Aortic Arch Reconstruction Using Nonvalved Femoral Vein Homograft in High-Risk Neonates.

Aortic arch obstruction is often present with complex concomitant congenital heart defects (CHDs). The use of nonvalved femoral vein homograft (FVH) to reconstruct the aortic arch has distinct surgical advantages, including simplified reconstruction. We present an intraoperative video of a Yasui procedure utilizing FVH for aortic reconstruction in a 12-day-old (2.2 kg) neonate with right ventricular outflow tract obstruction, malalignment ventricular septal defect, aortic valve atresia, aortic arch hypoplasia, atrial septal defect, and ductal dependent systemic circulation. Further, we report outcomes for a series of three additional neonatal patients with complex CHD and aortic arch obstruction who underwent FVH arch reconstruction.

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.

Isolated aortic arch anomalies are associated with defect severity and outcome in patients with congenital diaphragmatic hernia.

PURPOSE: Congenital diaphragmatic hernia (CDH) patients often have suspected isolated aortic arch anomalies (IAAA) on imaging. The purpose of this work was to describe the incidence and outcomes of CDH + IAAA patients. METHODS: Cardiovascular data were collected for infants from the CDH Study Group born between 2007 and 2019. IAAA were defined as coarctation of aorta, hypoplastic aortic arch, interrupted aortic arch, and aortic aneurysmal disease on early, postnatal echocardiography. Patients with major cardiac malformations and/or chromosomal abnormalities were excluded. Primary outcomes included the rate of aortic intervention, rates of extracorporeal life support (ECLS) utilization, and mortality. RESULTS: Of 6357 CDH infants, 432 (7%) were diagnosed with a thoracic aortic anomaly. Of these, 165 were diagnosed with IAAA, most commonly coarctation of the aorta (n = 106; 64%) or hypoplastic aortic arch (n = 58; 35%). CDH + IAAA patients had lower birthweights (3 kg vs. 2.9 kg) and Apgar scores (7 vs. 6) than patients without IAAA (both χ2 p < 0.001). CDH + IAAA were less likely to undergo diaphragm repair (72 vs. 87%, p < 0.001), and overall mortality was higher for CDH + IAAA infants (58 vs. 24%, p < 0.001). When controlling for defect size, birth weight, and Apgar, IAAA were significantly associated with mortality (OR 3.3, 95% CI 2.2-5.0; p < 0.01) but not associated with ECLS (OR 0.98, 95% CI 0.65-1.50; p = 0.90). Only 17% (n = 28) of CDH + IAAA patients underwent aortic intervention. CONCLUSIONS: IAAA in CDH are associated with increased mortality. This often simply reflects severity of the defect and thoracic anatomic derangement, as opposed to unique aortic pathology, given few CDH + IAAA patients undergo aortic intervention.

Impact on clinical outcomes from transcatheter closure of the Fontan fenestration: A systematic review and meta-analysis.

Background: Meta-analysis of the impact on clinical outcome from transcatheter closure of Fontan fenestration. Methods: Cochrane, Embase, MEDLINE, and Open-Gray were searched. Parameters such as changes in oxygen saturation, cavo-pulmonary pressure, maximum heart rate during exercise, exercise duration, and oxygen saturation after fenestration closure were pooled and statistical analysis performed. Results: Among 922 publications, 12 retrospective observational studies were included. The included studies involved 610 patients, of which 552 patients (90.5%) had a fenestration. Of those patients, 505 patients (91.5%) underwent attempt at trans-catheter closure. When it could be estimated, the pooled overall mean age at trans-catheter fenestration closure was 6.6 ± 7.4 years, and the mean follow-up time was 34.4 ± 10.7 months. There were 32 minor (6.3%) and 20 major (4.0%) complications during or after trans-catheter Fontan fenestration closure. The forest plots demonstrate that following fenestration closure, there was a significant increase in the mean arterial oxygen saturation of 7.9% (95% CI 6.4-9.4%, p < 0.01). There was also a significant increase in the mean cavo-pulmonary pressure of 1.4 mmHg (95% CI 1.0-1.8 mmHg, p < 0.01) following fenestration closure. The exercise parameters reported in 3 studies also favored closing the fenestration as well, yet the exercise duration increase of 1.7 min (95% CI 0.7-2.8 min, p < 0.01) after fenestration closure is probably clinically insignificant. Conclusion: Late closure of a Fontan fenestration has the impact of improving resting oxygen saturation, exercise oxygen saturation, and a modest improvement of exercise duration. These clinical benefits, however, may be at the expense of tolerating slightly higher cavo-pulmonary mean pressures.

Virtual Simulated Implantation of an Adult-Sized Left Ventricular Assist Device in a Pediatric Patient.

There is very limited experience with simulated virtual implantation of left ventricular assist devices (LVADs) to assess device fitness in pediatric patients. In this clinical vignette, we report the case of a 9-year-old male patient with dilated cardiomyopathy who underwent successful placement of an LVAD after virtual simulated implantation was performed. (Level of Difficulty: Advanced.).

Aortic Valve Neocuspidization in a Pulmonary Autograft.

Management of aortic valve (AV) disease in the pediatric population is challenged by a lack of attractive valve replacement options. The Ross procedure has been prominent for these patients; however, long-term reports have revealed concerns, namely neoaortic root dilation and regurgitation. More recently, AV neocuspidization has emerged as an alternative to the Ross operation or other valve replacement procedures in pediatric patients with congenital AV disease. We report a successful case of AV neocuspidization of a previously implanted pulmonary autograft.

Modern advances regarding interatrial communication in congenital heart defects.

BACKGROUND: The interatrial communication, one of the most frequent congenital heart defects, represents an important intracardiac shunt between systemic and pulmonary circulations. Direction and magnitude of the interatrial shunting depends upon several features, including defect size, shape and location, pressure difference between right and left atrium, and difference in right and left ventricular compliance. METHODS: In this review article, the presence or absence of interatrial communication, and its role, have been analyzed, as they can have a critical impact on the cardiovascular physiopathology, and the interatrial communication can prove to be either clinically harmful, useful or indispensable. Accordingly, the utility and role of the interatrial communication in modern congenital, pediatric and adult, disease has evolved, with modification of the indications to close, maintain patency, or create an interatrial communication. RESULTS: The interatrial communication and shunting can be manipulated to maximize the oxygen delivery to the tissues, accordingly with the underlying congenital heart defect. While not always relevant to patients with bi-ventricular circulations, this becomes extremely important in children and adults with complex congenital heart defects. CONCLUSIONS: With improving long-term survival for the vast majority of congenital heart patients, an advanced understanding of the role and utility of the interatrial communication, and of all the possibilities of its manipulation, is essential to improve the patient outcomes.

A narrative review of modern approach and outcomes evaluation in congenital heart defects.

OBJECTIVE: The purpose of this review is to highlight critical advances and innovative approaches to the most challenging clinical situations in congenital heart surgery, to establish a new perspective from which to evaluate current clinical practice patterns and contemporary United States program ranking systems. BACKGROUND: The past decades have witnessed substantial advances in the treatment of congenital heart defects. New strategies are deeply rooted in calculated risk-taking innovations. Pioneer surgeons developed, improved and refined critical operative skills and techniques to optimize cardiovascular physiology, decrease operative mortality and improve clinical outcomes. METHODS: Unfortunately, in the modern surgical era, supportive environments to allow surgeons to make similar gains and innovative contributions remain scarce. In the current practice, overall procedure volume is prioritized to safeguard quality metrics, including hospital survival and length of stay, surgical complications, and neurocognitive outcomes. As a result, exceptional surgical results have become translated and defined by public ranking systems such as the US News and World Report Best Children's Hospital National Ranking (USNWR) and the Congenital Heart Surgery Database of the Society for Thoracic Surgeons (CHSD-STS), primary based upon early post-operative mortality. This reality places surgeons in a vulnerable position where pressure to achieve a high clinical ranking contrasts with a surgeon definition of "acceptable" surgical risk. Currently, the most frequently used risk stratification tools do not factor in important differences in strategies, such as staged palliation versus complete repair, or bi-ventricular versus uni-ventricular physiology. This favors hospitals pursuing multistage surgical approaches, even if the result is worse long-term morbidity, mortality and increased resource utilization. This economy of ranking-based decision-making causes surgeons either avoid operating altogether or accept less advantageous multi-staged treatment strategies for patients with elevated expected mortality. Such an environment also might present much farther-reaching negative impacts on the growth and development of junior surgeons and trainees, as well as on the pursuit of new surgical innovations to aid future generations of patients. CONCLUSIONS: Risk aversive surgical behavior is creating an environment not favorable for the children born with truly complex congenital heart defects. KEYWORDS: Biventricular conversion; congenital heart surgery; multidisciplinary approach; risk-stratification; surgical outcomes.

Cardiac transplantation in adult congenital heart disease with prior sternotomy.

BACKGROUND: Adult congenital heart disease (ACHD) patients who require orthotopic heart transplantation are surgically complex due to anatomical abnormalities and multiple prior surgeries. In this study, we investigated these patients' outcomes using our institutional database. METHODS: ACHD patients who had prior intracardiac repair and subsequent heart transplant were included (2008-2018). Adult patients without ACHD were extracted as a control. A comparison of patients with functional single ventricular (SV) and biventricular (BV) hearts was performed. RESULTS: There were 9 SV and 24 BV patients. The SV group had higher central venous pressure/pulmonary capillary wedge pressure (P = .028), hemoglobin concentration (P = .010), alkaline phosphatase (P = .022), and were more likely to have liver congestion (P = .006). Major complications included infection in 16 (48.5%), temporary dialysis in 12 (36.4%), and graft dysfunction requiring perioperative mechanical support in 7 (21.2%). Overall in-hospital mortality was 15.2%. Kaplan-Meier analysis showed a higher, but not statistically significant, survival after 10 years between the ACHD and control groups (ACHD 84.9% vs. control 67.5%, P = .429). There was no significant difference in 10-year survival between SV and BV groups (78% vs. 88%, P = .467). CONCLUSIONS: Complex ACHD cardiac transplant recipients have a high incidence of early morbidities after transplantation. However, long-term outcomes were acceptable.

Neonatal Cone Valvuloplasty for Pulmonary Atresia/Intact Ventricular Septum and Ebsteinoid Valve.

Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare lesion with wide anatomic variability. A rare variant exists, marked by right-sided dilatation, an Ebsteinoid tricuspid valve, and severe tricuspid regurgitation. Neonatal cone valvuloplasty allows for a biventricular circulation and avoids complications of prosthetic valves; however, this technique inevitably requires reintervention to exchange the right ventricle-to-pulmonary artery conduit as the patient grows. We present a successful complete repair cone tricuspid valvuloplasty in a 9-day-old (2.8 kg) neonate with trisomy 21, pulmonary atresia with intact ventricular septum, and an Ebsteinoid tricuspid valve.

Weight impacts 1-year congenital heart surgery outcomes independent of race/ethnicity and payer.

Body mass index, race/ethnicity, and payer status are associated with operative mortality in congenital heart disease (CHD). Interactions between these predictors and impacts on longer term outcomes are less well understood. We studied the effect of body mass index, race/ethnicity, and payer on 1-year outcomes following elective CHD surgery and tested the degree to which race/ethnicity and payer explained the effects of body mass index. Patients aged 2-25 years who underwent elective CHD surgery at our centre from 2010 to 2017 were included. We assessed 1-year unplanned cardiac re-admissions, re-interventions, and mortality. Step-wise, multivariable logistic regression was performed.Of the 929 patients, 10.4% were underweight, 14.9% overweight, and 8.5% obese. Non-white race/ethnicity comprised 40.4% and public insurance 29.8%. Only 0.5% died prior to hospital discharge with one additional death in the first post-operative year. Amongst patients with continuous follow-up, unplanned re-admission and re-intervention rates were 14.7% and 12.3%, respectively. In multivariable analyses adjusting for surgical complexity and surgeon, obese, overweight, and underweight patients had higher odds of re-admission than normal-weight patients (OR 1.40, p = 0.026; OR 1.77, p < 0.001; OR 1.44, p = 0.008). Underweight patients had more than twice the odds of re-intervention compared with normal weight (OR 2.12, p < 0.001). These associations persisted after adjusting for race/ethnicity, payer, and surgeon.Pre-operative obese, overweight, and underweight body mass index were associated with unplanned re-admission and/or re-intervention 1-year following elective CHD surgery, even after accounting for race/ethnicity and payer status. Body mass index may be an important modifiable risk factor prior to CHD surgery.

Trisomy 21 Patients Undergoing Cavopulmonary Connections Need Improved Preoperative and Postoperative Care.

BACKGROUND: Patients with trisomy 21 (T21) often have elevated pulmonary vascular resistance, which may result in a suboptimal cavopulmonary connection (CPC) after a Glenn or Fontan operation. The purpose of this study was to assess, in a nationwide, multiinstitution cohort of patients with CPC, the impact of T21 on patient morbidity, mortality, and resource use. METHODS: A total of 23,271 pediatric patients with CPC (2004 to 2019) at 50 US hospitals were evaluated using the Pediatric Health Information System database. Univariable and multivariable regression analyses were used to assess risk-adjusted associations between Down syndrome and other risk factors and postoperative measures of morbidity, mortality, lengths of stay, and cost of hospitalization. RESULTS: The overall prevalence of T21 among patients who had undergone Glenn and Fontan procedures was 1.5% (199 of 13,268) and 0.8% (78 of 1003), respectively. Among both CPC cohorts, T21 status significantly increased unadjusted mortality, hospital lengths of stay, and total costs of hospitalization compared with the non-T21 CPC cohort (all P < .001). Patients with T21 also had a higher incidence of prolonged mechanical ventilation compared with patients without T21 in both Glenn and Fontan groups (P < .001). Multivariable regression analysis further estimated that patients with T21 are associated with a 5.5-fold increase in mortality (P < .001) compared with patients without T21. Finally, patients with T21 had increased long-term mortality compared with their peers. CONCLUSIONS: T21 significantly increases risk-adjusted morbidity, inpatient mortality, long-term mortality, and resource use after cavopulmonary connections. Further investigation is needed to clarify modifiable patient-level and center-specific risk factors to improve outcomes for patients with T21.

Neonates With Complex Cardiac Malformation and Congenital Diaphragmatic Hernia Born to SARS-CoV-2 Positive Women-A Single Center Experience.

BACKGROUND: Our understanding of the impact of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) on pregnancies and perinatal outcomes is limited. The clinical course of neonates born to women who acquired coronavirus disease 2019 (COVID-19) during their pregnancy has been previously described. However, the course of neonates born with complex congenital malformations during the COVID-19 pandemic is not known. METHODS: We report a case series of seven neonates with congenital heart and lung malformations born to women who tested positive for SARS-CoV-2 during their pregnancy at a single academic medical center in New York City. RESULTS: Six infants had congenital heart disease and one was diagnosed with congenital diaphragmatic hernia. In all seven infants, the clinical course was as expected for the congenital lesion. None of the seven exhibited symptoms generally associated with COVID-19. None of the infants in our case series tested positive by nasopharyngeal test for SARS-CoV-2 at 24 hours of life and at multiple points during their hospital course. CONCLUSIONS: In this case series, maternal infection with SARS-CoV-2 during pregnancy did not result in adverse outcomes in neonates with complex heart or lung malformations. Neither vertical nor horizontal transmission of SARS-CoV-2 was noted.

The Use of Hemostatic Blood Products in Children Following Cardiopulmonary Bypass and Associated Outcomes.

To describe the use of hemostatic transfusions in children following cardiac surgery with cardiopulmonary bypass and the association of hemostatic transfusions postoperatively with clinical outcomes. DESIGN: A retrospective cohort study. SETTING: PICU of a tertiary care center from 2011 to 2017. PATIENTS: Children 0-18 years old undergoing cardiac surgery with cardiopulmonary bypass. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Four-hundred twenty children underwent cardiac surgery with cardiopulmonary bypass. The median (interquartile range) age was 0.8 years (0.3-5 yr) and 243 (58%) were male. The majority of cases were classified as Risk Adjustment for Congenital Heart Surgery 2 (223, 54%) or Risk Adjustment for Congenital Heart Surgery 3 (124, 30%). Twenty-four percent of children (102/420) received at least one hemostatic transfusion with the most common first product being platelet transfusions (47/102), followed by plasma (44/102), and cryoprecipitate (11/102). The children who received hemostatic transfusions were younger (p = 0.006), had lower body weights (p = 0.004), less likely to be their initial operation with cardiopulmonary bypass (p = 0.003), underwent more complex surgeries (p = 0.001) with longer bypass runs (p < 0.001), and had more use of hypothermic circulatory arrest (p = 0.014). The receipt of hemostatic blood products postoperatively was independently associated with more days of mechanical ventilation (p < 0.001) and longer PICU lengths of stay (p = 0.001) but not with time receiving vasoactive mediations (p = 0.113) or nosocomial infections (p = 0.299). CONCLUSIONS: Nearly one-quarter of children undergoing cardiac repair with cardiopulmonary bypass receive hemostatic transfusions postoperatively. These blood products are independently associated with worse clinical outcomes. Larger studies should be performed to determine the hemostatic efficacy of these products, as well as to clarify associated morbidities, in order to inform proper blood management.

Identification of Risk Factors for Early Fontan Failure.

Despite improvements in operative and perioperative care, the risk of significant morbidity and mortality for children undergoing Fontan procedures persists. Previous investigations have identified peri-Fontan characteristics that may predict early adverse events. The purpose of this study was to identify characteristics from throughout a patient's lifespan, including all perioperative stages, that might predict early Fontan failure-defined as death, Fontan takedown, or listing for cardiac transplantation before hospital discharge or within 30 postoperative days. A single-center retrospective study of all patients undergoing a Fontan procedure was performed. Patient and intervention-related characteristics were examined from birth through Fontan. Data were described using standard summary statistics. Univariable, logistic regression was used to examine associations with early Fontan failure. In total, 191 patients met inclusion criteria. The incidence of early Fontan failure was 4% (n = 8: 6 deaths, 2 Fontan takedowns). Neonatal balloon atrial septostomy was the only patient characteristic significantly associated with Fontan failure. Patients who underwent balloon septostomy had 8.5 times higher odds of Fontan failure (confidence interval (CI) 2.6-28.1, P < 0.001) than those who did not. Children who require balloon septostomy as neonates remain at higher risk of Fontan takedown, listing for heart transplantation, or death in the early post-Fontan period.