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.

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.

Adjuvant chemoradiation for high-grade cardiac leiomyosarcoma in a child: Case report and review of literature.

A 13-year-old healthy girl presented with dizziness and palpitations, found to have a left atrial mass. An 8-cm tumor was removed en bloc. Pathology confirmed grade 3 leiomyosarcoma with multifocal positive margins. She received adjuvant ifosfamide and doxorubicin, followed by concurrent proton radiotherapy and ifosfamide. Radiotherapy included 66 Gy (RBE) in 33 fractions to the operative bed. Prospectively graded toxicities included Grade 2 esophagitis and Grade 1 anorexia, dermatitis, and fatigue. She completed six cycles of ifosfamide. Two years post operation, she had no evidence of disease, intermittent palpitations with normal cardiac function, and no other cardiopulmonary or esophageal symptoms.

Impact of Major Residual Lesions on Outcomes After Surgery for Congenital Heart Disease.

BACKGROUND: Many factors affect outcomes after congenital cardiac surgery. OBJECTIVES: The RLS (Residual Lesion Score) study explored the impact of severity of residual lesions on post-operative outcomes across operations of varying complexity. METHODS: In a prospective, multicenter, observational study, 17 sites enrolled 1,149 infants undergoing 5 common operations: tetralogy of Fallot repair (n = 250), complete atrioventricular septal defect repair (n = 249), arterial switch operation (n = 251), coarctation or interrupted arch with ventricular septal defect (VSD) repair (n = 150), and Norwood operation (n = 249). The RLS was assigned based on post-operative echocardiography and clinical events: RLS 1 (trivial or no residual lesions), RLS 2 (minor residual lesions), or RLS 3 (reintervention for or major residual lesions before discharge). The primary outcome was days alive and out of hospital within 30 post-operative days (60 for Norwood). Secondary outcomes assessed post-operative course, including major medical events and days in hospital. RESULTS: RLS 3 (vs. RLS 1) was an independent risk factor for fewer days alive and out of hospital (p ≤ 0.008) and longer post-operative hospital stay (p ≤ 0.02) for all 5 operations, and for all secondary outcomes after coarctation or interrupted arch with VSD repair and Norwood (p ≤ 0.03). Outcomes for RLS 1 versus 2 did not differ consistently. RLS alone explained 5% (tetralogy of Fallot repair) to 20% (Norwood) of variation in the primary outcome. CONCLUSIONS: Adjusting for pre-operative factors, residual lesions after congenital cardiac surgery impacted in-hospital outcomes across operative complexity with greatest impact following complex operations. Minor residual lesions had minimal impact. These findings may provide guidance for surgeons when considering short-term risks and benefits of returning to bypass to repair residual lesions.

Clinical outcomes of children receiving ABO-incompatible versus ABO-compatible heart transplantation: a multicentre cohort study.

BACKGROUND: ABO-incompatible heart transplantation increases donor availability in young children and is evolving into standard of care in children younger than 2 years. Previous smaller studies suggest similar outcomes to ABO-compatible heart transplantation, but persisting alterations of the immune system in ABO-incompatible recipients might increase the risk of some infections or benefit the graft owing to reduced HLA reactivity. We aimed to assess long-term outcomes in young children after they received ABO-incompatible or ABO-compatible heart transplantation. METHODS: In this multicentre, prospective cohort study, we analysed data from the Pediatric Heart Transplant Society registry to compare children who received ABO-incompatible or ABO-compatible heart transplantation before age 2 years between Jan 1, 1999, and June 30, 2018. Given significantly different clinical demographics between the two groups, we also matched each ABO-incompatible recipient to two ABO-compatible recipients using propensity score matching. We assessed patient and graft survival, coronary allograft vasculopathy, malignancy, acute rejection (any episode resulting in augmentation of immunosuppression), and infections (requiring intravenous antibiotic or antiviral therapy or life-threatening infections treated with oral therapy). FINDINGS: We included 2206 children who received a heart transplant before age 2 years, with 11 332·6 patient-years of cumulative observation time. Children who received an ABO-incompatible transplant (n=364) were younger and a larger proportion had congenital heart disease and ventilator and mechanical circulatory support than the ABO-compatible recipients (n=1842). After matching, only differences in blood group (more O in ABO-incompatible and more AB in ABO-compatible groups) and use of polyclonal induction therapy with anti-thymocyte globulins persisted. The two matched groups had similar post-transplantation graft survival (p=0·74), freedom from coronary allograft vasculopathy (p=0·75), and malignancy (p=0·51). ABO-incompatible recipients showed longer freedom from rejection (p=0·0021) in the overall cohort, but not after matching (p=0·48). Severe infections (p=0·0007), bacterial infections (p=0·0005), and infections with polysaccharide encapsulated bacteria (p=0·0005) that share immunological properties with blood group antigens occurred less frequently after ABO-incompatible heart transplantation. INTERPRETATION: ABO-incompatible heart transplantation for children younger than 2 years is a clinically safe approach, with similar survival and incidences of rejection, coronary allograft vasculopathy, and malignancy to ABO-compatible recipients, despite higher-risk pre-transplant profiles. ABO-incompatible transplantation was associated with less bacterial infection, particularly encapsulated bacteria, suggesting that the acquired immunological changes accompanying ABO tolerance might benefit rather than jeopardise transplanted children. FUNDING: Pediatric Heart Transplant Society.

Pretransplantation and Post-Transplantation Liver Disease Assessment in Adolescents Undergoing Isolated Heart Transplantation for Fontan Failure.

OBJECTIVE: To describe the assessment of Fontan-associated liver disease and determine the clinical and imaging measures that may identify hepatic morbidity risk in isolated heart transplantation candidates and trend those measures post-isolated heart transplantation. STUDY DESIGN: Retrospective analysis of pre-isolated heart transplantation and post-isolated heart transplantation Fontan-associated liver disease (FALD) status using blood tests, magnetic resonance imaging (MRI), and liver biopsy analysis within 6 months before isolated heart transplantation and 12 months after isolated heart transplantation in 9 consecutive patients with Fontan. Pre- and post-isolated heart transplantation standard laboratory values; varices, ascites, splenomegaly, thrombocytopenia (VAST) score; Fontan liver MRI score; liver biopsy scores; Model for End-stage Liver Disease (MELD); MELD excluding the International Normalized Ratio (MELD-XI); AST to platelet ratio index, and cardiac catheterization data were compared. RESULTS: Pretransplantation maximum MELD and MELD-XI was 15 and 16, respectively. Central venous pressures and VAST scores decreased significantly post-transplantation. In 5 paired studies, Fontan liver MRI score maximum was 10 pretransplantation and decreased significantly post-transplantation. Arterially enhancing nodules on MRI persisted in 2 patients post-transplantation. Pretransplantation and post-transplantation liver biopsy scores did not differ in 4 paired biopsy specimens. CONCLUSIONS: Patients with FALD and MELD <15, MELD-XI <16, Fontan liver MRI score <10, and VAST score ≤2 can have successful short-term isolated heart transplantation outcomes. Liver MRI and VAST scores improved post-transplantation. Post-transplantation liver biopsy scores did not change significantly. Pretransplantation liver biopsy demonstrating fibrosis alone should not exclude consideration of isolated heart transplantation. The persistence of hepatic vascular remodeling and fibrosis post-isolated heart transplantation suggests that continued surveillance for hepatic complications post-transplantation for patients with Fontan is reasonable.

Intracorporeal Biventricular Assist Devices Using the Heartware Ventricular Assist Device in Children.

We describe the experience with biventricular HeartWare VAD (HVAD) support in the pediatric population. As of May 2017, using the Heartware database, 11 centers were identified. Seven centers participated providing information for 10 patients (four females, six males). Median age at the time of implantation was 12.7 ± 4.6years (5.3-6.9), median body surface area was 1.56. The indications for biventricular assist device (BiVAD) support included: myocarditis (n = 1), cardiomyopathy (n = 8), and one posttransplant heart failure. Six patients had a primary BiVAD implantation. Out of 10 patients with BiVAD, five were transplanted, one is ongoing (postoperatively day 207), and none were weaned from the device. Two patients (20%) were discharged from hospital on BiVAD support. Median support time was 52 days (16-235). The overall success rate was 60% surviving to transplant (median support time: 51 days) or are ongoing. Reasons for death included bleeding (n = 2), intracerebral hemorrhage (n = 1), and multisystem organ failure (n = 1). Eight out of 10 patients had at least one major complication, that is, major bleeding requiring re-operation (n = 6), severe neurologic injury (n = 1), and pump thrombosis in two patients requiring device exchange (n = 1) or thrombolysis therapy (n = 1). BiVAD HVAD implantation in children is a rare procedure, with high mortality and low discharge rates. Bleeding requiring re-operation was the most common postoperative complication, despite fresh sternotomies in the majority of patients. The risk and benefit ratio of such an approach should be carefully reviewed, and compared with the standard strategy using the Berlin EXCOR.

Factors Associated With Survival Following Extracorporeal Cardiopulmonary Resuscitation in Children.

OBJECTIVES: We examined a large single-institution experience in extracorporeal cardiopulmonary resuscitation (ECPR) in children having cardiac arrest refractory to conventional resuscitation measures with focus on factors affecting survival. METHODS: Between 2002 and 2017, 184 children underwent ECPR at our institution. We entered demographic, anatomic, clinical, surgical, and ECPR support details into a multivariable logistic regression models to determine factors associated with mortality. RESULTS: Median age was 54 days (interquartile range [IQR]: 11-272). In all, 157 (85%) patients had primary cardiac disease, including 136 (74%) with congenital heart disease (71 with single ventricle). Extracorporeal cardiopulmonary resuscitation occurred following cardiac surgery in 124 (67%) patients. Median cardiopulmonary resuscitation (CPR) duration was 27 minutes (IQR: 18-40) and median support duration was 3.0 days (IQR: 1.6-5.3). Overall, ECPR was weaned in 115 (63%), with 79 (43%) surviving to hospital discharge. Survival for patients with congenital heart disease, noncongenital cardiac, and noncardiac pathologies was 44%, 71%, and 15%, respectively. On multivariable regression analysis, risk factors associated with mortality were presupport pH <7.1 (odds ratio [OR] = 3.7, 95% confidence interval [CI]: 1.11-12.41, P = .033), mechanical complications (OR = 8.33, 95% CI: 1.91-36.25, P = .005), neurologic complications (OR = 6.27, 95% CI: 1.40-28.10, P = .017), and renal replacement therapy (OR = 3.31, 95% CI: 1.03-10.66, P = .045). CONCLUSIONS: Extracorporeal cardiopulmonary resuscitation plays a valuable role salvaging children with refractory cardiac arrest. Survival varies with underlying pathology and can be expected even with relatively longer CPR durations. Efforts to improve systemic output before and after institution of ECPR might mitigate some of the significant risk factors for mortality.

A current era analysis of ABO incompatible listing practice and impact on outcomes in young children requiring heart transplantation.

BACKGROUND: Heart transplantation from ABO incompatible (ABOi) donors has evolved into a progressively accepted therapy in young children. We assessed the recent practice of ABOi listing impact on waitlist and post-transplant outcomes. METHODS: Using the Pediatric Heart Transplant Society registry, we compared clinical presentation, waitlist parameters, and post-transplant survival of children < 2 years of age listed for ABOi vs ABO compatible (ABOc) heart transplant between January 2010 and June 2018 with sub-analysis of blood group O recipients. RESULTS: Among 2,039 patients, ABOi listing increased significantly with time from 49% (2010) to 72% (2017). ABOi-listed patients had lower age and body surface area, and higher proportion of congenital heart disease, mechanical ventilation, and high urgency status (all p < 0.01). Use of mechanical circulatory support was similar between groups. Of 1,288 patients reaching transplant, 239 (18.6%) received an ABOi organ (15%-40%/year). Death while waiting, removal from the waitlist, and waitlist survival were similar between groups. Time to transplant was significantly shorter for ABOi listing in blood group O patients (p < 0.02), approaching significance (p = 0.057) for all blood groups. Post-transplant survival was similar except for lower survival of patients listed ABOc but transplanted ABOi. These patients showed increasing need for mechanical circulatory support and high urgency listing while waiting. CONCLUSIONS: In the current era, primary listing for ABOi heart transplant has become routine for the majority of children < 2 years old, resulting in shorter waitlist time, especially in blood group O. Post-transplant survival is similar despite ABOi-listed children still showing a higher risk profile.

Y-graft modification to the Fontan procedure: Increasingly balanced flow over time.

OBJECTIVE: The use of Y-grafts for Fontan completion is hypothesized to offer more balanced hepatic flow distribution (HFD) and decreased energy losses. The purpose of this study was to evaluate the hemodynamic performance of Y-grafts over time using serial cardiac magnetic resonance data and to compare their performance with extracardiac Fontan connections. METHODS: Ten Fontan patients with commercially available Y-graft connections and serial postoperative cardiac magnetic resonance data were included in this study. Patient-specific computational fluid dynamics simulations were used to estimate HFD and energy losses. Y-graft performance was compared with 3 extracardiac conduit Fontan groups (n = 10 for each) whose follow-up times straddle the Y-graft time points. RESULTS: Y-graft HFD became significantly more balanced over time (deviation from 50% decreased from 18% ± 14% to 8% ± 8%; P = .015). Total cavopulmonary connection resistance did not significantly change. Y-grafts at 3-year follow-up showed more balanced HFD than the extracardiac conduit groups at both the earlier and later follow-up times. Total cavopulmonary connection resistance was not significantly different between any Y-graft or extracardiac conduit group. CONCLUSIONS: Y-grafts showed significantly more balanced HFD over a 3-year follow-up without an increase in total cavopulmonary connection resistance, and therefore may be a valuable option for Fontan completion. Additional follow-up data at longer follow-up times are still needed to thoroughly characterize the potential advantages of Y-graft use.

The first cohort of prospective Fontan surgical planning patients with follow-up data: How accurate is surgical planning?

OBJECTIVE: Fontan surgical planning is an image-based, collaborative effort, which is hypothesized to result in improved patient outcomes. A common motivation for Fontan surgical planning is the progression (or concern for progression) of pulmonary arteriovenous malformations. The purpose of this study was to evaluate the accuracy of surgical planning predictions, specifically hepatic flow distribution (HFD), a known factor in pulmonary arteriovenous malformation progression, and identify methodological improvements needed to increase prediction accuracy. METHODS: Twelve single-ventricle patients who were enrolled in a surgical planning protocol for Fontan surgery with pre- and postoperative cardiac imaging were included in this study. Computational fluid dynamics were used to compare HFD in the surgical planning prediction and actual postoperative conditions. RESULTS: Overall, HFD prediction error was 17 ± 13%. This error was similar between surgery types (15 ± 18% and 18 ± 10% for revisions vs Fontan completions respectively; P = .73), but was significantly lower (6 ± 7%; P = .05) for hepatic to azygous shunts. Y-grafts and extracardiac conduits showed a strong correlation between prediction error and discrepancies in graft insertion points (r = 0.99; P < .001). Improving postoperative anatomy prediction significantly reduced overall HFD prediction error to 9 ± 6% (P = .03). CONCLUSIONS: Although Fontan surgical planning can offer accurate HFD predictions for specific graft types, methodological improvements are needed to increase overall accuracy. Specifically, improving postoperative anatomy prediction was shown to be an important target for future work. Future efforts and refinements to the surgical planning process will benefit from an improved understanding of the current state and will rely heavily on increased follow-up data.

An in vitro analysis of the PediMag and CentriMag for right-sided failing Fontan support.

OBJECTIVE: Right-sided mechanical circulatory support for failing Fontan physiology has been largely unsuccessful due to inherent hemodynamic differences between these patients and the target populations for most assist devices. This study uses advanced benchtop modeling of Fontan physiology to examine the use of PediMag and CentriMag to improve failing Fontan hemodynamics. METHODS: Each device was attached to a compliance-matched, patient-specific total cavopulmonary connection in vitro model that used resistances, compliances, and programmable waveforms to establish "failing Fontan" baseline hemodynamics (cardiac output [CO] = 3.5 L/min and central venous pressure ∼17 mm Hg). The ability of the assist devices to improve failing Fontan hemodynamics (reduce inferior vena cava pressure and augment CO) was investigated. RESULTS: Requiring complete Fontan pathway restriction, PediMag reduced inferior vena cava pressure by ∼10 mm Hg and supported CO augmentation up to 5 L/min. This was accompanied by an increase in superior vena cava pressure of ∼6 mm Hg. CentriMag produced similar hemodynamic changes without the need for pathway restriction or an increase in superior vena cava pressure. CONCLUSIONS: PediMag and CentriMag right-sided support led to a decrease in inferior vena cava pressure and augmentation of cardiac output. In the case of CentriMag, this is accomplished without an increase in superior vena cava pressure or the need for restrictive banding. This work provides further data to help with the optimal design of a Fontan assist device to ameliorate the growing need.

Fontan Surgical Planning: Previous Accomplishments, Current Challenges, and Future Directions.

The ultimate goal of Fontan surgical planning is to provide additional insights into the clinical decision-making process. In its current state, surgical planning offers an accurate hemodynamic assessment of the pre-operative condition, provides anatomical constraints for potential surgical options, and produces decent post-operative predictions if boundary conditions are similar enough between the pre-operative and post-operative states. Moving forward, validation with post-operative data is a necessary step in order to assess the accuracy of surgical planning and determine which methodological improvements are needed. Future efforts to automate the surgical planning process will reduce the individual expertise needed and encourage use in the clinic by clinicians. As post-operative physiologic predictions improve, Fontan surgical planning will become an more effective tool to accurately model patient-specific hemodynamics.

Local Hemodynamic Differences Between Commercially Available Y-Grafts and Traditional Fontan Baffles Under Simulated Exercise Conditions: Implications for Exercise Tolerance.

Fontan completion, resulting in a total cavopulmonary connection (TCPC), is accomplished using a lateral tunnel (LT), extracardiac conduit (ECC), or recently a bifurcated Y-graft. The local energetic differences between these graft types have not been substantially analyzed under exercise conditions. The present study evaluates the energetic performance of Y-grafts under simulated exercise conditions, compares their performance to the previous LT/ECC Fontan options, and discusses implications for exercise tolerance and hemodynamic predictability. Twenty Y-graft and 20 LT/ECC patients were analyzed. TCPC anatomies and flow waveforms were reconstructed using patient-specific cardiac magnetic resonance (CMR) images and phase-contrast CMR. Computational fluid dynamics simulations were performed to quantify indexed power loss (iPL) under both resting and simulated exercise conditions. Comparisons between graft types were investigated. iPL was significantly higher (p < 0.01) for Y-grafts at all activity levels. No significant interaction effects were observed between graft type and activity level. iPL at rest was strongly correlated (r 2 = 0.97, p < 0.001) with iPL at moderate exercise for Y-grafts, but less so for the LT/ECC cohort (r 2 = 0.66, p < 0.001). Similar results were seen for intense exercise, with a strong correlation for Y-grafts (r 2 = 0.94, p < 0.001) and a moderate correlation for LT/ECC (r 2 = 0.52, p < 0.001). Commercially available Y-grafts were found to have significantly higher iPL at all activity levels, suggesting worse exercise tolerance than the LT/ECC alternatives. Y-grafts offered impressive hemodynamic predictability which was not seen in the LT/ECC cohort. Our results encourage the further evaluation of an area-preserving Y-graft design to offer both improved energetic performance and hemodynamic predictability. Commercial Y-grafts show worse energetics, but more predictable responses than traditional Fontan connections under simulated exercise conditions. During simulated exercise conditions, commercially available Y-grafts show predictable but inferior energetic performance compared to lateral tunnel and extracardiac conduit Fontan connections, suggesting poorer exercise capacity. If Y-graft use is continued, these results encourage further evaluation of a cross sectional area-preserving Y-graft design as a additional alternative for Fontan completion.

How Is Physician Work Valued?

Strategies to value physician work continue to evolve. The Society of Thoracic Surgeons and The Society of Thoracic Surgeons National Database have an increasingly important role in this evolution. An understanding of the Current Procedural Terminology (CPT) system (American Medical Association [AMA], Chicago, IL) and the Relative Value Scale Update Committee (RUC) is necessary to comprehend how physician work is valued. In 1965, with the dawn of increasingly complex medical care, immense innovation, and the rollout of Medicare, the need for a common language describing medical services and procedures was recognized as being of critical importance. In 1966, the AMA, in cooperation with multiple major medical specialty societies, developed the CPT system, which is a coding system for the description of medical procedures and medical services. The RUC was created by the AMA in response to the passage of the Omnibus Budget Reconciliation Act of 1989, legislation of the United States of America Federal government that mandated that the Centers for Medicare & Medicaid Services adopt a relative value methodology for Medicare physician payment. The role of the RUC is to develop relative value recommendations for the Centers for Medicare & Medicaid Services. These recommendations include relative value recommendations for new procedures or services and also updates to relative value recommendations for previously valued procedures or services. These recommendations pertain to all physician work delivered to Medicare beneficiaries and propose relative values for all physician services, including updates to those based on the original resource-based relative value scale developed by Hsaio and colleagues. In so doing, widely differing work and services provided can be reviewed and comparisons of their relative value (to each other) can be established. The resource-based relative value scale assigns value to physician services using relative value units (RVUs), which consist of three components: work RVU, practice expense RVU, and malpractice RVU, also known as professional liability insurance RVU. The Centers for Medicare & Medicaid Services retains the final decision-making authority on the RVUs associated with each procedure or service. The purpose of this article is to discuss the role that the CPT codes and the RUC play in the valuation of physician work and to provide an example of how the methodology for valuation of physician work continues to evolve.

Completeness and Accuracy of Local Clinical Registry Data for Children Undergoing Heart Surgery.

BACKGROUND: Data routinely captured in clinical registries may be leveraged to enhance efficiency of prospective research. The quality of registry data for this purpose has not been studied, however. We evaluated the completeness and accuracy of perioperative data within congenital heart centers' local surgical registries. METHODS: Within 12 Pediatric Heart Network (PHN) sites, we evaluated 31 perioperative variables (and their subcategories, totaling 113 unique fields) collected via sites' local clinical registries for submission to The Society of Thoracic Surgeons Database, compared with chart review by PHN research coordinators. Both used standard STS definitions. Data were collected on 10 subjects for 2 to 5 procedures/site and adjudicated by the study team. Completeness and accuracy (agreement of registry data with medical record review by PHN coordinator, adjudicated by the study team) were evaluated. RESULTS: A total of 56,500 data elements were collected on 500 subjects. With regard to data completeness, 3.1% of data elements were missing from the registry, 0.6% from coordinator-collected data, and 0.4% from both. Overall, registry data accuracy was 98%. In total, 94.7% of data elements were both complete/non-missing and accurate within the registry, although there was variation across data fields and sites. Mean total time for coordinator chart review per site was 49.1 hours versus 7.0 hours for registry query. CONCLUSIONS: This study suggests that existing surgical registry data constitute a complete, accurate, and efficient information source for prospective research. Variability across data fields and sites also suggest areas for improvement in some areas of data quality.

Echocardiographic Predictors of Left Ventricular Outflow Tract Obstruction following Repair of Atrioventricular Septal Defect.

BACKGROUND: Left ventricular outflow tract obstruction (LVOTO) is a common complication following surgical repair of atrioventricular septal defect (AVSD). OBJECTIVE: We sought to determine predictors of LVOTO based on echocardiograms performed prior to initial repair of AVSD. METHODS: Of the 415 children that had repair of AVSD from 2003 to 2012, 17 children were identified with LVOTO that required surgical intervention. Thirty-four patients with repaired AVSD and no LVOTO served as controls. Patient demographics, cardiac surgery type, and echocardiogram results at last follow-up were collected. Off-line analysis of the echocardiogram prior to AVSD repair was done to obtain: left ventricular outflow tract (LVOT) and interventricular septal diameter, chordae across LVOT, aortoseptal angle, left ventricular (LV) inflow/outflow length ratio, inferior displacement of the atrioventricular (AV) valve, atrioventricular valve index (AVVI) and presence of coarctation of the aorta. RESULTS: The LVOTO group had significantly smaller indexed LVOT diameters (P < .001), increased likelihood of chordae crossing the LVOT (P = .010), decreased LV inflow/outflow length ratio (P < .001), decreased AVVI (P = .014) and increased presence of coarctation of the aorta (P = .003) compared to control patients. A multiple logistic regression model including presence of chordae in the LVOT [OR 5.32, 95% CI: (1.24-22.78, P = .024] and an indexed LVOT diameter ≤2.5 cm/m2 [OR 5.41, 95% CI: (1.15-25.39) P = .032] demonstrated each to be independently associated with the development of LVOT obstruction (area under the receiving operating curve = 0.77). CONCLUSIONS: Chordae across the LVOT and an indexed LVOT diameter of ≤2.5 cm/m2 are associated with a higher risk of development of LVOTO following repair of AVSD. Identification of these risk factors on echocardiogram prior to initial AVSD repair can be useful in evaluating for future risk of LVOTO and the need for closer clinical follow-up.

Early Extubation After Repair of Tetralogy of Fallot and the Fontan Procedure: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

BACKGROUND: There is increasing interest in reducing the duration of mechanical ventilation after pediatric cardiac operations. However, the contemporary use of an early extubation strategy and its effect on clinical outcomes is poorly understood. METHODS: Data from The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010 to 2013) were analyzed to determine the association of early extubation with postoperative length of stay (PLOS). Two operations were analyzed: complete repair of tetralogy of Fallot (TOF) in patients older than 45 days and the Fontan procedure. Centers were stratified into tertiles by frequency of early extubation, defined as less than 6 hours after leaving the operating room. Patients extubated after 48 hours were excluded. RESULTS: Among 92 centers, early extubation was performed in 31.5% (478 of 1,519) of children undergoing TOF repair and in 69.8% (1,153 of 1,653) of those undergoing the Fontan procedure. Early extubation after TOF repair was associated with heavier weight at operation (p < 0.001) and fewer preoperative risk factors (p = 0.016). After adjustment for covariates, average PLOS after TOF repair was shorter for centers in the highest tertile of early extubation rate than for the lowest tertile centers, which have low early extubation rate (p = 0.04). No association was found between center early extubation rate and PLOS for Fontan procedures (p = 0.08). CONCLUSIONS: Early extubation is common after repair of TOF and the Fontan procedure. A high institutional rate of early extubation after TOF repair is associated with shorter PLOS. Further analysis is needed to understand what effect early extubation might have on other meaningful measures such as resource use.