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Ventricular pacing can cause myocardial dysfunction, but how lead anchoring to the myocardium affects function has not been studied. The purpose of this study was to evaluate patterns of regional and global ventricular function in patients with a ventricular lead using cine cardiac computed tomography (CCT) and histology. This was a single-center retrospective study with 2 groups of patients with a ventricular lead: (1) those who underwent cine CCT from September 2020 to June 2021 and (2) those whose cardiac specimen was analyzed histologically. Regional wall motion abnormalities on CCT were assessed in relation to lead characteristics. For the CCT group, 122 ventricular lead insertion sites were analyzed in 43 patients (47% female; median age 19 years; range 3–57 years). Regional wall motion abnormalities were present at 51 of 122 lead insertion sites (42%) in 23 of 43 patients (53%). The prevalence of a lead insertion–associated regional wall motion abnormality was higher with active pacing (55% vs 18%; P <.001). Patients with lead insertion–associated regional wall motion abnormalities had a lower systemic ventricular ejection fraction (median 38% vs 53%; P <.001) than did those without regional wall motion abnormalities. For the histology group, 3 patients with 10 epicardial lead insertion sites were studied. Myocardial compression, fibrosis, and calcifications were commonly present directly under active leads. Lead insertion site–associated regional wall motion abnormalities are common and associated with systemic ventricular dysfunction. Histopathological alterations including myocardial compression, fibrosis, and calcifications beneath active leads may explain this finding. [Display omitted] [ABSTRACT FROM AUTHOR]

Outcomes after total anomalous pulmonary venous connection (TAPVC) repair remain suboptimal due to recurrent pulmonary vein (PV) obstruction requiring reinterventions. We sought to develop a clinical prediction rule for PV reintervention after TAPVC repair. Data from consecutive patients who underwent TAPVC repair at a single institution from January 1980 to January 2020 were retrospectively reviewed after Institutional Review Board approval. The primary outcome was postdischarge (late) unplanned PV surgical or transcatheter reintervention. Echocardiographic criteria were used to assess PV residual lesion severity at discharge (class 1: no residua; class 2: minor residua; class 3: major residua). Competing risk models were used to develop a weighted risk score for late reintervention. Of 437 patients who met entry criteria, there were 81 (18.5%) reinterventions at a median follow-up of 15.6 (interquartile range, 5.5-22.2) years. On univariable analysis, minor and major PV residua, age, single-ventricle physiology, infracardiac and mixed TAPVC, and preoperative obstruction were associated with late reintervention (all P <.05). The final risk prediction model included PV residua (class 2: subdistribution hazard ratio [SHR], 4.8; 95% CI, 2.8-8.1; P <.001; class 3: SHR, 6.4; 95% CI, 3.5-11.7; P <.001), age <1 year (SHR, 3.3; 95% CI, 1.3-8.5; P =.014), and preoperative obstruction (SHR, 1.8; 95% CI, 1.1-2.8; P =.015). A risk score comprising PV residua (class 2 or 3: 3 points), age (neonate or infant: 2 points), and obstruction (1 point) was formulated. Higher risk scores were significantly associated with worse freedom from reintervention (P <.001). A risk prediction model of late reintervention may guide prognostication of high-risk patients after TAPVC repair. [Display omitted] [ABSTRACT FROM AUTHOR]

The natural history of the dilated truncal root in repaired truncus arteriosus (TA) is incompletely understood. A single-center review of patients who underwent TA repair between January 1984 and December 2018 was performed. Echocardiographically determined root diameters and derived z scores were measured at the annulus, sinus of Valsalva (SoV), and sinutubular junction (STJ) immediately before TA repair and throughout follow-up. Linear mixed-effects models assessed trends in root dimensions over time. Of 193 patients who underwent TA repair at a median age of 12 days (interquartile range, 6-48 days) and survived to discharge, 34 (17.6%), 110 (57.0%), and 49 (25.4%) patients had bicuspid, tricuspid, and quadricuspid truncal valves, respectively. Median postoperative follow-up was 11.6 years (interquartile range, 4.4-22.0 years; range, 0.1-34.8 years). Truncal valve or root intervention was required in 38 patients (19.7%). The mean rates of annular, SoV, and STJ growth were 0.7 ± 0.3 mm/y, 0.8 ± 0.5 mm/y, and 0.9 ± 0.4 mm/y, respectively. Root z scores remained stable with time. At baseline, compared with patients with tricuspid leaflet anatomy, bicuspid patients had larger diameters at the SoV (P =.003) and STJ (P =.029), whereas quadricuspid patients had larger STJ diameters (P =.004). Over time, the bicuspid and quadricuspid cohorts demonstrated comparatively greater annular dilatation (both P <.05). Patients with ≥75th percentile root growth rates had a higher incidence of moderate-severe truncal regurgitation (P =.019) and truncal valve intervention (P =.002). Root dilatation in TA persisted for up to 30 years after primary repair. Patients with bicuspid and quadricuspid truncal valves demonstrated greater root dilatation over time and required more valve interventions. Continued longitudinal follow-up is warranted in this higher-risk cohort. [Display omitted] [ABSTRACT FROM AUTHOR]

Early postoperative catheterizations (EPOCs) within 6 weeks after a congenital heart surgical procedure can treat residual lesions and provide important clinical information. However, EPOCs are often assumed to impose additional risk on a vulnerable patient population. This study aimed to describe the EPOC population, evaluate procedural safety, compare EPOC patients with procedure-matched non-EPOC patients, and determine risk factors for poor outcomes using data from the Congenital Cardiac Catheterization Project on Outcomes registry. In a retrospective cohort, demographic, clinical, and procedural characteristics were analyzed for diagnostic and interventional catheterizations performed in 13 participating institutions from January 2014 to December 2017, excluding patients after heart transplant. The primary outcome was a high-severity adverse event (AE). Three distinct analyses included (1) describing the full cohort and EPOC patients, (2) comparing EPOC patients with and without a high-severity AE, and (3) comparing EPOC patients with controls matched on case type. This study included 17,776 catheterizations, with 1399 EPOCs. The high-severity AE rate was 6.4% overall, 8.9% in the EPOC cohort, and 8.4% in matched controls (P =.74). The association between EPOC status and high-severity AE was not significant in a multivariable model (P =.17). In EPOCs with a high-severity AE, median procedure duration was 30 minutes longer (P <.001), and median time from surgical procedure to catheterization was 3 days longer (P =.05). EPOC was not associated with additional risk. Individual patient characteristics of size and hemodynamic vulnerability may serve as informative predictors. Timely catheterization may preempt further clinical deterioration, and intraprocedure duration optimization may correlate with improved outcomes. [Display omitted] [ABSTRACT FROM AUTHOR]

Background: Coarctation of the aorta (COA) is associated with reduced aortic distensibility and systemic hypertension (HTN). 60–85% of COA patients have a bicuspid aortic valve (BAV). It is not known if the presence of a BAV accentuates the aortopathy and HTN in CoA patients. We examined whether patients with COA and a BAV had lower aortic distensibility by CMR, and a higher prevalence of systemic HTN compared with COA patients with a tricuspid aortic valve (TAV). Methods: In successfully repaired COA patients excluding those with residual COA, ascending aorta (AAO) and descending aorta (DAO) distensibility was calculated by CMR. HTN was assessed using standard pediatric and adult criteria. Results: Among 215 COA patients (median age 25.3 years), 67% had a BAV, and 33% had a TAV. Median AAO distensibility z-score was lower in the BAV group (− 1.2 versus − 0.7; p = 0.014) but DAO distensibility was similar in BAV and TAV patients. HTN prevalence was similar in BAV (32%) and TAV groups (36%, p = 0.56). On multivariable analysis controlling for confounders, HTN was not associated with BAV but was associated with male gender (p = 0.003) and older age at follow-up (p = 0.004). Conclusions: In young adults with treated COA, those with a BAV had stiffer AAO compared to those with a TAV, but DAO stiffness was similar. HTN was not related to BAV. These results suggest that although the presence of a BAV in COA exacerbates the AAO aortopathy, it does not exacerbate the more generalized vascular dysfunction and associated HTN. [ABSTRACT FROM AUTHOR]

The study objectives were to report on a growing experience of conduction system mapping during complex congenital heart surgery and create a predictive model of conduction anatomy. Patients undergoing complex cardiac repair with conduction mapping were studied. Intraoperative mapping used a multielectrode catheter to collect His bundle electrograms in the open, decompressed, beating heart. Patient anatomy, operative details, His bundle location, and postoperative conduction status were analyzed. By using classification and regression tree analysis, a predictive model of conduction location was created. A total of 109 patients underwent mapping. Median age and weight were 1.8 years (range, 0.2-14.9) and 10.8 kg (range, 3.5-50.4), respectively. Conduction was identified in 96% (105/109). Median mapping time was 6 minutes (range, 2-33). Anatomy included atrioventricular canal defect, double outlet right ventricle, complex transposition of the great arteries, and multiple ventricular septal defects. By classification and regression tree analysis, ventricular looping and visceroatrial situs were the greatest discriminators of conduction location. A total of 94 of 105 patients (89.5%) were free of complete heart block. Only 1 patient (2.9%) with heterotaxy syndrome developed complete heart block. The precise anatomic location of the conduction system in patients with complex congenital heart defects can be difficult for the surgeon to accurately predict. Intraoperative conduction mapping enables localization of the His bundle and adds to our understanding of the anatomic factors associated with conduction location. Predictive modeling of conduction anatomy may build on what is already known about the conduction system and help surgeons to better anticipate conduction location preoperatively and intraoperatively. [Display omitted] [ABSTRACT FROM AUTHOR]

Outcomes after first-stage palliation of single-ventricle heart disease are influenced by many factors, including the presence of residual lesions requiring reintervention. However, there is a dearth of information regarding the optimal timing of reintervention. We assessed if earlier reintervention would be favorably associated with in-hospital outcomes among patients requiring unplanned reinterventions after the Norwood operation. This was a single-center, retrospective review of all patients who underwent the Norwood procedure from January 1997 to November 2017 and required a predischarge unplanned surgical or transcatheter reintervention on 1 or more subcomponent areas repaired at the index operation. Outcomes of interest included in-hospital mortality or transplant, postoperative hospital length of stay, and inpatient cost. Associations between timing of reintervention and outcomes were assessed using logistic regression (mortality or transplant) or generalized linear models (postoperative hospital length of stay and cost), adjusting for baseline patient-related and procedural factors. Of 500 patients who underwent the Norwood operation, 92 (18.4%) required an unplanned reintervention. Median time to reintervention was 12 days (interquartile range, 5-35 days). There were 31 (33.7%) deaths or transplants, median postoperative hospital length of stay was 49 days (interquartile range, 32-87 days), and median cost was $328,000 (interquartile range, $204,000-$464,000). On multivariable analysis, each 5-day increase in time to reintervention increased the odds of mortality or transplant by 20% (odds ratio, 1.2; 95% confidence interval, 1.1-1.3; P =.004). Longer time to reintervention was also significantly associated with greater postoperative hospital length of stay (P <.001) and higher cost (P <.001). For patients requiring predischarge unplanned reinterventions after the Norwood operation, earlier reintervention is associated with improved in-hospital transplant-free survival and resource use. [Display omitted] [ABSTRACT FROM AUTHOR]

The utility of the intraoperative technical performance score (IO-TPS) in predicting outcomes after congenital cardiac surgery remains unknown. Data from patients undergoing surgery for congenital heart disease from January 2011 to December 2019 at a single institution were retrospectively reviewed. Intraoperative echocardiograms were used to assign IO-TPS for each index operation (class 1, no residua; class 2, minor residua; class 3, major residua). The primary outcome was a composite of in-hospital mortality, transplant, unplanned reintervention in the anatomic area of repair, and new permanent pacemaker implantation. Secondary outcomes included postdischarge (late) mortality or transplant and unplanned reintervention. Associations between IO-TPS and outcomes were assessed using logistic (primary) and Cox or competing risk (secondary) models, adjusting for preoperative patient- and procedure-related covariates. The primary outcome was observed in 784 (11.5%) of 6793 patients who met entry criteria. On multivariable analysis, IO-TPS was a significant predictor of the primary outcome (class 2: odds ratio, 1.7 [95% CI, 1.4-2.0; P <.001]; class 3: odds ratio, 6.0 [95% CI, 4.0-8.9; P <.001]). Among 6661 transplant-free survivors of hospital discharge observed for up to 10.5 years, there were 185 (2.8%) deaths or transplants and 1171 (17.6%) reinterventions. Class 3 patients had a greater adjusted risk of late mortality or transplant (hazard ratio, 2.2; 95% CI, 1.2-4.2; P =.012) and late reintervention (subdistribution hazard ratio, 2.5; 95% CI, 1.8-3.3; P <.001) vs class 1 patients. IO-TPS is significantly associated with adverse early and late outcomes after congenital heart surgery and may serve as an important adjunct for self-assessment and quality improvement. [Display omitted] [ABSTRACT FROM AUTHOR]

While the predischarge technical performance score (DC-TPS) is significantly associated with outcomes after congenital cardiac surgery, the utility of the intraoperative TPS (IO-TPS) remains unknown. This was a single-center retrospective review of consecutive patients who underwent congenital cardiac surgery from January 2011 to December 2019. Intraoperative and predischarge echocardiograms were used to assign IO-TPS and DC-TPS, respectively, for each index operation (class 1, no residua; class 2, minor residua; class 3, major residua). Anatomic modules identifying the principal residual lesion were assigned to all class 2/3 patients. Overall and module-specific TPS comparisons were made. Multivariable regression models with IO-TPS and DC-TPS as separate predictors of postoperative outcomes were compared. Of 6201 patients, overall agreement between IO-TPS and DC-TPS was observed in 4251 patients (68.6%); scores were likelier to be worse at discharge (P <.001). Paired comparative analyses revealed that among patients with at least class 2 atrioventricular and semilunar valve residua, IO-TPS was likelier to worsen than improve (both P <.001). Class 3 patients had a higher risk of in-hospital/early mortality (IO-TPS: odds ratio, 7.5; 95% CI, 2.4-23; DC-TPS: odds ratio, 6.6; 95% CI, 3.0-15), postdischarge/late mortality (IO-TPS: hazard ratio [HR], 3.1, 95% CI, 1.3-7.1; DC-TPS: HR, 2.3; 95% CI, 1.2-4.4), and late unplanned reintervention (IO-TPS: HR, 2.8; 95% CI, 1.9-4.0; DC-TPS: HR, 3.4; 95% CI, 2.8-4.2) vs class 1 (all P <.05). IO- and DC-TPS models were equivalent fits for predicting early and late mortality; the latter was a marginally better fit for late reintervention. IO-TPS and DC-TPS are both important adjuncts for quality improvement in congenital cardiac surgery. [Display omitted] [ABSTRACT FROM AUTHOR]

Background: Unplanned catheter-based or surgical reinterventions after congenital heart operations are independently associated with operative mortality and increased postoperative length of stay.Objectives: This study assessed the long-term outcomes of transplant-free survivors of hospital discharge requiring predischarge reinterventions after congenital cardiac surgery.Methods: Data from patients who required predischarge reinterventions in the anatomic area of repair after congenital cardiac surgery and survived to hospital discharge at a quaternary referral center from January 2011 to December 2019 were retrospectively reviewed. Previously published echocardiographic criteria were used to assess the severity of persistent residual lesions at discharge (Grade 1, no residua; Grade 2, minor residua; and Grade 3, major residua). Outcomes included postdischarge (late) mortality or transplant and unplanned reintervention. Associations between predischarge residual lesion severity and outcomes were assessed by using Cox or competing risk models, adjusting for baseline patient characteristics, case complexity, and preoperative risk factors.Results: Among the 408 patients who met entry criteria, there were 58 (14.2%) postdischarge deaths or transplants and 208 (51.0%) late reinterventions at a median follow-up of 3.0 years (IQR: 1.1-6.8 years). Greater predischarge residual lesion severity was associated with worse transplant-free survival and freedom from reintervention (both, P < 0.05). On multivariable analyses, Grade 3 patients had an increased risk of postdischarge mortality or transplant (HR: 4.8; 95% CI: 2.0-11; P < 0.001) and late reintervention (subdistribution HR: 2.1; 95% CI: 1.4-3.1; P < 0.001) vs Grade 1 patients.Conclusions: Among transplant-free survivors requiring predischarge reinterventions after congenital cardiac surgery, those with persistent major residua have significantly worse long-term outcomes. These high-risk patients warrant closer surveillance. [ABSTRACT FROM AUTHOR]

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.ahj.2007.07.037 Byline: Aphrodite Tzifa (a)(b)(c), Kimberlee Gauvreau (a)(b), Robert L. Geggel (a) Abstract: Clinical practice is discrepant regarding routine enlargement of the interatrial communication (IAC) in patients with right-sided atrioventricular valve atresia. We determined the percentage and risk factors of those who develop a restrictive IAC. Author Affiliation: (a) Department of Cardiology, Children's Hospital, Harvard Medical School, Boston, MA (b) Department of Pediatrics, Harvard Medical School, Boston, MA (c) Department of Congenital Heart Disease, Evelina Children's Hospital, Guy's & St Thomas' NHS Trust, London, United Kingdom Article History: Received 11 April 2007; Accepted 31 July 2007

We sought to simplify reporting of outcomes in congenital heart surgery that compares well-defined patient groups and accommodates multiple stakeholder needs while being easily understandable. We selected 19 commonly performed congenital heart surgeries ranging in complexity from repair of atrial septal defects to the Norwood procedure. Strict inclusion/exclusion criteria ensured the creation of 19 well-defined diagnosis/procedure cohorts. Preoperative, procedural, and postoperative data were collected for consecutive eligible patients from 9 centers between January 1, 2016, and December 31, 2021. Unadjusted operative mortality rates and hospital length of stay for each of the 19 diagnosis/procedure cohorts were summarized in aggregate and stratified by each center. Of 8572 eligible cases included, numbers in the 19 diagnosis/procedure cohorts ranged from 73 for tetralogy of Fallot repair after previous palliation to 1224 for ventricular septal defect (VSD) repair for isolated VSD. In aggregate, the unadjusted mortality ranged from 0% for atrial septal defect repair to 28.4% for hybrid stage I. There was significant heterogeneity in case mix and mortality for different diagnosis/procedure cohorts across centers (eg, arterial switch operation/VSD, n = 7-42, mortality 0%-7.4%; Norwood procedure, n = 16-122, mortality 5.3%-25%). Reporting of institutional case volumes and outcomes within well-defined diagnosis/procedure cohorts can enable centers to benchmark outcomes, understand trends in mortality, and direct quality improvement. When made public, this type of report could provide parents with information on institutional volumes and outcomes and allow them to better understand the experience of each program with operations for specific congenital heart defects. Simplifying outcomes reporting for congenital heart surgery. [Display omitted] [ABSTRACT FROM AUTHOR]

American Heart Association (AHA) guidelines for management of Kawasaki disease (KD) rely on coronary artery (CA) z-scores from echocardiograms. Compared with echocardiography, cardiac CT (CCT) offers better visualization of distal segments and evaluation for thrombosis and stenosis. Despite increasing use of CCT in KD, CA z-scores for CCT are not available and measurement concordance between imaging modalities is a critical knowledge gap. We retrospectively reviewed KD patients with CA aneurysms who had concurrent echocardiography and CCT between 2016 and 2020. Patients were included if they had history of CA z-scores of ≥3 on echocardiography during their clinical course. Agreement between CCT and echocardiography was assessed using Bland-Altman analysis. Paired CCT and echocardiography studies were available in 18 patients (21 studies). The largest CA aneurysms were large/giant (z-score ≥10) in 14 studies, medium (z-score ≥5, <10) in 3 studies, and small (z score ≥2.5, <5) in 2 studies. Intra- and inter-observer reliability for CCT measurements were high for all CA segments (ICC 99.7% and 98.6%). For the LMCA, proximal LAD and proximal and distal RCA there was high correlation between echocardiogram and CCT absolute measurements with wider variation between modalities for the distal LAD and circumflex. Overall, CCT measurements tended to be smaller than echocardiogram measurements, and led to a lower AHA z-score risk classification in 24% of studies. CCT and echocardiography have high agreement for absolute measurements of proximal CA segments, but more measurement discrepancy exists for distal CA segments with bias toward lower dimensions on CCT. [ABSTRACT FROM AUTHOR]

Background: Transvenous permanent pacemaker (PPM) implantation is an available option for Fontan patients with sinus node dysfunction. However, the thrombogenic potential of leads within the Fontan baffle is unknown.Objective: The purpose of this study was to compare the clot burden in Fontan patients with a transvenous atrial PPM to those without a PPM and those with an epicardial PPM.Methods: This was a retrospective cohort study of all transvenous PPM implantations in Fontan patients followed at our institution (2000-2018). We performed frequency matching on Fontan type and age group. Primary outcome was identification of intracardiac clot, pulmonary embolus, or embolic stroke.Results: Of 1920 Fontan patients, 58 patients (median age 23 years; interquartile range [25th-75th percentiles] 14-33) at the time of transvenous PPM implantation and 174 matched subjects formed our cohort. The type of Fontan performed in case subjects was right atrium-pulmonary artery or right atrium-right ventricle conduit (54%), lateral tunnel (43%), and extracardiac (3%). The cumulative incidence of clot was highest in patients with transvenous PPM, followed by patients with epicardial PPM and no PPM (1.2 vs 0.87 vs 0.67 per 100 person-years of follow-up, respectively). In multivariable analysis, anticoagulation and/or antiplatelet therapy were protective against clot and resulted in reduction of clot risk by 3-fold (incidence rate ratio 0.33; 95% confidence interval 0.21-0.53; P <.001).Conclusion: In a large cohort of Fontan patients matched for age and Fontan type, patients with transvenous PPM had a higher but not statistically significant incidence of clot compared to those with no PPM and epicardial PPM. Patients treated with warfarin/aspirin had lower clot risk. [ABSTRACT FROM AUTHOR]

The Technical Performance Score (TPS) can predict outcomes after congenital cardiac surgery. We sought to validate TPS as a predictor of both short- and long-term outcomes of the Norwood procedure. We conducted a retrospective review of patients who underwent the Norwood procedure from 1997 to 2017. We assigned TPS (class 1, no residua; class 2, minor residua; class 3, major residua or reintervention for major residua before discharge) based on subcomponent scores from discharge echocardiograms or unplanned reinterventions, or both. Multivariable Cox or competing risk analysis, adjusted for preoperative patient- and procedure-related covariates, examined the association of TPS with postoperative hospital length of stay, transplant-free survival, and postdischarge reinterventions. Among 500 patients, 319 (64%) were male, 54 (11%) were premature, 56 (11%) had noncardiac anomalies/syndromes, 146 (29%) had preoperative risk factors, and 480 (96%) were assigned TPS. On multivariable analysis, class 3 had greater hazard for reinterventions in transplant-free survivors (class 3: subdistribution hazard ratio [HR], 2.06; 95% confidence interval [CI] 1.34-3.16; P =.001) and was associated with increased hospital length of stay vs class 1 (HR, 0.25; 95% CI, 0.18-0.34; P <.001). Transplant-free survival after Norwood surgery was shorter for both class 2 (HR, 2.48; 95% CI, 1.68-3.66; P <.001) and class 3 (HR, 3.29; 95% CI, 2.18-4.95; P <.001). TPS predicts early and late outcomes after Norwood. Absence of residual lesions results in improved long-term prognosis for single-ventricle patients. TPS may improve outcomes after Norwood by identifying patients warranting closer follow-up and potentially earlier reintervention. [ABSTRACT FROM AUTHOR]

Several factors predict reintervention for subaortic stenosis (SubAS): age, preoperative left ventricular outflow tract gradient, distance from the obstructive subaortic ridge to the aortic valve, and peeling of membrane from the aortic/mitral valves. We sought to develop a prediction rule to categorize risk of reintervention for recurrent SubAS and guide follow-up in patients with discrete SubAS. We retrospectively reviewed patients who underwent SubAS resection between 1984 and 2016. Our primary outcome was reintervention for recurrent SubAS after discharge. Kaplan-Meier estimates were used for time-to-event analysis of any reintervention. Multivariable models were used to create a prediction rule. We excluded patients without 3 years of follow-up. Of 172 patients, 21 (12.2%) required reintervention. The characteristics predicting reintervention were age younger than 2 years (P <.001), preoperative left ventricular outflow tract gradient of 65 mm Hg or more (P =.011), peeling of membrane from the mitral valve (P <.001), distance from the membrane to the aortic valve of less than 5 mm (P <.001), prior complex operation (P =.035), other left-sided heart lesions (P =.008), and aortic annulus z-score of −2.5 or less (P <.001). Our final prediction rule includes age, membrane to aortic valve distance, and other left-sided heart lesions each scored as 1 point. For patients with a score of 1 or less, 4% required a reintervention compared with 34% with a score of 2 or more. A prediction rule that incorporates the patient's age at the index operation, membrane to aortic valve distance, and associated left-sided heart lesions can determine the likelihood of reintervention for recurrent SubAS. [ABSTRACT FROM AUTHOR]

We sought to develop a novel risk prediction model of 1-year mortality after congenital heart surgery that accounts for clinical, anatomic, echocardiographic, and socioeconomic factors. This was a single-center, retrospective review of consecutive index operations for congenital or acquired heart disease, from January 2011 to January 2021, among patients with known survival status at 1 year after discharge from the index hospitalization. The primary outcome was postdischarge mortality at 1 year. Variables of interest included age, prematurity, noncardiac anomalies or syndromes, the Childhood Opportunity Index, primary procedure, major adverse postoperative complications, and the Residual Lesion Score. Logistic regression was used to develop a weighted risk score for the primary outcome. Internal validation using a bootstrap-resampling approach was performed. Of 10,412 consecutive operations for congenital or acquired heart disease, 8808 (84.6%) cases met entry criteria, including survival to discharge. There were 190 (2.2%) deaths at 1 year postdischarge. A weighted risk score was formulated on the basis of the variables in the final risk prediction model, which included all aforementioned risk factors of interest. This model had a C-statistic of 0.82 (95% confidence interval, 0.80-0.85). The median risk score was 6 (interquartile range, 4-8) points. Patients were categorized as low (score 0-5), medium (score 6-10), high (score 11-15), or very high (score 16-20) risk. The expected probability of mortality was 0.4% ± 0.2%, 2.0% ± 1.1%, 10.1% ± 5.0%, and 36.6% ± 9.6% for low-risk, medium-risk, high-risk, and very high-risk patients, respectively. A risk prediction model of 1-year mortality may guide prognostication and follow-up of patients after discharge after surgery for congenital or acquired heart disease. [Display omitted] [ABSTRACT FROM AUTHOR]

Outcomes after the arterial switch operation (ASO) for dextro-transposition of the great arteries have improved significantly since its inception in the 1980s. This study reviews contemporaneous outcomes and predictors for late reinterventions after ASO. We retrospectively reviewed patients who underwent ASO for dextro-transposition of the great arteries from 1997 to 2017. Technical performance score (TPS) class (class 1, trivial or no residua; class 2, minor residua; class 3, major residua or reintervention) was assigned at discharge based on echocardiographic evaluation of components of the ASO. Multivariable Cox regression identified patient- and procedure-specific factors associated with postdischarge reinterventions. Among 598 patients, 410 (69%) underwent ASO and 188 (31%) underwent ASO with ventricular septal defect repair. Median age at surgery was 5 days (interquartile range, 3 to 7); median follow-up time was 8.2 years; 408 (68%) were male; 50 (8.3%) were premature; and 10 (1.7%) had noncardiac anomalies or syndromes. Survival to hospital discharge was 98% (n = 591). Among 349 patients with follow-up, freedom from unplanned reintervent2ion at 5 years was 99% for TPS class 1, compared with 84% for class 2 and 30% for class 3. On multivariable Cox regression, classes 2 and 3 had significantly higher hazard for reintervention (class 2 hazard ratio 10.6; 95% confidence interval, 2.5 to 44.2; P =.001; class 3 hazard ratio 58.2, 95% confidence interval, 13.1 to 259; P <.001). At our center, ASO was associated with relatively low mortality. Class 2 and class 3 TPS were the most important independent predictors of reinterventions after discharge. Therefore, TPS can serve as a tool for identifying high-risk patients who warrant closer follow-up. [ABSTRACT FROM AUTHOR]

Background: Aortic stiffness has been shown to be abnormal in patients with bicuspid aortic valve (BAV), and is considered a component of the aortopathy associated with this condition. Progressive aortic stiffening associated with aging has been previously described in normal adults. However, it is not known if aging related aortic stiffening occurs at the same rate in BAV patients. We determined the longitudinal rate of decline in segmental distensibility in BAV patients using serial cardiovascular magnetic resonance (CMR) studies, and compared to previously published results from a group of patients with connective tissue disorders (CTD). Methods: A retrospective review of CMR and clinical data on children and adults with BAV (n = 49, 73% male; 23 ± 11 years) with at least two CMRs (total 98 examinations) over a median follow-up of 4.1 years (range 1–9 years) was performed to measure aortic distensibility at the ascending (AAo) and descending aorta (DAo). Longitudinal changes in aortic stiffness were assessed using linear mixed-effects modeling. The comparison group of CTD patients had a similar age and gender profile (n = 50, 64% male; 20.6 ± 12 years). Results: Compared to CTD patients, BAV patients had a more distensible AAo early in life but showed a steeper decline in distensibility on serial examinations [mean 10-year decline in AAo distensibility (× 10−3 mmHg−1) 2.4 in BAV vs 1.3 in CTD, p = 0.005]. In contrast, the DAo was more distensible in BAV patients throughout the age spectrum, and DAo distensibility declined with aging at a rate similar to CTD patients [mean 10 year decline in DAo distensibility (× 10−3 mmHg−1) 0.3 in BAV vs 0.4 in CTD, p = 0.58]. Conclusions: On serial CMR measurements, AAo distensibility declined at significantly steeper rate in BAV patients compared to a comparison group with CTDs, while DAo distensibility declined at similar rates in both groups. These findings offer new mechanistic insights into the differing pathogenesis of the aortopathy seen in BAV and CTD patients. [ABSTRACT FROM AUTHOR]

The aim of this study was to evaluate early and mid-term outcomes (mortality and prosthetic valve reintervention) after mitral valve replacement with 15- to 17-mm mechanical prostheses. A multicenter, retrospective cohort study was performed among patients who underwent mitral valve replacement with a 15- to 17-mm mechanical prosthesis at 6 congenital cardiac centers: 5 in The Netherlands and 1 in the United States. Baseline, operative, and follow-up data were evaluated. Mitral valve replacement was performed in 61 infants (15 mm, n = 17 [28%]; 16 mm, n = 18 [29%]; 17 mm, n = 26 [43%]), of whom 27 (47%) were admitted to the intensive care unit before surgery and 22 (39%) required ventilator support. Median age at surgery was 5.9 months (interquartile range [IQR] 3.2-17.4), and median weight was 5.7 kg (IQR, 4.5-8.8). There were 13 in-hospital deaths (21%) and 8 late deaths (17%, among 48 hospital survivors). Major adverse events occurred in 34 (56%). Median follow-up was 4.0 years (IQR, 0.4-12.5) First prosthetic valve replacement (n = 27 [44%]) occurred at a median of 3.7 years (IQR, 1.9-6.8). Prosthetic valve endocarditis was not reported, and there was no mortality related to prosthesis replacement. Other reinterventions included permanent pacemaker implantation (n = 9 [15%]), subaortic stenosis resection (n = 4 [7%]), aortic valve repair (n = 3 [5%], and aortic valve replacement (n = 6 [10%]). Mitral valve replacement with 15- to 17-mm mechanical prostheses is an important alternative to save critically ill neonates and infants in whom the mitral valve cannot be repaired. Prosthesis replacement for outgrowth can be carried out with low risk. [ABSTRACT FROM AUTHOR]

Background: Validated protocols for diagnostic testing and management of pregnant women with cardiovascular disease (CVD) do not exist. Our objective was to establish a prospective standardized protocol for the clinical evaluation of pregnant women with CVD.Methods: The Standardized Outcomes in Reproductive Cardiovascular Care (STORCC) initiative prospectively enrolled pregnant women with CVD into a standardized diagnostic testing and assessment protocol. Detailed cardiac and obstetric data were collected during the antepartum, intrapartum, and postpartum periods. Each woman was assigned a STORCC color code of perceived risk at a monthly multidisciplinary conference.Results: In 250 pregnancies of 207 women with CVD, the standardized care protocol was followed in 136 and routine care in 114. The median age of the subjects was 32 years, and the most common form of heart disease was congenital heart disease (77%). Women enrolled in standardized care protocol had high compliance with second- and third-trimester visits (93%) and postpartum visits (76%). Maternal cardiac complications occurred in 10%. The STORCC cardiac and obstetric color codes predicted adverse outcomes within each respective category (P = .02, .01).Conclusions: The STORCC protocol for prospective diagnostic testing and follow-up of pregnant women with CVD was successfully established, and compliance was high. The strength of a standardized testing and care protocol as well as detailed classification of labor and delivery characteristics allows for robust analyses into specific questions regarding testing protocols, and mode and timing of delivery. [ABSTRACT FROM AUTHOR]

We sought to characterize the natural history of aortic root dilatation and aortic regurgitation in tetralogy of Fallot (TOF). A single-center review of patients who underwent TOF repair from January 1960 to December 2022 was performed. Morphology was categorized as TOF-pulmonary stenosis or TOF-variant (including TOF-pulmonary atresia and TOF-pulmonary atresia-major aortopulmonary collateral arteries). Echocardiographically determined diameters and derived z scores were measured at the annulus, sinus of Valsalva, and sinotubular junction immediately before TOF repair and throughout follow-up. Linear mixed-effects models assessed trends in dimensions over time. Of 2205 patients who underwent primary repair of TOF at a median age of 4.9 months (interquartile range, 2.3-20.5 months) and survived to discharge, 1608 (72.9%) patients had TOF-pulmonary stenosis and 597 (27.1%) patients had TOF-variant. At a median postoperative follow-up of 14.4 years (interquartile range, 3.3-27.6 years; range, 0.1-62.6 years), 313 (14.2%) patients had mild or greater aortic regurgitation and 34 (1.5%) patients required an aortic valve or root intervention. The overall mean rates of annular, sinus of Valsalva, and sinotubular junction growth were 0.5 ± 0.2, 0.6 ± 0.3, and 0.7 ± 0.5 mm/year, respectively. Root z scores remained stable with time. At baseline, patients with TOF-variant had larger diameters and z scores at the annulus, sinus of Valsalva, and sinotubular junction, compared with patients with TOF-pulmonary stenosis (all P values <.05). Over time, patients with TOF-variant demonstrated relatively greater annular (P =.020), sinus of Valsalva (P <.001), and sinotubular junction (P <.001) dilatation. Patients with ≥75th percentile root growth rates had a higher incidence of mild or greater aortic regurgitation (P <.001), moderate or greater aortic regurgitation (P <.001), and aortic valve repair or replacement (P =.045). Patients with TOF-variant are at comparatively greater risk of pathologic root dilatation over time, warranting closer longitudinal follow-up. [Display omitted] [ABSTRACT FROM AUTHOR]

Supravalvular aortic stenosis (SVAS) represents a heterogeneous group, including Williams syndrome, familial elastin arteriopathy, sporadic cases, and others. This study sought to evaluate long-term outcomes of SVAS repair. A total of 87 patients underwent surgical repair of congenital SVAS at Boston Children's Hospital in Boston, Massachusetts, between 1997 and 2017. A total of 41 patients had Williams syndrome, and 46 did not. Of the 46 patients who did not have Williams syndrome, 23 had sporadic SVAS, and 13 had familial elastin arteriopathy. Demographic data and outcomes were reviewed and analyzed from medical records. The median age at operation was 2.9 years. Mean z score of the sinotubular junction was -3.29 ± 1.42 and of the aortic root was −0.09 ± 1.19. A total of 26% (n = 22) patients had coronary ostium stenosis, and 41% (n = 9) of them required patch plasty. Survival rates at 5, 10, and 20 years were all 94.3%. Freedom from left ventricular outflow tract reoperation at 5, 10, and 20 years was 78.5%, 70.3%, and 70.3%, respectively. Freedom from aortic arch reintervention at 5, 10, and 20 years was 98.6%, 94.3%, 89.3%, respectively. In risk factors analysis, age younger than 1 year, z scores of the aortic valve and aortic root, and concomitant right ventricular outflow tract surgical repair were predictive of the need for reoperation and reintervention for left or right ventricular outflow tract obstruction. Excellent long-term survival rates can be achieved with surgical repair of SVAS. Age younger than 1 year, small aortic valve and aortic root z scores, and concomitant right ventricular outflow tract surgical repair were predictors of reoperation and reintervention. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the association of neighborhood socioeconomic status (SES) with time to intravenous immunoglobulin treatment, length of stay (LOS), and coronary artery aneurysms (CAAs) in patients with Kawasaki disease.Study Design: We examined the relationship of SES in 915 patients treated at a large academic center between 2000 and 2017. Neighborhood SES was measured using a US census-based score derived from 6 measures related to income, education, and occupation. Linear and logistic regression were used to examine the association of SES with number of days of fever at time of treatment, LOS, and CAA.Results: Patients in the lowest SES quartile were treated later than patients with greater SES (7 [IQR 5, 9] vs 6 [IQR 5, 8] days, P = .01). Patients in the lowest SES quartile were more likely to be treated after 10 days of illness, with an OR 1.9 (95% CI 1.3-2.8). In multivariable analysis, SES remained an independent predictor of the number of days of fever at time of treatment (P = .01). Patients in the lowest SES quartile had longer LOS than patients with greater SES (3 [IQR 2, 5] vs 3 [IQR 2, 4], P = .007). In subgroup analysis of white children, those in the lowest SES quartile vs quartiles 2-4 were more likely to develop large/giant CAA 17 (12%) vs 30 (6%), P = .03.Conclusions: Lower SES is associated with delayed treatment, prolonged LOS, and increased risk of large/giant CAA. Novel approaches to diagnosis and education are needed for children living in low-SES neighborhoods. [ABSTRACT FROM AUTHOR]

Successful repair of anomalous origin of coronary artery from the pulmonary artery (ACAPA) is generally associated with a good prognosis. However, risk factors for poor postoperative outcomes have not been well characterized. This study used a multicenter data set to determine predictors of mortality after ACAPA repair. A retrospective analysis was performed using The Society of Thoracic Surgeons Congenital Heart Surgery Database's Participant User File. After identification of all patients with ACAPA who underwent repair from 2007 to 2016, demographics, preoperative and intraoperative variables, and postoperative complications were compared between survivors and nonsurvivors. The primary outcomes included (1) in-hospital mortality and (2) the need for postoperative extracorporeal membrane oxygenation (ECMO) support. Multivariable logistic regression was used to determine preoperative and intraoperative risk factors for these outcomes. Of the 703 patients who underwent ACAPA repair, 20 (2.8%) died during the same hospitalization. The odds of mortality were increased if preoperative shock was present (odds ratio [OR], 4.6; 95% confidence interval [CI], 1.4 to 15.1; P =.01) and if postoperative ECMO was required (OR, 11.8; 95% CI, 3.6 to 38.4; P <.001). The odds of postoperative ECMO use were increased if preoperative shock was present (OR, 3.6; 95% CI, 1.6 to 7.6; P =.001). Lower weight was also a risk factor for both mortality and postoperative ECMO. Lower weight, preoperative shock, and postoperative ECMO use were identified as risk factors for in-hospital mortality in patients undergoing ACAPA repair. These important perioperative factors likely reflect the clinical severity of presentation and suggest a role for early consideration of postoperative mechanical circulatory support to improve outcomes. [ABSTRACT FROM AUTHOR]

Background: Fasciculoventricular fibers (FVFs) are responsible for 1%-5% of cases of asymptomatic preexcitation on the surface electrocardiogram (ECG). Unlike ventricular preexcitation seen in Wolff-Parkinson-White (WPW) syndrome, FVFs are not associated with sudden cardiac death from preexcited atrial fibrillation.Objective: The purpose of this study was to identify surface ECG variables that differentiate FVFs from true WPW syndrome.Methods: This is a retrospective case-control study comparing surface ECG characteristics of patients diagnosed with FVFs (cases) with those of patients with WPW syndrome and anteroseptal accessory pathways (controls) via intracardiac electrophysiology testing at a single institution from 2005 to 2017.Results: Twenty-four cases of FVFs confirmed by intracardiac electrophysiology testing were identified and compared with 48 consecutive controls with WPW syndrome and anteroseptal accessory pathways. Patients with WPW syndrome were found to have significantly higher delta wave amplitudes (4.8 ± 2.0 mm vs 1.9 ± 1.3 mm; P < .001), shorter PR intervals (94.6 ± 12.5 ms vs 106.8 ± 13.2 ms; P < .001), and longer QRS intervals (133.6 ± 19.0 ms vs 118.7 ± 24.7 ms; P = .006) than did those with FVFs. Multivariable logistic regression analysis identified the delta wave amplitude as the only independent predictor of WPW syndrome (odds ratio 3.1 per 1-mm increase; bootstrapped 95% confidence interval 1.5-6.4; c statistic 0.90; P = .002).Conclusion: The etiology of preexcitation in patients with an anteroseptal preexcitation pattern, whether because of a benign FVF or because of potentially serious WPW syndrome, can be noninvasively deduced using the surface ECG. A higher delta wave amplitude is an independent risk factor for the presence of WPW syndrome and can accurately distinguish WPW syndrome from a FVF with good test accuracy characteristics. [ABSTRACT FROM AUTHOR]

Previous reports of primary graft dysfunction (PGD) in pediatric heart transplant (HT) recipients are limited to descriptive series of children who required extracorporeal membrane oxygenation (ECMO) support shortly after HT. In this study we sought to determine the incidence, risk factors, and survival after severe PGD in pediatric HT recipients. We identified all children <18 years old who underwent HT in the United States during 1996 to 2015 using the Organ Procurement and Transplant Network database and then identified those who developed severe PGD by linking patient variables to Extracorporeal Life Support Organization registry data. Logistic regression models were used to assess risk factors for developing severe PGD. The overall incidence of severe PGD was 4.7% over 20 years (95% confidence interval 4.2% to 5.3%). The incidence was 4.1%, 4.5%, 5.3%, and 4.6%, respectively, in consecutive 5-year periods (p for trend = 0.48). Independent risk factors for developing severe PGD were younger age, congenital heart disease, HT while supported on ECMO, higher serum bilirubin, and graft ischemic time ≥4 hours. Ventricular assist device support as bridge to HT and available donor variables were not associated. Death (or graft loss) before discharge occurred in 40.6% of children with PGD (105 deaths, 7 re-transplants) and in 5.6% of children without PGD. Severe PGD remains an important clinical morbidity in pediatric HT recipients in the current era and is associated with high mortality. These findings highlight the need for research in preventing and treating PGD in pediatric HT recipients for improving overall post-transplant survival. [ABSTRACT FROM AUTHOR]

Background In the current era, coarctation repair is usually performed as soon as diagnosis is established. We sought to determine if neonatal isolated coarctation repair had a higher rate of intervention postdischarge when compared with older infants. Methods We conducted a retrospective review of neonates (≤30 days of age) and infants (1 to 6 months of age) undergoing isolated coarctation repair between January 1, 2000, and March 31, 2016. Preoperative and postoperative Z scores of arch, aortic valve, and isthmus; rates of reintervention; and length of stay (LOS) were compared between groups. Linear or Cox regression was used to determine predictors of postoperative intensive care unit and hospital LOS during index surgery and postdischarge interventions for the entire cohort. Results There were 213 (71.5%) neonates and 85 (28.5%) infants. There was no difference in aortic valve morphology between groups. There was a trend but no statistical difference in postdischarge coarctation reintervention (neonates 10.3% versus infants 4.7%; p = 0.1) or left ventricular outflow tract, aortic valve, or mitral valve interventions (9.9% versus 9.4%; p = 0.9). Median postoperative intensive care LOS (3.01 versus 2.28 days; p < 0.001) and postoperative hospital LOS (6.23 versus 4.85 days; p < 0.001) were significantly higher in neonates. Median follow-up was 3 (interquartile range, 0.2 to 9) years for the entire cohort. On multivariable modeling, preoperative hypoplastic arch was associated with arch reinterventions, particularly in infants (10%; p = 0.003). Preoperative left-sided catheter intervention, left-sided obstructive lesions, and age predicted postdischarge intervention on left-sided structures. Conclusions Coarctation repair can be safely performed in infants and neonates with acceptable postdischarge intervention. Severity of aortic arch hypoplasia is an important discriminator for reinterventions on the arch, particularly in infants. [ABSTRACT FROM AUTHOR]

Background Isolated reports from low- and middle-income countries (LMICs) for surgical results in tetralogy of Fallot (TOF) are available. The International Quality Improvement Collaborative for Congenital Heart Disease (IQIC) seeks to improve surgical results promoting reductions in infection and mortality in LMICs. Methods All cases of TOF in the IQIC database performed between 2010 and 2014 at 32 centers in 20 LMICs were included. Excluded from the analysis were TOF with any associated lesions. A logistic regression analysis was performed to identify risk factors for in-hospital mortality after surgery for TOF. Results A total of 2,164 patients were identified. There were 1,839 initial primary repairs, 200 with initial systemic-to-pulmonary artery shunt, and 125 underwent secondary repair after initial palliation. Overall mortality was 3.6% (78 of 2,164), initial primary repair was 3.3% (60 of 1,839), initial systemic-to-pulmonary artery shunt was 8.0% (16 of 200), and secondary repair was 1.6% (2 of 125; p = 0.003). Major infections occurred in 5.9% (128 of 2,164) of the entire cohort. Risk factors for death after the initial primary repair were oxygen saturation less than 90% and weight/body mass index for age below the fifth percentile (p < 0.001). The initial primary repair occurred after age 1 year in 54% (991 of 1,839). Older age at initial primary repair was not a risk factor for death (p = 0.21). Conclusions TOF patients are often operated on after age 1 year in LMICs. Unlike in developed countries, older age is not a risk factor for death. Nutritional and hypoxemic status were associated with higher mortality and infection. This information fills a critical knowledge gap for surgery in LMIC. Visual Abstract [ABSTRACT FROM AUTHOR]

Background Repair of partial or transitional atrioventricular septal defects (P/TAVSDs) has excellent outcomes; however, late reinterventions remain a concern. Technical performance score (TPS) measures residua after repair and has been associated with early/mid-term outcomes after congenital cardiac operation. Our study investigates TPS as a predictor of outcomes after P/TAVSD repair. Methods This was a single-center retrospective review of P/TAVSD repair from July 2000 to November 2015. Intraoperative and discharge TPS were assigned based on echocardiographic criteria: class 1, no residua; class 2, minor residua; and class 3, major residua or reintervention for major residua. Intensive care unit (ICU) hospital length of stay and reintervention after discharge were analyzed with Cox regression. Results In our cohort, 124 partial (68%) and 59 transitional (32%) AVSDs underwent repair. Median age was 1.5 years (interquartile range [IQR]: 0.6 to 3.8 years), median weight was 9.7 kg (IQR: 6.6 to 14.1 kg), and 96 (52%) were female. Twenty patients (11%) required reintervention after discharge. On multivariable modeling, patients with TPS class 3 spent more days in the ICU (hazard ratio [HR] 0.33, 95% confidence interval [CI]: 0.19 to 0.58, p < 0.001) and hospital (HR 0.33, 95% CI: 0.19 to 0.57, p < 0.001) and had shorter time to reintervention after discharge (HR 8.76, 95% CI: 1.03 to 74.7, p = 0.047). Conclusions Major residua, that is TPS class 3, were a predictor of in-hospital outcomes and unplanned reinterventions after discharge, with left atrioventricular valve regurgitation being the strongest predictor. Use of TPS as a tool for residual lesions may identify patients predisposed to prolonged ICU hospital stay and reinterventions after discharge, providing feedback on areas in need of improvement in technique and identification of patients who warrant closer follow-up. [ABSTRACT FROM AUTHOR]

Background Mitral valve replacement (MVR) in very young children is challenging. This study investigates the mechanisms for early bioprosthetic valve failure in very young patients through review of the macroscopic and microscopic findings in explanted bioprosthetic valves. Methods Patients who underwent MVR with a bioprosthetic valve at Boston Children's Hospital between January 2010 to April 2016 at <5 years of age were the subjects of this study. Valve failure was defined as prosthetic mitral valve explantation with mitral valve re-replacement. Results Bioprosthetic valves were used in 31 of 77 MVRs during the study period. Valve failure occurred in 10 patients (32%). Freedom from valve failure was 80% at 1 year and was associated with older age at implantation. On gross and microscopic evaluation, valve failure (predominantly stenosis) was found to be due to pannus deposition and intrinsic leaflet calcification. Conclusions Successful long-term use of bioprosthetic valves in the mitral position in very young children continues to be a challenge. In addition to intrinsic calcification, excessive pannus deposition can lead to early bioprosthetic valve failure in this population. Early exuberant pannus growth appears due to thrombus deposition on the valves themselves and to the host's reaction to foreign material. [ABSTRACT FROM AUTHOR]

Objectives Although pseudoaneurysm is an uncommon complication after right ventricle–to–pulmonary artery conduit placement, it has the potential to cause significant morbidity and mortality. Methods We performed a review of patients with pseudoaneurysms diagnosed at our institution in a 20-year period (from 1995 through 2015) and compared their clinical characteristics with a group of age- and sex-matched control patients. Results We found that younger age, smaller size, the diagnosis of tetralogy of Fallot, the use of a pulmonary homograft conduit, the presence of an unrestrictive ventricular septal defect after conduit placement, and having at least systemic right ventricular pressure were all more common in patients who had pseudoaneurysms develop. Conclusions This study is unique in identifying both patient and surgical factors that may predispose to pseudoaneurysm development and can help inform optimal strategies to monitor and evaluate this patient population. [ABSTRACT FROM AUTHOR]

Data regarding the influence of intraoperative residual lesions on extracorporeal membrane oxygenation (ECMO) following the Norwood procedure are limited. Moreover, the significance of postoperative ECMO timing on in-hospital outcomes remains incompletely characterized. This was a single-center, retrospective review of consecutive patients who underwent the Norwood operation from January 1997 to November 2017. Patients with at least minor residual lesions based on the intraoperative postcardiopulmonary bypass echocardiogram were identified. The association between residual lesions and postoperative ECMO was assessed with logistic regression, adjusting for age, weight, prematurity, various preoperative system-specific and procedural risk factors, shunt type, and era. Among patients receiving ECMO, associations between late ECMO (≥3 days post-Norwood) and in-hospital mortality or transplant, postoperative hospital length-of-stay, and cost of hospitalization were evaluated using logistic regression or generalized linear models with a gamma distribution and logarithmic link. Among 500 patients, 78 (15.6%) received ECMO postoperatively. On multivariable analysis, the presence of at least minor residual lesions (odds ratio, 4.4; 95% CI, 2.1-9.3; P <.001) was associated with postoperative ECMO. In the ECMO subpopulation, there were 44 (56.4%) deaths or transplants. Late ECMO was associated with increased risk of in-hospital mortality or transplant (adjusted odds ratio, 6.2; 95% CI, 1.5-26.0), longer postoperative hospital length of stay (regression coefficient, 0.7; 95% CI, 0.3-1.1), and greater cost (regression coefficient, 0.6; 95%, CI 0.4-0.7), versus early ECMO (all P values <.05). The presence of even minor intraoperative residua significantly increases the risk of ECMO following the Norwood operation. Among patients receiving ECMO postoperatively, early institution of ECMO is associated with lower mortality and resource utilization. [Display omitted] [ABSTRACT FROM AUTHOR]

Neo-aortic root dilatation can lead to significant late morbidity after the arterial switch operation (ASO) for dextro-transposition of the great arteries (d-TGA). We sought to examine the growth of the neo-aortic root in d-TGA. A single-center, retrospective cohort study of patients who underwent the ASO between July 1, 1981 and September 30, 2022 was performed. Morphology was categorized as dextro-transposition of the great arteries with intact ventricular septum (d-TGA-IVS), dextro-transposition of the great arteries with ventricular septal defect (d-TGA-VSD), and double-outlet right ventricle-transposition of the great arteries type (DORV-TGA). Echocardiographically determined diameters and derived z scores were measured at the annulus, sinus of Valsalva, and sinotubular junction immediately before the ASO and throughout follow-up. Trends in root dimensions over time were assessed using linear mixed-effects models. The association between intrinsic morphology and the composite of moderate-severe aortic regurgitation (AR) and neo-aortic valve or root intervention was evaluated with univariable and multivariable Cox proportional hazards models. Of 1,359 patients who underwent the ASO, 593 (44%), 666 (49%), and 100 (7%) patients had d-TGA-IVS, d-TGA-VSD, and DORV-TGA, respectively. Each patient underwent a median of 5 echocardiograms (Q1-Q3: 3-10 echocardiograms) over a median follow-up of 8.6 years (range: 0.1-39.3 years). At 30 years, patients with DORV-TGA demonstrated greater annular (P < 0.001), sinus of Valsalva (P = 0.039), and sinotubular junction (P = 0.041) dilatation relative to patients with d-TGA-IVS. On multivariable analysis, intrinsic anatomy, older age at ASO, at least mild AR at baseline, and high-risk root dilatation were associated with moderate-severe AR and neo-aortic valve or root intervention at late follow-up (all P < 0.05). Longitudinal surveillance of the neo-aortic root is warranted long after the ASO. [Display omitted] [ABSTRACT FROM AUTHOR]

Objectives Liver disease is an important contributor to morbidity and mortality in patients after Fontan surgery. There has been no large-scale survey of liver health in this population. We sought to explore the prevalence and predictors of liver disease in a multicenter cohort of adults with Fontan physiology. Methods Subjects were recruited from 6 adult congenital heart centers. Demographics; clinical history; and laboratory, imaging, and histopathology data were obtained. Results Of 241 subjects (median age 25.8 years [11.8-59.4], median time since Fontan 20.3 years [5.4-34.5]), more than 94% of those who underwent testing (208 of 221) had at least 1 abnormal liver-related finding. All hepatic imaging (n = 54) and liver histology (n = 68) was abnormal. Subjects with abnormal laboratory values had higher sinusoidal fibrosis stage (2 vs 1, P = .007) and higher portal fibrosis stage (3 vs 1, P = .003) compared with those with all normal values. Low albumin correlated with lower sinusoidal fibrosis stage (1 vs 2; P = .02) and portal fibrosis stage (0 vs 3, P = .002); no other liver studies correlated with fibrosis. Regenerative nodules were seen on 33% of histology specimens. Conclusions Regardless of modality, findings of liver disease are common among adults with Fontan circulation, even those appearing clinically well. Cirrhosis is present in up to one-third of subjects. Correlations between hepatic fibrosis stage and clinical history or findings on noninvasive testing are few. Further research is needed to identify patients at risk for more severe liver disease and to determine the best methods for assessing liver health in this population. [ABSTRACT FROM AUTHOR]