Standardization of the Norwood Procedure Improves Outcomes in a Medium-Sized Volume Center.

The Norwood operation has become common practice to palliate patients with hypoplastic left heart structures. Surgical technique and postoperative care have improved; yet, there remains significant attrition prior to stage II palliation. The objective of this study is to report outcomes before and after standardizing our approach to the Norwood operation. Patients who underwent the Norwood operation at Children's of Alabama were identified, those who underwent hybrid palliation operations were excluded. Pre- (2015-2020) and post- (2020-January 2023) standardization groups were compared and outcomes analyzed. Ninety-one patients were included (pre-standardization 44 (48.3%) and 47 (51.7%) post-standardization). There were no differences in baseline and intraoperative characteristics at Norwood between the pre- and post-standardization groups. Compared with pre-standardization, post-standardization was associated with decreased time to extubation (OR 0.87, 95%CI 0.79-0.96), inotrope duration (OR 0.92, 95%CI 0.86-0.98) and hospital length of stay (OR 0.98, 95%CI 0.96-0.99). There was a trend toward decreased cardiac arrest, reintervention rates, and interstage mortality for the post-standardization group. A standardized approach to complex neonatal cardiac operations such as the Norwood procedure may improve morbidity and decrease hospital resource utilization. We recommend establishing protocols at an institutional level to optimize outcomes in such high-risk patient populations.

Hybrid Thrombectomy and Central Extracorporeal Membrane Oxygenation for Massive Pulmonary Embolism in a Child.

We describe a hybrid thrombectomy and central extracorporeal membrane oxygenation for a child in cardiogenic shock due to a massive pulmonary embolism.

Hybrid Norwood for Hypoplastic Left Heart Syndrome With Rare Aortic Arch Anatomy.

We describe the hybrid Norwood as first-stage palliation for a patient with hypoplastic left heart syndrome, right aortic arch, right descending aorta, bilateral ductus arteriosus, and left innominate artery arising from the left ductus.

Surgical Experience With Nine Cases of Obstructed Right Aortic Arch.

BACKGROUND: Right-sided aortic arch obstruction is an extremely rare congenital anomaly. A variety of surgical approaches have been described. This study reviews our institutional experience over the last 30 years. METHODS: Our surgical database at the University of Alabama at Birmingham and Children's Hospital of Alabama from 1992 to 2022 was reviewed to include all patients who underwent surgical repair for right-sided aortic arch obstruction. RESULTS: A total of nine patients underwent surgical repair for right-sided aortic arch obstruction. Surgical approach was via thoracotomy (n = 2, 22%), sternotomy (n = 5, 56%), or combined (n = 2, 22%). Primary extended end-to-end anastomosis was utilized for patients with discrete coarctation (n = 1, 11%), reverse subclavian flap for coarctation with associated distal arch hypoplasia (n = 2, 22%), GORE-TEX® tube graft for circumflex aorta (n = 1, 11%), and aortic arch advancement (n = 5, 56%) with or without patch augmentation for those with an interrupted or severely hypoplastic aortic arch. Reintervention was required in one patient (11%) for recoarctation. All patients were discharged in good condition. There was no hospital mortality and at 10.5 years (mean) follow-up there was one late death. CONCLUSION: Right aortic arch obstruction is a rare entity. Surgical approach should be tailored to the anatomy and associated intracardiac defects. Preoperative imaging with a CT angiogram is useful for operative planning. Sternotomy with single-stage primary repair is safe, effective, and our preferred surgical approach for patients with right aortic arch obstruction and associated intracardiac pathology.

Association Between Venous Homografts and Allosensitization After Norwood Procedure.

BACKGROUND: Right ventricle (RV) to pulmonary artery (PA) shunts have become the shunt of choice at many centers for use during the Norwood procedure for single ventricle palliation. Some centers have begun to use cryopreserved femoral or saphenous venous homografts as an alternative to polytetrafluoroethylene (PTFE) for shunt construction. The immunogenicity of these homografts is unknown, and potential allosensitization could have significant implications on transplant candidacy. METHODS: All patients undergoing Glenn procedure at our center between 2013 and 2020 were screened. Patients who initially underwent Norwood procedure with either PTFE or venous homograft RV-PA shunt and had available pre-Glenn serum were included in the study. The primary outcome of interest was panel reactive antibody (PRA) level at the time of Glenn surgery. RESULTS: Thirty-six patients met inclusion criteria (N = 28 PTFE, N = 8 homograft). Patients in the homograft group had significantly higher median PRA levels at the time of Glenn surgery (0% [IQR 0-18] PTFE vs 94% [IQR 74-100] homograft, P = .003). There were no other differences between the two groups. CONCLUSIONS: Despite potential improvements in PA architecture, the use of venous homografts for RV-PA shunt construction at the time of Norwood procedure is associated with significantly elevated PRA level at the time of Glenn surgery. Centers should carefully consider the use of currently available venous homografts given the high percentage of these patients who may require future transplantation.

A Multidisciplinary Surgical Approach to Mediastinal Masses in Children.

The aim of this study is to demonstrate the safety and advantages of a multidisciplinary approach to surgical resection of mediastinal masses in children. Eight patients underwent resection of a mediastinal mass by a team involving both a pediatric general surgeon and pediatric cardiothoracic surgeon. One patient required rapid initiation of cardiopulmonary bypass to complete the tumor resection and repair an aortic injury that occurred when removing adherent tumor from the structure. Perioperative outcomes were excellent for all patients. This series shows that a multidisciplinary surgical approach can be potentially life saving.

Will previous palliative surgery for congenital heart disease be detrimental to subsequent pig heart xenotransplantation?

INTRODUCTION: Pig heart xenotransplantation might act as a bridge in infants with complex congenital heart disease (CHD) until a deceased human donor heart becomes available. Infants develop antibodies to wild-type (WT, i.e., genetically-unmodified) pig cells, but rarely to cells in which expression of the 3 known carbohydrate xenoantigens has been deleted by genetic engineering (triple-knockout [TKO] pigs). Our objective was to test sera from children who had undergone palliative surgery for complex CHD (and who potentially might need a pig heart transplant) to determine whether they had serum cytotoxic antibodies against TKO pig cells. METHODS: Sera were obtained from children with CHD undergoing Glenn or Fontan operation (n = 14) and healthy adults (n = 8, as controls). All of the children had complex CHD and had undergone some form of cardiac surgery. Seven had received human blood transfusions and 3 bovine pericardial patch grafts. IgM and IgG binding to WT and TKO pig red blood cells (RBCs) and peripheral blood mononuclear cells (PBMCs) were measured by flow cytometry, and killing of PBMCs by a complement-dependent cytotoxicity assay. RESULTS: Almost all children and adults demonstrated relatively high IgM/IgG binding to WT RBCs, but minimal binding to TKO RBCs (p < 0.0001 vs WT), although IgG binding was greater in children than adults (p < 0.01). All sera showed IgM/IgG binding to WT PBMCs, but this was much lower to TKO PBMCs (p < 0.0001 vs WT) and was greater in children than in adults (p < 0.05). Binding to both WT and TKO PBMCs was greater than to RBCs. Mean serum cytotoxicity to WT PBMCs was 90% in both children and adults, whereas to TKO PBMCs it was only 20% and < 5%, respectively. The sera from 6/14 (43%) children were cytotoxic to TKO PBMCs, but no adult sera were cytotoxic. CONCLUSIONS: Although no children had high levels of antibodies to TKO RBCs, 13/14 demonstrated antibodies to TKO PBMCs, in 6 of these showed mild cytotoxicity. As no adults had cytotoxic antibodies to TKO PBMCs, the higher incidence in children may possibly be associated with their exposure to previous cardiac surgery and biological products. However, the numbers were too small to determine the influence of such past exposures. Before considering pig heart xenotransplantation for children with CHD, testing for antibody binding may be warranted.

Hemodilution in high-risk cardiac surgery: Laboratory values, physiological parameters, and outcomes.

BACKGROUND: Acute normovolemic hemodilution (ANH) is a blood conservation strategy in cardiac surgery, predominantly used in coronary artery bypass graft (CABG) and/or valve procedures. Although higher complexity cardiac procedures may benefit from ANH, concerns for hemodynamic instability, and organ injury during hemodilution hinder its wider acceptance. Laboratory and physiological parameters during hemodilution in complex cardiac surgeries have not been described. STUDY DESIGN AND METHODS: This observational cohort (2019-2021) study included 169 patients who underwent thoracic aortic repair, multiple valve procedure, concomitant CABG with the aforementioned procedure, and/or redo sternotomies. Patients who received allogeneic blood were excluded. Statistical comparisons were performed between ANH (N = 66) and non-ANH controls (N = 103). ANH consisted of removal of blood at the beginning of surgery and its return after cardiopulmonary bypass. RESULTS: Intraoperatively, the ANH group received more albumin (p = .04) and vasopressor medications (p = .01), while urine output was no different between ANH and controls. Bilateral cerebral oximetry (rSO2 ) values were similar before and after hemodilution. During bypass, rSO2 were discretely lower in the ANH versus control group (right rSO2 p = .03, left rSO2 p = .05). No differences in lactic acid values were detected across the procedural continuum. Postoperatively, no differences in extubation times, intensive care unit length of stay, kidney injury, stroke, or infection were demonstrated. DISCUSSION: This study suggests hemodilution to be a safe and comparable blood conservation technique, even without accounting for potential benefits of reduced allogenic blood administration. The study may contribute to better understanding and wider acceptance of ANH protocols in high-risk cardiac surgeries.

The Genetically Engineered Heart as a Bridge to Allotransplantation in Infants Just Around the Corner?

BACKGROUND: Mortality for infants on the heart transplant waitlist remains unacceptably high, and available mechanical circulatory support is suboptimal. Our goal is to demonstrate the feasibility of utilizing genetically engineered pig (GEP) heart as a bridge to allotransplantation by transplantation of a GEP heart in a baboon. METHODS: Four baboons underwent orthotopic cardiac transplantation from GEP donors. All donor pigs had galactosyl-1,3-galactose knocked out. Two donor pigs had human complement regulatory CD55 transgene and the other 2 had human complement regulatory CD46 and thrombomodulin. Induction immunosuppression included thymoglobulin, and anti-CD20. Maintenance immunosuppression was rapamycin, anti-CD-40, and methylprednisolone. One donor heart was preserved with University of Wisconsin solution and the other three with del Nido solution. RESULTS: All baboons weaned from cardiopulmonary bypass. B217 received a donor heart preserved with University of Wisconsin solution. Ventricular arrhythmias and depressed cardiac function resulted in early death. All recipients of del Nido preserved hearts easily weaned from cardiopulmonary bypass with minimal inotropic support. B15416 and B1917 survived for 90 days and 241 days, respectively. Histopathology in B15416 revealed no significant myocardial rejection but cellular infiltrate around Purkinje fibers. Histopathology in B1917 was consistent with severe rejection. B37367 had uneventful transplant but developed significant respiratory distress with cardiac arrest. CONCLUSIONS: Survival of B15416 and B1917 demonstrates the feasibility of pursuing additional research to document the ability to bridge an infant to cardiac allotransplant with a GEP heart.

Publication Quality Following Short Summer Cancer Research Internships.

Publications of research conducted during short-term research education training programs serve as an outcome measure for students' successes and a program's value. We compared the impact of cancer publications from research conducted during a short-term cancer research education training program, to publications by program participants in the years following completion of the training program and earning an academic or professional degree. Bibliometrics and altmetrics from NIH, ResearchGate, Google Scholar and Scopus® that measure publication impact were collected on cancer publications by student interns in the CaRES (Cancer Research Experiences for Students) R25 program from 1999 to 2017; and on cancer publications in subsequent years by program alumni (students who completed CaRES). Publication characteristics and impact measures were described and compared. Of 558 publications, 206 (37%) were related to CaRES internship projects and 352 (63%) related to any cancer research in which program alumni engaged following their internships. CaRES project publications were cited more frequently and held a higher research interest score than later cancer publications by CaRES alumni but appeared in journals having lower impact factors (p < 0.05). A higher proportion of alumni were first authors of their publications as compared to first authorship by interns (p = 0.02). Research conducted during short-term cancer research programs can be scientifically meaningful and of comparable quality to publications by program alumni who engage in cancer research careers.

Outcomes in Pediatric Post-Cardiotomy ECMO Support With Modification of Systematic Support Strategy.

BACKGROUND: Utilization of extracorporeal membrane oxygenation (ECMO) support in the post-cardiotomy setting is vital to successful perioperative outcomes following pediatric cardiac surgery. Specific analysis of protocolized management strategies and staff preparedness is imperative to optimizing institutional ECMO outcomes. METHODS: All patients requiring post-cardiotomy ECMO support at a single institution from 2013 to 2019 were retrospectively reviewed. In 2015, several modifications were made to the ECMO support paradigm that addressed deficiencies in equipment, critical care protocols, and staff preparedness. Cases were stratified according to era of ECMO support; patients supported prior to paradigm change from 2013 to 2015 (Group EARLY, n = 20), and patients supported following the implementation of systematic modifications from 2016 to 2019 (Group LATE, n = 26). The primary outcomes of interest were survival to decannulation and hospital discharge. RESULTS: Median age at cannulation was 24.5 days (IQR 7-96) and median duration of support was 4 days (IQR 2-8). Overall survival to decannulation was 78.3% (65% EARLY vs. 88.5% LATE, P = .08) and overall survival to hospital discharge was 58.7% (35% EARLY vs. 76.9% LATE, P = .004). CONCLUSION: Systematic modifications to ECMO support strategy and staff preparation are associated with a significant increase in perioperative survival for pediatric patients requiring post-cardiotomy ECMO support.

Effects of acute normovolemic hemodilution on post-cardiopulmonary bypass coagulation tests and allogeneic blood transfusion in thoracic aortic repair surgery: An observational cohort study.

BACKGROUND AND AIM: Perioperative blood transfusion is associated with increased morbidity and mortality. Acute normovolemic hemodilution (ANH) is a blood conservation strategy associated with variable success, and rarely studied in more complex cardiac procedures. The study aim was to evaluate whether ANH improves coagulopathy and reduces blood transfusions in thoracic aortic surgeries. METHODS: Single-center observational cohort study comparing ANH and standard institutional practice in patients who underwent thoracic aortic repair with cardiopulmonary bypass (CPB) from 2019 to 2021. RESULTS: A total of 89 patients underwent ANH and 116 standard practice. There were no significant differences between the groups in terms of demographic or major perioperative characteristics. In the ANH group coagulation tests before and after transfusion of autologous blood showed decreased INR and increased platelets, fibrinogen, all with p < 0.0005. Coagulation results in the ANH and control groups were not statistically different. The average number of transfused allogeneic products per patient was lower in the ANH versus control group: FFP 1.1 ± 1.6 versus 1.9 ± 2.3 (p = 0.003), platelets 0.6 ± 0.8 versus 1.2 ± 1.3 (p = 0.0008), and cryoprecipitate 0.3 ± 0.7 versus 0.7 ± 1.1 (p = 0.008). Reduction in red blood cell transfusion was not statistically significant. The percentage of patients who received any transfusion was 53.9% in ANH and 59.5% in the control group (p = 0.42). There was no significant difference in major adverse outcomes. CONCLUSIONS: ANH is a safe blood conservation strategy for surgical repairs of the thoracic aorta. Laboratory data suggests ANH can improve some coagulation values after separation from CPB, and significantly reduce the number of transfused FFP, platelets and cryoprecipitate.

Impact of induction strategy change on first-year rejection in pediatric heart transplantation at a single center-From postoperative basiliximab to either postoperative anti-thymocyte globulin or preoperative basiliximab.

BACKGROUND: Our pediatric heart transplant center transitioned from post-bypass basiliximab (BAS) induction to either anti-thymocyte globulin (ATG) or pre-bypass BAS. The purpose of this study was to compare first-year rejection rates before and after this change. METHODS: A single-center retrospective analysis was conducted of pediatric heart transplant recipients from 2010 to 2019. Primary outcome was first-year rejection. Bivariate analysis, Kaplan-Meier curves, and multivariable regression were performed across eras. RESULTS: Forty-three early era patients (55%) received post-bypass BAS, and 35 late era patients (45%) received pre-bypass BAS (n = 17) or ATG (n = 18). First-year rejection decreased in the late era (31% vs 53%, p = .05). This finding was more pronounced after excluding infants (38% vs 73%, p = .006). Late era was associated with a decreased likelihood of rejection (all cohort OR 0.19, 95% CI 0.05-0.66; infants excluded OR 0.17, 95% CI 0.04-0.61). No differences in post-transplant lymphoproliferative disease, donor-specific antibody, or infection were observed. CONCLUSIONS: Fewer late era patients receiving ATG or pre-bypass BAS induction had first-year rejection compared to the early era patients receiving standard post-bypass BAS induction. This programmatic shift in induction strategy was readily achievable and potentially effective in reducing first-year rejection.

Tachyarrhythmia as a possible symptom of coronavirus in a neonate diagnosed with transposition of the great arteries.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic poses broad challenges to healthcare systems and providers. The manifestations of this disease are still being described in a variety of different contexts and patient populations. RESULTS: We report the case of a neonate who demonstrated COVID-19 after surgical correction of transposition of the great arteries. In addition, the patient demonstrated an evolving and persistent tachyarrhythmia consistent with neither the most likely postoperative complications nor typical COVID-19. DISCUSSION: The patient had negative preoperative testing for the virus and presented with profound oxygen desaturation and respiratory failure several days postoperatively. This raised concern for a complication of his arterial switch operation. It was found that one of the patient's caregivers was an asymptomatic carrier of COVID-19, and imaging ruled out intracardiac shunting. After initiating treatment for COVID-19, the patient's oxygen requirements and need for anti-arrhythmic agents improved. CONCLUSION: We propose that, despite negative preoperative testing, coronavirus infection may present as refractory tachyarrhythmia, and may be considered along with surgical complications as a cause for unexplained hypoxemia postoperatively.

Gender Differences in Cardiothoracic Surgery Interest Among General Surgery Applicants.

BACKGROUND: Although the overall gender gap in medicine is narrowing, significant gender disparities remain in the cardiothoracic surgery (CTS) field; women represent only 7% of practicing surgeons and 20% of residents. The purpose of this study was to identify gender differences in CTS exposure and interest among fourth year medical students applying to general surgery residency. METHODS: An anonymous survey was emailed to general surgery residency applicants at a major academic program for the 2019 and 2020 application cycles. Data were stratified by gender and analyzed using the χ2 and t tests. RESULTS: Of the 303 responders to the survey, 44% were women. A total of 58% of women were unlikely to be interested in or were definitely not interested in pursuing a career in CTS compared with 35% of men (P < .05). Men were 2.5 times more likely than women to be interested in CTS (odds ratio, 2.5; 95% confidence interval, 1.5 to 4.1). More men had rotated through CTS (55% vs 44%; P = .04) and shadowed a cardiothoracic surgeon (41% vs 29%; P = .03). More than 30% of women interested in CTS reported mentorship as the most important factor in their decision. Mentorship and CTS rotations were both independently associated with CTS interest after adjusting for gender. CONCLUSIONS: Interest in CTS is disappointingly low among women and represents a troublesome disparity that must be addressed. Early exposure to CTS and more mentorship from cardiothoracic surgeons are critical to reverse the current trend. Further studies are necessary to determine factors limiting female exposure to CTS rotations and dissuading female applicants from pursuing careers in CTS.

Attitudes to Cardiac Xenotransplantation by Pediatric Heart Surgeons and Physicians.

BACKGROUND: Cardiac transplantation in early childhood is limited by scarcity of organ donors. Advances in cardiac xenotransplantation (XTx) research suggest that xenografts may one day represent an alternative to allografts. We sought to determine the attitudes among surgeons and cardiologists in the field of pediatric cardiac transplantation toward the potential use of XTx if this clinical option were to become a reality. METHODS: A Likert-scale anonymous survey addressing the use of XTx in pediatric patients was sent to members of the Congenital Heart Surgeons (CHS) Society and the Pediatric Heart Transplant Society. Results were described and compared between the two surgeon/physician groups. RESULTS: Ninety-two CHS and 42 pediatric transplant cardiologists (PTC) responded (N = 134). The potential acceptance of XTx was high in both groups, assuming risks and results were similar to those of cardiac allotransplantation (88% CHS vs 81% PTC; P = .07). When asked if they would recommend a xenograft, if the results were anticipated to be inferior to those of cardiac allotransplantation, as a bridge to a human heart, potential acceptance fell dramatically but remained higher among CHS than PTC (41% vs 17%, p 0.02). Approximately only one-third of CHS and half of PTC preferred primary cardiac XTx for hypoplastic left heart syndrome if there was no waitlist time and had similar outcomes to allotransplantation. CONCLUSIONS: Our findings suggest that potential acceptance of XTx by CHS and PTC would not be a major barrier if XTx demonstrated similar outcomes to allotransplantation. Acceptance by other congenital heart stakeholders remains to be investigated.

Sociodemographic factors, health seeking behaviors, reproductive history, and knowledge of cervical screening among women in Swaziland.

BACKGROUND: Cervical cancer is the leading cause of cancer among women in Swaziland; however, a low rate of cervical screening in this population has led to high rates of morbidity and mortality from cervical cancer. OBJECTIVE: To identify factors associated with lack of cervical screening among women in Swaziland. METHODS: A cross-sectional study was conducted among 300 women aged 18-69 years attending clinics in three regions of Swaziland from May to August of 2014. An investigator-administered questionnaire was used to collect data on socioeconomic factors, health-seeking behaviors, reproductive history, and cervical screening history and knowledge from the women. RESULTS: Adjusted multivariable logistic regression analysis revealed that women < 30 years of age were less likely to receive a cervical exam compared to women ≥30 years of age (Odds Ratio 0.06, 95% Confidence Interval 0.01-0.67). Women who had a tertiary education were almost 6 times more likely to receive a cervical screening (OR 5.83, 95% CI 1.11-30.50). Women who said that they did not know when to receive cervical screening were 73% less likely to have a cervical exam (OR 0.27, 95% CI 0.01-0.74). CONCLUSIONS: Younger age, lower educational level, and lack of knowledge about when to receive a cervical screening affected whether women obtained a cervical screening. This indicates the need for educating women, particularly younger women, about the importance of cervical examinations. Addressing these barriers to screening should lead to a decrease in cervical lesions and cancer, especially in this high HIV-positive population.

Surgical Management of Heterotaxy Syndrome: Current Challenges and Opportunities.

INTRODUCTION: Heterotaxy syndrome presents a unique challenge in surgical management, even in the current era. We hypothesized that certain anatomic subsets merit novel strategies. METHODS: We analyzed morphologic details, surgeries, comorbidities, subsequent admissions, and survival using Kaplan-Meier methods and multivariable risk models from a single-institution experience of 103 consecutive patients with heterotaxy who underwent cardiac surgery between January 1, 1990, and May 31, 2016. RESULTS: Of the 103 patients (50 males and 53 females), 31 had left atrial isomerism, 64 had right atrial isomerism (RAI), and 8 patients' isomerism was indeterminate (IND), with first cardiac operation at a mean 1.0 year (standard deviation ±3.0 years) of age. Kaplan-Meier overall survival estimate was 83.1% at six months, 77.8% at one year, 65.9% at five years, and 52.1% at ten years. Survival was particularly low among RAI following repair of total anomalous pulmonary venous connection (TAPVC) at first operation, with one- and five-year survival of 57% and 46%, respectively. By multivariable analysis, the only risk factor for death during the early phase (hazard model) was repair of TAPVC at the first cardiac operation (hazard ratio [HR]: 4.4, P = .01), and risk factors during the longer term constant phase were atrioventricular valve (AVV) regurgitation (HR: 4.2, P < .01), male gender (HR: 3.7, P < .01), and two-ventricle repair (HR: 3.0, P = .02). Patients with heterotaxy undergoing the Fontan procedure had excellent subsequent survival (85% at ten years). CONCLUSIONS: This analysis of over 100 patients with heterotaxy identified TAPVC requiring initial repair as the major risk factor for early death and important AVV regurgitation as the major risk factor in the longer term. Survival with RAI and early repair of TAPVC were poor, with one-year mortality exceeding 40%. Patients with single ventricle completing the Fontan operation enjoyed outstanding ten-year survival (85%). Initial management of RAI requiring early repair of TAPVC remains challenging. For this high-risk subset, alternative strategies such as early referral for cardiac transplantation evaluation warrant consideration.

Repair of Rare Vascular Ring: Circumflex Aortic Arch with Associated Hypoplasia and Coarctation.

We describe a neonate with an unusual vascular ring formed by a right-sided aortic arch with associated coarctation and distal hypoplasia in the presence of an aberrant left subclavian artery. The descending aorta traveled behind the esophagus to descend on the left side of the spine. A left ductus arteriosus connected to the descending aorta completing the vascular ring, with notable esophageal compression. Surgical correction was accomplished through median sternotomy, resection of the hypoplastic circumflex arch, aortic arch advancement, and end-to-side anastomosis.