PICTORIAL ESSAY

Congenital Descending Aorta to Pulmonary Vein Fistula

Imaging Findings and Differential Diagnosis
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Miriam DeFilipp, MD and Stephen Machnicki, MD

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normal pulmonary artery distribution, and no pulmonary

Abstract: Congenital descending aorta to pulmonary vein fistula parenchymal abnormalities to suggest sequestration.9 The
(cDAPVF) is an extremely rare vascular abnormality that is usually following cases are used to highlight the relevant anatomy
diagnosed in infancy. Patients can become symptomatic, presenting and radiographic findings of cDPAVF.
with heart failure and hemoptysis. It is important to differentiate
cDAPVF from its potential mimickers due to differences in treat-
The first case is that of a 23-year-old woman with a past
ment implications. In this pictorial essay, we will highlight the medical history including ventricular septal defect (sponta-
pathophysiology and key imaging findings of cDAPVF and how to neously closed as an infant), mild dilated cardiomyopathy
separate it from its more common mimickers using cases that pre- (resolved), and asthma who was found to have a positive
sented at our institution. PPD and negative QuantiFERON-TB Gold at her annual
health follow-up. A chest x-ray was performed, which
Key Words: congenital descending aorta to pulmonary vein fistula, showed an abnormal opacity at her left lung base (Fig. 1).
pulmonary arteriovenous malformation, sequestration, anomalous Before we began our care of this patient, she was treated at
supply basal segment, vascular anomalies an outside institution with antibiotics for suspected pneu-
(J Thorac Imaging 2021;36:W62–W69) monia. The opacity persisted on follow-up chest x-ray. No
lab work is available from that time. However, the follow-up
lab work that was carried out once the patient was referred
to thoracic surgery at our institution was all within normal
ongenital descending aorta to pulmonary vein fistula
C (cDAPVF) falls under a spectrum of vascular abnor-
malities that involve systemic blood supply to a portion
limits.
A noncontrast chest CT scan was performed for further
evaluation of the persistent opacity and showed a 4.6 cm mass
of lung. A literature review showed 10 reported cases with linear central calcification in the infrahilar region of the
of cDAPVF. Four of the 10 cases were in adults and the rest left lower lobe, abutting the esophagus and descending aorta
in children and infants.1–9 Of the 10 reported cases, 6 were in (Fig. 2A). A follow-up CT chest with IV contrast was per-
male individuals and 4 were in female individuals. Nearly all formed to better evaluate the mass and revealed the lesion to
of the patients were treated with surgical ligation. While be composed of aberrant vasculature, with a systemic arterial
normally diagnosed in infancy, it can present later in life. supply arising from the descending aorta (Figs. 2B, C). No air
Common clinical presentations in infants and children bronchograms, ground-glass opacities, or bronchiectasis was
include heart failure and holosystolic murmur on
examination.7 Most adults are asymptomatic, but rarely can
present with hemopytysis. The differential diagnosis
includes pulmonary sequestration, anomalous arterial sup-
ply to normal basal segment of the lower lobe, pulmonary
arteriovenous malformations (PAVMs), scimitar syndrome,
and pulmonary varices. We will present 2 cases of patients
diagnosed with cDAPVF: one of a 23-year-old woman and
the other of a 41-year-old man, both found to have
cDAPVF incidentally on chest computed tomography (CT),
in order to highlight its key imaging findings. We will then
review the imaging findings, clinical presentations, and
treatments of pulmonary sequestration, anomalous arterial
supply to normal basal segment of the lower lobe, PAVMS,
scimitar syndrome, and pulmonary varices.

cDAPVF
cDAPVF is characterized by a descending aorta to
pulmonary vein fistula with enlarged pulmonary veins,

From the Department of Radiology, Northwell Health System, Lenox

Hill Hospital, New York, NY.
The authors declare no conflicts of interest.
Correspondence to: Miriam DeFilipp, MD, 100 East 77th Street,
New York, NY 10075 (e-mail: mdefilipp1@northwell.edu).
Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved. FIGURE 1. Posteroanterior chest x-ray demonstrates a retro-
DOI: 10.1097/RTI.0000000000000518 cardiac opacity as indicated by the red arrow.

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 J Thorac Imaging  Volume 36, Number 4, July 2021 Congenital Aorta to Pulmonary Vein Fistula

A B C
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FIGURE 2. Axial image from unenhanced chest CT with lung windows (A) shows a mass in the left infrahilar region abutting the esophagus
and aorta. There are a few internal calcifications (purple arrow). Coronal contrast-enhanced chest CT scan with MIP image and soft tissue
windows (B) shows show the aberrant vessel (red arrow) and enlarged draining vein (blue arrow). A normal draining pulmonary vein in the
right lung labeled with a green arrow for comparison. Sagittal contrast-enhanced chest CT with soft tissue windows (C) shows fistulous
connection (purple arrow) between the aberrant artery and pulmonary vein. MIP indicates maximum intensity projection.

present throughout the lungs. This was suspected to represent
an intralobar pulmonary sequestration in the left lower lobe.
Echocardiogram revealed trace mitral regurgitation, normal
cardiac function, and normal ascending aorta.
The patient was referred to a thoracic surgeon at our
institution who performed a left lower lobe lobectomy.
Pathology showed anomalous arterial supply with prom-
inent vessels. No definitive features of bronchopulmonary
sequestration were identified. While initial interpretation of
the CT scan concluded that the patient had pulmonary
sequestration, reevaluation shows that she had cDAPVF.
Imaging shows an anomalous artery from the descending
aorta with drainage via enlarged pulmonary veins. There is
normal pulmonary artery distribution to the left lower lobe.
There are no signs of abnormal lung tissue or consolidation
in the left lower lobe to indicate sequestration (Fig. 2).
The second case is that of a 41-year-old man with no
significant past medical history who presented to the ER at
our institution for left-sided rib pain status after an assault.
An initial noncontrast CT chest revealed a 3.5 cm left lower
lobe mass thought to be vascular in origin involving the
descending aorta and inferior pulmonary vein (Fig. 3A).
Additional findings (not shown) included an acute left rib
fracture, a small left pleural effusion, and a small amount of
atelectasis in the left lower lobe. A CT with IV contrast was
recommended for further evaluation and to rule out
underlying malignant mass. The follow-up CT showed an
anomalous vessel arising from the descending aorta with
drainage via enlarged left inferior pulmonary veins
(Figs. 3B, C). There was no abnormal lung tissue to suggest
sequestration. Normal pulmonary artery distribution to the
left lower lobe was present. The patient was instructed to
follow-up with thoracic surgery for evaluation and possible
treatment. He was lost to follow-up.
Understanding the pathophysiology of cDAPVF is key
to explaining the patients’ symptoms and how they are
treated. The fistula creates a left-to-left shunt in which blood
goes from the descending aorta to the pulmonary veins and
into the left atrium. As the shunt worsens, there will be
dilation of pulmonary veins, the left atrium, and potentially
the pulmonary artery. Hemoptysis is believed to result
from rupture of small pulmonary veins. In cDAPVF, the
lung parenchyma receives its blood supply from the bron-
chial artery and not the anomalous aortic branch (Fig. 4).
Thus, when treating patients with this congenital disorder,
surgeons can ligate the descending aortic branch without
concern that they will compromise blood flow to that
tissue.8,9

A B C

FIGURE 3. Axial unenhanced chest CT image with lung windows (A) showing a mass like opacity in the left infrahilar region associated
with the pulmonary vasculature. There are no associated pulmonary parenchymal abnormalities. Coronal (B) and sagittal (C) contrast-
enhanced chest CT with MIP image and soft tissue windows shows aberrant vessel of the descending pulmonary artery (red arrow) and
enlarged draining pulmonary veins (blue arrow). A normal draining pulmonary vein in the right lung is labeled with a green arrow on MIP
imaging for comparison. The fistulous connection between the aberrant artery and pulmonary vein is indicated with a purple arrow (C).
MIP indicates maximum intensity projection.

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FIGURE 4. Schematic diagram shows the pathophysiology of the left-left shunt from cDAPVF (highlighted by the green box). Dilated
pulmonary vein carries oxygenated blood back to the left atrium (LA). There is normal pulmonary artery supply and normal bronchial
anatomy. IVC indicates inferior vena cava; LV, left ventricle; RA, right atrium; RV, right ventricle; SVC, superior vena cava.

DIFFERENTIAL DIAGNOSIS formation separate from the tracheobronchial tree. This

It is important to differentiate cDAPVF from other
mimickers, which include pulmonary sequestration, anom-
alous arterial supply to normal basal segment of the lower
lobe, arteriovenous malformations, scimitar syndrome, and
pulmonary varices.
PULMONARY SEQUESTRATION
Pulmonary sequestration, which is divided into extra-
lobar or intralobar, is characterized by abnormal lung tissue
abnormal tissue has systemic arterial supply from the aorta,
but without normal pulmonary artery supply, and can have
multiple internal calcifications (Figs. 5, 6). Both intralobar
and extralobar sequestrations tend to have arterial
supply via an anomalous branch off the aorta. Intralobar
sequestration often has venous drainage via the pulmonary
veins, while the extralobar sequestration drains via systemic
veins into the right atrium. Extralobar sequestration is fur-
ther differentiated from intralobar sequestration by the
presence of its own pleura.10,11 The overall incidence of
pulmonary sequestration is extremely low, ∼1% of all
bronchopulmonary congenital anomalies, with the majority
of cases being intralobar.11 Some literature has reported a
4:1 male to female preference.12
Clinically patients with pulmonary sequestration tend
to present with repeat pulmonary infections as opposed to
heart failure.11 On CT imaging, sequestration can be dif-
ferentiated from cDAPVF by the presence of abnormal lung
tissue and abnormal bronchial distribution in that segment.
In addition, there will be lack of pulmonary artery dis-
tribution in the area of concern. Pulmonary veins will be
normal in caliber.9 With sequestration, the arterial supply to
the abnormal lung parenchyma comes primarily from the
aberrant aortic vessel and to a lesser extent from the bron-
chial artery. Because a large portion of the blood to the
sequestered parenchyma is supplied by the anomalous ves-
sel, if one were to treat the condition with ligation of the
artery, there is increased risk for parenchymal ischemia.
Therefore, sequestration is treated by surgical resection of
the abnormal parenchyma (Fig. 7).7

ANOMALOUS ARTERIAL SUPPLY TO NORMAL

BASAL SEGMENT OF THE LOWER LOBE
Another rare mimic of cDAPVF that needs to be ruled
FIGURE 5. Posteroanterior chest x-ray showing an opacity along out is anomalous arterial supply to normal basal segments
the left cardiac border (red arrow). of the lower lobe. Only a handful of cases of it have been

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A B
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FIGURE 6. Coronal (A) and coronal oblique (B) contrast-enhanced chest CT scan with MIP image and soft tissue windows shows the
previously seen opacity on chest x-ray in Figure 5 to be an area of pulmonary sequestration in the left lower lobe. Note the multiple
anomalous feeding vessels from the aorta (red arrows) and mass like area of sequestered lung tissue in the left lower lobe. B, Drainage of
the sequestered lung tissue via the pulmonary vein (blue arrow). IVC indicates inferior vena cava; LA, left atrium; LV, left ventricle; MIP,
maximum intensity projection; RA, right atrium; RV, right ventricle; SVC, superior vena cava.

FIGURE 7. Schematic diagram showing the pathophysiology of pulmonary sequestration (highlighted by the green box). Note that most of the
blood supply to the sequestered lung tissue comes from the aberrant vessel. Thus, ligation of that vessel as a treatment option runs the risk of
infarction of the sequestered tissue. There is no pulmonary arterial supply to the affected area, as indicated by the red slashes and green box.

A B

FIGURE 8. Axial contrast-enhanced chest CT image with soft tissue MIP windows (A) and coronal contrast-enhanced chest CT with MIP image and
lung windows (B) showing anomalous arterial supply to normal basal lower lobe segment. Small aberrant artery (red arrow) is seen branching off the
lower descending thoracic aorta. The draining pulmonary veins (blue arrow) are not enlarged. MIP indicates maximum intensity projection.

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FIGURE 9. Schematic diagram showing the pathophysiology of anomalous arterial supply to normal basal lobe segment of the lower
lobe (highlighted by the green box). While this vascular anomaly appears very similar to cDAPVF, it differs in that there is no pulmonary
arterial supply to that segment (red slashes and green box), and the draining pulmonary veins are normal in caliber. IVC indicates inferior
vena cava; LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle; SVC, superior vena cava.

reported in the literature with a slight male predominance.13
Similar to cDAPVF, in this congenital disorder, there is
anomalous arterial supply to a segment of lung (left lower
lobe more than right lower lobe) from a branch of the
descending aorta with drainage via the pulmonary veins
(Fig. 8). There is normal bronchial supply to the lung in that
region. The key differentiators between the two are that in
anomalous arterial supply to normal basal segment of the
lower lobe, there is a lack of pulmonary vein dilation and a
lack of normal pulmonary arterial flow to the affected area
(Figs. 8, 9). Patients may be asymptomatic, but could
present with hemoptysis. As the anomalous artery develops,
the proximal portion is muscular, while the more distal
portion within the lung parenchyma is elastic. Overtime,
with increased systemic arterial pressure, the anomalous
artery can develop atherosclerotic changes leading to
rupture causing hemoptysis. Treatment includes resection of
the abnormal lung segment.13
PULMONARY ARTERIOVENOUS
MALFORMATIONS
Pulmonary arteriovenous malformations can mimic
cDAPVFs. Because PAVMs are significantly more com-
mon than cDAPVF and its mimics, special attention should
be paid to rule out PAVMs. While significantly more
common than cDPAVF, PAVMs still have a relatively low
incidence rate of ∼3/15,000 people.14 PAVMs can present
with a single lesion, but, often, patients present with mul-
tiple PAVMs associated most commonly with hereditary
hemorrhagic telangiectasias. Patients with PAVMs can be
asymptomatic or often present with hemoptysis and

A B C

FIGURE 10. Posteroanteior chest x-ray (A) showing a subtle opacity (red arrow) in the left lower lobe. CT angiography of the same
patient shows on axial lung windows (B) 2 small nodular opacities in the lingula (green arrow). The sagittal contrast-enhanced MIP image
with soft tissue windows (C) shows that the nodular opacity is consistent with an arteriovenous malformations characterized by a feeding
artery (red arrow), nidus of vessels (green arrow), and draining vein (blue arrow) that bypasses the pulmonary capillary system. MIP
indicates maximum intensity projection.

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pulmonary artery to vein, effectively bypassing the capillary

bed (Figs. 10, 11). As PAVMs do not provide blood supply
to lung parenchyma, they are generally treated with either
coil or chemical embolization without fear of parenchymal
infarction.16
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Scimitar Syndrome
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Scimitar syndrome falls under the category of con-

FIGURE 11. Schematic diagram shows the pathophysiology of
PAVMs (highlighted by the green box). There is an abnormal
feeding artery that branches off the pulmonary artery, which is
connected to the draining vein via the cluster of abnormal vessels
(nidus). The draining vein will drain deoxygenated blood back
into the pulmonary vein, effectively bypassing the pulmonary
capillary system. IVC indicates inferior vena cava; LA, left atrium;
LV, left ventricle; RA, right atrium; RV, right ventricle; SVC, superior
vena cava.
shortness of breath. Arteriovenous malformations are a
type of high-flow venous malformation constituted by
abnormal pulmonary arterial and venous connections that
bypass capillary beds. The connection, also known as the
“nidus,” is a collection of vessels at the point of
communication.15 They are often located more peripherally
in the lung.14 On CT, they are characterized by an abnor-
mal cluster of vessels with abnormal feeding pulmonary
artery and draining pulmonary vein. Generally, they form
right to left shunts due to shunting of blood from the
genital anomalous pulmonary venous return abnormalities.
Because of the presence of abnormal vasculature, it is
an important anomaly to consider in the differential for
CDAPVF.
Scimitar syndrome is seen almost exclusively in the
right lung and is characterized by enlarged abnormal
pulmonary venous drainage into the systemic system,
hypoplastic right lung, dextrocardia, hypoplasia of the right
pulmonary artery, and abnormal systemic arterial supply
(Fig. 12). On x-ray, the large arching right pulmonary vein
was thought to look like the Middle Eastern sabre known as
a scimitar.
On CT, the enlarged anomalous pulmonary vein drains
most commonly into the IVC at the subdiaphragmatic level.
Rarely, it can be seen to drain into the hepatic and portal
veins. Meandering pulmonary vein also known as Pseudo-
Scimitar syndrome occurs when there is a similar array of
findings, as in Scimitar syndrome, but the tortuous right
pulmonary vein drains into the left atrium. Scimitar syn-
drome leads to a left to right shunt, which in severe cases can
lead to heart failure (Fig. 13).17 Scimitar can present at any
age, has a female predominance, and can be associated with
other congenital anomalies such as atrial septal defects.18
Most patients with scimitar are asymptomatic. However,
younger patients with more severe forms of the anomaly
tend to present with recurrent infections (due to hypoplastic
right lung) and symptoms of heart failure (due to shunt).
Because ligation of the artery results in high risk of
pulmonary infarction and edema, treatment generally
involves surgically redirecting the pulmonary vein back in to
the left atrium.17
Pulmonary Varices
Lastly, pulmonary varices should be considered in the
differential for cDAPVF and other pulmonary vascular

A B

FIGURE 12. Axial contrast-enhanced chest CT with soft tissue windows (A) shows hypoplasia of the right pulmonary artery (red arrow)
seen in the setting of scimitar syndrome. Normal left pulmonary artery is indicated by a green arrow for comparison. Coronal contrast-
enhanced chest CT with MIP image and lung windows (B) shows enlarged abnormal right pulmonary vein (blue arrow) and small
anomalous systemic artery supplying the right lower lobe (purple arrow). Note the hypoplastic right lung size in comparison with the left.
MIP indicates maximum intensity projection.

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FIGURE 13. Schematic diagram showing the pathophysiology of scimitar syndrome, which results in a left-to-right shunt from the right
pulmonary vein to the IVC (highlighted by larger green box). There is hypoplasia of the right pulmonary artery (as indicated by slashes).
In addition, there is abnormal systemic arterial supply (highlighted by the smaller green box) which arises most commonly from the infra-
diaphgramatic aorta and supplies the posterior segment of the right lower lobe. IVC indicates inferior vena cava; LA, left atrium; LV, left
ventricle; RA, right atrium; RV, right ventricle; SVC, superior vena cava.

abnormalities. Pulmonary varices are nearly always
asymptomatic. A small number of patients can present with
hemoptysis due to increased backpressure on the pulmonary
vein. Pulmonary varices are extremely rare with a true
incidence rate not reported in the literature. They can be
congenital or acquired in the setting of mitral regurgitation
or pulmonary hypertension. Imaging findings on CT include
a dilated segment of pulmonary vein that is connected to the
normal pulmonary venous vasculature (Figs. 14, 15). There
is no abnormal systemic arterial supply to the lung.19
Asymptomatic patients do not require therapeutic inter-
vention and can be monitored with interval CTs to evaluate
for stability. In those who develop varices in the setting of
heart failure, valvular replacement and treatment of con-
gestive heart failure can lead to spontaneous resolution of
pulmonary varix. Variceal resection is generally reserved for
patients with hemoptysis.20
CONCLUSIONS
While cDAPVF is an extremely rare congenital vas-
cular anomaly, it is important for radiologists to be aware of
this entity when one suspects a vascular anomaly in the chest
because of treatment implications. cDAPVF may be treated
with ligation, while common mimickers such as pulmonary
arteriovenous malformations are usually treated with
embolization. Other entities such as pulmonary sequestra-
tion, anomalous arterial supply to normal basal segment of
the lower lobe, and scimitar syndrome can be treated with
surgical intervention or resection. In contrast, pulmonary

A B

FIGURE 14. Coronal contrast-enhanced soft tissue MIP window. Normal pulmonary vein is indicated by the green arrow for comparison
(A) and axial contrast-enhanced lung window (B) shows focal segment of the dilated right pulmonary vein (blue arrow) consistent with
a varix. MIP indicates maximum intensity projection.

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FIGURE 15. Schematic diagram shows the pathophysiology of pulmonary varix (highlighted by the green box). There is a focal segment
of abnormally dilated pulmonary vein draining into the normal pulmonary vein vasculature. IVC indicates inferior vena cava; LA, left
atrium; LV, left ventricle; RA, right atrium; RV, right ventricle; SVC, superior vena cava.

varices rarely necessitate surgical intervention and are usu-
ally observed with follow-up imaging.
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