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Background: Postoperative infections contribute substantially to morbidity and mortality after congenital heart disease surgery and are often preventable. We sought to identify risk factors for postoperative infection and the impact on outcomes after congenital heart surgery, using data from the International Quality Improvement Collaborative for Congenital Heart Surgery in Developing World Countries.Methods and Results: Pediatric cardiac surgical cases performed between 2010 and 2012 at 27 participating sites in 16 developing countries were included. Key variables were audited during site visits. Demographics, preoperative, procedural, surgical complexity, and outcome data were analyzed. Univariate and multivariable logistic regression were used to identify risk factors for infection, including bacterial sepsis and surgical site infection, and other clinical outcomes. Standardized infection ratios were computed to track progress over time. Of 14 545 cases, 793 (5.5%) had bacterial sepsis and 306 (2.1%) had surgical site infection. In-hospital mortality was significantly higher among cases with infection than among those without infection (16.7% versus 5.3%; P<0.001), as were postoperative ventilation duration (80 versus 14 hours; P<0.001) and intensive care unit stay (216 versus 68 hours; P<0.001). Younger age at surgery, higher surgical complexity, lower oxygen saturation, and major medical illness were independent risk factors for infection. The overall standardized infection ratio was 0.65 (95% confidence interval, 0.58-0.73) in 2011 and 0.59 (95% confidence interval, 0.54-0.64) in 2012, compared with that in 2010.Conclusions: Postoperative infections contribute to mortality and morbidity after congenital heart surgery. Younger, more complex patients are at particular risk. Quality improvement targeted at infection risk may reduce morbidity and mortality in the developing world. [ABSTRACT FROM AUTHOR]

Objective We pursued a multimodality approach to the treatment of patients with pulmonary vein stenosis, incorporating sutureless surgical repair, catheter interventions, and adjunctive chemotherapy. We report our outcomes after surgery. Methods Between January 2007 and August 2013, 49 patients with multivessel pulmonary vein stenosis underwent operations at our institution. We retrospectively reviewed data from a pulmonary vein stenosis registry and the medical records. Results At the time of the index operation, the median patient age was 6 months (range, 32 days-48 months) and weight was 4.9 kg (range, 2.1-13.4 kg). Fourteen patients (28%) died during the follow-up period (median follow-up was 0.5 years [range, 0.04-4.9 years]). There were 2 deaths (4%) within 30 days. Age at repair <6 months, weight at repair <3 kg, and a preoperative right ventricular systolic pressure < ¾ systemic were found to be associated with mortality. One patient required repeat operation for recurrent stenosis. Thirty-nine patients (80%) underwent postoperative catheterizations. The median number of catheterizations per patient was 2 (range, 0-14). Twenty-nine patients (59%) underwent catheterizations with pulmonary vein intervention. The median number of catheterizations with intervention per patient was 1 (range, 0-14). There were no identifiable associations with need for or number of catheterizations with intervention. Ten patients were listed for lung transplantation: 4 patients were de-listed, 3 patients died waiting, and 3 patients underwent transplant. Conclusions Using a multimodality approach, we observed acceptable early survival after operation in patients with pulmonary vein stenosis, despite the need for catheter reinterventions. Lung transplantation remains a viable option. [ABSTRACT FROM AUTHOR]

The study objective was to evaluate outcomes of pulmonary vein stenosis repair in a large single-center cohort. Clinical data from a pulmonary vein stenosis registry were retrospectively reviewed identifying patients who underwent pulmonary vein stenosis repair. The primary/index operation was defined as the patient's first pulmonary vein stenosis operation during the study period. Between January 2007 and August 2019, 174 patients underwent pulmonary vein stenosis repair. Bilateral pulmonary vein stenosis occurred in 111 patients (64%); 71 patients (41%) had 4-vessel disease. Fifty-nine patients (34%) had primary pulmonary vein stenosis. Median age was 9 months (interquartile range, 5-27) and weight was 6.5 kg (4.7-10.2). Surgical techniques evolved and included ostial resection, unroofing, reimplantation, sutureless, modified sutureless, and a newer anatomically focused approach of pulmonary vein stenosis resection with lateralization or patch enlargement of the pulmonary vein–left atrium connection. Twenty-three patients (13%) required reoperation. Cumulative 2-year incidence of postoperative transcatheter intervention (balloon dilation ± stenting) was 64%. One-, 2-, and 5-year survivals were 71.2%, 66.8%, and 60.6%, respectively. There was no association between surgery type and reoperation rate (hazard ratio, 2.38, P =. 25) or transcatheter intervention (hazard ratio, 0.97, P =. 95). The anatomically focused repair was associated with decreased mortality on univariate (hazard ratio, 0.38, P =. 042) and multivariable analyses (hazard ratio, 0.19, P =. 014). Antiproliferative chemotherapy was also associated with decreased mortality (hazard ratio, 0.47, P =. 026). This large single-center surgical pulmonary vein stenosis experience demonstrates encouraging midterm results. A new anatomically focused repair strategy aims to alleviate pulmonary vein angulation to minimize turbulence and shows promising early outcomes. Continued follow-up is required to understand longer-term outcomes for this surgical approach. The PVs can take a long, angulated course as they pass from the lung parenchyma to the LA. This angulated course appears to occur due to the fulcrum effect of structures adjacent to the PVs (ie, descending aorta, airway, pulmonary artery, and pericardial reflection). The anatomically focused repair strategy aims to target this long, angulated PV course, which may be the nidus for turbulent blood flow and subsequent PVS. This repair involves extensive takedown of the pericardial reflection, resection of stenosing/scar tissue, and lateralizing/patch augmenting the PV–LA connection with the goal of creating a shorter and straighter pulmonary venous course. Early follow-up suggests improved survival with the anatomically focused repair strategy compared with conventional repair techniques (ie, sutureless and modified sutureless repair). [Display omitted] [ABSTRACT FROM AUTHOR]

Background: Surgical complexity and related morbidities may affect long‐term patient quality of life (QOL). Aristotle Basic Complexity (ABC) score and Risk Adjustment in Congenital Heart Surgery (RACHS‐1) category stratify the complexity of pediatric cardiac operations. The purpose of this study was to examine the relationship between surgical complexity and QOL and to investigate other demographic and clinical variables that might explain variation in QOL in pediatric cardiac surgical survivors. Methods and Results: Pediatric Cardiac Quality of Life (PCQLI) study participants who had undergone cardiac surgery were included. The PCQLI database provided sample characteristics and QOL scores. Surgical complexity was defined by the highest ABC raw score or RACHS‐1 category. Relationships among surgical complexity and demographic, clinical, and QOL variables were assessed using ordinary least squares regression. A total of 1416 patient–parent pairs were included. Although higher ABC scores and RACHS‐1 categories were associated with lower QOL scores (P<0.005), correlation with QOL scores was poor to fair (r=−0.10 to −0.29) for all groups. Ordinary least squares regression showed weak association with R 2=0.06 to R 2=0.28. After accounting for single‐ventricle anatomy, number of doctor visits, and time since last hospitalization, surgical complexity scores added no additional explanation to the variance in QOL scores. Conclusions: ABC scores and RACHS‐1 categories are useful tools for morbidity and mortality predictions prior to cardiac surgery and quality of care initiatives but are minimally helpful in predicting a child's or adolescent's long‐term QOL scores. Further studies are warranted to determine other predictors of QOL variation.

Objectives: This study sought to develop a method to adjust for case mix complexity in catheterization for congenital heart disease to allow equitable comparisons of adverse event (AE) rates. Background: The C3PO (Congenital Cardiac Catheterization Project on Outcomes) has been prospectively collecting data using a Web-based data entry tool on all catheterization cases at 8 pediatric institutions since 2007. Methods: A multivariable logistic regression model with high-severity AE outcome was built using a random sample of 75% of cases in the multicenter cohort; the models were assessed in the remaining 25%. Model discrimination was assessed by the C-statistic and calibration with Hosmer-Lemeshow test. The final models were used to calculate standardized AE ratios. Results: Between August 2007 and December 2009, 9,362 cases were recorded at 8 pediatric institutions of which high-severity events occurred in 454 cases (5%). Assessment of empirical data yielded 4 independent indicators of hemodynamic vulnerability. Final multivariable models included procedure type risk category (odds ratios [OR] for category: 2 = 2.4, 3 = 4.9, 4 = 7.6, all p < 0.001), number of hemodynamic indicators (OR for 1 indicator = 1.5, ≥2 = 1.8, p = 0.005 and p < 0.001), and age <1 year (OR: 1.3, p = 0.04), C-statistic 0.737, and Hosmer-Lemeshow test p = 0.74. Models performed well in the validation dataset, C-statistic 0.734. Institutional event rates ranged from 1.91% to 7.37% and standardized AE ratios ranged from 0.61 to 1.41. Conclusions: Using CHARM (Catheterization for Congenital Heart Disease Adjustment for Risk Method) to adjust for case mix complexity should allow comparisons of AE among institutions performing catheterization for congenital heart disease. [ABSTRACT FROM AUTHOR]

Introduction: The purpose of this study was to describe the cost burden of congenital heart disease (CHD) and the associated social impact as experienced by families. Method: Qualitative methods were used to collect and interpret data. Semi-structured interviews were conducted with parents of children with various degrees of CHD complexity and socioeconomic status currently admitted for congenital heart surgery at a large tertiary care regional center. Results: The meaning of cost burden as defined by participants resulted in the emergence of two major categories, lifestyle change and uncertainty. Cost was described beyond monetary terms and as a result, data in each category were further clustered into three underlying subcategories labeled financial, emotional, and family burden. The child's disease complexity and parent's socioeconomic status seem to be linked to higher levels of stress experienced in terms of finances, emotional drain, and family member burden. Prenatal diagnosis was noted to trigger early discussion of financial uncertainty, often resulting in altered personal spending prior to birth. Discussion: The cost experienced by parents of children with complex CHD was described as both life-changing and uncertain. Informing families of these types of additional stressors may allow issues of finances to be considered early in the overall preparation of caring for a child with complex CHD. [Copyright &y& Elsevier]

Tremendous advances have occurred in catheter-based interventions for congenital heart disease. Multicenter trials of these advances are either out of date or have been limited in scope. As such little is known on the application of these techniques in the current era. The IMPACT Registry™ (IMproving Pediatric and Adult Congenital Treatments) will allow us to measure variability in the performance and outcomes of both diagnostic and interventional cardiac catheterization procedures in all children and adults with congenital heart disease. The IMPACT Registry™ will be harmonized with the Society of Thoracic Surgeons Congenital Heart Disease Database, thereby allowing us to compare catheter-based interventions with surgical interventions when appropriate. The initial release of the registry will only include hospital-based outcomes, but ultimately it will transition to a longitudinal registry. The IMPACT Registry™ will provide the necessary benchmark tools for quality improvement activities for cardiac catheterization procedures in congenital heart disease. [Copyright &y& Elsevier]

Objective: No method of measuring technical performance exists for the stage I Norwood procedure. Hospital mortality is usually used as a surrogate for technical performance, but evidence is lacking to support this concept. A technical score was designed by expert consensus. Methods: The technical score included the following steps: (1) Stage I was divided into subprocedures according to anatomic areas where an intervention is performed. (2) For each subprocedure, three score categories (optimal, adequate, and inadequate) were defined on the basis of echocardiography, catheterization, and/or clinical data. (3) Subprocedures were analyzed for the whole group and by surgeon. (4) Overall repair was also scored: optimal if all attempted subprocedures were optimal, inadequate if any was inadequate, and adequate for everything in between. (5) All patients undergoing the stage I procedure from January 2004 to December 2006 were retrospectively studied. Results: One hundred ten patients were included (operated on by six surgeons), and 4 were excluded for lack of reliable postoperative data. Most subprocedures were scored as optimal. Subprocedures with the largest inadequate scores were distal arch reconstruction in 7 (6%) patients and aortopulmonary shunt in 3 (5%). No statistical differences were found among surgeons either by subprocedure or by overall outcome, although individual sample sizes were small. The overall score correlated with length of stay, extracorporeal membrane oxygenator support, and hospital mortality. Conclusions: Technical performance can be measured after the stage I procedure, and performance score correlates with early outcome. This score may also be useful as a self-assessment tool. [Copyright &y& Elsevier]

Background: Although adequacy of repair after congenital heart surgery is a crucial determinant of clinical outcome, there is no current method of assessment. We sought to develop a process to measure the adequacy of repair for a diverse group of congenital heart procedures. Methods: Selected surgical procedures, consisting of repair of ventricular septal defect (VSD), tetralogy of Fallot (TOF), complete common atrioventricular canal (CAVC), and arterial switch operation, were divided into component subprocedures, each of which was assessed separately. Three outcome categories of “optimal,” “adequate,” and “inadequate” were defined by consensus according to postprocedure echocardiographic assessment. Outcome categories for conduction disturbance were also created. All patients undergoing one of the four procedures in 2004 were identified, and each subprocedure was assessed. Other clinical data were obtained from medical records. Repairs were scored as “optimal” if all attempted subprocedures and conduction were optimal, and “inadequate” if any was inadequate. Results: A total of 138 procedures were included. VSD repair was done in 46 patients (33%), TOF repair in 33 (24%), arterial switch operation in 36 (26%), and CAVC repair in 23 (17%). Optimal technical score was found in 28 (20%), adequate in 106 (77%), and inadequate in 4 (3%) (2 VSD, 1 TOF, 1 CAVC). Median length of stay was 8 days, and no patients died. Conclusions: Despite procedural diversity and complexity, technical adequacy of repair can be assessed for congenital heart surgery. [Copyright &y& Elsevier]

There is widespread recognition that surgical outcomes differ by surgeon and institution; however, the definition and measurement of quality in pediatric cardiac surgery is in its infancy. This article discusses the definition of quality, what has been done to define and measure quality of pediatric cardiac surgery, and how to proceed. Descriptions of assessment of quality by evaluating structure, process, and outcome measures; efforts to establish a global congenital heart surgery database; and a comparison of risk-adjusted mortality rates using the Risk Adjustment for Congenital Heart Surgery method are included. [Copyright &y& Elsevier]

Objective: To evaluate the use of imatinib mesylate with or without bevacizumab targeting neoproliferative myofibroblast-like cells with tyrosine kinase receptor expression, as adjuncts to modern interventional therapies for the treatment of multivessel intraluminal pulmonary vein stenosis (PVS). We describe the 48- and 72-week outcomes among patients receiving imatinib mesylate with or without bevacizumab for multivessel intraluminal PVS.Study Design: This single-arm, prospective, open-label US Food and Drug Administration approved trial enrolled patients with ≥2 affected pulmonary veins after surgical or catheter-based relief of obstruction between March 2009 and December 2014. Drug therapy was discontinued at 48 weeks, or after 24 weeks of stabilization, whichever occurred later.Results: Among 48 enrolled patients, 5 had isolated PVS, 26 congenital heart disease, 5 lung disease, and 12 both. After the 72-week follow-up, 16 patients had stabilized, 27 had recurred locally without stabilization, and 5 had progressed. Stabilization was associated with the absence of lung disease (P = .03), a higher percentage of eligible drug doses received (P = .03), and was not associated with age, diagnosis, disease laterality, or number of veins involved. Survival to 72 weeks was 77% (37 of 48). Adverse events were common (n = 1489 total), but only 16 were definitely related to drug treatment, none of which were serious.Conclusion: Survival to 72 weeks was 77% in a referral population with multivessel intraluminal PVS undergoing multimodal treatment, including antiproliferative tyrosine kinase blockade. Toxicity specific to tyrosine kinase blockade was minimal. [ABSTRACT FROM AUTHOR]