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.

Neonatal lymphatic flow disorders: impact of lymphatic imaging and interventions on outcomes.

Neonatal chylothorax (NCTx) and central lymphatic flow disorder (CLFD) are historically challenging neonatal disorders with high morbidity and mortality. METHODS: We conducted a retrospective study of 35 neonates with pulmonary lymphatic abnormalities at our institution who underwent lymphatic evaluation between December 2015 and September 2018. Patients with only pulmonary lymphatic perfusion syndrome were classified as NCTx and those with multiple flow abnormalities were classified as CLFD. Demographics, clinical characteristics, and outcomes were compared using t-tests/Wilcoxon rank sum tests and Fisher's exact tests. RESULTS: All 35 patients had intranodal MR lymphangiography and 14 (40%) also had conventional fluoroscopic lymphangiography. Fifteen (42.8%) patients were diagnosed with NCTx and 20 (57.1%) were diagnosed with CLFD. Thirty-four (97.1%) patients had pleural effusions. None of the NCTx group had ascites, anasarca, or dermal backflow compared to 17 (85%) (p < 0.001), 8 (42.1%) (p: 0.004), and 20 (100%) (p < 0.001) of the CLFD group, respectively. In the NCTx group, 11 (73.3%) had ethiodized oil embolization and 4 (26.7%) received conservative therapy. Ten (50%) of the CLFD patients had an intervention; of those, two (10%) had ethiodized oil-only embolization. Eight had non-ethiodized oil embolizations (two (25%) had embolization with glue, three (37.5%) underwent surgical lymphovenous anastomosis, two (25%) underwent thoracic duct (TD) externalization, and one (12.5%) had a non-TD lymphatic channel drain placed). Complete resolution of pleural effusions was achieved in all 15 NCTx patients, whereas 9 (45%) of 20 CLFD patients had resolution of chylothorax (p: 0.001). CONCLUSIONS: Establishing a diagnosis of NCTx or CLFD is paramount in selecting treatment options and providing prognostic information. Development of lymphatic interventions represents a paradigm shift in our understanding of neonatal lymphatic flow disorders and may be associated with improved survival.

Longitudinal Improvements in Radiation Exposure in Cardiac Catheterization for Congenital Heart Disease: A Prospective Multicenter C3PO-QI Study.

BACKGROUND: The C3PO-QI (Congenital Cardiac Catheterization Project on Outcomes - Quality Improvement), a multicenter registry launched in 2015, instituted quality improvement (QI) initiatives to reduce patient radiation exposure. Through regular collaboration, this initiative would allow for harmony among active participants, maximizing efforts and efficiency at achieving radiation best practices. This study sought to report these efforts with a detailed methodology for which institutions can target initiatives, reducing radiation exposure, and increasing patient safety. METHODS: Data were collected prospectively by 8 C3PO-QI institutions between January 1, 2015 and December 31, 2017. Radiation exposure was measured in dose area product per body weight (dose area product/kg; µGy*m2/kg) and reported by expected radiation exposure categories (REC) and institution for 40 published unique procedure types. Targeted interventions addressing selected strategic domains for radiation reduction were implemented in the pediatric catheterization labs of the C3PO-QI institutions. RESULTS: The study consisted of 15 257 unique cases. Median exposure (dose area product/kg) was decreased by 30% for all procedures. Dose area product/kg was reduced in all 3 REC, with the greatest improvement observed in REC I (REC I, -37%; REC II, -23%; REC III, -27%). Although the baseline radiation exposures and exact percent decrease varied across all C3PO-QI sites, each institution demonstrated improvements in radiation dose over time. These improvements occurred with the implementation of institution-specific QI interventions accelerated by participation in the C3PO-QI multicenter collaborative. CONCLUSIONS: Substantial radiation dose reductions can be achieved using targeted QI methodology and interventions. Participation in a multicenter QI collaborative may accelerate improvement across all centers due to enhanced engagement and shared learning between sites.

Operator-Directed Procedural Sedation in the Congenital Cardiac Catheterization Laboratory.

OBJECTIVES: The purpose of this study was to evaluate the association between the method of procedural sedation and outcomes for congenital cardiac catheterization procedures. BACKGROUND: The safety of operator-directed sedation (ODS) in the pediatric/congenital cardiac catheterization laboratory has been questioned. To our knowledge, the relative safety of ODS versus general anesthesia (GA) in these cases has not to date been critically evaluated. METHODS: A single-center retrospective cohort study was performed to compare the relative safety, cost, and times of catheterization procedures performed with ODS and those performed with GA from a cardiac anesthesiologist. The risk of adverse outcomes was compared using propensity-score-adjusted models. Using the same propensity score, procedure times and relative charges were also compared. RESULTS: Over the study period, 4,424 procedures in 2,547 patients were studied. Of these, 27% of cases were performed with ODS. ODS procedures were 70% diagnostic procedures, 17% device closure of patent ductus arteriosus, 5% balloon pulmonary valvuloplasty, and 3% pulmonary artery angioplasty. The risk of adverse event in adjusted models for ODS cases was significantly lower than in GA cases (odds ratio: 0.66; 95% confidence interval: 0.45 to 0.95; p = 0.03). Total room time and case time were also significantly shorter (p < 0.001). Professional (charge ratio: 0.88; p < 0.001) and hospital (charge ratio: 0.84; p < 0.001) charges for ODS cases were also lower than those for GA cases. CONCLUSIONS: This study demonstrates that clinical judgment can identify subjects in whom ODS is not associated with increased risk of adverse events. The use of ODS was associated with reduced case times and charges. In combination, these findings suggest that the selective use of ODS can allow for greater efficiency and higher value care without sacrificing safety.

Imaging of central lymphatic abnormalities in Noonan syndrome.

BACKGROUND: Children with Noonan syndrome are known to have increased risk for lymphatic disorders, the extent and nature of which are poorly understood. OBJECTIVE: Our objective was to describe the imaging findings of the central lymphatic abnormalities in children with Noonan syndrome who underwent central lymphatic imaging. MATERIALS AND METHODS: We conducted a single-center retrospective review of all children with a confirmed history of Noonan syndrome who presented for lymphatic imaging over a 5-year period. Imaging evaluation was performed on unenhanced T2-weighted (T2-W) imaging, dynamic-contrast MR lymphangiography or conventional lymphangiography. Two readers evaluated the imaging in consensus for the distribution of fluid on T2-W imaging and for lymphatic flow of intranodal contrast agent and thoracic duct abnormalities on dynamic-contrast MR lymphangiography and conventional lymphangiography. We performed a chart review for clinical history and outcomes. RESULTS: We identified a total of 10 children, all but one of whom had congenital heart disease. Presenting symptoms included chylothorax (n=9) and ascites (n=1). Nine had T2-W imaging, seven had dynamic-contrast MR lymphangiography, and seven had conventional lymphangiography. All with T2-W imaging had pleural effusions. On both dynamic-contrast MR lymphangiography and conventional lymphangiography, perfusion to the lung was seen (n=6), with intercostal flow also seen on dynamic-contrast MR lymphangiography (n=6). The thoracic duct was not present in three children and the central thoracic duct was not present in three. A double thoracic duct was seen in two children. CONCLUSION: Children with Noonan syndrome and clinical evidence of lymphatic dysfunction have central lymphatic abnormalities characterized by retrograde intercostal flow, pulmonary lymphatic perfusion, and thoracic duct abnormalities.

Radiation dose benchmarks in pediatric cardiac catheterization: A prospective multi-center C3PO-QI study.

OBJECTIVES: This study sought to update benchmark values to use a quality measure prospectively. BACKGROUND: Congenital Cardiac Catheterization Outcomes Project - Quality Improvement (C3PO-QI), a multi-center registry, defined initial radiation dose benchmarks retrospectively across common interventional procedures. These data facilitated a dose metric endorsed by the American College of Cardiology in 2014. METHODS: Data was collected prospectively by 9 C3PO-QI institutions with complete case capture between 1/1/2014 and 6/30/2015. Radiation was measured in total air kerma (mGy), dose area product (DAP) (µGy*M2 ), DAP per body weight, and fluoroscopy time (min), and reported by age group as median, 75th and 95th %ile for the following six interventional procedures: (1) atrial septal defect closure; (2) aortic valvuloplasty; (3) treatment of coarctation of the aorta; (4) patent ductus arteriosus closure; (5) pulmonary valvuloplasty; and (6) transcatheter pulmonary valve implantation. RESULTS: The study was comprised of 1,680 unique cases meeting inclusion criteria. Radiation doses were lowest for pulmonary valvuloplasty (age <1 yrs, median mGy: 59, DAP: 249) and highest in transcatheter pulmonary valve implantation (age >15 yrs, median mGy: 1835, DAP: 17990). DAP/kg standardized outcome measures across weights within an age group and procedure type significantly more than DAP alone. Radiation doses decreased for all procedures compared to those reported previously by both median and median weight-based percentile curves. These differences in radiation exposure were observed without changes in median fluoroscopy time. CONCLUSIONS: This study updates previously established benchmarks to reflect QI efforts over time. These thresholds can be applied for quality measurement and comparison. © 2017 Wiley Periodicals, Inc.

Implementation of Methodology for Quality Improvement in Pediatric Cardiac Catheterization: A Multi-center Initiative by the Congenital Cardiac Catheterization Project on Outcomes-Quality Improvement (C3PO-QI).

The Congenital Cardiac Catheterization Project on Outcomes (C3PO) launched in 2007 as a multi-center collaborative to establish standardized and comparable metrics for pediatric cardiac catheterization procedures. The limitations of larger registries at the time led to the development of the next phase in 2013, C3PO-Quality Improvement (C3PO-QI), focusing on instituting QI initiatives within the field. The objective of this manuscript is to provide a detailed overview of C3PO-QI and report data on case characteristics and outcome metrics being explored. C3PO-QI was designed to cultivate institutional collaboration during implementation of its initiatives. A database and website were developed to support data entry and on-demand reporting. The registry prospectively captures pediatric cardiac catheterization data among 15 hospitals. The present study includes case demographic data (n) and quality metric reporting by case type, age, and radiation dose variables. This dataset includes 13,135 cases entered into the database between 1/1/2014 and 12/31/2015. Interventional cases make up the highest percentage by case mix distribution (48 %), and patients <1 years make up the highest percentage by age distribution (26 %). The ratio of diagnostic and interventional procedures performed changes by age group. Application of QI metric shows all procedure types surpassing metric goals. Large volume data collection, such as in C3PO-QI, allows for meaningful interpretation of data. C3PO-QI is uniquely poised to deliver fast-paced changes in the field. Although the project initiatives are specific to pediatric cardiac catheterization, the implementation of the project and utilization of real-time reporting is generalizable to other specialties and multi-center collaboratives.

Percutaneous Lymphatic Embolization of Abnormal Pulmonary Lymphatic Flow as Treatment of Plastic Bronchitis in Patients With Congenital Heart Disease.

BACKGROUND: Plastic bronchitis is a potentially fatal disorder occurring in children with single-ventricle physiology, and other diseases, as well, such as asthma. In this study, we report findings of abnormal pulmonary lymphatic flow, demonstrated by MRI lymphatic imaging, in patients with plastic bronchitis and percutaneous lymphatic intervention as a treatment for these patients. METHODS AND RESULTS: This is a retrospective case series of 18 patients with surgically corrected congenital heart disease and plastic bronchitis who presented for lymphatic imaging and intervention. Lymphatic imaging included heavy T2-weighted MRI and dynamic contrast-enhanced magnetic resonance lymphangiogram. All patients underwent bilateral intranodal lymphangiogram, and most patients underwent percutaneous lymphatic intervention. In 16 of 18 patients, MRI or lymphangiogram or both demonstrated retrograde lymphatic flow from the thoracic duct toward lung parenchyma. Intranodal lymphangiogram and thoracic duct catheterization was successful in all patients. Seventeen of 18 patients underwent either lymphatic embolization procedures or thoracic duct stenting with covered stents to exclude retrograde flow into the lungs. One of the 2 patients who did not have retrograde lymphatic flow did not undergo a lymphatic interventional procedure. A total of 15 of 17(88%) patients who underwent an intervention had significant symptomatic improvement at a median follow-up of 315 days (range, 45-770 days). The most common complication observed was nonspecific transient abdominal pain and transient hypotension. CONCLUSIONS: In this study, we demonstrated abnormal pulmonary lymphatic perfusion in most patients with plastic bronchitis. Interruption of the lymphatic flow resulted in significant improvement of symptoms in these patients and, in some cases, at least temporary resolution of cast formation.

Radiation safety protocol using real-time dose reporting reduces patient exposure in pediatric electrophysiology procedures.

Radiation exposure during pediatric catheterization is significant. We sought to describe radiation exposure and the effectiveness of radiation safety protocols in reducing exposure during catheter ablations with electrophysiology studies in children and patients with congenital heart disease. We additionally sought to identify at-risk patients. We retrospectively reviewed all interventional electrophysiology procedures performed from April 2009 to September 2011 (6 months preceding intervention, 12 months following implementation of initial radiation safety protocol, and 8 months following implementation of modified protocol). The protocols consisted of low pulse rate fluoroscopy settings, operator notification of skin entrance dose every 1,000 mGy, adjusting cameras by >5 at every 1,000 mGy, and appropriate collimation. The cohort consisted of 291 patients (70 pre-intervention, 137 after initial protocol implementation, 84 after modified protocol implementation) at a median age of 14.9 years with congenital heart disease present in 11 %. Diagnoses included atrioventricular nodal reentrant tachycardia (25 %), atrioventricular reentrant tachycardia (61 %), atrial tachycardias (12 %), and ventricular tachycardia (2 %). There were no differences between groups based on patient, arrhythmia, and procedural characteristics. Following implementation of the protocols, there were significant reductions in all measures of radiation exposure: fluoroscopy time (17.8 %), dose area product (80.2 %), skin entry dose (81.0 %), and effective dose (76.9 %), p = 0.0001. Independent predictors of increased radiation exposure included larger patient weight, longer fluoroscopy time, and lack of radiation safety protocol. Implementation of a radiation safety protocol for pediatric and congenital catheter ablations can drastically reduce radiation exposure to patients without affecting procedural success.