AJR 2003; 180:716-718
© American Roentgen Ray Society
Embolization of the Patent Ductus Venosus in an Adult Patient
Takuji Araki1,
Toshiyuki Konishi2,
Shinichiro Yasuda2,
Takanori Osada2 and
Tsutomu Araki1
1 Department of Radiology, Yamanashi Medical University, 1110 Shimokato, Tamaho,
Nakakoma-gun, Yamanashi, 4093898, Japan.
2 Department of Internal Medicine, Kofu Kyoritsu Hospital, 1-9-1 Takara,
Kofu-city, Yamanashi, 400-0034, Japan.
Received February 11, 2002;
accepted after revision August 27, 2002.
Address correspondence to Tsutomu Araki.
Introduction
In fetal circulation the ductus venosus is a connection between the left
portal vein and the inferior vena cava that closes immediately after birth.
Reports of patent ductus venosus in children are rare
[1,
2,
3,
4], and in adults, even rarer
[5,
6]. Although a patent ductus
venosus may be thought of as a kind of portosystemic shunt, most intrahepatic
portosystemic venous shunts, which often result in portosystemic
encephalopathy, are congenital shunts between the intrahepatic portal vein and
the peripheral hepatic vein [7,
8]. A patent ductus venosus may
be treated by conservative therapy or by surgical banding
[9]. To our knowledge, only
three reports about the transcatheter treatment of patients with this disease
have been published [3,
4,
6].
We present a case of a 45-year-old woman with direct communication between
the left portal vein and the inferior vena cava that was a patent ductus
venosus. An arterial portogram revealed a markedly dilated left portal vein
and a narrowed right portal vein. While the shunt was temporarily occluded by
an inflated balloon, the shunt was successfully treated by embolization with
the use of detachable coils via a retrograde transcaval route.
Case Report
In August 1997, a 45-year-old woman with a 2-year history of disturbance of
consciousness and headache presented to our hospital. She had been diagnosed
at another hospital as having hepatic encephalopathy due to hyperammonemia of
unknown origin and was conservatively treated with no improvement in her
condition. The results of routine laboratory tests were as follows: serum
albumin, 3.0 g/dL (normal, 3.4-4.9 g/dL); and total bilirubin, 1.7 mg/dL
(0.2-1.1 mg/dL). Blood ammonia was elevated at 193 µg/dL (12-66 µg/dL).
Laboratory results indicated that hepatitis C virus antibody and hepatitis B
surface antigen were not present. There was no clinical evidence of liver
cirrhosis.
Contrast-enhanced abdominal CT revealed an abnormal vein between the left
portal branch and the inferior vena cava passing through the fissure for the
ligamentum venosum (Figs. 1A
and 1B). An arterial portogram
revealed the abnormal vein connecting the umbilical portion of the portal vein
directly to the inferior vena cava. Although the main portal trunk and its
left branch were dilated, the right branch was narrowed
(Fig. 1C). A celiac arteriogram
revealed compensatory dilatation of the hepatic artery. There was no
communication between the abnormal vein and any hepatic veins, and the
diagnosis was a patent ductus venosus. The patient opted for a transcatheter
procedure, not surgery, for treatment of the patent ductus venosus.
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Fig. 1A. —45-year-old woman with patent ductus venosus. Contiguous
contrast-enhanced CT scans with 10-mm slice thickness reveal abnormal vein
(arrow, A) connecting large left portal vein to inferior vena
cava through fissure for ligamentum venosum.
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Fig. 1B. —45-year-old woman with patent ductus venosus. Contiguous
contrast-enhanced CT scans with 10-mm slice thickness reveal abnormal vein
(arrow, A) connecting large left portal vein to inferior vena
cava through fissure for ligamentum venosum.
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Fig. 1C. —45-year-old woman with patent ductus venosus. Portogram
obtained through superior mesenteric artery reveals abnormal vein
(arrow) following dilated left portal vein and narrow right portal
vein. Shunt near umbilical portion of left portal vein appears to be
aneurysmal.
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From the right internal jugular vein, a 6-French catheter with a
20-mm-diameter latex balloon was inserted into the abnormal vein. When the
abnormal shunt was temporarily occluded by the inflated balloon, the patient's
portal venous pressure increased from 18 to 20 mm Hg. An arterial portogram
obtained during temporary balloon occlusion showed good portal venous flow and
right portal branches that were slightly wider than those without balloon
occlusion. Therefore, we assumed that occlusion of the abnormal vein did not
affect the portal venous circulation. The diameter of the abnormal vein was 10
mm, and the aneurysmal dilatation was 16 mm near the umbilical portion of
portal vein.
A microcatheter (Renegade; Boston Scientific, Natick, MA) was introduced
through a balloon catheter into the aneurysmal portion of the abnormal vein. A
temporary occlusion balloon was inflated in the 10-mm-diameter shunt. We
prepared snares and cardiac muscle biopsy forceps to treat migration of coils
should that become necessary. Embolization was performed with detachable coils
(IDC [interlocking detachable coil]; Target, Fremont, CA) and microcoils
(Tornado; Cook, Bloomington, IN) under temporary balloon occlusion of the
shunt to control the blood flow (Fig.
1D). We placed nine interlocking detachable coils, ranging in
diameter from 8 to 14 mm and in length from 10 to 20 cm, and 12 microcoils
that were 6 mm in diameter and 6 cm long.
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Fig. 1D. —45-year-old woman with patent ductus venosus. Radiographic
fluorogram shows transcatheter embolization using detachable microcoils and
temporary balloon occlusion. Note inflated balloon (arrows).
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Before the occlusion balloon was completely deflated, the shunt venous
pressure measured through the balloon catheter was decreased by approximately
0-3 mm Hg, and no coil migration or movement was seen during slight deflation
of the balloon. The temporary occlusion balloon had been inflated for 3 hr by
the time the embolization was completed. An arterial portogram obtained after
embolization showed that the shunt was completely occluded, and the right
portal branches were wider than before embolization. Ten days after
embolization, the blood ammonia level had dropped from 193 to 55 µg/dL,
which is in the normal range. Three months after the intervention, an arterial
portogram revealed a normal portal vein and occlusion of the abnormal vein
(Fig. 1E). Four years after
embolization was performed, the patient's symptoms had not recurred.
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Fig. 1E. —45-year-old woman with patent ductus venosus. Arterial
portogram obtained 3 months after treatment reveals shunt to be completely
occluded and portal vein branches to be normally wide.
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Discussion
The ductus venosus is a fetal circulation system that provides a direct
connection between the portal vein and inferior vena cava or left hepatic
vein. A patent ductus venosus should differ from intrahepatic portosystemic
venous shunts without liver cirrhosis, which may be congenital in origin
[7]. In the embryo, when
remnants of anastomosis between the subcardinal venous and vitelline venous
systems are present, intrahepatic portosystemic venous shunts occur, usually
between intrahepatic portal veins and peripheral hepatic veins.
In our patient, the abnormal vein connected the umbilical portion of the
portal vein and the inferior vena cava directly through the fissure for the
ligamentum venosum. The portal vein at the umbilical portion connects with the
umbilical vein and ductus venosus in fetal circulation. According to Mitchell
et al. [1], the ductus venosus
is rapidly obliterated after birth and forms the ligament venosum. Unlike the
umbilical vein, the ductus venosus rarely reopens in patients with portal
hypertension because the ductal-portal junction closes early, whereas the
umbilical-portal junction is frequently patent after birth. Our patient had a
portal venous pressure of 18 mm Hg, but portal hypertension did not seem to
have caused the ductus venosus to reopen because other collateral veins or
splenomegaly was absent and because the abnormal vein was a patent ductus
venosus, not a reopened one.
A patent ductus venosus is important to recognize because it causes
portosystemic encephalopathy during childhood. However, only nine cases in
adults have been reported [5,
6]. Why portosystemic
encephalopathy often develops later in life—even when the shunt itself
is congenital—is not known
[2]. A patent ductus venosus
with mild symptoms of portosystemic encephalopathy or heart failure may be
conservatively treated. When these symptoms are not conservatively controlled,
the shunt must be closed by surgical banding or transcatheter procedures. Only
three reports about transcatheter procedures for treatment of patients with
this disease, including one adult, appear in the literature
[3,
4,
6]. Two of these reports
describe embolizations, and the other describes placement of a reduction stent
to decrease shunt volume.
Embolization can be expected to have the same effect as surgical banding,
but embolization is less invasive. Liver congestion leading to portal
thrombosis has been reported to occur in patients treated with surgical
banding [1,
9], and embolization may have
the same risk. To evaluate the risk, we measured the portal venous pressure
and performed arterial portography under temporary balloon occlusion of the
shunt before embolization. The patient's portal venous pressure was minimally
elevated, and arterial portography revealed good blood flow and wide right
portal branches. These findings might suggest that portal venous congestion
would not occur after embolization. Liver biopsy was not performed in our
patient, but confirmation of the absence of liver cirrhosis by biopsy might
provide a more accurate prognosis. On the other hand, placement of a reduction
stent can gradually occlude the shunt. Liver congestion may occur to a lesser
extent with stent placement than with embolization. Nevertheless, shunt flow
still poses the risk of pulmonary thrombosis, and one cannot predict when the
shunt will be completely occluded.
We performed embolization through the retrograde transcaval route. During
embolization, temporary balloon occlusion of the patent ductus venosus
prevented migration of the coils as a result of the high shunt flow. In
another study, temporary balloon occlusion reduced the likelihood of coil
migration and predicted portal hemodynamic changes after occlusion
[6]. We believe transcatheter
intervention through retrograde transcaval access to be the treatment of
choice for patients with symptomatic patent ductus venosus.
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Introduction
Case Report
