Congenital Communications of the RightPulmonary Veins with the Azygos Vein

Report of a Case with Surgical Correction

BY HOWARD N. ANDERSON, M.D., WARREN G. GUNTHEROTH. M.D.,LO1REN C. WINTERSCHEID, M.D., AND K. ALVIN MERENDILNO, M.D.

ULMONARY venous drainage into theazygos vein is a rare type of anomalous

pulmonary venous cardiac return. Brodylfound three cases reported in the medical lit-terature to 1942 and added another case.More recently, this subject has been reviewedby Stecken and Beyer, who diagnosed threecases by tomogram and confirmed the diagno-sis in one of these patients by angiocardiog-raphy.To our knowledge, surgical correctioni of

pulmonary venouis drainage inito the azygosvein has not been reported and for this reasonit was thouglht that the following case reportwould be of interest.

Case ReportP. W., a 6-year-old, 34-pound white girl, was

referred to the University Hospital for evaluationof a cardiac murmur first noted at age 2 years.She xvas essentially asymptomatic anid had neverbeen cyanotic. Physical examination revealed asmall child xvith abnormalities confined to theheart. The point of maximal impulse vas at thelower left sternal border. There was a grade-II/VI systolic murmuir heard in the right firstanid second interspaces adjacent to the sternumi!,a grade-Il/VI systolic murmuir and a grade-I/VIdiastolic mturmtr xvere heard along the left sternialborder. An electrocardiogram revealed incompleteright b-undle-branch block, right axis deviationlanid right ventricular hypertrophy. A cardiacseries revealed a moderately enlarged right atriumand ventricle and increased pulmonary vascular-

From the Departmients of Surgery and Pediatrics,University of Washington Sclhool of Mfedlicinie, Seat-tle, Washington.

Supported by Research Grant HE 03379, Post-doctoral Fellowship HPD-19,016, and Heart Train-ing Grant 2 TI HE 5194-06, U. S. Public HealthService.

Circulation, Valumne XXX, September 1964

ity. There wx as a prominient, ro-unded bulge iil ther,ighlt upper mnediastinum, thouglit to represcieIteither enlarged lymph nodes or an an-omnalous pul-monary vein (fig. 1). (The aorta was located onthe left on the basis of tracheal and esophagealposition.) Cardiac catheterization revealed anoxygen step-up of five volumes per cent betweenthe inferior vena cava and right atrium, a highsaturation of the superior vena caxval blood, andrno right-to-left slhunt. Trhe pollmonary blood flowx as txa ice the systemic blood flow. Right-sidedpressures were essentiallv normal. Pulmonaiy vas-cular resistance -,vas normal. A xenous anigiocardi-ograni revealed that all of the pulmonary venous(drainage fr-om the right luniig entered the azygosvein, which in turn entered the suiperior venacava (figs. 2 anid 3).

/

Figure 1

Preoperatiee roentgenogram showing a prominent,rounded bulge in the right superior mediastinum,right-sided cardiac enlargement, and prominent pul-monary arteries.

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Figure 2

Early phase of ccnotis ang,iocairdiogroatn. A dlilutionldefect is noted int the right psuprior portiont of thesuperior vena cava at the enltrance of the izygosvein.

OperationOn October 27, 1961, the first openi operationi

with extracorporeal support xvas performed viathe right fourth inltercostal space anteriorly. Therevas ani enilarged right atritum, superior ven-a cava,and a huge azvgos vein that emptied high in thesuperior vena cava close to the junction of theright and left innominate veins. No additional ves-sels vere noted entering the stuperior vena cava.The superior caval catheter vas placed through

the right atrium into the riglht innominate vein.The drainage via the left innominate vein xvasretrieved by retrograde drainage xxhen tlhce touirni-quet on the cavae vas occluided distal to theazygos vein. With the right atrium openied, apatent fossa ovalis vas noted. About 300 to 500ml. of blood per miniute returned via the azygosvein. There was conicern that other veniotus chan-nels not easily visualized might be contributingto this flox .. Additional dissection rexcvaled nione.At this point, howxvexr, there xxas a seriouis pro-gressive deterioration of the paticnt's clectro-

cardiogram. The operationl xVas rapidly terminatedafter enilar-ging the atrial septal defect.The1patieInts convalescence xvas uneventful.

Because of concern that the hepatic vein emptiedclii ectl inlto the atrium and that the inferior venacava]l return may have beeni cointiiblitinig to thelarge azygos vein flow nioted diring open cardi-otomyll a veniogramn was carried otit. No aniatol-m-icevidnc e xwas nioted to substantiate this thesis.

Oni jiune 20, 1962, the patient xxas re-exploredand the same fincdiings x, rec o1)served. The onlxVxaliati(Jn ill tehnlllic x\xas the placemenit of thesuiper'ior- ven(iious catheter inito the righit innominatevxeic from the jtiglarviati a small cervical incision.All sxvstemic chest wall veimis emptvinig inito theaix go.s veini xere doubly ligated and (livided. Axwoxven Dacri-on half-t innel xvas uitilized to channelaill aizk-gs) blood ilnto the left atritum via theatrial septal defect (fig. 4) Thlis created somelalrox inlg of the luIimei of the superior venacava. Coniseqtlentlyv, this asxby sututiringa diarmond-shapecd piece of xv70oveni Teflon at astrategic location in the stipericor vena cava. Thercimilind(ler of the operationx unevexntful.

Re-examination 1 eart- postoperatively revealedthe pattient to be muiech mi-ore actctiv(e phvsicallvthaln 1)reoperatively. No carldiomi(eglyxlvas pl)resentoni plysical examination. There xvas a giradle-I/VIsvstolic murmuir at the third left iiter-space. Chestfilmis r-evealed little change in cardiac size. Thepromincenit, roundcledl arlea of the light uipper medi-astinuIi, xxhich represented the disteinded azygosvein, xx-as no loneger xisible ancl pulmolary vascu-

Figure 3

Laite phase of ue1-ous auigiocar.diogrant showing rightpulmloonary veins draining ioito the azyigos vein.

(Cirulation, Voolumc XXX, Septcboer 196-4

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larity was decreased fromn that noted preopera-tively (fig. 5). Arterial saturation wias completelynormal, 98 per cent.

DiscussionAnomalouis pulmononary enoutis (Irainage vxia

the azygos vein is rare. WAZith one exceptionall reported cases Mlxae occuiir-edl in the fe-male. Of interest is the fact thatt it occIursin a variety of anatomic forms. Tlher.e arecases of partial drainage from the rigrlt lungtotal riglht pullmonary venous (Irainage, andtotal puilmonary venols drainiag,e (i.e., bothrighlt and left). This lesion appears to heassociatedi frequetntly xxithi otlher congenitalca,rdiovascutlar anormalies and abnormalitiesof lulng genesis and dceelopmnent. It seemiisto lhave been diagn(osedl on ti vice prior tothe atutlhors' case. Table 1, mlodlified fromnStecken andi Beyer, descriles thie cases re-corded in the medlcal literatiure to 1963 thatlhave been proved bAy angifri()car(iograplhly, op)-eration, or autopsy.The sturgical correction1 of this lesion is not

diffictlt. Hoxever, tlhere can be special prob-lents iii venotus camnulation for bVypss (ilie tothe proximilitv of tlhe azxygos orifice to the con-fluxence of the riglht andl left innominate veilesto form the suiperior vena cav a. This may re-qtiire jlar caiinnulation in the neck for

5uCpefor vena cava-

Azyfos Vlfln- 1.

Pater t toy ai e[l (IVa (irnto left ati UrIC

veins

DPllCC tulltelC

Figure 4

DiLag,a7mn?iutic reperseoftatiori of Sirtical (/10ir. Inset

u/t?orts patch ettlarJgel7)l( it of .s/ii Crin 7(i 211nac a 1.

(Crcilation, Vol/ut XX'X Sepuc,mber 196)(

Figure 5

Posto)jcltii.ett tolg go shottuing (dlis9l)p)pL0aa1lCof thle j)i.ooitiotett, l1IC(b11)d,g ein tltc right sit peiiorqIn (dieatl till M.

satisfactory xvenous returni. f-rom the upperbody (luringlbypass. In this patient, the inter-costal xvems were emnpty ing into the azygos inthleir n.ormal pattern. Obxviously, all systemicvenous channels emptyiing inlto the azygosmiust he dividcd and ligated. If this is not(lone, the atzygos flox of oxygenaited veniouisblood whlen reclianneled into the left atriumis contaminated by desaturated systeimic ve-nlouis blood. In slort, xxhile correcting theleft-to-rigl-ht shulint, one xx011( at the sametime he creatin(g a right-to-left shliunt. There-fore, a careful search withi (livision of all sys-temic venous connectionis to the azygos isnecessary. M7lbile one miglt guess that if thlisadvice were igniored, the riglht-to-left shulntwx ould be of little miiomeint, it can only bestated froim the experience of the first opera-tioii ii this patient that venouis retuirn froimthle azvyos xxas considerable. Unfortuntately.til(h SIMrgCOn did mi(t hlave sufficient presence,of mind to occlude temporarilv the riglht potl-inonary artery so that the contribtution to totaliygo()s floxx contributed 1by tlhei systemic flowcould he separatelvydeterminiedl. Normall,

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this volume is approximately 20 per cent ofthe total body flow.At surgery, one will be sorely tempted to

divide the intercostal veins, temporarily oc-clude the pulmonary artery, ligate and dividethe azygos vein at its junction with the su-perior vena cava, and anastomose the proxi-mal azygos to the left atrium. This was con-sidered at the time of surgery. It wasthought to be feasible but was discarded as apossible solution in this case. The decision notto do so was conditioned by the fear that

Table

due to the normally low intraluminal pressurein the vein, a small error in vein placementmight result in kinking with congestive ve-nous infarction of the right lung. Rather thanrisk this complication, a more familiar pro-cedure was used.

SummaryA case of total pulmonary venous drainage

of the right lung into the azygos vein, surgi-cally corrected, has been reported.There have been 10 previous cases of vary-

Pulmonary Venous Drainage into Azygos Vein

Year Age Sex Veins to azygos Other anomalies Confirmation

I Partial drainage of one lung

Guillabert3 1859 55 yr.

Brody 1

MI Right middle lobe

1942 56 yr. F Right upper lobe

Kjellberg9 1955 26 yr. F Two right upper

lobe veins

Steeken2 1963 18 yr. F One right lowerlobe vein

II Total drainage of either right or left lung

Theremin4 1884 11 da. F

Therernin 4 1884

Right lung

4 mo. F Right lung

Shepherd6 1890 30 yr. F Right lung

Hurwitz7 1937 7wk. F Left lung

Authors' case 1964 6 yr. F Right lung

III Total drainage of both right and left lung

Muira5 1889 6 mo. F

Edwards8 1953 10 yr. F

Right andleft lungs

Right andleft lungs

Patent foramen ovale;right upper lobe veins tosuperior vena cava

Agenesis of the left lung;one lobed right lung

Absent inferior vena cava;anomalous left innominatevein; pulmonarysequestration

Right upper lobe veinsto inferior vena cava

Agenesis of the left lung;one lobed right luing

Agenesis of the left lung;two lobed right lung

Arterial anomalies

Agenesis of the left lung;one lobed right lung

Patent foramen ovale

Patent ductus arteriosus;patent foramen ovale;superior vena cava

entered left atrium;arterial anomalies

Cor biloculare;subpulmonic stenosis

Autopsy

Autopsy

Catheterization,angiocardiogrami,and operation

Angiocardiogram

Autopsy

Autopsy

Autopsy

Autopsy

Catherization,angiocardiogram,and operation

Autopsy

Autopsy

Circulation, Volume XXX, September 1964

Author

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ing degrees of partial right, total right, tototal (bilateral) pulmonary venous drainageinto the azygos vein. The authors' case is thethird to be diagnosed during life and ap-parently the first case to be successfully cor-rected. With one exception, all previouslyreported cases have occurred in females.A review of previous cases and surgical

considerations in the authors' case have beenpresented.

References1. BRODY, H.: Drainage of the pulmonary veins

into the right side of the heart. Arch. Path.33: 221, 1942.

2. STECKEN, A., AND BEYER, A.: Roentgen diagnosisof anomalous insertion of the pulmonary veininto the azygos vein. Fortsch. Ceb. Rontgen-strahlen 98: 1, 1963.

3. GUILLABERT, V.: Montpellier med. 3: 241, 1859.Quoted by Brodyl and by Stecken.2

4. THEREMIN, E.: Rev. mens. mal enf. 2: 554,1884. Quoted by Stecken2 and by Hurwitz.7

5. MUIRA: Virchows Arch path. Anat. 115: 333,1889. Quoted by Brody.1

6. SHEPHERD, F. J.: Some vascular anomalies ob-served during the session 1888-89. J. Anat.Phys. 24: 69, 1890.

7. HURWITZ, S., AND STEPHENS, H. B.: Agenesis oflung. Review of literature and report of case.Am. J. M. Sc. 193: 81, 1937.

8. EDWARDS, J. E.: Pathologic and developmentalconsiderations in anomalous pulmonary venousconnections. Proc. Staff Meet., Mayo Clin.28: 441, 1953.

9. KJELLBERG, S., MANNHEIMER, R. E., RuDHE, U.,AND JONSSON, B.: Diagnosis of CongenitalHeart Disease. Chicago, The Year Book Pub-lishers, 1955.

Observations on TreatmentBy Richard Bright-1827

In the foregoing statements it has been my great object to establish the fact, thatcertain dropsical affections depend more on the derangement of the kidneys themselvesthan has generailly been supposed; and that the albuminous nature of the urine fre-quently points out the particular cases in which these organs are the seat of disease.I wish that I were now able to add any thing con-ipletely satisfactory to myself withregard to the mode of treating these diseases of the kidney. It will be very obviousfrom a review of the cases I have cited, that they sometimes present difficulties so for-midable as to defy the ordinary means of cure; indeed I am inclined to doubt whetherit be possible, after the decided organic change has taken a firm hold on the kidney,to effect a cure, or even to give such relief as may enable the patient to pursue for afew years the occupations of life; where, however, the mischief is less rooted, we mayundoubitedly do much. In the treatment of the disease, as it occurs in sudden attacksof anasarca from intemperance and exposure, in its early stages, and before organicchanges have taken place, we have two distinct indications to fulfil;-we have to restorethe healthy action of the kidney, and we have to guard continually against those danger-ous secondary consequences which may destroy the patient at any period of the disease.The two great sources of icasual danger will be found in inflammatory affections, more

particularly of the serous, sometimes of the mucous membranes, and in the effusion ofblood or serum into the brain, and the consequent occurrence of apoplexy.-OriginalPapers of Richard Bright on Renal Disease. Edited by A. ARNOLD OSMAN. London,Oxford University Press, 1937, pp. 71-72.

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WINTERSCHEID and K. ALVIN MERENDINOHOWARD N. ANDERSON, WARREN G. GUNTHEROTH, LOREN C.

Report of a Case with Surgical CorrectionCongenital Communications of the Right Pulmonary Veins with the Azygos Vein:

Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 1964 American Heart Association, Inc. All rights reserved.

is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231Circulation doi: 10.1161/01.CIR.30.3.439

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