Analysis of postoperative reoperation for congenital duodenal obstruction

OBJECTIVE: To analyse the risk factors for reoperation after initial surgical repair of congenital duodenal obstruction and demonstrate that they can be decreased with more careful attention and more advanced techniques during surgery. METHODS: The records of newborns and infants (aged 0-2 months) who had surgical therapy for congenital duodenal obstruction in the past 30 years were reviewed and analysed. Of the 298 patients, 132 (44%) were boys and 166 (56%) were girls. All patients who underwent repeat surgery postoperatively were evaluated by the reasons for surgery. The number of patients with various combination lesions of congenital duodenal obstruction was also calculated and the relationship to postoperative reoperation was analysed. RESULTS: Twenty patients (6.7%) had congenital duodenal obstruction with combination lesions including duodenal web, malrotation, annular pancreas and multiple duodenal web. Twelve patients required further operation 5 days to 2 years postoperatively for complications (n = 5) and other duodenal atresias that were not discovered initially (n = 7). CONCLUSION: More than half of reoperated patients (7/12) had multiple lesions of duodenal obstruction that were missed during the primary operation. The postoperative reoperation rate for congenital duodenal obstruction could be decreased with more careful attention to operative details and more preoperative and intraoperative evaluation of the gastrointestinal tract.     

Anterior translocation of the right pulmonary artery for relief of airway compression in the repair of distal aortopulmonary window and interrupted aortic arch

Airway compression by dilated right pulmonary artery (RPA) in infants with congenital heart disease can cause severe respiratory insufficiency and increase postoperative morbidity. Anterior aortopexy can be a good solution. However, in a case in which aortic arch repair is required, anterior aortopexy might not be effective or can be dangerous. Anterior translocation of the RPA can be a good option to avoid postoperative airway obstruction. We adopted this technique in a one-stage complete repair of distal aortopulmonary window, aortic origin of the RPA, interrupted aortic arch, and ventricular septal defect in a neonate with compression of both main bronchi. After 37 months of follow-up, no problem was encountered with her airway or RPA. We describe the technique used and the results obtained.     

婴幼儿先天性气管狭窄合并先天性心脏病一期手术纠治

目的总结婴幼儿先天性气管狭窄伴先心病一期纠治经验和效果。方法回顾性分析2012年我院收治的12例先天性气管狭窄合并先心病患儿的临床资料。患儿平均年龄15.75月,平均体重9.38 kg。所有病例均在浅低温或中低温体外循环下行先心病纠治术,同时行气管成形术。结果所有患儿手术均成功,其中10例采用Slide气管成形法,2例采用端端吻合法。术后并发症包括肺炎4例,气道分泌物增多5例,气道高反应4例,肉芽组织生长3例,其中2例最终呼吸衰竭死亡;其余患者术后恢复良好。结论小儿先天性气管狭窄的处理仍较困难,Slide手术方法是目前处理主气管狭窄的最佳手术方式,特别适合于长段和弥漫性气管狭窄的患者。但是,先天性气管狭窄患者的治疗仍需要以气管成形手术为中心的综合治疗。     

A glial-derived protein,S100B,in neonates and infants with congenital heart disease: evidence for preexisting neurologic injury

The glial-derived protein S100B is a serum marker of cerebral ischemia and correlates with negative neurological outcome after cardiopulmonary bypass (CPB) in adults. We sought to characterize the S100B release pattern before and after CPB in neonates and infants with congenital heart disease and correlate it with surgical mortality. Serum was collected before surgery and at 24 postoperative h from 109 neonates and infants with congenital heart disease. All patients had presurgical transthoracic echocardiograms and CPB with or without hypothermic circulatory arrest. S100B concentrations were determined using a two-site immunoluminometric assay (Sangtec 100). Thirty-day surgical mortality was observed. All neonates had significantly increased S100B concentrations before surgery that decreased by 24 postoperative h. Preoperative S100B concentrations in 32 neonates with hypoplastic left heart syndrome correlated inversely with the forward flow and size of the ascending aorta and postoperative mortality (r(2) = -0.63; P = 0.03). Among infants, increased pulmonary blood flow was associated with higher S100B levels before surgery than cyanosis. There was no correlation with postoperative S100B and time on CPB, hypothermic circulatory arrest, or 30-day surgical mortality. In conclusion, preoperative S100B concentrations correlate inversely with the size of the ascending aorta in hypoplastic left heart syndrome and may serve as a marker for preexisting brain injury and mortality. IMPLICATIONS: Neonates with hypoplastic left heart syndrome and no forward flow in the ascending aorta may have brain injury at birth before heart surgery.     

Surgical treatment of airway obstruction associated with congenital heart disease in infants and small children

In the last 12 years, 21 patients age 1 month to 5 years (median, 7 months) underwent surgical treatment for severe airway obstruction associated with congenital heart disease. Plico-suspension of the pulmonary artery was carried out in 14 patients with documented severe left bronchial compression by a distended pulmonary artery together with repair of ventricular septal defects (11 patients), repair of large coronary artery fistula (1), division of ductus (1) and pulmonary artery banding (1), with subsequent significant relief of compression. Aortopexy was used in 2 patients with documented severe tracheal compression by a right aortic arch. Five patients had congenital tracheal stenoses (3 extensive, 2 localized). Two of 3 patients with associated complex intracardiac anomalies underwent concomitant repair of both lesions with the use of cardiopulmonary bypass and 1 had a tracheoplasty and pulmonary artery banding. Tracheal reconstruction with a cartilaginous graft together with repair of pulmonary artery sling was carried out in 2 infants. Nineteen patients are alive and well, at a mean follow-up of 87 months. In conclusion, we advocate early aggressive surgical intervention to both lesions to obtain a better result in the management of infants and small children with this difficult and often fatal combination.     

Elective extracorporeal support for complex tracheal reconstruction in neonates

BACKGROUND: Congenital obstructive anomalies of the trachea present unique challenges in reconstruction and perioperative airway management. Complications include anastomotic breakdown, leak and granulation formation related to the complexity of the repair, and difficulties with perioperative airway management. We describe our technique of elective intraoperative and postoperative extracorporeal support to improve surgical exposure and postoperative healing. METHODS: We have performed complex tracheal reconstructions in 4 newborns (2.2 to 4.3 kg) for long segment tracheal stenosis and complete tracheal rings, diagnosed with bronchoscopy and computerized tomography. Three of the 4 infants had other significant anomalies including complex congenital heart disease, hydrocephalus, encephalomalacia, left lung agenesis, facial anomalies, vertebral anomalies, and hand and hip anomalies. The repairs were performed through a median sternotomy using an extracorporeal membrane oxygenation circuit for support. Venoarterial support was used for the sliding tracheoplasty reconstruction. Extracorporeal membrane oxygenation was converted to venovenous for postoperative "airway rest." After diuresis, the lungs were reexpanded and the 4 patients were ventilated and removed from extracorporeal membrane oxygenation at 4, 5, 8, and 9 days postoperatively. Bronchoscopy was performed to evaluate the airway. RESULTS: All patients had excellent healing of the trachea without granulation tissue. There were no complications of extracorporeal membrane oxygenation support or bleeding issues. All 4 patients survived the surgery and immediate postoperative period with 2 late deaths. The child with congenital heart disease expired after 8 weeks after having hepatorenal failure develop. The child with Goldenhar's syndrome and a single left lung died after 5 months in the hospital. The other 2 patients survived. Two of the infants required late tracheostomy for facial and laryngeal anomalies. CONCLUSIONS: Extracorporeal membrane oxygenation provides an excellent environment for complex tracheal reconstruction and promotes postoperative healing by minimizing trauma to the reconstructed airway.     

Preoperative risk-of-death prediction model in heart surgery with deep hypothermic circulatory arrest in the neonate

OBJECTIVE: Our goal was to generate a preoperative risk-of-death prediction model in selected neonates with congenital heart disease undergoing surgery with deep hypothermic circulatory arrest. METHODS: We completed a single-center, prospective, randomized, double-blind, placebo- controlled neuroprotection trial in selected neonates with congenital heart disease requiring operations for which deep hypothermic circulatory arrest was used. An extensive database was generated that included preoperative, intraoperative, and postoperative variables. Variables (delivery, maternal, and infant related) were evaluated to produce a preoperative risk-of-death prediction model by means of logistic regression. An operative risk-of-death prediction model including duration of deep hypothermic circulatory arrest was also generated. RESULTS: Between July 1992 and September 1997, 350 (74%) of 473 eligible infants were enrolled with 318 undergoing deep hypothermic circulatory arrest. The mortality was 52 of 318 (16.4%), unaffected by investigational drug. The resulting preoperative risk model contained 4 variables: (1) cardiac anatomy (two-ventricle vs single ventricle surgery, with/without arch obstruction), (2) 1-minute Apgar score (5), (3) presence of genetic syndrome, and (4) age at hospital admission for surgery (5 days). Mortality for two-ventricle repair was 3.2% (4/130). Mortality for single ventricle palliation was 25.5% (48/188) and was significantly influenced by Apgar score, genetic diagnosis, and admission age. The preoperative model had a prediction accuracy of 80%. The operative risk model included duration of deep hypothermic circulatory arrest, which significantly (P =.03) increased risk of death, with a prediction accuracy of 82%. CONCLUSIONS: In this selected population, postoperative mortality risk is significantly affected by preoperative conditions. Identification of infants with varying mortality risks may affect family counseling, therapeutic intervention, and risk stratification for future study designs.     

Rigid bronchoscopy in the pediatric age group: diagnostic effectiveness.

Over a 15-year period, 277 diagnostic bronchoscopic procedures were carried out at the Winnipeg Children's Hospital using rigid bronchoscopic instrumentation. The objective of the review is to examine contribution to final diagnosis made by the procedure. The patient population included 60% male and 40% female patients ranging from the first day of life to 18 years (mean age, 6 years). Indications for bronchoscopy fell into two large groups, including 60% of patients with evidence of lower airway disease and 30% of patients with evidence of upper airway obstruction. In patients with upper airway obstruction, half were found to have a congenital underlying cause and half were due to an acquired lesion. In 85% of patients, a specific diagnosis was reached and this proved to correlate positively with the preoperative diagnosis in 80% of patients and negatively in 20%. Definitive treatment in patients with upper airway obstruction included surgical intervention in one third of patients. Patients with lower airway disease were diagnosed as having consolidation in 43%, atelectasis in 39%, and bronchiectasis in 18%. Disease localized most frequently to the left lower and right upper lobes and in only 10 of 168 patients was a congenital cause determined. Among 168 patients, 30 had surgical treatment as the definitive management with the majority of patients treated medically. Bronchoscopy was shown to contribute to diagnosis in 88% of patients examined. Bronchoscopy was carried out with a complication rate of 3% and no mortality.     

Intraoperative transoesophageal echocardiography for paediatric cardiac surgery--an audit of 200 cases

We report out initial experience with intraoperative transoesophageal echocardiography (TOE) in 200 patients undergoing repair of congenital heart disease. Complications associated with probe insertion precluded a full study in 11 patients (5.5%) and included airway obstruction (n = 6), inability to pass the probe (n = 4) and vascular compression (n = 1). The preoperative diagnosis was confirmed by TOE in 176 of 189 cases (93%) with minor variances in 12 (6.3%) and one major variance (additional large muscular ventricular septal defect (VSD)). Unexpected abnormalities not diagnosed preoperatively were found at surgery in four cases (2%) and were all of trivial operative significance. Postoperative studies showed no residual defect in 96 (51%) and trivial or mild residual defects in 72 patients (38%). There were moderate or severe residual defects in 21 cases (11%). Ten cases (5.3%) returned to bypass for further surgery, with eight achieving complete or adequate amelioration. In six of the 10 cases, return to bypass was prompted by the TOE examination alone. There was one re-operation in the postoperative period for a VSD patch dehiscence. Routine TOE offers significant advantages in the management of patients undergoing repair of congenital heart disease, particularly in postoperative assessment. Careful monitoring of perfusion and ventilation is required, particularly during probe insertion in children weighing 5 kg or less.     

Cardiovascular causes of airway compression

Compression of the paediatric airway is a relatively common and often unrecognized complication of congenital cardiac and aortic arch anomalies. Airway obstruction may be the result of an anomalous relationship between the tracheobronchial tree and vascular structures (producing a vascular ring) or the result of extrinsic compression caused by dilated pulmonary arteries, left atrial enlargement, massive cardiomegaly, or intraluminal bronchial obstruction. A high index of suspicion of mechanical airway compression should be maintained in infants and children with recurrent respiratory difficulties, stridor, wheezing, dysphagia, or apnoea unexplained by other causes. Prompt diagnosis is required to avoid death and minimize airway damage. In addition to plain chest radiography and echocardiography, diagnostic investigations may consist of barium oesophagography, magnetic resonance imaging (MRI), computed tomography, cardiac catheterization and bronchoscopy. The most important recent advance is MRI, which can produce high quality three-dimensional reconstruction of all anatomic elements allowing for precise anatomic delineation and improved surgical planning. Anaesthetic technique will depend on the type of vascular ring and the presence of any congenital heart disease or intrinsic lesions of the tracheobronchial tree. Vascular rings may be repaired through a conventional posterolateral thoracotomy, or utilizing video-assisted thoracoscopic surgery (VATS) or robotic endoscopic surgery. Persistent airway obstruction following surgical repair may be due to residual compression, secondary airway wall instability (malacia), or intrinsic lesions of the airway. Simultaneous repair of cardiac defects and vascular tracheobronchial compression carries a higher risk of morbidity and mortality.     

低出生体重儿及早产儿心脏手术的早期疗效分析

目的分析60例先天性心脏病(先心病)低出生体重儿(low birth weight infant)和早产儿行外科手术治疗的早期疗效。方法回顾性分析自2003年5月至2011年10月广东省心血管病研究所60例患先天性心脏病的低出生体重儿(体重2 500 g)和早产儿施行心脏手术治疗的临床资料,其中男43例,女17例;年龄4～55(24.9±12.5)d;孕周26～42(33.5±4.1)周,其中47例为早产儿;出生时体重640～2 500(1 729.3±522.5)g,手术时体重650～2 712(1 953.2±463.6)g。术前所有患者均在新生儿重症监护病房(NICU)接受治疗,在全身麻醉下行先心病矫治手术,其中行非体外循环手术29例,体外循环手术31例。术后住NICU进行监护治疗,并观察并发症发生情况和死亡情况。结果住院期间共死亡13例,总病死率为21.7%(13/60)。其中术中死亡4例,术后早期(72 h内)死亡6例,放弃治疗3例。体外循环时间(121.0±74.7)min,升主动脉阻断时间(74.8±44.7)min,术后呼吸机辅助呼吸时间(136.9±138.1)h。术后延期关胸13例,床旁开胸探查止血8例,发生重症肺炎10例,肺动脉高压危象2例,低心排血量综合征8例,均经相应的治疗治愈或好转。随访47例,随访时间2～12个月,患者均生存。结论对危重先心病低出生体重儿或早产儿早期施行外科手术治疗是安全、有效的。     

787例小于6月龄先天性心脏病患者外科治疗的临床分析

目的回顾性总结婴幼儿先天性心脏病的手术时机和手术治疗方法,以进一步提高手术成功率和远期疗效。方法1988年1月～2003年6月,手术纠治年龄小于6个月的先天性心脏病患者787例。主要病种包括完全性大动脉错位109例,完全性肺静脉异位引流51例,肺动脉闭锁16例,主动脉缩窄33例,室间隔缺损伴肺动脉高压299例,法洛四联症44例,右心室双出口23例,室间隔完整型肺动脉闭锁9例等;对787例患者根据不同病种采取相应的手术方法纠治。结果手术死亡77例,手术死亡率9.78%(77/787)。随着手术方法的不断改进,手术总死亡率从1988～1995年的25%降至2003年的4.11%。随访完全性大动脉错位患者中发生VSD残余漏1例,术后3个月再次手术治愈;随访中发生肺动脉和主动脉瓣上狭窄2例。完全性肺静脉异位引流心内型患者中2例分别在术后4d和2个月出现肺静脉回流梗阻,1例死亡,1例再次手术解除梗阻。室间隔缺损患者中发生残余漏5例,分流量小,不需再次手术。其余病例随访资料不完整。结论对婴幼儿先天性心脏病施行手术治疗的时间非常重要,危重复杂型先天性心脏病患者如不早期手术,将失去手术机会,增加术后危险性和死亡率。     

Ketorolac after congenital heart surgery: does it increase the risk of significant bleeding complications?

BACKGROUND: The routine use of ketorolac after congenital heart surgery in infants and children is limited by concerns for postoperative bleeding complications. The object of this study was to determine if the use of ketorolac is associated with an increased risk of significant postoperative bleeding after congenital heart surgery in infants and children. METHODS: A retrospective chart review was performed. The exposure of interest was postoperative use of ketorolac after congenital heart surgery in infants and children. The outcome measured was postoperative bleeding requiring surgical exploration. The patients who received ketorolac were compared with an age- and diagnosis-matched comparison group who did not receive ketorolac. RESULTS: Records of 842 infants and children who underwent congenital heart surgery between July 2001 and October 2002 were reviewed. 94 (11.1%) patients were treated with ketorolac postoperatively. The comparison group consisted of 94 matched subjects selected from the patients that did not receive ketorolac. The mean age of patient in the ketorolac group was 8.5 (+/-6.1) years. No (0%) patients in the ketorolac group and four (4.2%) patients in the nonketorolac group developed postoperative bleeding requiring surgical exploration. The relative risk for postoperative bleeding that required surgical exploration in the ketorolac group compared with the nonketorolac group was 0.2 (95% CI 0.02-1.67). CONCLUSIONS: The use of ketorolac after congenital heart surgery in infants and children does not significantly increase the risk of bleeding complications requiring surgical exploration.     

Prenatal diagnosis of congenital heart disease affects preoperative acidosis in the newborn patient

OBJECTIVES: Congenital heart disease is the leading cause of death in the first year after birth. Prenatal diagnosis of the disease can optimize the preoperative condition of the patient and may help in the prevention of acidosis. In this retrospective study we compared the occurrence of metabolic acidosis in patients with and without prenatal diagnosis of a congenital heart disease. METHODS: Data of 408 patients who needed an operation for congenital heart disease within 31 days of life were analyzed retrospectively. Arterial blood gases at fixed time intervals and worst blood gas of 81 patients with and 327 patients without a prenatal diagnosis were compared, categorizing the patients on ductus dependency, anticipated univentricular or biventricular repair, and left-sided, right-sided, or no heart obstruction. RESULTS: In the overall group significant differences in lowest pH, lowest base excess, and highest lactate level were found, with metabolic acidosis more common among the patients with a postnatal diagnosis. In the group of patients with ductus-dependent congenital heart disease, the difference between patients receiving a prenatal and those receiving a postnatal diagnosis was more significant than in the group with non-ductus-dependent lesions. Analyzing patients with right-sided, left-sided, and no obstruction separately, significant differences were found in the group with left-sided heart obstruction for lowest pH and base excess and in the group with right-sided heart obstruction for lowest base excess. CONCLUSIONS: Prenatal diagnosis of congenital heart disease minimizes metabolic acidosis in patients with congenital heart disease and may be associated with improved long-term outcome and prevention of cerebral damage among this fragile group of patients, although no significant effect on direct surgical outcome was encountered.     

Congenital dacryocystocele: five clinical cases

Congenital dacryocystocele (CDC) is recognised as a cause of nasal airway obstruction or respiratory distress in newborns. CDC is caused by the distal obstruction of the lachrymal duct and presents as a cystic formation in the inferior meatus. We discuss five cases of dacryocystocele, together with surgical management and outcome. Endoscopic endonasal marsupialisation and appropriate postoperative care resulted in definitive recovery for all patients. In newborns or infants with nasal obstruction, CDC should be considered in the differential diagnosis, and prompt endoscopic endonasal marsupialisation is mandatory.     

Posterior aortic plication for tracheal compression by aortic dilatation in infants with congenital heart disease

Two infants with congenital heart disease developed life-threatening airway obstruction due to progressive dilatation of the aorta. Both underwent posterior aortic plication with extracorporeal bypass (9 and 4 months of age). After surgery, the arterial diameter was adequately reduced with a smooth aortic route leading to a wide airway space. Both patients were weaned from the ventilator shortly after surgery and are currently asymptomatic.     

Management of ureteropelvic junction obstruction in infants and neonates

From June 1980 to October 1985 we performed 22 ureteropyeloplasties on 20 infants and neonates with ureteropelvic junction obstruction. All patients were males less than 2 years old; 12 were less than 1-month-old. The diagnosis was suspected on the basis of maternal ultrasonography in 10 patients (50%), a palpable abdominal mass was the presenting symptom in 7 (35%), and obstruction was detected during evaluation of congenital heart disease in 3 (15%). This change in mode of presentation is expected to become even more pronounced as the use of fetal ultrasonography increases. All patients in whom the diagnosis was suspected antenatally underwent ultrasonography after birth to confirm the presence of hydronephrosis. All 22 obstructed kidneys were repaired by a dismembered technique under optical magnification. There were no operative mortalities. Of 21 postoperative intravenous pyelograms available, 3 demonstrated marked improvement, 17 showed improvement with residual hydronephrosis, and 1 showed deterioration. The methods used to diagnose, treat and evaluate ureteropelvic junction obstruction in 20 infants and neonates are presented. Given the existing clinical and experimental data we advocate early postnatal surgical correction of ureteropelvic junction obstruction to achieve a maximum recovery of renal function.     

Compression of the central airways by a dilated aorta in infants and children with congenital heart disease.

BACKGROUND: Children with congenital heart disease often experience respiratory symptoms in the preoperative and perioperative periods, which can complicate their management. An uncommon but important cause of respiratory insufficiency in such children is external airway compression. METHODS: We operated on 5 patients (median age, 6 months) with significant respiratory distress attributable to compression of the central airways by a dilated ascending aorta before or after repair of concomitant cardiovascular defects. Four of these patients had right aortic arch and 3 had pulmonary atresia with a ventricular septal defect and major aortopulmonary collaterals. In all patients, aortopexy was performed at the time of operation for the cardiovascular defects (n = 3) or after symptoms developed in the postoperative period (n = 2). The 3 patients in whom airway compression produced symptoms preoperatively also underwent reduction ascending aortoplasty. RESULTS: Symptoms resolved immediately after operation in 3 patients, whereas symptoms persisted in the other 2 patients and tracheostomy was required. At follow-up of 20 months to 5 years, all patients are alive and well, with mild or moderate respiratory symptoms in the 2 patients who required tracheostomy, both of whom were decannulated within 13 months. CONCLUSIONS: External airway compression can cause significant morbidity in patients with congenital heart defects other than vascular rings. In patients with respiratory symptoms in the context of a lesion that involves increased aortic outflow during intrauterine life and consequently, an enlarged ascending aorta, such as tetralogy of Fallot with pulmonary atresia, airway compression should be considered as a cause, especially if a right aortic arch is present or the patient also has pulmonary atresia with a ventricular septal defect and collaterals. Attempts to address this problem surgically may provide substantial relief, but increasing duration of airway compression is likely to lead to tracheal or bronchial malacia and persistent symptoms even after the compression is relieved.     

Inguinal hernia repair in the perinatal period and early infancy: clinical considerations

Contemporary neonatal intensive care has resulted in survival of many seriously ill preterm and older infants that frequently present with symptomatic inguinal hernia. Controversy exists concerning timing and safety of early repair in prematures or other neonates, especially those hospitalized with concurrent illness. This study examines this topic by evaluating predisposing factors, presentation, and postoperative complications in 100 recent consecutive hernia repairs in previously hospitalized infants less than 2 months of age. There were 85 boys and 15 girls. Thirty percent were premature (less than 36 wks gestation). Forty-two infants were hospitalized for RDS with assisted ventilation in 16 infants, hydrocephalus and ventriculoperitoneal (VP) shunt in 7 infants, and congenital heart disease (CHD) in 19 infants. Clinical presentation was on the right side in 44 infants, bilateral in 42, and on the left side in 14. Incarceration occurred in 31 cases with nine babies having overt intestinal obstruction. The incidence of cryptorchidism was 12.9%. All (VP) shunt, CHD patients, and incarcerated cases were treated with preoperative antibiotics. Following discharge, 49 preterm or previously ill infants developed a symptomatic hernia at home and were readmitted. Nine full-term infants were treated as outpatients. Bilateral inguinal exploration was performed in 92 cases with second hernia or patent processus found in 81. Seven of eight with unilateral exploration had acute incarceration with obstruction at the time of the procedure. Three subsequently required a second hernia repair. Two infants with incarceration and cryptorchid testis or ovarian slider had gonadal infarction. There were eight postoperative complications.(ABSTRACT TRUNCATED AT 250 WORDS)     

The significance of baseline cerebral oxygen saturation in children undergoing congenital heart surgery

OBJECTIVES: Despite recent significant improvement in outcome, children undergoing surgery for correction of congenital heart defects have a persistent and troublesome mortality rate and incidence of neurologic complications. Recent data suggest that some congenital heart defects are associated with abnormal brain development and with low cerebral blood flow. We hypothesized that some children with congenital heart disease have an abnormally low baseline (preoperative) cerebral oxygen saturation (ScO2). METHODS: ScO2 was continuously recorded intraoperatively in 143 infants and children (age <18 years) undergoing repair of congenital heart defects on cardiopulmonary bypass. Baseline saturation was obtained prior to induction of anesthesia. Preoperative and postoperative saturations were correlated with the patient's physiology (cyanotic vs. acyanotic, presence of ventricular- or arterial-level left-to-right shunts) and outcome. RESULTS: Patient age ranged from 2 days to 17 years (median 8 months). Mean baseline ScO2 was 64%. Preoperative ScO2 was lower in infants with left to right shunt physiology (P < .01), but not in cyanotic infants without left-to-right shunts. Perioperative death was associated with baseline saturation less than 50%. CONCLUSIONS: Baseline ScO2 is lower in patients with left-to-right shunt physiology. Postoperative saturation is lower in patients with left-to-right shunt physiology and in cyanotic patients. Low baseline ScO2 predicts perioperative mortality in children with congenital heart disease. Measurement of ScO2 preoperatively will provide additional information for parent counseling, and preoperative optimization of ScO2 may improve outcome.