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Patients who have undergone the Fontan procedure in later adolescence and adulthood represent a unique population at risk for significant morbidity and mortality. The optimal strategy for long-term management of such patients is unknown. The aim of this study was to evaluate outcomes of patients who had undergone Fontan surgery later in life, focusing on late survivorship, mode of death, and predictors of mortality. Eighty-eight patients were identified who had their initial Fontan operation from 1973 to 2007 at ≥15 years of age. A standardized tiered contact protocol was followed to capture the recent health status of each patient; the probability of survival was 83%, 71%, and 66% at 5, 10, and 15 years of follow-up, respectively. Despite focused efforts, the modes of death were not available in 48% of the patients. A prolonged intensive care unit stay at the time of operation was the single predictor of mortality (p = 0.0123). In conclusion, this investigation highlights the significant mortality that exists in patients who undergo a Fontan procedure later in life and the difficulties in achieving standardized medical follow-up for this high-risk group of patients. more...

Background— Significant morbidities, including angina, symptomatic heart failure, and myocardial infarction, have been reported after coronary artery fistula (CAF) closure; however, predictors that may be associated with adverse outcomes have not been established. The goal of this investigation is to describe the long-term outcomes witnessed in patients with either treated or untreated CAF at our institution and to investigate whether certain features predicted adverse outcomes. Methods and Results— The records and angiograms of patients with CAF who underwent a diagnostic cardiac catheterization at Children’s Hospital Boston from 1959 through 2008 were reviewed. Of 76 patients identified, 20% were associated with additional congenital heart disease. Forty-four underwent transcatheter closure, 20 underwent surgical repair, and no intervention was performed in the remaining 12 subjects. Three patients who had initially undergone surgical closure had a second intervention, 1 underwent repeat surgery, and 2 underwent transcatheter closure. One patient who had undergone transcatheter closure underwent a second transcatheter closure for residual fistula. Major complications, including myocardial infarction, angina with coronary thrombosis, and symptomatic cardiomyopathy, occurred in 11 (15%) patients. The sole angiographic feature that was predictive of adverse outcome was drainage of the CAF into the coronary sinus ( P P P =0.006), diabetes ( P =0.05), systemic hypertension ( P P Conclusions— Long-term complications of CAF closure may include coronary thrombosis, myocardial infarction, and cardiomyopathy. CAF that drain into the coronary sinus are at particularly high-risk of long-term morbidities after closure, and strategies including long-term anticoagulation should be considered. more...

Background. Human-administered clinical scales are the accepted standard for quantifying motor performance of stroke subjects. Although they are widely accepted, these measurement tools are limited by interrater and intrarater reliability and are time-consuming to apply. In contrast, robot-based measures are highly repeatable, have high resolution, and could potentially reduce assessment time. Although robotic and other objective metrics have proliferated in the literature, they are not as well established as clinical scales and their relationship to clinical scales is mostly unknown. Objective. To test the performance of linear regression models to estimate clinical scores for the upper extremity from systematic robot-based metrics. Methods. Twenty kinematic and kinetic metrics were derived from movement data recorded with the shoulder-and-elbow InMotion2 robot (Interactive Motion Technologies, Inc), a commercial version of the MIT-Manus. Kinematic metrics were aggregated into macro-metrics and micro-metrics and collected from 111 chronic stroke subjects. Multiple linear regression models were developed to calculate Fugl-Meyer Assessment, Motor Status Score, Motor Power, and Modified Ashworth Scale from these robot-based metrics. Results. Best performance—complexity trade-off was achieved by the Motor Status Score model with 8 kinematic macro-metrics ( R = .71 for training; R = .72 for validation). Models including kinematic micro-metrics did not achieve significantly higher performance. Performances of the Modified Ashworth Scale models were consistently low ( R = .35-.42 for training; R = .08-.17 for validation). Conclusions. The authors identified a set of kinetic and kinematic macro-metrics that may be used for fast outcome evaluations. These metrics represent a first step toward the development of unified, automated measures of therapy outcome. more...