surgical Era

To determine the incidence of postoperative junctional ectopic tachycardia (JET) in a modern cohort of pediatric patients, evaluate possible risk factors for JET, and examine the effects of JET on postoperative morbidity and mortality. JET is common after congenital heart surgery. JET-related mortality has been a rare event at our center, which is different from previous reports. We reviewed records for pediatric patients who had postoperative arrhythmias between January 2006 and June 2010 at a large tertiary-care children’s hospital. We performed a matched case–control study to identify risk factors for JET and a matched-cohort study to compare outcomes between patients and controls. Whenever possible, each JET case was randomly matched to two controls on the basis of lesion, repair, and surgical period. We identified 54 patients with JET (incidence = 1.4 %). After multivariate logistic regression analysis, low operative weight, cardiopulmonary bypass (CPB) duration >100 min, and immediate postoperative serum lactic acid level >20 mg/dl were associated with increased odds of developing JET. Patients with JET had longer mechanical ventilation time, cardiac intensive care unit (CICU) stay, and hospital stay. There was only one death in JET group (1.8 %) with no significant difference compared with the control group. JET remains a relatively common postoperative arrhythmia, but it is less frequent than previously reported. JET occurs more commonly in smaller patients with longer CPB runs and significant postoperative lactic acidosis levels. Mortality associated with JET is lower than historically reported, but morbidity remains high.     

先天性心脏病并肺动脉高压患儿心脏直视手术的麻醉管理

目的探讨婴幼儿先天性心脏病(CHD)并肺动脉高压(PH)心脏直视手术的麻醉管理方法。方法对1999年7月~2002年9月CHD并PH手术婴幼儿87例,加强麻醉手术前准备、合理的选择麻醉药物及给药方法、术中严密监测循环及呼吸、应用血管活性药物,麻醉后密切观察和处理并发症。结果4例因感染和无法脱离体外循环机死亡,余均痊愈出院。结论CHD并PH的婴幼儿心脏直视手术的麻醉管理非常重要。     

Psychosocial Outcomes for Preschool Children and Families After Surgery for Complex Congenital Heart Disease

The purpose of the current study was to assess the psychosocial outcomes of preschool-aged survivors (ages 3–6 years) of hypoplastic left heart syndrome (HLHS; n = 13) and transposition of the great arteries (TGA; n = 13). Parents completed the following measures: Pediatric Quality of Life Inventory, Impact on the Family Scale, Parenting Stress Index, Parent Behavior Checklist, and Child Behavior Checklist. Quality of life scores did not differ from those of healthy controls. Parents of children with HLHS reported more negative impact of the child’s illness on the family and more parenting stress than parents of children with TGA. Parents of both groups of children were more permissive in their parenting style than parents of healthy controls. Children with HLHS had higher rates of attention and externalizing behavior problems than children with TGA. The results highlight the need for practitioners working with these children and families to ask about parental stress, family functioning, and behavioral expectations for the child in the context of routine medical/cardiac follow-up.     

Tissue Doppler Imaging Detects Impaired Biventricular Performance Shortly After Congenital Heart Defect Surgery

Cardiac surgery with cardiopulmonary bypass is associated with the development of a systemic inflammatory response, which can lead to myocardial damage. However, knowledge concerning the time course of ventricular performance deterioration and restoration after correction of a congenital heart defect (CHD) in pediatric patients is sparse. Therefore, the authors perioperatively quantified left ventricular (LV) and right ventricular (RV) performance using echocardiography. Their study included 141 patients (ages 0–18 years) undergoing CHD correction and 40 control subjects. The study assessed LV systolic performance (fractional shortening) and diastolic performance (mitral Doppler flow) in combination with RV systolic performance [tricuspid annular plane systolic excursion (TAPSE)] and diastolic performance (tricuspid Doppler flow). Additionally, systolic (S′) and diastolic (E′, A′, E/E′) tissue Doppler imaging (TDI) measurements were obtained at the LV lateral wall, the interventricular septum, and the RV free wall. Echocardiographic studies were performed preoperatively, 1 day postoperatively, and at hospital discharge after 9 ± 5 days. Although all LV echocardiographic measurements showed a deterioration 1 day after surgery, only LV TDI measurements were impaired in patients at discharge versus control subjects (S′: 5.7 ± 2.0 vs 7.1 ± 2.7 cm/s; E′: 9.8 ± 3.9 vs 13.7 ± 5.1 cm/s; E/E′: 12.2 ± 6.4 vs 8.8 ± 4.3; p < 0.05). In the RV, TAPSE and RV TDI velocities also were impaired in patients at discharge versus control subjects (TAPSE: 9 ± 3 vs 17 ± 5 mm; S′: 5.2 ± 1.7 vs 11.4 ± 3.4 cm/s; E′: 7.3 ± 2.5 vs 16.3 ± 5.2 cm/s; E/E′: 12.5 ± 6.8 vs 4.8 ± 1.9; p < 0.05). Furthermore, longer aortic cross-clamp times were associated with more impaired postoperative LV and RV performance (p < 0.05). In conclusion, both systolic and diastolic biventricular performances were impaired shortly after CHD correction. This impairment was detected only by TDI parameters and TAPSE. Furthermore, a longer-lasting negative influence of cardiopulmonary bypass on myocardial performance was suggested.     

Renal Function in Children with Cyanotic Congenital Heart Disease: Pre- and Post-Cardiac Surgery Evaluation

Objective: Cyanotic congenital heart diseases (CCHDs) are a series of cardiac anomalies that have long been recognized as a potential cause of nephropathy. There have been few reports on renal impairment in patients with CCHD before and after corrective cardiac surgery. The aim of this study was to evaluate the prevalence of renal dysfunction before and after cardiac surgery and the impact of some risk factors on final renal outcome. Methods: Thirty children with CCHD who had done corrective cardiac surgery in the previous 6 months were enrolled in this study. All data prior to surgery were collected from the charts. Post-operation data including blood and spot urine samples were taken simultaneously for CBC, Cr, and uric acid and 24 hour urine was collected for microalbumin and Cr during the follow up visits. Pre- and post-operation parameters were compared to study the impact of cardiac surgery on renal function. Findings : Pre- and post-operative GFRs were not significantly different. Final GFR was significantly and inversely associated with pre- and post-operation age (P=0.008 r=-0.48, P=0.03 r=-0.38). Three (10%) patients had microalbuminuria. The prevalence of microalbuminuria in children older than 10 years was 30%. There was no link between microalbuminuria and age, GFR, and hematocrit (P=0.1, P=0.3, P=0.3, respectively). Patients with preoperation hematocrit >45 had a significantly lower final GFR compared to children with HCT <45 (83.7±6.5 vs 111.10.2, P=0.001). The mean uric acid fraction (FEua) excretion was 8.21±4.75. Pre-operative HCT was inversely associated to FEua (P=0.01, r=-0.44). There was no relationship between FEua and age, serum uric acid, and GFR (P=0.7, P=0.4, P=0.2). Conclusion: Children with CCHD are at increased risk of renal injury which is related more to the duration of cyanosis and higher degree of hematocrit level. To lower the risk, corrective cardiac surgery is recommended to be done as soon as possible to improve renal function and stop more renal impairment.Key Words: Congenital Heart Disease, Nephropathy, Cardiac Surgery, Cyanosis, Microalbuminuria     

Neurodevelopmental Outcomes Following Congenital Heart Surgery

Advances in both surgical techniques and perioperative care have led to improved survival outcomes in infants and children undergoing surgery for complex congenital heart disease. An awareness is emerging that early and late neurological morbidities complicate the outcome of these operations. Adverse neurological outcomes after neonatal and infant cardiac surgery are related to both fixed and modifiable mechanisms. Fixed factors include many variables specific to the individual patient, including genetic predisposition, gender, race, socioeconomic status, and in utero central nervous system development. Modifiable factors include not only intraoperative variables (cardiopulmonary bypass, deep hypothermic circulatory arrest, and hemodilution) but also such variables as hypoxemia, hypotension, and low cardiac output. The purpose of this review is to examine these mechanisms as they relate to available outcome data.     

Regionalization in Neonatal Congenital Heart Surgery: The Impact of Distance on Outcome After Discharge

Studies have shown improved perioperative outcomes after neonatal heart surgery at centers with greater surgical volumes. The impact of increasing distance from such centers on outcome after discharge has not yet been reported. Chart review and cross-sectional survey were performed on children discharged or transferred after undergoing neonatal congenital heart surgery as neonates (<30 days of age) from January 2005 to June 2006. The association of distance from center with mortality and adverse events was analyzed by univariate and multivariate regression and stratified by the Risk Adjustment for Congenital Heart Surgery-1 for complexity. Among 217 patients, those living further from the surgical center were smaller and older at surgery and more likely to be RACHS-1 class 6. Overall mortality was 8% (16 of 202) and was not associated with distance. Surveys were completed by 109 (54%) families with mean follow-up of 24 (±3) months. Unplanned admissions after discharge and unplanned interventions occurred in 45% and 40% of patients, respectively. After adjusting for case complexity, living 90–300 min away from the surgical center was associated with fewer unplanned admissions compared with those living <90 min away. After neonatal cardiac surgery, adverse events were common. Distance from the surgical center was not associated with mortality, but it was associated with morbidity in a nonlinear fashion. This relation, its mediators, and its possible impact on mortality and later outcomes warrant further study to aid in planning appropriate patient follow-up.     

Pressure-Regulated Volume Control vs Volume Control Ventilation in Infants After Surgery for Congenital Heart Disease

The objective of this investigation was to compare how two modes of positive pressure ventilation affect cardiac output, airway pressures, oxygenation, and carbon dioxide removal in children with congenital heart disease in the immediate postoperative period. The investigation used a one group pretest–post-test study design and was performed in the pediatric cardiac intensive care unit in a university-affiliated children's hospital. Nine infants were enrolled immediately after repair of tetralogy of Fallot (2) or atrioventricular septal defects (7) with mean weight = 5.5 kg (4.2–7.3 kg). Children were admitted to the pediatric cardiothoracic intensive care unit after complete surgical repair of their cardiac defect and stabilized on a Siemen's Servo 300 ventilator in volume control mode (VCV1) (volume-targeted ventilation with a square flow wave pattern). Tidal volume was set at 15 cc/kg (total). Hemodynamic parameters, airway pressures and ventilator settings, and an arterial blood gas were measured. Patients were then changed to pressure-regulated volume control mode (PRVC) (volume-targeted ventilation with decelerating flow wave pattern) with the tidal volume set as before. Measurements were repeated after 30 minutes. Patients were then returned to volume control mode (VCV2) and final measurements made after 30 minutes. The measurements and results are as follows: After correction of congenital heart defects in infants, mechanical ventilation using a decelerating flow wave pattern resulted in a 19% decrease in peak inspiratory pressure without affecting hemodynamics, arterial oxygenation, or carbon dioxide removal.     

Completion Angiography After Cardiac Surgery for Congenital Heart Disease: Complementing the Intraoperative Imaging Modalities

Residual structural pathology after cardiac surgery for congenital heart disease may complicate postoperative recovery. Completion angiograms obtained in the operating room may facilitate early detection and therapy of residual structural abnormalities. Our objective here is to report our institutional experience performing completion angiograms after cardiopulmonary bypass surgery. Between October 2007 and August 2008, 31 patients underwent completion angiograms after 32 cardiac surgical procedures. The median age was 7.5 months (range, 50 days to 31.2 years) and the median weight was 6.5 kg (range, 3.1–153 kg). Type of procedure, angiographic findings, and therapeutic decision were retrospectively reviewed. Procedures (proc) evaluated through completion angiography included comprehensive stage II or Glenn (n = 13), aortic arch reconstruction/conduit (n = 3), repair/palliation of tetralogy of Fallot or pulmonary atresia with ventricular septal defect (n = 4), PVR or conduit replacement (n = 5), and others (n = 7). Unexpected pathology was identified in 18 of 32 (56.3%) proc, which included left pulmonary artery (LPA) stenoses (n = 15), right pulmonary artery (RPA) stenoses (n = 11), and stenosis impairing coronary blood flow (after DKS; n = 1). In 9 of 32 (28.1%) proc, findings may have led to a change in therapeutic management. This included surgical revision (n = 1), ‘Hybrid’ therapy in the same setting (n = 2: LPA stent, 1; RPA balloon, 1), early catheterization within 3 months (n = 4), and change in medical management (n = 2: change in anticoagulation, 1; early CT, 1). Complications related to completion angiography were seen in only a single procedure (LPA staining). In conclusion, completion angiograms using a dedicated Hybrid cardiac operating suite may aid in early diagnosis and therapy of postsurgical abnormalities. They complement other methods of intraoperative imaging and may reduce the potential need for early surgical or transcatheter reintervention.     

Congenital Hydrocolpos Combined with Unilateral Renal Agenesis

Herein we report an infantile case of congenital hydrocolpos combined with left renal agenesis which presented with an abnormal mass protruding from the vaginal introitus. The diagnosis was made shortly after birth by ultrasonography and confirmed by other radiologic diagnostic methods. This case highlights the value of ultrasonography in diagnosing hydrocolpos and determining the coexistence of renal anomalies.     

Relation Between Red Cell Distribution Width and Clinical Outcome After Surgery for Congenital Heart Disease in Children

Recent studies have reported a strong association between increased red cell distribution width (RDW) and the risk of adverse outcomes for adults with heart failure. This study investigated the association between preoperative RDW and postoperative clinical outcomes for children with cardiac disease. The relation between preoperative RDW and the length of postoperative stay was tested with 688 consecutive children undergoing surgery for congenital heart disease (CHD). The RDW was significantly higher in patients who died during the postoperative hospital stay (mean, 18.34?±?4.69 vs 16.12?±?2.84; p?=?0.004). The risk of postoperative death was five times higher for patients with an RDW of 16% or more. In the general study population, RDW correlated with the intensive care unit (ICU) stay (p?<?0.0001) and with the total hospital stay in the local population (p?<?0.0001). The correlation between RDW and ICU stay was stronger for patients with acyanotic CHD (p?<?0.0001) than for those with cyanotic CHD (p?=?0.0007), and for the subpopulation of patients with acyanotic CHD and normal hemoglobin level (p?<?0.0001) than for anemic patients with acyanotic CHD (p?=?0.025). Preoperative RDW is a strong predictor of an adverse outcome in children undergoing surgery for CHD, especially in nonanemic patients, for whom it reflects an underlying inflammatory stress.