대 한 방 사 선 의 학 회 지 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 [J，ηiversity 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 p비monary vein atresia . We reviewed seven cases of pulmonary vein obstruction , fi ve of which ac

companied other cardiac anomalies. Righ t p비monary 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

PuJmonary Veins , stenosis

Pulmonary veins, abnormalities

Children , cardiovascular system

INTRODUCTION

a re caused by long-standing pulmonary conges­

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

* 이 연구는 1991년도 서울대학교병원 특진연구비 지원에 의한 결과임(과제변호 : 02-91-171)

**서울대학교 의과대학 소아과학교실

** Department 01 Pediatrics, Seoul National Universiψ College 01 Mediciηe

*** 인제대학교 의과대학 병리학교실 *** Departmeηt 01 Pathology, 1ηje University College 01 Medicine

**** 서울대학교 의과대학 병리학교실 **** Department 01 Pathology, Seoul Natioηal University College 01 Mediciηe

이 논문은 1992년 8월 12일 접수하여 1992년 10월 5일에 채택되었음.

Received August 12, Accepted October 5, 1992

뼈

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 .

MATERIALS AND METHODS

Authors experienced seven cases ofpulmonary

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.

Chest radiographs of seven children 씨le re

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

5/F

2 lIF

3 9/M

4 5/F

5 0+ 1/F

6 6/M

7 131M

Symptoms & Signs

recurrent hemoptysis

and pneumonia

recurrent hemoptysis

and pneumonia

recurrent hemoptys is and pneumonia

cyanosls

recurrent hemoptysis

and pneumonia

dys pnea

cardiac murmur

dyspnea

PDA

cyanosls growth retardation

Accompanied Anom alies

tracheaJ bronchus

(right upper lobe)

partiaJ cor triatriatu m ,

PDA , biJateral SVC , multipJe hemangioma in

skin , di gital anomaly

left SVC pure red cell apJas ia

( - )

multiple VSD and ASD

PDA , pulmonary sequestration vertebr‘ aJ anomaJy

secundum ASD

PDA

(Eisenmenger complex)

secundum ASD

ear , eye anomaly

undescended testicle

In voJvcd Veins

right

r ight

ri ght left (foca l)

right

right

ri ght u ppel

(fo caJ)

ri ght uppel

(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

The clinical features in seven cases of

pulmona ry vein obstruction is summarized in

table 1. Right pulmonary veins were obstructed

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

야ω mω %

98

w” 겐

mw LU

객υ

. m m u t

쩍

m f o

@ d

n ·m 뼈 없

w

Journal of Korean Radiological Society 1993 ; 29 ( 1) 165~ 173

tr띠‘

C hest rad비iographs showed decreased lung

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

c

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 (ar­rowheads) 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

ary arterial hypertension in six cases (not

available in case 5). Pulmonary arterial wedge

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

arteries of the involved side without peripheral

filling (“dead tree" appearance) (6 cases) , reflux

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).

- 169-

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). Secun­dum ASD(arrowheads)is also demonstrated.

cardiac anomalies (Table 1)

Pulmonary perfusion scans in 3 cases (case

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 p비monaryvem

obstructon.

DISCUSSION

Anomalies which can cause pulmonary

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 (foc띠 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" p비monary 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 i바ancy (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 、이ledge

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 circula­tio 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 이 , 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 per­fusion 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 tl띠 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

In summary , we reviewed seven cases of

pulmonary vein obstruction in which right lungs

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

angiography may be still essential for the

diagnosis of pulmonary vein obstuction , MR irn­

aging is thought to be a useful modality for the

diagnosis of this disease .

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선천성 폐정맥 폐쇄의 방사선학적 고찰

서울대학교 의과대학 방사선과학교실， 소아과학교실* 병리학교실*** 인제대학교 의과대학 병리학교실**

김우선 • 연경모 • 킴인원 • 윤용수* • 서연림** • 지제근***

폐정맥 폐쇄는 만성적 폐울혈과 폐동맥 고혈압을 초래할 수 있으며， 폐정맥 환류이상이 없는 선천성 폐정맥 폐쇄

에는 일측성 폐정맥 폐쇄증 또는 폐정맥 협착증이 포함된다. 저자들이 경험한 폐정맥 폐쇄 7례는 전례에서 우측 폐

정맥에 발생하였으며 l례는 양측성 이었다. 폐쇄 정도는 5례에서 완전폐쇄. 3례(양측성 1례 포함)에서는 부분폐쇄였

다. 5례는 다른 선천성 심장질환을 통반했다. 4례는 유아기부터의 재발성의 폐렴과 각혈이 주증상이었고 이들의 평

균 나이는 5세였다. 나머지 3례는 동반된 심장 질환으로 인한 증상이 주로 나타났다. 단순흉부촬영상 폐정맥 완전폐

쇄시는 동측의 일측성 폐용적 감소， 폐부종， 망상형 간질성병변， 늑막변화가 관찰되었다. 부분폐쇄의 경우는 정맥폐

쇄 부위 폐야의 폐정맥 확장이 주소견이었다. 폐관류 스캔 (n=3)상 폐정맥이 완전 폐쇄된 폐야는 완전관류 결손으로

나타났다. 심도자술(n=6)상 폐동맥 고혈압 소견이 전례에서 있었으며 부분폐쇄의 경우는 폐쇄원위부와 화심방 사이

에 혈압경사가 관찰되었다. 완전폐쇄의 경우 폐동맥 조영술상 병변측 폐동맥의 직경 감소와 원위부로 부터의 조영제

의 역류가 있었고， 말단 폐동맥이 조영되지 않아 “ dead tree" 모양이었으며. levo-phase에는 동측의 폐정맥이 전

혀 관찰되지 않았다. 대동맥 조영술(n=2)상 전신동맥 으로부터 폐동맥으로의 측부혈류가 관찰되었다. 부분폐쇄의

경우 이에 통반된 심방중격결손을 통한 우상 폐정맥조영술(n=2) 로 폐정맥협착이 관찰되었다. 자기공명영상(n=2)

소견상 2례의 폐정맥-좌심방 접합부위의 완전폐쇄와 1례의 막성 부분폐쇄가 관찰되었다(양측성 폐쇄 1례 포함) . 2

례에서수술과조직학적검사가시행되었고1 다른 2례는 심장수술중 폐정맥 폐쇄가 확인되었다. 폐정맥폐쇄 (특히 완전

폐쇄)는 특이한임상적， 방사선학적소견을보여증상과 단순흉부촬영 소견만으로도 이 질환의 가능성을 생각할 수 있

을 것으로 생각된다. 또한 자기공명영상은 폐정맥폐쇄의 진단에 매우 유용할 것으로 예상된다.

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