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
ABDOMINAL ULTRASOUND
To confirm the outside CT findings,
a limited abdominal ultrasound
was performed at our institution.
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
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)
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.
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.
Description
II
IIa
IIb
IIc
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?
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.
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.
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.
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.
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.
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