Unilateral absence or extreme hypoplasia ofpulmonary veins · 150 predominantlyontheleft side.Onchestx-rayfilmsthe left lung now looked smaller than the right, and at times there

Br Heart J 1983; 49: 148-53

Unilateral absence or extreme hypoplasia of pulmonaryveins

H M KINGSTON, R G PATEL, G H WATSON

From the Royal Manchester Children's Hospital, Pendlebury, Nr Manchester

SUMMARY Three children with recurrent chest symptoms and signs caused by unilateral atresia orabsence of the pulmonary veins are described, with a discussion of the seven reported cases ofunilateral congenital pulmonary venous obstruction. The condition is rare but should enter into thedifferential diagnosis of recurrent unilateral pulmonary infection or oedema. Pulmonary arteriogra-phy is necessary and pulmonary artery wedge angiography is the most informative diagnosticprocedure.

Congenital obstruction of the pulmonary veins fre-quently occurs in patients with total anomalous pul-monary venous drainage,' but is otherwise a rare ano-maly. Various forms have been described, involvingone to four veins and ranging.from localised stenosesto atresia. Bilateral obstruction, unless minimal, leadsto pulmonary congestion and oedema and pulmonaryarterial hypertension, the clinical course dependingon the degree of obstruction. Unilateral obstruction,however, causes less severe symptoms and a clinicalpicture which may mislead.We report three cases of unilateral pulmonary ven-

ous obstruction, two of which also had congenital car-diac anomalies, and comment on the diagnostic fea-tures.

Case reports

CASE 1This boy was seen at the age of 14 months shortlyafter arrival in England from India. His symptomsbegan at the age of 3 months after which he had recur-rent bouts of cough with some wheezing, and alsoseveral severe haemoptyses, for which he received ablood transfusion on four occasions. Physical exami-nation disclosed a left leg slightly thinner than theright but no other asymmetry or dysmorphic features;there were a few rales in the left lung. Investigations,including a sweat test and immunoglobulin levels.were normal. A chest x-ray film showed a small leftlung, with more prominent vascularity on the right.An isotope scan showed normal ventilation and perfu-sion of the right lung; the left lung showed very little

Accepted for publication 21 October 1982

148

perfusion and some diminished ventilation.Cardiac catheterisation showed no shunts or

intracardiac abnormalities. Oxygen saturations of 68to 69% were found in the superior vena cava, rightheart, and right pulmonary artery; that in the leftpulmonary artery was not significantly different-70%. Pulmonary arterial pressures were normal (24/16 mmHg), though the left pulmonary arterial wedgepressure was higher than that in the right (14 and 5mmHg). The left atrium could not be entered. Onangiocardiography an injection into the main pulmo-nary artery showed a large right pulmonary artery; theleft pulmonary artery was relatively small but in abso-lute terms was only slightly less than the expected size(Fig. 1). The right lung drained normally by largeright pulmonary veins into the left atrium, but for-ward flow in the left lung was very slow, and oscilla-tory, and arterial branches remained opacified formany seconds after the venous phase on the right. Noleft pulmonary veins could be seen but there was asuggestion of small bronchial veins draining the leftlung (Fig. 2). Aortography was normal and showed noevidence of sequestration or enlarged bronchialarteries.

Bronchoscopy showed a hyperaemic left bronchialmucosa with increased secretions but the right sidewas normal.A left pneumonectomy showed that the left lung

was small, there were some prominent bronchialveins, and two tiny veins ran from the left lungtowards the left atrium. On examination of theexcised left lung no pulmonary veins could be found.Histological examination showed evidence of someold and recent haemorrhage in the lung parenchyma,and a very small area of cystic malformation in the left

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Unilateral absence or extreme hypoplasia ofpulmonary veins

Fig. 1 Case 1: pulmonary arteriogram. Right pulmonaryartery large, left slighty smaU with poorfiUing oflateralbranches.

Fig. 2 Case 1: laevphase following Fig. 1. Right pulmonaryveins weU seen; on the left side some continuing opacification oflower lobe pulmonary artery branches, and some small irregularvessels, but no pulmonary veins visible.

upper lobe, but there was no fibrosis. No pulmonaryveins could be seen, but some small veins were

present in the peribronchial positions; these veinsseemed normal in structure. Some contrast mediumwhich had been injected into the pulmonary arteryafter removal of the lung could be seen in dilatedperibronchial lymphatic vessels.The child recovered uneventfully and remained

well when last heard of at the age of 3 years.

CASE 2This boy presented at the age of 1 day with cyanosis.Investigations at that time showed transposition of thegreat arteries, and anomalous drainage of the rightupper lobe pulmonary vein into the upper part of theright superior vena cava. Pressures recordedincluded: right ventricle 70/2, aorta 70/40, and leftventricle 40/2 mmHg. Right ventricular and aortic

Fig. 3 Case 2: left ventricular angiogram at age 4 months. Allmedium enters pulmonary artery but most then passes throughright lung; left pulmonary artery branches poorlyfiled.angiograms excluded a ventricular septal defect; therewas a small persistent ductus which later closed spon-taneously. A left ventricular angiogram showed con-trast medium all passing through an unobstructedoutflow tract into unremarkable pulmonary arteries,but the venous return on the left side was obscured byrefilling of the left ventricle. The left lung, however,was a little more translucent than the right though ofnormal size.

After a balloon septostomy his progress was at firstsatisfactory, but because of increasing cyanosis he wasrecatheterised at the age of 4 months. After a furtherballoon septostomy the aortic oxygen saturation was62%; the pressures had not changed significantly fromthose recorded at the age of 1 day. A left ventricularangiogram (Fig. 3) showed most of the medium enter-ing the right pulmonary artery, and indeed very littlemedium passed into the left lung. The right sidedpulmonary veins could all be seen but not those on theleft, though the reopacified left ventricle obscured theview.At the age of 14 months a modified Mustard opera-

tion was performed, diverting the systemic venousreturn to the left ventricle and the pulmonary returnto the right ventricle. No left sided pulmonary veinscould be found. It was not found possible to channelblood from the anomalous right upper lobe pulmo-nary vein into the pulmonary venous atrium.Nevertheless he made good progress after the opera-tion for about a year but subsequently developed signsof inferior vena caval obstruction with hepatomegaly,and evidence of a right to left shunt with somecyanosis. He also had recurrent episodes of cough anddyspnoea with signs suggesting pulmonary oedema

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predominantly on the left side. On chest x-ray films theleft lung now looked smaller than the right, and attimes there was evidence of pulmonary oedema.

Catheterisation at the age of 3 years (Table 1)showed moderate pulmonary hypertension, with anincreased pulmonary flow resulting from the anomal-ous drainage of the right upper lobe pulmonary vein.There was stenosis of the inferior vena caval pathwayand also of the right lower lobe pulmonary vein path-way in its intracardiac part. Left pulmonary arteryangiography showed most contrast medium flowingbackwards to the right side; medium oscillated in theleft pulmonary artery branches and there was littleadvance. No pulmonary veins could be seen on theleft side though there was a suggestion of small bron-chial veins on the later frames (Fig. 4). There was noincrease in the systemic arterial supply to the leftlung.

Table 1 Case 2, cardiac catheterisaion data at age 3 years

Site Oxygen Pressuresaturation (%) (mmHg)

Innominate veins 64 12Low superior vena cava 80 10Left ventricle 79 55/4Left pulmonary artery 80 52/17Right pulmonary artery 77 50/18Right pulmonary artery wedge 16Right lower pulmonary vein 95 16Pulmonary venous pathway 94 0

(near tricuspid valve)Right ventricle 86 80/4Aorta 88 85/55

Fig. 4 Case 2: frane taken nine heart beats after injection.Left low lobe pulmonaty artery branches stll visible. No left

pulmonay vetins seen.

Kingston, Patel, Watson

Further surgery was undertaken, at which the ven-ous obstructions were relieved and the right upperlobe pulmonary venous blood was redirected into thepulmonary venous atrium, but unfortunately thechild did not regain consciousness, and died afterrenal failure. Necropsy showed a satisfactory anatom-ical reconstruction. No left sided pulmonary veinscould be found. The right lung weighed 520 g, the leftonly 175 g. Histological examination showed thechanges of pulmonary hypertension in the small pul-monary arterioles and areas of bronchopneumonia onboth sides. In addition the left lung showed interstitialfibrosis, some areas of infarction, and dilated lympha-tic vessels; there were some veins of normal structurebut only in peribronchial positions.

CASE 3This girl had cyanosis from shortly after birth and wasinvestigated elsewhere at the age of 4 days. On a chestx-ray film at this time the lungs were translucent butotherwise unremarkable. Investigations showed isol-ated pulmonary atresia, with a small right ventricle;the pulmonary arteries were not well seen. Aftertransfer here a right Waterston-Cooley procedure(anastomosis of ascending aorta to right pulmonaryartery) and a Brock procedure (enlargement of rightventricular outflow) were performed. A continuousmurmur from the anastomosis subsequently becameaudible and has remained since.

She has remained small in build and moderatelycyanosed but otherwise her progress was satisfactoryat first. From the age of 18 months she had recurrentepisodes of cough and dyspnoea with left sided chestcrepitations. Examination showed cyanosis at rest,some clubbing, and bilateral congenital ptosis. Chestx-ray film showed variable changes compatible withpulmonary oedema in the left lung, which by the ageof 3 years looked smaller than the right.Two dimensional echocardiography showed the

features of isolated pulmonary atresia; the right lowerlobe pulmonary vein was seen, but no left sided pul-monary vein could be found.At catheterisation at the age of 3 years (Table 2) the

catheter passed through the right ventricle into theleft pulmonary artery but not the right; and through acentral atrial septal defect into the left atrium, leftventricle, and right lower lobe pulmonary vein, butnot into any left pulmonary veins. There was a vari-able right ventricular and pulmonary systolic hyper-tension, with a high end-diastolic right ventricularpressure, and similar tracings in the right ventricleand pulmonary artery. The left pulmonary arterialwedge pressure was very high. There was a rise inoxygen saturation to nearly the aortic level in the rightventricle and left pulmonary artery, and a right to leftatrial shunt.



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Table 2 Case 3, cardiac catheteisation data at age 3 years

Site Oxygn Pressresaturat (%) (mmg)

Superior vena cava 62 -Right atrium 62 7Right ventricle 75 32/8 to 55/10Left pulmonary artery 76 34/8 to 55/10Left pulmonary artery (wedge) 75 50Right lower pulmonary vein 95 9Left ventricle 78 96/2

A left atrial angiogram showed no reflux ofmediuminto any of the pulmonary veins and there was wash-out from the right lung though none from the left. Aleft ventricular angiogram excluded a ventricular sep-tal defect, all the contrast medium passing into a largeascending aorta; the Waterston anastomosis suppliedonly the right pulmonary artery. A few small bron-chial arteries ran to the left lung. A right ventricularangiogram showed a small poorly functioning cavity;medium advanced into the main pulmonary artery butdid not advance into the lungs and flowed back freelyinto the right ventricle in diastole. An injection intothe left pulmonary artery showed some advance ofcontrast medium into small arterial branches duringsystole (Fig. 5), but medium then oscillated back indiastole into the main pulmonary artery and right ven-tricle. As may happen through distortion by the oper-ation the right pulmonary artery was completely obs-tructed proximal to the Waterston anastomosis. Con-trast medium injected through an end-hole catheter inthe left pulmonary artery wedge position could be

Fig. 5 Case 3: age 3 years. Left pulonary artoam,systole. Opacqfication ofsmal artrie, ofmain pulmonamyarry, and ofpromal part of right pulmonay arty whichtapers to occusin.

=,,,,.,.... _ g . .

Fig. 6 Case 3: injection drough end-hok catheter uwdged inleft lower lobe pulmonary artey. Some ha-y capitaiyfjilling,and opaccation ofsmaU disualpulmo y arteris and ofirregular vessels running proximally.

seen passing from the small distal pulmonary arterybranches into bronchial arteries (Fig. 6) and some of itthen flowed back into the pulmonary arteries moreproximally (Fig. 7). No pulmonary veins opacifed;later frames (Fig. 8) showed slow filling of whatappeared to be small bronchial veins in subpleuralpositions and also along the left main bronchus to thetrachea and even over to the right side.The little girl remains in much the same health;

further operation seemed inadvisable, so no anatomi-cal data are available.

Fig. 7 Case 3: laterframe following Fig. 6. Furtheropacfication ofdistal pulmonay artial tree and ofsmaUirregular vessels more proximaly, from some ofwhich contrastmedium has enrd the main stem ofthe left pulmonary artery.No pulmonary veins visible.

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Fig. 8 Case 3: laterframefolowing Fig. 7. Hazy capillaryblush, and opac#ication ofbronchial vwins rnning along leftmain bronchus and ofsubpkural veins, but no pulmonary veinsvisible.

Discussion

Each of the three children described had left sidedpulmonary venous obstruction. In one the pulmonaryveins were absent, in one they were extremely small atoperation and could not be found after the lung wasremoved, and in one case there was no anatomicalevidence but good investigatory evidence that theveins were either atretic, extremely small, or absent.A review of the published reports has disclosed only

a few cases of congenital unilateral pulmonary venousobstruction and we have listed these in Table 3,

Kingston, Patel, Watson

including those with stenosis as well as those withatresia, as some of the features are similar. We haveexcluded that of Samanek et a19 in which the rightpulmonary veins were atretic, because congenital obs-truction of the left pulmonary veins, which drainedinto the portal vein, led to severe pulmonary hyper-tension and a different clinical picture. We havefound no case reports of unilateral absence of pulmo-nary veins exemplified by our case 2.

In general, patients have presented with unilateralcongestive symptoms and signs or haemoptyses, withx-ray changes which have sometimes suggested infec-tion and at times pulmonary venous hypertension.Gradual relative diminution in size of the affectedlung occurred in two of our cases but has not beencommented on previously, though the affected lungsappear small in the cases of Bouchard et al.6 andNasrallah et al.8Two dimensional echocardiography has not been

used in the reported cases. In our case 3 we were ableto show a large right lower lobe pulmonary vein andcould not detect any left pulmonary vein. We are notyet certain, however, that failure to picture a pulmo-nary vein would be a reliable indication of its absenceor hypoplasia, and of course demonstration of a nor-mal pulmonary vein would not exclude an intrapul-monary obstruction.

At cardiac catheterisation mild pulmonaryhypertension has been present in some casesuncomplicated by major abnormalities, but has notcorrelated closely with symptoms. Bronchial arterieswhich commonly communicate with the smallpulmonary artery branches are presumably

Table 3 Cases ofunlateral pulmonary venous obstruction with unobstructed contralateral veins

Author Age Sex Side Nature of Other Synptoms caused Outcome Pulmonary areryobstructio anomalies by obstrution pressure (mmHg)

Free Wedge

R L

Emslie-Smith 14 y F R Diaphragms, P ductus Congestion Died Systemic- -et al.' 1 atretic

Andrews3 3 y F L Atresia Tricuspid None Diedatresia

Nakibetal.4 2 M L Localised Aortic None Died - - -stenosis coarct

Snellen and 13 y F R Membranous VSD Congestion ? Raised -Bruinss obstruction

Bouchard 4 y F R Diaphragm None Haemoptyses Venoplasty, 40/20 30 12et a16 cured

Laboux et al.' 7 y F R ? None Haemoptyses Died 35/10 20 10

Nasrallah 16 m M R Atresia None Congestion Pneumonectomy, 28/14 20 12et al.3 cured

Present case 1 15 m M L Extreme None Haemotypes, Pneumonectomy, 20/12 5 14hypoplasia congestion cured

Present case 2 4 y M L Absence Transp of Congestion Died 50/18 16 -gt arteries

Present case 3 4 y F L ? Pulmonary Congestion Unchanged 46/12 - 50atresia



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responsible for the raised pulmonary artery wedgepressure and the high pulmonary artery oxygensaturation which may be characteristically found onthe affected side.

Pulmonary arteriography on the affected side showsvery little forward flow, and contrast medium mayoscillate or flow backwards (68 and our three cases).Medium may not enter the affected lung at all, or mayremain in it for many seconds. Pulmonary veins arenot seen if they are atretic though they may be ifmerely stenosed.5 It may be very difficult, however,to see the pulmonary veins even if present and moreinformation has been obtained from wedge angiogra-phy. Pulmonary artery wedge angiography may showfilling of small bronchial arteries which may rejoinand opacify the pulmonary arterial tree more proxi-mally (our case 3). Subsequently a bronchial venousplexus may be seen, but no true pulmonary veins (8and our case 3) unless they are merely stenosed as incase described by Bouchard et al.6 and also as in a caseof acquired stenosis.'°

Histological changes have included oedema, con-gestion, dilatation of lymphatic vessels, patchy infarc-tion, probably responsible for some of the radiologicalshadows,8 and fibrosis3 (and our case 2). Intrapulmo-nary veins were sometimes normal (our cases 1 and 2)but sometimes fibrosed8 and hypertensive changeswere present in the intrapulmonary arteries in somecases.3 8Treatment would in general be pneumonectomy

should symptoms be severe enough, though surgicalrepair of localised stenosis of veins has been per-formed successfully in one case.6

Conclusion

Congenital unilateral pulmonary venous obstruction,though rare, should be considered in the differentialdiagnosis of recurrent bouts of illness suggestingunilateral pneumonia or oedema. Pulmonary arterialwedge angiography is the most helpful single diagnos-tic measure in determining the nature of the venousobstruction.

We are grateful to Mr John Dark for the surgicalfindings and to Dr K V Lodge and Dr M Lendon forthe pathological data.

References

1 Lucas RV, Schmidt RE. In: Moss AJ, Adams FH,Emmanouilides GC, eds. Heart disease in infants, childrenand adolescents. 2nd ed. Baltimore: Williams and Wil-kins, 1978: 458.

2 Emslie-Smith D, Hill IGW, Lowe KG. Unilateral mem-brafrous pulmonary venous occlusion, pulmonary hyper-tension, and patent ductus arteriosus. Br Heart J 1955;17: 79-84.

3 Andrews EC Jr. Five cases of an undescribed form ofpulmonary interstitial fibrosis caused by obstruction ofthe pulmonary veins. BullJohns Hopkins Hosp 1957; 100:28-42.

4 Nakib A, Moller JH, Kanjuh VI, Edwards JE.Anomalies of the pulmonary veins. Am J Cardiol 1967;20: 77-90.

5 Snellen HA, Bruins C. In: Watson H, ed. Paediatric car-diology. London: Lloyd-Luke, 1968: 428.

6 Bouchard F, Binet JP, Chetochine F, Conso JF, Lang-lois J, Pottemain M. Un cas de st6nose des veines pul-monaires diagnostique et traite avec succes. Arch MalCoeur 1972: 65: 1143-53.

7 Laboux L, Michaud JL, Cornet E. Atr6sie unilateraledes veines pulmonaires. Signes cliniques, h6modynami-ques et angiographiques a propos d'une observation.Arch Mal Coeur 1972; 65: 1155-8.

8 Nasrallah AT, Mullins CE, Singer D, Harrison G,Mcnamara DG. Unilateral pulmonary vein atresia: diag-nosis and treatment. Am J Cardiol 1957; 36: 969-73.

9 gamanek M Tdma S, Benesova D, Povysilova V,Pralzsky F, tapova E. Atresia of right pulmonary veinsand anomalous left pulmonary venous drainage into por-tal circulation. Thorax 1974; 29: 446-50.

10 Bini RM, Bargeron LM Jr. Visualisation of pulmonaryvein obstruction by pulmonary artery wedge injection.Pediatr Cardiol 1982; 2: 161-2.

Requests for reprints to Dr G H Watson, Royal Man-chester Children's Hospital, Pendlebury, Near Man-chester M27 IHA.

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Unilateral absence or extreme hypoplasia ofpulmonary veins · 150 predominantlyontheleft side.Onchestx-rayfilmsthe left lung now looked smaller than the right, and at times there