Search

Patch augmentation of the right ventricular outflow tract (RVOT) and pulmonary artery (PA) arterioplasty are relatively common procedures in the surgical treatment of patients with congenital heart disease. To date, several patch materials have been applied with no agreed upon clinical standard. Each patch type has unique performance characteristics, cost, and availability. There are limited data describing the various advantages and disadvantages of different patch materials. We performed a review of studies describing the clinical performance of various RVOT and PA patch materials and found a limited but growing body of literature. Short-term clinical performance has been reported for a multitude of patch types, but comparisons are limited by inconsistent study design and scarce histologic data. Standard clinical criteria for assessment of patch efficacy and criteria for intervention need to be applied across patch types. The field is progressing with improvements in outcomes due to newer patch technologies focused on reducing antigenicity and promoting neotissue formation which may have the ability to grow, remodel, and repair.

Patients with single ventricle heart defects requires a series of staged open-heart procedures, termed Fontan palliation. However, while lifesaving, these operations are associated with significant morbidity and early mortality. The attendant complications are thought to arise in response to the abnormal hemodynamics induced by Fontan palliation, although the pathophysiology underlying these physicochemical changes in cardiovascular and other organs remain unknown. Here, we investigated the microRNA (miRNA) content in serum and serum-derived extracellular vesicles (EVs) by sequencing small RNAs from a physiologically relevant sheep model of the Fontan operation. The differential expression analysis identified the enriched miRNA clusters in (1) serum vs. serum-derived EVs and (2) pre-Fontan EVs vs. post-Fontan EVs. Metascape analysis showed that the overexpressed subset of EV miRNAs by Fontan procedure target liver-specific cells, underscoring a potentially important pathway involved in the liver dysfunction that occurs as a consequence of Fontan palliation. We also found that post-Fontan EV miRNAs were associated with senescence and cell death, whereas pre-Fontan EV miRNAs were associated with stem cell maintenance and epithelial-to-mesenchymal transition. This study shows great potential to identify novel circulating EV biomarkers from Fontan sheep serum that may be used for the diagnosis, prognosis, and therapeutics for patients that have undergone Fontan palliation.

We present the first-in-paediatric uses of a mechanical aspiration system for percutaneous removal of right atrial masses in three patients, including central line-related thrombus and metastatic tumour. Percutaneous mechanical removal of right atrial masses can be performed safely and effectively.

The aim of the study was to determine the variables associated with high-quality (HQ) versus low-quality (LQ) three-dimensional rotational angiography (3DRA) and create guides for optimization of approach to 3DRA in congenital cardiac catheterization (CCC). CCC has adopted 3DRA as a mainstay, but there has not been systematic analysis of approach to and factors associated with HQ 3DRA. This was a single-center, retrospective study of 3DRAs using Canon Infinix-I platform. Reconstructions were graded by 3 interventionalists. Quality was dichotomized into HQ and LQ. Univariable analyses and multivariable logistic regression models were performed. From 8/2016 to 12/2018, 208 3DRAs were performed in 195 CCCs; median age 7 years (2, 16), weight 23 kg (12, 57). The majority of 3DRAs were performed in patients with biventricular physiology (N = 137, 66%) and in pulsatile sites (N = 144, 69%). HQ 3DRA (N = 182, 88%) was associated with greater total injection volume [2.20 mL/kg (1.44, 3.29) vs. 1.62 mL/kg (1.10, 1.98), p = 0.005] and more dilute contrast solution [60% (50, 100) vs. 100% (60, 100), p = 0.007], but not with contrast volume administered (p = 0.2) on univariable analysis. On multivariable logistic regression, HQ 3DRA was significantly associated with patient weight [OR 0.97 (95% CI (0.94, 0.99), p = 0.018], total injection volume [OR 1.04 (95% CI 1.01, 1.07) p = 0.011], and percent contrast solution [OR 0.97 (95% CI 0.95, 1.00), p = 0.022]. These data resulted in creation of scatter plots and a novel 3DRA Nomogram for estimating the probability of HQ 3DRA. This is the first study to create evidence-based contrast dose guides and nomogram for 3DRA in CCC. HQ 3DRA was associated with lower weight, higher total injection volumes, and more dilute contrast solution.

The aim of this study was to evaluate the accuracy of cardiac magnetic resonance (CMR) in predicting coronary artery (CA) compression during transcatheter pulmonary valve implantation (TPVi).TPVi is a widely available option to treat dysfunctional right ventricle (RV)-to-pulmonary artery (PA) conduits, but CA compression is an absolute contraindication. CMR can evaluate coronary anatomy, but its utility in predicting CA compression is not well established.After Institutional Review Board approval was obtained, all patients at 9 centers with attempted TPVi in RV-PA conduits and recent CMR (≤12 months) were analyzed. A core laboratory reviewed all CMR studies for the shortest orthogonal distance from a CA to the conduit, the shortest distance from a CA to the most stenotic area of the conduit, and subjective assessment of CA compression risk.Among 231 patients, TPVi was successful in 198 (86%); in 24 (10%), balloon testing precluded implantation (documented CA compression or high risk). Distance to the RV-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 CA compression. Subjective assessment had the highest AUC (0.78), with 96% negative predictive value. Both distances and qualitative assessment remained independently associated with CA compression when controlling for abnormal coronary anatomy or degree of conduit calcification.CMR can help predict the risk for CA compression during TPVi in RV-PA conduits but cannot completely exclude CA compression. CMR may assist in patient selection and counseling families prior to TPVi, although balloon testing remains essential.

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.

Three-dimensional rotational angiography has become a mainstay of congenital cardiac catheterisation. Augmented reality is an exciting and emerging technology that allows for interactive visualisation of 3D holographic images in the user’s environment. This case series reports on the feasibility of intraprocedural augmented reality visualisation of 3D rotational angiography in five patients with CHD.

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.

While bedside training has always presented its own unique challenges, the COVID-19 pandemic era has intensified barriers to suitable provider and trainee experiences for both patient care and medical education.This project introduced an innovative solution with the Extended Reality International Grand Rounds, a collaboration between the University of Michigan Center for Medical and Surgical Extended Reality and Imperial College London. Three complex cases were presented to trainees through a wireless, extended reality (XR) headset and augmented by holographic visual aids and expert commentary. This pilot rounding experience was performed through the first-person view of one clinician at the bedside.In 2020, 140 attendees participated in XR International Grand Rounds, and 82 (59%) and 61 (44%) completed pre- and postsurveys, respectively. Survey analysis showed that the majority of respondents (65, 79.3%) had very little to no baseline experience with XR technologies and nearly all (75, 91.5%) agreed that the development and implementation of XR curricula are important in medical training, indicating an unmet need. Nearly all respondents (59, 96.7%) found value in the ability to visualize patients' clinical findings in the XR rounding experience and 60 (98.4%) found value in the ability to visualize patient-specific imaging and test findings in an XR format. Limiting exposure to high-risk patients and care team members with this innovative format was believed to be important to 79 (96.3%) respondents at baseline and that perception was unchanged following the event.This solution to a long-standing dilemma, newly stressed by a unique era in medicine, was a successful collaboration using state-of-the-art XR technology. Next steps will include introducing more advanced physical exam visualization and detection and comprehensive evaluation of the patient experience, as well as expanding the international experience in a format that is scalable to other interested institutions.

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.

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.

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.

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.

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.