Overview
Congenital systemic venous atrium refers to anomalies involving the systemic venous return to the atrium, often associated with complex portasystemic shunts and other congenital vascular malformations. These conditions can lead to systemic venous congestion and affect organ perfusion, particularly in critically ill patients 1.Diagnosis
Point-of-care Ultrasound (POCUS): Utilize the Venous Excess Ultrasound (VExUS) score to assess gallbladder wall thickening indicative of systemic venous congestion 1.
Imaging Studies: Consider CT or MRI for detailed anatomical assessment of portasystemic anomalies, especially in cases requiring transplantation 2.
Clinical Correlation: Differentiate from acute cholecystitis based on clinical context and imaging findings 1.Management
Diuretic Therapy: Administer diuretics to manage fluid overload and reduce systemic venous congestion 1.
Surgical Intervention: Careful surgical management is crucial in cases like liver transplantation, focusing on preventing mesenteric edema due to portal pressure changes 2.
Monitoring: Closely monitor for signs of mesenteric congestion and adjust fluid management accordingly 2.Special Populations
Critically Ill Patients: POCUS is particularly valuable in critically ill patients for early detection and differentiation of systemic venous congestion 1.
Liver Transplant Recipients: Special attention to portasystemic pressure dynamics and mesenteric edema management is essential 2.Key Recommendations
Utilize POCUS with the VExUS score for early identification of systemic venous congestion in critically ill patients (Evidence: Moderate 1).
In patients with congenital absence of the portal vein undergoing liver transplantation, meticulously manage portal pressure to prevent mesenteric edema (Evidence: Weak 2).
Employ diuretic therapy as a first-line treatment for managing fluid overload associated with systemic venous congestion (Evidence: Expert opinion 1).References
1 Cheong I, Tamagnone FM. Assessing Gallbladder Wall Thickening due to Systemic Venous Congestion Using the VExUS Score With Point-Of-Care Ultrasound. Journal of clinical ultrasound : JCU 2026. link
2 Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. Journal of pediatric surgery 1994. link90812-5)