24. De Blanche LE, Schmitz ML, Johnson CE, et al. Successful 43. Matsuzaki K, Koishizawa T, Hiramatsu Y.
iramatsu Y. A case report of pul-
surgical management of a neonate with a saddle pulmonary
embolus. Ann Thorac Surg. 2004;78(1):1-2.
25. Deehring R, Kiss AB, Garrett A, Hillier AG. Extracorporeal
membrane oxygenation as a bridge to surgical embolectomy
in acute fulminant pulmonary embolism. Am J Emerg Med.
2006;24(7):879-880.
26. Burgoyne LL, Anghelescu DL, Tamburro RF, De Armendi AJ.
A pediatric patient with a mediastinal mass and pulmonary
embolus. Pediatric Anesthesia. 2006;16(4):487-491.
27. Sur JP, Garg RK, Jolly N. Rheolytic percutaneous thrombec-
tomy for acute pulmonary embolism in a pediatric patient.
Catheter Cardiovasc Interv. 2007;70(3):450-453.
28. Van den Heuvel-Eibrink MM, Lankhorst B, Egeler RM,
Corel LJ. Sudden death due to pulmonary embolism as pre-
senting symptom of renal tumors. Pediatr Blood Cancer.
2008;50(5):1062-1064.
29. Jean N, Labombarda F, De La Gastine G, Raisky O,
Boudjemline Y. Successful pulmonary embolectomy in a
4-year-old girl with antithrombin III deficiency. Pediatr Cardiol.
2010;31(5):711-713.
30. Baldursdottir S, Torfason B, Sigfusson G, Benediktsdottir K,
Bjarnason R. Pulmonary embolism in a teenage girl [in
Icelandic]. Laeknabladid. 2011;97(2):97-99.
31. Kamijo Y, Soma K, Nagai T, Kurihara K, Ohwada T. Acute
massive pulmonary thromboembolism associated with risperi-
done and conventional phenothiazines. Circ J. 2003;67(1):46-48.
32. Kawahito K, Adachi H. Balloon catheter pulmonary embolec-
tomy under direct visual control using a choledochoscope.
Ann Thorac Surg. 2011;91(2):621-623.
33. Ngaage DL, Shah R, Sanjay SP, Cale AR. Cardiopulmonary
endoscopy: an effective and low risk method of examining
the cardiopulmonary system during cardiac surgery. Eur J
Cardiothorac Surg. 2001;19(2):152-155.
34. Wilkens H, Lang I, Behr J, et al. Chronic thromboembolic
pulmonary hypertension (CTEPH): updated recommenda-
tions of the Cologne Consensus Conference 2011. Int J
Cardiol. 2011;154(suppl 1):S54-S60.
35. Kim NH, Lang IM. Risk factors for chronic thromboembolic
pulmonary hypertension. Eur Respir Rev. 2012;21(123):27-31.
36. Beckman D, Solmos B, Herod G, Siderys H. Intraoperative
pulmonary angioscopy using the flexible fiberoptic choledo-
choscope. Ann Thorac Surg. 1986;41(5):563-564.
37. Morshuis WJ, Jansen EW, Vincent JG, Heystraten FJ,
Lacquet LK. Intraoperative fiberoptic angioscopy to evaluate
the completeness of pulmonary embolectomy. J Cardiovasc
Surg (Torino). 1989;30(4):630-634.
38. Yamanaka K, Miki S, Kusuhara K, Ueda Y, Okita Y, Tahata T.
Intraoperative pulmonary angioscopy to undergo pulmo-
nary embolectomy for acute massive pulmonary embolism
[in Japanese]. Nippon Kyobu Geka Gakkai Zasshi. 1994;
42(10):1940-1943.
39. Kawahito K, Murata S, Ino T, Fuse K. Angioscopic pul-
monary embolectomy and ECMO. Ann Thorac Surg. 1998;
66(3):980-989.
40. Uno Y, Horikoshi S, Emoto H, Koyanagi K. Successful direct
embolectomy for acute massive pulmonary thromboembolism.
Nippon Kyobu Geka Gakkai Zasshi. 1996;44(10):1958-1961.
41. Dartevelle P, Fadel E, Chapelier A, et al. Pulmonary throm-
boendarterectomy with video-angioscopy and circulatory
arrest: an alternative to cardiopulmonary transplantation and
post-embolism pulmonary artery hypertension [in French].
Chirurgie. 1998;123(1):32-40.
42. Dartevelle P, Fadel E, Chapelier A, Macchiarini P. Angio-
scopic video-assisted pulmonary endarterectomy for post-
embolic pulmonary hypertension. Eur J Cardiothorac Surg.
1999;16(1):38-43.
journal.publications.chestnet.org
Downloaded From: http://journal.publications.chestnet.org/ by David Kinnison on 02/08/2013
monary embolectomy using an endoscope for the detection of
residual emboli [in Japanese]. Kyobu Geka. 1998;51(6):461-463.
44. Soyer R, Brunet AP, Redonnet M, Borg JY, Hubscher C,
Letac B. Follow-up of surgically treated patients with mas-
sive pulmonary embolism—with reference to 12 operated
patients. Thorac Cardiovasc Surg. 1982;30(2):103-108.
An Occult Congenital Fistula
Between the Descending Aorta
and the Left Pulmonary Vein
in an Adult Presenting With
Recurrent Episodes of
Hemoptysis
Yijie Hu, MD, PhD; Qianjin Zhong, MD, PhD;
Zhiping Li, MD, PhD; Jianming Chen, MD, PhD;
Cheng Shen, MD, PhD; and Yi Song, MM
We report a case of recurrent hemoptysis due to
an occult congenital fistula between the descending
aorta and the left pulmonary vein in a 25-year-old
female patient. The anomaly was confirmed by contrast-
enhanced CT scan and angiography. No abnormality
was noted in the bronchia and pulmonary arteries.
The patient was successfully managed by simple liga-
tion of the fistula. To our knowledge, this is the first
reported case of adult-onset hemoptysis caused by
an occult congenital fistula between the descending
aorta and a pulmonary vein.
CHEST 2013; 143(2):549–553
Abbreviations: DAPVF 5 descending aorta-pulmonary vein fistula
H emoptysis can be caused by a variety of conditions,
including tracheobronchial diseases, pulmonary paren-
chymal lesions, primary vascular anomalies, and systemic
coagulopathies.1 Here, we present the first report, to our
knowledge, of an adult case of hemoptysis due to congen-
ital descending aorta-pulmonary vein fistula (DAPVF).
Case Report
A 25-year-old female teacher was admitted to our hospi-
tal because of recurrent hemoptysis over 1 year. Initially the
patient complained about expectoration of blood-streaked
mucus, which occurred several times over 6 months. The
Manuscript received December 20, 2011; revision accepted June 1,
2012.
Affiliations: From the Department of Cardiovascular Surgery,
Institute of Surgery Research, Daping Hospital, Third Military
Medical University, Chongqing, China.
Correspondence to: Qianjin Zhong, MD, PhD, No. 10 Changjiang
Zhi Rd, Yuzhong District, Chongqing 400042, China; e-mail:
zhongqianjin@qq.com
© 2013 American College of Chest Physicians. Reproduction
of this article is prohibited without written permission from the
American College of Chest Physicians. See online for more details.
DOI: 10.1378/chest.11-3229
CHEST / 143 / 2 / FEBRUARY 2013 549

Figure 1. Preoperative chest radiograph shows a smooth and highly
dense opacity in the left lower lobe behind the heart (black arrow).
situation worsened in the subsequent month, and the patient
reported one to three expectorations of a scant volume of
bright red blood (about 5 mL) or blood clot. Exercise or
menstruation did not appear to have any apparent effect
on these episodes. The patient denied having fever, chest
pain, night sweats, shortness of breath, or any anticoagu-
lant use. On physical examination, she appeared to be in
good general condition.
A left parasternal grade 3/6 systolic murmur was heard
over the fourth intercostal space, with a normal pulmo-
nary valve closure (P2) sound. Echocardiography showed
a normal cardiac contour without any defect or anomalous
communication. Chest radiograph (Fig 1) and fluoroscopy
(Video 1) revealed a smooth, highly dense opacity in the
left lower lobe. Contrast-enhanced CT scan of the chest
showed an anomalous arterial branch, 9 mm in the narrow-
est inner diameter, that arose from the descending aorta
and was connected with the left pulmonary vein before its
entry into the left atrium (Figs 2A-D). The pulmonary vein
was dilated; the bronchial tree (Fig 2E) and pulmonary
arterial branches (Fig 2F) appeared normal. Angiography
showed that the contrast agent entered the lower tributaries
of the left inferior pulmonary vein through the DAPVF
(Fig 3A) before returning to the left inferior pulmonary
vein and joining the left atrium (Fig 3B, Videos 2, 3).
Proliferating vessels suffused the basal segments of
the left inferior lobe obviously (Fig 4A). An anomalous
large vessel originated from the descending aorta and then
entered the left inferior lobe (Figs 4B, 4C). After ligation
of the anomalous vessel through a left thoracotomy using
the same surgical procedure as for patent ductus arteriosus,
the aneurysmatic part of the anomalous vessel in the lung
disappeared (Fig 4D). Intraoperative biopsy was not per-
formed because the patient refused to provide informed
consent.

Figure 2. Contrast-enhanced CT scan shows the anatomic structure of DAPVF, the left bronchial tree,
and left pulmonary branches. A, The anomalous systemic artery (*) arises from the descending aorta.
B, The anomalous systemic artery (*) is connected with the left inferior pulmonary vein. C, The dilated
inferior pulmonary vein (black arrow) joins the left atrium. D, Three-dimensional reconstruction of
DAPVF. The narrowest inner diameter of DAPVF is 9 mm. E, The normal left bronchial tree. F, The
normal left pulmonary arterial branches. DA 5 descending aorta; DAPVF 5 descending aorta-pulmonary
vein fistula; LPA 5 left pulmonary artery.

550 Selected Reports

Downloaded From: http://journal.publications.chestnet.org/ by David Kinnison on 02/08/2013

 Figure 3. A, Angiography shows that the contrast agent has entered the lower tributaries of the left inferior
pulmonary vein, but not the tributaries of the superior segment, through the DAPVF. B, It then returns to
the left inferior pulmonary vein and joins the left atrium. See Figure 2 legend for expansion of abbreviation.

The patient recovered smoothly and was free of hemop-
tysis during the subsequent 6 months of follow-up. Postop-
erative angiography (Fig 5A, Video 4) and CT scan (Fig 5B)
revealed no evidence of DAPVF, and the pulmonary circu-
lation and tracheobronchial structure also appeared normal
(Fig 5C, Video 5).
Discussion
Major hemodynamic changes in DAPVF include left-
to-left shunt and increased pulmonary venous pressure.
The left-to-left shunt, which produces left parasternal sys-
tolic murmur over the fourth intercostal space at the same
horizontal level as DAPVF, results in enlargement of the
left atrium and left ventricle and, eventually, heart fail-
ure. Cardiac failure is the presenting feature in the only
reported case of neonatal DAPVF.2 The other pathophysi-
ologic change is elevated venous pressure of the involved
pulmonary vein, which is largely due to the communica-
tion between the pulmonary vein and the descending aorta,
thus providing a passage for retrograde perfusion of the
lung parenchyma and blood drainage to the left atrium.

Figure 4. Intraoperative view of DAPVF. A, Proliferating vessels suffuse the basal segments of the left
inferior lobe obviously (white arrow), but not the superior segment (black arrow) or left superior lobe.
B, An anomalous large vessel originates from the descending aorta and then enters the left inferior lobe,
aneurysmatically dilated (white arrow). C, The anomalous vessel is separated (white arrow). D, The
anomalous vessel is ligated with the same surgical procedure as for patent ductus arteriosus. E, After
ligation of the anomalous vessel, the aneurysmatic part of anomalous vessel in the lung has disappeared
(white arrow). See Figure 2 legend for expansion of abbreviation.

journal.publications.chestnet.org CHEST / 143 / 2 / FEBRUARY 2013 551

Downloaded From: http://journal.publications.chestnet.org/ by David Kinnison on 02/08/2013

 Figure 5. Postoperative angiography and CT scan reconstruction. A, Angiography demonstrates no
DAPVF. B and C, CT scan reconstruction shows that the pulmonary vein and tracheobronchial struc-
ture are normal. See Figure 2 legend for expansion of abbreviation.

Interestingly, pressure at the ostium of segmental veins pulmonary sequestration. Currarino et al10 reported three
varies with their specific positions. This was proved by cases of congenital fistula between a systemic artery and a
preoperative angiography, which showed that the contrast pulmonary vein, and they found no evidence of pulmo-
agent entered the lower tributaries of the left inferior pul- nary sequestration. Consequently, ligation of the fistula is
monary vein, but not the tributaries of the superior seg- a proper surgical treatment to forestall the development
ment. As a result, proliferating vessels covering different of irreversible pulmonary hypertension, with no need for
segments were dilated to various degrees, as seen in the resection of lung tissues.
operation. Persistently elevated venous pressure, varied at In conclusion, DAPVF is a rare, congenital, thoracic dis-
different venous segments, could lead to segmental, lobar, ease, which can induce hemoptysis or even heart failure.
or lateral pulmonary arterial hypertension. Rupture of pro- Contrast-enhanced CT scan and angiography can aid in the
liferating and dilated vessels of the basal segments secondary correct diagnosis of this uncommon entity. We are more
to elevation of pulmonary venous pressure likely underlay inclined to regard it only as a type of vascular anomaly,
hemoptysis in our patient. rather than a special type of pulmonary sequestration. Sim-
There is a question of whether congenital DAPVF belongs ple ligation of DAPVF, without resection of lung tissues,
to the spectrum of “pulmonary sequestrations,” despite its is an effective treatment option.
obscure embryonic origin. Traditionally, pulmonary seques-
tration encompasses abnormal arterial connection and the
spectrum of pulmonary abnormalities usually associated
with it.3,4 The current thinking holds that pulmonary seques-
trations are a spectrum of anomalies ranging from an area
of sequestered lung with normal pulmonary artery supply
to an area of normal lung with systemic artery supply,5-7
including type 1 intralobar pulmonary sequestration, in
which the anomalous systemic artery supplies to the nor-
mal lung. In fact, the anatomic structures of DAPVF and
type 1 intralobar pulmonary sequestration are different.
Although both are covered with the visceral pleura, the
anomalous systemic artery supplies the pulmonary paren-
chyma in type 1 intralobar pulmonary sequestration (Fig 6A),
whereas it is connected with the pulmonary vein outside
the parenchyma in DAPVF (Fig 6B). Based on that, sim-
ple occlusion or ligation of the anomalous artery in type 1
intralobar pulmonary sequestration runs the risk of pulmo-
nary infarction8 because it accounts for a considerable part
of arterial supply to the pulmonary parenchyma. Generally,
lobectomy needs to be considered.9 However, pulmonary
arterial branches are completely normal in DAPVF, although
there is some retrograde perfusion through the pulmonary
vein because of the pressure gradient between the descend-
ing aorta and pulmonary vein (shown in preoperative angi- Figure 6. The schematic view of type 1 intralobar pulmonary
ography). Therefore, occlusion or ligation of the anomalous sequestration and DAPVF. A, An anomalous systemic artery
artery in DAPVF will restore the normal pulmonary circu- supplies the pulmonary parenchyma in type 1 intralobar pulmo-
nary sequestration. B, The anomalous systemic artery is connected
lation without the risk of pulmonary infarction. with the pulmonary vein outside the parenchyma in the reported
We are more inclined to regard congenital DAPVF only case, although both of them enter the visceral pleura. See Figure 2
as a type of vascular anomaly, rather than a special type of legend for expansion of abbreviation.

552 Selected Reports

Downloaded From: http://journal.publications.chestnet.org/ by David Kinnison on 02/08/2013

 Acknowledgments
Financial/nonfinancial disclosures: The authors have reported
to CHEST that no potential conflicts of interest exist with any
companies/organizations whose products or services may be dis-
cussed in this article.
Additional information: The Videos can be found in the
“Supplemental Materials” area of the online article
References
1. Bidwell JL, Pachner RW. Hemoptysis: diagnosis and manage-
ment. Am Fam Physician. 2005;72(7):1253-1260.
2. Shebani SO, Khan MD, Tofeig MA. A congenital fistula
between the descending aorta and the right pulmonary vein
in a neonate presenting with heart failure. Cardiol Young.
2007;17(5):563-564.
3. Van Raemdonck D, De Boeck K, Devlieger H, et al. Pulmo-
nary sequestration: a comparison between pediatric and adult
patients. Eur J Cardiothorac Surg. 2001;19(4):388-395.
4. Nicolette LA, Kosloske AM, Bartow SA, Murphy S. Intra-
lobar pulmonary sequestration: a clinical and pathological
spectrum. J Pediatr Surg. 1993;28(6):802-805.
journal.publications.chestnet.org
Downloaded From: http://journal.publications.chestnet.org/ by David Kinnison on 02/08/2013
5. Zumbro GL, Treasure RL, Seitter G, Strevey TE, Brott W,
Green DC. Pulmonary sequestration. A broad spectrum of
bronchopulmonary foregut abnormalities. Ann Thorac Surg.
1975;20(2):161-169.
6. Flye MW, Conley M, Silver D. Spectrum of pulmonary seques-
tration. Ann Thorac Surg. 1976;22(5):478-482.
7. Thilenius OG, Ruschhaupt DG, Replogle RL, Bharati S,
Herman T, Arcilla RA. Spectrum of pulmonary sequestration:
association with anomalous pulmonary venous drainage in
infants. Pediatr Cardiol. 1983;4(2):97-103.
8. Chabbert V, Doussau-Thuron S, Otal P, et al. Endovascular
treatment of aberrant systemic arterial supply to normal bas-
ilar segments of the right lower lobe: case report and review
of the literature. Cardiovasc Intervent Radiol. 2002;25(3):
212-215.
9. Ando K, Maehara T, Adachi H, et al. Intralobar pulmonary
sequestration supplied by an anomalous aneurysmal artery.
Ann Thorac Surg. 2012;93(1):319-322.
10. Currarino G, Willis K, Miller W. Congenital fistula between
an aberrant systemic artery and a pulmonary vein without
sequestration. A report of three cases. J Pediatr. 1975;87(4):
554-557.
CHEST / 143 / 2 / FEBRUARY 2013 553