Abstract

ObjectiveAfter the Fontan operation (ie, direct anastomosis of the caval veins to the pulmonary arteries, constituting right ventricular bypass circulation), high central venous pressure can lead to peripheral venous stasis and venous valvar insufficiency. We hypothesized that post-Fontan patients are at a higher risk of developing lower extremity venous lesions detectable using duplex ultrasound, even if clinical signs of chronic venous disease might not be evident. MethodsA total of 87 transplantation-free survivors after the Fontan procedure who reached adolescence or young adulthood (current age, 15-30 years) participated in a leg vein duplex ultrasound study. The median age at the Fontan procedure, median age at the vein study, and median interval between the two were 3.65 years (interquartile range [IQR], 3.1-5.3 years), 21.7 years (IQR, 18.9-24.7 years), and 16.6 years (IQR, 14.9-19.4 years), respectively. Duplex ultrasound scanning was performed using a venous ultrasound imaging system (Logiq P7; GE Healthcare). The patients were categorized according to the presence of venous reflux (VR) in the superficial, deep, or perforating venous systems: no VR, superficial VR (SVR), deep VR (DVR), perforating VR (PVR), and a combination of multiple venous systems. Correlation of the duplex ultrasound-detected venous lesions with clinical severity using the modified CEAP (clinical, etiological, anatomical, pathophysiological) classification was analyzed using Spearman's correlation analysis. ResultsLeg pain was reported by 48 of 87 patients (55.2%). The duplex ultrasound findings for the cohort were no VR in 21 patients (24.1%), SVR in 22 (25.3%), isolated PVR in 21 (24.1%), and reflux of multiple venous systems in 23 patients, including SVR and PVR in 19, DVR and PVR in 1, and SVR, PVR, and DVR in 3 patients. Although the patients with advanced venous lesions detected by duplex ultrasound tended to have a higher CEAP clinical class (P < .001), VR of any venous system on duplex ultrasound was present even in patients with a lower CEAP clinical class. The CEAP clinical class was C0 for 66 patients (76%), and VR of any venous system was present on duplex ultrasound in 66 patients (76%). ConclusionsThe prevalence of lower extremity venous lesions detected by duplex ultrasound is strikingly high in post-Fontan adolescents and young adults, and duplex ultrasound-detected venous abnormalities can precede clinical manifestations. Early detection and timely intervention for leg vein problems are mandatory for post-Fontan patients, especially for those considered to have risk factors for developing chronic lower extremity venous disease.

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