Doppler ultrasound of the portal system - Pathological findings

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Cirrhosis, portal hypertension, portal vein thrombosis, Budd-Chiari syndrome, portal vein aneurysm, TIPS, hereditary hemorrhagic telangiectasia, arteriovenous fistula, SOS.

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  • Most prevalent portal systemic collateral present in 80% to 90%of patients with portal hypertension.Most clinically important ofthe portal systemic collaterals because its presence implies an increased risk for variceal hemorrhage.
  • Determination of flow direction in splenic vein increases the diagnostic confidence of S-R shunt.
  • Hepatic artery and the portal vein have blood flowing in opposite directions.
  • Although the role of Doppler sonography has decreased in the evaluation of the hepatic lesions with recent advances in CT andMR imaging, it should be kept in mind that Doppler sonography has the advantage over CT and MRI of demonstrating the direction of the flow of the hepatic vasculature.
  • Acceleration resulting from focal compression by regenerative nodulesValue of 0.6 of DI showed a sensitivity of 75.9% and a specificity of 81.8% for the presence of severe portal hypertension (hepatic venous pressure gradient >12mmHg) (AUC = 0.860).
  • Absolute contraindications to TIPS:1- Severe hepatic encephalopathy and liver failure2- Chronic portal vein thrombosis, especially those with narrowed and fibrotic veins or cavernous transformation Experienced centers are often successful in placing a shunt in patients with acute or subacute thrombosis.3- Severe right-heart failure with elevated central venous pressureRelative contraindications to TIPS1- Polycystic liver disease2- Systemic hepatic infections3- Hypervascular liver tumorsTechnical success rate for placement of TIPS is greater than 90%.The procedural complication rate ranges from 10 to 16%.Mortality related to the TIPS procedure is usually less than 2%.Primary patency:1 year: 25 - 66%2 years: 5 - 42% 3 years: 21%4 years: 13%5 years 13%Radiologic revision of malfunctioning shunt usually successful, resulting in primary assisted patency rate of approximately1 year: 85%2 year: 61%3 years: 46%4 years: 42%5 years: 36%
  • Early stenosis would be missed if one waited until the stent velocity dropped to 50 to 60 cm/second.
  • Important unanswered questions in cirrhotic portal vein thrombosis:Does occurrence of PVT alter the natural history of cirrhosis and therefore should asymptomatic patients be treated withthe goal of recanalization or prevention of further thrombus extension?Should all patients with cirrhosis and PVT be aggressively anticoagulated?Should this apply only to patients on transplantation waiting list?If recanalization does not occur should patients be offered second-line treatment with transjugularintrahepaticportosystemic shunts?How long should the interval be whilst being anticoagulated before considering therapy to have failed?How should patients be monitored?Is oral warfarin better than low-molecular weight heparin?
  • Consider only direct visualization of obstruction, and/or collaterals, of a hepatic vein or inferior vena cava, as definite evidence for the diagnosis.
  • Term “spiderweb” was initially used in description of angiographic findings in Budd-Chiari syndrome, and it means presence of very small interwoven veins.Interwoven: منسوجة
  • Hypertrophied subcapsular veins may shunt blood from liver to systemic veins (azygos vein, intercostal veins) or directly to inferior vena cava creating new intrahepatic and extrahepatic circulation.
  • “Bicolored” hepatic vein
  • HCC appears to be a significant long-term complication (11 of 97 patients followed-up for a mean of 5 years).αFP more specific for dg than with other liver diseases. Risk of HCC in long-standing IVC obstruction 70-fold higher than those with pure hepatic vein involvement.
  • HVOD frequently develops before day 20 of bone marrow transplantation.clinically suspected when there is jaundice, painful hepatomegaly, and fluid retention. Intensive myeloablativechemotherapy or radiation therapy before transplantation is presumed to be the cause.Clinical criteria for diagnosis: Seattle Criteria & Baltimore Criteria (weight gain – ascites – hepatomegaly - hyperbilirubinemia). The diagnosis may be supported by imaging, which will demonstrate the presence of hepatomegaly and ascites and rule out biliary obstruction due to benign or malignant causes, but imaging is currently not diagnostic by itself. The best-studied modality is gray-scale and color Doppler ultrasonography. The majority of studies suggest that no single ultrasound parameter is diagnostic for SOS.Findings that are highly suggestive of SOS are reversal of portal venous flow, attenuation of hepatic venous flow, gallbladder wall edema, and perhaps increased resistive indices to hepatic artery flow. A composite score of gray-scale and color Doppler ultrasound criteria has been proposed, but may be too cumbersome for routineclinical use. One study has suggested that the presence of flow in the para-umbilical vein is more common in moderate and severe SOS, but this will need to be validated by other investigators.
  • spoke-wheel:
  • Infradiaphragmatic total anomalous pulmonary venous return (TAPVR)
  • All or part of EHBT atretic – 1 in 15 000 live birthsMoyer et al introduced general guidelines for evaluation of cholestatic jaundice in infants.High-spatial resolution real-time US serves as a first-line screening tool with which to determine the cause of jaundice. Presence of triangular cord (TC) sign and an abnormal gallbladder (GB) on high-spatial resolution real-time US images is widely accepted as the diagnostic criterion for BA.Length of the GB on longitudinal scanning: GB length of at least 1.5 cm considered normal.Thickness of EARPV just proximal to RPV bifurcation site: triangular cord sign is EARPV thicker than 4 mm on longitudinal US images.
  • Causes of porto-systemic shuntCirrhosis Most frequent – Associated with HETrauma Blunt or penetratingPV aneurysm Rupture into hepatic veinCongenitalExtrahepatic “Abernethy malformation”Intrahepatic “Park classification” HHT Hereditary Hemorrhagic Telangiectasia
  • Medical literature contains approximately 100 published cases of congenital portosystemic shunts. First study conducted by Abernethy in 1793.Morgan and Superina classified extrahepaticportosystemic shunts into two types (1994).
  • The first type is the most common. Blood flow volumes are measured by multiplying the lumen area by the mean velocity at a given point. Portovenous shunt ratio is calculated by dividing the total blood flow volume in the shunt by that in the portal vein. Although the presence of a portosystemic shunt is considered abnormal in all cases, it has been demonstrated that shunt ratios of less than 24%–30% do not cause liver encephalopathy, even in cirrhotic patients.Portosystemic venous shunting causes hypergalactosemia, which, if it persists over a long period of time, leads to cataract formation.Portosystemic encephalopathy usually develops in adults.The natural history of portosystemic shunting depends on shunt ratio and patient age. Spontaneous closure is expected to occur in the first 2 years of life when an intrahepaticportosystemic shunt is found; close follow-up is recommended.In patients of all ages, shunt ratios above 60% should be corrected due to the risk of encephalopathy and liver dysfunction.Both embolization and surgical correction of the shunt have been described. When these measures fail, liver transplantation is the only therapeutic option.
  • DV is embryonic vascular structure that connects umbilical vein to IVC bypassing liver during the fetal life & closes immediately after birth.Functional closure is completed within the first few minutes of life, but true obliteration may not be complete for 15 to 20 dayMeasurement of postprandial serum galactose and ammonia concentrations can be used as screening tests in infants suspected of having a portosystemic shunt through the ductusvenosus.16 cases of PDV in children, excluding of neonates, have been reported. Most children were Japanese.The initial presentation included hypergalactosemia, liver dysfunction, encepalopathy, hypoxia, and tachypnea.Ultrasonography and angiography are useful in diagnosing PDV.
  • Causes of arterio-portal shuntTrauma Blunt or penetratingIatrogenic Liver biopsy – PTC Cirrhosis With or without HCCFocal liver lesion Benign or malignantAneurysm Rupture of arterial or venous aneurysmCongenital HHT – Ehlers-Danlos – Biliaryatresia
  • Causes of arterio-systemic shuntHepato-cellular carcinomaLarge hemangiomaHereditary Hemorrhagic Telangiectasia (HHT)
  • Causes of sytsemic-to-systemic shunt:BCSHHT
  • Causes of enlargement of hepatic artery:1- Cirrhosis2- Hepatic diseases associated with alcoholism3- Vascular tumors4- Hereditary hemorrhagic telangiectasia
  • Doppler ultrasound of the portal system - Pathological findings

    1. 1. Doppler of the portal system Pathological findings Samir Haffar M.D. Assistant Professor of Gastroenterology
    2. 2. Doppler of the portal system  Portal hypertension  Portal vein thrombosis  Budd-Chiari syndrome & SOS*  Suspicion of cardiac disease  Characterization of hepatic mass  Miscellaneous * SOS: Sinusoidal Obstruction Syndrome
    3. 3. Causes of portal hypertension Pre-sinusoidal Congenital hepatic fibrosis Sarcoidosis Schistosomiasis Lymphoma Hyperdynamic Arterio-portal fistula or malformation Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13. Intra-hepatic Post-sinusoidal Cirrhosis Causes Disease Extra-hepatic Portal vein thrombosis or compression most common cause Supra-hepatic Budd-Chiari syndrome Right heart insufficiency
    4. 4. Doppler US signs of PHT in cirrhosis • P-S collaterals Highly sensitive & specific • Portal vein Dilated PV Decreased mean velocity (< 15 cm/sec) To-and-fro flow /Hepatofugal flow Increased pulsatility (VPI) Arterio-portal fistula • Hepatic vein Compression (Pseudo-portal flow) • Hepatic artery Enlargement & tortuosity Increased RI & PI Harkanyi Z. Ultrasound Clin 2006 ; 1 : 443 – 455.
    5. 5. Porto-systemic collaterals High sensitivity & specificity for PHT • Tributary collaterals “Drain normally into PS” Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13. Coronary vein (left gastric) Short gastric veins Branches of SMV & IMV • Developed collaterals “Developed or recanalized” Recanalized umbilical vein Spleno-renal collateral Gastro-renal collateral Spleno-retroperitoneal collateral
    6. 6. Common spontaneous porto-systemic collaterals More than 20 P-S collaterals described Patnquin1 H et al. Am J Roentgenol 1987 ; 149 : 71 – 76. Most common: LGV – PUV – Spleno-renal – Gastro-renal
    7. 7. P-S collaterals / Coronary vein Most prevalent (80-90%) – Most clinically important Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13. Sagittal view slightly superior Tortuosity of CV as it extends superiorly toward GE junction Sagittal paramedial view Flow in CV directed superiorly & away from splenic vein
    8. 8. P-S collaterals / Gastroesophageal collateral Gastroesophageal collateral veins close to diaphragm McGahan J et al. Diagnostic ultrasound, Informa Healthcare, 2nd edition, 2008. Longitudinal view of left liver lobe
    9. 9. Normal umbilical vein anatomy UV communicates with umbilical segment of LPV Travels down anterior abdominal wall toward umbilicus Eventually drains into systemic system via inferior epigastric vein Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
    10. 10. Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13. Hepatofugal flow within UV Similar color Doppler viewLongitudinal US of LLL Dilated umbilical vein (10 mm) P-S collaterals / Recanalized umbilical vein PUV observed only in hepatic or suprahepatic blockage
    11. 11. Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13. N Engl J Med 2005 ; 353 : e19. Sagittal panoramic view PUV traveling to periumbilical region where it becomes tortuous P-S collaterals / Recanalized umbilical vein Caput medusae
    12. 12. Recanalized UV in ligamentum teres UV ramifies into smaller PU collaterals when it proceeds inferiorly Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13. Sagittal color Doppler view US of LLL P-S collaterals / Recanalized umbilical vein
    13. 13. Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13. Detectable flow within UV Flow directed away from LPV Indicating recanalization & PHT Similar color Doppler viewLongitudinal US of LLL UV extending from LPV Diameter: 1.8 mm P-S collaterals / Recanalized umbilical vein
    14. 14. Porto-systemic collaterals • Coronary vein & umbilical vein are the easiest & most productive to analyze • Other collaterals detected sonographically albeit with more difficulty in some cases Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
    15. 15. P-S collaterals / Spleno-renal collateral Yamada M et al. Abdom Imaging 2006 ; 31:701 – 705. Mansour MA et al. Vascular Diagnosis. Elsevier-Saunders, Philadelphia, 1st edition, 2005. Transverse color Doppler US Splenic vein feeding large splenorenal collaterals Flow direction from SV to LRV Reversed or to-and-fro flow in SV Schematic drawing
    16. 16. P-S collaterals / Spleno-renal collateral Flow inversion in splenic vein Flow inversion in SV increases dg of spleno-renal shunt Mansour MA et al. Vascular Diagnosis. Elsevier-Saunders, Philadelphia, 1st edition, 2005
    17. 17. P-S collaterals / Short gastric veins Sato T et al. J Gastroenterol 2002 ; 37 : 604 – 610. Short gastric vein as inflowing vessel to gastric varices
    18. 18. P-S collaterals / Gastro-renal collateral Yamada M et al. Abdom Imaging 2006 ; 31 : 701 – 705. Maruyama H et al. Acad Radiol 2008 ; 15 : 1148 – 1154. From cranial & dorsal side to caudal & ventral side into LRV Long-axis view of GRS GRS LRV From SV at confluence coursing backward to join LRV Schematic drawing
    19. 19. P-S collaterals / Lumbar & epigastric collaterals Mansour MA et al. Vascular Diagnosis. Elsevier-Saunders, Philadelphia, 1st edition, 2005 Large collateral vein between LK & lower pole of spleen shunting blood from splenic hilum to lumbar & epigastric veins
    20. 20. P-S collaterals / Superior mesenteric vein Flow toward SMV in sup branch Flow away from SMV in inf branch Color Doppler view 2 mesenteric branches of superior mesenteric vein Semicoronal view of SMV Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
    21. 21. P-S collaterals / Inferior mesenteric vein Mansour MA et al. Vascular Diagnosis. Elsevier-Saunders, Philadelphia, 1st edition, 2005. Hepatofugal flow in IMV originating from PV confluence
    22. 22. P-S collaterals / IMV & rectal venous drainage Wachsberg RH. Am J Roentgenol 2005 ; 184 : 481 – 486. Peri-rectal varices Transverse US through bladderLeft parasagittal CDUS Hepatofugal flow in dilated IMV
    23. 23. P-S collaterals / Gallbladder varices Harkanyi Z. Ultrasound Clin 2006 ; 1 : 443 – 455. Sepentine area in wall of GB Cystic vein to anterior abdominal wall or patent PV branches Most commonly observed in PV thrombosis (30%)
    24. 24. Mansour MA et al. Vascular Diagnosis. Elsevier-Saunders, Philadelphia, 1st edition, 2005 P-S collaterals / Omental varices Transverse view with linear transducer (7-MHz) Omental varices just beneath abdominal wall
    25. 25. P-S collaterals / Spleno-retroperitoneal collateral Prominent varices surrounding posterior aspect of spleen Owen C et al. J Diag Med Sonography 2006 ; 22 : 317 – 328.
    26. 26. Cirrhosis & PHT / Diameter of portal vein 1 Weinreb J et al. Am J Roentgenol 1982 ; 139 : 497 – 499. 2 Goyal AK et al. J Ultrasound Med 1990 ; 9 : 45 – 48. Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13. Diameter: 16.9 mm Sign of portal hypertension Longitudinal view of MPV Contoversy on normal PV diameter Up to 13 mm in one study1 Up to 16 mm in another study2 Unusual large PV: good sign of PHT Normal PV size: do not exclude PHT
    27. 27. Cirrhosis & PHT / Portal vein velocity Low velocity: good indicator of PHT Normal velocity: do not exclude PHT Controversy on normal PV velocity Difficult to rely on velocity for dg Normal mean velocity: 15 – 18 cm/sec Swart J et al. Ultrasound Clin 2007 ; 2 : 355 – 375. Shrunken liver & irregular margin Vmax: 10 cm/s Diagnosis of PHT Triplex image of PV
    28. 28. Portal vein pseudoclot – Incorrect velocity Cirrhotic patient with portal hypertension Slower flow in portal vein demonstrated Velocity scale: 7 cm/s Rubens DJ et al. Ultrasound Clin 2006 ; 1 : 79 – 109. Velocity scale: 20 cm/s Good flow in HA anteriorly No flow in adjacent PV
    29. 29. Cirrhosis & PHT / Portal vein flow Normal flow Kok Th et al. Scand J Gastroenterol 1999 ; 34 (Suppl 230) : 82 – 88. Reversed flow Advanced PHT SOS Porto-systemic shunt To and fro flow Advanced PHT Heart failure Arterio-portal fistula
    30. 30. Cirrhosis & PHT / To-and-fro flow in PV Cardiac cycle Hepatopetal & hepatofugal with each heart beat Seen before frank hepatofugal flow Wachsberg RH et al. RadioGraphics 2002 ; 22 : 123 – 140. Duplex US of LPV during suspended respiration
    31. 31. Cirrhosis & PHT / To-and-fro flow in PV Respiratory cycle Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13. On real-time US, these alterations corresponded to respiratory cycle Transverse color Doppler US of left portal vein Hepatopetal flow Hepatofugal flow
    32. 32. Cirrhosis & PHT / To-and-fro flow in PV Compression Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13. Transverse CDUS of left portal vein Hepatopetal flow Hepatofugal flow
    33. 33. Causes of to-and-fro flow Exaggerated pulsatility Minimum velocity below baseline Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13. - Portal hypertension - Tricuspid regurgitation - Right heart failure - Aerterio-portal vein fistula
    34. 34. Cirrhosis & PHT / Reversed flow of PV Hepatopetal flow in HA & hepatofugal flow in PV Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13. Not pathognomonic feature of cirrhosis Severe PHT – Rare
    35. 35. Hepatopetal flow in HA Hepatofugal flow in PV Color Doppler of peripheral liver Arterial flow above baseline Portal venous below baseline Duplex Doppler of same area Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13. Cirrhosis & PHT / Reversed flow in PV branches
    36. 36. Cirrhosis & PHT / Reversed flow in PV branches Mansour MA et al. Vascular Diagnosis. Elsevier-Saunders, Philadelphia, 1st edition, 2005. Right anterior PV branch Hepatofugal flow Right posterior PV branch Hepatopetal flow
    37. 37. Hepatofugal flow in portal vein Portal vein flow away from liver • Cirrhosis • Budd-Chiari syndrome & SOS • TIPS • Arterio-portal fistula Tumor: HCC – Hemangioma Percutaneous liver biopsy Percutaneous biliary drainage Rupture vein aneurysm Rendu-Osler-Weber disease Hwang HJ et al. J Clin Ultrasound 2009 ; 37 : 511 – 524.
    38. 38. Hepatofugal portal / TIPS Right portal vein to right hepatic vein Hwang HJ et al. J Clin Ultrasound 2009 ; 37 : 511 – 524. Reversion of hepatofugal flow Stent devoid of color signals Malfunction of TIPS 1 week after TIPS Hepatofugal flow in RPV Vigorous color flow in stent Immediately after TIPS
    39. 39. Arterio-portal fistula / High-flow hemangioma Hwang HJ et al. J Clin Ultrasound 2009 ; 37 : 511 – 524. 65-year-old man with high-flow hemangioma in LLL Hypoechoic nodule with intratumoral flow Peritumoral hepatofugal flow in segmental PV Hepatopetal flow in proximal PV
    40. 40. Arterio-portal fistula / Post-liver biopsy Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538. Vascular lesion between HA & PV branches Inverted flow in PV Oblique CDUSOblique gray-scale US Focal echogenic area in region of biopsy Spectral Doppler US High-velocity flow Low-resistance flow Turbulent flow
    41. 41. Arterio-portal fistula / Rendu-Osler-Weber Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538. Low-resistance arterial flow Arterialized & inverted PV flow Dilated tortuous structures Dilated vascular structures with aliasing
    42. 42. Helical portal vein flow Near bifurcation • Normal subjects 2% • Severe liver disease 20% • TIPS • Post-liver transplantation Donor PV > recipient PV • Portal vein stenosis Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
    43. 43. Helical portal vein flow If not properly recognized, it can produce the mistaken impression of PV flow reversal Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
    44. 44. Helical portal vein flow Mimic of hepatofugal flow Wachsberg RH et al. RadioGraphics 2002 ; 22 : 123 – 140. Hepatopetal flow within liver confirms that net flow is hepatopetal
    45. 45. Cirrhosis & PHT / Prominent hepatic artery Enlarged HA with tortuous or „„corkscrew‟‟ appearance Increased flow in HA to compensate decreased flow in PV Swart J et al. Ultrasound Clin 2007 ; 2 : 355 – 375.
    46. 46. Causes of enlargement of hepatic artery • Cirrhosis • Hepatic diseases associated with alcoholism • Congenital hepatic fibrosis • Vascular tumors • Hereditary hemorrhagic telangiectasia Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55.
    47. 47. Parallel channel sign von Herbay A et al. J Clin Ultrasound 1999 ; 27 : 426 – 432. Gray-scale US IH parallel channel sign Suspicious of dilated IHBD Color & pulsed Doppler US Flow in both intra-hepatic lumina Portal vein & hepatic artery Absence of dilated intra-hepatic bile duct
    48. 48. Parallel channel sign von Herbay A et al. J Clin Ultrasound 1999 ; 27 : 426 – 432. Gray-scale US IH parallel channel sign Suspicious of dilated IHBD Color & pulsed Doppler US Blood flow in anterior structure No flow in posterior structure Confirmation of dilated intra-hepatic bile duct
    49. 49. Cirrhosis & PHT / Changes of hepatic artery flow Kok Th et al. Scand J Gastroenterol 1999 ; 34 (Suppl 230) : 82 – 88. Decreased diastolic flow ESLD Reversed diastolic flow ESLD Normal flow Normal in most patients
    50. 50. Cirrhosis & PHT / Pulsatility index of HA Cirrhotic patients vs controls – Correlation with HVPG Schneider AW et al. J Hepatol 1999 ; 30 : 876 – 881. PI: 0.85 20 controls 0.92 ± 0.1 PI: 1.22 50 cirrhotic patients 1.14 ± 0.18 P< 0.001 Directly correlated with HVPG
    51. 51. Cirrhosis & PHT / Changes of hepatic vein flow Kok Th et al. Scand J Gastroenterol 1999 ; 34 (Suppl 230) : 82 – 88. Triphasic Biphasic Cirrhosis Budd-Chiari syndrome Metastases Ascites Healthy subjects Monophasic Cirrhosis Budd-Chiari syndrome Metastases Ascites Healthy subjects
    52. 52. Damping index of HV waveform Severe portal hypertension : HVPG > 12 mmHg Kim MY et al. Liver International 2007 ; 27 : 1103 – 1110. Minimum velocity of downward HV Maximum velocity of downward HV Damping index = Normal value: < 0.6 Severe portal hypertension: ≥ 0.6
    53. 53. Damping index of HV waveform in cirrhosis DI: 0.26 HVPG: 7 mmHg DI: 0.72 HVPG: 15 mmHg Kim MY et al. Liver International 2007 ; 27 : 1103 – 1110. DI of 0.6: Sen 76%, Sp 82, & AUC 0.86 for severe PHT
    54. 54. Doppler in cirrhosis / PHT Prognostic implications • Collaterals PUV High bleeding risk in surgery Reversed LGV High bleeding risk of EV S-R shunt Low bleeding risk of EV • Portal vein Low flow High risk of HE Inversed flow CI for TIPS & porto-caval shunt Congestion index High bleeding risk of EV • Hepatic artery Increased PI ESLD • Hepatic vein Monophasic ESLD Increased DI Severe PHT (> 12 mmHg)
    55. 55. Transjugular Intrahepatic Portosystemic Shunt TIPS Highly effective for – Reducing ascites – Recurrent variceal hemorrhage – Improving quality of life High rate of stenosis or thrombosis High rate of hepatic encephalopathy
    56. 56. Normal Doppler parameters for TIPS • Portal vein Hepatopedal flow – Velocity > 30 cm/sec • IHPV Hepatofugal flow • Hepatic artery Increased PSV • Stent Flow completely filling the stent Monophasic pulsatile flow Vmin: 90 cm/sec – Vmax: 190 cm/sec Vmax – Vmin: 50 – 100 cm/sec Temporal changes: ↑ or ↓ less 50 cm/sec Middleton WD et al. Ultrasound Quarterly 2003 ; 19 : 56 – 70.
    57. 57. Follow-up of TIPS by Doppler US • 24 to 48 hours (baseline) • 3 months • 6 months • 12 months • Annually thereafter Middleton WD et al. Ultrasound Quarterly 2003 ; 19 : 56 – 70. Real goal of surveillance Detect stenosis before complete thrombosis
    58. 58. TIPS / Normal Middleton WD et al. Ultrasound Quarterly 2003 ; 19 : 56 – 70. Stent within liver parenchyma Hepatopetal flow in MPV Hepatofugal flow in RPV Color Doppler of TIPS Color & pulsed Doppler of TIPS Monophasic pulsatile flow Velocity: 106 cm/sec
    59. 59. TIPS / Mirror image artifact If not recognized: migration into heart (emergency intervention) If uncertainty persists: chest radiograph Wachsberg RH. Ultrasound Quarterly 2003 ; 19 : 139 – 148. Stent on either side of diaphragm Mirror image artifact Variant of mirror image artifact Stent above diaphragm True TIPS visible by rotating probe
    60. 60. TIPS / migration Middleton WD et al. Ultrasound Quarterly 2003 ; 19 : 56 – 70. Proximal portion migrated out of PV into parenchymal tract This resulted in complete thrombosis of stent Longitudinal view of TIPS
    61. 61. TIPS – Stenosis Middleton WD et al. Ultrasound Quarterly 2003 ; 19 : 56 – 70. Mid TIPS Mean portal vein Right portal vein Mid TIPS Distal TIPS Vel 26 cm/sec Aliasing 371 cm/sec 98 cm/sec Hepatopetal flow
    62. 62. TIPS / occlusion Ricci P et al. J Ultrasound 2007 ; 10 : 22 – 27. Homogeneous hyperechoic intraluminal material without any color flow within TIPS
    63. 63. Doppler of the portal system  Portal hypertension  Portal vein thrombosis  Budd-Chiari syndrome & SOS*  Suspicion of cardiac disease  Characterization of hepatic mass  Miscellaneous * SOS: Sinusoidal Obstruction Syndrome
    64. 64. Portal vein thrombosis • Etiology Extra-hepatic: multiple causes Cirrhosis ± HCC: complete – partial Budd-Chiary syndrome: 15% – poor prognosis • Sensibility Equal to CT – Power Doppler increase Sen • False positive Very low portal flow • Partial Gray scale better than color Doppler • Indications Before hepatic surgery Before porto-caval shunt Before hepatic transplantation
    65. 65. Splenic vein thrombosis Gastric cancer
    66. 66. Superior mesenteric vein thrombosis Pancreatic cancer Sagittal view of pancreas & SMV Thrombosed SMV Mass in Pancreatic neck Shunt between SMV & systemic venous return http://www.sonographers.ca
    67. 67. Superior mesenteric vein thrombosis Transverse image of SMA & SMV http://www.ultrasoundcases.info SMA SMV
    68. 68. Intestinal infarction Considered from presentation until resolution of pain • Ascites • Thinning of intestinal wall • Lack of mucosal enhancement of thickened wall • Development of multi-organ failure Intestinal infarction is likely Surgical exploration should be considered
    69. 69. Ultrasound in ischemic bowel Thickening of small bowel wall Loss of layering structure of wall Chen MJ et al. J Med Ultrasound 2006 ; 14 : 79 – 85. Thickening of small bowel wall Bright flecks within the wall
    70. 70. Portal vein gas Acute transmural mesenteric infarction Tritou I et al. J Clin Ultrasound 2011 (in press). Wiesner W et al. Radiology 2003 ; 226 : 635 – 650. Intrahepatic PV gas in periphery of both lobes CECT scan Tiny echogenic foci in liver parenchyma Gray-scale US Vertical bidirectional spikes on PV waveform Duplex of MPV
    71. 71. Acute thrombosis of portal vein Complete thrombosis http://www.sites.tufts.edu Echogenic material visualized within portal vein Increased diameter of portal vein
    72. 72. Partial thrombosis of portal vein Echogenic material occluding lumen of PV by ≈ 50% Sacerdoti D et al. J Ultrasound 2007 ; 10 : 12 – 21.
    73. 73. Partial thrombosis of portal vein Swart J et al. Ultrasound Clin 2007 ; 2 : 355 – 375. Black & white ultrasound Partial echogenic thrombus Color & pulsed Doppler Complete filling of main PV obscuring the clot
    74. 74. Non-malignant PV thrombosis in cirrhosis Systematic review – Many unresolved issue • Incidence 10 – 25% • Pathophysiology Cirrhosis no longer hypocoagulable state • Clinical findings Asymptomatic disease Life-threatening condition • Management Not addressed in any consensus publication 1st line treatment: warfarin or LMWH 2nd line treatment: thrombectomy, TIPS Tsochatzis EA et al. Aliment Pharmacol Ther 2010; 31 : 366 – 374.
    75. 75. Diagnosis of malignant PV thrombosis • Color Doppler US* PV > 23 mm in diameter “AASLD” Arterial-like flow on Doppler Increased serum α-FP • FNA CT- or US-guided • CEUS Contrast-Enhanced Ultrasound * DeLeve L et al. AASLD practice guidelines: Vascular disorders of the liver. Hepatology 2009 ; 49 : 1729 – 1764.
    76. 76. Portal vein thrombus in HCC Swart J et al. Ultrasound Clin 2007 ; 2 : 355 – 375. FNA of portal vein thrombus confirmed HCC Gray-scale US image Thrombus in PV & its branches Color Doppler image Vascularity within thrombus Low-resistance arterial waveform
    77. 77. Malignant PV thrombosis / CEUS 38 pts (15 benigns - 23 malignants) – Conclusive (37/38) Dănilă M et al. Medical Ultrasonography 2011 ; 13 : 102 – 107. Gray-scale US Malignant PVT Arterial phase Enhancement Portal phase Wash-out Late phase Wash-out Contrast-Enhanced US
    78. 78. Portal vein pseudoclot – Augmentation Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13. Color Doppler US of main portal vein At rest No detectable flow Compression of lower abdomen Augmented portal venous flow
    79. 79. Portal vein pseudoclot – Incorrect angle Velocity: 24 cm/sec Wall filter: medium Angle 90° Velocity: 7 cm/sec Wall filter: medium Angle < 90° Radiol Clin N Am 2006 ; 44 : 805 – 835.
    80. 80. Chronic portal vein thrombosis Portal cavernoma Parikh et al. Am J Med 2010 ; 123 : 111 – 119. Hepatopetal collaterals around thrombosed portal vein
    81. 81. Portal cavernoma Gray-scale ultrasound Color & pulsed Doppler
    82. 82. Tchelepi H et al. Ultrasound Clin 2007 ; 2 : 415 – 422. Transverse color US of stomach Multiple dilated gastric varices P-S collaterals / Isolated gastric varices Collaterals via short gastric veins Isolated gastric varices Hepatopetal flow in LGV Splenic vein thrombosis
    83. 83. P-S collaterals / Transcapsular collaterals Chronic PVT due to necrotizing pancreatitis or surgery Seeger M et al. Radiology 2010 ; 257 : 568 – 578. Transcapuslar collateral from SB varices to PVs Color Doppler image Submucosal varices in small-bowel loop US image Ectopic intestinal varices & transcapsular collaterals Schematic diagram
    84. 84. Portal cholangiopathy • Definition Biliary & GB abnormalities in EHPVO • Frequency 70 – 100% (symptomatic or not) • Mechanism Mechanical extrinsic compression Biliary ischemic injury • Manifestation Majority asymptomatic RUQ quadrant pain Cholestasis & cholangitis Secondary biliary cirrhosis • Management Directed to symptomatic patients only Besa C et al. Abdom Imaging 2011 (in press).
    85. 85. Portal cholangiopathy Biliary & GB wall abnormalities in EHPVO Gallbladder varices producing wall thickening Cavernoma of portal vein Associated with dilated bile ducts Besa C et al. Abdom Imaging 2011 (in press). Cholangiographic images essential to confirm diagnosis
    86. 86. MRC in portal cholangiopathy Besa C et al. Abdom Imaging 2011 (in press). Multiple smooth strictures due to extrinsic compression Stenosis with marked dilatation of proximal biliary tree Differentiate from bile duct cancerDifferentiate from PSC
    87. 87. Doppler of the portal system  Portal hypertension  Portal vein thrombosis  Budd-Chiari syndrome & SOS*  Suspicion of cardiac disease  Characterization of hepatic mass  Miscellaneous * SOS: Sinusoidal Obstruction Syndrome
    88. 88. Budd-Chiari syndrome Narayanan Menon KV et al. N Engl J Med 2004 ; 350 : 578 – 85. Occlusion of a single hepatic vein usually clinically silent Two or three hepatic veins can be occluded without clear symptoms
    89. 89. Diagnosis of BCS AASLD practice guidelines* • Doppler US Most effective & reliable diagnostic mean Experienced examiner aware of dg suspicion • MRI or CT Confirmatory study Experienced Doppler examiner not available • Liver biopsy Diagnosis not done by non-invasive imaging • Venography When diagnosis remains uncertain * DeLeve L et al. AASLD practice guidelines: Vascular disorders of the liver Hepatology May 2009 ; 49 : 1729 – 1764.
    90. 90. Doppler US in BCS Obstruction &/or collaterals of HV or IVC* * DeLeve L et al. AASLD practice guidelines. Hepatology 2009 ; 49 : 1729 – 1764. Obstructed HV Presence of solid endoluminal material Hyperechoic cord replacing normal vein Reversed flow in large hepatic vein Dilatation of vein upstream to obstacle HV collaterals Sipder web in vicinity of HV ostia Subcapsular or HV to intercostal or HV veins Caudate lobe hypertrophy with dilated veins IVC Web – Thrombosis – Inversion of flow
    91. 91. BCS / Solid endoluminal material in HV Solid endoluminal material in middle & left hepatic veins Narrowing at distal end of middle hepatic vein as it joins IVC Chaubal N et al. J Ultrasound Med 2006 ; 25 : 373 – 379. Transverse subcostal image
    92. 92. BCS / Hyperechoic cord Hepatic veins transformed to fibrotic cords “Hepatic vein star” Boozari B et al. J Hepatol 2008 ; 49 : 572 – 580.
    93. 93. BCS / Reversed flow in large HV Inverted flow in right hepatic vein Normal flow in middle hepatic vein Right intercostal view Bargalló X et al. Am J Roentgenol 2006 ; 187 : W33 – W42.
    94. 94. BCS / Reversed flow in HV upstream to obstacle Solid endo-luminal material in distal part of MHV Reverse flow in proximal part of MHV
    95. 95. BCS / Sipder web in vicinity of HV ostia Vilgrain V. Eur Radiol 2001 ; 11 : 1563 – 1577. Segev D L. Liver Transpl 2007 ; 13 : 1285 – 1294. Gray-scale US Small interwoven veins near IVC Hepatic venogram Typical “spider web” pattern
    96. 96. BCS / Large subcapsular vein Large tortuous subcapsular vein draining into IVC Bargalló X et al. Am J Roentgenol 2006 ; 187 : W33 – W42.
    97. 97. BCS / HV draining into another HV Occluded RHV draining through collateral vessel into MHV Flow away & toward transducer in same vessel “Bicolored hepatic vein” Bargalló X et al. Am J Roentgenol 2006 ; 187 : W33 – W42.
    98. 98. BCS / Collateral from HV to caudate lobe vein Brancatelli G et al. Am J Roentgenol 2007 ; 188 : W168 – W176. Transverse Doppler US at level of caudate lobe Lack of flow in distal portion of MHV Collateral from MHV to caudate lobe vein
    99. 99. BCS / Suggestive intra-hepatic collateral Erden A. Eur J Radiol 2007 ; 61 : 44 – 56. Undulated course “h-shaped” Hockey-stick Curvilinear Curved
    100. 100. BCS / Caudate lobe hypertrophy Erden A. Eur J Radiol 2007 ; 61 : 44 – 56. Sagittal gray-scale US Enlarged caudate lobe Antero-posterior diameter: 7.6 cm
    101. 101. BCS / Dilated caudate lobe vein 75% of cases Bargalló X et al. Am J Roentgenol 2003 ; 181 : 1641 – 1645. Mildly dilated caudate vein 7 mm Largely dilated caudate vein 21 mm Caudate vein (≥ 3 mm) suggests diagnosis Except for cardiac failure
    102. 102. BCS / Membranous obstruction of IVC Kandpal H et al. RadioGraphics 2008 ; 28 : 669 – 689. 30-year-old woman, abdominal pain & distention of 3 y duration Ostial HV narrowing Multiple IH collaterals Tapered IVC occlusion at cavo-atrial junction Reversed flow in IVC Loss of cardiac pulsations
    103. 103. Budd-Chiari syndrome & liver hydatid disease Retrospective study of 13 patients with HDL & BCS Yilmaz C et al. Radiol Oncol 2009 ; 43 : 225 – 232. Heterogeneous mass representing degenerated & collapsed membranes Large subcapsular vein draining into suprahepatic IVC
    104. 104. BCS / IVC thrombosis Behçet disease – Secondary BCS Sagittal image of IVC distended with echogenic thrombus Secondary BCS due to renal cell carcinoma Rumack CM, Wilson SR, & Charboneau JW. Diagnostic Ultrasound. Elsevier-Mosby, St. Louis, Missouri, USA, 3rd edition, 2005.
    105. 105. BCS / Thrombosis of PV 15% of patients – Poor prognosis Bargalló X et al. Am J Roentgenol 2006 ; 187 : W33 – W42. Thrombosis of portal vein Hepatofugal flow in right portal vein Dilated hepatic artery
    106. 106. BCS / Benign regenerative nodules Multiple (> 10) – Small ( < 4 cm) – Hypervascular Vilgrain V et al. Radiology 1999 ; 210 : 443 – 450. Bargalló X et al. Am J Roentgenol 2006 ; 187 : W33 – W42. Two iso- & hyperechoic nodules surrounded by thin hypoechoic halo Low resistance arterial waveform with high velocity
    107. 107. Proposed diagnostic strategy for BCS Valla DC. Gut 2008 ; 57 : 1469 – 1478. DeLeve L et al. AASLD practice guidelines. Hepatology 2009 ; 49 : 1729 – 1764.
    108. 108. Doppler US in SOS Non specific • Main PV Decreased, to-and-fro, or reversed flow • Hepatic artery Significant elevation of RI (> 0.80) • Hepatic veins Normal direction – Monophasic flow • IVC Patent with flow toward heart McGahan J et al. Diagnostic ultrasound, Informa Healthcare, 2nd edition, 2008. * Lassau N et al. Radiology 1997 ; 204 : 545 – 552. • US findings Thickened GB wall – Ascites
    109. 109. Sinusoidal obstruction syndrome (SOS) BMT for acute myelogenous leukemia Desser TS et al. Am J Roentgenol 2003 ; 180 : 1583 – 1591. Contrast-enhanced CT Heterogeneous hepatic enhancement Color & duplex US of HV Monophasic flow in MHV Imaging currently not diagnostic by itself
    110. 110. Doppler of the portal system  Portal hypertension  Portal vein thrombosis  Budd-Chiari syndrome & SOS*  Suspicion of cardiac disease  Characterization of hepatic mass  Miscellaneous * SOS: Sinusoidal Obstruction Syndrome
    111. 111. Respiratory variation of IVC Berzigotti A & Piscaglia F. Ultraschall Med 2011 ; 32 : 548 – 571. Dilated IVC diameter (> 20 mm) Absence of normal respiratory variation of IVC diameter Being similar in expiration & inspiration Longitudinal scan of IVC
    112. 112. Tricuspid regurgitation Type 1 (mild) S wave < D wave Scheinfeld MH et al. RadioGraphics 2009 ; 29 : 2081 – 2098. Type 2 (moderate) No flow in ventricular systole Type 3 (severe) Retrograde flow in ventr systole
    113. 113. Combined tricuspid regurgitation & right-sided HF Biphasic waveform Scheinfeld MH et al. RadioGraphics 2009 ; 29 : 2081 – 2098. Retrograde A, S, & V waves combined to form a single wave Single antegrade wave & single retrograde wave
    114. 114. Constrictive pericarditis Retrograde pulse between D & A Scheinfeld MH et al. RadioGraphics 2009 ; 29 : 2081 – 2098. Typical of constrictive pericarditis CT scanSchematic drawing Pulsed Doppler
    115. 115. Severe right heart failure Increased pulsatility in portal vein Dietrich CF. Ultrasound of the liver. EFSUMB – European Course Book Cardiac dysfunction if Vmin reaches 0 or reversed Correlate PV waveforms with HV waveforms
    116. 116. Doppler of the portal system  Portal hypertension  Portal vein thrombosis  Budd-Chiari syndrome & SOS*  Suspicion of cardiac disease  Characterization of hepatic mass  Miscellaneous * SOS: Sinusoidal Obstruction Syndrome
    117. 117. Brannigan M et al. RadioGraphics 2004 ; 24 : 921 – 935. Hemangioma / CEUS Baseline US Arterial phase Peripheral enhancement Sustained enhancement Portal vein phase
    118. 118. Focal nodular hyperplasia Venturi A et al. J Ultrasound 2007 ; 10 : 116 – 127. “spoke-wheel pattern” Arteries radiating toward periphery Color Doppler US Power Doppler US
    119. 119. Brannigan M et al. RadioGraphics 2004 ; 24 : 921 – 935. Focal nodular hyperplasia / CEUS Arterial phase Hypervascular mass Stellate vascular pattern Equal to liver enhancement Central nonenhancing scar Portal vein phaseBaseline US Hypoechoic mass in caudate lobe
    120. 120. Brannigan M et al. RadioGraphics 2004 ; 24 : 921 – 935. Hepato-cellular carcinoma / CEUS Baseline US Arterial phase More echogenic Hypervascular mass Less echogenic “Washed out” Portal phase
    121. 121. Brannigan M et al. RadioGraphics 2004 ; 24 : 921 – 935. Hepatic metastasis / CEUS Arterial phase US image Less echogenic Hypovascular masses Metastatic colon cancer Baseline conventional US Very subtle lesion
    122. 122. Doppler of the portal system  Portal hypertension  Portal vein thrombosis  Budd-Chiari syndrome & SOS*  Suspicion of cardiac disease  Characterization of hepatic mass  Miscellaneous * SOS: Sinusoidal Obstruction Syndrome
    123. 123. Miscellaneous • Biliary atresia • Portal vein aneurysm • Vascular shunts Porto-systemic PV to HV Arterio-portal HA to PV Arterio-systemic HA to HV Portal-to-portal PV to PV Systemic-to-systemic HV to HV • HHT Hereditary Hemorrhagic Telangiectasia • TAPVR Total Anomalous Pulmonary Vein Return * Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538.
    124. 124. US of biliary atresia High-spatial resolution real-time US • Typical GB absent or small (< 15 mm) Triangular cord sign (EARPV > 4 mm) Hepatic subcapsular flow • Others IHBD or CBD not dilated Associated choledochal cyst Cyst in porta hepatis (10%) Pre-duodenal portal vein Interrupted IVC: does not join right atrium Situs inversus EARPV: Echogenic Anterior wall of Right Portal Vein Lee MS et al. Radiology 2009 ; 252 : 282 – 289.
    125. 125. Biliary atresia 29 biliary atresia – 35 neonatal cholestasis – 19 controls EARPV: Echogenic Anterior wall of Right Portal Vein Lee MS et al. Radiology 2009 ; 252 : 282 – 289. GB length 1.4 cm Hepatic subcapsular flowPositive TC sign EARPV: 4 mm Sen: 62% Sp: 100% Sen: 100% Sp: 86%
    126. 126. Portal vein aneurysm Prevalence: 0.6 per 1 000 persons • Sites Confluence – Main PV – Intra-hepatic PV • Pathogenesis Congenital: Marfan – Cutis laxa Acquired: CLD, PHT, pancreatitis, trauma • Diagnosis Extra-hepatic: focal dilatation > 21 mm Intra-hepatic: focal dilatation > 9 mm • Manifestation Small: asymptomatic Large: thrombosis, rupture, compression • Treatment Follow-up – anticoagulation – PC treatment Koc Z et al. Am J Roentgenol 2007 ; 189 : 1023 – 1030.
    127. 127. Portal vein aneurysm Swart J et al. Ultrasound Clin 2007 ; 2 : 355 – 375. Bidirectional flow “yin yang sign” Color Doppler US Focal dilatation of PV 4 cm in diameter Gray-scale US Duplex Doppler US Portal venous type flow Bidirectional flow
    128. 128. Thrombosed portal vein aneurysm Santana P et al. J Ultrasound Med 2002 ; 21: 701 – 704. Power Doppler USTransverse US of SP confluence No flow within aneurysmGiant portal vein aneurysm SV
    129. 129. Aneurysm of portal vein bifurcation Koc Z et al. Am J Roentgenol 2007; 189 : 1023 – 1030. Diameter of portal vein bifurcation 21 mm Significantly larger than remaining segments of same vein Turbulent flow
    130. 130. Porto-systemic shunt Portal vein to hepatic vein Tri- or biphasic wave in PV suggests diagnosis Cirrhosis – Trauma – Aneurysm – Congenital – HHT
    131. 131. Porto-systemic shunt / Cirrhosis Most frequent cause – Associated with HE Direct communication between portal vein & hepatic vein Power Doppler US PV HV Lane MJ et al. Am J Roentgenol 2000 ; 174 : 125 – 131.
    132. 132. Congenital extrahepatic porto-systemic shunts Abernethy malformation ( 1793) Type I Absence of intra-hepatic portal veins Ia SMV & SV drain separately into IVC Ib SMV & SV form common trunk drained by IVC Type II Hypoplastic intra-hepatic portal veins Morgan G et al. J Pediatr Surg 1994 ; 29 : 1239 – 1241.
    133. 133. Schematic drawing of Abernethy malformation Hu GH et al. World J Gastroenterol 2008 ; 14 : 5969 – 5979. Type IIType I bType I a
    134. 134. Abernethy malformation type Ib Konstasa AA et al. Eur J Radiol 2010 (in press). Color Doppler US Direct emptying of portal vein into IVC CECT Direct emptying of portal vein into IVC Biopsy (portal space) Presence of bile duct & HA Absence of PV
    135. 135. Congenital intra-hepatic porto-systemic shunts Park’s classification (1990) Type 1 Single tubular connection between RPV & IVC Type 2 Multiple peripheral shunts within one segment Type 3 Porto-systemic shunt through PV aneurysm Type 4 Multiple peripheral shunts diffusely in 2 lobes Park JH et al. Am J Roentgenol 1990 ; 155 : 527 – 8. Type 5 Persistent ductus venosus (considered 5th type)
    136. 136. Park classification – Type 1 Single tubular connection between RPV & IVC Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538. Large vascular channel connecting RPV & MHV RHV MHV Channel Oblique color Doppler US
    137. 137. Park classification – Type 2 Multiple peripheral shunts within one segment De Gaetano AM et al. Abdom Imaging 2007 ; 32 : 463 – 469. Gray-scale US Multiple serpiginous vessels in segment 3 between LPV & LHV Waveform within shunt Continuous portal-like flow Waveform of LHV Turbulent high velocity flow
    138. 138. De Gaetano AM et al. Abdom Imaging 2007 ; 32 : 463 – 469. Park classification – Type 3 Porto-systemic shunt through PV aneurysm Normal triphasic pattern Waveform of HV branch Phasic pattern Transmission from heart Waveform of PV branch Aneurysm between PV & HV branches Color Doppler US
    139. 139. Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538. Monophasic portal flow Triphasic HV flow Spectral Doppler of shuntIntercostal RLL Portal branch opens into peripheral HV Transverse LLL Portal branches open into peripheral HV Park classification – Type 4 Multiple peripheral shunts diffusely in both lobes
    140. 140. Patent ductus venosus 17 reported cases in children* * Yoshimoto Y et al. J Pediatr Surg 2004 ; 39 : E1 – E5. Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538. Venous duct connecting left portal vein & IVC Oblique color Doppler US IVC MPV LPV DV
    141. 141. Arterio-portal shunt Hepatic artery to portal vein Low resistance flow in HA & arterialization of PV Trauma – Cirrhosis – Aneurysm – Congenital – HHT
    142. 142. Arterio-portal shunt / Aneurysm Dilated splenic artery 14 mm Dilated splenic vein 22 mm Splenic hilum SV aneurysm
    143. 143. Arterio-portal shunt / Aneurysm Splenic artery in hilum RI: 0.41 Intra-splenic artery RI: 0.61 Splenic vein in hilum Arterialisation
    144. 144. Arterio-portal shunt / Splenic aneurysm Splenic arterio-venous fistula Complication of splenic vein aneurysm Arteriography
    145. 145. Arterio-systemic shunt Hepatic artery to hepatic vein HCC – Large hemangioma – HHT
    146. 146. Arterio-systemic shunt / HHT Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55. Dilated hepatic artery branch & right hepatic vein Shunt of hepatic artery branch to right hepatic vein
    147. 147. Portal-to portal shunt Portal vein to portal vein Rare – Cirrhosis
    148. 148. Portal-to portal shunt / Cirrhosis Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538. Cirrhotic patient with portal cavernoma Shunt between cavernoma & ascending portion of LPV Transverse CDUS LPV Shunt Cavernoma
    149. 149. Systemic-to systemic shunt Hepatic vein to hepatic vein BCS – HHT
    150. 150. Systemic-to-systemic shunt / HHT Oblique color Doppler US Large communication between right & middle hepatic veins RHV MHV Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538.
    151. 151. Hereditary Hemorrhagic Telangiectasia HHT
    152. 152. Curaçao diagnostic criteria for HHT 1. Epistaxis Spontaneous, recurrent nose bleeds 2. Telangiectases Multiple, at characteristic sites: Lips, oral cavity, nose, fingers 3. VMs GI, pulmonary, hepatic, cerebral, spinal 4. Family history First degree relative with HHT Definite diagnosis If three criteria are present Possible diagnosis If two criteria are present Unlikely If fewer than two criteria are present Criteria Shovlin CL et al. Am J Med Genet 2000 ; 91 : 66 – 7.
    153. 153. US Doppler findings in HHT Prevalence of hepatic involvement: at least 33% • HA Dilated main HA & its branches High-velocity flow (aliased or turbulent) • Vms Porto-systemic shunt Arterio-portal shunt Arterio-systemic shunt Systemic-to-systemic shunt Liver biopsy should be avoided (high risk of bleeding) Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55.
    154. 154. HHT / Dilated main hepatic artery Buscarini E et al. Dig Liver Dis 2005 ; 37 : 635 – 645. Dilated hepatic artery: 8.7 mm Measured 2 cm distal to its origin from celiac trunk
    155. 155. Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55. HHT / Dilated HA branches – Straight Transverse US of left lobe “double channel sign” Dilated hepatic artery branch Power Doppler US
    156. 156. HHT / Dilated HA branches – Tortuous Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55. Power Doppler USGray-scale US
    157. 157. HHT / IH arterial hypervascularization Buscarini E et al. Dig Liver Dis 2005 ; 37 : 635 – 645. Tortuous small arterial branches Color Doppler USGray-scale US No abnormality demonstrated
    158. 158. Slightly dilated HA: 6.2 mm High PSV: 129 cm/sec Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55. HHT / High velocity flow in HA Duplex US of hepatic artery
    159. 159. US criteria for hepatic involvement in HHT Criteria Major criteria Dilated common hepatic artery (> 7 mm) Intrahepatic arterial hypervascularization Caselitz M et al. Hepatology 2003 ; 37 : 1139 – 1146. Diagnosis: two major criteria or one major with two minor Minor criteria PSV of proper hepatic artery > 110 cm/s RI of proper hepatic artery < 0.60 Vmax of portal vein > 25 cm/s Tortuous course of extrahepatic hepatic artery Facultative findings Dilated portal vein (> 13 mm) Dilated liver veins (> 11 mm) Hepatomegaly (> 15 cm in MCL) Nodular liver margin
    160. 160. Total Anomalous Pulmonary Venous Return TAPVR
    161. 161. Total Anomalous Pulmonary Venous Return TAPVR Infradiaphragmatic TAPVR the least common type Pulmonary veins to left HV or left PV by large common channel Flow almost always inhibited by one or more stenoses at this channel Gallego C et al. RadioGraphics 2004 ; 24 : 755 – 772.
    162. 162. Infra-diaphragmatic TAPVR Newborn with cyanosis & respiratory distress CPV: Common Pulmonary Vein Gallego C et al. RadioGraphics 2004 ; 24 : 755 – 772. Transverse US of liver Stenosis of CPV just before it enters LHV A LHV Stenosis CPV Longitudinal midline US Vessel coming from thorax with flow as in aorta CPV A Aliasing in stenotic segment of CPV & LHV Oblique image of liver A CPV LHV
    163. 163. Thank You

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