Case report
Systemic arteriopulmonary venous fistula (APVF):
1
Radiology, Peninsula Radiology
Academy, Plymouth, UK
2
Radiology, University Hospitals
Plymouth NHS Trust, Plymouth,
UK
Correspondence to
Dr Paul Jenkins;
​pjenkins1@​nhs.​net
Accepted 1 July 2021
© BMJ Publishing Group
Limited 2021. No commercial
re-­use. See rights and
permissions. Published by BMJ.
To cite: Jenkins P,
Dissanayake P,
Riordan R. BMJ Case
Rep 2021;14:e241644.
doi:10.1136/bcr-2021-
241644
case presentation of an asymptomatic individual and
a review of the literature
Paul Jenkins ‍ ‍,1 Prageeth Dissanayake,2 Richard Riordan2
SUMMARY
Abnormal communications between the systemic and
pulmonary venous systems are rare but can present
as a opacity on chest radiograph. A solitary vessel
communicating as a fistula directly between the systemic
arterial circulation and the pulmonary venous system
is not widely described. These may have significant
implications in the long-­term cardiovascular health of
an individual acting as a left to right shunt. There is no
clear consensus as to the management, but surgical
management and endovascular embolisation have been
successfully used. We present a case where a systemic
arteriaopulmonary fistula originating from the abdominal
aorta and connecting to the right inferior pulmonary vein
manifested as an incidental finding on a chest radiograph
and was further evaluated on cross-­sectional imaging
in a young patient. Chest radiographs are non-­specific
and it is important to be aware of the less frequent but
important pathologies that can be picked up on plain
chest radiographs, which inturn should warrant further
investigation. This is presented in conjunction with a
review of the available literature along with a discussion
regarding the differential diagnosis and management
applicable to the general clinician.
BACKGROUND
Abnormal vascular communications as an unusual
but potential diagnosis of a chest X-­ ray opacity
is often not considered. An angiographic phase
contrast-­enhanced chest CT provides clarity
regarding abnormal vascular connections. The asso-
ciations and treatment of a variety of chest vessel
malformations depend on the exact location and
connections as well as an assessment of physiolog-
ical impact. An arterial pulmonary fistula is a rare
anomaly, which has limited literature base and no
management consensus. It is important to recog-
nise as it can develop elevated right heart pressures
with associated sequalae. Due to concerns about
right-­sided cardiac failure embolisation, surgery or
conservative management have been considered as
a treatment option.1–4
The fundamental issue with pulmonary and
systematic vascular communications is a physiolog-
ical shunting of blood either from the right-­sided
circulation to the left, thereby bypassing the lungs
or from the left back to the right without passing
through the peripheral circulation. Symptoms of
shunts are well established and primarily related to
the specific type of shunting, whether left to right
or right to left.5
Jenkins P, et al. BMJ Case Rep 2021;14:e241644. doi:10.1136/bcr-2021-241644
Right to left shunts can include dyspnoea,
haemoptysis, chest pain, cough and paradoxical
embolism (thrombotic and infective), whereas left
to right shunts can lead to right-­sided heart failure
and pulmonary hypertension. Due to increased
pressures, ‘Eisenmenger’ phenomena whereby the
right-­sided pressure exceeds the left-­sided pressures,
resulting in flow reversal and decompensation.6
CASE PRESENTATION
A 40-­year-­old woman with no significant previous
medical history or radiological imaging received an
emergency chest X-­ray due to concerns regarding
aspiration after excessive alcohol intake. An ill-­
defined density was noted within the right lower
zone on an AP radiograph, and interval Posterior-­
Anterior (PA) chest radiograph was advised. The
subsequent film demonstrated a well-­ defined
opacity with tubular conformation in the right
lower zone, mid clavicular line (figure 1). A
contrast-­enhanced CT study of the thorax was
arranged (figure 2A–E). This demonstrated a
right-­sided systemic arterial to pulmonary venous
communication between the subdiaphragmatic
aorta and the right inferior pulmonary vein, which
passed through the right lower lobe. Use of recon-
struction (figure 3) and maximal intensity projec-
tion (MIP) significantly aided the determination of
the vascular connections. These were also appre-
ciable on coronal and axial MIP (figure 4). This
vessel measured maximally 15 mm in diameter
and enhanced during the systemic arterial phase
imaging that was acquired. The right lower lobe
pulmonary veins and some upper lobe pulmo-
nary veins drain into this dilated structure, which
returned blood to the left atrium. Discussion with
the interventional radiology team and cardiotho-
racic team regarding management was undertaken
with a decision to monitor the patient for any
developing of right-­sided heart failure.
INVESTIGATIONS
Chest radiographs and chest CT are standard
studies used in daily practice by a large number of
clinicians. Unexpected findings such as soft tissue
densities with tubular conformations are unusual,
but an awareness of a wide differential and possibil-
ities is essential to ensure appropriate management.
In our individual, plain film findings were signifi-
cantly abnormal and differential diagnosis of a
bronchocoele, arterio venous malformation (AVM),
1
 Case report
Figure 1 PA chest radiograph demonstrating the right mid zone
opacity.
pulmonary sequestration, rounded pneumonia, focal consolida-
tion and nodular lesion were considered.
There were no previous films to assess growth or longevity
and further imaging was appropriately sought. Subsequent eval-
uation with a chest CT demonstrated a large intrapulmonary
vessel (figure 2). MIP reconstruction demonstrated the vessel
anatomy (figure 3) with its originating and destination connec-
tions appreciated. Further assessment of the physiological impact
of the abnormal vasculature was performed by echocardiogram,
which demonstrated no significant evidence of right ventricular
hypertrophy or strain.
DIFFERENTIAL DIAGNOSIS
While unusual, an accurate diagnosis may be achieved through
understanding of an appropriate differential and recognition of
the origin and insertion of the abnormal vasculature. The prime
differentials of abnormal cardiopulmonary vasculature would
include pulmonary AVM (PAVM), pulmonary sequestration,
Scimitar syndrome, partial anomalous venous return or pulmo-
nary vein varix.
Pulmonary arteriovenous malformations are a capillary-­based
lesion with abnormal connection between the pulmonary artery
and pulmonary vein considered to result from a defect in the
terminal capillary loops, causing dilation and formation of thin-­
walled vascular sacs. They are usually congenital and comprise
of a feeding vessel or vessels and an efferent venule. Multiple
PAVMs are commonly associated with hereditary haemorrhagic
telangiectasia.7 8 While physiologically acting as a low pressure
shunt, PAVMs (figure 4) are distinct from systemic arterial to
pulmonary venous fistulae (APVF) as the latter do not involve
the capillary bed and are connected to the systemic arterial
system.
Other differentials of abnormal pulmonary vasculature include
pulmonary sequestration, which may appear similar on the chest
radiograph, although it would demonstrate a segmental of lung
tissue without connection to the bronchial tree on CT supplied
by the systemic arterial circulation. Further differentials included
scimitar syndrome, a specific type of partial anomalous pulmo-
nary venous return (PAPVR) characterised by a hypoplastic lung
drained by an anomalous vein into the systemic circulation,
so-­called after its characteristic radiographic appearances of a
Figure 2 Axial image reconstruction on lung windows with maximal
intensity projection demonstrating abnormal right-­sided vessel
connecting to the left inferior pulmonary vein.
2 Jenkins P, et al. BMJ Case Rep 2021;14:e241644. doi:10.1136/bcr-2021-241644
Figure 3 Reconstruction of right lower lobe vessel communicating
with the right inferior pulmonary vein and aorta.
‘Turkish sword’ like opacity. This similarly leads to a right to
left shunt with associated complications and is almost exclusively
right sided and is related to a hypoplastic lung that was absent
in this case. Similar scimitar syndrome drains the pulmonary
veins into the systemic venous system rather than a connection
between the systemic arterial system and pulmonary veins in this
patient.
PAPVR is a range of rare congenital cardiovascular condition
in which some of the pulmonary veins draining oxygenated
blood back from the lungs, drain into the systemic circulation
rather than in the left atrium. This can cause a left to right
shunt with associated sequalae. This is distinct from a systemic
APVF, whereby the abnormal connection is between the systemic
circulation and a pulmonary vein feeding back towards the left
atrium, not connected to lung parenchyma. Pulmonary vein
varix, a localised aneurysmal dilation of a pulmonary vein, is a
further differential that should be considered with regards to the
finding of a dilated tubular pulmonary structure. This would be
distinct from a fistula by its normal communication between the
lung parenchyma and the left atrium.
TREATMENT
Due to the scarcity of the literature, there is little consensus as
to the management of APVFs. Conservative management may
be appropriate in vessels of small calibre where volume of blood
shunted is unlikely to have a lasting impact on right atrial pres-
sures. Similarly, depending on the presentation, comorbidities
and life expectancy of the individual at presentation, many
communications are unlikely to have a long-­term health conse-
quences and overtreatment is a real possibility. MR angiography
may be used to demonstrate the flow gradient to guide appro-
priately timed intervention.9 Although cases have been surgical
resected and embolised under 7Interventional Radiology within
the literature,1–4 this patient was managed conservatively. A
case by case management plan tailored to the individual patient
requirements based on their risk profile and multi disciplianary
team discussion is mandatory as these are complex lesions.
OUTCOME AND FOLLOW-UP
The patient was seen in clinic and following discussion, a
plan was adopted to keep this finding under surveillance with
follow-­up imaging with a rapid access pathway put in place—
as the patient is relatively young and the proposed treatment
Figure 4 Diagram from Shovlin6 indicating the major functions of the
pulmonary capillary bed that are bypassed in the setting of pulmonary
arteriovenous malformations (red arrow).

options have significant risks and complications. The patient has
had serial echocardiograms, which showed normal right and left
heart pressures with no significant evidence of volume overload
to indicate a significant extra cardiac shunt. The patient was
alive and well at 2 years follow-­up.
DISCUSSION
Although rare, the differential of an aberrant intrapulmonary
vessel is considerable and diligent tracing of the vessel origin
and insertion is essential. The use of multiplanar reformats and
MIP techniques can be useful in determining the connections.
Although digital subtraction angiography is considered the gold
standard of angiographic imaging9 contrast-­enhanced CT should
suffice.
A congenital fistula between the infradiaphragmatic aorta and
the right inferior pulmonary vein returning to the left atrium is
rarely described within the literature, with only one other case
presenting as a fistula between the abdominal aorta and the right
inferior pulmonary vein.1 Two further cases presented as a fistula
between the descending thoracic aorta and the right (one inferior
and one superior) pulmonary veins2 3 and four cases presenting
as fistulas between the descending thoracic aorta and the left
Learning points
►► Abnormal communications between the systemic arterial
circulation and the pulmonary venous system are rare, with
a limited literature base relating to systemic arterial to
pulmonary venous fistula.
►► CT scans with the use of multiplanar reconstructions and
maximum intensity projection is essential to accurately
identify the vascular origin and connections.
►► Left to right shunts can have long-­term health effects and
consideration to treatment, in particular embolisation, should
be considered.
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Jenkins P, et al. BMJ Case Rep 2021;14:e241644. doi:10.1136/bcr-2021-241644
Case report
inferior pulmonary vein.4 10 11 All the cases were adults except
one neonate2 and were alive at the publication of the reports.
Twitter Paul Jenkins @pjenkins200
Contributors PD supplied the cases. PJ wrote the manuscript and reviewed the
cases. PD and RR reviewed the cases and edited the manuscript.
Funding The authors have not declared a specific grant for this research from any
funding agency in the public, commercial or not-­for-­profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
ORCID iD
Paul Jenkins http://​orcid.​org/​0000-​0002-​4490-​316X
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