Cardiac Magnetic Resonance Imaging (MRI) in Children is Safe with Most Pacemaker Systems, Including Those with Epicardial Leads.

Magnetic resonance imaging (MRI) of patients with pacemakers remains concerning because of possible magnetic field effects on the device. Many pacemaker models are labeled as non-conditional, or contraindicated for MRI, or do not have any specific safety guidelines listed. This study describes our experience with pacemaker function and adverse events in pediatric and young adult patients after clinically indicated MRI scanning at 1.5 Tesla (T). We hypothesized that generator battery voltage, pacemaker lead threshold, and lead impedance would not be altered by MRI. This was a retrospective review of Children's Wisconsin clinical MRI data for all patients with pacemakers scanned between January 1, 2010 and March 31, 2018. Pacemakers were interrogated by the Electrophysiology Team before and immediately after MRI and at outpatient follow up. Twenty-one patients underwent forty-four MRI scans. No significant immediate changes were seen in any pacemaker parameter for any manufacturer/model/lead at the time of MRI. At first clinical follow up post MRI, (median 4.4 months, range 0.2-12.3), battery voltage was reduced (2.78 V pre-MRI versus 2.77 V at follow up, p = 0.02), but there were no other significant changes. No adverse events were noted. Pediatric patients with pacemakers, including those with epicardial leads, can be scanned at 1.5 T safely without alteration in pacemaker function. Using appropriate precautions, pediatric patients with pacemakers can be imaged with MRI.

Anatomic Repair of Congenitally Corrected Transposition of the Great Arteries: Single-Center Intermediate-Term Experience.

We present our experience for patients who have undergone an anatomic repair (AR) for congenitally corrected transposition of the great arteries (CCTGA) at the Children's Hospital of Wisconsin. A retrospective chart review of patients who underwent AR for CCTGA from 2001 to 2015 was performed. The cohort consisted of 15 patients (74% male). Median age of anatomic repair was 15 months (range 4.5-45.6 months). Four patients had a bidirectional Glenn (BDG) prior to AR. At the time of AR,-9 (60%) underwent Senning/Rastelli procedure, 4 (26.6%) had double switch operation, and 2 (13.3%) underwent only Senning with VSD closure. Median duration of follow-up was 5.5 years (0.05-14 years). Reoperations prior to discharge included BDG, revision of pulmonary venous baffle, closure of residual VSD, and pacemaker placement. Late reoperations included left ventricular outflow tract obstruction repair, conduit replacement, melody valve placement, and pacemaker implantation. At their most recent follow-up, no patient had heart failure symptoms and only 1 had severely diminished function that improved with cardiac resynchronization therapy. Moderate mitral regurgitation was noted in 15% (2/13), and severe in 7% (1/13). Moderate tricuspid regurgitation was noted in 15% (2/13). One patient, 7% (1/13), developed moderate aortic insufficiency. There was a 100% survival at the time of the most recent follow-up. Patients with CCTGA who have undergone AR have excellent functional status and mid-term survival but reinterventions are common. Longer term studies are needed to determine both the extent and spectrum of reinterventions as well as long term survival.

The Impact of Cardiac Motion on Aortic Valve Flow Used in Computational Simulations of the Thoracic Aorta.

Advancements in image-based computational modeling are producing increasingly more realistic representations of vasculature and hemodynamics, but so far have not compensated for cardiac motion when imposing inflow boundary conditions. The effect of cardiac motion on aortic flow is important when assessing sequelae in this region including coarctation of the aorta (CoA) or regurgitant fraction. The objective of this investigation was to develop a method to assess and correct for the influence of cardiac motion on blood flow measurements through the aortic valve (AoV) and to determine its impact on patient-specific local hemodynamics quantified by computational fluid dynamics (CFD). A motion-compensated inflow waveform was imposed into the CFD model of a patient with repaired CoA that accounted for the distance traveled by the basal plane during the cardiac cycle. Time-averaged wall shear stress (TAWSS) and turbulent kinetic energy (TKE) values were compared with CFD results of the same patient using the original waveform. Cardiac motion resulted in underestimation of flow during systole and overestimation during diastole. Influences of inflow waveforms on TAWSS were greatest along the outer wall of the ascending aorta (AscAo) (∼30 dyn/cm2). Differences in TAWSS were more pronounced than those from the model creation or mesh dependence aspects of CFD. TKE was slightly higher for the motion-compensated waveform throughout the aortic arch. These results suggest that accounting for cardiac motion when quantifying blood flow through the AoV can lead to different conclusions for hemodynamic indices, which may be important if these results are ultimately used to predict patient outcomes.

Droperidol transiently prolongs the QT interval in children undergoing single ventricle palliation.

Historically, droperidol was commonly used for postoperative sedation of critically ill children. A FDA black box warning regarding its arrhythmogenic potential greatly reduced its use. We hypothesized that administration of neuroleptic dose droperidol during volatile anesthesia would transiently prolong the corrected QT interval (QTc) in patients undergoing single ventricle palliation. As part of a prospective study in children undergoing stage 2 or 3 single ventricle palliation, we recorded electrocardiograms preoperatively, after induction of volatile anesthesia, immediately after completion of 30 min intravenous infusion of 75 mcg/kg droperidol, and shortly after arrival in the cardiac intensive care unit. Mean absolute QT intervals and heart rate data were analyzed in a blinded fashion and the longest QT interval was determined. QT intervals were corrected for heart rate (QTc) with the Bazett and Friderici formulae. Any perioperative arrhythmias were recorded. Complete data were available for 62 patients. Volatile anesthesia was associated with significant prolongation of the QTc interval. Administration of droperidol after cardiopulmonary bypass was associated with further significant QTc prolongation. All QTc changes were transient and the postoperative QTc, while still prolonged relative to baseline, was significantly shorter than the QTc immediately postdroperidol. No episodes of Torsades de Pointes (TdP) or ventricular arrhythmias were observed. The administration of a neuroleptic dose of droperidol during volatile anesthesia in patients undergoing single ventricle palliation was associated with a significant prolongation of QTc, which was transient and did not result in TdP or other ventricular arrhythmias in our study population.

Infant cardiac magnetic resonance imaging using oscillatory ventilation: safe and effective.

Cardiac magnetic resonance imaging (CMR) for infants and young children typically requires sedation. General anesthesia with controlled ventilation can eliminate motion artifact with breath-holds during imaging to limit respiratory artifact, but these may lead to atelectasis or other complications. High-frequency oscillatory ventilation (HFOV) provides ventilation with near-constant mean airway pressure and minimal movement of chest wall and diaphragm, thus obviating the need for breath-holding. Clinical data were collected for 8 infants who underwent CMR with HFOV and 8 controls who underwent CMR with conventional ventilator and breath-hold technique. Data included demographic information, adverse events, and scan-acquisition time. Studies were reviewed for image quality by two cardiologists who were blinded to type of ventilation. There were no significant differences in patient characteristics between the two groups. There was no significant difference in average image quality for cine short-axis or black blood imaging. Total CMR scan time was not significantly different between groups, but the short-axis cine stack was acquired more quickly in the HFOV group (1.8 ± 0.8 vs. 5.0 ± 3.6 min). There were no adverse events in the HFOV group, but scans were terminated early for two patients in the conventional ventilator group. HFOV during CMR is feasible and well tolerated. Image quality is equivalent to that obtained with conventional ventilation with breath-holding technique and allows shorter cine scan times for some sequences.

Late complications after Takeuchi repair of anomalous left coronary artery from the pulmonary artery: case series and review of literature.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart lesion that if left untreated, results in high mortality. Surgical treatment options include reimplantation of the anomalous coronary ostia, use of extracardiac arterial blood supply, or the Takeuchi procedure. The Takeuchi procedure involves creation of an aortopulmonary window and an intrapulmonary tunnel that baffles the aorta to the ostium of the anomalous left coronary artery. The incidence of late complications following the Takeuchi repair is unknown. The goal of our study was to determine the long-term outcome after palliation of ALCAPA using the Takeuchi Procedure. A total of 9 patients with a history of ALCAPA palliated with the Takeuchi procedure were identified from our surgical database. Chart review was performed. The mean age at time of Takeuchi procedure was 49.6 months (range 5 weeks-14.6 years). There was one late death, of unknown cause. Of the remaining 8 patients, the mean length of follow-up after surgery was 15.9 years (13.5-19.7 years). All 8 survivors had some degree of main pulmonary artery stenosis in the area of the intrapulmonary baffle, with moderate stenosis in 2 and severe stenosis in 1. Three late survivors (38 %) had a baffle leak. Two patients (25 %) had decreased left ventricular systolic function and 3 (38 %) had developed at least moderate mitral valve regurgitation. Three of the 8 late survivors (38 %) required a reoperation for repair of mitral valve regurgitation, baffle leak, and main pulmonary artery (MPA) stenosis. Review of literature demonstrated similar complication rates and need for reoperation following the Takeuchi procedure. Compared with patients after direct reimplantation for ALCAPA at our institution, there was no significant difference in late survival or freedom from reoperation. The Takeuchi procedure is a method to establish a two-coronary repair for ALCAPA. Late complications are common, necessitating lifelong care in a center experienced with caring for adults with congenital heart disease.

A Pilot Study Characterizing Flow Patterns in the Thoracic Aorta of Patients With Connective Tissue Disease: Comparison to Age- and Gender-Matched Controls via Fluid Structure Interaction.

Prior computational and imaging studies described changes in flow patterns for patients with Marfan syndrome, but studies are lacking for related populations. This pilot study addresses this void by characterizing wall shear stress (WSS) indices for patients with Loeys-Dietz and undifferentiated connective tissue diseases. Using aortic valve-based velocity profiles from magnetic resonance imaging as input to patient-specific fluid structure interaction (FSI) models, we determined local flow patterns throughout the aorta for four patients with various connective tissue diseases (Loeys-Dietz with the native aorta, connective tissue disease of unclear etiology with native aorta in female and male patients, and an untreated patient with Marfan syndrome, as well as twin patients with Marfan syndrome who underwent valve-sparing root replacement). FSI simulations used physiological boundary conditions and material properties to replicate available measurements. Time-averaged WSS (TAWSS) and oscillatory shear index (OSI) results are presented with localized comparison to age- and gender-matched control participants. Ascending aortic dimensions were greater in almost all patients with connective tissue diseases relative to their respective control. Differences in TAWSS and OSI were driven by local morphological differences and cardiac output. For example, the model for one twin had a more pronounced proximal descending aorta in the vicinity of the ductus ligamentum that impacted WSS indices relative to the other. We are optimistic that the results of this study can serve as a foundation for larger future studies on the connective tissue disorders presented in this article.

The association between cardiac magnetic resonance-derived aortic stiffness parameters and aortic dilation in young adults with bicuspid aortic valve: With and without coarctation of aorta.

Background: Bicuspid aortic valve (BAV) is associated with progressive aortic dilation. Studies in aortopathies have shown a correlation between increased aortic stiffness and aortic dilation. We aimed to evaluate aortic stiffness measures as predictors of progressive aortic dilation by cardiac magnetic resonance (CMR) in BAV patients. Methods: This is a retrospective study of 49 patients with BAV (median age 21.1 years at first CMR visit) with ≥2 CMR at the Wisconsin Adult Congenital Heart Disease Program (WAtCH). Circumferential aortic strain, distensibility, and ß-stiffness index were obtained from CMR-derived aortic root cine imaging, and aortic dimensions were measured at aortic root and ascending aorta. A linear mixed-model and logistic regression were used to identify important predictors of progressive aortic dilation. Results: Over a median of 3.8 years follow-up, the annual growth rates of aortic root and ascending aorta dimensions were 0.25 and 0.16 mm/year, respectively. Aortic strain and distensibility decreased while ß-stiffness index increased with age. Aortic root strain and distensibility were associated with progressive dilation of the ascending aorta. Baseline aortic root diameter was an independent predictor of >1 mm/year growth rate of the aortic root (adjusted OR 1.34, 95 % CI 1.03-1.74, p = 0.028). Most patients (61 %) had coexisting coarctation of aorta. Despite the higher prevalence of hypertension in patients with aortic coarctation, hypertension or coarctation had no effect on baseline aorta dimensions, stiffness, or progressive aortic dilation. Conclusion: Some CMR-derived aortic stiffness parameters correlated with progressive aortic dilation in BAV and should be further investigated in larger and older BAV cohorts.

A medical strategy to reduce persistent chest tube drainage after the fontan operation.

A standardized medical regimen aimed at reducing pleural effusions after the Fontan operation was compared with a randomly selected retrospective cohort. The duration of chest tube drainage, hospital stay, and the need for pleural sclerosis were significantly reduced, indicating that postoperative management plays an important role in reducing this morbidity.

Early cavopulmonary anastomosis in very young infants after the Norwood procedure: impact on oxygenation, resource utilization, and mortality.

BACKGROUND: The optimal timing of second-stage palliation after Norwood operations remains undefined. Advantages of early cavopulmonary anastomosis are early elimination of volume load and shortening the high-risk interstage period. Potential disadvantages include severe cyanosis, prolonged pleural drainage and hospitalization, and excess mortality. We reviewed our recent experience to evaluate the safety of early cavopulmonary anastomosis. METHODS: Eighty-five consecutive patients undergoing post-Norwood operation cavopulmonary anastomosis were divided into group I (cavopulmonary anastomosis at <4 months; n = 33) and group II (cavopulmonary anastomosis at >4 months; n = 52). Groups were compared for age; size; early and late mortality; preoperative, initial postoperative, and discharge oxygen saturation; and duration of mechanical ventilation, intensive care unit stay, pleural drainage, and hospitalization. RESULTS: Group I patients were younger than group II patients (94 +/- 21 days vs 165 +/- 44 days, respectively; P <.001) and smaller (4.8 +/- 0.8 kg vs 5.8 +/- 0.9 kg; P <.001). The preoperative oxygen saturation was not different (group I, 75% +/- 10%; group II, 78% +/- 8%; P =.142). The oxygen saturation was lower immediately after surgery in group I compared with group II (75% +/- 7% vs 81% +/- 7%, respectively; P <.001) but not by discharge (group I, 79% +/- 4%; group II, 80% +/- 4%). Younger patients were ventilated longer (62 +/- 86 hours vs 19 +/- 42 hours; P =.001), in the intensive care unit longer (130 +/- 111 hours vs 104 +/- 94 hours; P =.049), hospitalized longer (12.5 +/- 11.5 days vs 10.3 +/- 14.8 days; P =.012), and required longer pleural drainage (106 +/- 45 hours vs 104 +/- 93 hours; P =.046). Hospital survival was 100% in both groups. Actuarial survival to 12 months was 96% +/- 4% for group I and 96% +/- 3% for group II. CONCLUSIONS: Early cavopulmonary anastomosis after the Norwood operation is safe. Younger patients are more cyanotic initially after surgery and have a longer duration of mechanical ventilation, pleural drainage, intensive care unit stay, and hospitalization.

Chest pain in children and adolescents.

The focus of this article is to explore chest pain in children and adolescents. Common causes of chest pain and the approach to its evaluation are reviewed. Special emphasis is given to the cardiac causes of pediatric chest pain and how it can be differentiated from more common benign etiologies.

Exercise recommendations and risk factors for sudden cardiac death.

This article reviews the risk factors that are associated with exercise that can lead to sudden cardiac death and what can be done to identify those who are at risk. Additionally, exercise recommendations to reduce the chance of sudden cardiac death and comments about restrictions and quality of life issues are addressed.

Home monitoring of infants after stage one palliation for hypoplastic left heart syndrome.

Despite improved early results with the Norwood procedure (stage one palliation), patients remain with at-risk anatomy and interstage mortality continues to be a limitation of staged single ventricle palliation. Retrospective analyses have implicated residual or recurrent anatomic lesions as well as intercurrent illness as causes of interstage mortality. We hypothesized that potentially life-threatening anatomic lesions and illnesses would be manifest before serious physiologic impact by alteration in arterial saturation, failure to gain weight or in the case of dehydration, acute weight loss. As a result, we developed a home monitoring program of daily weights and oxygen saturations to earlier identify those patients at increased risk for interstage death. Frequent monitoring of these physiologic variables between stage one and two palliation identified life-threatening anatomic lesions and illness and permitted timely intervention that ultimately improved survival. All 36 survivors of the stage one palliation discharged from the hospital and entered into the home monitoring program survived the interstage period.

Restrictive versus nonrestrictive physiology following repair of tetralogy of Fallot: is there a difference?

BACKGROUND: Long-term outcome in repaired tetralogy of Fallot (TOF) is related to chronic pulmonary insufficiency (PI), right ventricular (RV) dilation, and deterioration of RV function. The aim of this study was to characterize clinical differences between restrictive and nonrestrictive RV physiology in young patients with repaired TOF. METHODS: Patients were prospectively enrolled from February 2008 to August 2009. Each had a clinic visit, brain natriuretic peptide assessment, exercise test, cardiac magnetic resonance study, and echocardiographic examination with assessment of regional myocardial mechanics. Consistent antegrade diastolic pulmonary arterial flow with atrial contraction identified restrictive RV physiology. RESULTS: Twenty-nine patients (median age, 12 years; range, 8-33 years; nine male patients) were studied. Twelve had restrictive RV physiology. The median time since initial TOF repair was 12 years (range, 5-27 years). Restrictive physiology appeared more prevalent after transannular patch repair and was not influenced by other demographic features. The restrictive group had more PI (46% vs 28%, P = .002), larger RV end-diastolic volumes (128 vs 98 mL/m(2), P = .046), but similar ejection fractions, brain natriuretic peptide levels, New York Heart Association classes, and exercise capacity. RV basal and mid free wall peak diastolic strain rate differed between groups, negatively correlating with exercise time and positively correlating with PI in patients with restrictive physiology. CONCLUSIONS: Restrictive RV physiology correlates with a larger right ventricle and increased PI after TOF repair but does not negatively affect other markers of myocardial health. Diastolic regional RV myocardial mechanics, particularly diastolic velocity and peak diastolic strain rate, differ for postoperative TOF patients with restrictive and nonrestrictive RV physiology; longitudinal study is necessary to understand the relationship of regional myocardial mechanics and patients' clinical status.

Including aortic valve morphology in computational fluid dynamics simulations: initial findings and application to aortic coarctation.

Computational fluid dynamics (CFD) simulations quantifying thoracic aortic flow patterns have not included disturbances from the aortic valve (AoV). 80% of patients with aortic coarctation (CoA) have a bicuspid aortic valve (BAV) which may cause adverse flow patterns contributing to morbidity. Our objectives were to develop a method to account for the AoV in CFD simulations, and quantify its impact on local hemodynamics. The method developed facilitates segmentation of the AoV, spatiotemporal interpolation of segments, and anatomic positioning of segments at the CFD model inlet. The AoV was included in CFD model examples of a normal (tricuspid AoV) and a post-surgical CoA patient (BAV). Velocity, turbulent kinetic energy (TKE), time-averaged wall shear stress (TAWSS), and oscillatory shear index (OSI) results were compared to equivalent simulations using a plug inlet profile. The plug inlet greatly underestimated TKE for both examples. TAWSS differences extended throughout the thoracic aorta for the CoA BAV, but were limited to the arch for the normal example. OSI differences existed mainly in the ascending aorta for both cases. The impact of AoV can now be included with CFD simulations to identify regions of deleterious hemodynamics thereby advancing simulations of the thoracic aorta one step closer to reality.

Computational simulations demonstrate altered wall shear stress in aortic coarctation patients treated by resection with end-to-end anastomosis.

BACKGROUND: Atherosclerotic plaque in the descending thoracic aorta (dAo) is related to altered wall shear stress (WSS) for normal patients. Resection with end-to-end anastomosis (RWEA) is the gold standard for coarctation of the aorta (CoA) repair, but may lead to altered WSS indices that contribute to morbidity. METHODS: Computational fluid dynamics (CFD) models were created from imaging and blood pressure data for control subjects and age- and gender-matched CoA patients treated by RWEA (four males, two females, 15 ± 8 years). CFD analysis incorporated downstream vascular resistance and compliance to generate blood flow velocity, time-averaged WSS (TAWSS), and oscillatory shear index (OSI) results. These indices were quantified longitudinally and circumferentially in the dAo, and several visualization methods were used to highlight regions of potential hemodynamic susceptibility. RESULTS: The total dAo area exposed to subnormal TAWSS and OSI was similar between groups, but several statistically significant local differences were revealed. Control subjects experienced left-handed rotating patterns of TAWSS and OSI down the dAo. TAWSS was elevated in CoA patients near the site of residual narrowings and OSI was elevated distally, particularly along the left dAo wall. Differences in WSS indices between groups were negligible more than 5 dAo diameters distal to the aortic arch. CONCLUSIONS: Localized differences in WSS indices within the dAo of CoA patients treated by RWEA suggest that plaque may form in unique locations influenced by the surgical repair. These regions can be visualized in familiar and intuitive ways allowing clinicians to track their contribution to morbidity in longitudinal studies.

Characterization of cardiac tumors in children by cardiovascular magnetic resonance imaging: a multicenter experience.

OBJECTIVES: The aim of this study was to report the results of an international multicenter experience of cardiac magnetic resonance imaging (MRI) evaluation of cardiac tumors in children, each with histology correlation or a diagnosis of tuberous sclerosis, and to determine which characteristics are predictive of tumor type. BACKGROUND: Individual centers have relatively little experience with diagnostic imaging of cardiac tumors in children, because of their low prevalence. The accuracy of cardiac MRI diagnosis on the basis of a pre-defined set of criteria has not been tested. METHODS: An international group of pediatric cardiac imaging centers was solicited for case contribution. Inclusion criteria comprised: 1) age at diagnosis ≤18 years; 2) cardiac MRI evaluation of cardiac tumor; and 3) histologic diagnosis or diagnosis of tuberous sclerosis. Data from the cardiac MRI images were analyzed for mass characteristics. On the basis of pre-defined cardiac MRI criteria derived from published data, 3 blinded investigators determined tumor type, and their consensus diagnoses were compared with histologic diagnoses. RESULTS: Cases (n = 78) submitted from 15 centers in 4 countries had the following diagnoses: fibroma (n = 30), rhabdomyoma (n = 14), malignant tumor (n = 12), hemangioma (n = 9), thrombus (n = 4), myxoma (n = 3), teratoma (n = 2), and paraganglioma, pericardial cyst, Purkinje cell tumor, and papillary fibroelastoma (n = 1, each). Reviewers who were blinded to the histologic diagnoses correctly diagnosed 97% of the cases but included a differential diagnosis in 42%. Better image quality grade and more complete examination were associated with higher diagnostic accuracy. CONCLUSIONS: Cardiac MRI can predict the likely tumor type in the majority of children with a cardiac mass. A comprehensive imaging protocol is essential for accurate diagnosis. However, histologic diagnosis remains the gold standard, and in some cases malignancy cannot be definitively excluded on the basis of cardiac MRI images alone.

Perioperative monitoring in high-risk infants after stage 1 palliation of univentricular congenital heart disease.

OBJECTIVE: Survival of high-risk patients with univentricular heart disease after Norwood palliation is reduced. We hypothesized that early goal-directed monitoring with venous oximetry and near-infrared spectroscopy would offset their increased vulnerability and improve survival. METHODS: A prospective database of patients undergoing stage 1 palliation was used to assess differences in outcomes across risk groups in the setting of a comprehensive, goal-directed monitoring program. High-risk criteria included gestational age 35 weeks or less, birth weight less than 2.5 kg, and additional cardiac or extracardiac anomalies. Outcomes included survival to defined end points and measures of postoperative support. RESULTS: From September 2000 to September 2008, 162 patients underwent stage 1 palliation: 28% (45/162) high-risk and 72% (117/162) standard-risk patients. Lesions other than hypoplastic left heart syndrome were more common among high-risk patients (38%, 17/45, vs 15%, 18/117, P = .003). Operative survival was not statistically different(87%, 39/45, high risk vs 95%, 111/117, standard risk, P = .1). High-risk patients were more likely to receive inpatient treatment until stage 2 palliation (24%, 11/45, vs 10%, 12/117, P = .001) and had lower 1-year survival (78% vs 93%, P = .01) and survival to date (71% vs 92%, P = .001). CONCLUSIONS: Intensive monitoring partially offset biologic vulnerability of high-risk patients, helping attain comparable early outcomes. Vulnerability persisted throughout the interstage period, however, and increased mortality beyond cavopulmonary shunt was seen only among high-risk patients. Although enhanced monitoring reduced early mortality, high resource use and attrition after stage 2 palliation suggest an ongoing need to evaluate our current palliative strategy for this subset of patients.

Near-infrared spectroscopy in neonates before palliation of hypoplastic left heart syndrome.

BACKGROUND: Neonates with hypoplastic left heart syndrome have circulatory vulnerability that results in shock and high risk of mortality without intervention. High arterial saturation (SaO(2)) is often used as a proxy for inadequate systemic oxygen delivery and triggers the use of invasive therapies to restore circulatory balance. We hypothesized that preoperative use of near-infrared spectroscopy (NIRS) would reduce the need for invasive therapies, including controlled ventilation and inspired gas manipulation. METHODS: A Human Research Review Board-approved retrospective review of patients who had stage 1 palliation from January 2000 to January 2006 was conducted. Preoperative patient characteristics, cardiorespiratory support, and monitored data were collected for all patients. Cerebral and somatic tissue oxyhemoglobin saturations were recorded for patients with preoperative NIRS monitoring. RESULTS: The studied cohort included 92 patients, 47 without and 45 with preoperative NIRS. Patient characteristics were similar between groups. Differences were observed in preoperative respiratory support. Controlled ventilation was less common in the NIRS group (51% versus 79%, p = 0.005) as was the use of inspired nitrogen (16% versus 70%, p = 0.001). The NIRS patients had higher mean SaO(2) (92% versus 88%, p = 0.001). Age at surgery was similar between groups (5.7 +/- 3.2 versus 6.5 +/- 5.2 days, p = 0.3). Early survival was 96% in each group. CONCLUSIONS: Near-infrared spectroscopy monitoring of patients with hypoplastic left heart syndrome awaiting palliation provides noninvasive assessment of oxygen delivery and simplified management, with reduced use of controlled ventilation and inspired gas. Higher SaO(2) in the NIRS group was not associated with impaired systemic oxygen delivery, and did not lead to earlier palliation or postoperative mortality.