Three-Dimensional Printing Facilitates Successful Endovascular Closure of a Type II Abernethy Malformation Using an Amplatzer Atrial Septal Occluder Device

Accepted Manuscript Three-Dimensional Printing Facilitates Successful Endovascular Closure of a Type II Abernethy Malformation Using an AMPLATZER Atrial Septal Occluder Device Jeffrey Forris Beecham Chick, MD, MPH, DABR, Shilpa N. Reddy, MD, Alice C. Yu, BS, Tatiana Kelil, MD, Ravi N. Srinivasa, MD, Kyle J. Cooper, MD, Wael E. Saad, MBBCh, FSIR PII:

S0890-5096(17)30014-6

DOI:

10.1016/j.avsg.2017.02.012

Reference:

AVSG 3282

To appear in:

Annals of Vascular Surgery

Received Date: 7 January 2017 Accepted Date: 4 February 2017

Please cite this article as: Beecham Chick JF, Reddy SN, Yu AC, Kelil T, Srinivasa RN, Cooper KJ, Saad WE, Three-Dimensional Printing Facilitates Successful Endovascular Closure of a Type II Abernethy Malformation Using an AMPLATZER Atrial Septal Occluder Device, Annals of Vascular Surgery (2017), doi: 10.1016/j.avsg.2017.02.012. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

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Three-Dimensional Printing Facilitates Successful Endovascular Closure of a Type II Abernethy Malformation Using an AMPLATZER Atrial Septal Occluder Device

Jeffrey Forris Beecham Chick, MD, MPH, DABR1; Shilpa N. Reddy, MD2; Alice C. Yu, BS3; Tatiana Kelil, MD4; Ravi N. Srinivasa, MD1; Kyle J. Cooper, MD1; Wael E. Saad, MBBCh, FSIR1

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Department of Radiology, Division of Vascular and Interventional Radiology, University of Michigan Health System, 1500 East Medical Center Drive, Ann Arbor, MI 48109 2

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Radiology Associates of the Main Line, Division of Vascular and Interventional Radiology, Main Line Health System, Bryn Mawr Hospital, 130 South Bryn Mawr Avenue, Bryn Mawr, PA 19010, USA 3

Case Western Reserve University, School of Medicine, 2109 Adelbert Road, Cleveland, OH 44106 4

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Department of Radiology, Brigham and Women’s Hospital, Harvard Medical School, 75 Francis Street, Boston, Massachusetts 02115

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All authors have read and contributed to this manuscript. The authors have no relevant disclosures.

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Correspondence/Reprints: Jeffrey Forris Beecham Chick, MD, MPH, DABR Department of Radiology Division of Vascular and Interventional Radiology University of Michigan Health System 1500 East Medical Center Drive Ann Arbor, MI 48109 [email protected]

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Three-Dimensional Printing Facilitates Successful Endovascular Closure of a Type II Abernethy Malformation Using an AMPLATZER Atrial Septal Occluder Device

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ABSTRACT Type II Abernethy malformations, characterized by side-to-side portosystemic

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shunting with preserved intrahepatic portal venous system, have been treated with

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shunt closure surgically and endovascularly. Three-dimensional printing has been used

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to develop highly accurate patient-specific representations for surgical and

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endovascular planning and intervention. This innovation describes three-dimensional

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printing to successfully close a flush-oriented type II Abernethy malformation, with

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discrepant dimensions on computed tomography, conventional venography, and

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intravascular ultrasound, using a 12-mm AMPLATZER atrial septal occluder device.

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KEYWORDS Three-Dimensional Printing; Endovascular Closure; Abernethy Malformation;

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Type II; Atrial Septal Occluder Device; Interventional Radiology

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ABBREVIATIONS CT = computed tomography; IVUS = intravascular ultrasound; IVC = inferior vena

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cava; DICOM = Digital Imaging and Communications in Medicine; STL = Standard

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Tesselation Language; TPA = tissue plasminogen activator; TIPS = transjugular

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intrahepatic portosystemic shunt

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INTRODUCTION Abernethy malformations, characterized as type I or type II, are congenital

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portosystemic venous anomalies (Figure 1). Type I, often seen in females with complex

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congenital abnormalities including heart disease, duodenal and biliary atresia,

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malrotation, annular pancreas, polysplenia, situs inversus, and genitourinary and

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musculoskeletal anomalies, is classically described as an end-to-side shunt in the

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absence of an intrahepatic portal venous system (1–3). Type II, on the other hand, is

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heralded by a side-to-side portocaval shunt with a preserved, but often hypoplastic,

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intrahepatic portal venous system (1,4).

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While often asymptomatic, Abernethy malformations may present with hepatic encephalopathy, pulmonary hypertension, hypoxemia, and hepatopulmonary syndrome,

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and may lead to the development of hepatocellular carcinoma and hepatoblastomas.

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Due to absence of the portal vein, liver transplantation remains the only definitive

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treatment for type I malformations (5). Surgical or endovascular shunt closure, however,

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may be a viable treatment for patients with symptomatic type II Abernethy

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malformations (1,2,4,6,7). Few cases of endovascular portocaval shunt closure have

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been described (4,6,8).

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Three-dimensional printing has played an increasing role in procedural planning,

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medical device design, and the delivery of complex medical and surgical care (9). Most

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often described with respect to dentistry and craniofacial surgeries, three-dimensional

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printing has improved surgical planning and implant fabrication in the brain, thorax, and

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cardiovascular and musculoskeletal systems (9). 6

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This report describes the first case of three-dimensional printing used to facilitate

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single-session AMPLATZER atrial septal occluder device closure of a type II Abernethy

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malformation. This report also highlights that, with the advent of three-dimensional

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planning techniques, increasing complex endovascular procedures are possible, often

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obviating the need for more invasive open surgical techniques.

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CASE REPORT Institutional review board approval was not required for preparation of this report.

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A 9-year-old male initially presented to an institution in Puerto Rico for a tibial fracture

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and was found to be hypoxic with oxygen saturations of 85% on room air. Ventilation-

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perfusion scan showed activity within the brain and kidneys suggestive of a right-to-left

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shunt. Cardiac catheterization showed multiple enlarged pulmonary arteries with

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findings suspicious for pulmonary arteriovenous malformations. The patient was then

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referred to this institution for additional management of presumed pulmonary

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arteriovenous malformations.

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Upon presentation, the patient was noted to be hypoxic with oxygen saturation of 82% on room air. Physical examination was notable for digital clubbing. Six-minute

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walking evaluation demonstrated a baseline oxygen saturation of 91% with average

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oxygen saturation of 72% upon walking. The patient reported “squeezing and burning

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sensations” throughout his chest during the entire examination. Computed tomography

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(CT) of the chest, abdomen, and pelvis with intravenous contrast demonstrated the

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presence of a 1.9 x 1.5 cm nearly-flush side-to-side portocaval shunt consistent with a

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type II Abernethy malformation. The CT also showed prominent pulmonary vasculature

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but no evidence of pulmonary arteriovenous malformations (Figures 1 and 2). The

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patient’s symptoms and presentation were therefore most consistent with

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hepatopulmonary syndrome in the setting of a congenital portocaval shunt.

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Patient was seen for initial consultation by an attending interventional radiologist in the interventional radiology clinic. All procedures were performed under general 8

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anesthesia with endotracheal intubation. Procedures were completed by two attending

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interventional radiologists in a biplane angiography suite containing an Artis Zee

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interventional angiography system (Siemens; Munich, Germany). Via a femoral

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approach, the patient underwent portocaval venography in multiple projections, which

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demonstrated a large side-to-side portocaval shunt. The shunt diameter ranged from

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10-24mm as measured in multiple different projections (Figure 2). Intravascular

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ultrasound (IVUS) (Visions PV; Volcano Corp; San Diego, CA) showed the portocaval

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shunt measuring 12 mm. Initial non-occluded manometry demonstrated a portal vein

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pressure of 13 mmHg and an inferior vena cava (IVC) pressure of 9 mmHg, yielding a

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portosystemic gradient of 4 mmHg. Multiple attempts were made to occlude the

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portosystemic shunt using 10-mm, 18-mm, and 20-mm balloons, but were unsuccessful

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in achieving complete occlusion of the shunt. Manometry performed with near-complete

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occlusion using a 20-mm balloon showed a portal vein pressure of 24 mmHg and an

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IVC pressure of 10 mmHg, yielding a portosystemic gradient of 14 mmHg.

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Given the portal vein pressure of 24 mmHg, a decision was made to perform

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single-session endovascular shunt closure as opposed to multi-staged surgical or

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endovascular closure as supported by available literature (10,11). Given the nearly-flush

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configuration of the side-to-side portocaval coupled with variable measurements of the

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shunt dimensions despite use of CT, conventional venography, and IVUS, three-

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dimensional printing of the portocaval shunt was performed to plan future single-session

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endovascular closure.

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Three-dimensional printing required the conversion of the CT chest, abdomen, and pelvis Digital Imaging and Communications in Medicine (DICOM) images into a

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Standard Tesselation Language (STL) standard file format as the currently available

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three-dimensional printers do not directly recognize DICOM files (Figure 3). The

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anatomy of interest, namely the portocaval shunt, was selected, segmented, and

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thresholded by placement of regions of interest using an open source image analysis

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software (Three-Dimensional Slicer 2.6; www.slicer.org). The selected and segmented

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data was then converted to an STL format using the same software. The generated STL

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data was further refined using a Computer-Aided Design software, Meshmixer

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(Autodesk Inc; Waltham, MA). The refined STL file was then printed using a

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stereolithography three-dimensional printer (Form 2; Formlabs; Cambridge, MA) using

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liquid photo-curable resin (Clear Resin; Formlabs; Cambridge, MA) (Figure 3).

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Based on the three-dimensional printed model, closure was planned following experimentation with multiple devices including: AMPLATZER Vascular Plugs (St. Jude

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Medical; Saint Paul, MN), AMPLATZER atrial septal occluder devices (St. Jude

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Medical), and AMPLATZER muscular ventricular septal defect closure (St. Jude

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Medical) devices. Ultimately AMPLATZER atrial septal occluder devices, ranging in size

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from 10-16 mm, were felt to be the most appropriate closure device.

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To perform the endovascular closure, a 14-French sheath was placed via a right

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femoral vein approach and repeat portocaval venography was performed. Based on the

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results, and in effort to successfully perform endovascular closure, transhepatic access

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was obtained, a 7-French sheath was placed, and additional venography of the 10

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portocaval shunt was performed (Figure 4). Repeated venography demonstrated a

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variable-diameter portocaval shunt ranging from 14-17 mm. Initial non-occluded

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manometry demonstrated a portal vein pressure of 14 mmHg and an IVC pressure of 13

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mmHg, yielding a portosystemic gradient of 1 mmHg. Based on three-dimensional

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planning, a 16-mm AMPLATZER atrial septal occluder device was placed; however, it

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was too large, with waist compressed to 8.5 mm, and it was subsequently recaptured

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and removed. Based on the 8.5 mm waist, a 10-mm AMPLATZER atrial septal occluder

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device was deployed; however, it was too small and prolapsed through the portocaval

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shunt. It was subsequently re-sheathed and removed. A 12-mm AMPLATZER atrial

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septal occluder device was then placed. Portocaval venography showed markedly

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reduced, but mildly persistent flow through the portocaval shunt. Therefore, a balloon

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was inflated from the portal venous end in order to buttress the AMPLATZER atrial

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septal occluder device against the open communication. Repeat portal venography and

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inferior vena cavography demonstrated complete occlusion of the portocaval shunt with

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no residual flow across the communication. Both the portal venous system and IVC

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were widely patent. Competition manometry demonstrated a portal vein pressure of 31

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mmHg and an IVC pressure of 19 mmHg, yielding a portosystemic gradient of 12

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mmHg. The transhepatic tract was embolized using 2 mL of 1:1 ethiodized oil (Ethiodol;

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Guerbet, Villepinte, France) and N-butyl cyanoacrylate glue (Histoacryl; B. Braun,

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Melsungen, Germany).

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Hepatic ultrasound with Doppler, performed immediately after the procedure, showed patent portal and hepatic veins with normal waveforms and direction of flow and 11

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without evidence of flow across the occluded portocaval shunt. Repeat hepatic

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ultrasound with Doppler, completed ten hours later, showed echogenic thrombus within

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the distal splenic vein, distal superior mesenteric vein, and proximal portal vein

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extending to the AMPLATZER atrial septal occluder. Contrast enhanced CT of the

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abdomen and pelvis, completed 12 hours after closure, demonstrated poor opacification

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of the splenic, superior mesenteric, and portal veins suggestive of thrombosis with

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wedged-shaped regions of hypoattenuation throughout the liver and spleen consistent

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with infarction (Figure 5).

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Based on these results, emergent thrombolysis was initiated. Via a right

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transjugular approach, a 16-gauge Colapinto needle (Cook Medical; Bloomington, IN)

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was used to access the right portal vein using the AMPLATZER device as an anatomic

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landmark (Figure 6). A flush catheter was placed and portography demonstrated

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extensive thrombus throughout the splenic, superior mesenteric, and portal veins.

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Pharmacomechanical thrombectomy was performed throughout the splenic, superior

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mesenteric, and portal veins using the Angiojet thrombectomy system (Boston

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Scientific; Marlborough, MA) in both pulse spray and suction thrombectomy modes

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using 20 mg tissue plasminogen activator (TPA) (alteplase; Genentech; South San

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Francisco, CA). Due to incomplete thrombus clearance, two 4-French UniFuse lysis

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catheters (Angiodynamics; Latham, NY) were placed within the splenic and superior

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mesenteric veins with 0.25 mg TPA/hour instilled through each catheter and 500 units of

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heparin/hour infused through the right internal jugular vein sheath.

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Three hours later, the patient was noted to have oozing surrounding the right internal jugular vein access sheath with associated drop in hemoglobin from 11.5 mg/dL

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to 6.9 mg/dL. TPA and heparin were immediately discontinued. Contrast enehnaced CT

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of the abdomen and pelvis demonstrated improved patency of the splenic, superior

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mesenteric, and extrahepatic portal veins with some remaining intrahepatic portal

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venous thrombus, but no evidence of contrast extravasation. The existing lysis

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catheters were removed and portography demonstrated near-complete resolution of the

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previously seen splenic, superior mesenteric, and portal vein thrombus. Repeat

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pharmacomechanical thrombectomy was performed throughout the splenic, superior

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mesenteric, and portal veins using the Angiojet thrombectomy device in both pulse

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spray and suction thrombectomy modes.

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A decision was then made to place a transjugular portosystemic shunt (TIPS)

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shunt. Pre-TIPS manometry demonstrated a portal vein pressure of 22 mmHg and a

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right atrial pressure of 11 mmHg, yielding a portosystemic gradient of 11 mmHg. TIPS

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placement was performed with the deployment of two overlapping 8 mm x 37 mm

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Express LD Stents (Boston Scientific). Post-stenting venography demonstrated widely

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patent stents. Post-TIPS manometry demonstrated a portal vein pressure of 13 mmHg

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and a right atrial pressure of 12 mmHg, yielding a portosystemic gradient of 1 mmHg.

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The patient was extubated the following day and hemoglobin remained stable.

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Repeat hepatic ultrasound with Doppler showed patency of the splenic, superior

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mesenteric, and portal veins as well as the TIPS with a small amount of residual

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eccentric thrombus within the intrahepatic main portal vein. Repeat venography of the 13

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portal system, performed from the TIPS access one week later, demonstrated

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opacification of the splenic, superior mesenteric, and portal veins as well as the TIPS

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stents. Manometry demonstrated a portal vein pressure of 17 mmHg and a right atrial

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pressure of 12 mmHg, yielding a portosystemic gradient of 5 mmHg.

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The patient was discharged home without anticoagulation. Repeat hepatic

ultrasound with Doppler performed at 2-months showed patency of splanchnic system

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and TIPS stents. Six-minute walking evaluation demonstrated a baseline oxygen

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saturation of 96% with average oxygen saturation of 94% upon walking at 4-months.

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Staged TIPS closure is planned in the future.

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DISCUSSION Type II Abernethy malformation is characterized by a side-to-side portosystemic

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shunt with preserved intrahepatic portal venous system. Patency of the intrahepatic

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portal venous system allows for consideration of surgical and endovascular shunt

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closure options in symptomatic patients. A few case reports and case series have

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described endovascular techniques for portocaval shunt closure (4,6,8,10). Anatomy of

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the portocaval shunt with respect to the portal vein or portal vein branch involved and

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IVC may present several technical challenges for endovascular closure. Two studies

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thus far described placement of a stent-graft in the IVC across the shunt for complete

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exclusion of the shunt (4,6). Proximity of the portocaval shunt to the hepatic or renal

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veins; however, may preclude safe placement of an IVC stent-graft in many instances.

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One study also described a multi-staged approach in which progressively smaller

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tailored stents were placed in the portocaval shunt until shunt closure was complete (8).

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The present technical innovation describes the novel use of three-dimensional printing for planning of portosystemic shunt closure. Three-dimensional printing has

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been previously described and available data suggest that highly accurate models may

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be created for patient-specific simulation of both surgical and percutaneous

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interventions including treatment of hypertrophic cardiomyopathy, transcatheter aortic

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valve replacement, and vertebral cryoablation (12,13). In this case, three-dimensional

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printing facilitated planning and enabled simulated experimentation with various shunt

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closure devices including AMPLATZER atrial septal occluder and AMPLATZER

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muscular ventricular septal defect closure devices. This report also represents the first

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known use of an AMPLATZER atrial septal occluder placement for single-staged

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portocaval shunt occlusion. Benefits of using the vascular occluder included ability to

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retrieve the device if and when deployment was suboptimal. Utilization of a vascular

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occluder also obviated the need for an IVC stent-graft, which has known long-term risks

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of in-stent stenosis and thrombosis (14). Furthermore, use of a vascular occluder may

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have the advantage over IVC stent-graft placement in maintaining integrity of the native

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IVC in the setting that liver transplantation is needed in the future.

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Aside from the technical challenges of portocaval shunt closure in patients with

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type II Abernethy malformations, this case also highlights the clinical challenges of

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shunt closure in these patients. Available studies have suggested that patients with type

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II Abernethy malformations have varying degrees of functional intrahepatic portal

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venous systems. Some patients may have hemodynamically functional intrahepatic

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portal veins while other patients may have diminutive intrahepatic portal veins that are

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functionally quiescent (4,6). In one report, a patient was misclassified as having type I

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Abernethy malformation based on both preoperative imaging and histopathology until

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direct catheterization of the portal vein and venography demonstrated a network of

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minute intrahepatic portal veins (6). Although the presence of portal veins allows

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patients to be considered for shunt closure, clinical success of shunt closure depends

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on the ability of the intrahepatic portal veins to respond to rapid hemodynamic changes

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in the portal system that occur immediately following shunt closure (6). Specifically,

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portosystemic shunt closure in patients with immature or inadequate intrahepatic portal

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veins may have fatal consequences as a result of abrupt portal hypertension, sluggish

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portal blood flow, and ultimately thrombosis of the portal and mesenteric veins (6,15). For this reason, studies have suggested a multi-staged approach to

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portosystemic shunt closure in patients with known or suspected diminutive intrahepatic

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portal veins (6,8). Multi-staged approaches to shunt closure have been shown to allow

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for gradual increases in intrahepatic portal venous blood flow and resultant remodeling

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and maturation of the intrahepatic portal venous system (6). Determining which patients

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may tolerate single-session shunt closure and which patients require multi-staged

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closure; however, may be challenging. Several algorithms and criteria for determining

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single-session versus multi-staged shunt closure have been proposed. A few studies

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have suggested utilizing absolute portal vein pressure and portosystemic shunt gradient

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during a shunt occlusion test (4,8,10,11). One study suggested that patients with an

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absolute portal vein pressure greater than 32 mmHg should undergo multi-phased

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shunt closure while another study suggested that patients with portal vein pressures

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greater than 25 mmHg should undergo multi-phased shunt closure (10,11). In another

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study, staged endovascular shunt closure was performed in patients with portosystemic

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shunt gradients above 18 mmHg during a shunt occlusion test (8). The only other report

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describing single-staged endovascular shunt closure utilized findings of normal

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intrahepatic portal veins on histopathology as well as low portosystemic gradient to

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guide the decision for a single-session shunt closure (4). In the present study, despite a

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portosystemic gradient of 14 mmHg and an absolute portal vein pressure of 24 mmHg

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during the shunt occlusion test, and portography following shunt closure demonstrating

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brisk intrahepatic portal venous flow, the patient almost immediately developed

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splanchnic venous thrombosis. Of note, the absolute portal venous pressure in this case

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increased to 31 mmHg immediately following shunt closure, suggesting that the shunt

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occlusion test may not have accurately assessed portal vein pressure. As such, marked

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increases in absolute portal venous pressures following shunt closure may be an

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indicator of clinical success following shunt closure and may guide the decision for

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concurrent TIPS placement in certain cases.

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This study also highlights the importance of post-shunt closure surveillance for

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evaluation of complications of shunt closure. Immediate intervention in this case with

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pharmacomechanical thrombolysis and TIPS placement was critical in preventing lethal

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consequences of splanchnic venous thrombosis.

Limitations of this study include single-center experience in a single patient.

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Furthermore, long-term follow-up and clinical success is currently pending. Additional

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studies are necessary to validate this technique.

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CONCLUSION This technical innovation highlights the use of three-dimensional printing to

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facilitate the first single-session endovascular closure of a type II Abernethy

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malformation using an AMPLATZER Atrial Septal Occluder. Such complex

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endovascular portocaval closures are feasible, but require vigilance for post-procedure

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complications including splanchnic thrombosis and occlusion.

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Neglén P, Hollis KC, Olivier J, Raju S. Stenting of the venous outflow in chronic venous disease: long-term stent-related outcome, clinical, and hemodynamic result. J Vasc Surg. 2007 Nov;46(5):979–90.

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Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg. 1994 Sep;29(9):1239–41.

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COI STATEMENT The authors whose names are listed immediately below certify that they have NO affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript. All authors have read and contributed to this manuscript.

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The authors have no relevant disclosures.

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ACKNOWLEDGEMENT The authors thank Alice C. Yu, BS for creation of the Figure 1 schematic diagram.

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FIGURES:

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Figure 1: (A) Type 1a end-to-side shunt with no intrahepatic portal veins where splenic

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vein (SV) and superior mesenteric vein (SMV) drain separately into the inferior vena

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cava (IVC). (B) Type 1b end-to-side shunt with no intrahepatic portal veins where SV 25

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and SMV join before draining into the IVC. (C) Type 2 side-to-side shunt with preserved

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intrahepatic portal veins. (D) Presented patient with type 2 side-to-side communication

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with the IVC and with preserved intrahepatic portal venous system. Compared to the

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type 2 schematic (C) the side-to-side portocaval shunt was shorter and wider (D).

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Figure 2: (A) Three dimensional Vitrea-reformatted image demonstrating a side-to-side

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communication (arrow) between the main portal vein (MPV) and the IVC. (B) Coronal

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CT image in the portal venous phase showing the area of communication between the

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MPV and the IVC (arrow). (C) Digital subtraction angiography image with a marker flush

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catheter positioned within the splenic vein from a jugular vein approach. The catheter

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traverses through the defect between the IVC and portal venous system (arrow). (D) An

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occlusion balloon is used to aid in measuring the diameter of the defect to determine the

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appropriate size plug to acquire.

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Figure 3: (A) Standard tessellation language (STL) file created from CT data using a

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segmentation and computer-aided detection software depicting the portal system in

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green and the venous system in blue. (B and C) Three-dimensional printed model

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created from the STL data using a stereolithography printer and clear resin.

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Figure 4: (A) A catheter passes from a femoral venous approach through the defect

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into the IVC and into a small right portal branch. A snare is used for targeting in order to

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obtain a transhepatic access with a Chiba needle. (B-D) Various AMPLATZER occluder 30

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devices of different sizes are used to attempt to occlude the shunt. (B) A device that is

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too large (arrow) and (C) a device that is too small (arrow). (D) Portal venography

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through the transhepatic catheter positioned in the splenic vein demonstrates persistent

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patency of the portosystemic communication. These devices were all re-sheathed and

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removed. (E and F) A final 12 mm AMPLATZER atrial septal occluder device is

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deployed within the portal venous system and the tail end deployed into the IVC

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(arrows). (F) A portal venogram performed after this device is deployed demonstrates

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markedly reduced flow within the portosystemic communication with some mild

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persistent flow noted. (G) A balloon inflated from the portal venous end in order to

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buttress the portal venous side of the AMPLATZER atrial septal occluder device against

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the open communication. (H) Repeat portal venography and (I) IVC cavography

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demonstrating complete occlusion of the portosystemic shunt with no residual flow

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across the communication. The portal venous system and IVC were widely patent.

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Figure 5: (A) Axial CT image through the liver performed the morning after shunt

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embolization demonstrating an area of wedge-shaped hypodensity within the left lobe of

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the liver concerning for developing ischemia and infarction. (B) There is expansion of

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the SV with filling defect (arrows) as well as (C) expansion of the portal vein with filling

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defect (arrow) compatible with thrombosis of the splanchnic circulation. Notably the

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SMV also had thrombosis (not shown). Only arterial phase CT imaging was performed

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due to poor bolus timing.

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Figure 6: The patient was immediately taken for creation of a transjugular intrahepatic

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portosystemic shunt (TIPS) in order to improve outflow in the now sluggish portal

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circulation. (A) From a right internal jugular approach a hepatic vein was cannulated and

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a Colapinto needle was used to puncture into the portal venous system using the

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Amplatzer device as a target. A wire can be seen coiled within the thrombosed portal

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vein (arrow). (B) Through the TIPS access, Angiojet rheolytic thrombectomy with pulse

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spray of tissue plasminogen activator was performed with improved flow obtained. (C)

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Two unifuse lysis catheters were left in place, one within the SV and one within the SMV

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in order to perform overnight thrombolysis. (D) Due to bleeding from the puncture site

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as well as dropping fibrinogen, the patient was brought back later that night for

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termination of thrombolysis. (E) Initial portosplenic venography demonstrated a patent

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portomesenteric and splenic circulation with small residual thrombus. Repeat Angiojet

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rheolytic thrombectomy was performed with evacuation of the majority of the thrombus.

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(E) A TIPS shunt was left in place following the procedure and is demonstrated to be

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patent post-placement and angioplasty.

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