Thorax (1974), 29, 446. Atresia of right pulmonary veins and anomalous left pulmonary venous drainage into portal circulation M. SAMA4NEK, S. TUMA, BENE§OVA, POVY'ILOVA, F. PRAZSKY, and E. CAPoVA Cardiopulmonary Laboratory, University Hospital Motol and Departments of Paediatrics and Pathology, Faculty of Paediatrics, Charles University, Prague, Czechoslovakia Samainek, M., Tuoma, S., Benesova, D., Povfyilova', V., Prazsky, F., and Capova, E. (1974). Thorax, 29, 446-450. Atresia of right pulmonary veins and anomalous left pulmonary venous drainage into portal circulation. An anomaly of pulmonary venous drainage in a male newborn infant is described whereby the left pulmonary veins entered the portal vein and the right pulmonary veins were atretic. A correct diagnosis was made by detecting high-oxygen satura- tion in the hepatic veins, right-to-left shunt at atrial level, and increased pulmonary artery wedge pressure in comparison with the left atrial pressure, and was confirmed by angiography. Atresia of one pulmonary vein or of the common pulmonary vein is an extremely rare condition. Postmortem findings in two cases of pulmonary venous atresia were described by Benesova (1955). Pulmonary veins converged to form a trunk which terminated as a capillary network in the posterior mediastinum, obviously representing a collateral route. Lucas et al. (1962) reported three similar cases of pulmonary venous atresia diagnosed at necropsy. The present report concerns an infant with atresia of the right pulmonary veins, and anomalous drain- age of the left pulmonary veins into the portal circulation, diagnosed during life. To our knowledge no case identical with this one has been reported. CASE REPORT J. B., a male infant of young parents, was born spontaneously following a 34-week gestational period complicated by eclampsia. The birth weight and length were 2,000 g and 48 cm. Cyanosis and tachy- pnoea were the first symptoms noticed shortly after birth. The child was referred immediately to the Department of Paediatrics at the district hospital. On admission he was cyanotic with a respiratory rate of 80/min and pulse rate of 154/min. Fine moist rales were detected at the base of the right lung. There was no audible heart murmur. The liver was not enlarged. Acidosis with reduction of standard bicarbonate and increased PCO2 was corrected, but mild acidosis of the 'respiratory type' persisted (Table I). The chest radiograph showed a normal TABLE I BLOOD GAS TENSION AND ACID-BASE EQUILIBRIUM Bicarbonates (mEq/l) Age Pco, Po, SO2 (weeks) (torr) (torr) % pH Standard Actual 2 69 - - 7*200 18-7 26 3 4 43 52 77 7*222 16 2 17-1 6 57 40 68 7 308 2454 2830 7 61 50 80 7*308 2545 3021 cardiac silhouette. Increased density and diffuse granular opacification throughout the right lung field contrasted with the normal appearance of the left lung. During the first three weeks the condition improved slightly. Unilateral neonatal pneumonia was diagnosed clinically and the patient was treated with antibiotics and oxygen. The clinical condition deteriorated progressively at the end of the third week of life and the infant was admitted to the University Hospital Motol. The child was mildly cyanotic and dyspnoeic, the respira- tory rate was about 90/min and the heart rate 1 50/min. The peripheral pulses were normal. Inspira- tory and expiratory moist rales spread from the base to the whole right lung. Normal auscultatory findings persisted in the left lung. The first heart sound was normal in intensity and the second sound was accentuated at the upper left sternal border. A soft grade 2/6 ejection systolic murmur was heard in the second to third left intercostal space at the sternal border. The liver was palpable 4 cm below the right costal margin. 446 on October 23, 2020 by guest. Protected by copyright. http://thorax.bmj.com/ Thorax: first published as 10.1136/thx.29.4.446 on 1 July 1974. Downloaded from
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Thorax (1974), 29, 446.
Atresia of right pulmonary veins and anomalous leftpulmonary venous drainage into portal circulationM. SAMA4NEK, S. TUMA, BENE§OVA, POVY'ILOVA,
F. PRAZSKY, and E. CAPoVACardiopulmonary Laboratory, University Hospital Motol and Departments of Paediatrics and
Pathology, Faculty of Paediatrics, Charles University, Prague, Czechoslovakia
Samainek, M., Tuoma, S., Benesova, D., Povfyilova', V., Prazsky, F., and Capova, E. (1974).Thorax, 29, 446-450. Atresia of right pulmonary veins and anomalous left pulmonary venousdrainage into portal circulation. An anomaly ofpulmonary venous drainage in a male newborninfant is described whereby the left pulmonary veins entered the portal vein and the rightpulmonary veins were atretic. A correct diagnosis was made by detecting high-oxygen satura-tion in the hepatic veins, right-to-left shunt at atrial level, and increased pulmonary arterywedge pressure in comparison with the left atrial pressure, and was confirmed by angiography.
Atresia of one pulmonary vein or of the commonpulmonary vein is an extremely rare condition.Postmortem findings in two cases of pulmonaryvenous atresia were described by Benesova (1955).Pulmonary veins converged to form a trunk whichterminated as a capillary network in the posteriormediastinum, obviously representing a collateralroute. Lucas et al. (1962) reported three similarcases of pulmonary venous atresia diagnosed atnecropsy.The present report concerns an infant with atresia
of the right pulmonary veins, and anomalous drain-age of the left pulmonary veins into the portalcirculation, diagnosed during life. To our knowledgeno case identical with this one has been reported.
CASE REPORT
J. B., a male infant of young parents, was bornspontaneously following a 34-week gestational periodcomplicated by eclampsia. The birth weight andlength were 2,000 g and 48 cm. Cyanosis and tachy-pnoea were the first symptoms noticed shortly afterbirth. The child was referred immediately to theDepartment of Paediatrics at the district hospital.On admission he was cyanotic with a respiratory
rate of 80/min and pulse rate of 154/min. Fine moistrales were detected at the base of the right lung. Therewas no audible heart murmur. The liver was notenlarged. Acidosis with reduction of standardbicarbonate and increased PCO2 was corrected, butmild acidosis of the 'respiratory type' persisted(Table I). The chest radiograph showed a normal
TABLE IBLOOD GAS TENSION AND ACID-BASE EQUILIBRIUM
cardiac silhouette. Increased density and diffusegranular opacification throughout the right lungfield contrasted with the normal appearance of theleft lung. During the first three weeks the conditionimproved slightly. Unilateral neonatal pneumoniawas diagnosed clinically and the patient was treatedwith antibiotics and oxygen.The clinical condition deteriorated progressively at
the end of the third week of life and the infant wasadmitted to the University Hospital Motol. Thechild was mildly cyanotic and dyspnoeic, the respira-tory rate was about 90/min and the heart rate1 50/min. The peripheral pulses were normal. Inspira-tory and expiratory moist rales spread from the baseto the whole right lung. Normal auscultatoryfindings persisted in the left lung. The first heartsound was normal in intensity and the second soundwas accentuated at the upper left sternal border. Asoft grade 2/6 ejection systolic murmur was heard inthe second to third left intercostal space at thesternal border. The liver was palpable 4 cm belowthe right costal margin.
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A tresia of right pulmonary veins and anomalous left pulmonary venous drainage 447
Electrocardiograms obtained up to the fourth weekof life did not differ from normal. At the sixth weekthe frontal QRS axis was + 1040, and a mild right ANventricular hypertrophy was noted without any Pwave abnormality (Fig. 1).
Multiple chest radiographs did not reveal any jcardiac enlargement (Fig. 2). Increased density anddiffuse granular opacification in the right lung was apermanent finding. The right cardiac border wasobscured and distinct Kerley B lines were revealed inthe right costophrenic angle. The right pulmonaryartery and its branches were not visible. The radio-graphic appearance of the left lung differed from thatof the right lung. The pulmonary vascular markingswere abundant, especially in the hilar region. Theleft lung was more radiolucent than the right andhad a reticular rather than granular pattern. KerleyB lines were absent. The liver shadow was enlarged.Lung scanning demonstrated a marked decrease
in the right pulmonary blood flow (Fig. 3). Theperfusion of the left lung was evenly distributed.Blood gas tension and acid-base equilibrium were
repeatedly abnormal (Table I) with a tendency to a
- FIG. 2. Plain chest radiograph, aged 4 weeks. For descrip-* r 4Q <* O. s tion, see text. Distinct Kerley B lines were seen only on the
T r 1 vi original x-ray picture.I_v_1_
4-~~~~~~~~~~~~~~~5A
It;li i
i i t'.. A ICi-A AIl.Z.1 1JU
t1+To~id It16
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cIVL J V2.4.... FIG. 3. Lung scan, aged 5 weeks. Macroaggregatedv- -' ti human serum albumin tagged with technetium-99mwas given intravenously in a dose of 50 MCi. The distri-
4++bution of radioactivity was detected by Pho/Dot II-t ~~vs? rectilinear scanner (Nuclear, Chicago) equipped with
aVF a 127-hole collimator. For description, see text.
- - -> ~- -Z 3~progressive of Pco2 value. labora-
1.1ijit ^{ I gr t s zetorytests were normal.V6 -Cardiac catheterization was performed through
ft - the right saphenous vein (TableIt). The pressuretr-acings showed pulmonary arterial hypertension
FIG 1 Electrocardiogram, aged 6 weeks. of the third grade, elevation of the right atrial
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pressure to the level of the left atrial pressure, and amarked increase ofthe right pulmonary artery wedgepressure. The difference in blood oxygen saturationin the inferior vena cava proximal and distal to theorifices of the hepatic veins and the 90% saturationof hepatic venous blood were suggestive of anoma-lous pulmonary venous drainage into the portalcirculation. Oxygen saturation in the left heart washigher than in the right atrium and ventricle. Anattempt to enter the pulmonary veins from the leftatrium was not successful.The right ventricular biplane angiogram demon-
strated, in the early phase, a marked differencebetween the right and left pulmonary vascular beds(Fig. 4a). The right pulmonary artery was hypo-plastic and the peripheral branches appeared to benarrow and deficient. In contrast, hypervascularityand engorgement of the pulmonary arteries weredetected in the left lung. The contrast medium fromthe left pulmonary veins drained into a commonvertical channel entering the portal vein. The anoma-lous common pulmonary venous trunk is bestshown in the later phase of angiography (Fig. 4b).Its terminal branching is well outlined and there isopacification of the liver. Delayed clearing ofcontrast inedium from both pulmonary vascularbeds, and particularly from the right pulmonaryartery, was observed. A narrowing at the site of theentrance of the pulmonary venous trunk into theportal vein was not seen.
Regional lung volume studied by a modification ofxenon-133 equilibration technique (Ball, Stewart,Newsham, and Bates, 1962; Ruth and gaminek, 1973)was lower in the right lung than in the correspondingzones of the left lung (Fig. 5; Table III). The volumeof the lower regions was less than that of the upperregions in both lungs. Radioactive gas washout wasfaster in the right lung than in the left. There was only
~~~~~~~~~~~~I
FIG. 4. Right ventricular angiogram, frontal view: (a)earlyphase; (b) later phase. For description see text.
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A tresia of right pulmonary veins and anomalous left pulmonary venous drainage
Vr
I1O c1s .- -
v-.WVV
100 cps
I RL
'I0secFIG. 5. Xenon-133 scintigraphy. Rebreathing of oxygenlabelled with 0 5 UCi '33Xe per litre in a closed systemequipped with a C02 absorber. Regional radioactivity afterequilibration is proportional to the volume of the airspacesunder the detectors communicating with the atmosphere.Coupled detectors of i-inch diameter were located in thecentre of the upper and lower halves of both lungs. Afterequilibrium was reached the patient was switched to theinhalation of air, and the regional washout curves of 133Xewere recorded.
TABLE II]REGIONAL DISTRIBUTION OF LUNG VOLUME
AND VENTILATION
Lung Volume Washout Half-timeLung Region ('% of total) (seconds)
Right upper 17 7-3lower 10 7-8
Left upper 44 10-8lower 29 10-4
an insignificant difference between the individualregions within one lung.On the basis of the clinical picture, catheterization
data, chest radiographs, and angiography, a diagnosiswas made of right pulmonary venous atresia,anomalous drainage of the left pulmonary veins intothe portal vein, and interatrial communication.Surgical intervention was not recommended becauseof the unfavourable prognosis of the combination ofanomalous pulmonary venous drainage and atresia.The infant died at the age of 7 weeks. At necropsy
the heart was normal in size and weight. The rightatrium and ventricle, the superior and inferior venaecavae, all valves, and the position of the greatarteries did not exhibit any abnormality. The ductusarteriosus was contracted. A widely patent foramen
ovale enabled good communication between theright and left heart. The left atrium was hypoplasticand the left ventricle was slightly smaller than theright ventricle. The right pulmonary artery wasnarrow. A common pulmonary venous trunkemerging from the left lung descended in the posteriormediastinum to the diaphragm and passed throughthe oesophageal hiatus to terminate in the portalvein (Fig. 6). The right pulmonary veins convergedto form a common trunk leading from the right lung,passing beneath the right pulmonary artery towardsthe left main bronchus. From a blind recess on theend of the trunk only, several small vessels disappear-ing in the mediastinal tissue were identified. Macro-scopically there was a honeycomb appearance of theright lung due to multiple holes formed by cysticlymphangiectases. Prominence of the dilated lym-phatics was evident on inspection of the pleuralsurface of both lungs. Histologically, dilated cystic
FIG. 6. Posterior view of the specimen demonstrates thepulmonary venous trunk (PVT) entering theportal vein (VP).Right pulmonary veins (PV) end as a blind recess. Note theappearance ofthe lungs. For details, see text.
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M. gamacnek, S. Teima, D. Benesova, V. Povysilova, F. Prazsky, and E. (ipova
FIG. 7. Microscopic section ofsubpleural tissue in right lung showing dilated lymphaticspaces and thickening ofinterlobular connective tissue (x 120).
lymphatics were found mostly under the visceralpleura and along the bronchi in the thickenedperibronchial tissue (Fig. 7). All changes weredeveloped more in the right lung than in the left. Theliver was enlarged and its weight was increased.
COMMENTS
Recent advances in the surgical correction of totalanomalous pulmonary venous drainage emphasizethe importance of an early precise diagnosis of thismalformation. In our patient the delay in making acorrect diagnosis was due mainly to the difference inthe auscultatory and x-ray findings between theright and left lung, supporting the diagnosis of aunilateral pneumonia. The decreased volume of thepatent airspaces in the right lung as compared with theleft lung can be explained by pulmonary venousatresia leading to unilateral pulmonary oedema.Significant unilateral or regional obstruction of theairways could be excluded by evaluating regionalwashout curves. Higher pulmonary vascular resist-ance in the right than in the left lung was thought to beresponsible for the abnormal pulmonary blood flowdistribution proved by the isotope techniques.However, lung scanning was not able to differentiatebetween precapillary and postcapillary pulmonaryvascular obstruction. The definite diagnosis wasmade by cardiac catheterization and angiography.An interesting finding at catheterization was the
preferential distribution of the inferior vena cavalblood to the left atrium which maintained arterialblood saturation at about 80%. High postcapillaryresistance was indicated by elevation of thepulmonary wedge pressure. An obstruction in theheart was excluded by the normal values of the leftatrial and ventricular pressures. The localization ofthe resistance in the hepatic circulation as well as theprecise anatomic diagnosis were made by angio-graphy.
REFERENCES
Ball, W. C., Stewart, P. B., Newsham, L. G. S., andBates, D. V. (1962). Regional pulmonary functionstudied with xenon 133. Journal of Clinical Investiga-tion, 41, 519.
Lucas, R. V. Woolfrey, B. F., Anderson, R. C., Lester,R. G., and Edwards, J. E. (1962). Atresia of thecommon pulmonary vein. Pediatrics, 29, 729.
Ruth, C. and SamAnek, M. (1973). Investigation ofregional lung function using stationary radiationdetectors. In Investigation of the Regional LungFunction, edited by M. SamAnek, pp. 51-58. Czecho-slovak Society of Clinical Physiology of Respiration,Karlovy Vary.
Requests for reprints to: Dr. Milan SamAnek, UniversityHospital Motol, 150 06 Prague, Czechoslovakia.
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