Quantification of lymphatic burden in patients with Fontan circulation by T2 MR lymphangiography and associations with adverse Fontan status.

AIMS: To quantify thoracic lymphatic burden in paediatric Fontan patients using MRI and correlate with clinical status. METHODS AND RESULTS: Paediatric Fontan patients (<18-years-old) with clinical cardiac MRI that had routine lymphatic 3D T2 fast spin echo (FSE) imaging performed from May 2017 to October 2019 were included. 'Lymphatic burden' was quantified by thresholding-based segmentation of the 3D T2 FSE maximum intensity projection image and indexed to body surface area, performed by two independent readers blinded to patient status. There were 48 patients (27 males) with median age at MRI of 12.9 (9.4-14.7) years, time from Fontan surgery to MRI of 9.1 (5.9-10.4) years, and follow-up time post-Fontan surgery of 9.4 (6.6-11.0) years. Intraclass correlation coefficient between two observers for lymphatic burden was 0.96 (0.94-0.98). Greater lymphatic burden correlated with post-Fontan operation hospital length of stay and duration of chest tube drainage (rs = 0.416, P = 0.004 and rs = 0.439, P = 0.002). Median lymphatic burden was greater in patients with chylous effusions immediately post-Fontan (178 (118-393) vs. 113 (46-190) mL/m2, P = 0.028), and in patients with composite adverse Fontan status (n = 13) defined by heart failure (n = 3), transplant assessment (n = 2), recurrent effusions (n = 6), Fontan thrombus (n = 2), and/or PLE (n = 6) post-Fontan (435 (137-822) vs. 114 (51-178) mL/m2, P = 0.003). Lymphatic burden > 600 mL/m2 was associated with late adverse Fontan status with sensitivity of 57% and specificity of 95%. CONCLUSION: Quantification of MR lymphatic burden is a reliable tool to assess the lymphatics post-Fontan and is associated with clinical status.

MRI Phase-Contrast Blood Flow in Fasting Pediatric Patients with Fontan Circulation Correlates with Exercise Capacity.

Purpose: To assess regional blood flow in fasting pediatric patients with Fontan circulation by using MRI and to explore associations with clinical parameters. Materials and Methods: In this retrospective study, pediatric patients who had undergone the Fontan procedure (<18 years of age) and had undergone clinical cardiac MRI, performed after at least 4 hours of fasting, between 2018 and 2021 were included. Regional blood flow was compared with published healthy volunteer data (n = 19) and assessed in relation to hemodynamic parameters and clinical status. Data are presented as medians, with first to third quartiles in parentheses. Mann-Whitney U, Kruskal-Wallis, χ2, and Spearman rank correlation tests were used. Results: Fifty-five patients (38 boys) with median age at MRI of 14 years (IQR, 11-16 years) and median time from Fontan procedure to MRI of 10 years (IQR, 8-12 years) were included. Patients after Fontan procedure had lower ascending aortic, inferior vena cava, and total systemic blood flow compared with healthy volunteers (3.00 L/min/m2 [IQR, 2.75-3.30 L/min/m2] vs 3.61 L/min/m2 [IQR, 3.29-4.07 L/min/m2]; 1.73 L/min/m2 [IQR, 1.40-1.94 L/min/m2] vs 2.24 L/min/m2 [IQR, 2.06-2.75 L/min/m2]; 2.78 L/min/m2 [IQR, 2.45-3.10 L/min/m2] vs 3.95 L/min/m2 [IQR, 3.20-4.30 L/min/m2], respectively; P < .001). Portal vein flow was greater than hepatic vein flow in 25% of patients. Fontan blood flow was inversely correlated with pre-Fontan mean pulmonary artery pressure (Spearman rank correlation coefficient [rs ]= -0.42, P = .005) and ventricular end diastolic pressure (rs = -0.33, P = .04) and positively correlated with post-Fontan percent predicted oxygen consumption at peak workload (rs = 0.34, P = .02). Conclusion: Reference ranges are provided for regional systemic blood flow derived by using MRI in fasting pediatric patients with Fontan circulation, who had lower systemic blood flow compared with healthy volunteers. Lower fasting Fontan blood flow correlated with lower exercise capacity.Keywords: Pediatrics, Heart, Congenital, MR Imaging, Hemodynamics/Flow Dynamics, Cardiac Supplemental material is available for this article. © RSNA, 2022.

PCBs and cognitive functioning of Mohawk adolescents.

This paper reports on the relationships between the cognitive functioning and PCB current body burdens of adolescents in the Mohawk Nation of Akwesasne where there is concern about industrial pollution of the environment. Three cognitive tests (Woodcock Johnson-Revised, Test of Memory and Learning, and Ravens Progressive Matrices) provide 13 subtests that allow a variety of cognitive outcomes to be assessed. A summary measure of PCB level was created from the congeners detected in at least 50% of the participants. The most notable finding was the significant negative relationship between PCB levels and two separate measures of long term memory. There was also a negative relationship with a measure of comprehension and knowledge. Significant relationships were not large, but provide evidence of subtle negative effects of PCB exposure.

Organochlorines, lead, and mercury in Akwesasne Mohawk youth.

Most humans have detectable body burdens of polychlorinated biphenyls (PCBs), hexachlorobenzene (HCB), and p,p'-dichlorophenyldichloroethylene p,p'-DDE), a metabolite of p,p'-dichlorodiphenyltrichloroethane (DDT). Native American communities may be at increased risk of exposure through subsistence-based diets and greater physical contact with contaminated soil and water. In this article we describe the levels of toxicants (PCBs, p,p'-DDE, HCB, mirex, lead, and mercury) among youth 10-17 years old (n = 271) of the Akwesasne Mohawk Nation. Ultratrace, congener-specific PCB analysis of human serum quantitated 83 PCB congeners (plus 18 as pairs/triplets), in addition to p,p'-DDE, HCB, and mirex, and included all major Aroclor-derived congeners typically present in human samples. Twenty congeners (in 16 chromatographic peaks) were detected in 50% or more of the individuals sampled [geometric mean (GM) of the sum of these congeners = 0.66 ppb]. Thirteen congeners (in 10 peaks) were detected in 75% or more of the samples (GM = 0.51 ppb). Of the 20 congeners detected in 50% or more of the samples, 17 had five or more chlorine substitutions. International Union for Pure and Applied Chemistry congeners 118, 101(+90), and 153 were detected in nearly all participants (GM = 0.06 ppb, 0.05 ppb, 0.09 ppb, respectively). p,p'-DDE and HCB were detected in 100% and 98% of the samples (GM: p,p'-DDE = 0.37 ppb; HCB = 0.03 ppb). Mirex was detected in approximately 46% of the samples (GM = 0.02 ppb). No cases of elevated lead level were observed. One participant had a mercury level marginally higher than the U.S. Environmental Protection Agency's current level of concern (0.50 microg/dL). Although differences in analytic methods and participant ages limit comparability, toxicant levels from the Mohawk youth are lower than those associated with severe food contamination (Yusho and Yu-cheng) but similar to other chronically exposed groups.