Associations between Child Opportunity Index and Pediatric Cardiac Surgical Outcomes.

OBJECTIVE: To assess the relationship between the Child Opportunity Index (COI), a comprehensive measurement of social determinants of health, and specific COI domains on patient-specific outcomes following congenital cardiac surgery in the metropolitan region of Atlanta, Georgia. STUDY DESIGN: In this retrospective chart review, we included patients who underwent an index operation for congenital heart disease between 2010 and 2020 in a single pediatric health care system. Patients' addresses were geocoded and mapped to census tracts. Descriptive statistics, univariable analysis, and multivariable regression models were employed to assess associations between variables and outcomes. RESULTS: Of the 7460 index surgeries, 3798 (51%) met eligibility criteria. Presence of an adverse outcome, defined as either mortality or 1 of several other major postoperative morbidities, was significantly associated with COI in the univariable model (P = .008), but not the multivariable regression model (P = .39). Postoperative hospital length of stay was significantly associated with COI (P < .001) in univariable and multivariable regression models. There was no significant association between COI and readmission within 30 days of hospital discharge in univariable (P < .094) and multivariable (P = .49) models. CONCLUSION: COI is associated with postoperative hospital length of stay but not all outcomes in patients after congenital heart surgery. By understanding the role of COI in outcomes related to cardiac surgery, targeted interventions can be developed to improve health equity.

Neonatal heart transplantation in the United States: Trends and outcomes.

BACKGROUND: Heart transplantation in the neonatal period is associated with excellent survival. However, outcomes data are scant and have been obtained primarily from two single-center reports within the United States. We sought to analyze the outcomes of all neonatal heart transplants performed in the United States using the United Network for Organ Sharing (UNOS) dataset. METHODS: The UNOS dataset was queried for patients who underwent infant heart transplantation from 1987 to 2021. Patients were divided into two groups based on age - neonates (<=31 days), and older infants (32 days-365 days). Demographic and clinical characteristics were analyzed and compared, along with follow up survival data. RESULTS: Overall, 474 newborns have undergone heart transplantation in the United States since 1987. Freedom from death or re-transplantation for neonates was 63.5%, 58.8% and 51.6% at 5, 10, and 20 years, respectively. Patients in the newborn group had lower unadjusted survival compared to older infants (p < .001), but conditional 1-year survival was higher in neonates (p = .03). On multivariable analysis, there was no significant difference in survival between the two age groups (p = .43). Black race, congenital heart disease diagnosis, earlier surgical era, and preoperative mechanical circulatory support use were associated with lower survival among infant transplants (p < .05). CONCLUSIONS: Neonatal heart transplantation is associated with favorable long-term clinical outcomes. Neonates do not have a significant survival advantage over older infants. Widespread applicability is limited by the small number of available donors. Efforts to expand the donor pool to include non-standard donor populations ought to be considered.

A comparison of high-flow nasal cannula versus non-invasive positive pressure ventilation for respiratory support in infants following cardiac surgery.

BACKGROUND: Following cardiac surgery, infants often remain endotracheally intubated upon arrival to the cardiac ICU. High-flow nasal cannula and non-invasive positive pressure ventilation are used to support patients following extubation. There are limited data on the superiority of either mode to prevent extubation failure. METHODS: We conducted a single-centre retrospective study for infants (<1 year) and/or <10 kg who underwent cardiac surgery between 3/2019-3/2020. Data included patient and clinical characteristics and operative variables. The study aimed to compare high-flow nasal cannula versus non-invasive positive pressure ventilation following extubation and their association with extubation failure. Secondarily, we examined risk factors associated with extubation failure. RESULTS: There were 424 patients who met inclusion criteria, 320 (75%) were extubated to high-flow nasal cannula, 104 (25%) to non-invasive positive pressure ventilation, and 64 patients (15%) failed extubation. The high-flow nasal cannula group had lower rates of extubation failure (11%, versus 29%, p = 0.001). Infants failing extubation were younger and had higher STAT score (p < 0.05). Compared to high-flow nasal cannula, non-invasive positive pressure ventilation patients were at 3.30 times higher odds of failing extubation after adjusting for patient factors (p < 0.0001). CONCLUSIONS: Extubation failure after cardiac surgery occurs in smaller, younger infants, and those with higher risk surgical procedures. Patients extubated to non-invasive positive pressure ventilation had 3.30 higher odds to fail extubation than patients extubated to high-flow nasal cannula. The optimal mode of respiratory support in this patient population is unknown.

Milrinone for treatment of elevated lactate in the pre-operative newborn with hypoplastic left heart syndrome.

BACKGROUND: There is a paucity of information reported regarding the use of milrinone in patients with hypoplastic left heart syndrome prior to the Norwood procedure. At our institution, milrinone is initiated in the pre-operative setting when over-circulation and elevated serum lactate levels develop. We aimed to review the responses associated with the administration of milrinone in the pre-operative hypoplastic left heart syndrome patient. Second, we compared patients who received high- versus low-dose milrinone prior to Norwood procedure. METHODS: Single-centre retrospective study of patients diagnosed with hypoplastic left heart syndrome between January 2000 and December 2019 who underwent Norwood procedure. Patient characteristics and outcomes were compared. RESULTS: During the study period, 375 patients were identified; 79 (21%) received milrinone prior to the Norwood procedure with median lactate 2.55 mmol/l, and SpO2 93%. Patients who received milrinone were older at the time of Norwood procedure (6 vs. 5 days) and were more likely to be intubated and sedated. In a subset analysis stratifying patients to low- versus high-dose milrinone, median lactate decreased from time of initiation (2.39 vs 2.75 to 1.6 vs 1.8 mmol/l) at 12 hours post-initiation, respectively. Repeated measures analysis showed a significant decrease in lactate levels by 4 hours following initiation of milrinone, that persisted over time, with no significant difference in mean arterial pressure. CONCLUSIONS: The use of milrinone in the pre-operative over-circulated hypoplastic left heart syndrome patient is well tolerated, is associated with decreased lactate levels, and was not associated with significant hypotension or worsening of excess pulmonary blood flow.

Comparison of Patent Ductus Arteriosus Stent and Blalock-Taussig Shunt as Palliation for Neonates with Sole Source Ductal-Dependent Pulmonary Blood Flow: Results from the Congenital Catheterization Research Collaborative.

Patent ductus arteriosus (PDA) stenting is an accepted method for securing pulmonary blood flow in cyanotic neonates. In neonates with pulmonary atresia and single source ductal-dependent pulmonary blood flow (SSPBF), PDA stenting remains controversial. We sought to evaluate outcomes in neonates with SSPBF, comparing PDA stenting and surgical Blalock-Taussig shunt (BTS). Neonates with SSPBF who underwent PDA stenting or BTS at the four centers of the Congenital Catheterization Research Collaborative from January 2008 to December 2015 were retrospectively reviewed. Reintervention on the BTS or PDA stent prior to planned surgical repair served as the primary endpoint. Additional analyses of peri-procedural complications, interventions, and pulmonary artery growth were performed. A propensity score was utilized to adjust for differences in factors. Thirty-five patients with PDA stents and 156 patients with BTS were included. The cohorts had similar baseline characteristics, procedural complications, and mortality. Interstage reintervention rates were higher in the PDA stent cohort (48.6% vs. 15.4%, p < 0.001).

Feeding outcomes after paediatric cardiothoracic surgery: a retrospective review.

BACKGROUND: Feeding difficulty is a known complication of congenital heart surgery. Despite this, there is a relative sparsity in the available data regarding risk factors, incidence, associated symptoms, and outcomes. METHODS: In this retrospective chart review, patients aged 0-18 years who underwent congenital heart surgery at a single institution between January and December, 2017 were reviewed. Patients with feeding difficulties before surgery, multiple surgeries, and potentially abnormal recurrent laryngeal nerve anatomy were excluded. Data collected included patient demographics, feeding outcomes, post-operative symptoms, flexible nasolaryngoscopy findings, and rates of readmission within a 1-year follow-up period. Multivariable regression analyses were performed to evaluate the risk of an alternative feeding plan at discharge and length of stay. RESULTS: Three-hundred and twenty-six patients met the inclusion criteria for this study. Seventy-two (22.09%) were discharged with a feeding tube and 70 (97.22%) of this subgroup were younger than 12 months at the time of surgery. Variables that increased the risk of being discharged with a feeding tube included patient age, The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery score, procedure group, aspiration, and reflux. Speech-language pathology was the most frequently utilised consulting service for patients discharged with feeding tubes (90.28%) while other services were not frequently consulted. The median length of stay was increased from 4 to 10 days for patients who required an enteral feeding tube at discharge. DISCUSSION: Multidisciplinary management protocol and interventions should be developed and standardised to improve feeding outcomes following congenital heart surgery.

Registry-based trials: a potential model for cost savings?

BACKGROUND/AIMS: Registry-based trials have emerged as a potentially cost-saving study methodology. Early estimates of cost savings, however, conflated the benefits associated with registry utilisation and those associated with other aspects of pragmatic trial designs, which might not all be as broadly applicable. In this study, we sought to build a practical tool that investigators could use across disciplines to estimate the ranges of potential cost differences associated with implementing registry-based trials versus standard clinical trials. METHODS: We built simulation Markov models to compare unique costs associated with data acquisition, cleaning, and linkage under a registry-based trial design versus a standard clinical trial. We conducted one-way, two-way, and probabilistic sensitivity analyses, varying study characteristics over broad ranges, to determine thresholds at which investigators might optimally select each trial design. RESULTS: Registry-based trials were more cost effective than standard clinical trials 98.6% of the time. Data-related cost savings ranged from $4300 to $600,000 with variation in study characteristics. Cost differences were most reactive to the number of patients in a study, the number of data elements per patient available in a registry, and the speed with which research coordinators could manually abstract data. Registry incorporation resulted in cost savings when as few as 3768 independent data elements were available and when manual data abstraction took as little as 3.4 seconds per data field. CONCLUSIONS: Registries offer important resources for investigators. When available, their broad incorporation may help the scientific community reduce the costs of clinical investigation. We offer here a practical tool for investigators to assess potential costs savings.

The Evolution of an Adult Congenital Heart Surgery Program: The Emory System.

The Emory Adult Congenital Heart (Emory University, Atlanta, GA) program was founded in 2001. In 2004, the surgical component transitioned from a pediatric facility to an adult facility. The aim of this article is characterize the program as a whole, outline changes in the program, and discuss the challenges of the transition process. Between 2001 and 2015, changes in program structure and personnel were evaluated. There has been significant growth of the program between 2001 and 2015. There are currently 19 half-day clinics per week, with 2,700 clinic visits per year. There are six cardiologists, three congenital cardiac surgeons, two sonographers, one advanced practice provider, and one social worker dedicated to the program. There are Accreditation Council for Graduate Medical Education-accredited adult congenital cardiology and congenital cardiac surgery fellowships. One thousand forty-four operations were performed between 2001 and 2015. There were 828 open-heart operations, of which 581 (70%) were re-operations. Over the study period, the number of yearly operations increased from 30 to 119, and the mean age at surgery increased from 22 to 35 years. Over time, more of the operations were performed at the adult hospital: increasing from 3% in 2001 to 82% in 2015, and more of the operations were performed by congenital cardiac surgeons: 87% (114 of 131) before the 2004 transition to 97% (881 of 913) afterward. The Emory Adult Congenital Heart program has undergone significant growth and change, including transition of the surgical component from the pediatric to the adult facility. While numerous obstacles have been overcome and great progress has been made, additional challenges remain.

A 30-year experience with mixed-type total anomalous pulmonary venous connection: a word of caution.

BACKGROUND: Patients with total anomalous pulmonary venous connection can be problematic, particularly those with mixed-type pathology. We aimed to describe a cohort of patients with mixed-type anomalous drainage, highlighting the treatment challenges, and identifying risk factors for poor outcome. METHODS: We reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015. RESULTS: A total of 19 patients were identified. The median age and weight of patients at surgery were 18 days (with a range from 1 to 185) and 3.4 kg (with a range from 1.9 to 6.5), respectively. Venous anatomy included a combination of duplicate supracardiac (four), supracardiac and cardiac (11), and supracardiac and infracardiac (four) drainage. Out of 19 patients, six (32%) died within 30 days or the initial hospital stay; two additional patients died from progressive pulmonary vein stenosis at 72 and 201 days, respectively, resulting in 42% mortality within the 1st year. Follow-up data were available for 8/11 long-term survivors. The median follow-up period was 7.3 years (with a range from 1.8 to 15.7). Only one patient underwent re-intervention for recurrent pulmonary vein stenosis. For surgical mortality, no statistically significant risk factors were identified, although the risk trended to be higher (p⩽0.1) with lower age and weight, an infracardiac component, and prolonged cardiopulmonary bypass. For 1-year mortality, the risk became significant (p⩽0.05) with a lower weight (p=0.01), an infracardiac component (p=0.03), and prolonged cardiopulmonary bypass (p=0.04). CONCLUSION: The surgical and 1-year mortality in patients with mixed-type total anomalous pulmonary venous connection is high. On the other hand, among patients who survive past the 1st year, most have good outcomes without subsequent sequelae.

Pulmonary Artery Banding and Arch Repair vs Norwood for Unbalanced Atrioventricular Canal Defect.

BACKGROUND: The outcomes of single-ventricle palliation in unbalanced atrioventricular canal defect with coarctation of aorta (uAVC+CoA) have not been well studied. Systemic ventricle outflow tract obstruction has a propensity to develop in these patients after aortic arch repair with pulmonary artery banding (arch-PAB), which may adversely affect survival and Fontan candidacy. METHODS: A retrospective review was performed of patients who underwent single-ventricle palliation for uAVC+CoA from 2000 to 2022. Patients were divided into 2 groups based on initial palliation: (1) arch-PAB and (2) Norwood procedure. Demographic and clinical characteristics were analyzed and compared along with survival data. RESULTS: Stage 1 palliation for uAVC+CoA was performed in 41 patients. Arch-PAB was performed in 14 infants and Norwood in 27 infants. Arch-PAB patients had more chromosomal abnormalities (28.6 vs 7.4%, P < .009) and less severe systemic ventricle outflow tract obstruction on baseline echocardiogram (0.0 vs 70.4%, P < .001). Survival to stage 3 palliation was lower for the arch-PAB group (28.6% vs 66.6%, P = .02). Arch-PAB remained a significant risk factor for mortality (hazard ratio, 2.93; 95% CI, 1.05-8.53; P = .04) after adjusting for chromosomal abnormalities and atrioventricular valve regurgitation. After arch-PAB, systemic ventricle outflow tract obstruction was diagnosed in 13 of 14 patients. Echocardiography underestimated the degree of outflow tract obstruction in 10 of 13 arch-PAB patients. CONCLUSIONS: Arch-PAB has worse outcomes than Norwood for uAVC+CoA. Systemic ventricle outflow tract obstruction develops in almost all patients after arch-PAB. Outflow tract obstruction is underestimated by the echocardiogram and requires a high index of suspicion, along with advanced imaging, to ensure timely diagnosis and management.

Short-Term Outcomes, Functional Status, and Risk Factors for Requiring Extracorporeal Life Support After Norwood Operation: A Single-Center Retrospective Study.

Patients requiring extracorporeal life support (ECLS) post-Norwood operation constitute an extremely high-risk group. We retrospectively described short-term outcomes, functional status, and assessed risk factors for requiring ECLS post-Norwood operation between January 2010 and December 2020 in a high-volume center. During the study period, 269 patients underwent a Norwood procedure of which 65 (24%) required ECLS. Of the 65 patients, 27 (41.5%) survived to hospital discharge. Mean functional status scale (FSS) score at discharge increased from 6.0 on admission to 8.48 (p < 0.0001). This change was primary in feeding (p < 0.0001) and respiratory domains (p = 0.017). Seven survivors (26%) developed new morbidity, and two (7%) developed unfavorable functional outcomes. In the regression analysis, we showed that patients with moderate-severe univentricular dysfunction on pre-Norwood transthoracic echocardiogram (odds ratio [OR] = 6.97), modified Blalock Taussig Thomas (m-BTT) shunt as source of pulmonary blood flow (OR = 2.65), moderate-severe atrioventricular valve regurgitation on transesophageal echocardiogram (OR = 8.50), longer cardiopulmonary bypass time (OR = 1.16), longer circulatory arrest time (OR = 1.20), and delayed sternal closure (OR = 3.86), had higher odds of requiring ECLS (p < 0.05). Careful identification of these risk factors is imperative to improve the care of this high-risk cohort and improve overall outcomes.

Novel Dosing and Monitoring of Aspirin in Infants With Systemic-to-Pulmonary Artery Shunt Physiology: the SOPRANO Study.

OBJECTIVES: Provision of pulmonary blood flow with a systemic-to-pulmonary artery shunt is essential in some patients with cyanotic congenital heart disease. Traditionally, aspirin (ASA) has been used to prevent thrombosis. We evaluated ASA dosing with 2 separate antiplatelet monitoring tests for accuracy and reliability. METHODS: This is a retrospective, pre-post intervention single center study. Two cohorts were evaluated; the pre-intervention group used thromboelastography platelet mapping (TPM) and post-intervention used VerifyNow aspirin reactivity unit (ARU) monitoring. The primary endpoint was to compare therapeutic effect of TPM and ARU with regard to platelet inhibition. Inadequate platelet inhibition was defined as TPM <50% inhibition and ARU >550. RESULTS: Data from 49 patients were analyzed: 25 in the TPM group and 24 in the ARU group. Baseline characteristics were similar amongst the cohorts. The TPM group had significantly more patients with inadequate platelet inhibition (14 [56%] vs 2 [8%]; p = 0.0006) and required escalation with additional thromboprophylaxis (15 [60%] vs 5 [21%]). There was no difference in shunt thrombosis (1 [2%] vs 0 [0%]; p = 0.32), cyanosis requiring early re-intervention (9 [36%] vs 14 [58%]; p = 0.11), or bleeding (15 [60%] vs 14 [58%]; p = 0.66). CONCLUSION: With similar cohorts and the same ASA-dosing nomogram, ARU monitoring resulted in a reduced need for escalation of care and concomitant thromboprophylaxis with no difference in adverse outcomes. Our study suggests ARU monitoring compared with TPM may be a more reliable therapeutic platelet inhibition test for determining ASA sensitivity in children with congenital heart disease requiring systemic-to-pulmonary artery shunt.

Successful Medical-Surgical Management of Intracardiac Thrombosis and Pulmonary Pseudoaneurysms in an Adolescent With Hughes-Stovin Syndrome.

We present an adolescent male with a single intracardiac mass and pulmonary emboli, complicated by peripheral venous thrombosis and subsequent development of pulmonary pseudoaneurysms, leading to diagnosis of Hughes-Stovin syndrome. Remission was achieved with cyclophosphamide, corticosteroids, and pseudoaneurysm resection and maintained with infliximab and methotrexate.

External airway splint placement for severe pediatric tracheobronchomalacia.

OBJECTIVE: To present external airway splinting with bioabsorbable airway supportive devices (ASD) for severe, life-threatening cases of pediatric tracheomalacia (TM) or tracheobronchomalacia (TBM). METHODS: A retrospective cohort was performed for 5 pediatric patients with severe TM or TBM who underwent ASD placement. Devices were designed and 3D-printed from a bioabsorbable material, polycaprolactone (PCL). Pre-operative planning included 3-dimensional airway modeling of tracheal collapse and tracheal suture placement using nonlinear finite element (FE) methods. Pre-operative modeling revealed that triads along the ASD open edges and center were the most effective suture locations for optimizing airway patency. Pediatric cardiothoracic surgery and otolaryngology applied the ASDs by suspending the trachea to the ASD with synchronous bronchoscopy. Respiratory needs were trended for all cases. Data from pediatric patients with tracheostomy and diagnosis of TM or TBM, but without ASD, were included for discussion. RESULTS: Five patients (2 Females, 3 Males, ages 2-9 months at time of ASD) were included. Three patients were unable to wean from respiratory support after vascular ring division; all three weaned to room air post-ASD. Two patients received tracheostomies prior to ASD placement, but continued to experience apparent life-threatening events (ALTE) and required ventilation with supraphysiologic ventilator settings. One patient weaned respiratory support successfully after ASD placement. The last patient died post-ASD due to significant respiratory co-morbidity. CONCLUSION: ASD can significantly benefit patients with severe, unrelenting tracheomalacia or tracheobronchomalacia. Proper multidisciplinary case deliberation and selection are key to success with ASD. Pre-operative airway modeling allows proper suture placement to optimally address the underlying airway collapse.

Bioprosthetic Pulmonary Valve Dysfunction in Congenital Heart Disease.

BACKGROUND: We aim to identify the incidence and timing of dysfunction and failure of stented bioprosthetic valves in the pulmonary position in congenital heart disease patients. METHODS: A total of 482 congenital heart disease patients underwent 484 stented bioprosthetic pulmonary valve implantations between 2008 and 2018. There were 164 porcine valves (Porcine) and 320 bovine pericardial valves (Pericardial) implanted. Primary endpoints were survival, valve dysfunction, and valve failure. RESULTS: Pericardial valves were implanted in older patients (22.0, interquartile range [IQR] 14-33 vs 16.0, IQR 11-23 years, P < 0.001). Five-year survival (96.7% vs 97.9%) for the Pericardial and Porcine groups, respectively, were similar, P > 0.05. Forty-six (34%) Porcine and 75 (27%) Pericardial group patients met criteria for valve dysfunction at a median echocardiographic follow-up time of 7.43 years (IQR 4.1-9.5 years) and 3.26 years (IQR 1.7-4.7 years), respectively. More Pericardial group patients suffered from at least mild late PR while late median peak gradient was higher in the Porcine group, P < .001 for both. Risk factors for valve dysfunction included decreasing patient age for the entire cohort (hazard ratio [HR] 1.02, 95% confidence interval [CI] 1.00-1.04, P = .015) and lack of anticoagulation at discharge for the Porcine group (HR 3.06, 95% CI 1.03-9.10, P = .044) but not the Pericardial group. Five-year cumulative incidence of dysfunction was 39% for the Pericardial group and 17% for the Porcine group. CONCLUSIONS: Porcine stented and bovine pericardial stented valves can be implanted in the pulmonary position in all age groups safely. However, despite similar rates of valve failure, bovine pericardial stented valves have a higher incidence of valve dysfunction at mid-term follow-up.

Age at surgery and outcomes following neonatal cardiac surgery: An analysis from the Pediatric Cardiac Critical Care Consortium.

OBJECTIVE: The optimal timing for neonatal cardiac surgery is a potentially modifiable factor that may affect outcomes. We studied the relationship between age at surgery (AAS) and outcomes across multiple hospitals, focusing on neonatal operations where timing appears is not emergency. METHODS: We studied neonates ≥37 weeks' gestation and ≥2.5 kg admitted to a treating hospital on or before day of life 2 undergoing selected index cardiac operations. The impact of AAS on outcomes was evaluated across the entire cohort and a standard risk subgroup (ie, free of preoperative mechanical ventilation, mechanical circulatory support, or other organ failure). Outcomes included mortality, major morbidity (ie, cardiac arrest, mechanical circulatory support, unplanned cardiac reintervention, or neurologic complication), and postoperative cardiac intensive care unit and hospital length of stay. Post hoc analyses focused on operations undertaken between day of life 2 and 7. RESULTS: We studied 2536 neonates from 47 hospitals. AAS from day of life 2 through 7 was not associated with risk adjusted mortality or major morbidity among the entire cohort and the standard risk subgroup. Older AAS, although associated with modest increases in postoperative cardiac intensive care unit and hospital length of stay in the entire cohort, was not associated with hospital length of stay in the standard risk subgroup. CONCLUSIONS: Among select nonemergency neonatal cardiac operations, AAS between day of life 2 and 7 was not found to be associated with risk adjusted mortality or major morbidity. Although delays in surgical timing may modestly increase preoperative resource use, studies of AAS and outcomes not evident at the time of discharge are needed.

The impact of prematurity and associated comorbidities on clinical outcomes in neonates with congenital heart disease.

Prematurity is a common risk factor in children, affecting approximately 10% of live births, globally. It is more common in children with critical congenital heart disease (CCHD) and carries important implications in this group of patients. While outcomes have been improving over the years, even late preterm birth is associated with worse outcomes in children born with critical congenital heart disease compared to those without. Infants with both prematurity and CCHD are at particularly high risk for important comorbidities, including: necrotizing enterocolitis, intraventricular hemorrhage, white matter injury, neurodevelopmental anomalies and retinopathy of prematurity. Lesion-specific intensive care management of these infants, interventional and peri-operative management specifically tailored to their needs, and multidisciplinary care all have the potential to improve outcomes in this challenging group.

Cardiac surgery in children with trisomy 13 or trisomy 18: How safe is it?

Objective: Surgery for heart defects in children with trisomy 13 or 18 is controversial. We analyzed our 20-year experience. Methods: Since 2002, we performed 21 operations in 19 children with trisomy 13 (n = 8) or trisomy 18 (n = 11). Age at operation was 4 days to 12 years (median, 154 days). Principal diagnosis was ventricular septal defect in 10 patients, tetralogy of Fallot in 7 patients, arch hypoplasia in 1 patient, and patent ductus arteriosus in 1 patient. Results: The initial operation was ventricular septal defect closure in 9 patients, tetralogy of Fallot repair in 7 patients, pulmonary artery banding in 1 patient, patent ductus arteriosus ligation in 1 patient, and aortic arch/coarctation repair in 1 patient. There were no operative or hospital deaths. Median postoperative intensive care and hospital stays were 189 hours (interquartile range, 70-548) and 14 days (interquartile range, 8.0-37.0), respectively, compared with median hospital stays in our center for ventricular septal defect repair of 4.0 days and tetralogy of Fallot repair of 5.0 days. On median follow-up of 17.4 months (interquartile range, 6.0-68), 1 patient was lost to follow-up after 5 months. Two patients had reoperation without mortality. There have been 5 late deaths (4 with trisomy 18, 1 with trisomy 13) predominately due to respiratory failure from 4 months to 9.4 years postoperatively. Five-year survival was 66.6% compared with 24% in a group of unoperated patients with trisomy 13 or 18. Conclusions: Cardiac operation with an emphasis on complete repair can be performed safely in carefully selected children with trisomy 13 or trisomy 18. Hospital resource use measured by postoperative intensive care and hospital stays is considerably greater compared with nontrisomy 13 and 18.

The utility of handheld ultrasound as a point-of-care screening tool to assess vocal fold impairment following congenital heart surgery.

INTRODUCTION: Vocal fold motion impairment (VFMI) is a known potential complication of congenital heart surgery (CHS). Flexible nasolaryngoscopy (FNL) is the gold standard for evaluation of vocal fold movement but has risks, including epistaxis, desaturation, and changes in heart rate. Laryngeal ultrasound (LUS) has begun to emerge as a diagnostic tool and has been shown to have high accuracy in the evaluation of VFMI. We sought to assess the utility of hand-held LUS as a point-of-care screening tool to assess VFMI in pediatric patients following congenital heart surgery. METHODS: Using a prospective cohort design, children under 18 years who were undergoing congenital heart surgery at a tertiary care pediatric hospital were enrolled. All patients underwent postoperative LUS and FNL. All studies were reviewed by two otolaryngology reviewers blinded to the clinical diagnosis. Higher quality studies were reviewed by two cardiology reviewers also blinded to the clinical diagnosis. Accuracy and inter-rater reliability were calculated. RESULTS: Sixty-two children were screened. Fourteen children with VFMI were identified via FNL. When comparing LUS and FNL, both individual accuracy (90.3% and 75.8%) and interrater agreement (79% overall, 96% for high quality videos) were high for the otolaryngology reviewers. The cardiology reviewers were able to obtain 100% accuracy for high quality videos. CONCLUSION: Handheld LUS has utility as a point-of-care screening tool to assess VFMI. This may have benefit in low-resource settings, for universal screening in cardiac intensive care units, or in settings where otolaryngology consultation may be difficult to obtain.

The Society of Thoracic Surgeons Congenital Heart Surgery Database: 2021 Update on Outcomes and Research.

The Society of Thoracic Surgeons Congenital Heart Surgery Database is a comprehensive clinical outcomes registry that captures almost all pediatric cardiac surgical operations in the United States. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and improvement of quality in this subspecialty. This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery performed between July 1, 2016, and June 30, 2020. The reported data on aggregate national outcomes are exemplified by an analysis of 10 prespecified benchmark operation groups performed. This report further reviews related activities in the areas of data collection and analysis, quality measurement, performance improvement, and research.