Images in Cardiovascular Medicine

Aorta–to–Pulmonary Vein Fistula in an

Asymptomatic 25-Year-Old Man
Arun Dahiya, MD; Patrick Collier, MB, PhD; Richard Krasuski, MD;
Vidyasagar Kalahasti, MD; Pedro del Nido, MD; William J. Stewart, MD

A 25-year-old man with no significant medical history was

found to have a continuous murmur along the left sternal
border on routine physical examination performed in prepa-
flow ratio of 1.8, consistent with a large left-to-left shunt.
Cardiac computed tomography (CT) confirmed the presence
of a large fistulous connection between the abdominal aorta
ration for running a marathon. Echocardiography showed and the right inferior pulmonary vein (Figure 2 and Movie
a dilated left ventricle with normal function and continu- III in the online-only Data Supplement) located in the right
ous high-velocity flow in an aberrant vessel in close vicin- lower lobe (Figure 3A). Although there was no distinct lung
ity to the abdominal aorta (Movie I in the online-only Data parenchyma changes on CT and MRI, the presence of con-
Supplement). Chest x-ray (posterior-anterior and lateral pro- glomerated fistula vessels in the right lower lung lobe limited
jections, Figure 1) disclosed conglomeration of tubular densi- the ability to exclude associated intralobar lung sequestration.
ties in a scimitar pattern in the right lower lobe suggestive of Therefore, cardiac catheterization (Movie IV in the online-
anomalous pulmonary venous return of right lower lobe (scim- only Data Supplement) was subsequently performed before
itar syndrome; anomalous drainage of right lung pulmonary consideration of percutaneous closure of the fistula. However,
veins into the inferior vena cava). Cardiac magnetic resonance this treatment option was abandoned because of concerns that
imaging (MRI) not only demonstrated normal drainage of all perfusion of the right lower lung lobe was fistula dependent.
4 pulmonary veins into the left atrium but also revealed an The patient was then referred for open surgery to determine
aberrant vessel (Movie II in the online-only Data Supplement) whether the fistula could be closed safely, which was done
extending between the abdominal aorta and the right inferior by temporarily occluding the fistula with a snare and observ-
pulmonary vein with an elevated systemic-to-pulmonary ing the color of the segment of the lung after 5 minutes of

Figure 1. Chest x-ray

(posterior-anterior and lateral
projections) revealing conglom-
eration of tubular densities in
a scimitar pattern in the right
lower lobe (arrow).

From the Department of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, OH (A.D., P.C., R.K., V.K., W.J.S.); Department of
Cardiovascular Surgery, Children Hospital Boston, MA (P.d.N.); School of Medicine, University of Queensland, Queensland, Australia (A.D.); and Royal
Brisbane and Women’s Hospital, Queensland, Australia (A.D.).
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.
112.125757/-/DC1.
Correspondence to Arun Dahiya, MD, Department of Cardiology, Royal Brisbane and Women’s Hospital, Herston, Queensland 4029 Australia. E-mail
[email protected]
(Circulation. 2013;127:1727-1729.)
© 2013 American Heart Association, Inc.
Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIRCULATIONAHA.112.125757

1727
1728  Circulation  April 23, 2013

Figure 2. Volume-rendered cardiac computed tomography

revealing the presence of a fistula (arrow) between the abdomi-
nal aorta and the right inferior pulmonary vein.

occlusion. Arterial blood gases were also obtained at that time.

Because no changes were detected, the fistula was then per-
manently ligated. Although inspection of the lung surface dur-
ing fistula occlusion is not a direct indication of the presence
of normal pulmonary artery blood supply to that segment, it
can confirm that pulmonary infarct is unlikely to occur. This
was the only concern and the reason for the test occlusion.
Ultimately, surgical ligation resulted in no complications,
and there was no need for lung resection. Postoperative car-
diac CT subsequently showed that the left ventricular size Figure 3. A, Presurgical cardiac computed tomography (CT)
reverted to normal and that no residual fistula was present images demonstrating a fistula within the posterior right lower
lung lobe (yellow arrow). Postsurgical cardiac CT shows normal
(Figure 3A and 3B). lungs after successful ligation of the fistula. B, Presurgical car-
An abdominal aorta–to–pulmonary vein fistula is a very diac CT images demonstrating dilated left ventricle (red arrow).
rare congenital anomaly that previously had been described Postsurgical cardiac CT shows a reduction in the size of the left
only in infancy.1,2 To the best of our knowledge, this is the ventricle after successful ligation of the fistula.
first case report of a large systemic circulation–to–pulmonary
vein fistula associated with chamber dilation and a high- main consideration in our case. Lung sequestration if present
volume shunt in an asymptomatic adult. This case highlights usually requires lobectomy6 or embolization.7 In contrast,
the key role of multimodality imaging in the diagnosis and patients without sequestration can be managed by surgical
management of a rare congenital anomaly. Tomographic ligation1 or percutaneous closure of the fistula.2 CT is excellent
investigations such as cardiac CT or MRI should be considered at depicting parenchymal abnormalities associated with lung
in cases when echocardiography fails to identify the cause of sequestration, which has a typical appearance of a complex
a murmur, particularly if chamber dilation is present. More mass with and without cystic changes. CT angiogram and
specifically, in the setting of a systemic–to–pulmonary vein multiplanar MRI not only can demonstrate parenchymal lung
fistula, cardiac CT or MRI can also diagnose the presence changes but also can delineate its systemic arterial supply and
of concomitant lung sequestration,3–5 which is critical with venous drainage. Invasive angiography may be indicated if
regard to subsequent disease management. Lung sequestration noninvasive evaluation is nonconfirmatory.8 Rarely, in patients
is defined as a nonfunctioning mass of normal lung tissue that with hemodynamically significant aberrant pulmonary vessel,
lacks normal communication with the pulmonary arterial noninvasive and invasive evaluation is unable to confidently
system and the tracheobronchial tree3 and receives its blood rule out associated lung sequestration (as in this case),
supply from 1 or more anomalous systemic arteries. On the warranting the safer approach of surgical ligation because it
basis of its pleural covering, lung sequestration is further facilitates direct inspection of the lung surface for infarction
divided into 2 subtypes. Extralobar lung sequestration has during temporary fistula occlusion, which in turn dictates the
a distinct pleural covering resulting in complete anatomic final surgical strategy.
separation of the mass from adjacent normal lung tissue,
and intralobar sequestrations are masses of lung tissue in Disclosures
continuation with adjoining normal lung, which was the None.
 Dahiya et al   Aorta–to–Pulmonary Vein Fistula   1729

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