Radiological Evaluation of Congenital Pulmonary Vein Obstruction

1993 ; 29 ( 1) : 165~ 173
Journal of Korean Radiological Society, January, 1993
Radiological Evaluation of Congenital Pulmonary Vein Obstruction *
Woo Sun Kim , M.D. , Kyung Mo Yeon , M.D. , In-One Kim , M.D. ,
Yong 800 Yoon , M.D. **, Yeon-Lim 8uh, M.D. ***, Je G. Chi , M.D. ****
Departmeηt 01 Radiology, Seoμl National [Jηiversity CoUege 01 M edicine
- Abstract-
Congenital obstruction of pulmonary vein without anomalous drainage can cause long-standing pulmonary
congest ion and pulmonary arterial hypertension , and it may include stenosis of individual pulmonary veins
or total pmonary vein atresia . We reviewed seven cases of pulmonary vein obstruction , fi ve of which ac
companied other cardiac anomalies. Righ t pmonary veins were involved in all seven cases including one
bilateral case . Pulmonary veins were occluded totally in five and partially in three lungs .
Pulmonary catheterization and angiography were done for diagnosis . Chest radiographs of total occlu
sion cases showed decreased lung volume , features ofpulmonary edema , interstitiallesions , and pleural thicken
ing, which were quite specific , whereas pulmonry venous dilatation was dominant findings in partial obstruction
cases. Pulmonary perfusion scans (n = 3) showed total perfusion defects in the cases oftotal occlusion ofveins.
MR imaging (n = 2) demonstrated total occlusion of pulmonary veins in the venoatrial junction in two , and
membranous focal obstruction in one lung. Two patients had pneumonectomy and histological confirma­
tion . AJthough catheterization and angiography are essential for the diagnosis , MR imaging is thought to
be useful for the diagnosis of pulmonary ve in obstruction
Index Words: Pulmonary veins
tion (1-13). Although catheterization and
angiography are essential for the diagnosis of
pulmonary vein obstruction , they do not always
show obstructed pulmonary veins itself, especial­
ly in cases with total oclusion (9-13). W e per­
formed MR imaging in two recent cases to
Pulmonary vein obstruction can be congenital
o r acquired (1-22). Congenital obstruction of
pulmona ry vein is presented as chronic cough , h emoptysis, and recurrent pneumonias , which
* 1991 ( : 02-91-171)
**
** Department 01 Pediatrics, Seoul National Universiψ College 01 Mediciηe
*** *** Departmeηt 01 Pathology, 1ηje University College 01 Medicine
**** **** Department 01 Pathology, Seoul Natioηal University College 01 Mediciηe
1992 8 12 1992 10 5 .
Received August 12, Accepted October 5, 1992

Journal of Korean Radiological Society 1993; 29 (1) 165~ 173
visualize obstructed pulmonary veins. W e
reviewed clinical and radiological features of
seven cases with pulmonary vein obstruction a nd
discussed about the role of MR imaging in this
disease .
vein obstruction without anomalous drainage in
Seoul Nationa l University Children's H ospita l
frm 1982 to 199 1. Among seven children , four
(case 1-4) h ad mainly pulmonary symptoms of
long. standing cough , rccurrent pneumon ia , a nd
h emoptys is from their infan cy . Their m can agc
was 5 years. The earli est clinical presenta tion was
h emoptysis at seven days of age in case 3. In thc
other three children (case 5-7) cardiac sym ptom s
due to associated cardiac anomalies we rc
predominan t.
reviewed. In two cases (case 2 and 4) , follow -up
chest radiographs for three years were available
Cardiac catheterization and cine-angiography in­
cluding pulmonary a n giography were perform­
ed in all cases . Aortograph ies were also done in
two cases (case 1 a nd 5). Radioi sotope pulmo n-
Table 1. Summary o[ Clinical Features in Seven Cases o[ Pulmonary Vein Obstruction
Patient Age/Sex
cyanosls
skin , di gital anomaly
( - )
secundum ASD
right
right
(focal)
* PA: pulmonary artery , PV: pulmonary vein , WP: wedge pressure , SVC: superior ve na cava , ASD ‘ atriaJ septal defect. VSD: vetricular septal defect , PDA: patent ductus arteriosus
- 166 -
Woo Sun Kim , et al : Radiological Eva luation of Congenital Pulmonary Ve in Obstruction
ary perfusion scans using technetium-99m
macroaggregate albumin were done in three
cases. 1n recent two cases (case 3 and 4) , MR
imagings were performed with a 2 .OT supercon­
ductive system (Spectro-20000; Goldstar, Seoul, Korea) . Axial and coronal T 1 weighted imag­
ings with cardiac gating were obtained. 1n case
3 , chest CT (10mm thickness , lOmm interval)
was done. Two cases (case 1 and 4) had
pneumonectomies and histologic studies. 1n two
cases (case 2 and 7) , pulmonary vein obstruc­
tion were observed during cardiac surgeries.
RESULTS
in all seven cases , total occlusion in fi ve cases
(case 1-5) and focal partial obstruction at the
right superior venoatrial junction in two cases
with large atrial septal defects (ASD) (case 6 and
7) . Left pulmonary veins were also partially in ­
volved in case 3. 1n case 2, left pulmonary venous
drainage was also obstructed partially by a
diaphragm in the left artium (partia l cor
Pulmonary Arterial O 2 Saturation Operations Pressure(mm H g) ( % )
main PA 32/8 79 ri ght pneumonectomv left PA 30/12 77 right PA 40/8 main PA 68/28(48) 64 cord-like right PV left P A 68/28(44) 64 PDA Iigat ion left PA WP (16) resection of abe rrent muscl e
in left atrium main P A 50/28 82 left PA 50/2.1 81 left PV 16 96 left atri um 11 main PA 25/ 12 78 right pneumonectom y right PA 27/18 82 right PA VP 29/2 1 88 left PA WP 17/8 Not Availabe
main PA 130/75 left P A 120/80
83 83
main PA 30/15 left PA 25/ 15 right upper PV 26/12 left u pper PV 14/7
left atrium 5
ω m ω %
Journal of Korean Radiological Society 1993 ; 29 ( 1) 165~ 173
tr‘
volume (6 cases) , small hila (5 cases) , diffues hazy
densities (4 cases) , reticulonodular den sities (4
case) , multiple K erley ’ s lines (4 cases) , cystic le­
sions (air cysts) (4 cases) , and pleural changes
(5 cases) in the involved side (Fig. la) . Car-
a b
Fig. 1. Patient 4
diomegaly was noted in three cases. In case 7, dila ted pulmon ary vein in the right uppe r lung
fi eld was the only finding . Pulmona ry venou s
dialtation was also n oted in the left lung of case
3 . In two cases , follow up radiographs for three
years showed progressive volume decrease and
d evelopment of reticulonodular densities
d
a. C hest radiograph shows decreased right lung volume with hern iation of left lung(arrows) . The right lung shows reticulonodular densities , multiple Kerley ’ s lines , cystic lesions, and pleural thickening. b . Right pulmonary arteriogram shows small sized arteries without peripheral filling (“dead tree" appearance) (arrows) , ref1 ux of contrast media to the left pulmonary artery , and systemic-pulmonary arterial fistula (ar­ rowheads) c . Levo-phase of pulmonary arteriogram (b) shows the left pulmonary veins and the left atrium without visuali zation of the right pulmonary vein. See the right border of the left atrium (arrowsheads) d . Pulmonary perfusion scan shows a complete perfusion defect in the right lung.
- 168-
Woo Sun Kim , et al : Radiological Evaluation of Congenital Pulmonary Vein Obstruction
le
e. Axial MR imaging on the level 0 1' the left atrium shows cord-like occlusion of the right superior pulmonary vein at its venoatrial junction and extrapulmonic segment (arrowheads). Decreased right lung volume and pleural thickening are also noted . f. CT with lung-window setting shows irregular reticular interstital changes , cystic lesions , and thickening of interlobular septa in the right lung.
Pulmonary catheterization revealed pulmon­
pressure of the involved lung was available in
case 4 and was elevated. In cases with focal
obstruction , pressure gradients were detected
between the left atrium and obstructed
pulmonary veins (case 7 and the left lung of case
3) (Table 1). Pulmonary arteriography
demonstrated small peripheral pulmonary
of contrast media from ipsilateral peripheral
pulmonary arteries (5 cases) , delayed pulmonary
transit time (5 cases) , and systemic - pulmonary
arterial collaterals (2 cases) (Fig. 1 b) . In levo­
phase of pulmonary angiography , involved
pulmonary veins were not visualized in five cases
(Fig. 1 c). Left pulmonary veins of case 3 show­
ed engorgemerit and delayed drainage to the left
atrium. In case 6 and 7, the right upper
pulmonary venography through ASD demon­
strated focal stenoses in the superior pulmonary
veins (Fig. 3). Aortography in case 1 and 5 show­
ed multiple systemic collaterals and retrograde
opacification of right pulmonary arteries from
the ir peripheries. Five cases accompanied other
Fig. 2. Coronal MR imaging of Patient 3 with bilateral pulmonary vein obstruction shows septum like structure in the left venoatrial junction (arrow) and engorgement of the left pulmonary vein. No evidence of draining veins in the right border of the left atrium(open arrows).
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Journal of Korean Radiological Society 1993; 29 (1) 165~ 173
Fig. 3. Right upper pulmonary venogram (four chamber view) ofPatient 6 shows focal narrowing in the right superior pulmonary vein(arrows). Secun­ dum ASD(arrowheads)is also demonstrated.
cardiac anomalies (Table 1)
1,3 , and 4) showed total perfusion defects in the
involved lungs (Fig. 1d) . Perfusion in the left
lun g, however , was preserved in the case o[ par­
tial obstruction of the left pulmonary vein (case
3) MR imaging showed total occlusion o[ right
pulmonary veins in their venoatrial junctions and
extra pulmonary segements (case 3 and 4) and
cord- Iike intrapulmonary pulmonary veins (case
4) . In case 3, membranous obstruction at the left
venoatrial junction (Fig. 2) and left -sided
superior vena cava was also demonstrated . MR imaging also showed small right pulmonary
arteries and pleural thickening in both cases. CT
with lung window setting in case 4 demonstrated
decreased right lung volume with diffus haziness , reticular in terstitial densities , multiple small air
cysts, and thickening of interlobular septa, ma­
jor fissure , and the pleura (Fig . 1f). Pulmonary vein obstructions were confirm­
ed by pneumonectomies and histological ex­
aminations in case 1 and case 4. In case 2 , a
cord-like occlusion of right pulmonary veins were
observed during the cardiac surgery. In case 7, a redundant septum obstructing the orfice of the
right upper pulmonary vein was noted and it was
excised during the operation. Each of obstructed
pulmonary vein s had a normal site of connec­
tion with the left atrium in all four operated cases.
Gross pathological examinations in case 1 and
case 4 showed consolidated lungs with occluded
cord-Iike pulmonary ve ins, cystic parenchymal
changes , and pleural thickening and adhesion , On histological examination , irregular fibrous
intimal proliferation , m edial hypertrophy , and
organized thrombi were observed in pulmonary
veins , and these changes were more pronounc­
ed in larger veins. These change were not observ­
ed in pulmonary venules. I-l istological features
were compatible with congenital pmonaryvem
obstructon.
DISCUSSION
arterial hypertension b y obstruction of
pulmonary venous drainage may include con­
genital anomalous pulmonary venous connec­
tions , cor triatriatum , mitral stenosis or atresia , and pulmonary vein obstuction (1 ,2). Pulmonary
vein obstuction without anomalous return is very rare and may include ste nosis 0 1' individual
pulmonary veins (1-12) and atresia 0 1' pulmonary
vein (1, 2,13). Pulmonary venous obstruction may be acqu ired 1'orm pulmonary veno-occlusive
disease or 1'rom compli cations of some diseases , such as constrictive pericarditis , mediastinitis, pulmonary venous phlebitis (as in tuberculosis) , and invasion by malignant tumor (2 ,5 ,14-23)
Individual pulmonary vein stenosis and 'atresia
of pulmonary vein are thought to be caused by
anomalous incorporation of the common pul­
monary veins into the left atrium (1-3). They
may occur in the same patient and one or more
ve ins may be involved (1-5 , 10-13). They may
be present in isolation or in association with other
congenital cardiac anomalies (50 % ) (1,2,1 0-13)
- 170 -
Woo Sun Kim. et al : Radiological Evaluation of Congenital Pulmonary Vein Obstruction
T he obstruction usually occurs at the venoatrial
junction , but it O1ay extend for01 this point to
the periphery (1 ,2,5,10). Obstruction in the for01
of a diaphrag01 (foc stenosis) or occlusion of
varing length O1 ay be present aloneor in coo1-
bination (2 ,5,10). Histologically fibrous inti01al
thickening is present and the ve in O1ay exist as
a fibrous cord (2 ,3 ,5 ,9-10). Thro01bus for01 a­
tion in the stenotic veins secondary to stasis of
blood also can be observed (4 ,13). These changes
are discovered O1ainly in large veins with less in­
volvement or sparing of small vein s or venules
Interst itial les ions , air-cys t for01ation , fíbrosis , and dilatation 0 1" pmonary lY01pha tics and
bronchial ve in can be noted (3-5 ,11 ,13). The
develop01ent of collateral circulation through the
bronchial vesselleads to early and .severe he01op­
tysis (4 ,11 ,13). The O1ost co0101on sY01pto01s of
congenita l pulO1onary vein obstruction are recur­
rent respiratory trac t infections and he010ptysis
for01 the earl y iancy (4 ,, 8 ,9 ,11 ,13) Radiological features of congenital pulO1onary
vein atres ia and significant stenosis are similar
to each other (11-13). D ecreased lung volu01e, pulO1ona ry ede01a , reticula r densiti es , O1 ultiple
air cysts , O1ultiple Kerley ’s B lines , accentuation
f fi ssures , and pleural changes , all of which are
discovered in the in volved lung , are unique
radiographic findings (Fig. 1a) (5 ,11-13). These fïndin gs represe nt chron ic pulO1onary venous
obstuction and fibrotic changes of the lun g. In
cases with on ly pa rtial obstruct ion (case 6,7, and
the left lung of case 3) , these findings were not
shown , and pulO1onary venous congestion or
engorge01ent was the only fínding. PulO1onary
arte r‘ ial catheter ization reveals pulO1 ona ry
arter‘ ial hypertension and elevated arterial ledge
pressu re of the in volved lung (5 ,11). Pul O1onary
angiogra01 shows s01all ipsilateral pulO1onary
artery wi thout peripheral filling producting the
dead tree appearance (Fig. 1 b) , delayed circula­ tio l1 , and diversion of blood fro01 the affected
pulO1on ar y a rtery (2 ,11 ,12). 111 levo- phase of
pulO1onary angiography, visualization of involv­
ed pulO1o l1 ary ve il1 s is ab5ent or delayed (Fig
1 c) (2 ,11-13). Although visualization o ffo call y
obstructing lesion is possible by selective
pulO1onary venography (Fig. 3) (2 ,12) or by
pulO1onary artery wedge angiography (2 ,1 , it is not alway possible , especially in cases with ex­
tensive occlusion to the perphery (Fig. 1c)
(9 ,11 ,13). Syste01ic-pulO1onary collaterals can be
noted in aortography (3 ,4,11). Radioisotope per­ fusion scans show abse nt or di01inished perfu­
sion of affected lobes (Fig. 1d) (11 ,13)
PulO1onary veno-occlusive disease is an ac­
quired disease without difinitely known etiology
(2 ,14-21). The s01all veins or venules (so01eti01es
also the major veins) are predo01inantly narrow­
ed or occluded by fibrous tissue on the basis of
organized thro01bi (14-22). It usually involves
the lung bilaterally (14-17 , 20-22). but occa­
sionally the involve01ent of O1ajor veins O1ay be
li01ited to one lung (18-19). Cardiac ano01alies
are absent and it usually affects older children
or adults (1 4-15 , 17-20). Radiographic findings
include card io01egaly , pulO1 onary ede01a , K erley ’ s lines and interlobar effusion and/or subpleural ede01a (14-15 , 23). Catheterization
reveals pulO1onary arterial hypertension , nor01al
or only slightly elevated pulO1onary venous
pressure , but pulO1onary wedge pressure is paradoxically nor01al (14-15 ,17 ,21). PulO1onary
angiography reveals dilated pulO1onary arte ries , prolonged ci rculation ti01e , and nor01al
pulmonary vemLung perfusion scan is usually normal in
pulO1onary veno-occlusive disease (14 ,15)
In two cases (case 1 and 4) of tl study who
had operations , history of recurrent infections and he010ptysis fro01 the infancy , unilateral in­
volve01ent , decreased lung volu01e with fibrotic
changes , cord-like O1ajor pulmonary veins , unilateral total perfusion defects in perfusion
scan , elevated pulO1onary wedge pressure of the
involved side (case 4) , and involvem ent of large
veins with less changes in small veins and spar­
ing of venules on histological exa01 ination sup­
ported the congenital nature of pulmonary ve in
obstruction. Other cases in this study also had
m
Journal of Korean Radiological Society 1993; 29 (1 ) 165~ 173
symptoms from the infancy and accompanied
other anomalies (especially cardiac) , which sug­
gested the congenital nature of lesions.
MR imaging in the evaluation of pulmonary
vein obstuction has been reported recently , but
mainly in cases with anomalous pulmonary
venous return (24) . Masui et al. (25) reported
that MR imaging can demonstrate anatomic ab­
normality in pulmonary vein stenosis and cor
triatriatum. Two MR studies in our study show­
ed total occlusion of pulmonary veins at
venoatrial junctions, cord-like veins in their ex­
trapulmonary segments (Fig. l e) , and small
pulmonary artery of the involved lung. Mem­
branous structure at the venoatrial junction was
demonstrated in the case with focal obstruction
(Fig. 2). In cases with totally occluded pulmonary
veins , MR imaging could visualize the state of
veins which angiography could not show (Fig
le). MR imaging could also reveal other cardiac
anomalies which are often accompanied by this
disease
were involved predorninantly. Findings on chest
radiographs which included decreased lung
volume and pulmonary edema with reticular and
cystic changes were very unique and they could
strongly suggest the possibility of pulrnonary vein
occlusion. Although catheterization and
aging is thought to be a useful modality for the
diagnosis of this disease .
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