CLINICAL INVESTIGATION ARTERIAL INTERVENTIONS
Endovascular Treatment of Congenital Arterioportal Fistulas
Anna P. Lillis
Horacio M. Padua
Rush H. Chewning
Ahmad I. Alomari
Received: 21 January 2018 / Accepted: 24 February 2018 / Published online: 6 March 2018
Ó Springer Science+Business Media, LLC, part of Springer Nature and the Cardiovascular and Interventional Radiological Society of Europe
Purpose To characterize anatomy of congenital arterio-
portal ﬁstulas (CAPF) and correlate this with technique and
outcomes of transcatheter embolization (TCE).
Materials and Methods Retrospective review was con-
ducted of children with CAPF that underwent TCE in a
10-year period. Medical records, imaging and procedure
details were reviewed. TCE was performed via transarterial
(n = 5), portal (n = 5) or patent ductus venosus (n =1)
approach. Embolic agents used were coils (n = 10), Onyx
(n = 1) and Amplatzer septal occluder (n = 1).
Results A total of 7 patients were included (4 female).
Median age at treatment was 4 months (2 month–3 year).
Most common symptoms were GI bleeding (n = 4), anemia
(n = 4) and ascites (n = 3). Hepatopetal ﬂow was seen in
all on pre-procedure sonography. On angiography, a simple
lesion, with direct ﬁstulous connections, from hepatic
arterial (HA) branches to portal venous (PV) system was
seen in 3. A complex lesion with multiple connections was
identiﬁed in 4. In simple lesions, cessation of shunting was
achieved in 1 procedure, with embolization of afferent
vessels. In complex lesions, multiple procedures were
required in 3 of 4 patients. All 4 with complex connections
required embolization of the aneurysmal PV segment.
There were no major complications. Two minor compli-
cations were noted: localized biliary leak in 1 and femoral
pseudoaneurysm in 1. There was resolution of symptoms in
Conclusions TCE is effective as primary treatment of
CAPF. Patients with simple arteriovenous connections can
be treated with embolization of afferent vessels, but chil-
dren with multiple complex connections usually require
embolization of the dilated PV segment.
Keywords Arterioportal ﬁstula Á Embolization Á
Pediatric intervention Á Hepatic intervention
Congenital arterioportal ﬁstulas (CAPF) are a rare cause of
portal hypertension in infancy and early childhood. CAPF
have been deﬁned as intrahepatic communications between
the systemic arteries and portal venous system, without any
communication with the systemic venous circulation .
The majority of arterioportal ﬁstulas described in the lit-
erature are acquired, secondary to underlying liver disease,
trauma and surgical procedures, with less than 10% thought
to be congenital [2, 3]. Various classiﬁcations have been
proposed for CAPF, based either on location  or sup-
plying vessels .
Endovascular treatment of CAPF, with embolization of
the abnormal communications, has been shown to be safe
and effective, resulting in obliteration of the shunt and
restoration of normal portal hemodynamics. It is now
considered as ﬁrst-line therapy for this entity [5–7].
& Gulraiz Chaudry
Division of Vascular and Interventional Radiology, Boston
Children’s Hospital and Harvard Medical School, 300
Longwood Avenue, Boston, MA 02115, USA
Cardiovasc Intervent Radiol (2018) 41:1021–1028