Outcomes in Primary Repair of Truncus Arteriosus with Significant Truncal Valve Insufficiency: A Systematic Review and Meta-analysis.

Data regarding the effect of significant TVI on outcomes after truncus arteriosus (TA) repair are limited. The aim of this meta-analysis was to summarize outcomes among patients aged ≤ 24 months undergoing TA repair with at least moderate TVI. A systematic literature search was conducted in PubMed, Scopus, and CINAHL Complete from database inception through June 1, 2022. Studies reporting outcomes of TA repair in patients with moderate or greater TVI were included. Studies reporting outcomes only for patients aged > 24 months were excluded. The primary outcome was overall mortality, and secondary outcomes included early mortality and truncal valve reoperation. Random-effects models were used to estimate pooled effects. Assessment for bias was performed using funnel plots and Egger's tests. Twenty-two single-center observational studies were included for analysis, representing 1,172 patients. Of these, 232 (19.8%) had moderate or greater TVI. Meta-analysis demonstrated a pooled overall mortality of 28.0% after TA repair among patients with significant TVI with a relative risk of 1.70 (95% CI [1.27-2.28], p < 0.001) compared to patients without TVI. Significant TVI was also significantly associated with an increased risk for early mortality (RR 2.04; 95% CI [1.36-3.06], p < 0.001) and truncal valve reoperation (RR 3.90; 95% CI [1.40-10.90], p = 0.010). Moderate or greater TVI before TA repair is associated with an increased risk for mortality and truncal valve reoperation. Management of TVI in patients remains a challenging clinical problem. Further investigation is needed to assess the risk of concomitant truncal valve surgery with TA repair in this population.

Factors Associated With Mortality and Adverse Outcomes After Truncus Arteriosus Repair.

BACKGROUND: Truncus arteriosus repair is associated with higher morbidity and mortality compared with many other congenital heart operations. We sought to determine factors associated with mortality and adverse outcomes in infants undergoing truncus arteriosus repair. METHODS: We used the Pediatric Health Information System Database to identify infants aged < 90 days who underwent truncus arteriosus repair from 2004 to 2019. The primary outcome was hospital mortality. Secondary outcomes were prolonged postoperative length of stay (>30 days) and hospital readmission within 90 days. Multivariable logistic regression models were used to identify associated factors for adverse outcomes. RESULTS: A total of 1645 subjects were included. Hospital mortality occurred in 164 (10%). Factors independently associated with mortality included birth weight < 3 kg, admit age < 48 hours, truncal valve surgery, cardiac arrest, extracorporeal membrane oxygenation, acute kidney injury, cardiac catheterization, tracheostomy, and earlier era. Prolonged postoperative length of stay occurred in 508 patients (31%). Factors independently associated with prolonged postoperative length of stay included prematurity, DiGeorge syndrome, admit age < 48 hours, later surgical era, acute kidney injury, infection, cardiac catheterization, vocal cord paralysis, tracheostomy, and gastrostomy. Readmission within 90 days occurred in 511 of 1481 surviving patients (34%). DiGeorge syndrome, cleft lip/palate, cardiac catheterization, and extracorporeal membrane oxygenation were factors independently associated with hospital readmission. CONCLUSIONS: We identified multiple factors associated with hospital mortality and adverse outcomes in infants undergoing truncus arteriosus repair. This information is useful for quality improvement initiatives, perioperative counseling, and discharge planning.

ABO-incompatible orthotopic heart transplant: a case report☠.

BACKGROUND: ABOi heart transplant has become routine for the majority of children <2 years old. An 8-month-old child with complex congenital heart disease presented to the Medical University of South Carolina Shawn Jenkins Children's Hospital in need of transplantation. METHODS: This case report describes the use of ABOi transplantation and describes the details of the total exchange transfusion prior to cardiopulmonary bypass. RESULTS: After a successful intraoperative total exchange transfusion following the ABOi protocol, the patient's isohemagglutinin titers were 1 VC on postoperative day (POD) 1, and isohemagglutinin titer was <1 VC on POD 14. The patient had no signs of rejection and continued to recover. CONCLUSIONS: Successful ABOi transplantation requires planning, an interdisciplinary approach, and clear closed-loop communication. Planning with the surgical and anesthesia teams is necessary for the hemodynamic stability of the patient during the total volume exchange as well as precautions put in place to ensure the blood products used in this procedure are correct. Planning with the lab and blood bank is also necessary to ensure they are prepared with enough blood products and can run isohemagglutinin titers.

Outcomes of Gastrostomy and Tracheostomy in Infants Undergoing Truncus Arteriosus Repair: Database Study Using the Pediatric Health Information System.

OBJECTIVES: We sought to determine the prevalence of and factors associated with gastrostomy tube placement and tracheostomy in infants undergoing truncus arteriosus repair, and associations between these procedures and outcome. DESIGN: Retrospective cohort study. SETTING: Pediatric Health Information System database. PATIENTS: Infants less than 90 days old who underwent truncus arteriosus repair from 2004 to 2019. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Multivariable logistic regression models were used to identify factors associated with gastrostomy tube and tracheostomy placement and to identify associations between these procedures and hospital mortality and prolonged postoperative length of stay (LOS; > 30 d). Of 1,645 subjects, gastrostomy tube was performed in 196 (11.9%) and tracheostomy in 56 (3.4%). Factors independently associated with gastrostomy tube placement were DiGeorge syndrome, congenital airway anomaly, admission age less than or equal to 2 days, vocal cord paralysis, cardiac catheterization, infection, and failure to thrive. Factors independently associated with tracheostomy congenital airway anomaly, truncal valve surgery, and cardiac catheterization. Gastrostomy tube was independently associated with prolonged postoperative LOS (odds ratio [OR], 12.10; 95% CI, 7.37-19.86). Hospital mortality occurred in 17 of 56 patients (30.4%) who underwent tracheostomy versus 147 of 1,589 patients (9.3%) who did not ( p < 0.001), and median postoperative LOS was 148 days in patients who underwent tracheostomy versus 18 days in those who did not ( p < 0.001). Tracheostomy was independently associated with mortality (OR, 3.11; 95% CI, 1.43-6.77) and prolonged postoperative LOS (OR, 9.85; 95% CI, 2.16-44.80). CONCLUSIONS: In infants undergoing truncus arteriosus repair, tracheostomy is associated with greater odds of mortality; while gastrostomy and tracheostomy are strongly associated with greater odds of prolonged postoperative LOS.

Association Between Birth Weight Z-Scores and Early Outcomes Following Truncus Arteriosus Repair.

In infants undergoing truncus arteriosus (TA) repair, we sought to determine associations between fetal growth restrictions as measured by birth weight Z-score and early outcomes. We utilized the Pediatric Health Information System (PHIS) database to identify infants < 90 days old who underwent TA repair from 2004 to 2019. The primary exposure variable was birth weight Z-score, calculated based on gestational age at birth, gender, and birth weight. The primary outcome was postoperative hospital mortality. Secondary outcomes included major complications, prolonged postoperative length of hospital stay (LOS; > 30 days), and hospital readmission within 1 year. Generalized estimating equation (GEE) models were used to identify adjusted associations between birth weight Z-score, small for gestational age (SGA) status, and mortality and included were 1039 subjects. Median birth weight was 2960 g, gestational age at birth was 38 weeks, and birth weight Z-score was - 0.47. SGA was present in 21% of subjects. Hospital mortality occurred in 104 patients (10%). By multivariable analysis, lower birth weight Z-score was associated with higher hospital mortality [for each unit decrease in birth weight Z-score below - 1.0, adjusted OR 1.71 (95% CI 1.10-4.25)]. SGA status was associated with increased hospital mortality (adjusted OR 2.17; 95% CI 1.39-3.40). Birth weight Z-scores and SGA status were not significantly associated with occurrence of cardiac arrest, ECMO use, gastrostomy tube placement, tracheostomy, seizures, infection, prolonged postoperative LOS, or hospital readmission. In infants undergoing TA repair, lower birth weight Z-scores and SGA status were strongly associated with increased hospital mortality.

Partial heart transplantation for pediatric heart valve dysfunction: A clinical trial protocol.

Congenital heart defects are the most common type of birth defects in humans and frequently involve heart valve dysfunction. The current treatment for unrepairable heart valves involves valve replacement with an implant, Ross pulmonary autotransplantation, or conventional orthotopic heart transplantation. Although these treatments are appropriate for older children and adults, they do not result in the same efficacy and durability in infants and young children for several reasons. Heart valve implants do not grow with the. Ross pulmonary autotransplants have a high mortality rate in neonates and are not feasible if the pulmonary valve is dysfunctional or absent. Furthermore, orthotopic heart transplants invariably fail from ventricular dysfunction over time. Therefore, the treatment of irreparable heart valves in infants and young children remains an unsolved problem. The objective of this single-arm, prospective study is to offer an alternative solution based on a new type of transplant, which we call "partial heart transplantation." Partial heart transplantation differs from conventional orthotopic heart transplantation because only the part of the heart containing the heart valve is transplanted. Similar to Ross pulmonary autotransplants and conventional orthotopic heart transplants, partial heart transplants contain live cells that should allow it to grow with the recipient child. Therefore, partial heart transplants will require immunosuppression. The risks from immunosuppression can be managed, as seen in conventional orthotopic heart transplant recipients. Stopping immunosuppression will simply turn the growing partial heart transplant into a non-growing homovital homograft. Once this homograft deteriorates, it can be replaced with a durable adult-sized mechanical implant. The protocol for our single-arm trial is described. The ClinicalTrials.gov trial registration number is NCT05372757.

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.

Effect of cardiac graft rejection on semilunar valve function: implications for heart valve transplantation.

BACKGROUND: The treatment of neonates with unrepairable heart valve dysfunction remains an unsolved problem because there are no growing heart valve replacements. Heart valve transplantation is a potential approach to deliver growing heart valve replacements. Therefore, we retrospectively analysed the semilunar valve function of orthotopic heart transplants during rejection episodes. METHODS: We included children who underwent orthotopic heart transplantation at our institution and experienced at least one episode of rejection between 1/1/2010 and 1/1/2020. Semilunar valve function was analysed using echocardiography at baseline, during rejection and approximately 3 months after rejection. RESULTS: Included were a total of 31 episodes of rejection. All patients had either no (27) or trivial (4) aortic insufficiency prior to rejection. One patient developed mild aortic insufficiency during a rejection episode (P = 0.73), and all patients had either no (21) or trivial (7) aortic insufficiency at follow-up (P = 0.40). All patients had mild or less pulmonary insufficiency prior to rejection, which did not significantly change during (P = 0.40) or following rejection (P = 0.35). Similarly, compared to maximum pressure gradients across the valves at baseline, which were trivial, there was no appreciable change in the gradient across the aortic valve during (P = 0.50) or following rejection (P = 0.42), nor was there any meaningful change in the gradient across the pulmonary valve during (P = 0.55) or following rejection (P = 0.91). CONCLUSIONS: This study demonstrated that there was no echocardiographic evidence of change in semilunar valve function during episodes of rejection in patient with heart transplants. These findings indicate that heart valve transplants require lower levels of immune suppression than orthotopic heart transplants and provide partial foundational evidence to justify future research that will determine whether heart valve transplantation may deliver growing heart valve replacements for children.

Differences in outcomes between surgical pericardial window and pericardiocentesis in children with postpericardiotomy syndrome.

Children with postpericardiotomy syndrome may develop hemodynamically significant pericardial effusions warranting drainage by surgical pericardial window or pericardiocentesis. The optimal approach is unknown. We performed a retrospective observational study at two pediatric cardiac centers. We included 42 children aged <18 years who developed postpericardiotomy syndrome following cardiac surgery between 2014 and 2021. Thirty-two patients underwent pericardial window and 10 underwent pericardiocentesis. Patients in the pericardial window group presented with postpericardiotomy syndrome sooner than those who underwent pericardiocentesis (median 7.5 days vs. 14.5 days, P = 0.03) and tended to undergo earlier intervention (median 8 days vs. 16 days, P = 0.16). No patient required subsequent drainage. There were no differences between groups in days of pericardial tube duration (median 4 days), complications, and subsequent days of intensive care or hospitalization. For children with postpericardiotomy syndrome with a pericardial effusion warranting drainage, these data suggest that pericardial window and pericardiocentesis have similar efficacy, safety, and resource utilization.

Corrections to "Risks and Benefits of Using a Commercially Available Ventricular Assist Device for Failing Fontan Cavopulmonary Support: A Modeling Investigation".

In [1], there are errors in Table I and equation (3) which are corrected here. This does not affect the study results. We sincerely apologize for the errors and any confusion they may have caused. \begin{equation*} \Delta {\mathrm {P}} = {\mathrm {A}}{\mathrm {Q}}^3 + \mathrm {B}{\mathrm {Q}}^2 + \text{CQ} + \mathrm {D} \tag{3} \end{equation*}.

A review of animal models for post-operative pericardial adhesions.

Post-operative pericardial adhesions remain a serious complication after cardiac surgery that can lead to increased morbidity and mortality. Fibrous adhesions can destroy tissue planes leading to injury of surrounding vasculature, lengthening of operation time, and increased healthcare costs. While animal models are necessary for studying the formation and prevention of post-operative pericardial adhesions, a standardized animal model for inducing post-operative pericardial adhesions has not yet been established. In order to address this barrier to progress, an analysis of the literature on animal models for post-operative pericardial adhesions was performed. The animal model, method used to induce adhesions, and the time to allow development of adhesions were analyzed. Our analysis found that introduction of autologous blood into the pericardial cavity in addition to physical abrasion of the epicardium caused more severe adhesion formation in comparison to abrasion alone or abrasion with desiccation (vs. abrasion alone p = 0.0002; vs. abrasion and desiccation p = 0.0184). The most common time frame allowed for adhesion formation was 2 weeks, with the shortest time being 10 days and the longest being 12 months. Finally, we found that the difference in adhesion severity in all animal species was similar, suggesting the major determinants for the choice of model are animal size, animal cost, and the availability of research tools in the particular model. This survey of the literature provides a rational guide for researchers to select the appropriate adhesion induction modality, animal model, and time allowed for the development of adhesions.

Immunosuppressive regimens in porcine transplantation models.

Pigs, or Sus scrofa domestica, are commonly used animal models in translational transplantation research due to their anatomical, physiological, and immunological similarities to humans. In solid organ transplantation studies, immunosuppressive medications may be administered to pigs to prevent rejection. We provide an overview of the immunosuppressive regimens used in allogeneic solid organ transplantation in pigs, including heart, lung, kidney, bowel and cotransplanted organs and focus on the use of tacrolimus, mycophenolate mofetil, and corticosteroids.

Diversity in the Adult and Pediatric Heart Transplant Surgeon Workforce between 2000 and 2020.

There is a paucity of literature evaluating trends in the demographic composition of the cardiothoracic surgery workforce. Using the United Network for Organ Sharing database, we retrospectively analyzed the changes in sex, race, and ethnicity of surgeons performing heart transplantations between 2000−2020. Surgeons performing heart transplantations for adult (≥18 years) and pediatric (<18 years) patients between 2000−2020 were identified and stratified by sex (male, female) and by race/ethnicity (non-Hispanic White, non-Hispanic Black, non-Hispanic Asian, Hispanic of any race). Between 2000−2020, the proportion of non-White and female cardiothoracic surgeons performing adult and pediatric heart transplantations increased. Nevertheless, there remains a lack of diversity in the workforce, particularly when compared to the general United States population.

Nine-Year Experience With the Arterial Switch Operation With Closed Coronary Transfer.

BACKGROUND: Coronary artery transfer is a critical step of the arterial switch operation (ASO) for transposition of the great arteries (TGA). Strategies for coronary transfer include open transfer before neoaortic anastomosis and closed transfer after neoaortic anastomosis. This study reports outcomes of ASO with closed coronary transfer at a single institution. METHODS: A retrospective analysis was performed of patients undergoing ASO for TGA from November 2006 to September 2015. Closed coronary transfer was universally employed. Patients were assigned to simple vs complex coronary anatomy groups. The primary outcome was overall survival. Secondary outcomes included reoperation-free survival, coronary reintervention, and aortic insufficiency. RESULTS: Ninety-six consecutive patients underwent ASO for TGA. Median follow-up was 5.8 years. Thirty-five (36%) patients had complex coronary anatomy, which was associated with significantly longer cardiopulmonary bypass and aortic cross-clamp time. Overall survival was 97.4%, and reoperation-free survival was 83.6%. There was no difference in survival or reoperation-free survival of patients with simple vs complex coronary anatomy. Hispanic ethnicity, side-by-side great arteries, and urgent or emergent operation were significantly associated with the composite outcome of reoperation or mortality. There were no coronary interventions after ASO, and the incidence of moderate or greater aortic insufficiency was 2.1% at hospital discharge and 1.5% in follow-up. CONCLUSIONS: Closed coronary transfer during ASO has excellent short-term and midterm results. Despite variable and often complex coronary anatomy, coronary ischemic events after ASO are avoidable. Closed coronary transfer has a low risk of aortic valve injury or insufficiency.

Ross Operation in Neonates: A Meta-analysis.

BACKGROUND: The Ross operation is the preferred treatment for aortic valve replacement in children. However previous studies indicate that outcomes in neonates are poor. This meta-analysis examines the pooled outcomes of the Ross operation in neonates. METHODS: Four major databases (PubMed/MEDLINE, EMBASE, Scopus, and ScienceDirect) were searched from inception until May 1, 2020 for studies describing outcomes of the Ross operation in neonates. The primary outcome was early mortality, and secondary outcomes were late mortality and mechanical support. Random-effects models were used to account for possible heterogeneity between studies, and continuity corrections were used to include zero total event trials. RESULTS: Eighteen studies comprising outcomes data on 181 neonates were included in the analysis. Meta-analysis showed a pooled early mortality rate of 24% (95% confidence interval, 12%-38%; I2 = 52%, P for heterogeneity = .01). Meta-regression analysis showed that more recently published studies reported significantly worse early mortality (P = .03). The pooled incidence of postoperative mechanical support was 15% (95% confidence interval, 5%-28%; I2 = 28%, P for heterogeneity = .22). No evidence of publication bias was found according to Egger's test (bias coefficient = 0.21, P = .57). CONCLUSIONS: The neonatal Ross operation carries a high early mortality rate. The treatment of unrepairable aortic valves in neonates remains an unsolved problem in congenital cardiac surgery.

Intraoperative Methylprednisolone and Neurodevelopmental Outcomes in Infants After Cardiac Surgery.

BACKGROUND: Neurodevelopmental impairment is an important consequence for survivors of surgery for critical congenital heart disease. This study sought to determine whether intraoperative methylprednisolone during neonatal cardiac surgery is associated with neurodevelopmental outcomes at 12 months of age and to identify early prognostic variables associated with neurodevelopmental outcomes. METHODS: We performed a planned secondary analysis of a 2-center, double-blind, randomized, placebo-controlled trial of intraoperative methylprednisolone in neonates undergoing cardiac surgery. A brain injury biomarker was measured during surgery. Bayley Scales of Infant and Toddler Development-III (BSID-III) were performed at 12 months of age. Two-sample t tests and generalized linear models were used. RESULTS: There were 129 participants (n = 61 methylprednisolone; n = 68 placebo). There were no significant differences in BSID-III scores and brain injury biomarker levels between treatment groups. Participants who underwent a palliative (versus corrective) procedure had lower mean BSID-III cognitive (101 ± 15 versus 106 ± 14; P = .03) and motor scores (85 ± 18 versus 94 ± 16; P < .01). Longer ventilation time was associated with lower motor scores. Longer cardiac intensive care unit stay was associated with lower cognitive, language, and motor scores. Cardiopulmonary bypass time, aortic cross-clamp time, and deep hypothermic circulatory arrest were not associated with BSID-III scores. CONCLUSIONS: Neurodevelopmental outcomes were not associated with intraoperative methylprednisolone or intraoperative variables. Participants who underwent a neonatal palliative (versus corrective) procedure had longer cardiac intensive care unit stays and worse neurodevelopmental outcomes at 1 year. This work suggests that interventions focused solely on the operative period may not be associated with a long-term neurodevelopmental benefit.

Adhesion barriers in cardiac surgery: A systematic review of efficacy.

BACKGROUND: Postoperative pericardial adhesions have been associated with increased morbidity, mortality, and surgical difficulty. Barriers exist to limit adhesion formation, yet little is known about their use in cardiac surgery. The study presented here provides the first major systematic review of adhesion barriers in cardiac surgery. METHODS: Scopus and PubMed were assessed on November 20, 2020. Inclusion criteria were clinical studies on human subjects, and exclusion criteria were studies not published in English and case reports. Risk of bias was evaluated with the Cochrane Risk of Bias Tool. Barrier efficacy data was assessed with Excel and GraphPad Prism 5. RESULTS: Twenty-five studies were identified with a total of 13 barriers and 2928 patients. Polytetrafluoroethylene (PTFE) was the most frequently evaluated barrier (13 studies, 67% of patients) with adhesion formation rate of 37.31% and standardized tenacity score of 26.50. Several barriers had improved efficacy. In particular, Cova CARD had a standardized tenacity score of 15.00. CONCLUSIONS: Overall, the data varied considerably in terms of study design and reporting bias. The amount of data was also limited for the non-PTFE studies. PTFE has historically been effective in preventing adhesions. More recent barriers may be superior, yet the current data is nonconfirmatory. No ideal adhesion barrier currently exists, and future barriers must focus on the requirements unique to operating in and around the heart.

Pentalogy of Cantrell With a Left Ventricular Diverticulum and Muscular VSD.

Pentalogy of Cantrell (POC) is a rare congenital disorder characterized by defects of the anterior diaphragm, midline supraumbilical abdominal wall, diaphragmatic pericardium, lower sternum, and heart. The low incidence and significant heterogeneity of POC make it difficult for most surgeons to gain consistent experience in treating these congenital heart defects. Here, we describe the treatment of a patient with POC, tetralogy of Fallot, left ventricular diverticulum, and a muscular ventricular septal defect. An innovative approach through a left ventricular diverticulotomy was utilized, which provided excellent exposure for repair of the muscular ventricular septal defect.