Thorax (1958), 13, 272.

ABSENT LEFT PULMONARY ARTERY AND RIGHT-SIDEDAORTIC ARCH IN EISENMENGER'S COMPLEX

BY

H. A. FLEMINGFrom the Cardiac Department, Brompton Hospital, London

(RECEIVED FOR PUBLICATION JANUARY 6, 1958)

Absence of a pulmonary artery is relativelyrare, and when McKim and Wiglesworth reportedsix examples in 1954 they could find only 11others in the literature since 1868. Most of themhad been discovered at operation or necropsy. AsMaier (1954) pointed out, the anomaly is foundmuch more frequently in patients submitted toangiocardiography than at necropsy, when it caneasily be ov erlooked. By 1956 Emanuel andPattinson were able to find 46 cases reported inthe literature. Of these, 18 were associated withFallot's tetralogy, and in all but one it was theleft pulmonary artery which was absent; in theexception, there was dextrocardia and situsinversus. The absence of the left pulmonaryartery was associated with a right-sided aorta in60% of these cases. Most of the other cases thathave been reported have also been associated witha major cardiac or vascular defect, althoughMaier (1954) mentioned one in which no otheranomaly could be found. Wyman (1954) likewisereported a case in which absence of the pul-monary artery was the sole defect, and Steinberg,Dotter, and Lukas (1953) described two others inpatients aged 26 and 53 years. No associatedcongenital abnormalities in other systems havebeen cited.

Nadas, Rosenbaum, Wittenborg, and Rudolph(1953) stated that all cases of absent pulmonaryartery without a cardiac defect occurred onthe right side; one of the two cases describedby Steinberg and others (1953), however, had anabsent left pulmonary artery. McKim andWiglesworth (1954) noted that the acwtic archcommonly lies on the side opposite to the absentpulmonary artery.Although studies of associated cardiac lesions

have often been incomplete, there is evidence thatFallot's tetralogy is particularly common with thisabnormality, a point that should be borne in mindwhen contemplating Blalock's anastomosis (Nadasand others, 1953). Other associated lesions so far

reported include atrial septal defect, patent ductusarteriosus, coarctation of the aorta, pulmonarystenosis, absence of the transverse aortic arch,and persistent truncus arteriosus (Humphreys,1932; Sweet and White, 1950; Kjellberg, Mann-heimer, Rudhe, and Jonsson, 1955). Eisenmenger'scomplex has been recorded only once, in anecropsy on a 20-months-old boy with an absentleft pulmonary artery and a right-sided aorticarch (McKim and Wiglesworth, 1954).The purpose of the present paper is to present

a case of Eisenmenger's complex with absent leftpulmonary artery and right-sided aortic arch inwhich the diagnosis was established during life.This is believed to be the first such case reported.

CASE REPORT

The patient was a little girl aged 7 years, fromTurkey. Since she was not accompanied by herparents or anyone speaking English, it was not pos-sible to obtain a complete history. Cyanosis datedfrom birth and was increasing; it was particularlynoticeable when she was tired or when she exertedherself. There was a dubious history of precordialdiscomfort on exertion. In the ward she wasobserved to squat very frequently and she tiredeasily.

She was small for her age, being 3 ft. 7j in.(110 cm.) in height and weighing 35 lb. (16 kg.). Thesternum protruded markedly, but she had no otherexternal congenital abnormalities. There was moder-ate central cyanosis and clubbing of the fingers andtoes. All peripheral pulses were normal. The bloodpressure was 120/80 mm. Hg in both arms. Thejugular venous pressure was not raised, but the awave was abnormally conspicuous. The apex beatwas displaced to the left, being formed by a hyper-dynamic right ventricle felt in the anterior axillaryline. The pulmonary artery impulse was impalpable.On auscultation there was a loud right ventricularthird heart sound, increasing on inspiration. In thesecond left intercostal space was a loud systolic clickpreceding a short systolic ejection murmur. The

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ABSENT LEFT PULMONARY ARTERY AND RIGHT-SIDED AORTIC ARCH 273.. ..1.V

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FIG. 1.-Electrocardiogram showing considerable right ventricular hypertrophy.

second sound was loud and single. The movementsand the breath sounds on both sides of the chest werenormal, and other systems were normal. The haemo-globin was 16.6 g./ 100 ml. (112%). An electrocardio-gram (Fig. 1) confirmed sinus rhythm and showedconsiderable right ventricular hypertrophy. Radio-graphy revealed a small left lung with displacement ofthe heart to the left (Fig. 2). The aortic arch wasright sided, the main pulmonary artery was wellshown, and the right ventricle was moderatelyenlarged. The left branch of the pulmonary arterycould not be seen, and the vessels in the left lung wereextremely small and spidery. The arteries in the rightlung appeared to be of normal distribution and wereoverfilled.

Angiocardiography, using 25 ml. of 70% sodiumacetrizoate (Fig. 3), showed a large right atrialappendix. The pulmonary artery and aorta filledsimultaneously from the right ventricle, but the bulkof the contrast medium entered the pulmonary artery.No left pulmonary artery could be seen. The right-

sided aortic arch crossed to the left of the spine abovethe diaphragm. The main pulmonary artery mergeddirectly into the right pulmonary artery to forma single uninterrupted curve. The plethoric appear-ance of the right lung was confirmed and no filling ofthe vessels in the left lung was seen even in the laterfilms.Cardiac catheterization was carried out under local

anaesthesia and from the right arm. The catheterpassed easily from the right ventricle into the aortawhence it was advanced to the descending aorta andthe various branches of the arch. It was also passedinto the pulmonary artery and wedged in a branchin the right upper lobe. Repeated attempts to enterthe aorta from the pulmonary artery were unsuccess-ful. The pulmonary and aortic valves lay at a ratherlow level. No other defects could be entered. Pres-sures were measured from the sternal angle. Theoxygen content of the samples was measured by themethod of Van Slyke and Neill. The results areshown in Table I.

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H. A. FLEMING

FIG. 2.-Postero-anterior radiograph showing displacement of theheart to the left, right-sided aortic arch, plethoric right lung, andsmall, translucent left lung.

TABLE IRESULTS OF CARDIAC CATHETERIZATION

Systolic 02 Contentj xyeSite Diastolic Mean of Blood SaturationPressures (mm. Hg) Sample-i~ /

(mm. Hg) (ml. I.)

Aorta .. .. 82/50 142 72 8Pulmonary artery.. 82155 70 129 66-5Right ventricle 8515 120 61 6Pulmonary capillary +2 -1 0 - -

Right atrium .. +4-5 115 59-0

02 capacity.195 ml. 1.02uptake. 118 ml. min. (calculated)Systemic blood flow.4.4 I./min.Pulmonary blood flow.2.1 .,'min.Pulmonary vascular resistance .. > 30 units (normal, < 2 units)

DISCUSSIONThe initial bedside diagnosis in this case was

Fallot's tetralogy with cardiac displacement to theleft associated with thoracic deformity; but theconspicuous a wave, hyperdynamic right ventricle,diastolic gallop, and very short ejection murmurwere disturbing. The click was attributed to theaorta. The radiograph showed that the displace-ment of the heart to the left was due to an absentleft pulmondry artery and poorly expanded leftlung, but did not otherwise alter the diagnosis;indeed, the right-sided aortic arch seemed ratherto confirm it. The slightly plethoric right lung

FIG. 3.-Angiocardiogram showing absent left pulmonary artery andsimultaneous filling of the aorta and pulmonary artery.

was attributed to the fact that the entire pul-monary blood flow was passing through it.Similar appearances were noticed by Nadas andothers (1953) when Fallot's tetralogy was com-plicated by absence of the left pulmonary artery.

Transposition, persistent truncus arteriosus, andEisenmenger's complex had to be excluded, andto this end the angiocardiogram was carried out.This confirmed the gross anatomical observationsalready made and eliminated transposition. Per-sistent truncus arteriosus was excluded by thesesame anatomical appearances and also by thestrikingly greater opacification of the pulmonaryartery than of the aorta in the early films. Thisis in contrast to the angiocardiogram reproducedby Kjellberg and others (1955) in their case ofpersistent truncus arteriosus with absent right pul-monary artery. The differential diagnosis betweenFallot's tetralogy and Eisenmenger's complex,however, was by no means resolved by the angio-cardiogram, for no pulmonary stenosis could beseen. Lowe (1953) found no diagnostic differ-ences between these two conditions on angio-cardiography in the absence of visible stricture,and Doyle, Goodwin, Harrison, and Steiner (1957)state that it may be impossible to recognize pul-monary hypertension in congenital heart disease

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ABSENT LEFT PULMONARY ARTERY AND RIGHT-SIDED AORTIC ARCH 275

from the radiological appearances of the pul-monary vessels.

Cardiac catheterization solved the problem bydemonstrating the absence of pulmonary stenosis,the identity of the systolic pressures in the rightventricle, aorta and pulmonary artery, and a con-siderable right-to-left shunt at ventricular level.The pulmonary vascular resistance was greatly inexcess of the systemic, this being the situation inthe Eisenmenger syndrome (Wood, 1956). Noother defects could be entered and the inabilityto enter one great vessel from the other was takenas further evidence against persistent truncusarteriosus.No treatment was advised and the child

returned to her home country.

FUNCTIONAL PATHOLOGYIn the few cases of unilateral absence of the

pulmonary artery in which the matter has beenstudied, the major bronchi to the avascular lungare normal and show no more than minora natomical variations. Madoff, Gaensler, andStrieder (1952) and Nadas and others (1953) havedemonstrated this by bronchography. It has alsobeen demonstrated by dissection at necropsy.The lung is usually of less than normal volumebut clinically appears to ventilate well; on

fluoroscopy there is no mediastinal swing withrespiration. Maier (1954) described a case inwhich the avascular lung decreased progressivelyin size and when finally removed at operation wasfound to be diffusely fibrosed. There may beherniation of the normal lung through the medi-astinum, but there are no ill effects from this.Steinberg and others (1953) could find only slightphysiological evidence of emphysema in a womanaged 53 years. The most complete physiologicalstudies have been made by Madoff and others(1952), who found that the subdivisions of lungvolume were near normal, but the minute ven-

tilation at rest and on exercise was increased, theabnormal lung ventilating without participating inthe oxygen uptake, as was demonstrated bybronchospirometry.

In many reported cases enlarged bronchialvessels on the affected side have been demon-strated by means of angiocardiography or atnecropsy; in a few, a large anomalous branch ofthe innominate artery has entered the avascularlung. Madoff and others (1952) estimated that17% of the total output of the left heart enteredthe affected lung in their case, and Findlay andMaier (1951) suggested that this increase in thebronchial supply could lead to heart failure. In

our case the lung vessels were particularly smalland did not opacify at any time in the angio-cardiogram. This was so also in cases reportedby Steinberg and others (1953) and by Madoff(1954), who checked his observations at necropsy.

In all necropsy cases there has been no centralportion of the affected pulmonary artery thoughthe intrapulmonary portion was normally deve-loped. McKim and Wiglesworth (1954) andEmanuel and Pattinson (1956) offer a develop-mental explanation for this. The former describe,in their left-sided cases, an anomalous obliteratedvessel arising from the innominate artery andpassing to the intrapulmonary vessels at the hilum.This, they suggest, is an obliterated ductusarteriosus.

CONCLUSIONS

Cases of unilateral absence of the left pul-monary artery are commonly associated withmajor cardiac defects (Emanuel and Pattinson,1956) and a right-sided aortic arch (McKim andWiglesworth, 1954). They thus present a cardio-logical problem.

Cases of unilateral absence of the right pul-monary artery commonly have a normal left-sidedaortic arch and a normal heart, though there maybe other anomalies of the great vessels. Theypresent as a respiratory problem.The isolated condition is benign and is com-

patible with normal life and good respiratoryfunction. It may be an incidental finding atroutine examination. In these circumstancesdysgenesis of the lung, lobar collapse with com-pensatory over-distension of the rest of the-lung, bronchiectasis and unilateral transradiancy(Macleod, 1954) may need to be eliminated.Tomography, bronchography, and broncho-spirometry will be helpful in confirming thediagnosis, and angiocardiography will rarely benecessary.

SUMMARY

A case of Eisenmenger's complex with a right--sided aortic arch and absent left pulmonary arterydiagnosed in life is described. The literature isreviewed and the functional pathology and differ-ential diagnosis are discussed.

My thanks are due to Dr. Paul Wood and SirRussell Brock, under whose care this patient was in-vestigated, and to Dr. Paul Wood for much helpfulcriticism. I am grateful to Sister V. G. Jones andMrs. P. M. Milne for technical help with the angio-cardiogram and the catheterization. Mr. D. F. Kemp-produced the photographs.

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H. A. FLEMING

ADDENDUMSince this paper was submitted Elder, Brofman,

Kohn, and Charms (1958) have reported five casesof unilateral absence or hypoplasia of the pul-monary artery without major cardiac defect. Fourof these affected the left side. Two cases hadgross bronchiectasis and the others all sufferedfrom haemoptysis with or without pulmonaryinfection.

REFERENCES

Doyle, A. E., Goodwin, J. F., Harrison, C. V., and Steiner, R. E.(1957). Brit. Heart J., 19, 353.

Elder, J. C., Brofman, B. L., Kohn, P. M., and Charms, B. L. (1958).Circulation, 17, 557.

Emanuel, R. W., and Pattinson, J. N. (1956). Brit. Heart J., 18, 289.Findlay, C. W., and Maier, H. C. (1951). Surgery, 29, 604.Humphreys, E. M. (1932). Arch. Path. (Chicago), 14, 671.Kjellberg, S. R., Mannheimer, E., Rudhe, U., and Jonsson, B. (1955).The Diagnosis of Congenital Heart Disease. Year Book Pub-

lishers, Chicago.Lowe, J. B. (1953). Brit. Heart J., 15, 319.McKim, J. S., and Wiglesworth, F. W. (1954). Amer. Heart J., 47,

845.Macleod, W. M. (1954). Thorax, 9, 147.Madoff, I. M. (1954). J. thorac. Surg., 28, 161.- Gaensler, E. A., and Strieder, J. W. (1952). New Engl. J. Med.,

247, 149.Maier, H. C. (1954). J. thorac. Surg., 28, 145.Nadas, A. S., Rosenbaum, H. D., Wittenborg, M. H., and Rudolph,

A. M. (1953). Circulation, 8, 328.Steinberg, I., Dotter, C. T., and Lukas, D. S. (1953). J. Amer. med.

Ass., 152, 1216.Sweet, R. H., and White, P. D. (1950). New Engl. J. Med., 242, 258.Wood, P. (1956). Diseases of the Heart and Circulation, 2nd ed.

Eyre and Spottiswoode, London.Wyman, S. M. (1954). Radiology, 62, 321.

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