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Computational fluid dynamics (CFD) is a modeling technique that enables calculation of the behavior of fluid flows in complex geometries. In cardiovascular medicine, CFD methods are being used to calculate patient-specific hemodynamics for a variety of applications, such as disease research, noninvasive diagnostics, medical device evaluation, and surgical planning. This paper provides a concise overview of the methods to perform patient-specific computational analyses using clinical data, followed by a case study where CFD-supported surgical planning is presented in a patient with Fontan circulation complicated by unilateral pulmonary arteriovenous malformations. In closing, the challenges for implementation and adoption of CFD modeling in clinical practice are discussed.

Biykem Bozkurt, MD, PhD, FACC, FAHA, Chair H. Vernon Anderson, MD, FACC, FAHA Garth N. Graham, MD, FACC Hani Jneid, MD, FACC, FAHA Gail K. Jones, MD David Kao, MD, FAHA Michael Kutcher, MD, FACC Leo Lopez, MD, FACC Gregory Marcus, MD, FACC, FAHA Jennifer Rymer, MD James E. Tcheng, MD

Background Infants who require extracorporeal membrane oxygenation (ECMO) support after a Norwood operation are at increased risk for early and late death compared with patients who do not require ECMO post-Norwood. Little is known about the effect that ECMO post-Norwood has on functional status and quality of life among long-term survivors. Methods We prospectively evaluated functional status and health-related quality of life in 12 surviving patients (cases) and 19 corresponding patients (controls) from a previous retrospective case-control assessment of long-term survival in patients requiring ECMO post-Norwood. Functional status was assessed with the Vineland Adaptive Behavior Scale-II, and health-related quality of life was assessed with the Pediatric Quality of Life Inventory (PedsQL) core and cardiac modules. Results There were no differences in demographics, extracardiac or genetic anomalies, or age at follow-up assessment between ECMO cases and non-ECMO controls. The Vineland Adaptive Behavior Scale-II scores were comparable between groups, with both groups demonstrating function in the normal range in all four domains tested. The only difference in PedsQL scores between cases and controls was perceived physical appearance, which was lower among ECMO survivors by both patient and proxy report. PedsQL scores of both groups were comparable to published scores for patients with single-ventricle congenital heart disease but generally lower than scores for the healthy population. Conclusions The requirement for ECMO support after a Norwood operation does not appear to significantly affect functional status or quality of life among the subset of patients who achieve long-term survival.

Objectives To describe our 20-year experience with intraoperative pulmonary artery (PA) stent placement and evaluate long-term patient outcomes, specifically the need and risk factors for reintervention. Background Intraoperative PA stent placement is an alternative to surgical patch arterioplasty and percutaneous angioplasty or stent placement to treat branch PA stenosis. Methods We performed a retrospective review of all intraoperative PA stents placed at our institution from 1994-2013. Patient and stent characteristics and outcome data were collected. Risk factors associated with reintervention were identified using univariate cox regression analysis. Results Eighty-one PA stents were placed in 68 patients. The procedural complication rate was 4.4%. During a median follow-up period of 6 years (interquartile range [IQR] 0.9-12.7), 30 patients (44%) underwent reintervention on the stented PA with a median time to first reintervention of 2.6 years (IQR 0.7–4.4 years). The first reintervention was surgical in 30% and catheter-based in 70%. Risk factors for reintervention included age

The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of 10 benchmark operations performed from January 2012 to December 2015. This analysis documents the overall aggregate operative mortality (interquartile range among all participating programs) for the following procedural groups: off-bypass coarctation repair, 1.3% (0.0% to 1.8%); ventricular septal defect repair, 0.6% (0.0% to 0.9%); tetralogy of Fallot repair, 1.1% (0.0% to 1.4%); complete atrioventricular canal repair, 3.0% (0.0% to 4.7%); arterial switch operation, 2.7% (0.0% to 4.1%); arterial switch operation and ventricular septal defect repair, 5.3% (0.0% to 6.7%); Glenn/hemi-Fontan, 2.5% (0.0% to 4.5%); Fontan operation, 1.2% (0.0% to 1.2%); truncus arteriosus repair, 9.4% (0.0% to 16.7%); and Norwood procedure, 15.7% (8.9% to 25.0%).

Previous studies have evaluated recurrent coarctation after the Norwood procedure (NP) in patients with single-ventricle (SV) anatomy. Extended patch augmentation of the aorta, a Norwood-type arch reconstruction (NTAR), is also used in patients with 2 ventricles and a diffusely hypoplastic arch; however, outcomes after NTAR in these patients are not well described.A retrospective review was performed of neonates with SV and 2-ventricle (2V) anatomy who underwent an NP or NTAR at our institution from 2000 to 2010. The incidence of recurrent coarctation requiring intervention and associated risk factors were evaluated.Overall, 101 patients with 2V anatomy and 361 patients with SV anatomy were included. Eighteen patients with 2V anatomy (17.8%) and 35 patients with SV anatomy (9.7%) required intervention for recurrent coarctation at a median of 0.5 years (interquartile range [IQR], 0.3-1.2 years) after the initial operation. Independent risk factors associated with recurrent coarctation in patients with 2V anatomy included weight less than 2.5 kg (hazard ratio [HR], 6.05; p = 0.001) and peak aortic arch gradient (PAAG) on the discharge echocardiogram greater than 10 mm Hg (HR, 3.07; p = 0.03). In patients with SV anatomy, shunt type (HR, 6.42; p0.0001 for right ventricle to pulmonary artery [RV-PA] shunt compared with others) and peak gradient on the discharge echocardiogram greater than 10 mm Hg were found to be significant (HR, 7.40; p0.0001). There was no survival difference in patients with and those without recurrent coarctation.Recurrent coarctation is common after NTAR. Small patient size and shunt type were found to be independent risk factors for recurrent coarctation in the 2V and SV populations, respectively, and discharge aortic arch gradient was a significant risk factor in both populations.

Background Previous studies have demonstrated the effect of adult nursing skill mix, staffing ratios, and level of education on patient deaths, complication rates, and failure to rescue (FTR). To date, only one known study had examined the effect of nursing experience and education on postoperative pediatric cardiac operations. Methods Nursing survey data were linked to The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database for patients undergoing cardiac operations (2010 to 2011). Logistic regression models were used to estimate associations of nursing education and years of clinical experience with in-hospital mortality rates, complication rates, and FTR. Generalized estimating equations and robust standard error estimates were used to account for within-center correlation of outcomes. Results Among 15,463 patients (29 hospitals), the in-hospital mortality rate was 2.8%, postoperative complications occurred in 42.4%, and the FTR rate was 6.4%. After covariate adjustment, pediatric critical care units with a higher proportion of nurses with a Bachelor of Science degree or higher had lower odds of complication (odds ratio for 10% increase, 0.85; 95% confidence interval, 0.76 to 0.96; p = 0.009). Units with a higher proportion of nurses with more than 2 years of experience had lower mortality rates (odds ratio for 10% increase, 0.92; 95% confidence interval, 0.85 to 0.99; p = 0.025). Conclusions This is the first study to demonstrate that higher levels of nursing education and experience are significantly associated with fewer complications after pediatric cardiac operations and aligns with our previous findings on their association with reduced deaths. These results provide data for pediatric hospital leaders and reinforce the importance of organization-wide mentoring strategies for new nurses and retention strategies for experienced nurses.

Large volumes of data and multiple computing platforms are now universal components of paediatric cardiovascular medicine, but are in a constant state of evolution. Often, multiple sets of related data reside in disconnected “silos”, resulting in clinical, administrative, and research activities that may be duplicative, inefficient, and at times inaccurate. Comprehensive and integrated data solutions are needed to facilitate these activities across congenital heart centres. We describe methodology, key considerations, successful use cases, and lessons learnt in developing an integrated data platform across our congenital heart centre.

Background There are limited data regarding long-term outcomes after the Ross procedure in children. We evaluated mortality and reintervention in a large pediatric cohort. Methods A retrospective analysis of all patients aged younger than 18 years who underwent the Ross procedure at our institution (1991 to 2013) was conducted. Kaplan-Meier curves and Cox proportion hazard models were used to evaluate long-term outcomes and associated risk factors. Results Included were 240 consecutive patients undergoing a Ross/Ross-Konno procedure: 18% infants, 48% children, and 33% adolescents. Infants were more likely to have complex left heart disease ( p = 0.005). Overall survival to hospital discharge was 96%; infants had the highest mortality (18%). Long-term survival status was known for 99.6% (median follow-up, 10.7 years). Overall 15-year survival was 87% (lowest in infants, 72%; p = 0.003). Reintervention status was known in 87%. Overall 15-year freedom from any left ventricular outflow tract reintervention was 59%; 85% still had their autograft valve at the latest follow-up. Left ventricular outflow tract reintervention was uncommon in infants (n = 2). Overall 15-year freedom from right ventricular outflow tract reintervention was 53%, and was lower in infants (19%) than in children (51%) and adolescents (76%; p Conclusions Outcomes after the Ross procedure in children vary by age. Infants more commonly have complex left heart disease and experience higher mortality but have excellent long-term autograft durability. Children and adolescents have higher rates of left ventricular outflow tract reintervention, whereas infants are at highest risk of right ventricular outflow tract reintervention.

Background: Abdominal cardiac implantable electronic device maintenance procedures are traditionally performed by cardiac surgeons. The University of Michigan Congenital Heart Center transitioned from this practice model to one in which electrophysiologists perform the majority of these procedures. This study presents the outcomes of these procedures during this transition. Methods: Retrospective cohort study of all patients undergoing abdominal device maintenance procedure, inclusive of generator change, removal, or placement with preexisting leads from January 2005 to July 2013. Procedures involving epicardial lead placement were excluded. The primary outcome was major complications defined as any intraoperative complication, the requirement of an additional operative intervention, or hospitalization for device-related infection. Results: There were 113 procedures on 93 patients. Of these, 84 (74%) procedures were on patients with congenital heart disease. Cardiac surgeons performed 54 (48%) procedures and electrophysiologists performed 59 (52%). Mean age was 16 ± 11 years. The groups were similar regarding age and proportion with congenital heart disease (CHD). Major complications occurred in 3 (5.5%) cardiac surgeon procedures and 2 (3.4%) electrophysiologist procedures. There is no difference in the risk of major complications between groups ( P = .59). Conclusion: This 8.5-year period encompassed a practice model transition from cardiac surgeon-performed abdominal device procedures to primarily electrophysiologist-performed abdominal device procedures. There was no difference in the risk of complications between services. This suggests that electrophysiologist-performed abdominal cardiac device maintenance procedures are a viable practice model, provided there is support and collaboration from the cardiac surgery service.

Although outcomes for infants with complex single ventricle heart defects have steadily improved in recent decades, there is still a significant risk for mortality and morbidity during the interstage period between stage 1 Norwood hospitalization discharge and stage 2 palliation. Home monitoring programs, which involve parental surveillance of daily weight and oxygen saturations during the interstage period, have been shown to significantly improve survival rates. This article describes the potential risk factors or causes of interstage mortality and reviews the role of home monitoring in early detection and potential prevention of adverse outcomes.

The American College of Cardiology (ACC) and the American Heart Association (AHA) support their members’ goal to improve the care of patients with cardiovascular disease through professional education, research, and development of guidelines and standards and by fostering policies that support optimal patient outcomes. The ACC and AHA recognize the importance of the use of clinical data standards for patient management, assessment of outcomes, and conduct of research, and the importance of defining the processes and outcomes of clinical care, whether in randomized trials, observational studies, registries, or quality improvement initiatives. Clinical data standards strive to define and standardize data relevant to clinical concepts, with the primary goal of facilitating uniform data collection by providing a platform of clinical terms with corresponding definitions and data elements. Broad agreement on a …

Background: Resource utilization in congenital heart surgery is typically assessed using administrative data sets. Recent analyses have called into question the accuracy of coding of cases in administrative data; however, it is unclear whether miscoding impacts assessment of associated resource use. Methods: We merged data coded within both an administrative data set and clinical registry on children undergoing heart surgery (2004-2010) at 33 hospitals. The impact of differences in coding of operations between data sets on reporting of postoperative length of stay (PLOS) and total hospital costs associated with these operations was assessed. Results: For each of the eight operations of varying complexity evaluated (total n = 57,797), there were differences in coding between data sets, which translated into differences in the reporting of associated resource utilization for the cases coded in either data set. There were statistically significant differences in PLOS and cost for seven of the eight operations, although most PLOS differences were relatively small with the exception of the Norwood operation and truncus repair (differences of two days, P < .001). For cost, there was a >5% difference for three of the eight operations and >10% difference for truncus repair (US$10,570; P < .01). Grouping of operations into categories of similar risk appeared to mitigate many of these differences. Conclusion: Differences in coding of cases in administrative versus clinical registry data can translate into differences in assessment of associated PLOS and cost for certain operations. This may be minimized through evaluating larger groups of operations when using administrative data or using clinical registry data to accurately identify operations of interest.

Purpose: A link has been created between the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and the Congenital Heart Surgeons’ Society Database (CHSS-D). Five matrices have been created that facilitate the automated identification of patients who are potentially eligible for the five active CHSS studies using the STS-CHSD. These matrices are now used to (1) estimate the denominator of patients eligible for CHSS studies and (2) compare “eligible and enrolled patients” to “potentially eligible and not enrolled patients” to assess the generalizability of CHSS studies. Methods: The matrices were applied to 40 consenting institutions that participate in both the STS-CHSD and the CHSS to (1) estimate the denominator of patients that are potentially eligible for CHSS studies, (2) estimate the completeness of enrollment of patients eligible for CHSS studies among all CHSS sites, (3) estimate the completeness of enrollment of patients eligible for CHSS studies among those CHSS institutions participating in each CHSS cohort study, and (4) compare “eligible and enrolled patients” to “potentially eligible and not enrolled patients” to assess the generalizability of CHSS studies. The matrices were applied to all participants in the STS-CHSD to identify patients who underwent frequently performed operations and compare “eligible and enrolled patients” to “potentially eligible and not enrolled patients” in following five domains: (1) age at surgery, (2) gender, (3) race, (4) discharge mortality, and (5) postoperative length of stay. Completeness of enrollment was defined as the number of actually enrolled patients divided by the number of patients identified as being potentially eligible for enrollment. Results: For the CHSS Critical Left Ventricular Outflow Tract Study (LVOTO) study, for the Norwood procedure, completeness of enrollment at centers actively participating in the LVOTO study was 34%. For the Norwood operation, discharge mortality was 15% among 227 enrolled patients and 16% among 1768 nonenrolled potentially eligible patients from the 40 consenting institutions. Median postoperative length of stay was 31 days and 26 days for these enrolled and nonenrolled patients. For the CHSS anomalous aortic origin of a coronary artery (AAOCA) study, for AAOCA repair, completeness of enrollment at centers actively participating in the AAOCA study was 40%. Conclusion: Determination of the denominator of patients eligible for CHSS studies and comparison of “eligible and enrolled patients” to “potentially eligible and not enrolled patients” provides an estimate of the extent to which patients in CHSS studies are representative of the overall population of eligible patients; however, opportunities exist to improve enrollment.

Premature infants with very low birth weight with tetralogy of Fallot and pulmonary atresia (TOF/PA) have been shown to have poor surgical outcomes. Palliating these patients by stenting the right ventricular outflow tract (RVOT) has been shown to be safe and effective. In patients with very low birth weight, a hybrid perventricular approach offers the ability to perform pulmonary valve perforation and RVOT stent placement while avoiding cardiopulmonary bypass and femoral vessel complications in the neonatal period. We present a hybrid perventricular treatment of a patient weighing 1.3 kg with TOF/PA.

Purpose: The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) is the largest Registry in the world of patients who have undergone congenital and pediatric cardiac surgical operations. The Congenital Heart Surgeons’ Society Database (CHSS-D) is an Academic Database designed for specialized detailed analyses of specific congenital cardiac malformations and related treatment strategies. The goal of this project was to create a link between the STS-CHSD and the CHSS-D in order to facilitate studies not possible using either individual database alone and to help identify patients who are potentially eligible for enrollment in CHSS studies. Methods: Centers were classified on the basis of participation in the STS-CHSD, the CHSS-D, or both. Five matrices, based on CHSS inclusionary criteria and STS-CHSD codes, were created to facilitate the automated identification of patients in the STS-CHSD who meet eligibility criteria for the five active CHSS studies. The matrices were evaluated with a manual adjudication process and were iteratively refined. The sensitivity and specificity of the original matrices and the refined matrices were assessed. Results: In January 2012, a total of 100 centers participated in the STS-CHSD and 74 centers participated in the CHSS. A total of 70 centers participate in both and 40 of these 70 agreed to participate in this linkage project. The manual adjudication process and the refinement of the matrices resulted in an increase in the sensitivity of the matrices from 93% to 100% and an increase in the specificity of the matrices from 94% to 98%. Conclusion: Matrices were created to facilitate the automated identification of patients potentially eligible for the five active CHSS studies using the STS-CHSD. These matrices have a sensitivity of 100% and a specificity of 98%. In addition to facilitating identification of patients potentially eligible for enrollment in CHSS studies, these matrices will allow (1) estimation of the denominator of patients potentially eligible for CHSS studies and (2) comparison of eligible and enrolled patients to potentially eligible and not enrolled patients to assess the generalizability of CHSS studies.

Objectives To examine acute and mid-term patient outcomes following the hybrid approach to pulmonary atresia with intact ventricular septum (PA-IVS) compared with the standard surgical approach. Background A subset of PA-IVS patients with the prospect of biventricular circulation typically undergo surgical or transcatheter right ventricular (RV) outflow tract opening. A recently described hybrid procedure, involving perventricular pulmonary valve perforation, was shown to be safe and effective in single-center series. Methods A single-center retrospective review of all patient with PA-IVS who underwent either surgical or hybrid RV decompression between January 2002 and December 2011 was completed and acute and mid-term patient outcomes were compared between the surgical and hybrid cohorts. Additionally, a systematic literature review was completed to compare a transcatheter cohort to the hybrid cohort. Results Seven patients with PA-IVS underwent a hybrid procedure; the procedure was technically successful in all attempts, and none required CPB. No patients required surgical re-intervention prior to hospital discharge, and none died during the study period. Surgical RV decompression was performed in 17 patients with a median CPB time of 80 min. Patient outcomes were nearly identical between cohorts. By systematic review, the transcatheter approach has a procedural success of 75–95% but up to 75% of patients require operation in the neonatal period. Conclusions The hybrid approach is a safe and feasible alternative to the standard surgical and transcatheter approaches to PA-IVS. Acute and mid-term patient outcomes are comparable with those treated with a standard surgical approach and neonatal CPB is completely avoided. © 2013 Wiley Periodicals, Inc.

Objectives We evaluated the use of, and outcomes associated with, balloon angioplasty (BA) for recurrent coarctation in single ventricle (SV) and two ventricle (2V) patients following a Norwood-type aortic arch reconstruction (NTAR). Background Extended patch augmentation of the aorta, a NTAR, is utilized in SV patients undergoing the Norwood procedure (NP) as well as 2V patients with a diffusely hypoplastic aorta. While many studies have evaluated recurrent coarctation following the NP, the incidence of recurrent coarctation and outcomes associated with BA in 2V patients following NTAR are unclear. Methods A retrospective review was performed of all neonates who underwent a NTAR at our institution between 2000 and 2010. The incidence of recurrent coarctation requiring intervention and factors associated with successful BA were evaluated. Results A NTAR was performed in 361 SV patients and 88 2V patients. The incidence of recurrent coarctation requiring intervention was 19.3% in 2V vs. 9.7% in SV patients (P = 0.01) at a median of 0.5 (interquartile range 0.3–1.2) years from initial surgery. BA was successful in 25 SV patients (81%) and 10 2V patients (71%; P = 0.70). Of the characteristics evaluated, lower initial peak-to-peak gradient (P = 0.02), larger balloon size for angioplasty (P = 0.02) and larger diameter of the descending aorta (P = 0.01) were associated with BA success. Conclusions Recurrent coarctation following NTAR is more common in 2V patients than in SV patients. BA for recurrent coarctation has similar success in both groups and should continue to be utilized in this population. © 2013 Wiley Periodicals, Inc.

Congenitally corrected transposition of the great arteries or l-transposition of the great arteries is characterized by discordance of both the atrioventricular and ventriculoarterial connections. Physiologic repair of associated conditions, whereby the morphologic right ventricle remains the systemic ventricle, has resulted in unsatisfactory long-term outcomes due to the development of right ventricular failure and tricuspid valve regurgitation. While intuitively attractive, anatomic repair also has inherent challenges and risks, particularly for those patients who present late and require left ventricular retraining. Although early and intermediate-term outcomes for anatomic repair have been encouraging, longer-term follow-up has demonstrated concern for late left ventricular dysfunction in this subgroup of patients. Continued monitoring of this challenging patient population will clarify late outcomes and inform clinical management in the future.

Background: In the early postoperative period after stage II palliation, patients with single ventricle physiology can have cyanosis due to a variety of causes. This cyanosis can be significant and necessitate cardiac catheterization to determine etiology and attempt treatment. Our objective was to determine the etiology of early postoperative cyanosis and outcomes in patients referred to the catheterization laboratory from the cardiac intensive care unit (CICU) after stage II palliation. Methods: We performed a retrospective analysis of all patients referred for cardiac catheterization from the CICU for evaluation of early cyanosis after stage II palliation. Etiology for hypoxemia, treatment strategy, and patient outcomes were examined for each patient. Results: Between January 1, 2006, and December 31, 2011, 244 patients underwent stage II palliation of which 22 required cardiac catheterization during the early postoperative period because of severe cyanosis. The etiologies for cyanosis were venovenous collaterals (n = 12), cavopulmonary pathway thrombosis (n = 3), hemi-Fontan pathway baffle leak (n = 2), and undetermined (n = 5). Overall, transplant-free survival to hospital discharge was 50% and survival to hospital discharge with stage II physiology was 32%. Venovenous collateral occlusion, cavopulmonary anastomosis takedown, and addition of a second source of pulmonary blood flow were not associated with improved outcome. Conclusions: Regardless of the etiology or treatment strategy, severe cyanosis in the early postoperative period after stage II palliation imparts high mortality and usually indicates failing stage II physiology. Venovenous collateral occlusion and thrombectomy are usually futile, and those who survive have a low likelihood of having stage II physiology at hospital discharge.

Staged palliation for neonates born with hypoplastic left heart syndrome (HLHS) and other single ventricle heart defects has provided a viable form of treatment with everimproving short- and long-term outcomes. The second-stage palliation for single ventricle heart defects, the hemi-Fontan, or bidirectional Glenn operation removes the volume overload on the ventricle from the systemic shunt. The incorporation of an early superior cavoplumonary connection to remove the deleterious effects of volume overload has dramatically improved long-term ventricular function in this patient population and is thought to be one of the largest contributors to improved outcomes in the modern era of staged palliation. Important components of this procedure include creating an unobstructed connection from the superior vena cava (SVC) to the pulmonary arteries (PAs) to provide adequate pulmonary blood flow, augmentation of the central PAs to remove any potential stenosis, avoidance of conduction disturbances, and anticipating the future connection of the inferior vena cava (IVC) to the PAs. Our technique differs from the original hemi-Fontan description 1,2 with 2 modifications. First, the incision on the right atrium is limited to the right atrial appendage and is therefore not carried onto the SVC. This minimizes the risk of transected the artery to the sinoatrial node, and we have demonstrated that this has reduced short- and long-term arrhythmias. 3 Second, the homograft patch augmentation is limited to the central PAs and a separate polytetraflouroethylene (PTFE) patch is utilized to exclude the SVC return from the right atrium. This simplifies the reestablishment of this connection while performing Fontan procedure. At the University of Michigan, the hemi-Fontan is preferred over the bidirectional Glenn as the second-stage operation of choice for patients with amenable anatomy. The reasons for this preference include the ability to correct any potential risk factors for poor long-term outcome such as PA distortion due to the systemic shunt, the maintenance of a stable circulation free of right ventricular volume overload, and the creation of a large eventual pathway for connection of the IVC to the PAs. The volume-unloading second-stage palliation for single ventricle cardiac lesion is generally performed at 4-6 months of age. The timing of surgery is dependent on the patient’s saturations, somatic growth, and need for intervention for associated defects, such as progressive tricuspid valve regurgitation. Preoperative cardiac catheterization is performed to evaluate right ventricular function, tricuspid regurgitation, residual arch or atrial septal obstruction, branch PA anatomy, and pulmonary vascular resistance. The hemi-Fontan procedure is certainly more complex than the bidirectional Glenn, but the routine augmentation of the branch PA associated with our technique of the hemiFontan ensures optimal PA anatomy and the effort dramatically simplifies the ultimate Fontan procedure. In addition, by performing a more complex operation at the time of stage 2 rather than at the Fontan procedure when the postoperative hemodynamics are more demanding, the postoperative course can be optimized as well.

To describe our 20-year experience with intraoperative pulmonary artery (PA) stent placement and evaluate long-term patient outcomes, specifically the need and risk factors for reintervention.Intraoperative PA stent placement is an alternative to surgical patch arterioplasty and percutaneous angioplasty or stent placement to treat branch PA stenosis.We performed a retrospective review of all intraoperative PA stents placed at our institution from 1994-2013. Patient and stent characteristics and outcome data were collected. Risk factors associated with reintervention were identified using univariate cox regression analysis.Eighty-one PA stents were placed in 68 patients. The procedural complication rate was 4.4%. During a median follow-up period of 6 years (interquartile range [IQR] 0.9-12.7), 30 patients (44%) underwent reintervention on the stented PA with a median time to first reintervention of 2.6 years (IQR 0.7-4.4 years). The first reintervention was surgical in 30% and catheter-based in 70%. Risk factors for reintervention included age 18 months (Hazard ratio [HR] 2.97, P = 0.005) and body surface area 0.47 mIntraoperative PA stent implantation is a safe and effective alternative to percutaneous stent implantation and offers several advantages, including the ability to implant adult-size stents in small patients while avoiding injury to peripheral vessels, to position stents to facilitate future percutaneous stent redilation, and to access the PAs directly, which eliminates radiation exposure. © 2017 Wiley Periodicals, Inc.

The combination of double outlet right ventricle and complete atrioventricular septal defect (DORV-AVSD) is a rare congenital heart malformation. Double outlet right ventricle with complete atrioventricular septal defect is commonly associated with heterotaxy but is distinct from DORV as associated with tetralogy of Fallot. Biventricular repair of DORV-AVSD is challenging because it requires not only correction of the complete AVSD and DORV, but often also involves resection of the outlet portion of the ventricular septum to create an unobstructed left ventricular outflow tract as well as repair of the frequently associated systemic and pulmonary venous connection anomalies and right ventricular outflow tract obstruction. Because of this high degree of complexity, functional single ventricle palliation has been recommended as a treatment option for DORV-AVSD by some groups. However, biventricular repair in these patients, despite its complexity, can be accomplished with excellent results. Reoperation is common in this difficult group of patients, most commonly for conduit replacement. Reintervention for recurrent left ventricular outflow tract obstruction or atrioventricular valve stenosis or regurgitation is uncommon. We feel that biventricular repair of DORV-AVSD is the preferred treatment option, although further studies are needed to define long-term outcomes.

The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This article summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of 10 benchmark operations performed from January 2011 to December 2014 and documenting overall discharge mortality (interquartile range among programs with more than 9 cases): off-bypass coarctation, 1.0% (0.0% to 0.9%); ventricular septal defect repair, 0.7% (0.0% to 1.1%); tetralogy of Fallot repair, 1.0% (0.0% to 1.7%); complete atrioventricular canal repair, 3.2% (0.0% to 6.5%); arterial switch operation, 2.7% (0.0% to 5.6%); arterial switch operation plus ventricular septal defect, 5.3% (0.0% to 6.7%); Glenn/hemiFontan, 2.1% (0.0% to 3.8%); Fontan operation, 1.4% (0.0% to 2.4%); truncus arteriosus repair, 9.6% (0.0 % to 11.8%); and Norwood procedure, 15.6% (10.0% to 21.4%).

Background Accurate hospital outcome measures in congenital heart surgery are important to multiple initiatives. While methods have been developed to account for differences in procedural case-mix, characteristics patients bring into the operation that may also vary across hospitals and influence outcome have received less attention. We evaluated the impact of these characteristics in a large cohort. Methods Patients undergoing congenital heart surgery at centers participating in The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010 to 2013) with adequate data quality were included. Variation across hospitals in important patient characteristics was examined, and hospital operative mortality rates were compared with and without adjustment for patient characteristics. Results Overall, 86 centers (52,224 patients) were included. There was greater than twofold variation across hospitals for nearly all patient characteristics examined. For example, the proportion of a center's surgical population comprised of neonates ranged from 12.8% to 26.6% across hospitals; the proportion with a non-cardiac anomaly ranged from 0.7% to 5.0%. When hospital mortality rankings were evaluated based on "standard" (adjustment for differences in procedural case-mix alone) versus "full" models (adjustment for both differences in procedural case-mix and patient characteristics), 14.0% changed their ranking for mortality by 20 or greater positions, 34.9% of centers changed which mortality quartile they were classified in, and 14.0% changed their statistical classification (statistically higher, lower, or same-as-expected mortality). Conclusions Characteristics of patients undergoing congenital heart surgery vary across centers and impact hospital outcomes assessment. Methods to assess outcomes and relative performance should account for these characteristics.

Interstage mortality remains significant for patients undergoing staged palliation for hypoplastic left heart syndrome and other related single right ventricle malformations (HLV). The purpose of this study was to identify factors related to demographics, socioeconomic position, and perioperative course associated with post-Norwood hospital discharge, pre-stage 2, interstage mortality (ISM). Medical record review was conducted for patients with HLV, born from 1/2000 to 7/2009 and discharged alive following the Norwood procedure. Sociodemographic and perioperative factors were reviewed. Patients were determined to have ISM if they died between Norwood procedure hospital discharge and stage 2 palliation. Univariable and multivariable logistic regressions were performed to identify risk factors associated with ISM. A total of 273 patients were included in the analysis; ISM occurred in 32 patients (12%). Multivariable analysis demonstrated that independent risk factors for interstage mortality included teen mothers [adjusted odds ratio (AOR) 6.6, 95% confidence interval (CI) 1.9-22.5], single adult caregivers (AOR 4.1, 95% CI 1.2-14.4), postoperative dysrhythmia (AOR 2.7, 95% CI 1.1-6.4), and longer ICU stay (AOR 2.7, 95% CI 1.2-6.1). Anatomic and surgical course variables were not associated with ISM in multivariable analysis. Patients with HLV are at increased risk of ISM if born to a teen mother, if they lived in a home with only one adult caregiver, suffered a postoperative dysrhythmia, or experienced a prolonged ICU stay. These risk factors are identifiable, and thus these infants may be targeted for interventions to reduce ISM.

To determine the prevalence of and risk factors for hemorrhagic complications in children with cardiac disease requiring extracorporeal membrane oxygenation.Retrospective review of the Extracorporeal Life Support Organization Registry (2002-2013).Participating Extracorporeal Life Support Organization centers.Patients less than 18 years old on extracorporeal membrane oxygenation.None.Of 21,845 patients requiring extracorporeal membrane oxygenation during the study period, 8,905 (41%) had cardiac disease, and 79% of whom (6,995) had cardiac surgery. Hemorrhagic complications occurred in 8,480 patients (39% of overall cohort), with higher rates in cardiac versus noncardiac patients (49% vs 32%; p0.0001) related to cannulation and surgical site bleeding. Cardiac surgical patients had higher rates of hemorrhage compared with cardiac medical patients (57% vs 38%; p0.0001), and cardiac patients with hemorrhage had higher extracorporeal membrane oxygenation mortality compared with those without (42% vs 22% in medical patients and 34% vs 20% in surgical patients; both p0.0001). In multivariable analysis in both the cardiac medical and surgical groups, hemorrhage risk was higher in children greater than 1 year old and in patients with longer extracorporeal membrane oxygenation duration. Additional independent risk factors for hemorrhage in cardiac surgical patients included pre-extracorporeal membrane oxygenation mediastinal exploration (odds ratio, 3.6; 95% CI, 2.1-6.3), Society of Thoracic Surgeons morbidity category 4-5 (odds ratio, 1.2; 95% CI, 1.03-1.5), cannulation less than 24 hours after surgery (odds ratio, 1.6; 95% CI, 1.3-1.9), and longer cardiopulmonary bypass time (≥ 282 min [upper quartile]; odds ratio, 1.5; 95% CI, 1.3-1.9).In this large, multicenter analysis, hemorrhagic complications occurred in nearly half of children with heart disease on extracorporeal membrane oxygenation and were associated with a significant mortality risk. Several factors were associated with hemorrhagic complications in cardiac surgical patients including pre-extracorporeal membrane oxygenation mediastinal exploration, greater surgical complexity, early postoperative cannulation, and longer bypass times. Whether these risks can be mitigated by modifying or delaying systemic anticoagulation requires further investigation.

Stroke is a common complication of extracorporeal membrane oxygenation (ECMO), and pediatric cardiac surgical patients may be at higher risk. Epidemiology and risk factors for stroke in these patients are not well characterized.We analyzed pediatric (18 years) cardiac ECMO cases in the Extracorporeal Life Support Organization Registry from 2002 to 2013. Cardiac surgical patients were identified, and procedures were stratified according to The Society of Thoracic Surgeons morbidity categories. The primary outcome was any stroke (hemorrhagic or infarction) identified by neuroimaging. Risk factors were identified through multivariable logistic regression.We analyzed 3,517 cardiac surgical patients; 81% with cyanotic disease, and 57% in high-risk categories from The Society of Thoracic Surgeons (categories 4 and 5). Overall, 12% experienced stroke while receiving ECMO, and those with stroke had greater in-hospital mortality (72% versus 51%; p0.0001). In multivariable analysis, neonatal status (adjusted odds ratio, 1.8; 95% confidence interval, 1.3 to 2.4), lower weight-for-age z score (adjusted odds ratio, 1.1 for each 1-point decrease; 95% confidence interval, 1.04 to 1.25), and longer ECMO duration (upper quartile [≥ 167 hours] adjusted odds ratio, 1.4; 95% confidence interval, 1.1 to 1.8) were independently associated with increased stroke risk, whereas cyanotic disease, The Society of Thoracic Surgeons category, and bypass time were not.This multicenter analysis demonstrates that pediatric cardiac surgical patients on ECMO are at high risk of stroke; younger or underweight patients and those with longer ECMO duration are at greatest risk, independent of procedural complexity. Future study is necessary to determine how anticoagulation or other clinical practices can be modified to reduce stroke incidence.

Background In congenital heart surgery, hospital performance has historically been assessed using widely available administrative data sets. Recent studies have demonstrated inaccuracies in case ascertainment (coding and inclusion of eligible cases) in administrative versus clinical registry data; however, it is unclear whether this impacts assessment of performance on a hospital level. Methods Merged data from The Society of Thoracic Surgeons (STS) database (clinical registry) and the Pediatric Health Information Systems (PHIS) database (administrative data set) for 46,056 children undergoing cardiac operations (2006–2010) were used to evaluate in-hospital mortality for 33 hospitals based on their administrative versus registry data. Standard methods to identify/classify cases were used: Risk Adjustment in Congenital Heart Surgery, version 1 (RACHS-1) in the administrative data and STS–European Association for Cardiothoracic Surgery (STAT) methodology in the registry. Results Median hospital surgical volume based on the registry data was 269 cases per year; mortality was 2.9%. Hospital volumes and mortality rates based on the administrative data were on average 10.7% and 4.7% lower, respectively, although this varied widely across hospitals. Hospital rankings for mortality based on the administrative versus registry data differed by 5 or more rank positions for 24% of hospitals, with a change in mortality tertile classification (high, middle, or low mortality) for 18% and a change in statistical outlier classification for 12%. Higher volume/complexity hospitals were most impacted. Agency for Healthcare Quality and Research (AHRQ) methods in the administrative data yielded similar results. Conclusions Inaccuracies in case ascertainment in administrative versus clinical registry data can lead to important differences in assessment of hospital mortality rates for congenital heart surgery.

There is an increasing focus on optimizing health care quality and reducing costs. The care of children undergoing heart surgery requires significant investment of resources, and it remains unclear how costs of care relate to quality. We evaluated this relationship across a multicenter cohort.Clinical data from The Society of Thoracic Surgeons Database were merged with cost data from the Pediatric Health Information Systems Database for children undergoing heart surgery (2006 to 2010). Hospital-level costs were modeled using Bayesian hierarchical methods adjusting for case-mix, and hospitals were categorized into cost tertiles. The primary quality metric evaluated was in-hospital mortality.Overall, 27 hospitals (30,670 patients) were included. Median adjusted cost per case was $82,360 and varied fivefold across hospitals, while median adjusted mortality was 3.4% and ranged from 2.4% to 5.0% across hospitals. Overall, hospitals in the lowest cost tertile had significantly lower adjusted mortality rates compared with the middle and high cost tertiles (2.5% vs 3.8% and 3.5%, respectively, both p0.001). When assessed at the individual hospital level, most (75%) but not all hospitals in the lowest cost tertile were also in the lowest mortality tertile. Similar relationships were seen across the spectrum of surgical complexity. Lower cost hospitals also had shorter length of stay and trends toward fewer major complications.Lowest cost hospitals generally deliver the highest quality care for children undergoing heart surgery, although there is some variation in this relationship. This information is important in the design of initiatives aiming to optimize health care value in this population.

Although extracorporeal membrane oxygenation (ECMO) is traditionally used to emergently manage respiratory and/or cardiovascular failure, ECMO can also support cardiorespiratory function in nonemergent settings. Here we present the elective use of ECMO to support a 4.9-kg pediatric patient undergoing a complex percutaneous catheter-based intervention.

Background Infants supported by extracorporeal membrane oxygenation (ECMO) after a Norwood operation face in-hospital mortality rates of 60% to 70%. There are limited data on completion of staged palliation for the subset of patients who survive to hospital discharge. Methods We performed a retrospective case-control study of 64 sequential patients at a single institution supported by ECMO after a Norwood operation. Primary endpoints were survival to hospital discharge, stage II palliation, and stage III palliation. Predictors of non-survival to each endpoint were identified with logistic regression. Survival was compared with a 3:1 era-matched group of control patients who underwent a Norwood operation but did not require ECMO. Results Survival to hospital discharge, stage II palliation, and stage III palliation was 43.8%, 35.9%, and 25.4%, respectively for ECMO cases. Factors independently associated with non-survival to hospital discharge included female gender, ECMO 7 days or greater, and need for renal replacement therapy on ECMO. Non-Caucasian race and ECMO 7 days or greater were independently associated with non-survival to stage II, while non-Caucasian race, lower birth weight, and ECMO 7 days or greater were independently associated with non-survival to stage III. Extracorporeal membrane oxygenation was associated with decreased survival at each endpoint. Patients who survived ECMO had increased interstage mortality between hospital discharge and stage II palliation. Conclusions Extracorporeal membrane oxygenation after a Norwood operation can be life-saving but ultimate survival through staged palliation remains suboptimal. The elevated mortality risk for patients supported by ECMO persists after hospital discharge. Both socioeconomic factors and ECMO-related morbidity may contribute to midterm mortality.

To examine acute and mid-term patient outcomes following the hybrid approach to pulmonary atresia with intact ventricular septum (PA-IVS) compared with the standard surgical approach.A subset of PA-IVS patients with the prospect of biventricular circulation typically undergo surgical or transcatheter right ventricular (RV) outflow tract opening. A recently described hybrid procedure, involving perventricular pulmonary valve perforation, was shown to be safe and effective in single-center series.A single-center retrospective review of all patient with PA-IVS who underwent either surgical or hybrid RV decompression between January 2002 and December 2011 was completed and acute and mid-term patient outcomes were compared between the surgical and hybrid cohorts. Additionally, a systematic literature review was completed to compare a transcatheter cohort to the hybrid cohort.Seven patients with PA-IVS underwent a hybrid procedure; the procedure was technically successful in all attempts, and none required CPB. No patients required surgical re-intervention prior to hospital discharge, and none died during the study period. Surgical RV decompression was performed in 17 patients with a median CPB time of 80 min. Patient outcomes were nearly identical between cohorts. By systematic review, the transcatheter approach has a procedural success of 75-95% but up to 75% of patients require operation in the neonatal period.The hybrid approach is a safe and feasible alternative to the standard surgical and transcatheter approaches to PA-IVS. Acute and mid-term patient outcomes are comparable with those treated with a standard surgical approach and neonatal CPB is completely avoided.

Blood product transfusion during cardiopulmonary bypass has been demonstrated to be associated with increased morbidity and mortality in adult cardiac surgery populations. The aim of this study was to characterize the risk-adjusted occurrence of postoperative complications and mortality in relation to intraoperative blood product transfusion in our pediatric cardiac surgery population.A retrospective review was performed on 1,631 consecutive cardiopulmonary bypass cases to determine the effects of intraoperative blood product transfusion on selected outcomes. After adjusting for patient and operative risk factors, multivariate analysis was performed to determine the association between blood product transfusion and postoperative complications. Cox proportional hazards model was used to examine the relationship of packed red blood cell transfusion to hospital length of stay.Red blood cell and fresh frozen plasma transfusion was associated with pulmonary complications (adjusted odds ratio, 1.55; 95% confidence interval, 1.05 to 2.28; p=0.03). Red blood cell transfusion also correlated with prolonged hospital stay (p0.01). Cryoprecipate transfusion was associated with postoperative pulmonary complications (adjusted odds ratio, 1.79; 95% confidence interval, 1.13 to 2.55; p=0.01), but decreased incidence of 30-day mortality (adjusted odds ratio, 0.44; 95% confidence interval, 0.23 to 0.85; p=0.02). Platelet transfusion was associated with decreased 30-day mortality (adjusted odds ratio, 0.51; 95% confidence interval, 0.28 to 0.93; p=0.04), but not overall mortality.Blood product transfusion was associated with an increased incidence of postoperative pulmonary complications and prolonged hospital length of stay, but not overall mortality. These findings suggest that minimizing blood product transfusion would be beneficial in the pediatric cardiopulmonary bypass surgery patient population.

Objective The optimal timing for neonatal cardiac surgery is unknown. We aimed to determine the relationship between age at surgery and perioperative outcomes, hypothesizing that earlier intervention would be associated with lower morbidity and mortality. Methods A retrospective review was performed of neonates who had undergone an arterial switch operation, stage 1 palliation for functional single ventricle, or systemic-to-pulmonary shunt for obstructed pulmonary blood flow from January 1, 2005, to December 31, 2010. The subjects with clinical indications for delayed surgery or prematurity were excluded. Age at surgery was evaluated as both a continuous and a categorical variable. The primary outcome was a composite endpoint of mortality or prolonged intensive care stay. Results Of 344 subjects, 286 (77 arterial switch operation, 124 stage 1 palliation, 85 systemic-to-pulmonary shunt) met the inclusion criteria. In each group, age at surgery was not associated with the primary composite endpoint. The patients who died after systemic-to-pulmonary shunt had a median age at surgery of 3 days versus 6 days for the survivors ( P = .04). A similar, but nonsignificant, pattern was seen for patients undergoing arterial switch operations (4.5 vs 7 days; P = .09). Earlier surgery was not associated with a reduced duration of vasoactive support, mechanical ventilation, or intensive care unit length of stay in any group. Stage 1 palliation subjects in the upper age quartile (≥8 days) at surgery were less likely to require prolonged mechanical ventilation ( P = .03). Conclusions Younger age at intervention in the neonatal period was not associated with reduced morbidity or mortality in any procedural subgroup studied. In our cohort, earlier systemic-to-pulmonary shunt for obstructed pulmonary blood flow was associated with a greater likelihood of a poor outcome.

Background Patients requiring extracorporeal membrane oxygenation (ECMO) support after a Norwood operation constitute an extremely high-risk group. Data regarding risk factors for the requirement for ECMO post-Norwood are limited, however. We retrospectively assessed risk factors for requiring ECMO support after a Norwood operation during a 10-year period in a high-volume center. Methods Retrospective case-control study of 64 consecutive patients requiring ECMO support after a Norwood operation at a single institution during a 10-year period (January 2001-December 2010), with a 3:1 era-matched control group of patients who underwent a Norwood but did not require ECMO. Results In univariate analysis, ascending aorta less than 2.0 mm, longer cardiopulmonary bypass (CPB) time, intraoperative shunt revision, and right ventricle to pulmonary artery conduit were associated with the need for postoperative ECMO. A single left ventricle was protective compared with single right ventricle anatomy. By multivariate logistic regression, birth weight less than 2.5 kg and longer CPB time were independently associated with the need for postoperative ECMO. Receiver-operating characteristic curve analysis identified a peak lactate of 9 mmol/L and a peak vasoactive inotrope score (VIS) of 27 within 48 hours of surgery as most prognostic of the need for ECMO. Conclusions Birth weight less than 2.5 kg and longer CPB time are independently associated with the need for ECMO after a Norwood operation. Peak serum lactate and peak VIS may be useful in stratifying risk for ECMO. Risk factors for ECMO post-Norwood appear to be similar to the risk factors for early mortality post-Norwood.