Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for centers performing pediatric heart surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Risk Stratification for Congenital Heart Surgery for ICD-10 Administrative Data (RACHS-2).

BACKGROUND: As the cardiac community strives to improve outcomes, accurate methods of risk stratification are imperative. Since adoption of International Classification of Disease-10th Revision (ICD-10) in 2015, there is no published method for congenital heart surgery risk stratification for administrative data. OBJECTIVES: This study sought to develop an empirically derived, publicly available Risk Stratification for Congenital Heart Surgery (RACHS-2) tool for ICD-10 administrative data. METHODS: The RACHS-2 stratification system was iteratively and empirically refined in a training dataset of Pediatric Health Information Systems claims to optimize sensitivity and specificity compared with corresponding locally held Society of Thoracic Surgeons-Congenital Heart Surgery (STS-CHS) clinical registry data. The tool was validated in a second administrative data source: New York State Medicaid claims. Logistic regression was used to compare the ability of RACHS-2 in administrative data to predict operative mortality vs STAT Mortality Categories in registry data. RESULTS: The RACHS-2 system captured 99.6% of total congenital heart surgery registry cases, with 1.0% false positives. RACHS-2 predicted operative mortality in both training and validation administrative datasets similarly to STAT Mortality Categories in registry data. C-statistics for models for operative mortality in training and validation administrative datasets-adjusted for RACHS-2-were 0.76 and 0.84 (95% CI: 0.72-0.80 and 0.80-0.89); C-statistics for models for operative mortality-adjusted for STAT Mortality Categories-in corresponding clinical registry data were 0.75 and 0.84 (95% CI: 0.71-0.79 and 0.79-0.89). CONCLUSIONS: RACHS-2 is a risk stratification system for pediatric cardiac surgery for ICD-10 administrative data, validated in 2 administrative-registry-linked datasets. Statistical code is publicly available upon request.

Widening our Focus: Characterizing Socioeconomic and Racial Disparities in Congenital Heart Disease.

BACKGROUND: Socioeconomic and racial (SER) disparities among patients with congenital heart disease (CHD) may limit access to high-quality care. We characterized the national SER landscape and its relationship to early outcomes and identified interactions among determinants mitigating adverse outcome. METHODS: The Pediatric Health Information System (PHIS) database was queried for patients (age <26 years) with CHD between 2016 and 2018. International Classification of Diseases, 10th Revision, codes were mapped to diagnostic categories for complexity adjustment. Correlational and hierarchical regression analyses identified risk factors and characterized interactions. RESULTS: We identified 166,599 unique admissions from 52 hospitals, with 58,395 having interventions. Median age was 0 years (interquartile range [IQR], 4 years). Race/ethnicity was predominantly White (59%), Hispanic (20%), and Black (16%). Median neighborhood household income (NHI) was $41,082 and varied among hospitals. Patient NHI had a parabolic relationship with mortality, with both higher and lower values having increased risk. Black patients had significantly higher death, and this relationship was potentiated by lower NHI and complexity. Hospital length of stay was longer among Black neonates (median, 51 days; IQR, 93 days) compared with neonates of other ethnic groups (median, 32 days; IQR, 71 days; P < .0001. Care pathways, including permanent feeding tubes, were also more prevalent among Black neonates (17.8%) compared with White neonates (15%; P = .02). CONCLUSIONS: Interactions among SER disparities modify CHD outcomes. Specific hospitals have more SER fragile patients but may have developed care pathways that prolong length of stay to mitigate risk among Black neonates. Adverse outcomes among SER-disadvantaged patients are magnified in complex CHD, suggesting tangible benefits to targeted resource allocation and population health initiatives.

Slide Tracheoplasty for Repair of Complex Tracheoesophageal Fistulas.

OBJECTIVES/HYPOTHESIS: Repair of large, recurrent, and complex tracheoesophageal fistulas (TEFs) is challenging and numerous different surgical approaches exist. These various techniques each carry a set of risks and possible complications such as fistula recurrence, tracheal stenosis or pouches, esophageal stenosis, and recurrent laryngeal nerve injury. Slide tracheoplasty is a reconstructive technique successfully used in many different airway pathologies, including TEF repair. This study examines the success, limits, and complications related to slide tracheoplasty for repair of complex TEFs. STUDY DESIGN: Retrospective chart review. METHODS: Patients undergoing TEF repair using a cervical or thoracic approach slide tracheoplasty, at a single institution, between July 2008 and December 2019 were retrospectively reviewed. Demographic data, comorbidities, TEF etiology and surgical history, slide tracheoplasty details and outcomes, and postoperative complication data were examined using descriptive statistics. RESULTS: Twenty-six patients underwent 27 slide tracheoplasties for TEF (20 cervical approaches, 7 thoracic approaches) with a mean age of 5.2 years (IQR 0.7-7.6) at time of surgery. The most common TEF etiologies included congenital (n = 13), tracheostomy tube erosion (n = 5), and button battery ingestion (n = 4). Fistulas ranged in size from <0.5 mm to 4 cm and 59% had previous endoscopic or open repairs. There were two TEF recurrences (7.4%), one of which was successfully revised and the other which was treated with stent placement. Postoperative complications included dehiscence (3.7%), unilateral vocal fold paralysis (3.7%), and mild tracheal stenosis (18.5%). CONCLUSIONS: Slide tracheoplasty is an effective surgical technique for treating complex congenital and acquired TEFs with lower rates of complications when compared to other techniques. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:1542-1547, 2022.

A mapping algorithm for International Classification of Diseases 10th Revision codes for congenital heart surgery benchmark procedures.

BACKGROUND: Administrative billing data are critical to many initiatives in congenital heart surgery. Mapping algorithms for International Classification of Disease, 10th Revision diagnosis and procedure codes to clinical registry procedure definitions will allow identification of surgical cases and account for patient and procedural factors within administrative data. Our objectives were to develop mapping logic to crosswalk International Classification of Disease, 10th Revision procedure codes to 10 Society of Thoracic Surgeons Congenital Heart Surgery Database benchmark and beta-test the algorithm. METHODS: Patients undergoing Society of Thoracic Surgeons Congenital Heart Surgery Database benchmark procedures from 2015 to 2019 were identified and served as the gold standard. Cases were linked on direct identifiers to cases from the Pediatric Health Information System Database. Two independent teams developed International Classification of Disease, 10th Revision-based algorithms for cases capture. Algorithms were compared and iteratively refined to optimize sensitivity and specificity. Operative mortalities for cases identified in the administrative versus registry data were compared. RESULTS: Overall sensitivity was 91% and specificity was 99% for capture of benchmark operations using International Classification of Diseases 10th Revision codes. Sensitivity was more than 90% in identifying 6 of the 10 individual benchmark procedures and more than 98% sensitive in identifying Fontan, Glenn, and arterial switch with ventricular septal defect procedures. Specificity was more than 98% for all benchmark operations. There were no statistical differences in operative mortality between cases identified in the administrative versus the registry data. CONCLUSIONS: Novel mapping algorithm for International Classification of Disease, 10th Revision procedure codes enables identification of congenital heart benchmark procedures within administrative billing data. This crosswalk facilitates population-based congenital heart surgical research and quality assessment.

Risk of Pediatric Cardiac Surgery Increased in Patients Undergoing Tracheal Surgery During the Same Hospitalization.

BACKGROUND: The impact of complex tracheal surgery (TS) on outcomes of children undergoing cardiac surgery has rarely been investigated in large national databases and could impact anticipated outcomes in those patients. METHODS: The Pediatric Health Information System database was reviewed from January 1, 2005 to December 31, 2014, for pediatric (<18 years) patients undergoing select cardiac surgical procedures using International Classification of Disease-9 procedural coding. Patients were divided based upon having TS (excluding tracheostomy) during the same hospitalization. Patients with tracheal and cardiac surgeries (CHS + TS) were propensity matched in a 1 : 2 fashion to patients undergoing isolated cardiac surgeries without TS (CHS) based upon cardiac procedure(s), age at surgery, gender, and discharge year. Hospital mortality and other outcomes were compared between the 2 matched groups. RESULTS: The 283 CHS + TS patients were similar to the 566 CHS patients in gender, race, age, cardiac procedures, and presence of chromosomal abnormalities, all P > .05. Hospital mortality was 13.8% (n = 39) for CHS + TS and 5.8% (n = 33) for CHS patients with an unadjusted 2.58 hazard ratio of death (95% confidence interval: 1.59-4.20; P < .001). CHS + TS was also associated with a greater length of stay (63 days vs 12 days), a higher cost per hospitalization ($322 402 vs $80 273), and more readmissions (64.3% [n = 182] vs 41.8% [n = 243]), all P < .001. CONCLUSIONS: Patients undergoing cardiac and TS in the same hospitalization are at greater risk of in-hospital mortality than patients undergoing similar cardiac surgeries alone and incur higher resource utilization thereafter. The increased risk of mortality is currently underappreciated, but it is important to recognize when discussing expectations with families and providers.

The use of mechanical assist devices in the pediatric population.

The last two decades have witnessed an expansion in the devices, support strategies, and outcomes for pediatric patients who require mechanical circulatory support. The use of large registries that house data on these devices and the development of shared learning networks have provided clinicians with the ability to critically assess outcomes for emerging and existing technology. The purpose of this review is to provide the reader with perspective on the most contemporary devices utilized for pediatric mechanical circulatory support. It will examine existing support strategies and the most contemporary outcomes regarding these devices including those in high risk patients.

Tracheal surgery for airway anomalies associated with increased mortality in pediatric patients undergoing heart surgery: Society of Thoracic Surgeons Database analysis.

OBJECTIVES: Airway anomalies are common in children with cardiac disease but with an unquantified impact on outcomes. We sought to define the association between airway anomalies and tracheal surgery with cardiac surgery outcomes using the Society of Thoracic Surgery Congenital Heart Surgery Database. METHODS: Index cardiac operations in children aged less than 18 years (January 2010 to September 2018) were identified from the Society of Thoracic Surgery Congenital Heart Surgery Database. Patients were divided on the basis of reported diagnosis of an airway anomaly and subdivided on the basis of tracheal lesion and tracheal surgery. Multivariable analysis evaluated associations between airway disease and outcomes controlling for covariates from the Society of Thoracic Surgery Congenital Heart Surgery Database Mortality Risk Model. RESULTS: Of 198,674 index cardiovascular operations, 6861 (3.4%) were performed in patients with airway anomalies, including 428 patients (0.2%) who also underwent tracheal operations during the same hospitalization. Patients with airway anomalies underwent more complex cardiac operations (45% vs 36% Society of Thoracic Surgeons/European Association for Cardiothoracic Surgery Congenital Heart Surgery Mortality category ≥3 procedures) and had a higher prevalence of preoperative risk factors (73% vs 39%; both P < .001). In multivariable analysis, patients with airway anomalies had increased odds of major morbidity and tracheostomy (P < .001). Operative mortality was also increased in patients with airway anomalies, except those with malacia. Tracheal surgery within the same hospitalization increased the odds of operative mortality (adjusted odds ratio, 3.9; P < .0001), major morbidity (adjusted odds ratio, 3.7; P < .0001), and tracheostomy (adjusted odds ratio, 16.7; P < .0001). CONCLUSIONS: Patients undergoing cardiac surgery and tracheal surgery are at significantly higher risk of morbidity and mortality than patients receiving cardiac surgery alone. Most of those with unoperated airway anomalies have higher morbidity and mortality, which makes it an important preoperative consideration.

Heart-Lung Transplant via an Eighth-Time Sternotomy.

We report a combined heart-lung transplantation following seven prior sternotomies in a patient born with a transitional atrioventricular septal defect. Previous surgeries to repair and replace the mitral valve led to pulmonary vein stenosis and pulmonary vascular disease. Eighth-time sternotomy and significant vascular adhesions led to a prolonged operation and to placing the heart-lung block anterior to the phrenic nerves. Despite this, the patient was ready for discharge after two weeks and continues to do well over nine months later. As more patients survive multiple cardiac palliations with some developing pulmonary vascular disease, heart-lung transplantation may become relevant again.

Higher Flow on Cardiopulmonary Bypass in Pediatrics Is Associated With a Lower Incidence of Acute Kidney Injury.

Adequate perfusion is of paramount concern during cardiopulmonary bypass (CPB) and different methodologies are employed to optimize oxygen delivery. Temperature, hematocrit, and cardiac index (CI) are all modulated during CPB to ensure appropriate support. This study examines 2 different perfusion strategies and their impact on various outcome measures including acute kidney injury (AKI), urine output on CPB, ICU length of stay, time to extubation, and mortality. Predicated upon surgeon preference, the study institution employs 2 different perfusion strategies (PS) during congenital cardiac surgery requiring CPB. One method utilizes a targeted 2.4 L/min/m2 CI and nadir hematocrit of 28% (PS1), the other a 3.0 L/min/m2 CI with a nadir hematocrit of 25% (PS2). This study retrospectively examines CPB cases during which the 2 perfusion strategies were applied to determine potential differences in packed red blood cell administration, urine output during CPB, AKI post-CPB as defined by the KDIGO criteria, and operative survival as defined by the Society of Thoracic Surgeons. Significant differences were found in urine output while on CPB (P < 0.01) and all combined stages of postoperative AKI (P = 0.01) with the PS2 group faring better in both measures. No significant difference was found between the 2 groups for packed red blood cell administration, mortality, time to extubation, or ICU length of stay. Avoiding a nadir hematocrit less than 25% has been well established but maintaining anything greater than that may not be necessary to achieve adequate oxygen delivery on CPB. Our results indicate that higher CI and oxygen delivery on CPB are associated with a lower rate of AKI and this may be achieved with increased flow rather than increasing the hematocrit thus avoiding unnecessary transfusion.

Adult Congenital Heart Disease: Current Early Expectations After Cardiac Transplantation.

BACKGROUND: Early mortality has plagued the otherwise good outcomes of heart transplantation in patients with adult congenital heart disease (ACHD), but perioperative care is improving. This study sought to identify risk factors for 1-year mortality currently and examine the results of patients without those risk factors compared with patients who did not have ACHD (nACHD). METHODS: The United Network of Organ Sharing database was searched for all adult (>17 years of age) heart transplant recipients from 2000 to 2018. They were divided into an early era and a late era. A multivariate analysis identified risk factors for 1-year mortality in the late era. Patients without these risk factors were compared with the nACHD group by Kaplan-Meier analysis. RESULTS: A total of 495 patients with ACHD were identified from 2000 to 2008, and 666 were identified from 2009 to 2018. The recent era had better 1-year survival (P <.001) and overall survival (P = .003) than did the era from 2000 to 2008. Patients with ACHD were different from the nACHD population in age (37 years vs 57 years), body mass index greater than 25 kg/m2 (45% vs 66%), incidence of renal dysfunction (23% vs 28%) and liver dysfunction (29% vs 23%), sensitization (38% vs 29%), and ischemic times (3.5 hours vs 3.1 hours). Multivariate analysis identified body mass index greater than 25 kg/m2 (hazard ratio [HR], 1.79), renal dysfunction (HR, 1.85), liver dysfunction (HR, 1.69), and longer ischemic time (HR, 1.46) as risk factors for early mortality. Patients with only 1 of the first 3 categorical risk factors had 1-year survival comparable to that reported in patients with nACHD. CONCLUSIONS: Patients with ACHD had better early and long-term outcomes in the recent era. When only 1 of 3 pretransplant risk factors for early mortality was present, patients had survival equal to that of nACHD patients and perhaps better long-term survival.