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Coronavirus disease 2019 created unintended but significant experiential barriers for surgical learners to interact at the bedside for teaching/case presentations. We hypothesized that an international grand rounds using the Microsoft HoloLens 2 extended reality (XR) headset would create an improved bedside-learning experience compared to traditional grand rounds formats.From December 2020 to March 2021, the world's first 2 international mixed reality grand rounds events using the HoloLens 2 headset were held, broadcasting transatlantically (between the University of Michigan and the Imperial College of London) bedside rounding experiences on 5 complex surgical patients to an international audience of 325 faculty, residents, and medical trainees. Participants completed pre- and post-event surveys to assess their experience.Of the 325 participants, 267 (80%) completed pre-surveys, and 95 (29%) completed both the pre- and post-surveys. Respondents (average age, 38 y; 44% women, 56% men; 211 US, 56 UK) included 92 (34%) medical students and residents and 175 faculty and staff. In the pre-event survey, 76% had little or no earlier experience with XR devices, and 94% thought implementation of XR into medical curricula was valuable. In the post-survey, 96% thought telerounding using XR technology was important for the current era, and 99% thought the ability to visualize the examination, imaging, and laboratory results at bedside via XR rounding was highly valuable and that this format was superior to traditional grand rounds.Almost all of the participants in the mixed reality international grand rounds felt the immersive XR experiences-allowing visualization of clinical findings, imaging, and laboratory results at the patient's bedside-were superior to a traditional grand rounds format, and that it could be a valuable tool for surgical teaching and telerounding.

Decreasing flood damages in a basin and achieving sustainable development reveal the necessity of integrated management of flood. Integrated flood management requires the co-implementation of both structural and non-structural flood mitigation strategies. Watershed management, building dams and levees, and increasing channel capacity are considered among structural measures. While, flood forecasting, developing flood early warning systems, and establishing reservoir operation rules can fall into the non-structural measures group. The main goal of this study is to reveal the importance of co-implementation of structural and non-structural measures for managing the flood in the whole system. Structural measures are divided here into two main groups (1) dams and (2) watershed management, and non-structural measures focus more on reoperation of the system of reservoirs. The system dynamics (SD) approach has been used to model the integration of a series of structural and non-structural flood control measures for the Karkheh Basin, Iran. The performance of the developed model is evaluated based on historical floods, including the recent 2019 flooding event in this basin, and the performance of flood mitigation strategies is evaluated facing these flood events. Results indicate that both structural and non-structural solutions should be considered in flood management portfolios simultaneously, and none solely can be the silver bullet. The magnitude of a 200-year flood can be reduced from 7956 to 3021 m3/s, if integrated structural and non-structural strategies would be considered in Karkheh Basin. This study identifies the key role of structural and non-structural measures for integrated flood management in Karkheh Basin, which and provides effective information for appropriate and timely operation and decision making in the future.

Complete thrombosis of a pulmonary artery interposition graft in an adolescent with complex repaired CHD was treated successfully with a combination of a novel mechanical thrombectomy system, stent implantation, and thrombolysis. This thrombectomy system used a flexible catheter with a built-in mechanism to attenuate blood loss, while providing effective recanalisation of a foreign graft.

This case report describes a rare etiology of cardiogenic shock, particularly in the pediatric population. A healthy 17 year old male presents from an outside hospital in undifferentiated shock requiring vasopressor support. Ruptured sinus of Valsalva aneurysm was diagnosed by echocardiogram and the patient went emergently to the operating room for surgical repair. We discuss the anatomy, incidence, and risk factors for sinus of Valsalva aneurysms, along with the range of clinical presentations and Emergency Department management of symptomatic rupture of sinus of Valsalva aneurysms.

3D visualization technologies have evolved to become a mainstay in the management of congenital heart disease (CHD) with a growing presence within multiple facets. Printed and virtual 3D models allow for a more comprehensive approach to educating trainees and care team members. Computational fluid dynamics can take 3D modeling to the next level, by predicting post-procedural outcomes and helping to determine surgical approach. 3D printing and extended reality are developing resources for pre-procedural planning and intra-procedural guidance with the potential to revolutionize decision-making and procedural success. Challenges still remain within existing technologies and their applications to the CHD field. Addressing these gaps, both by those within and outside of CHD, will transform education and patient care within our field.

Purpose Ectodermal dysplasias are characterized by developmental abnormalities in ectodermal structures. Hypohidrotic ectodermal dysplasias (HED) are the most common subtype. They are most commonly inherited via X-linked recessive routes. We report on a novel ectodysplasin-A (EDA) mutation that is expected to be involved in pathogenesis of HED. Methods Hypohidrotic ectodermal dysplasia genes, including EDA, EDAR and EDARADD, were analyzed using next-generation sequencing (NGS). The detected mutation on the EDA gene was confirmed in the patient and his mother using Sanger sequencing. Results The patient presented with adontia, absence of gum development, hyperthermia and hypohidrosis. Our genetic analysis of the patient revealed a novel frameshift hemizygous mutation (c.898_924 + 8del35ins4CTTA) on the EDA gene. The patient’s mother showed a mild HED phenotype. Direct sequencing of the EDA gene in the region where her son had the mutation showed the same mutation in a heterozygous state. Conclusion We identified a novel frameshift mutation in the EDA gene in an Iranian patient affected by X-linked HED. The difference between our patient’s symptoms and those recorded for some previous subjects may be due to the differences in the mutations involved. Electronic supplementary material The online version of this article (10.1186/s11658-019-0174-9) contains supplementary material, which is available to authorized users.

The Gore Viabahn VBX balloon-expandable endoprosthesis (W. L. Gore & Associates, Flagstaff, AZ) is a flexible covered stent that is FDA-approved for the treatment of iliac artery stenosis, including lesions at the aortic bifurcation. In this case series, we report the first use of the VBX covered stent in congenital heart disease, highlighting several of its unique advantages.

IntroductionA 2-year-old female with hypoplastic left heart syndrome (HLHS)-variant, a complex congenital heart defect (CHD) characterized by the underdevelopment of the left ventricle, presented with complications following single ventricle palliation. Diagnostic work-up revealed elevated Fontan pathway pressures, as well as significant dilation of the inferior Fontan pathway with inefficient swirling flow and hepatic venous reflux. Due to the frail condition of the patient, the clinical team considered an endovascular revision of the Fontan pathway. In this work, we performed a computational fluid dynamics (CFD) analysis informed by data on anatomy, flow, and pressure to investigate the hemodynamic effect of the endovascular Fontan revision.MethodsA patient-specific anatomical model of the Fontan pathway was constructed from magnetic resonance imaging (MRI) data using the cardiovascular modeling software CardiovasculaR Integrated Modeling and SimulatiON (CRIMSON). We first created and calibrated a pre-intervention 3D-0D multi-scale model of the patient’s circulation using fluid-structure interaction (FSI) analyses and custom lumped parameter models (LPMs), including the Fontan pathway, the single ventricle, arterial and venous systemic, and pulmonary circulations. Model parameters were iteratively tuned until simulation results matched clinical data on flow and pressure. Following calibration of the pre-intervention model, a custom bifurcated endograft was introduced into the anatomical model to virtually assess post-intervention hemodynamics.ResultsThe pre-intervention model successfully reproduced the clinical hemodynamic data on regional flow splits, pressures, and hepatic venous reflux. The proposed endovascular repair model revealed increases of mean and pulse pressure at the inferior vena cava (IVC) of 6 and 29%, respectively. Inflows at the superior vena cava (SVC) and IVC were each reduced by 5%, whereas outflows at the left pulmonary artery (LPA) and right pulmonary artery (RPA) increased by 4%. Hepatic venous reflux increased by 6%.ConclusionOur computational analysis indicated that the proposed endovascular revision would lead to unfavorable hemodynamic conditions. For these reasons, the clinical team decided to forgo the proposed endovascular repair and to reassess the management of this patient. This study confirms the relevance of CFD modeling as a beneficial tool in surgical planning for single ventricle CHD patients.

Children with hypoplastic left heart syndrome (HLHS) must undergo multiple surgical stages to reconstruct the anatomy to a sustainable single ventricle system. Stage I palliation, or the Norwood procedure, provides circulation to both pulmonary and systemic vasculature. The aorta is reconstructed and attached to the right ventricle and a fraction of systemic flow is redirected to the pulmonary arteries (PAs) through a systemic-to-PA shunt. Despite abundant hemodynamic data available 4–5 months after Norwood palliation, data is very scarce immediately following stage I. This data is critical in determining post-operative success. In this work, we combined population data and computational fluid dynamics (CFD) to characterize hemodynamics immediately following stage I (post-stage I) and prior to stage II palliation (pre-stage II). A patient-specific model was constructed as a baseline geometry, which was then scaled to reflect population-based morphological data at both time-points. Population-based hemodynamic data was then used to calibrate each model to reproduce blood flow representative of HLHS patients. The post-stage I simulation produced a PA pressure of 22 mmHg and high-frequency oscillations within the flow field indicating highly disturbed hemodynamics. Despite PA mean pressure dropping to 14 mmHg, the pre-stage II model also produced high-frequency flow components and PA wall shear stress increases. These suboptimal conditions may be necessary to ensure adequate PA flow throughout the pre-stage II period, as the shunt becomes relatively smaller compared to the patient’s somatic growth. In the future, CFD can be used to optimize shunt design and minimize these suboptimal conditions.

To investigate whether transcatheter device closure of patent ductus arteriosus (PDA) is safe in children with pulmonary artery hypertension, we retrospectively analyzed our experience with 33 patients who underwent the procedure from January 2000 through August 2015. Pulmonary artery hypertension was defined as a pulmonary vascular resistance index (PVRI) >3 WU · m2. All 33 children (median age, 14.5 mo; median weight, 8.1 kg) underwent successful closure device implantation and were followed up for a median of 17.2 months (interquartile range [IQR], 1.0–63.4 mo). During catheterization, the median PVRI was 4.1 WU · m2 (IQR, 3.6–5.3 WU · m2), and the median mean pulmonary artery pressure was 38.0 mmHg (IQR, 25.5–46.0 mmHg). Premature birth was associated with pulmonary vasodilator therapy at time of PDA closure ( P=0.001) but not with baseline PVRI (P=0.986). Three patients (9.1%) had device-related complications (one immediate embolization and 2 malpositions). Two of these complications involved embolization coils. Baseline pulmonary vasodilator therapy before closure was significantly associated with intensive care unit admission after closure (10/12 [83.3%] with baseline therapy vs 3/21 [14.3%] without; P We conclude that transcatheter PDA closure can be performed safely in many children with pulmonary artery hypertension and improve symptoms, particularly in patients born prematurely. Risk factors for adverse outcomes are multifactorial, including coil use and disease severity. Multicenter studies in larger patient populations are warranted.

Introduction: Transcatheter pulmonary valve implantation (TPVi) is a widely available option to treat right ventricular outflow tract (RVOT) conduits, but coronary artery compression is an absolute contraindication. Cardiac magnetic resonance (CMR) may evaluate coronary anatomy, but its utility in predicting coronary compression is not well established. Hypothesis: CMR prior to TPVi can accurately predict coronary compression risk. Methods: We analyzed all patients with a recent CMR (≤ 12 months) and attempted TPVi in an RVOT conduit at 9 centers from 2007-2016. A core lab reviewed all CMRs for the shortest orthogonal distance from a coronary artery to the conduit, the shortest distance from a coronary artery to the most stenotic area of the conduit, and subjective assessment of coronary compression risk. Receiver operating characteristic curve was used to determine optimal predictive distances. Univariate and independent associations of the distances and qualitative assessment with coronary compression were examined using logistic regression. Results: Of 231 patients (62% male, median age 19.0 years), TPVi was successful in 198 (86%); in 24 (10%) balloon testing (documented coronary compression or high risk) precluded implantation. Distance to the RV to PA conduit ≤ 2.1 mm (area under the curve [AUC] 0.70) and distance to most stenotic area ≤ 13.1 mm (AUC 0.69) predicted coronary compression (Table). Subjective assessment had the highest AUC (0.78), with 96% negative predictive value. Both distances and qualitative assessment remained independently associated with coronary compression when controlling for abnormal coronary anatomy. Conclusions: CMR can predict the risk of coronary compression during TPVi in RVOT conduits but cannot completely exclude the risk of coronary compression. CMR may assist in patient selection and counselling families prior to TPVi, although balloon testing prior to TPVi remains essential.

Background Debriefing after pediatric rapid response team activations (RRT-As) in a tertiary care children's hospital was identified to occur only sporadically. The lack of routine debriefing after RRT-As was identified as a missed learning opportunity. Objective We implemented a formal debriefing program and assessed staff attitudes toward and experiences with debriefing after pediatric RRT-As. Methods Real-time feedback for pediatrics residents captured clinical and debriefing data for each RRT-A from July 2014 to June 2016. The debriefing on physiology, team communication, and anticipation of clinical deterioration was introduced in July 2015. To assess debriefing perceptions, residents, intensive care fellows, nurses, and respiratory therapists participated in anonymous preintervention and postintervention surveys. We also developed a workshop to teach residents how to lead debriefing. Results Debriefing after RRT-As increased from 26% preintervention to 46% postintervention (P Conclusions Debriefing frequency after pediatric RRT-As significantly increased with the introduction of a formal debriefing program. A majority of health professionals and trainees reported this practice was a valuable experience.

An 8-year-old boy with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals status post complete repair including a fenestrated ventricular septal defect patch presented to the catheterization laboratory for fenestration closure. During the procedure, the catheterization wire was found to have an unusual intracardiac loop and was unable to be straightened within the heart. Three-dimensional transesophageal echocardiography revealed the wire was looped around a right ventricular papillary muscle. The wire was readjusted, and the fenestration was successfully closed. Three-dimensional imaging was essential in unambiguously defining the catheter course and assisting in fenestration closure.

Quantitative control and safe exploitation of water resources are crucial components for the sustainable development of any region due to increasing water scarcity, especially in arid and semiarid regions. A proper water resources management program of the Tabriz plain is necessary because of the groundwater excessive exploitation and the surface water limitations. The present study aims to develop a system dynamics (SD) model not only to identify safe groundwater level fluctuations and exploitation but also to determine the amount of supply and deficits of municipal-industry and agricultural uses by considering various water management strategies. The integrated water resources including surface and groundwater resources were included in the analyses. The considered alternatives are the development of sewage collection networks, enhancement of agricultural efficiency, climate changes effects, artificial recharge and changes of the allocation of the surface and groundwater in supplying water needs. SD modeling application showed that if the current management policy was continued, the users will face a crisis of the water supply for various uses in the future. Increase in agricultural efficiency, reducing in groundwater extraction and artificial recharge in Tabriz plain must be considered to prevent the crisis. The current model provides a better understanding of the principal challenge of balancing supply and demand with regards to drastic changes of water resources in arid or semiarid regions.

There is no better representation of the need for personalization of care than the breadth and complexity of congenital heart disease. Advanced imaging modalities are now standard of care in the field, and the advancements being made to three-dimensional visualization technologies are growing as a means of pre-procedural preparation. Incorporating emerging modeling approaches, such as computational fluid dynamics, will push the limits of our ability to predict outcomes, and this information may be both obtained and utilized during a single procedure in the future. Artificial intelligence and customized devices may soon surface as realistic tools for the care of patients with congenital heart disease, as they are showing growing evidence of feasibility within other fields. This review illustrates the great strides that have been made and the persistent challenges that exist within the field of congenital interventional cardiology, a field which must continue to innovate and push the limits to achieve personalization of the interventions it provides.

Objective We describe the technical considerations of transcatheter implantation of the CardioMEMS™ HF System (Abbott, Abbott Park, IL) in adult patients with complex palliated congenital heart disease (CHD) and advanced heart failure (HF). Background Ambulatory pulmonary artery (PA) pressure monitoring with implantable hemodynamic monitors (IHMs) has been shown to reduce HF-related hospital admissions in non-CHD populations. HF is a common late cardiovascular complication in adult CHD necessitating better understanding of IHM application in this population. Methods We analyzed adults with complex CHD and advanced HF who were referred for CardioMEMS™ device implantation (2015-2018). Feasibility of device implantation, defined by successful device implantation and calibration, and procedural outcomes were evaluated. Results CardioMEMS™ was successfully implanted in all 14 adults (35.5 ± 9.2 years old, 72 ± 12 kg) with complex CHD (single ventricle/Fontan, n = 8 [57%]; d-transposition of the great arteries/atrial switch, n = 6 [43%]). The device was delivered via femoral venous access in 13 (93%) patients and implanted in the left PA in 12 (86%). A long sheath was used in 8 (57%) patients, including 5/6 with an atrial switch operation. There was one device migration that did not require retrieval. Conclusions Transcatheter implantation of an IHM is feasible in select complex adult CHD patients with advanced HF. Further studies evaluating integration of ambulatory hemodynamics and the impact on clinical care are needed. This technology has the potential to improve medical management of advanced HF in patients with Fontan and atrial switch physiologies and provide new insights into their ambulatory hemodynamics.

Transcatheter stent implantation for SVC obstruction following OHT has been well described, particularly in pediatric patients. This study describes a large single-center pediatric heart transplant experience that investigates the risk factors for SVC stenosis requiring stent implantation and its associated outcomes. All pediatric OHTs between January 1, 2000, and December 12, 2016, were examined for risk factors. Twelve of 349 (3.4%) OHTs required SVC stent implantation. Patients who required stents were younger (2.5 years vs 10.0 years, P = 0.0097), weighed less (8.6 kg vs 26.0 kg, P = 0.0024), and were more likely to have had CHD (83% vs 32%, P = 0.001) or previous SCPA (58% vs 18%, P = 0.002). Bicaval anastomosis was not associated with subsequent SVC stent implantation. Symptoms included SVC syndrome and chylous effusions. All 12 patients had evidence of significant SVC obstruction by both echocardiographic Doppler interrogation and transcatheter angiography. There were no acute procedural complications; however, reinterventions were common (four of 12 patients) and occurred at a mean of 5.7 ± 3.6 months poststent implantation. In conclusion, transcatheter SVC stent implantation is safe and effective after OHT. There were significant associations between SVC stent implantation and younger age, smaller weight, CHD, and history of SCPA, but not with bicaval anastomosis or donor-recipient weight ratio.

Background Children with heterotaxy syndrome and intestinal rotational anomalies (IRA) are at risk for midgut volvulus and ischemia. Controversy exists regarding risks and benefits of prophylactic Ladd procedures. The purpose of this study is to assess the impact of Ladd procedures on adverse events for children with heterotaxy and IRA. Methods A retrospective cohort study using the Pediatric Health Information System (PHIS) database was performed. All children with heterotaxy and IRA admitted at age ≤30days and discharged between 1/1/2004 and 1/1/2011 were included with a minimum 2-year follow-up period. The primary outcome was major morbidity , defined as a composite variable for intestinal obstruction/volvulus, ischemia, or resection, or inhospital mortality . Univariable and multivariable analyses were performed. Results There were 325 patients who met the inclusion criteria, including 92 (28%) patients with single ventricles. Mean gestational age was 38.0±2.1weeks and birth weight was 3.1±0.6kg. Ladd procedure was performed during initial hospitalization on 188 (58%) children. In multivariable analyses, Ladd procedure on initial hospitalization was associated with a 2.2 times increased odds of adverse events on subsequent admissions (95% CI 1.3–4.0, p =0.007). Conclusions Ladd procedure is associated with increased odds of adverse events for children with heterotaxy and IRA.

PURPOSE OF REVIEW Procedural technique and short-term outcomes of transcatheter pulmonary valve implantation (TPVI) have been widely described. The purpose of this article is to provide an update on current valve technology, and to focus on recent data surrounding TPVI in the dilated right ventricular outflow tract (RVOT), hybrid interventions, significant outcomes, and procedural costs. RECENT FINDINGS Transcatheter valve technology has expanded with current trials evaluating self-expandable valves that can be implanted in dilated RVOTs. Until those valves are widely available, hybrid techniques have been shown to offer a potential alternative in these patients, as well as in patients of small size. Although medium-term results of TPVI have shown 5-year freedom from reintervention or replacement of 76%, new data have underlined some concerns relating to bacterial endocarditis after the procedure. Procedural costs remain a concern, but vary greatly between institutions and healthcare systems. SUMMARY TPVI has emerged as one of the most innovative procedures in the treatment of patients with dysfunctional RVOT and pulmonary valves. Further device development is likely to expand the procedure to patients of smaller size and with complex, dilated RVOTs.

Aim: Chronic periodontitis (CP) is a multifactorial disease and the most common type of periodontitis mainly caused by microbial plaque. CP can be brought on by, and progresses with, insufficient oral hygiene, and environmental and genetic susceptibilities. The aim of the present study was to investigate the association between interleukin (IL)-2 (T-330G), IL-16 (T-295C), and IL-17 (A-7383G) gene polymorphisms and the susceptibility to CP in an Iranian population.Methods: Ninety-nine cases diagnosed with CP and 75 matched healthy controls engaged in the present study. 3 cc peripheral blood samples were obtained for DNA isolation. Genotype analysis was performed using restriction fragment length polymorphism polymerase chain reaction. Genotype distribution and allele frequencies within groups were compared using chi(2)-test, and logistic regression analysis was used to recognize the independent relation between the disease and the absence or presence of alleles.Results: There was no polymorphism in IL-2 (T-330G) among our patients, and the TT genotype was present in both study groups. Moreover, none of the studied genotypes and alleles of IL-16 (T-295C) and IL-17 (A-7383G) was significantly associated with CP.Conclusion: The present study demonstrated no association between IL-2 (T-330G), IL-16 (T-295C), and IL-17 (A-7383G) genotypes and CP in an Iranian population.

Risk stratification of patients with Wolff–Parkinson–White syndrome for sudden death is a complex process, particularly in understanding the utility of the repeat exercise stress test. We report a case of an 18-year-old patient who was found to have a high-risk pathway by both invasive and exercise stress testing after an initial exercise stress test showing beat-to-beat loss of pre-excitation.

Cardiopulmonary bypass (CPB) can be a potential cause of morbidity in patients for several reasons, including significantly higher gaseous microemboli (GME) formation than extracorporeal life support (ECLS) and physiological circulation, diverted blood flow from the patient via an open purge line of the arterial filter, and pressure drop across the oxygenator that is used in the circuit. Using a combined oxygenator and arterial filter may minimize these harmful factors and can effectively reduce the chances for postoperative morbidity. This study investigated the new QUADROX-i Neonatal Oxygenator (D-72145, Maquet, Hirrlingen, Germany) with an integrated arterial filter in terms of the hemodynamic properties and ability to clear GME in response to hypothermic versus normothermic conditions, open versus closed arterial filter purge line, and varying flow rates in a simulated CPB circuit identical to that of the clinical setting. A flow probe, pressure transducer, and Emboli Detection and Classification (EDAC) quantifier transducer were placed upstream and downstream to the oxygenator to measure changes in each parameter. The circuit was primed with fresh human blood with an hematocrit (Hct) of 26% diluted with Ringer’s lactate solution. Five milliliters of air were injected proximal to the venous cardiotomy reservoir, under non-pulsatile perfusion, with flow rates of 500 ml/min, 750 ml/min, and 1000 ml/min. A total of 8 air bolus injections were made at each individual set of conditions for a total of 96 injections. Results showed that the QUADROX-i Neonatal Oxygenator with an integrated filter has excellent hemodynamic properties with extremely low pressure drops and blood flow diverted from the patient, as well as high rates of GME capturing. The arterial filter purge line has a significant effect on the degree of blood flow diverted from the patient (p < 0.001), but does not affect pressure drop across the oxygenator.

PURPOSE To evaluate the utility of skin conductance (SC) as a measure of autonomic arousal to sound stimuli in preterm infants. DESIGN A pilot cross-sectional, correlations study. SUBJECTS Eleven preterm infants with a mean gestational age of 31.6 weeks without anomalies or conditions associated with neurodevelopmental delay composed the sample. METHODS On days 5-7 of life, the following infant responses were simultaneously recorded in response to naturally occurring sound stimuli in the NICU: real-time measurements of heart rate, respiratory rate, and oxygen saturations; sympathetic-mediated sweating via SC; and behavioral responses using the Newborn Individualized Developmental Care and Assessment Program naturalistic observation. Baseline sound levels (BSL, 65 dBA) were measured to index patterns of response during a nonhandling period preceding care. RESULTS Mean heart rate during precare was directly associated with higher SC increases to sound stimuli (r[10] = 0.697, P = .017). The SC during HSL was significantly higher than that during BSL (P < .0001). Males demonstrated higher SC increases to sound stimuli than females (P = .030). Changes in SC induced by increases in sound intensity were associated with lower attention responses (r[10] = -0.92, P < .0001) and lower summated behavioral responses (r[10] = -0.59, P = .054). CONCLUSION SC provides a noninvasive, sensitive measure of sympathetic arousal that may not be apparent in behavioral cues or states, or determined by standard physiological responses alone.

Objectives Our aim was to examine the effects of surgical timing on major morbidity, mortality, and total hospital reimbursement for late preterm and term infants with hypoplastic left heart syndrome (HLHS) undergoing stage 1 palliation within the first 2 weeks of life. Methods We conducted a retrospective cohort study of infants aged ≥35 weeks gestation, with HLHS, admitted to our institution at age ≤5 days, between January 1, 2003, and January 1, 2013. Children with other cardiac abnormalities or other major comorbid conditions were excluded. Univariable and multivariable analyses were performed to determine the association between age at stage 1 palliation and major morbidity, mortality, and hospital reimbursement. Results One hundred thirty-four children met inclusion criteria. Mortality was 7.5% (n = 10). Forty-three percent (n = 58) experienced major morbidity. Median costs were $97,000, in 2013 dollars (interquartile range, $72,000-$151,000). Median age at operation was 5 days (interquartile range, 3-7 days; full range, 1-14 days). All deaths occurred in patients operated on between 4 and 8 days of life. For every day later that surgery was performed, the odds of major morbidity rose by 15.7% (95% confidence interval, 2.5%-30.7%; P = .018) and costs rose by 4.7% (95% confidence interval, 0.9%-8.2%; P Conclusions Delay of stage 1 palliation for neonates with HLHS is associated with increased morbidity and health care costs, even within the first 2 weeks of life.