Outcomes for Children With Vascular Ring Repair Using a Simple Surgical Approach.

INTRODUCTION: Vascular Rings (VRs) are congenital malformations of the aortic arch that can compress the trachea and esophagus producing symptoms. Approximately, 5%-10% of patients experience persistent symptoms postrepair and 9% require reintervention. There are various approaches to repair-simple ligation and division of the ring or more complex procedures. Our objective was to describe outcomes for VR repair using a "simple" surgical approach. METHODS: We identified patients who underwent VR repair from 2012 to 2022 at our institution. Clinical and surgical data, demographics, intraoperative, and postoperative outcomes were extracted from their electronic medical records. Outcomes were analyzed and regression analysis was used to identify risk factors for residual symptoms after repair. RESULTS: Ninety three patients with VR repair were included. Prevalence of symptoms were reduced following surgery (80% preoperative versus 13% postoperative, P 0.03). Tracheomalacia also decreased from 16% to 7% postrepair. No deaths were reported in our cohort and 2% of our patients required reintervention. Multivariable regression analysis showed that preoperative tracheomalacia was associated with having persistent symptoms after VR repair (odds ratio 6.2, 95% CI 1.02-37.6). CONCLUSIONS: Our institutional experience using a simple surgical approach showed a significant decrease in symptoms, a very low reintervention rate and no mortality. Preoperative tracheomalacia can be a risk factor for persistent symptoms postrepair. We believe a simple surgical approach for children with complete VR repair can be effective in relieving symptoms.

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.

Central Extracorporeal Membrane Oxygenation Support Following Calcium Channel Blocker Overdose in Children.

Refractory vasodilatory shock (RVS) following massive calcium channel blocker (CCB) overdose remains a challenging clinical entity. Peripheral venoarterial extracorporeal membrane oxygenation (ECMO) has proven useful in several cases of CCB intoxication, however, its use in the pediatric population poses unique challenges given the generally small size of pediatric peripheral vasculature in comparison to the high flow rates necessary for adequate mechanical circulatory support. As a result of these challenges, our group has adopted a "primary" central ECMO cannulation approach to the treatment of children and adolescents admitted to our center with profound RVS after CCB ingestion. We present four cases within the last year using this approach. All patients were successfully discharged from the hospital with no late morbidity at most recent follow-up. Central ECMO support in cases of massive vasodilatory shock following CCB overdose is safe and effective and should be considered early in the clinical course of these critically ill patients.

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.

Report of the 2022 Society of Thoracic Surgeons Congenital Heart Surgery Practice Survey.

BACKGROUND: The Society of Thoracic Surgeons Workforce on Congenital Surgery performed a practice survey to analyze contemporary data. METHODS: An electronic survey was sent to congenital heart surgeons in North America. Details on demographics, training paradigm, clinical practice, and work satisfaction were queried, tabulated, and analyzed. RESULTS: Of 312 unique contacts, 201 (64.4%) responded. Of these, 178 (89%) were practicing. The median age was 52 years (interquartile range, 43, 59 years), and 157 (88%) were male. The number of female respondents increased from 12 (7%) in 2015 to 18 (11%) at present. Practice composition was predominantly mixed pediatric and adult (141; 79%), although 15 (8%) surgeons practiced exclusively pediatric surgery. Most surgeons (154; 87%) reported performing the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category 4 and 5 procedures. One-fourth (42; 24%) reported performing fewer than 50 pediatric cases per year, and 18 (10%) stated that their primary role was as a surgical assistant. Individual surgeon case volume was most commonly 100 to 149 total cases (29%). Although one-half (91; 51%) reported their volume as being "just right," 74 (42%) reported that their case volume was "too small." Seventy-six (43%) reported too many surgeons in their region. Of the 201 practicing surgeons, 30 (14.9%) plan retirement in the next 5 years. Most described career satisfaction, with 102 (57%) being very satisfied and 48 (27%) somewhat satisfied. CONCLUSIONS: Although most congenital heart surgeons in North America are satisfied with their careers, more than 40% believe that their caseload is inadequate and that there are too many surgeons in their region. Further analysis is warranted regarding career dissatisfaction and diversity.

Health-Related Quality of Life After Single Ventricle Palliation or Tetralogy of Fallot Repair.

Single ventricle (SV) cardiac lesions and tetralogy of Fallot (TOF) are both common forms of cyanotic congenital heart disease. With advances in perioperative care and longitudinal follow-up, survival of these patients has dramatically improved and the majority survive to adulthood. This study compares health-related quality of life (HRQoL) of adult SV and TOF patients to each other and the general population. HRQoL of all surviving, non-transplanted SV and TOF patients 21 years of age and older at our institution was assessed with the SF-36 questionnaire via phone. Additional data including demographic parameters and information related to comorbidities and healthcare utilization were also analyzed. Among 81 eligible SV patients and 207 TOF patients, 33 (41%) and 75 (36%) completed the SF-36 phone survey, respectively. The mean age of SV patients was 32 vs. 38 years in the TOF group (p=0.01). SV patients reported more hepatic, pulmonary, and renal comorbidities. TOF patients were more likely to complete advanced degrees and more likely to have children (p=0.03). SV physical functioning scores were worse compared to TOF. In other domains of the SF-36 questionnaire, SV and TOF scores were similar. Compared to the general population, both groups reported worse bodily pain and mental health, but other aspects of psychosocial and general health were comparable. Overall HRQoL is good for both SV and TOF patients through early and mid-adulthood. Some QoL metrics were modestly worse in the SV patients. While these patients may have some physical limitations, psychosocial wellbeing appears preserved.

Norwood Operation: Immediate vs Delayed Sternal Closure.

BACKGROUND: The Norwood operation is a complex neonatal surgery. There are limited data to inform the timing of sternal closure. After the Norwood operation, delayed sternal closure (DSC) is frequent. We aimed to examine the association of DSC with outcomes, with a particular interest in how sternal closure at the time of surgery compared with the timing of DSC. Our outcomes included mortality, length of ventilation, length of stay, and postoperative complications. METHODS: This retrospective study included neonates who underwent a Norwood operation reported in the Pediatric Cardiac Critical Care Consortium registry from February 2019 through April 2021. Outcomes of patients with closed sternum were compared to those with sternal closure prior to postoperative day 3 (early closure) and prior to postoperative day 6 (intermediate closure). RESULTS: The incidence of DSC was 74% (500 of 674). The median duration of open sternum was 4 days (interquartile range 3-5 days). Comparing patients with closed sternum to patients with early sternal closure, there was no statistical difference in mortality rate (1.1% vs 0%) and the median hospital postoperative stay (30 days vs 31 days). Compared with closed sternum, patients with intermediate sternal closure required longer mechanical ventilation (5.9 days vs 3.9 days) and fewer subsequent sternotomies (3% vs 7.5%). CONCLUSIONS: For important outcomes following the Norwood operation there is no advantage to chest closure at the time of surgery if the chest can be closed prior to postoperative day 3.

Cardiac and Pulmonary Histopathology in Baboons Following Genetically-Engineered Pig Orthotopic Heart Transplantation.

BACKGROUND Although improving, survival after pig orthotopic heart transplantation (OHTx) in baboons has been mixed and largely poor. The causes for the high incidence of early failure remain uncertain. MATERIAL AND METHODS We have carried out pig OHTx in 4 baboons. Two died or were euthanized within hours, and 2 survived for 3 and 8 months, respectively. There was evidence of a significant 'cytokine storm' in the immediate post-OHTx period with the elevations in IL-6 correlating closely with the final outcome. RESULTS All 4 baboons demonstrated features suggestive of respiratory dysfunction, including increased airway resistance, hypoxia, and tachypnea. Histopathological observations of pulmonary infiltration by neutrophils and, notably, eosinophils within vessels and in the perivascular and peribronchiolar space, with minimal cardiac pathology, suggested a role for early lung acute inflammation. In one, features suggestive of transfusion-related acute lung injury were present. The 2 longer-term survivors died of (i) a cardiac dysrhythmia with cellular infiltration around the conducting tissue (at 3 months), and (ii) mixed cellular and antibody-mediated rejection (at 8 months). CONCLUSIONS These initial findings indicate a potential role of acute lung injury early after OHTx. If this response can be prevented, increased survival may result, providing an opportunity to evaluate the factors affecting long-term survival.

Program characteristics of cardiothoracic surgery departments versus divisions.

BACKGROUND: The organizational structure of cardiothoracic surgery practices varies among different programs throughout the United States (U.S.). We aimed to investigate the characteristics of the top ranked programs within the specialty and the surgeons practicing within each. METHODS: The top 50 hospitals for adult cardiology and heart surgery were identified using the US News and World Report 2019-20 ranking. There were 590 hospitals reported on, with 50 top rated programs. Data was collected from each hospital's website, analyses conducted using SAS 9.4 with statistical significance set at p ≤ 0.05. RESULTS: When comparing cardiothoracic surgery program organizational structures, 21 of the top 50 ranked programs were departments and 24 were divisions within their respective Department of Surgery. Mean number of surgeons was 11 with no statistical difference when analyzed by division versus department. Overall, 9% of practicing cardiothoracic surgeons were female. Between programs that are a department versus division, general thoracic surgery was included in 58% of divisions and 52% of departments (p = ns). Among programs that were departments, approximately 6% of surgeons had attained a Ph.D., while in divisions approximately 4% of surgeons had attained a Ph.D. CONCLUSIONS: The top 50 Cardiothoracic Surgery programs in the U.S. have approximately the same number of surgeons within the group and are organized similarly. This study group had a slightly higher percentage of female surgeons than has previously been noted in cardiothoracic surgery, with general thoracic surgery trending toward higher gender diversity. The presence of physician scientists was low, though similar amongst the study groups.

The World Society for Pediatric and Congenital Heart Surgery: 2021 Update of the World Database for Pediatric and Congenital Heart Surgery.

BACKGROUND: The World Database for Pediatric and Congenital Heart Surgery (WDPCHS), sponsored by the World Society for Pediatric and Congenital Heart Surgery (WSPCHS), provides complex programmatic outcomes analyses for all members of the WSPCHS. METHODS: The Data center, currently at Kirklin Institute for Research in Surgical Outcomes (KIRSO), University of Alabama, Birmingham (USA), provides biannual reports to all active members of the database. This report presents a descriptive analysis of these procedures submitted from January 1, 2017 to December 31, 2020. RESULTS: A total of 37,386 procedures were submitted with an overall mortality of 4.3%. The majority of submissions were from Asian countries. The majority of cases submitted from these countries were of Society of Thoracic Surgeons (STS)-European Association for Cardio-Thoracic Surgery (STAT) Mortality Categories I and II. CONCLUSIONS: The WSPCHS accomplished one of its missions in 2017 when the WDPCHS began accepting data from pediatric and congenital heart surgery programs across the globe. In doing so, it became one of the first organizations to create a platform for the exchange of knowledge and experience, regardless of the socioeconomic status of the particular program or country.

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.

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.

Early Versus Late Sternal Closure in Infants-Perioperative Associations and Outcomes.

BACKGROUND: Delayed sternal closure (DSC) has been used for patients who develop bleeding, chest wall edema, and malignant arrhythmia following cardiopulmonary bypass. Multiple factors can influence the timing of when to perform DSC. We aimed to describe our DSC experience in neonates and infants by comparing outcomes between patients undergoing early (<48 hours) versus late DSC (> 48 hours). We explored the associations between specific clinical and laboratory variables and the timing of DSC. METHODS: Retrospective chart review of neonates and infants (

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.

Examining racial and socioeconomic disparity in the pediatric heart transplant evaluation.

Racial disparities have been reported among pediatric patients waitlisted for and undergoing heart transplantation but have not been studied further upstream in the transplant candidate evaluation process. We retrospectively studied our single-center experience in order to investigate any potential biases in the evaluation process. Results of the heart transplant evaluation in children ≤18 years old at our institution were analyzed. Primary outcome was final disposition to waitlist or not. Race was defined by family self-identification. Descriptive and comparative statistical analyses were performed. From 2013 to 2019, 133 unique patients were referred for listing consideration. While Black patients comprised 44% of the referral population and had more markers of socioeconomic disadvantage, they comprised 43% of the patients who were listed for transplantation with no significant difference between these proportions (p = .96). Black and White patients made up a similar proportion of patients deemed too well or too ill for listing. Black patients had lower annual household income estimates and rates of household marriage. Despite identifying significant social challenges in 27 patients (18 of them Black), only five patients (3 Black and 2 White) were turned down for listing due to social barriers. While limited by the small number of patients turned down for social barriers, our transplant evaluation process does not appear to result in racial disparities in access to listing. Further studies are needed using national cohorts to explore possible racial disparities upstream from waitlisting and transplantation, such as during the referral and evaluation.