Abnormal Blood Flow Colors and Characteristics on Liver Ultrasound in Polycythemia Vera
In patients with polycythemia vera, Doppler ultrasound should specifically evaluate for hepatic vein thrombosis (Budd-Chiari syndrome) and portal vein thrombosis, as these represent the most critical vascular complications requiring immediate recognition and treatment. 1
Key Abnormal Doppler Findings Indicating Vascular Complications
Hepatic Vein Obstruction (Budd-Chiari Syndrome)
The following Doppler features are considered specific for hepatic vein obstruction and should raise immediate concern in PV patients 1:
- Absent flow signal in a large hepatic vein, appearing as a hyperechoic cord replacing the normal vein 1
- Reversed or turbulent flow within hepatic veins instead of normal hepatopetal flow 1
- Large intrahepatic or subcapsular collaterals with continuous flow connecting hepatic veins to diaphragmatic or intercostal veins 1
- Spider-web appearance typically located near hepatic vein ostia, combined with absence of normal hepatic vein visualization in that area 1
- Absent or flat hepatic vein waveform without the normal respiratory phasic variation (fluttering) 1
Portal Vein Thrombosis
Portal vein thrombosis is a common thrombotic complication in PV patients and presents with characteristic ultrasound findings 1:
- Absence of visible portal vein lumen with replacement by numerous serpiginous vascular channels in the porta hepatis (cavernomatous transformation) 1
- Echogenic material within the portal vein representing thrombus 1
- Absence of normal portal venous flow on color Doppler interrogation 1
Critical Pitfall to Avoid in PV Patients
Never assume normal hematocrit excludes active PV when evaluating for thrombosis. Iron deficiency from chronic bleeding (including from hereditary hemorrhagic telangiectasia if co-occurring) can mask the elevated red cell mass of PV, and patients may present with splanchnic vein thrombosis while appearing anemic 2, 3. This "masked PV" represents a diagnostic trap where the prothrombotic state persists despite normal-appearing blood counts 3, 4.
Additional Vascular Abnormalities in Hepatic Vascular Malformations
While less relevant to PV specifically, if hereditary hemorrhagic telangiectasia co-occurs (a rare but documented scenario), additional abnormal Doppler findings include 1:
- Dilated hepatic artery (>6 mm extrahepatic diameter) with peak flow velocity >80 cm/sec 1
- Low resistivity index (<0.55) in hepatic arteries 1
- Complex arteriovenous shunting with marked flow abnormalities in both arteries and veins 1
- Dilated hepatic and/or portal veins with marked flow abnormalities indicating decompensation of arteriovenous shunts 1
Practical Approach to Ultrasound Evaluation
When performing liver ultrasound in a patient with known or suspected PV 1:
- Systematically evaluate all three hepatic veins (right, middle, left) for patency, flow direction, and waveform characteristics
- Assess the main portal vein and its branches for echogenic thrombus and flow presence
- Examine for collateral vessels suggesting chronic venous obstruction
- Evaluate caudate lobe size, as hypertrophy occurs in approximately 75% of Budd-Chiari cases due to separate venous drainage 1
- Document inferior vena cava caliber and flow, as narrowing at the intrahepatic portion suggests caudate lobe enlargement from chronic hepatic vein obstruction 1
The sensitivity of Doppler ultrasound for detecting hepatic venous outflow obstruction exceeds 75% when performed by an experienced sonographer, making it the appropriate first-line investigation 1. If ultrasound findings are equivocal or the operator is inexperienced, proceed directly to contrast-enhanced CT or MRI for definitive characterization 1.