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BOY WITH SHORTNESS OF BREATH

Resident(s): Alexandria S. Jo, MD

Attending(s): Narasimham L Dasika, MD


Program/Dept(s): University of Michigan Department of Interventional Radiology

DISCLOSURES
No conflict of interest and relationships to disclose for this presentation
I may be discussing products that are investigational or not labeled by FDA for such
use under discussion

HPI AND PHYSICAL EXAMINATION


7-year old male in the 2nd grade, with 1 year history of shortness of breath upon
mild exertion (e.g. dressing in the morning).
Normal birth and development with no significant past medical/surgical history.

Examination demonstrated normal growth and development without cognitive


deficits.
However, he noted to be severely hypoxic in clinic (87% standing and 83% with
minimal exertion).

A chest X-ray revealed increased interstitial markings and reticulonodular pattern.


CTA of the chest was performed to rule out pulmonary AVM.

CTA OF THE CHEST


Findings:
Cardiomegaly and slight
prominence of the
pulmonary arteries in the
periphery of the lungs,
mostly in left lower lobe.
No pulmonary arteriovenous
malformation identified.
These findings led to a CT of
the abdomen and pelvis with
IV contrast.

CT ABDOMEN AND PELVIS with IV CONTRAST

CT ABDOMEN AND PELVIS with IV CONTRAST


No early venous filling of portal vein to
suggest mesenteric AVM or arteriovenous
fistulae.
Portosystemic shunt with a branch of the left
portal vein extending through the liver and
connecting to a dilated vascular structure
which then empties into the hepatic veins and
inferior vena cava.
Hepatic veins isodense to portal vein
suggestive of shunting.
Liver and spleen are not enlarged and are
unremarkable.
Link to Video (L)

Link to Video (R)

ABDOMINAL ULTRASOUND
To confirm the outside CT findings,
a limited abdominal ultrasound
was performed at our institution.

US revealed a large focally ectatic


patent left portal vein with internal
turbulent venous flow, which
appears to be connected to the left
hepatic vein.

ADDITIONALS FINDINGS
With increasing concerns for hepatopulmonary syndrome, ECHO was performed.
ECHO: Positive IV agitated saline contrast injection with early filling of the left
atrium after 3 cardiac cycles, diagnostic of intrapulmonary arteriovenous
malformation.
CBC
LFT
BMP
Labs: WNL

WBC

6.0

Na

140

AST

47

HGB

15.4

4.3

ALT

26

HCG

42.6

Cl

110

Alk Phos

253

PLT

219

CO2

22

T. Bili

0.6

BUN

10

D. Bili

0.2

Cr

0.28

Ammonia

48

Albumin

4.2

Note that the patients ammonia level


is within normal range for his age.

QUESTION SLIDE
Based on our findings so far, what is our patients diagnosis?
1. Abernethy Malformation
2. Congenital intrahepatic portosystemic venous shunt (IPSVS)
3. Arterioportal fistula
4. Arteriovenous malformation (AVM)

SORRY, THATS INCORRECT


Abernethy Malformation
Abernethy malformation is a rare congenital malformation of the splanchnic venous
system where there is an extrahepatic portosystemic shunt as a result of persistent
embryonic vessels.

TRY AGAIN
Nacif LS, Paranagua-Vezozzo DC, Galvao FHF, et al. Significance of CT scan and color Doppler duplex ultrasound in the assessment of Abernethy malformation. BMC Medical Imaging.
2015; 15:37.

SORRY, THATS INCORRECT


Arterioportal fistulas
Rare congenital anomaly associated with hereditary
hemorrhagic telangiectasia, Ehlers-Danlos syndrome, and
biliary atresia.
Usually symptomatic within 1 year of life.
Initially develop portal hypertension from hepatofugal
flow develops in the portal vein which becomes
arterialized.
US shows enlargement of the hepatic artery and
dilatation of the segment of the portal vein where the
fistula is located.
There maybe pulsatile hepatofugal flow in the portal vein
and color speckling in the hepatic parenchyma adjacent to
the fistula.
TRY AGAIN
Gallego C, Miralles M, Marin C, et al. Congenital hepatic shunts. Radiographics 2004;24(3):755 - 72.

SORRY, THATS INCORRECT


Arteriovenous Malformation (AVM)
Formation of blood vessels that shunt blood through direct
arteriovenous connections without abnormal neoplastic tissue
between the anomalous vessels. Usually localized to one lobe of
the liver.
US shows nest of tortuous enlarged vessels located in one lobe
of the liver with high peak Doppler shifts in both arteries and
veins, low arterial RI and increased pulsatility of veins.
CT shows enhancement in arterial or early portal venous phase
with rapid washout of contrast material.

TRY AGAIN
Gallego C, Miralles M, Marin C, et al. Congenital hepatic shunts. Radiographics 2004;24(3):755 - 72.

CORRECT
Congenital intrahepatic portosystemic venous shunt
Abnormal intrahepatic connection between branches of the portal vein
and the hepatic vein.
Presumed basis for IPSVS is a persistent communication between the
vitelline veins of the emphalomesenteric system and the sinus venosus
due to a focal absence of sinusoid formation.

CONTINUE WITH CASE

TYPES OF CONGENITAL PORTOSYSTEMIC SHUNT


Type

Description

No intrahepatic portal flow (CAPV or type I Abernethy malformation)

II

Partial shunt with preserved hepatic portal flow (type II Abernethy


malformation)

IIa

Fistula arising from left or right portal vein (includes


patent ductus venosus)

IIb

Fistula arising from main portal vein (including its bifurcation or


splenomesenteric confluence)

IIc

Fistula arising from the mesenteric, gastric, or splenic veins

Lautz TB, Tantemsapya N, Rowell E, Superina RA (2011) Management and classification of type II congenital portosystemic shunts. J Pediatr Surg 46:308314Senocak E, Oguz B, Edger T, Cila A. Congenital
intrahepatic portosystemic shunt with variant inferior right hepatic vein. Diagn Interv Radiol 2008; 14: 9799.

HEPATOPULMONARY SYNDROME
Liver is connected in series with the lung and portal
system, receiving all venous effluents from the portal
system and directing the metabolites to the lungs.
Exact mechanism is unknown but is thought to be due to a
combination of upregulation of nitric oxide (NO)
production and inability for the liver to metabolize
vasoactive mediators, resulting in V/Q mismatch, diffusion
limitation and shunting through pulmonary AVMs.
These patients often present with cyanosis, clubbing,
polycythemia, and impaired exercise tolerance from
hypoxemia.
Serious complications include systemic embolization,
pulmonary hemorrhage, or cerebral abscesses.

Morikawa, N. et al. Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an abernethy malformation. J. Pediatr. Surg. 43, e35e38 (2008)
http://www.nature.com/nrcardio/journal/v7/n9/images/nrcardio.2010.99-f3.jpg

CLINICAL DECISION
This was an usual clinical presentation of congenital intrahepatic portosystemic shunt
where the patient presented with isolated hepatopulmonary syndrome without clinical
evidence of hepatic encephalopathy, which is often the presenting symptom.
Although CTA of the chest did not show presence of arteriovenous malformations (AVMs),
ECHO findings were diagnostic of intrapulmonary AVMs.
Pulmonary AVM formation was most likely secondary to the presence of a large congenital
portosytemic shunt resulting in increased vasoactive mediators.
Despite the presence of a large portosystemic shunt, the patients hepatic function, as
determined by LFTs, was well preserved.

Patient was transferred to our institution and admitted under the transplant team for
evaluation.
IR was consulted for possible other minimally invasive intervention.

CLINICAL EVALUATION BY IR
Closure of our patients congenital intrahepatic portosystemic shunt is technically feasible.
However, the following questions remained unanswered so far in this patient:
How developed are the hepatic veins?
How developed is the peripheral portal system in the rest of the liver?
Pre-procedure direction and quantity of flow to the right liver lobe?
What will be the change in the portal pressures following occlusion and will the patient be
able to tolerate sudden occlusion of the shunt?

We decided to obtain answers to these questions by temporarily occluding the shunt


intraoperatively with a balloon. Depending on the result, we would decide to proceed
with a one-step or staged occlusion of the congenital shunt.
The back-up plan was to consider liver transplantation if hepatic veins and intrahepatic
portal system is poorly developed.

IR INTERVENTION:
INITIAL HEPATIC VENOGRAM
Left IJ was accessed for stable
intravascular sheath position and 9 Fr
Pinnacle sheath was placed.
The right and middle hepatic veins were
cannulated and a hepatic venogram was
performed.

The free and wedge hepatic venogram


revealed well-developed right and
middle hepatic veins.
The shunt was then cannulated through
the left hepatic vein with a coaxial 7 Fr
double angle 45 cm sheath for stability.

IR INTERVENTION:
CANNULATION OF THE PORTAL VEIN
Through the shunt, a 5 French glide
Cobra catheter was finally
advanced into the main portal vein
and direct portal venogram was
performed.
Small but patent right branch of
the portal vein with hepatopetal
flow and well-developed
intrahepatic portal branches were
documented.

IR INVERVENTION:
TEMPORARY BALLOON OCCLUSION OF THE
PORTOSYSTEMIC SHUNT
We then performed occlusion
portogram by inflating a properly
sized balloon in the shunt for 20
minutes.

Contrast portogram and direct


portal pressures were obtained at
the end of 20 minutes.
Given the satisfactory flow and
increase of porto-systemic gradient
by only 7mm , we decided to
proceed with a one-step occlusion
of the shunt.

IR INVERVENTION:
OCCLUSION OF THE PORTOSYSTEMIC SHUNT
Intrahepatic portosystemic venous shunt
was closed with two 2nd generation
16mm Amplatzer plugs.
Final venogram demonstrated total
occlusion of the shunt.

FOLLOW-UP: IMMEDIATE POSTOP


He was admitted to PICU for post operative monitoring.
He was initially on 2L NC but was weaned to room air overnight and was
saturating at 92-93% with ambulation.

He was then downgraded to floor status the next day.


Repeat ultrasound demonstrated complete occlusion of previously demonstrated
shunt with no residual shunting.
Stable HGB and LFTS.
He was discharged home 2 days after IR intervention in a stable condition.

FOLLOW-UP: 2 YEARS POSTOP


Most recent US: No left portal vein
signal was identified in the region of the
left portal vein.

Most recent update, 2 years after


intervention: Patient is in the 3rd grade
this year. He is doing well in school and is
enjoying playing baseball. He has no
shortness of breath. He seems to be
thriving.

CONCLUSION
Congenital intrahepatic portosystemic shunt is a very rare congenital anomaly that can
result in hepatic encephalopathy from hyperammonemia, heart failure, and fatty
degeneration of the liver from lack of nutrition in the hepatic cells due to reduced inflow.
Hepatopulmonary syndrome is an uncommon complication resulting from IHPSS, and is
believed to be due to increased NO and vasoactive mediators.

Congenital intrahepatic portosystemic shunt can be differentiated from Abernethy


malformation by the presence of an intrahepatic shunt from the portal to the systemic
circulation.
Our patient presented with clinically symptomatic hepatopulmonary syndrome. He had
no signs of liver dysfunction and demonstrated sufficient blood supply to the liver
parenchyma to proceed with shunt occlusion. Patient demonstrated almost immediate
improvement in symptoms and continues to be asymptomatic.
Orthotopic liver transplantation may not be necessary to correct hepatopulmonary
syndrome. Early congenital intrahepatic portosystemic shunt occlusion can serve as a
minimally invasive option for pediatric patients who demonstrate noncirrhotic intrahepatic
vascular abnormalities and early reversible pulmonary vasodilatation.

REFERENCE
Alonso J, Sierre S, Lipsich J et al. Endovascular treatment of congenital portal vein fistulas with the Amplatzer occlusion device. J Vasc Interv Radiol 2004; 15:989993.
Florio F, Nardella M, Balzano S, Giacobbe A, Perri F. Congenital intrahepatic portosystemic shunt. Cardiovasc Intervent Radiol 1998; 21:421 424.
Gallego C, Miralles M, Marin C, et al. Congenital hepatic shunts. Radiographics 2004;24(3):755 - 72.
Konstas AA, Digumarthy SR, Avery LL, et al.. Congenital portosystemic shunts: imaging findings and clinical presentations in 11 patients. Eur J Radiol 2010.
Lautz TB, Tantemsapya N, Rowell E, Superina RA (2011) Management and classification of type II congenital portosystemic shunts. J Pediatr Surg 46:308314Senocak E, Oguz B,
Edger T, Cila A. Congenital intrahepatic portosystemic shunt with variant inferior right hepatic vein. Diagn Interv Radiol 2008; 14: 9799.
Lee SA, Lee YS, Lee KS, Jeon GS. Congenital intrahepatic portosystemic venous shunt and liver mass in a child patient: successful endovascular treatment with an Amplatzer vascular
plug (AVP). Korean J Radiol 2010; 11 (5) 583-586.
Morikawa, N. et al. Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an abernethy malformation. J. Pediatr. Surg 2008;43,
e35e38.
Park JH, Cha SH, Han JK, Han MC. Intrahepatic portosystemic venous shunt. AJR Am J Roentgenol 1990;155(3):527528.
Scalabre A, Gorincour G, Hery G, Gamerre M, Guys JM, de Lagausie P. Evolution of congenital malformations of the umbilical-portal-hepatic venous system. J Pediatr Surg
2012;47:14905.
Senocak E, Ouz B, Edger T, Cila A. Congenital intrahepatic portosystemic shunt with variant inferior right hepatic vein. Diagn Interv Radiol 2008; 14: 9799.
Stringer MD. The clinical anatomy of congenital portosystemic venous shunts. Clin Anat 2008;21:14757.

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