Publications by authors named "Christopher E Mascio"

89 Publications

Effects of circulatory arrest and cardiopulmonary bypass on cerebral autoregulation in neonatal swine.

Pediatr Res 2021 May 4. Epub 2021 May 4.

Department of Pediatrics, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.

Background: Cerebral autoregulation mechanisms help maintain adequate cerebral blood flow (CBF) despite changes in cerebral perfusion pressure. Impairment of cerebral autoregulation, during and after cardiopulmonary bypass (CPB), may increase risk of neurologic injury in neonates undergoing surgery. In this study, alterations of cerebral autoregulation were assessed in a neonatal swine model probing four perfusion strategies.

Methods: Neonatal swine (n = 25) were randomized to continuous deep hypothermic cardiopulmonary bypass (DH-CPB, n = 7), deep hypothermic circulatory arrest (DHCA, n = 7), selective cerebral perfusion (SCP, n = 7) at deep hypothermia, or normothermic cardiopulmonary bypass (control, n = 4). The correlation coefficient (LDx) between laser Doppler measurements of CBF and mean arterial blood pressure was computed at initiation and conclusion of CPB. Alterations in cerebral autoregulation were assessed by the change between initial and final LDx measurements.

Results: Cerebral autoregulation became more impaired (LDx increased) in piglets that underwent DH-CPB (initial LDx: median 0.15, IQR [0.03, 0.26]; final: 0.45, [0.27, 0.74]; p = 0.02). LDx was not altered in those undergoing DHCA (p > 0.99) or SCP (p = 0.13). These differences were not explained by other risk factors.

Conclusions: In a validated swine model of cardiac surgery, DH-CPB had a significant effect on cerebral autoregulation, whereas DHCA and SCP did not.

Impact: Approximately half of the patients who survive neonatal heart surgery with cardiopulmonary bypass (CPB) experience neurodevelopmental delays. This preclinical investigation takes steps to elucidate and isolate potential perioperative risk factors of neurologic injury, such as impairment of cerebral autoregulation, associated with cardiac surgical procedures involving CPB. We demonstrate a method to characterize cerebral autoregulation during CPB pump flow changes in a neonatal swine model of cardiac surgery. Cerebral autoregulation was not altered in piglets that underwent deep hypothermic circulatory arrest (DHCA) or selective cerebral perfusion (SCP), but it was altered in piglets that underwent deep hypothermic CBP.
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http://dx.doi.org/10.1038/s41390-021-01525-3DOI Listing
May 2021

Commentary: Easy, Breezy TCPC.

Semin Thorac Cardiovasc Surg 2021 Mar 15. Epub 2021 Mar 15.

Division of Pediatric Cardiothoracic Surgery, Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address:

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http://dx.doi.org/10.1053/j.semtcvs.2021.03.015DOI Listing
March 2021

Large aneurysms and pseudoaneurysms of surgically reconstructed right ventricular outflow tracts.

Cardiol Young 2021 Mar 17:1-3. Epub 2021 Mar 17.

Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Aneurysm and pseudoaneurysm development is a known, albeit uncommon, complication after right ventricular outflow tract surgical reconstruction. Large right ventricular outflow tract aneurysms and pseudoaneurysms have not been extensively described in recent literature and we report our experience with this unusual complication in five patients at our institution over the last 8 years. Although uncommon, this complication has potentially important clinical implications. Thus, clinicians should be aware of its potential, particularly in certain anatomic conditions.
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http://dx.doi.org/10.1017/S1047951121001025DOI Listing
March 2021

Comparison of management strategies for neonates with symptomatic tetralogy of Fallot and weight <2.5 kg.

J Thorac Cardiovasc Surg 2021 Feb 3. Epub 2021 Feb 3.

UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pa.

Objective: To compare management strategies for neonates <2.5 kg with tetralogy of Fallot and symptomatic cyanosis who either undergo staged repair (SR) (initial palliation followed by later complete repair) or primary repair (PR).

Methods: Consecutive neonates with tetralogy of Fallot and symptomatic cyanosis weighing <2.5 kg at initial intervention and between 2005 and 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Primary outcome was mortality and secondary outcomes included component (eg, initial palliation, complete repair, or primary repair) and cumulative (SR: initial palliation followed by later complete repair) hospital and intensive care unit lengths of stay, durations of ventilation, inotrope use, cardiopulmonary bypass time, procedural complications, and reintervention. Outcomes were compared with propensity score adjustments with PR as the reference group.

Results: The cohort included 76 SR (initial palliation: 53 surgical and 23 transcatheter) and 44 PR patients. The observed risk of overall mortality was similar between SR and PR groups (15.8% vs 18.2%: P = .735). The adjusted hazard of mortality remained similar between groups overall (hazard ratio, 0.59; 95% confidence interval, 0.26-1.36; P = .214), as well as during short-term (<4 months: hazard ratio, 0.37; 95% confidence interval, 0.13-1.09; P = .071) and midterm (>4 months: hazard ratio, 1.32; 95% confidence interval, 0.30-5.79; P = .717) follow-up. Reintervention in the first 18 months was common in both groups (53.2% vs 48.4%; hazard ratio, 1.69; 95% confidence interval, 0.96-2.28; P = .072). Adjusted procedural complications and neonatal morbidity burden were overall lower in the SR group. Cumulative secondary outcome burdens largely favored the PR group.

Conclusions: In this study comparing SR and PR treatment strategies for neonates with tetralogy of Fallot and symptomatic cyanosis and weight <2.5 kg, mortality and reintervention burden was highly independent of treatment strategy. Other potential advantages were observed with each approach.
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http://dx.doi.org/10.1016/j.jtcvs.2021.01.100DOI Listing
February 2021

Modeling Tool for Rapid Virtual Planning of the Intracardiac Baffle in Double-Outlet Right Ventricle.

Ann Thorac Surg 2021 Jun 6;111(6):2078-2083. Epub 2021 Mar 6.

Department of Anesthesia and Critical Care, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Division of Pediatric Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Electronic address:

Purpose: Biventricular repair of double-outlet right ventricle (DORV) necessitates the creation of a complex intracardiac baffle. Creation of the optimal baffle design and placement thereof can be challenging to conceptualize, even with 2-dimensional and 3-dimensional images. This report describes a recently developed methodology for creating virtual baffles to inform intraoperative repair.

Description: A total of 3 heart models of DORV were created from cardiac magnetic resonance images. Baffles were created and visualized using custom software.

Evaluation: This report demonstrates application of the tool to virtual planning of the baffle for repair of DORV in 3 cases. Models were examined by a multidisciplinary team, on screen and in virtual reality. Baffles could be rapidly created and revised to facilitate planning of the surgical procedure.

Conclusions: Virtual modeling of the baffle pathway by using cardiac magnetic resonance, creation of physical templates for the baffle, and visualization in virtual reality are feasible and may be beneficial for preoperative planning of complex biventricular repairs in DORV. Further work is needed to demonstrate clinical benefit or improvement in outcomes.
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http://dx.doi.org/10.1016/j.athoracsur.2021.02.058DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8154721PMC
June 2021

Comparison of Management Strategies for Neonates With Symptomatic Tetralogy of Fallot.

J Am Coll Cardiol 2021 Mar;77(8):1093-1106

Department of Pediatrics, University of California San Francisco, San Francisco, California, USA.

Background: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention.

Objectives: This study sought to perform a balanced multicenter comparison of staged repair (SR) (initial palliation [IP] and subsequent complete repair [CR]) versus primary repair (PR) treatment strategies.

Methods: Consecutive neonates with sTOF who underwent IP or PR at ≤30 days of age from 2005 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was death. Secondary outcomes included component (IP, CR, PR) and cumulative (SR): hospital and intensive care unit lengths of stay; durations of cardiopulmonary bypass, anesthesia, ventilation, and inotrope use; and complication and reintervention rates. Outcomes were compared using propensity score adjustment.

Results: The cohort consisted of 342 patients who underwent SR (IP: surgical, n = 256; transcatheter, n = 86) and 230 patients who underwent PR. Pre-procedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more common in the SR group (p ≤0.01). The observed risk of death was not different between the groups (10.2% vs 7.4%; p = 0.25) at median 4.3 years. After adjustment, the hazard of death remained similar between groups (hazard ratio: 0.82; 95% confidence interval: 0.49 to 1.38; p = 0.456), but it favored SR during early follow-up (<4 months; p = 0.041). Secondary outcomes favored the SR group in component analysis, whereas they largely favored PR in cumulative analysis. Reintervention risk was higher in the SR group (p = 0.002).

Conclusions: In this multicenter comparison of SR or PR for management of neonates with sTOF, adjusted for patient-related factors, early mortality and neonatal morbidity were lower in the SR group, but cumulative morbidity and reinterventions favored the PR group, findings suggesting potential benefits to each strategy.
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http://dx.doi.org/10.1016/j.jacc.2020.12.048DOI Listing
March 2021

Venous Flow Variation Predicts Preoperative Pulmonary Venous Obstruction in Children with Total Anomalous Pulmonary Venous Connection.

J Am Soc Echocardiogr 2021 Feb 16. Epub 2021 Feb 16.

Division of Pediatric Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia and Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.

Background: Identifying preoperative pulmonary venous obstruction in total anomalous pulmonary venous connection is important to guide treatment planning and risk prognostication. No standardized echocardiographic definition of obstruction exists in the literature. Definitions based on absolute velocities are affected by technical limitations and variations in pulmonary venous return. The authors developed a metric to quantify pulmonary venous blood flow variation: pulmonary venous variability index (PVVI). The aim of this study was to demonstrate its accuracy in defining obstruction.

Methods: All patients with total anomalous pulmonary venous connection at a single institution were identified. Echocardiograms were reviewed, and maximum (V), mean (V), and minimum (V) velocities along the pulmonary venous pathway were measured. PVVI was defined as (V - V)/V. These metrics were compared with pressures measured on cardiac catheterization. Echocardiographic measures were then compared between patients with and without clinical preoperative obstruction (defined as a need for preoperative intubation, catheter-based intervention, or surgery within 1 day of diagnosis), as well as pulmonary edema by chest radiography and markers of lactic acidosis. One hundred thirty-seven patients were included, with 22 having catheterization pressure recordings.

Results: V and V were not different between patients with catheter gradients ≥ 4 and < 4 mm Hg, while PVVI was significantly lower and V higher in those with gradients ≥ 4 mm Hg. The composite outcome of preoperative obstruction occurred in 51 patients (37%). Absolute velocities were not different between patients with and without clinical obstruction, while PVVI was significantly lower in patients with obstruction. All metrics except V were associated with pulmonary edema; none were associated with blood gas metrics.

Conclusions: The authors developed a novel quantitative metric of pulmonary venous flow, which was superior to traditional echocardiographic metrics. Decreased PVVI was highly associated with elevated gradients measured by catheterization and clinical preoperative obstruction. These results should aid risk assessment and diagnosis preoperatively in patients with total anomalous pulmonary venous connection.
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http://dx.doi.org/10.1016/j.echo.2021.02.007DOI Listing
February 2021

Attrition between the superior cavopulmonary connection and the Fontan procedure in hypoplastic left heart syndrome.

J Thorac Cardiovasc Surg 2020 Oct 22. Epub 2020 Oct 22.

Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, Philadelphia, Pa. Electronic address:

Objective: We investigated the incidence and predictors of failure to undergo the Fontan in children with hypoplastic left heart syndrome who survived superior cavopulmonary connection.

Methods: The cohort consists of all patients with hypoplastic left heart syndrome who survived to hospital discharge after superior cavopulmonary connection between 1988 and 2017. The primary outcome was attrition, which was defined as death, nonsuitability for the Fontan, or cardiac transplantation before the Fontan. Subjects were excluded if they were awaiting the Fontan, were lost to follow-up, or underwent biventricular repair. The study period was divided into 4 eras based on changes in operative or medical management. Attrition was estimated with 95% confidence intervals, and predictors were identified using adjusted, logistic regression models.

Results: Of the 856 hospital survivors after superior cavopulmonary connection, 52 died, 7 were deemed unsuitable for Fontan, and 12 underwent or were awaiting heart transplant. Overall attrition was 8.3% (71/856). Attrition rate did not change significantly across eras. A best-fitting multiple logistic regression model was used, adjusting for superior cavopulmonary connection year and other influential covariates: right ventricle to pulmonary artery shunt at Norwood (P < .01), total support time at superior cavopulmonary connection (P < .01), atrioventricular valve reconstruction at superior cavopulmonary connection (P = .02), performance of other procedures at superior cavopulmonary connection (P = .01), and length of stay after superior cavopulmonary connection (P < .01).

Conclusions: In this study spanning more than 3 decades, 8.3% of children with hypoplastic left heart syndrome failed to undergo the Fontan after superior cavopulmonary connection. This attrition rate has not decreased over 30 years. Use of a right ventricle to pulmonary artery shunt at the Norwood procedure was associated with increased attrition.
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http://dx.doi.org/10.1016/j.jtcvs.2020.10.053DOI Listing
October 2020

Resource Use and Outcomes of Pediatric Congenital Heart Disease Admissions: 2003 to 2016.

J Am Heart Assoc 2021 Feb 6;10(4):e018286. Epub 2021 Feb 6.

Division of Cardiology Cardiac Center, the Children's Hospital of PhiladelphiaUniversity of Pennsylvania Perelman School of Medicine Philadelphia PA.

Background Children with congenital heart disease (CHD) are known to consume a disproportionate share of resources, yet there are limited data concerning trends in resource use and mortality among admitted children with CHD. We hypothesize that charges in CHD-related admissions increased but that mortality improved over time. Methods and Results This study, including patients <18 years old with CHD, examined inpatient admissions from the nationally representative Kids' Inpatient Database from 2003 to 2016 in order to assess the frequency, medical complexity, and outcomes of CHD hospital admissions. A total of 859 843 admissions of children with CHD were identified. CHD admissions increased by 31.8% from 2003 to 2016, whereas overall pediatric admissions decreased by 13.4%. Compared with non-CHD admissions, those with CHD were more likely to be <1 year of age (80.5% versus 63.3%), and to have ≥1 complex chronic condition (39.7% versus 9.3%). For CHD admissions, mortality was higher (2.97% versus 0.31%) and adjusted median charges greater ($48 426 [interquartile range (IQR), $11.932-$161 048] versus $4697 [IQR, $2551-$12 301]) (<0.0001 for all). Among CHD admissions, whereas adjusted median charges increased from $35 577 (IQR, $9303-$110 439) to $61 696 (IQR, $15 212-$219 237), mortality decreased from 3.2% to 2.7% ( <0.0001). CHD admissions accounted for an increased proportion of all inpatient deaths, from 18.0% in 2003 to 24.5% in 2016. Conclusions Children admitted with CHD are 10 times more likely to die than those without CHD and have higher charges. Although the rate of mortality in CHD admissions decreased, children with CHD accounted for an increasing proportion of all pediatric inpatient deaths. Effective resource allocation is critical to optimize outcomes in these high-risk patients.
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http://dx.doi.org/10.1161/JAHA.120.018286DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7955343PMC
February 2021

Does supply meet demand? A comparison of perfusion strategies on cerebral metabolism in a neonatal swine model.

J Thorac Cardiovasc Surg 2020 Dec 11. Epub 2020 Dec 11.

Department of Anesthesiology and Critical Care Medicine, Children's Hospital of Philadelphia, Philadelphia, Pa.

Objective: We aimed to determine the effects of selective antegrade cerebral perfusion compared with other perfusion strategies on indices of cerebral blood flow, oxygenation, cellular stress, and mitochondrial function.

Methods: One-week-old piglets (n = 41) were assigned to 5 treatment groups. Thirty-eight were placed on cardiopulmonary bypass. Of these, 30 were cooled to 18°C and underwent deep hypothermic circulatory arrest (n = 10), underwent selective antegrade cerebral perfusion at 10 mL/kg/min (n = 10), or remained on continuous cardiopulmonary bypass (deep hypothermic cardiopulmonary bypass, n = 10) for 40 minutes. Other subjects remained on normothermic cardiopulmonary bypass (n = 8) or underwent sham surgery (n = 3). Novel, noninvasive optical measurements recorded cerebral blood flow, cerebral tissue oxyhemoglobin concentration, oxygen extraction fraction, total hemoglobin concentration, and cerebral metabolic rate of oxygen. Invasive measurements of cerebral microdialysis and cerebral blood flow were recorded. Cerebral mitochondrial respiration and reactive oxygen species generation were assessed after the piglets were killed.

Results: During hypothermia, deep hypothermic circulatory arrest piglets experienced increases in oxygen extraction fraction (P < .001), indicating inadequate matching of oxygen supply and demand. Deep hypothermic cardiopulmonary bypass had higher cerebral blood flow (P = .046), oxyhemoglobin concentration (P = .019), and total hemoglobin concentration (P = .070) than selective antegrade cerebral perfusion, indicating greater oxygen delivery. Deep hypothermic circulatory arrest demonstrated worse mitochondrial function (P < .05), increased reactive oxygen species generation (P < .01), and increased markers of cellular stress (P < .01). Reactive oxygen species generation was increased in deep hypothermic cardiopulmonary bypass compared with selective antegrade cerebral perfusion (P < .05), but without significant microdialysis evidence of cerebral cellular stress.

Conclusions: Selective antegrade cerebral perfusion meets cerebral metabolic demand and mitigates cerebral mitochondrial reactive oxygen species generation. Excess oxygen delivery during deep hypothermia may have deleterious effects on cerebral mitochondria that may contribute to adverse neurologic outcomes. We describe noninvasive measurements that may help guide perfusion strategies.
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http://dx.doi.org/10.1016/j.jtcvs.2020.12.005DOI Listing
December 2020

Impact of Treatment Strategy on Outcomes in Isolated Pulmonary Artery of Ductal Origin.

Pediatr Cardiol 2021 Mar 4;42(3):533-542. Epub 2021 Jan 4.

Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Isolated pulmonary artery (PA) of ductal origin (IPADO) is a rare cardiac defect which requires surgical repair, with or without preceding palliation. We sought to determine the impact of treatment strategy on outcomes. Retrospective study of consecutive patients with IPADO that underwent staged or primary repair from 1/05 to 9/16 at 6 Congenital Cardiac Research Collaborative centers. Patients with single ventricle physiology, major aortopulmonary collaterals, or bilateral IPADO were excluded. Primary outcome was isolated PA z-score at late follow-up. Secondary outcomes included PA symmetry index (isolated:confluent PA diameter) and reintervention burden. Propensity score adjustment was used to account for baseline differences. Of 60 patients in the study cohort, 26 (43%) underwent staged and 34 (57%) primary repair. The staged and primary repair groups differed in weight at diagnosis and presence of other heart disease but not in baseline PA dimensions. Staged patients underwent ductal stent (n = 16) or surgical shunt (n = 10) placement followed by repair at 210 vs. 21 days in the primary repair group (p < 0.001). At median follow-up of 4.5 years post-repair, after adjustment, isolated PA z-score (- 0.74 [- 1.75, - 0.26] vs. - 1.95 [- 2.91, - 1.59], p = 0.012) and PA symmetry index (0.81 [0.49, 1.0] vs. 0.55 [0.48, 0.69], p = 0.042) significantly favored the staged repair group. Freedom from PA reintervention was not different between groups (adjusted HR 0.78 [0.41, 1.48]; p = 0.445). A staged approach to repair of IPADO is associated with superior isolated PA size and symmetry at late follow-up. Consideration should be given to initial palliation in IPADO patients, when feasible.
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http://dx.doi.org/10.1007/s00246-020-02511-yDOI Listing
March 2021

Increased cerebral mitochondrial dysfunction and reactive oxygen species with cardiopulmonary bypass.

Eur J Cardiothorac Surg 2021 Jun;59(6):1256-1264

Division of Anesthesia and Critical Care Medicine, Children's Hospital of Philadelphia, Philadelphia, PA, USA.

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Objectives: Neurodevelopmental injury after cardiac surgery using cardiopulmonary bypass (CPB) for congenital heart defects is common, but the mechanism behind this injury is unclear. This study examines the impact of CPB on cerebral mitochondrial reactive oxygen species (ROS) generation and mitochondrial bioenergetics.

Methods: Twenty-three piglets (mean weight 4.2 ± 0.5 kg) were placed on CPB for either 1, 2, 3 or 4 h (n = 5 per group) or underwent anaesthesia without CPB (sham, n = 3). Microdialysis was used to measure metabolic markers of ischaemia. At the conclusion of CPB or 4 h of sham, brain tissue was harvested. Utilizing high-resolution respirometry, with simultaneous fluorometric analysis, mitochondrial respiration and ROS were measured.

Results: There were no significant differences in markers of ischaemia between sham and experimental groups. Sham animals had significantly higher mitochondrial respiration than experimental animals, including maximal oxidative phosphorylation capacity of complex I (OXPHOSCI) (3.25 ± 0.18 vs 4-h CPB: 1.68 ± 0.10, P < 0.001) and maximal phosphorylating respiration capacity via convergent input through complexes I and II (OXPHOSCI+CII) (7.40 ± 0.24 vs 4-h CPB: 3.91 ± 0.20, P < 0.0001). At 4-h, experimental animals had significantly higher ROS related to non-phosphorylating respiration through complexes I and II (ETSCI+CII) than shams (1.08 ± 0.13 vs 0.64 ± 0.04, P = 0.026).

Conclusions: Even in the absence of local markers of ischaemia, CPB is associated with decreased mitochondrial respiration relative to shams irrespective of duration. Exposure to 4 h of CPB resulted in a significant increase in cerebral mitochondrial ROS formation compared to shorter durations. Further study is needed to improve the understanding of cerebral mitochondrial health and its effects on the pathophysiology of neurological injury following exposure to CPB.
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http://dx.doi.org/10.1093/ejcts/ezaa439DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8203249PMC
June 2021

Commentary: Truncus among us.

J Thorac Cardiovasc Surg 2020 May 30. Epub 2020 May 30.

Division of Pediatric Cardiothoracic Surgery, Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pa. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2020.05.050DOI Listing
May 2020

Preoperative echocardiographic parameters predict primary graft dysfunction following pediatric lung transplantation.

Pediatr Transplant 2021 Mar 19;25(2):e13858. Epub 2020 Oct 19.

Division of Critical Care Medicine, Department of Anesthesiology and Critical Care Medicine, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.

The importance of preoperative cardiac function in pediatric lung transplantation is unknown. We hypothesized that worse preoperative right ventricular (RV) systolic and worse left ventricular (LV) diastolic function would be associated with a higher risk of primary graft dysfunction grade 3 (PGD 3) between 48 and 72 hours. We performed a single center, retrospective pilot study of children (<18 years) who had echocardiograms <1 year prior to lung transplantation between 2006 and 2019. Conventional and strain echocardiography parameters were measured, and PGD was graded. Area under the receiver operating characteristic (AUROC) curves and logistic regression were performed. Forty-one patients were included; 14 (34%) developed PGD 3 and were more likely to have pulmonary hypertension (PH) as the indication for transplant (P = .005). PGD 3 patients had worse RV global longitudinal strain (P = .01), RV free wall strain (FWS) (P = .003), RV fractional area change (P = .005), E/e' (P = .01) and lateral e' velocity (P = .004) but not tricuspid annular plane systolic excursion (P = .61). RV FWS (AUROC 0.79, 95% CI 0.62-0.95) and lateral e' velocity (AUROC 0.87, 95% CI 0.68-1.00) best discriminated PGD 3 development and showed the strongest association with PGD 3 (RV FWS OR 3.87 [95% CI 1.59-9.43], P = .003; lateral e' velocity OR 0.10 [95% CI 0.01-0.70], P = .02). These associations remained when separately adjusting for age, weight, primary PH diagnosis, ischemic time, and bypass time. In this pilot study, worse preoperative RV systolic and worse LV diastolic function were associated with PGD 3 and may be modifiable recipient risk factors in pediatric lung transplantation.
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http://dx.doi.org/10.1111/petr.13858DOI Listing
March 2021

Mechanical Support of the Failing Fontan Circulation.

Semin Thorac Cardiovasc Surg 2021 Summer;33(2):454-458. Epub 2020 Sep 23.

Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address:

The Fontan is the final common pathway for most single ventricle lesions. It is not a durable circulation and many Fontan patients eventually present for advanced heart failure therapies including mechanical circulatory support. While there have been advancements in available durable devices, many pediatric patients have size, anatomical, and physiologic constraints that make placement of adult ventricular assist devices challenging. And, Fontan failure comes in different varieties, including failure with preserved systolic function. This subset of patients requires either a subpulmonary assist device or a total artificial heart. Unique operative maneuvers, including atrial cannulation and atrioventricular valve manipulation have allowed many failing Fontan patients to be bridged to transplant after recovery of end-organ dysfunction and improved nutrition. The Advanced Cardiac Therapies Improving Outcomes Network is a multicenter learning network of pediatric hospitals implanting, managing, and studying ventricular assist devices in children and adults with congenital heart disease. This network is advancing the knowledge of outcomes and approaches to mechanical circulatory support in those with congenital heart disease, including Fontans. The congenital heart disease community continues to work toward and hope for a smaller durable device that will help this complex cohort of patients.
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http://dx.doi.org/10.1053/j.semtcvs.2020.09.005DOI Listing
September 2020

Decreasing Interstage Mortality After the Norwood Procedure: A 30-Year Experience.

J Am Heart Assoc 2020 10 23;9(19):e016889. Epub 2020 Sep 23.

Division of Cardiothoracic Surgery Department of Surgery The Children's Hospital of Philadelphia, and Perelman School of Medicine at the University of Pennsylvania Philadelphia PA.

Background The superior cavo-pulmonary connection was introduced at our institution in 1988 for infants undergoing surgery for hypoplastic left heart syndrome. Patients with hypoplastic left heart syndrome remain at high risk for mortality in the time period between the Norwood procedure and the superior cavo-pulmonary connection. The primary objectives of this study were to compare interstage mortality across 4 eras and analyze factors that may impact interstage mortality. Methods and Results Patients with hypoplastic left heart syndrome who underwent the Norwood procedure, were discharged from the hospital, and were eligible for superior cavo-pulmonary connection between January 1, 1988, and December 31, 2017, were included. The study period was divided into 4 eras based on changes in operative or medical management. Mortality rates were estimated with 95% CIs. Adjusted and unadjusted logistic regression models were used to identify risk factors for mortality. There were 1111 patients who met the inclusion criteria. Overall, interstage mortality was 120/1111 (10.8%). Interstage mortality was significantly lower in era 4 relative to era 1 (4.6% versus 13.4%; =0.02) during the time that age at the superior cavo-pulmonary connection was the lowest (135 days; <0.01) and the interstage monitoring program was introduced. In addition, use of the right ventricle to pulmonary artery shunt was associated with decreased interstage mortality (=0.02) and was more routinely practiced in era 4. Conclusions During this 30-year experience, the risk of interstage mortality decreased significantly in the most recent era. Factors that coincide with this finding include younger age at superior cavo-pulmonary connection, introduction of an interstage monitoring program, and increased use of the right ventricle to pulmonary artery shunt.
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http://dx.doi.org/10.1161/JAHA.120.016889DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7792374PMC
October 2020

Development and Validation of a Seizure Prediction Model in Neonates After Cardiac Surgery.

Ann Thorac Surg 2021 Jun 29;111(6):2041-2048. Epub 2020 Jul 29.

Department of Biostatistics and Epidemiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.

Background: Electroencephalographic seizures (ESs) after neonatal cardiac surgery are often subclinical and have been associated with poor outcomes. An accurate ES prediction model could allow targeted continuous electroencephalographic monitoring (CEEG) for high-risk neonates.

Methods: ES prediction models were developed and validated in a multicenter prospective cohort where all postoperative neonates who underwent cardiopulmonary bypass (CPB) also underwent CEEG.

Results: ESs occurred in 7.4% of neonates (78 of 1053). Model predictors included gestational age, head circumference, single-ventricle defect, deep hypothermic circulatory arrest duration, cardiac arrest, nitric oxide, extracorporeal membrane oxygenation, and delayed sternal closure. The model performed well in the derivation cohort (c-statistic, 0.77; Hosmer-Lemeshow, P = .56), with a net benefit (NB) over monitoring all and none over a threshold probability of 2% in decision curve analysis (DCA). The model had good calibration in the validation cohort (Hosmer-Lemeshow, P = .60); however, discrimination was poor (c-statistic, 0.61), and in DCA there was no NB of the prediction model between the threshold probabilities of 8% and 18%. By using a cut point that emphasized negative predictive value in the derivation cohort, 32% (236 of 737) of neonates would not undergo CEEG, including 3.5% (2 of 58) of neonates with ESs (negative predictive value, 99%; sensitivity, 97%).

Conclusions: In this large prospective cohort, a prediction model of ESs in neonates after CPB had good performance in the derivation cohort, with an NB in DCA. However, performance in the validation cohort was weak, with poor discrimination, poor calibration, and no NB in DCA. These findings support CEEG of all neonates after CPB.
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http://dx.doi.org/10.1016/j.athoracsur.2020.05.157DOI Listing
June 2021

Standardization of the Perioperative Management for Neonates Undergoing the Norwood Operation for Hypoplastic Left Heart Syndrome and Related Heart Defects.

Pediatr Crit Care Med 2020 09;21(9):e848-e857

Department of Pediatrics, Division of Cardiology, Perelman School of Medicine at the University of Pennsylvania, Children's Hospital of Philadelphia, Philadelphia, PA.

Objectives: In-hospital complications after the Norwood operation for single ventricle heart defects account for the majority of morbidity and mortality. Inpatient care variation occurs within and across centers. This multidisciplinary quality improvement project standardized perioperative management in a large referral center.

Design: Quality improvement project.

Setting: High volume cardiac center, tertiary care children's hospital.

Patients: Neonates undergoing Norwood operation.

Interventions: The quality improvement team developed and implemented a clinical guideline (preoperative admission to 48 hr after surgery). The composite process metric, Guideline Adherence Score, contained 13 recommendations in the guideline that reflected consistent care for all patients.

Measurements And Main Results: One-hundred two consecutive neonates who underwent Norwood operation (January 1, 2013, to July 12, 2016) before guideline implementation were compared with 50 consecutive neonates after guideline implementation (July 13, 2016, to May 4, 2018). No preguideline operations met the goal Guideline Adherence Score. In the first 6 months after guideline implementation, 10 of 12 operations achieved goal Guideline Adherence Score and continued through implementation, reaching 100% for the last 10 operations. Statistical process control analysis demonstrated less variability and decreased hours of postoperative mechanical ventilation and cardiac ICU length of stay during implementation. There were no statistically significant differences in major hospital complications or in 30-day mortality. A higher percentage of patients were extubated by postoperative day 2 after guideline implementation (67% [30/47] vs 41% [41/99], respectively; p = 0.01). Of these patients, reintubation within 72 hours of extubation significantly decreased after guideline implementation (0% [0/30] vs 17% [7/41] patients, respectively; p = 0.02).

Conclusions: This initiative successfully implemented a standardized perioperative care guideline for neonates undergoing the Norwood operation at a large center. Positive statistical process control centerline shifts in Guideline Adherence Score, length of postoperative mechanical ventilation, and cardiac ICU length of stay were demonstrated. A higher percentage were successfully extubated by postoperative day 2. Establishment of standard processes can lead to best practices to decrease major adverse events.
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http://dx.doi.org/10.1097/PCC.0000000000002478DOI Listing
September 2020

Increasing Pump Speed During Exercise Training Improves Exercise Capacity in Children with Ventricular Assist Devices.

ASAIO J 2021 04;67(4):449-456

From the Division of Cardiology, Children's Hospital of Philadelphia; Philadelphia, Pennsylvania.

Exercise rehabilitation during pediatric ventricular assist device (VAD) support aims to improve musculoskeletal strengthening while awaiting heart transplantation (HT). This study aimed to determine whether increasing VAD pump speed during exercise testing and training improves exercise capacity. A single-center cohort study was performed comparing changes in exercise capacity on serial cardiopulmonary exercise testing (CPET) after exercise training at a fixed VAD pump speed (historical cohort from 2014 to 2017) compared with a prospective cohort (2017-2019) who underwent increasing pump speed during exercise training. All children were supported with intracorporeal continuous-flow VAD. Four subjects (13 ± 2.8 years) were included in the historical cohort, and 6 subjects (14 ± 1.7 years) were enrolled in the prospective cohort. Ninety percent had dilated cardiomyopathy, and one had single ventricle Fontan physiology. Baseline maximal oxygen consumption (VO2) was 19 ± 6.3 ml/kg/min. After exercise training with increased pump speed, there was substantial improvement in aerobic capacity (maximal VO2 increased 42% vs. decreased 3%, respectively) and working capacity (maximal work increased 49% vs. 13%, respectively) compared with fixed pump speed. There were no adverse events reported in either the fixed or increased pump speed cohorts. Increasing VAD pump speed during exercise training results in substantial improvement in both physical working and aerobic capacity compared a fixed pump speed in children on VAD support regardless of single or biventricular ventricle physiology. Further study of a larger cohort is needed to validate these findings to improve the approach to pediatric cardiac rehabilitation in this population.
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http://dx.doi.org/10.1097/MAT.0000000000001231DOI Listing
April 2021

Outcomes for the superior cavopulmonary connection in children with hypoplastic left heart syndrome: a 30-year experience.

Eur J Cardiothorac Surg 2020 10;58(4):809-816

Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Objectives: The objective of this study was to estimate hospital mortality and length of stay (LOS) for children with hypoplastic left heart syndrome undergoing superior cavopulmonary connection (SCPC).

Methods: All hypoplastic left heart syndrome interstage survivors who underwent SCPC between 1 January 1988 and 31 December 2017 were included. The study period was divided into 4 eras based on changes in operative or medical management. Mortality rates were estimated using standard binomial proportions. Adjusted and unadjusted logistic regression models were used to identify risk factors for mortality and LOS.

Results: The most common procedures for the cohort (n = 958) were Hemi-Fontan (57.3%) or Bidrectional Glenn shunt (35.7%). The mortality was 4.1% overall and decreased in all 3 later eras compared to era 1. Factors associated with mortality in a multiple covariate model included longer total support time, earlier gestational age, longer LOS at the Norwood Procedure and need for additional procedures. Overall, the median LOS was 7.0 days with a decrease from eras 1 to 2 and plateaued in eras 3 and 4. Predictors of longer LOS included genetic anomaly, longer Norwood LOS, additional procedures, lower weight at surgery and longer total support time. The type of SCPC was not associated with mortality or LOS.

Conclusions: In this large cohort of patients with hypoplastic left heart syndrome undergoing SCPC, hospital mortality has decreased significantly. LOS initially declined but plateaued in recent eras. The risk factors for mortality and longer LOS are related to patient and procedural complexity, especially the need for additional procedures at the time of SCPC.
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http://dx.doi.org/10.1093/ejcts/ezaa117DOI Listing
October 2020

Commentary: You like potato and I like potahto.

J Thorac Cardiovasc Surg 2021 02 20;161(2):400-401. Epub 2020 Apr 20.

Division of Pediatric Cardiothoracic Surgery, Children's Hospital of Philadelphia, and Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pa. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2020.04.029DOI Listing
February 2021

Three-Dimensional Modeling of Surgically Implanted Stent-Based Valves in the Mitral Position in Children.

Ann Thorac Surg 2020 08 18;110(2):670-675. Epub 2020 Mar 18.

Department of Anesthesia and Critical Care, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Division of Pediatric Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Electronic address:

Purpose: In children with a mitral annulus too small to accommodate traditional prostheses, surgical implantation of stent-based valves is a promising option. However no reliable preoperative methods exist to guide patient selection, device sizing, and positioning. We describe a novel methodology to visualize and quantify device fit in 3-dimensional echocardiogram (3DE)-derived heart models.

Description: Heart models were created from existing preoperative 3DEs using custom software. Valve models were virtually implanted into the models, and both device fit and left ventricular outflow tract (LVOT) area were quantified.

Evaluation: The 3DEs of 3 patients who underwent Melody valve placement in the mitral position were retrospectively modeled: 1 with LVOT obstruction, 1 with perivalvar leak, and 1 without complications. In all cases 2-dimensional measurements underestimated 3D annular dimensions, and the patient with clinical LVOT obstruction had the lowest predicted LVOT area-to-aortic area ratio (0.5).

Conclusions: 3DE-based preoperative modeling of surgical implantation of stent-based valves in the mitral position may improve quantification of mitral valve dimensions and inform risk stratification for potential LVOT obstruction.
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http://dx.doi.org/10.1016/j.athoracsur.2020.02.020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8028013PMC
August 2020

Early experience with the HeartMate 3 continuous-flow ventricular assist device in pediatric patients and patients with congenital heart disease: A multicenter registry analysis.

J Heart Lung Transplant 2020 06 13;39(6):573-579. Epub 2020 Feb 13.

Cincinnati Children's Hospital Medical Center, Heart Institute, Cincinnati, Ohio.

Background: The HeartMate 3 ventricular assist device (VAD) is a newer centrifugal continuous-flow VAD used for bridge-to-transplant and destination therapy in adults. However, there is limited experience regarding its use in children and adults with complex congenital heart disease (CHD).

Methods: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) is a multicenter learning network comprised of pediatric hospitals implanting VADs in children and adults with complex CHD. We examined the outcomes of patients undergoing HeartMate 3 implantation at an ACTION center between December 2017 and September 2019.

Results: The HeartMate 3 was implanted in 35 patients at 9 ACTION centers, with a median age of 15.7 (8.8-47.3) years, median weight of 65.7 (19.1-114.1) kg, and median body surface area (BSA) of 1.74 (0.78-2.36) m. Of the cohort, 14 patients (40%) weighed <60 kg. Diagnoses included dilated cardiomyopathy (63%), dilated cardiomyopathy in neuromuscular disease (20%), and CHD (17%). Of those with CHD, most had a Fontan circulation. With a median 78 days of follow-up, there was 1 death on device (97% survival); 20 out of 35 (57%) underwent transplantation with no post-transplantation mortality. There were no episodes of stroke or pump thrombosis.

Conclusions: Use of the HeartMate 3 in ACTION centers was associated with a low incidence of mortality and adverse events. Patients as small as 19 kg (BSA 0.78 m) were successfully implanted and supported, indicating that this device may be appropriate for older children and small adults.
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http://dx.doi.org/10.1016/j.healun.2020.02.007DOI Listing
June 2020

Pulse Oximetry Screening Has Not Changed Timing of Diagnosis or Mortality of Critical Congenital Heart Disease.

Pediatr Cardiol 2020 Jun 27;41(5):899-904. Epub 2020 Feb 27.

Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA.

This study evaluates the effectiveness of mandatory pulse oximetry screening. The objective is to evaluate whether mandatory pulse oximetry testing had decreased the late critical congenital heart disease (CCHD) diagnosis rate and reduced mortality in neonatal subjects. This was a single center, retrospective cohort study comparing the timing of diagnosis of CCHD between neonates undergoing cardiac surgery in 2009-2010, prior to mandatory pulse oximetry screening, and neonates in 2015-2016, after mandatory pulse oximetry screening was instituted. Follow-up was for 1 year. We defined CCHD as lesions requiring surgical correction within 30 days of life. Exclusions included: pacemaker insertions, vascular ring divisions, closure of patent ductus arteriosus, arterial cutdown, or extracorporeal membrane oxygenation cannulation without structural heart disease as the sole procedure, or if subjects were born at home. Infants diagnosed prior to discharge from birth hospital were defined as early postnatal; late postnatal subjects were diagnosed after birth hospital discharge. In-hospital mortality and 1-year mortality were measured. A total of 527 neonates were included; 251 (47.6%) comprised the pre-mandatory pulse oximetry screening cohort (2009-2010). Only 3.6% of the 2009-2010 cohort and 4.3% of the 2015-2016 cohort were diagnosed late (p = 0.66). One-year mortality decreased during the study period (17.2% in 2009-2010 vs 10.5% in 2015-2016, p = 0.03). There were no deaths in the late CCHD diagnosis groups. Mandatory pulse oximetry screening legislation has not changed the late postnatal diagnosis rate at our institution. Mortality for neonatal CCHD has declined, but this decline is not attributable to mandatory pulse oximetry screening.
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http://dx.doi.org/10.1007/s00246-020-02330-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7319863PMC
June 2020

Commentary: Unicorns and leprechauns.

J Thorac Cardiovasc Surg 2020 04 23;159(4):1462-1463. Epub 2019 Dec 23.

Division of Pediatric Cardiothoracic Surgery-Department of Surgery, Children's Hospital of Philadelphia, Philadelphia, Pa; Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pa. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2019.12.017DOI Listing
April 2020

Extracorporeal membrane oxygenation as a novel management strategy for interventricular septal hematoma following ventricular septal defect repair.

J Thorac Cardiovasc Surg 2020 05 16;159(5):1936-1940. Epub 2019 Oct 16.

Division of Cardiothoracic Surgery, Department of Surgery, The Children's Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pa.

Objectives: Interventricular septal hematoma (IVSH) is a rare complication, which may result from ventricular septal defect (VSD) repair. IVSH can result in conduction and/or hemodynamic abnormalities related to impaired ventricular filling or outflow tract obstruction. We report the novel use of extracorporeal membrane oxygenation (ECMO) for management.

Methods: Echocardiography reports (January 1980 to December 2016) were searched for the term "hematoma" in our institutional database and reviewed to determine appropriate cases. Charts and imaging (reports) data were abstracted. All intraoperative and select postoperative echocardiograms were reread by a pediatric cardiologist.

Results: N = 12 patients with IVSH. Mean age and weight at surgery were 59 ± 41 days and 3.4 (2.9-5.1) kg, respectively, while the most frequent diagnosis was tetralogy of Fallot. Although all patients had intraoperative transesophageal echocardiography (TEE), only 55% (6 of 11, missing [m] = 1) of IVSH were discovered intraoperatively. Of the 5 patients not discovered intraoperatively (m = 1), IVSH was postoperatively detected secondary to arrhythmia/decompensation by echocardiogram 10.1 ± 7.9 hours postoperatively. Five patients (42%) were managed with ECMO (1 unable to separate from bypass). Overall mortality was 33%. For patients in whom ECMO was used, 2 of 5 (40%) survived. Mean time to IVSH resolution in all survivors was 20 ± 185 days.

Conclusions: IVSH from VSD repair can result in clinical decompensation and mortality. This may relate to the high proportion missed intraoperatively. ECMO should be considered an important modality, which can allow for IVSH resolution. However, considerations must be made to allow for appropriate anticoagulation to avoid hematoma expansion and repeat imaging during ECMO to continually assess the interventricular septum.
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http://dx.doi.org/10.1016/j.jtcvs.2019.09.150DOI Listing
May 2020

Staged Versus Complete Repair in the Symptomatic Neonate With Tetralogy of Fallot.

Ann Thorac Surg 2020 03 26;109(3):802-808. Epub 2019 Nov 26.

Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address:

Background: The optimal management of tetralogy of Fallot (TOF) in symptomatic neonates remains unknown. We compared outcomes for those undergoing palliation vs complete repair in the neonatal period.

Methods: In a retrospective cohort study of symptomatic neonates with TOF who had a neonatal complete repair (group 1, n = 112) or staged repair (group 2, n = 26) from 2000 to 2013, we compared outcomes at 4 time points: neonatal complete repair vs palliation (group 1 vs 2A), neonatal vs later complete repair (group 1 vs 2B), the single vs combined admissions to achieve a complete repair (group 1 vs group 2A + 2B), and cumulative events 2 years after complete repair for both groups.

Results: Demographics, anatomy, comorbidities, surgical approach, and mortality were similar between groups 1 and 2. Group 1 had a longer duration of cardiopulmonary bypass and deep hypothermic circulatory arrest and more postprocedure cardiac events compared with group 2A; a longer duration of intubation, intensive care, and postprocedure hospital stay compared with groups 2A and 2B; and a longer total hospital stay compared with group 2B. With combined admissions for group 2, there was no difference in the total duration of intensive care, total hospital stay, or reinterventions compared with group 1.

Conclusions: Both management options result in similar survival; however, early morbidity was greater with neonatal complete repair. The impact of increased neonatal exposures, such as cardiopulmonary bypass, deep hypothermic circulatory arrest, and intensive care, on neurocognitive development requires further study but should be considered when choosing an optimal strategy.
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http://dx.doi.org/10.1016/j.athoracsur.2019.10.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7054157PMC
March 2020

Commentary: Unicorns and leprechauns.

J Thorac Cardiovasc Surg 2019 Oct 3. Epub 2019 Oct 3.

Division of Pediatric Cardiothoracic Surgery, Department of Surgery, Children's Hospital of Philadelphia, Philadelphia, Pa; Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pa. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2019.09.112DOI Listing
October 2019

Thirty years and 1663 consecutive Norwood procedures: Has survival plateaued?

J Thorac Cardiovasc Surg 2019 07 28;158(1):220-229. Epub 2019 Feb 28.

Division of Cardiothoracic Surgery, Department of Surgery, Children's Hospital of Philadelphia, Philadelphia, Pa.

Objective: Hypoplastic left heart syndrome is one of the most common and challenging lesions requiring surgical intervention in the neonatal period. The Norwood procedure for hypoplastic left heart syndrome was first reported in 1983. The objective of this study was to describe early outcomes after the Norwood procedure at a single institution over 30 years.

Methods: This retrospective cohort study included all patients with hypoplastic left heart syndrome (and variants) who underwent the Norwood procedure between January 1984 and May 2014 at a single institution. The study period was divided into 6 eras: era 1, 1984 to 1988; era 2, 1989 to 1993; era 3, 1994 to 1998; era 4, 1999 to 2003; era 5, 2004 to 2008; and era 6, 2009 to 2014. The primary outcome was in-hospital mortality after the Norwood procedure. Binomial point estimates complete with 95% confidence intervals (CL) were computed for the entire cohort and by era.

Results: During the study period, 1663 infants underwent the Norwood procedure. Overall in-hospital mortality was 25.9% (CL, 23.8-28.0). Mortality by chronologic era was 40.4% (CL, 34.9-45.9), 33.6% (CL, 29.2-37.9), 28.7% (CL, 22.8-34.6), 14.9% (CL, 10.4-19.3), 11.2% (CL, 7.4-15.0), and 15.7% (CL, 10.3-21.1). Survival was improved in eras 4 to 6 compared with eras 1 to 3 (P all < .03). Anomalous pulmonary drainage, moderate to severe atrioventricular valve regurgitation, lower birth weight, earlier era, younger gestational age, genetic anomaly, preterm birth, race other than white or African-American, and lower weight at the Norwood procedure were associated with increased mortality. Mortality was greatest in patients with 3 or more risk factors. In the best-fitting multiple covariate model, anomalous pulmonary venous drainage, gestational age in weeks, genetic anomaly, and race other than white and African American were statistically significant contributors, after adjusting for era.

Conclusions: Survival after the Norwood procedure has plateaued despite improvements in diagnosis, perioperative care, and surgical techniques. Nonmodifiable patient characteristics are important determinants of the risk of mortality.
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http://dx.doi.org/10.1016/j.jtcvs.2018.12.117DOI Listing
July 2019