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Red cell distribution width (RDW), a measure of variability in red cell size, predicts adverse outcomes in acquired causes of heart failure. We examined the relation of RDW and outcomes in adults with congenital heart disease. We performed a prospective cohort study on 696 ambulatory patients ≥18years old enrolled in the Boston Adult Congenital Heart Disease Biobank between 2012 and 2016 (mean age 38.7 ± 13.5 years; 49.9% women). The combined outcome was all-cause mortality or nonelective cardiovascular hospitalization. Most patients had moderately or severely complex congenital heart disease (42.5% and 38.5%, respectively). Mean RDW was 14.0 ± 1.3%. RDW >15% was present in 81 patients (11.6%). After median 767days of follow-up, 115 patients sustained the primary combined outcome, including 31 who died. Higher RDW predicted both the combined outcome (hazard ratio [HR] for RDW >15% = 4.5, 95% confidence interval [CI] 3.0 to 6.6; HR per + 1SD RDW = 1.8, 95% CI 1.6 to 2.0, both p <0.0001) and death alone (HR for RDW >15% = 7.1, 95% CI 3.5 to 14.4; HR per + 1SD RDW = 1.8, 95% CI 1.6 to 2.0, both p <0.0001). RDW remained an independent predictor of the combined outcome after adjusting for age, cyanosis, congenital heart disease complexity, ventricular systolic function, New York Heart Association functional class, hemoglobin concentration, mean corpuscular volume, high-sensitivity C-reactive protein and estimated glomerular filtration rate (HR per + 1SD RDW = 1.5, 95% CI 1.2 to 1.9, p <0.0001). RDW also remained an independent predictor of mortality alone after adjustment for age plus each variable individually. In conclusion, elevated RDW is an independent predictor of all-cause mortality or nonelective cardiovascular hospitalization in adults with congenital heart disease. This simple clinical biomarker identifies increased risk for adverse events even among patients with preserved functional status., (Copyright © 2018. Published by Elsevier Inc.)

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., (Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Thrombosis occurs in up to 26% of patients with congenital heart disease after cardiac surgery and is associated with increased morbidity and mortality. Aspirin is commonly administered to reduce the risk of thrombosis, yet aspirin responsiveness is rarely assessed. In this study, we hypothesize that inadequate response to aspirin is associated with increased risk of thrombosis after selected congenital cardiac procedures considered to be high risk for thrombosis., Methods: Patients undergoing high-risk congenital cardiac surgery who received postoperative aspirin (N = 95) were studied. Response to aspirin was determined using the VerifyNow system several days after administration. Patients were monitored prospectively for 30 days for the development of a thrombosis event and the relationship between aspirin unresponsiveness and a thrombosis event was determined by the Fisher exact test., Results: Rate of aspirin unresponsiveness (≥550 aspirin reaction units [ARU]) was 10 of 95 (10.5%) and was highest in patients weighing less than 5 kg given 20.25 mg/d of aspirin. Thrombosis events occurred in 7 patients (7.4%). Thrombosis was observed in 6 of 10 (60%) patients who were unresponsive to aspirin, compared with 1 of 85 (1.2%) patients who were responsive to aspirin (P < .001). In 2 patients who were unresponsive to the initial aspirin dose, an increase in dose resulted in an adequate therapeutic aspirin response (ARU < 550), suggesting insufficiency rather than true resistance in a subset of patients., Conclusions: Postoperative thrombosis is associated with aspirin unresponsiveness in this patient population. In high-risk patients, monitoring of aspirin therapy and consideration of dose adjustment or alternative agents for unresponsive patients may be justified and warrants further investigation in a prospective trial., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: We have previously shown that surgical Technical Performance Scores (TPS) are important predictors of early postoperative morbidity across a wide spectrum of procedures and that intraoperative recognition and intervention of residual defects resulted in improved outcomes. We hypothesized that these scores would also be important predictors of midterm outcomes., Methods: Neonates and infants aged younger 6 months were prospectively followed from the index surgery for a minimum of 1 year. The TPS were calculated using previously published criteria, including intraoperative course, predischarge echocardiograms or catheterizations, and clinical data, and graded as optimal, adequate, or inadequate. Case complexity was determined by the Risk Adjustment for Congenital Heart Surgery-1 category. The primary outcome was mortality, and the secondary outcome was the need for unplanned reinterventions. Outcomes were analyzed using nonparametric methods and a logistic regression model., Results: A total of 166 patients were included in our study, with 7 early deaths. The remaining 159 patients (Risk Adjustment for Congenital Heart Surgery-1 category 4-6, 76 [48%]; neonates, 78 [49%]) were followed for a minimum of 1 year after surgery. There were 14 late deaths or late transplantations and 55 late reinterventions. On univariate analysis, the TPS were associated with mortality (P < .001) and reintervention (P = .04). On logistic regression analysis, inadequate TPS was associated with late mortality (P < .001; odds ratio, 7.2; 95% confidence interval, 2.2-23.6), and Risk Adjustment for Congenital Heart Surgery-1 category (P = .004; odds ratio, 3.7; 1.5-8.8) at index surgery was associated with need for late unplanned reintervention., Conclusions: Technical performance affects midterm survival after infant heart surgery. Inadequate TPS can be used to prospectively identify patients at ongoing risk of demise and the need for reintervention. An aggressive approach to diagnosing and treating residual lesions at the initial operation is warranted., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: Survival of children with in-hospital cardiac arrest that does not respond to conventional cardiopulmonary resuscitation (CPR) is poor. We report on survival and early neurological outcomes of children with heart disease supported with rapid-response extracorporeal membrane oxygenation (ECMO) to aid cardiopulmonary resuscitation (ECPR)., Methods and Results: Children with heart disease supported with ECPR were identified from our ECMO database. Demographic, CPR, and ECMO details associated with mortality were evaluated using multivariable logistic regression. Pediatric overall performance category and pediatric cerebral performance category scores were assigned to ECPR survivors to assess neurological outcomes. There were 180 ECPR runs in 172 patients. Eighty-eight patients (51%) survived to discharge. Survival in patients who underwent ECPR after cardiac surgery (54%) did not differ from nonsurgical patients (46%). Survival did not vary by cardiac diagnosis and CPR duration did not differ between survivors and nonsurvivors. Factors associated with mortality included noncardiac structural or chromosomal abnormalities (OR, 3.2; 95% CI, 1.3-7.9), use of blood-primed ECMO circuit (OR, 7.1; 95% CI, 1.4-36), and arterial pH <7.00 after ECMO deployment (OR, 6.0; 95% CI, 2.1-17.4). Development of end-organ injury on ECMO and longer ECMO duration were associated with increased mortality. Of pediatric overall performance category/pediatric cerebral performance category scores assigned to survivors, 75% had scores ≤2, indicating no to mild neurological injury., Conclusions: ECPR may promote survival in children with cardiac disease experiencing cardiac arrest unresponsive to conventional CPR with favorable early neurological outcomes. CPR duration was not associated with mortality, whereas patients with metabolic acidosis and noncardiac structural or chromosomal anomalies had higher mortality.