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Patients with single-ventricle CHD undergo a series of palliative surgeries that culminate in the Fontan procedure. While the Fontan procedure allows most patients to survive to adulthood, the Fontan circulation can eventually lead to multiple cardiac complications and multi-organ dysfunction. Care for adolescents and adults with a Fontan circulation has begun to transition from a primarily cardiac-focused model to care models, which are designed to monitor multiple organ systems, and using clues from this screening, identify patients who are at risk for adverse outcomes. The complexity of care required for these patients led our centre to develop a multidisciplinary Fontan Management Programme with the primary goals of earlier detection and treatment of complications through the development of a cohesive network of diverse medical subspecialists with Fontan expertise.
ABSTRACT IMPACT: Preliminary results will inform the formal evaluation of the reliability of point-of-care ultrasound (POCUS) done by the gastroenterologist compared to standard of care methods such as MR-Enterography. OBJECTIVES/GOALS: Evaluation of mucosal healing is standard for pediatric patients with inflammatory bowel disease (IBD). Point-of-care ultrasound is a non-invasive, cost-efficient tool for assessing intestinal inflammation. We aim to evaluate the agreement between POCUS and typical cross-sectional imaging, such as MR-Enterography (MRE). METHODS/STUDY POPULATION: In this cross-sectional study, we recruited consecutive patients newly diagnosed with IBD, presenting to the specialty outpatient clinic or hospitalized in a pediatric tertiary care center between August to November 2020. They underwent POCUS performed by a single gastroenterologist, in addition to MRE. The sonographer was blinded to MRE results. Bowel wall thickness (BWT) was measured across different bowel segments and recorded twice in longitudinal view and twice in axial view. An average segmental BWT of the four measurements of more than 3 mm was considered inflamed. Agreement between sections of the bowel measured as inflamed were compared to inflamed bowel segments seen by MRE, using Cohen’s kappa. RESULTS/ANTICIPATED RESULTS: Eight of 12 patients completed both MRE and POCUS.A total of 40 bowel segments were assessed, namely the terminal ileum, ascending, transverse, descending and sigmoid colon. There were 4 girls with a median age of 15 years (IQR 14.25-16 years), and 6 patients were diagnosed with Crohn’s disease. Median PCDAI was 32.5 (IQR 30.6-40), and median PUCAI was 75 (72.5-77.5). Agreement between MRE and point-of-care ultrasound was substantial to perfect for the terminal ileum *(Îº= 0.75, 95%CI 0.31-1), transverse colon (Îº= 1, 95%CI 1-1) and sigmoid colon (Îº= 1, 95%CI 1-1). The agreement was poor for the ascending (Îº= 0, 95%CI 0-0) and moderate for the descending colon. (Îº= 0.6, 95%CI -0.07-1) DISCUSSION/SIGNIFICANCE OF FINDINGS: In pediatric patients with IBD, we found a high agreement between POCUS and MRE for imaging of the terminal ileum, transverse and sigmoid colon, areas commonly involved in IBD. This reinforces adult data, outlining the potential of POCUS as an evaluation tool of disease activity in clinical practice.
Systemic venous hypertension and low cardiac output are believed to be important mediators of liver injury after the Fontan procedure. Pulmonary vasodilators have the potential to improve such haemodynamics. The aim of this study was to assess the acute effects of exercise on liver stiffness and venous pressures and to assess the impact of inhaled Treprostinil on this response.
In this prospective, double-blind, placebo-controlled, crossover trial, 14 patients with a Fontan circulation were randomised to inhalation of placebo and Treprostinil. Incremental and constant work rate exercise tests were performed to assess the effect of Treprostinil on exercise tolerance. Venous pressures were measured throughout and liver stiffness at rest and immediately after peak exercise.
Mean age was 27.8 ± 7.9 years and 66% were females. Exercise acutely increased liver stiffness by 30% (mean shear wave speed: 2.38 ± 0.71 versus 2.89 ± 0.51 ms, p = 0.02). Peripheral venous pressures increased acutely during both incremental (12.1 ± 2.4 versus 22.6 ± 8.0 mmHg, p < 0.001) and constant work rate exercise (12.5 ± 2.5 versus 23.4 ± 5.2 mmHg, p < 0.001). Overall, Treprostinil failed to attenuate exercise-induced increases in liver stiffness. Compared with placebo, Treprostinil did not significantly impact venous pressure responses, VO2peak, nor exercise endurance times.
Peripheral venous pressure increased acutely during exercise by an average of 88% above baseline and was not altered by administration of inhaled Treprostinil. Liver stiffness measured immediately post-exercise increased acutely by an average of 30%, with no attenuation following Treprostinil inhalation.
Being overweight is associated with reduced functional capacity in Fontan patients. Increased adiposity leads to accumulation of epicardial and intra-abdominal visceral fat, which produce proinflammatory cytokines and may affect endothelial function. This retrospective study to evaluate the association between visceral fat and Fontan haemodynamics included 23 Fontan patients >18 years old with MRI and catheterization data available. Epicardial fat volume indexed to body surface area was measured by cardiac MRI, and intra-abdominal visceral fat thickness and subcutaneous fat thickness were derived from abdominal MRI. Stepwise regression models were used to determine univariable and multivariable associations between fat measures and haemodynamics. Mean age was 28.2 ± 9.5 years and body mass index was 26 ± 4 kg/m2. Mean central venous pressure was 13 ± 3 mmHg and pulmonary vascular resistance index was 1.23WU·m2 (interquartile range: 0.95–1.56). Epicardial fat volume was associated with age (r2 = 0.37, p = 0.002), weight (r2 = 0.26, p = 0.013), body mass index (r2 = 0.27, p = 0.011), and intra-abdominal visceral fat (r2 = 0.30, p = 0.018). Subcutaneous fat thickness did not relate to these measures. There was modest correlation between epicardial fat volume and pulmonary vascular resistance (r2 = 0.27, p = 0.02) and a trend towards significant correlation between intra-abdominal fat thickness and pulmonary vascular resistance (r2 = 0.21, p = 0.06). Subcutaneous fat thickness was not associated with Fontan haemodynamics. In multivariable analysis, including age and visceral fat measures, epicardial fat was independently correlated with pulmonary vascular resistance (point estimate 0.13 ± 0.05 per 10 ml/m2 increase, p = 0.03). In conclusion, in adults with Fontan circulation, increased visceral fat is associated with higher pulmonary vascular resistance. Excess visceral fat may represent a therapeutic target to improve Fontan haemodynamics.
Lymphopenia is common in adults who have had a Fontan operation although its aetiology and clinical implications remain unknown. Previous work suggests an association between lymphopenia and both liver disease and splenomegaly. The objective of this study was to assess the prevalence of lymphopenia in adults with a Fontan circulation and evaluate its associations with risk factors and clinical outcomes. Using a retrospective cohort study design, we studied 73 adult Fontan patients (age 25.0 ± 8.4 years) who had a complete blood count and abdominal imaging performed. Patients with protein-losing enteropathy were excluded. Clinical data were extracted from hospital records. The mean white blood cell count was 6580 ± 220/ml with a mean lymphocyte count of 1223 ± 508/ml. Lymphopenia, defined as lymphocyte count <1000/ml, was present in 23 (32%) patients. Patients with lymphopenia had a lower total white blood cell count (5556 ± 2517 versus 7136 ± 1924/ml, p = 0.009) and a lower platelet count (162 ± 69 versus 208 ± 69 k/ml, p = 0.008). Lymphopenia was also associated with findings of portal hypertension, including splenomegaly (36 versus 14%, p = 0.04), varices (22 versus 6%, p = 0.04), and ascites (39 versus 14%, p = 0.02). Lymphopenia did not correlate with any cardiac imaging, haemodynamic or exercise testing variables. In conclusion, lymphopenia is common in adult Fontan patients and is associated with markers of portal hypertension. Larger studies are needed to better define the relationship between lymphopenia and clinical outcomes.
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