auniquepulmonary venous abnormality: with · circuitous route from the periphery, before uniting...

Thorax (1976), 31, 190.

A unique pulmonary venous abnormality:with associated pulmonary arterial anomaly and arteriovenous malformation

MICHAEL HONEY, MICHAEL C. PEARSON,and J. N. PATTINSON

Departments of Cardiology and Radiology, Brompton Hospital, London SW3,and London Chest Hospital, London E2

Honey, M., Pearson, M. C., and Pattinson, J. N. (1976). Thorax, 31, 190-196. A uniquepulmonary venous abnormality: with associated pulmonary arterial anomaly and arterio-venous malformation. A man aged 29 was first seen at the age of 11, when he was foundto have a congenital anomaly of the pulmonary veins which follow an abnormal course

to the hilum. On the left side, two large veins descend peripherally before turningmedially towards the left atrium; on the right, several smaller veins follow a similarcircuitous route from the periphery, before uniting into two main veins which enter theleft atrium. The proximal pulmonary arteries are normal in their pattern of branchingand distribution but are dilated and tortuous and taper abruptly: the left lower lobeartery is abnormally large almost down to the diaphragm. There is also a systemicarterio/pulmonary venous fistula. There is no haemodynamic disturbance, but gastransfer is slightly impaired, and there is slight arterial desaturation.

This congenital anomaly is unique. Reported cases described as having pulmonaryvenous varices are a heterogeneous group, but one patient described as such showsresemblances to our case.

The patient reported here has a hitherto undes-cribed pulmonary venous abnormality, in whichall the major pulmonary veins follow a very ab-normal intrapulmonary course but drain normallyinto the left atrium. This was associated withabnormal pulmonary arteries and a systemicarterio/pulmonary venous malformation in theright lung, arising from the descending aorta.

CASE REPORT

The patient, now 29 years old, was first seen as aboy of 11 in 1956 after a pulmonary vascular ab-normality had been recognized at the local chestclinic following mass radiography at school. Hewas symptomless. A continuous murmur washeard below the right clavicle, but there were noother abnormal physical signs. After investigationhe remained under observation as an outpatientfor six years. He was re-examined at the age of25, when no change was found. In 1972 he wasrefused life assurance and was then re-admittedfor further investigation. He still has no symptomsof cardiovascular or respiratory disease but has

recently developed migraine associated withtransient visual field defect, aphasia, andhemianaesthesia.On examination in 1973 there was no cyanosis

nor clubbing. The arterial pulse was normal andregular, and the blood pressure 150/90 mmHg.The venous pressure and pulse were normal. Therewas still a quiet continuous murmur heard in thesecond right intercostal space and below the rightclavicle. The heart was not enlarged; the heartsounds were normal, and there were no othermurmurs. The lungs were clinically normal.The electrocardiogram showed borderline vol-

tage changes of left ventricular hypertrophy butwas otherwise normal. The chest radiograph isdescribed below and illustrated (Fig. 1).Cardiac catheterization (Table I) showed normal

intracardiac pressures. There was no detectableleft-to-right shunt, but arterial blood gas analysisshowed a slightly reduced oxygen tension. Thepulmonary arteriogram and aortogram are des-cribed below. Respiratory function tests (Table II)showed no significant ventilatory abnormality, butgas transfer was slightly reduced.

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A unique pulmonary venous abnormality

RADIOLOGICAL OBSERVATIONS

The 1956 chest radiograph shows a normal heartsize and cardiac contour. The right hilar vesselsare prominent, but peripherally in the right lungthe vessels are normal in size. On the left sidethe upper lobe vessels appear enlarged, and a largeartery is visible behind the heart. Two abnormalvessels, presumably venous, pass vertically down-wards before making an abrupt turn medially to-wards the heart. A previous film in 1947 hadshown slight cardiac enlargement, and the abnor-mal vascular pattern could be recognized in retro-spect. Serial chest radiographs between 1956 and

(a) (b)FIG. 1. (a) Chest radiograph in 1973; (b) magnified view of left hemithorax.

1973 show no significant change though the abnor-mal vessels are more clearly seen (Fig. 1).Pulmonary angiograms were obtained in 1956

by peripheral venous injection, and in 1973 byselective injection into the main pulmonary trunk.These show the proximal pulmonary arteries to betortuous and enlarged but normal in anatomicaldistribution. With the exception of the left lowerlobe artery, the arterial calibre decreases suddenlyat the subsegmental level; the left lower lobeartery remains large until just above the dia-phragm where it divides into several smallbranches (Fig. 2).The pulmonary veins are grossly abnormal

(Fig. 3). On the left side, middle and lower veinscourse vertically downwards peripherally beforeturning sharply inwards towards the heart; an

TABLE ICARDIAC CATHETERIZATION DATA

OxygenSaturation

Site Pressure (mmHg)' per cent

SVC 66IVC 69, 74RA a 9,x 2, v 7, y 5, mean 6 70RV 27/4-9 73PA 26/13, mean 19 73PA wedge a 14, x 10, v 14, y 10, mean 12LV 160/7-16'Aorta 160/100' 96

Oxygen consumption 129 ml/min per m2 BSACardiac index 2-8 I/min per m2 BSAPulmonary arteriolar resistance 2 5 units per m2 BSA

'Reference zero: mid-thorax.Conversion to Pascal units: I mmHg - 0-133 kPa.'After angiography.

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M. Honey, M. C. Pearson, and J. N. Pattinson

TABLE IIRESPIRATORY FUNCTION TESTS

FEV, 3410 mlFVC 3740 mlFEV,/FVC 91%VC' 3800 mlFRC' 3760 mlTLC' 6340 mlRV/TLC 40%Airways resistance' 2-59 cmH,O-' s (0 259 kPa-' s)SGaw 0-103 s-' cmH,O- (103 s-I kPa-1)TF3 24-2 ml min-' mmHg-" (8-0 mmol min-' kPa-')Pao, 70 mmHg (9 3 kPa)

'Body plethysmograph (DuBois et al., 1956).2Body plethysmograph (DuBois et al., 1956; Briscoe andDuBois, 1958).'Single breath method of Ogilvie et al. (1957).

upper vein descends more medially to join themiddle vein. On the right side, more numerousveins in the middle and lower zones follow asimilar course, descending before turning mediallyor medially and upwards; these unite to formlower and middle pulmonary veins, the latter thenjoining the vein from the upper lobe. All the pul-monary veins can be seen to enter the left atrium.The aortogram in 1973 shows an abnormal

systemic artery (Fig. 4a) which had also beennoted on the late films of the venous angiogramin 1956. This arises from the descending aorta, issimilar in position to but larger than a bronchialartery, and passes to the right upper lobe. A tortu-ous vessel resembling a pulmonary venous varixfills from it (Fig. 4b), though the angiographicsequence is not sufficiently long to determine itsfinal drainage site. These vessels are partly respon-sible for the abnormality of the vascular patternabove the right hilum on the plain films.

Anteroposterior and left lateral tomogramsshow that the abnormal vessels lie within the lungsubstance and not on the pleural surface or in thefissures. They also show calcification in the wallof the draining vein of the systemic arterio/pulmonary venous malformation (Fig. 5).

DISCUSSION

This patient has an abnormality of the pulmonaryveins unlike any other we have found in the litera-ture. Normal pulmonary veins take a direct coursefrom the periphery to the hilum of the lung. Inour case, abnormal pulmonary veins descend to-wards the bases before turning medially or medi-ally and upwards: on the left side, there are threemajor vessels, the upper and middle of which unitebefore entering the left atrium; on the right side,there are multiple abnormal veins, dilated,elongated, and sinuous, but generally not tortuous,which join to constitute middle and lower veins,the former then joining a relatively normal upper

FIG. 2. (a) Pulmonary arteriogram: arterial phase;(b) magnified view of left lower and middle zones.

pulmonary vein. In addition, the proximal pul-monary arteries are abnormal in appearance, beingdilated and tortuous, and tapering abruptly, andthe left lower lobe artery is very large, though the

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(a)

(b)

(C.)FIG. 3. (a) Pulmonary arteriogram: venous phase; (b) magnified view of lower half of bothlungs; (c) line drawing of pulmonary veins.

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FIG. 4. (a) A ortogram: arterial phase, showing tor-tuous systemic artery; (b) aortogram: venous phase,showing dilated and tortuous venous channel.

FIG. 5. Tomogram showing calcification of wall ofvenous varicosity in right upper lobe.

course and distribution of the vessels is normal.These changes resemble those seen in pulmonaryarterial hypertension which has been shown not to

be present. In the absence of pulmonary hyper-tension they suggest a structural defect of thevessel wall. Finally, there is a systemic arterio/pulmonary venous anomaly in the right upperlobe. Abnormal pulmonary veins on the left sideand the vascular anomaly above the right hilumwere apparent on the earliest radiograph takenwhen the patient was only 3 years old. Thoughall the radiological changes have become moreobvious as the patient has grown, there can be nodoubt that the pulmonary arterial, pulmonaryvenous, and arteriovenous anomalies have all beenpresent from birth.

Pulmonary venous varices have been reportedby Hipona and Jamshidi (1967), Rizk, Melhem,and Dagher (1970), Brocard, Vannier, and Gal-louedec (1970), and Bartram and Strickland(1971), and these authors have reviewed a numberof similar malformations recorded in the litera-ture. Further cases have been reported morerecently by Papamichael et al. (1972), Castell6Perez Gomez, and Marazuela (1972), Beamish(1972), Perrott and Shin (1974), and Davia et al.(1974) These varices vary greatly: they areusually dilated, elongated, and more or lesstortuous proximal pulmonary veins, draining asingle lobe or segment but following an essenti-ally normal course; the peripheral ramificationsare normal. The dilatation may be localized,forming a sac, or may extend along the length ofthe vein. The other pulmonary veins are normal,as also are the pulmonary arteries. In a few cases,the varices are associated with acquired or con-genital heart disease. Many of these present withsmooth rounded or lobulated lesions seen on theplain chest radiograph which may resembletumour or pulmonary arteriovenous fistula; thesemay be single or multiple but show no resemblanceto the venous abnormality in this case. In othercases, there is a single dilated, tortuous vein(Mouquin et al., 1951; Kozuka and Nosaki, 1968).In the first case of Brocard et al. (1970), the dilatedright lower pulmonary vein descended verticallyfrom the upper part of the lung, turning mediallythrough a right-angle close to the diaphragm; theupper vein was dilated and followed a corkscrew-like course, but its direction was generally normal;both veins entered the left atrium. Bronchographysuggested that the right lung was unilobar. In theirthird case, a large left pulmonary vein which ap-peared to drain the entire lung descended verti-cally, turning medially and then upwards to enterthe left atrium. In their second case, also with ahypoplastic right lung, an anomalous right pul-monary vein entered the inferior vena cava, and

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a second vein draining the right lung was said tofollow an abnormal course to the left atrium but isnot clearly shown in their illustration. On thebasis of their experience, Brocard et al. (1970)suggest that pulmonary varices of the type theyhave encountered are usually part of a complexmalformation, involving also pulmonary arteriesand bronchi, and as such are related to thescimitar syndrome. Though there are points ofsimilarity, none of these cases closely resemblesthe one we have described.On the other hand, Kaindl, Kotscher, and

Lobenwein (1971) have described a patient inwhom the pulmonary venous abnormality is morelike that seen in our patient. In their case 4, theright lung veins were dilated and unusually long,and followed a sinuous course from periphery tohilum with siphon-like loops turning back onthemselves and few branches. The central pul-monary veins were normal and entered the leftatrium. The pulmonary venous anatomy on theleft side is not well shown on their illustrations.As in our case, the proximal pulmonary arterieson both sides were dilated and tortuous.Our view is that the pulmonary venous abnor-

mality in our case represents a congenital anomalyof the course and distribution of the pulmonaryveins. The pulmonary venous system evolves froma primitive plexus on the ventral aspect of thelung bud of the embryo. We can offer no explana-tion of the factors determining the pattern andcourse of the lobar and segmental veins either inthe normal lung or in our case. At an early stagein development there are anastomoses betweenthe pulmonary plexus and the posterior and com-mon cardinal veins, which may persist and lead topartial anomalous pulmonary venous drainage tosystemic veins on either side. In our case, thoughthe development of the normal direct pathwayto the hilum of the lung has been inhibited bysome unknown mechanism, the alternative andcircuitous route reaches a normal destination.

This curious pulmonary venous anomaly has notresulted in any haemodynamic disturbance andindeed none would be expected. It seems likelythat the slightly reduced arterial oxygen tensionand saturation results from a ventilation/perfusionimbalance. Though distribution of ventilation hasnot been measured, there is no significant distur-bance of overall pulmonary ventilation, and it isprobable that the distortion of the orderly ana-tomical pattern of pulmonary venous drainage hasresulted in an abnormal distribution of pulmonaryperfusion. The resulting impairment of gas ex-change is, however, slight, unaccompanied by any

subjective disability and unlikely to affect prog-nosis adversely. Though haemorrhage from pul-monary varices is reported, this has occurred inthose patients in whom the varix is localized andsac-like, and there seems to be no reason to antici-pate this complication in our case. Possibly, how-ever, reduced velocity of blood flow and the latedevelopment of degenerative changes in the veinwall might predispose to pulmonary venous throm-bosis. It is unlikely that the development of mi-graine albeit with severe prodromal symptoms isrelated in any way to the pulmonary venousanomaly, though a congenital cerebral vascularlesion has not been excluded.

REFERENCES

Bartram, C. and Strickland, B. (1971). Pulmonaryvarices. British Journal of Radiology, 44, 927.

Beamish, W. E. (1972). Pulmonary venous varicosity.Journal of the Canadian Association of Radiolo-gists, 23, 136.

Briscoe, W. A. and DuBois, A. B. (1958). The relation-ship between airway resistance, airway conduct-ance, and lung volume in subjects of differentage and body size. Journal of Clinical Investiga-tion, 37, 1279.

Brocard, H., Vannier, R., and Gallouedec, Ch. (1970).Les varices pulmonaires, elements possibles d'ano-malies pulmonaires congenitales complexes.Annales de Medecine Interne, 121, 217.

Castell6, J., Perez G6mez, F., and Marazuela, A.(1972). Varicosided pulmonar. Revista Espanolade Cardiologia, 25, 175.

Davia, J. E., Golden, M. S., Price, H. L., Hastings,J. E., and Cheitlin, M. D. (1974). Pulmonaryvarix: a diagnostic pitfall. Circulation, 49, 1011.

DuBois, A. B., Botelho, S. Y., Bedell, G. N., Marshall,R., and Comroe, J. H. Jr. (1956). A rapid plethys-mographic method for measuring thoracic gasvolume: a comparison with a nitrogen washoutmethod for measuring functional residual capacityin normal subjects. Journal of Clinical Investiga-tion, 35, 322.

and Comroe, J. H. Jr. (1956). A newmethod for measuring airway resistance in manusing a body plethysmograph: values in normalsubjects and in patients with respiratory disease.Journal of Clinical Investigation, 35, 327.

Hipona, F. A. and Jamshidi, A. (1967). Observationson the natural history of varicosity of pulmonaryveins. Circulation, 35, 471.

Kaindl, F., Kotscher, E., and Lobenwein, E. (1971).Zur Radiologie pulmonaler Gefassfehlbildungen.Radiologe, 11, 189.

Kozuka, T. and Nosaki, T. (1968). A pulmonary veinanomaly: unusual connection and tortuosity ofthe right lower lobe vein. British Journal ofRadiology, 41, 232.

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Mouquin, M., Hebrard, H., Damasio, R., Jouvet, P.,Durand, M., and Piequet, J. (1951). Varice dupoumon diagnostiquee par l'angiocardiographie.Bulletins et Me'moires de la Socie'te Medicaledes Hopitaux de Paris, 67, 1091.

Ogilvie, C. M., Forster, R. E., Blakemore, W. S., andMorton, J. W. (1957). A standardized breath hold-ing technique for the clinical measurement of thediffusing capacity of the lung for carbon mon-oxide. Journal of Clinical Investigation, 36, 1.

Papamichael, E., Ikkos, D., Alkalais, K., and Yanna-copoulos, J. (1972). Pulmonary varicosity associ-

ated with other congenital abnormalities. Chest,62, 107.

Perrott, W. W., and Shin, M. S. (1974). Pulmonaryvarix. Journal of Thoracic and CardiovascularSurgery, 68, 318.

Rizk, G., Melhem, R., and Dagher, I. (1970). Bilateralpulmonary varicosities associated with coarctationof the aorta. Thorax, 25, 97.

Requests for reprints to: Dr. Michael Honey, Bromp-ton Hospital, Fulham Road, London SW3.

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auniquepulmonary venous abnormality: with · circuitous route from the periphery, before uniting...

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