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Objectives To investigate the pathophysiology of chronic venous insufficiency in adults with a Fontan circulation. Background Chronic venous insufficiency (CVI) is prevalent in adults with a Fontan circulation, however the underlying pathophysiology has not been identified. We defined the prevalence of venous reflux and obstruction in a Fontan population, identified associated factors, and assessed the hypothesis that venous reflux correlates with clinical CVI in adults with a Fontan circulation. Methods Fifty-one adult Fontan subjects from the Congenital Heart Disease in Adults: Lower Extremity Systemic Venous Health in Fontan Patients (CALF) study, ten control subjects without cardiac disease, and ten comparator subjects with repaired tetralogy of Fallot underwent lower extremity Doppler venous ultrasound. We investigated 10 venous segments in each subject for venous obstruction and reflux. Results The prevalence of venous reflux was significantly greater in the Fontan population compared to healthy controls (51% vs. 10%, p=0.03). Venous flow patterns demonstrated a range of pulsatility and biphasic flow in many subjects. There was no evidence of venous obstruction in any subject. Venous reflux did not correlate with clinical venous insufficiency; 49% of Fontan subjects with observed venous reflux demonstrated no clinical signs of venous disease. Predictors of venous reflux in multivariate analysis were single right ventricle (p=0.03), use of anti-arrhythmic medications (p=0.04), and family history of venous disease (p=0.003). Conclusions Venous reflux is highly prevalent and venous obstruction is absent in adults with a Fontan circulation. Clinical CVI underestimates pathophysiologic venous insufficiency in adults with a Fontan circulation.

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.

Duplication of medical testing results in a financial burden to the healthcare system. Authors undertook a retrospective review of duplicate testing on patients receiving coordinated care across two institutions, each with its own electronic medical record system. In order to determine whether duplicate testing occurred and if such testing was clinically indicated, authors analyzed records of 85 patients transferred from one site to the other between January 1, 2006 and December 31, 2007. Duplication of testing (repeat within 12 hours) was found in 32% of the cases examined; 20% of cases had at least one duplicate test not clinically indicated. While previous studies document that inaccessibility of paper records leads to duplicate testing when patients are transferred between care facilities, the current study suggests that incomplete electronic record transfer among incompatible electronic medical record systems can also lead to potentially costly duplicate testing behaviors. The authors believe that interoperable systems with integrated decision support could assist in minimizing duplication of testing at time of patient transfers.