Stenosis of a Branch of the Pulmonary ArteryAn Additional Cause of Continuous Murmurs over the Chest

By FREDERIC ELDRIDGE, M.D., ARTHUR SELZER, M.D., AND HERBERT HULTGREN, M.D.

Three patients have been demonstrated by cardiac catheterization to have stenosis of a mainbranch of the pulmonary artery. Two exhibited continuous ductus-like murmurs at the chestwall over the site of stenosis. Demonstration of a pressure gradient throughout the cardiac cycleat the site of stenosis in these 2 patients and the experimental production in the dog of a continuousmurmur at the site of partial constriction of a pulmonary artery prove that the murmur is due tothe stenosis. This anomaly should be added to the differential diagnosis of continuous murmurs overthe chest.

STENOSIS of a main branch of the pulmo-nary artery has been previously considered

very rare. Bredt' and Monckeberg' believedthat it never occurred independently of stenosisof the pulmonary orifice. Schwalbel in hiscomprehensive survey of malformations re-ferred to 4 reported instances, but a review ofthe original reports reveals only 2 in whichnarrowing of a branch of an otherwise normalpulmonary artery was present,4 6 the other 2being patients with absence of either the rightor left pulmonary artery. In 1938 Oppen-heimer6 reported a stricture of the right pul-monary artery and of the lower branch of theleft pulmonary artery in a 17-month-oldinfant. Four patients with stenosis at the areaof the bifurcation of the pulmonary artery andinsertion of the ligamentum arteriosum havebeen discovered at operation by Schumaker andLurie7 and S0ndergaard.8 Kjellberg and hisassociates9 mention having seen a case ofsupravalvular stricture of the main pulmonaryartery. Arvidsson and his associates10-'2 re-cently reported 4 patients with pulmonaryhypertension in whom multiple stenoses of themain branches of the pulmonary artery weredemonstrated by selective angiography. In 1of these patients a continuous murmur waspresent over the upper chest.

In this communication 3 additional patientswith stenotic lesions of the main branches of

From the Department of Medicine of the StanfordUniversity School of Medicine, San Francisco, Calif.

Frederic Eldridge and Herbert Hultgren areScholars in Medical Science of the John and Mary R.Markle Foundation.

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the pulmonary artery are described including 2patients manifesting continuous murmurs.Experimental evidence is also presented that acontinuous murmur can be produced by con-striction of the main branches of the pulmonaryartery.

CASE REPORTSCase 1. G.T., a 7-year-old Negro girl, entered the

Stanford University Hospital in February 1956 withthe history of a heart murmur and congenital ab-sence of the right radius having been noted at theage of 1 year. She had been asymptomatic exceptfor several episodes of pneumonitis.

She was an alert, active girl. Her brachial bloodpressure was 94/66 mm. Hg. There was shorteningand radial deviation of the right forearm. The lungswere clear. The heart was regular. At the pulmonicarea the second sound was loud and slightly split.There was a grade III murmur filling systole overthe left precordium, and a loud continuous murmurof grade IV intensity was heard at the right secondintercostal space just medial to the midelavicularline. This murmur was well transmitted to the rightaxilla and back.The urinalysis and blood count were not remark-

able. The electrocardiogram showed high voltage Rwaves over the right precordium, compatible withright ventricular hypertrophy. X-ray picturesshowed marked pulmonary plethora, right ventricu-lar enlargement, and widening of the superior medi-astinum. A phonocardiogram (fig. 1) demonstrateda continuous murmur over the second intercostalspace, just medial to the right midelavicular lineand a loud, slightly split second sound in the pul-monic area. The continuous murmur was essentiallysimilar to the machinery murmur produced by theusual uncomplicated patent ductus. The murmurappeared about 0.06 second after the peak of theapical first sound, increased to maximal intensityjust before or at the time of the second sound, andfaded away in diastole. The diastolic component

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STENOSIS OF PULMONARY ARTERY BRANCH

PULMONARY I 1STALwCQ4 4IRIKYT PULMONARY ARTIRY

, ~ ~~MAIN

IPROXIMAL 1,jPULMONARY .Lfl)SK1Tam"IRISK PULMONARY ARTERtY ARTCRY

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2 1 2 1 2 1 2RISK5M b...

INTIRCOSYAL L_-*PAI _

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FIG. 1. Case 1. The top tracing shows pressures obtained during withdrawal of the cardiac catheterfrom right lung. The pressure in the distal right pulmonary artery shows a systolic dip due to theVenturi effect. Next is a sharp rise in pressure as the catheter tip is withdrawn to the proximal rightpulmonary artery, and then a further sharp rise of pressure in the main pulmonary artery. Both sharprises of pressure indicate sites of stenosis. The lower 2 tracings are phonocardiograms showing thecontinuous murmur heard in the right second intercostal space and the heart sounds as heard in thepulmonic area. The first and second heart sounds are shown on both tracings by the numbers 1 and 2.

appeared to diminish in intensity readily duringexpiration.

Cardiac catheterization revealed a high oxygensaturation in the superior vena cava (86 per cent)and the right atrial oxygen saturation was higherthan that in the inferior vena cava. In addition,there were arterial anoxemia, moderate pulmonaryhypertension, and at least 2 sites of stenosis in theright main pulmonary artery (table 1 and fig. 1).The pressure tracing recorded during the withdrawalof the catheter from the wedged position revealedfirst a typical, normal, wedged-pressure tracing(fig. 1, far left), then an abrupt change in the pres-sure curve with a distinct dip in pressure duringsystole and then an abrupt increase in pressure witha positive pressure pulse during systole, and finallya second abrupt rise in pressure. The systolic dip inthe pressure trace is probably due to the blood flowthrough the constricted vessel producing a Venturieffect, such as is occasionally seen when the cathetertip is at the stenotic orifice in valvular pulmonicstenosis.13' 14 This portion of the tracing andthe 2 abrupt rises in pressure in the pulmonaryartery conclusively establish the presence of 2areas of stenosis in the right branch of the pulmonaryartery.

Venous angiocardiography subsequently con-firmed the presence of a total anomalous pulmonaryvenous return to the right atrium by means of apersistent left superior vena cava, and demonstrateda moderate dilatation of the right pulmonary artery.The first portion of the right pulmonary artery waspoorly seen on the film because it was located overthe spine. No distinct area of stenosis could be seen.

Comment. This patient had multiple con-genital anomalies. Two sites of stenosis of thepulmonary artery were demonstrated bycatheterization. The continuous murmur waslocated directly over the point where thestenosis of the right main pulmonary artery wasdemonstrated. The quality of the murmur wasquite unlike that of a venous hum, which hasbeen occasionally described over the area ofinsertion of anomalous pulmonary veins intothe right atrium or venae cavae.15 The rise in theintensity of the murmur a short interval afterthe first heart sound and the decrease in inten-sity during diastole correspond to the phasic

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TABLE 1-Catheterization Findings in Case 1

Left axillary vein........Superior vena cava*......Inferior vena cava........Right atrium*............Right ventricle*..........Main pulmonary artery*..Left pulmonary artery....Proximal right pulmonary

artery..................Distal right pulmonaryartery ..................

Pulmonary artery wedge..

Femoral artery...........Arterial oxygen satura-

tion ....................

Oxygencontent,

ml./100 ml.

16.015.711.513.513.613.613.9

13.8

14.0

13.9

77%

TABLE 2.-Catheterization Findings in Case 2

Pressure,mm. Hg

9 (mean)82/1182/2989/29

39/13

19/814 (mean)

Superior vena cava.......Inferior vena cava........Right atrium*............Right ventricle*..........Main pulmonary artery...Right pulmonary artery.Left pulmonary artery....

Femoral artery...........Arterial oxygen satura-

tion....................

Oxygencontent,

ml./100 ml.

13.212.512.013.513.913.3

15.0

91%

Pressure,mm. Hg

2 (mean)20/321/621/615/8

115/60

* These figures represent the average of 2 or moresamples.

TABLE 3.-Catheterization Findings in Case 3

* These figures represent the average value of 2or more samples.

I 2 S&.M 2

PULUONICARE A

EKO

FIG. 2. Case 2. Phonocardiogram taken in the pul-monary area showing the systolic ejection murmurand the diastolic murmur. The location of the heartsounds is shown by the numbers 1 and 2.

pressure changes recorded from the pulmonaryartery at the point of the demonstrated steno-sis.

Case 2. R.W., a 7-year-old boy, was first seen atthe Stanford University Hospital at the age of 6months, when he was noted to have a thrill at thefourth intercostal space along the left sternal borderand a loud systolic murmur in the same area. At thepulmonic area a systolic murmur of grade III in-tensity was followed by a short early diastolic mur-mur. While not a truly continuous murmur, severalobservers believed it so on the basis of auscultationalone, and a diagnosis of a probable patent ductusarteriosus was made. At the age of 2 years a thora-cotomy was performed at this hospital. The surgeondiscovered a small ligamentum arteriosum andligated it. There were no changes in the murmursfollowing this surgery.The patient remained asymptomatic and again

entered the hospital at the age of 71f years for fur-ther studies. He appeared well. His brachial blood

Superior vena cava.......Inferior vena cava........Right atrium*............Right ventricle*..........Main pulmonary artery...Left pulmonary artery....Right pulmonary artery.

Femoral artery...........Arterial oxygen satura-tion....................

Oxygencontent,

ml/100 ml.

10.611.012.612.312.6

12.2

14.0

87%

Pressure,mm. Hg

7 (mean)42/838/1041/920/8

94/50

* These figures represent the average of 2 or moresamples.

pressure was 98/60 mm. Hg. There was a systolicthrill and a harsh grade IV systolic murmur loudestat the left sternal border in the fourth intercostalspace. In the pulmonic area a grade III systolicmurmur was immediately followed by a grade IIIdiastolic murmur of diminishing intensity duringdiastole.

Routine laboratory studies were normal. Theelectrocardiogram revealed somewhat high QRSvoltage over the precordium but was otherwise nor-mal for a child of 7 years. X-rays showed somewhatprominent right and left ventricles and a large mainpulmonary artery. The pulmonary vasculature wasprominent. A phonocardiogram (fig. 2) revealed aloud crescendo systolic murmur compatible with ashunt through an interventricular septal defect. Atthe pulmonic area a systolic ejection murmur withits maximum intensity in midsystole was recorded.It was followed by a murmur of lesser intensity con-tinuing into diastole.

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STENOSIS OF PULMONARY ARTERY BRANCH

RIGHT MAINPULMONARY ARTERY ±PULUC

LEFT , RIGHT

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FIG. 3. Pressures obtained at cardiac catheterization in case 3. There is a rise in pressure betweenthe right pulmonary artery and the main pulmonary artery indicating the site of the stenosis.

Carliac catheterization wvas performed and indi-cated a ventricular septal defect with a small left-to-right shunt (table 2). During the procedure it wasnoted that the catheter passed in an unusual fashionposteriorly and laterally into the left pulmonaryartery and that there was a definite sudden pressuredrop in this portion of the left main pulmonaryartery (table 2).

Comment. The ventricular septal defect wasthe principal cardiac lesion in this case. Thestenosis of the left pulmonary artery was anincidental finding, and was probably the causeof the systolic and diastolic murmur closelyresembling a continuous murmur that hadpreviously led to a surgical exploration for apatent ductus arteriosus. Since the murmur waspresent before the thoracotomy, it is unlikelythat the operation produced the narrowing ofthe pulmonary artery. It is of interest thatpulmonary hypertension was not present.

Case 3. J.C., a 5-year-old boy, was examined inApril 1956. A heart murmur had been heard at birthbut he had developed normally and had experiencedno symptoms except questionable easy fatigability.He looked well. His brachial blood pressure was

100/60 mm. Hg. There was a slight precordial bulgeof the left chest wall parasternally. A thrill and agrade IV systolic murmur were present along theleft sternal border in the third and fourth intercostalspaces. The pulmonic second sound was loud.

Routine laboratory studies were not unusual. Theelectrocardiogram revealed a pattern consistent withincomplete right bundle-branch block, right ven-tricular hypertrophy, and abnormal P waves. X-raystudies revealed a prominent main pulmonary artery,increased pulmonary vascularity, and enlarged rightand left ventricles.

Cardiac catheterization demonstrated an atrialseptal defect with a left-to-right shunt (table 3) andmild pulmonary hypertension. In addition, therewas a definite drop of systolic pressure in the rightpulmonary artery 4 to 5 cm. beyond the bifurcationof the main pulmonary artery (fig. 3) indicating thepresence of a stenosis of the right pulmonary artery.

Mild arterial unsaturation was thought to be due tohvpoventilation a(ccompany ing the tribromoethanol(Avrertin) anesthesia.

Comment. The atrial septal defect was theprincipal lesion in this patient. Since a con-tinuous murmur was not present, there appearsto be nothing in the clinical findings that wouldenable one to make the diagnosis of stenosis ofthe right pulmonary artery.

DIscussIoN

The exact nature of such stenotic lesionsinvolving the pulmonary artery distal to thevalve is obscure because of the rarity of suchlesions and the paucity of descriptions of theirpathologic anatomy. Partial obstruction bythrombosis or embolus or a congenital develop-mental defect are the 2 most likely possibilities.

Partial occlusion by thrombosis, secondaryto trauma of the chest wall, of the main pul-monary artery in an adult has been describedby Dimond and Jones.16 The resulting clinicalpicture resembled that of pulmonary stenosiswith an intact septum. Pulmonary thrombosisor embolism with partial recanalization couldresult in single or multiple areas of narrowingof the branches of the pulmonary artery; sinceresorption of an occluding thrombus or embolusmay be variable, complete restitution of thelumen may occur or a residual area of stenosismay remain.'7 18 Saphir,19 for example, hasconcluded that small bands or valvelike struc-tures found occasionally in the pulmonaryartery represent vestiges of resorbed muralthrombi. Partially resorbed multiple thrombior emboli similar to those illustrated in Moller'spaper' could easily give the curious rounded orscalloped appearance demonstrated so clearlyin the angiograms of one of Arvidsson's pa-

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ELDRIDGE, SELZER, ANI) HULTGREN

tients'2 (case 1). However, it seems unlikelythat this was the etiology of the stenotic lesionspresent in our patients, for there was no clinicalhistory compatible with pulmonary thrombosisor embolism. Although such a process couldhave occurred during intra-uterine develop-ment, there are no descriptions in the literatureof any such lesions in the fetus or newborn.A developmental, congenital defect of the

pulmonary artery seems more likely. The occur-rence in childhood, the association with othercongenital cardiac lesions, and the absence ofany evidence of an acquired etiologic process allsuggest this possibility. S0ndergaard' suggestedthat the cause of stenosis of the pulmonaryartery at its bifurcation was due to the incorpo-ration of the pulmonary artery by the tissue ofthe closing ductus arteriosus (the Skodaictheory). This theory was first suggested byCraigie20 and later supported by Skoda toaccount for the origin of the adult type ofcoarctation of the aorta. Although one of thepatients in the present report (case 2) probablyhad a single area of stenosis at the bifurcation,such a theory would not explain the stenosis ofthe more peripheral branch of the right pul-monary artery (case 3) and the multiplestenoses observed in case 1.The only detailed report of a patient with

stenosis of the main branches of the pulmonaryartery in which a complete autopsy study wasdescribed is by Oppenheimer.6 In that 17-month-old child, the right pulmonary arteryand the lower branch of the left pulmonaryartery were constricted by thickened intimaltissue beneath which were extensive depositsof calcium in the media. The right pulmonaryartery at the stenotic area was completely en-circled by a ring of calcium. Calcification wasalso present in the media of the aorta. Fiveother instances of calcification of the pulmonaryartery were described but in none of these pa-tients was there any narrowing of the pul-monary arteries. The author suggested acongenital origin for these lesions. It is possiblethat a similar process is present in our patients,but a careful review of the x-ray studies re-vealed no evidence of calcifications either inthe pulmonary arteries or aorta.The question of the proper terminology of

such lesions of the pulmonary artery should beconsidered. S0ndergaard' suggested the term"coarctation of the pulmonary artery" becauseof the resemblance in structure and, as hebelieved, in origin to the adult variety of aorticcoarctation. This term would be desirable if thestenosis were isolated and located near thebifurcation and if the Skodaic theory were theproper explanation for such a lesion. Many ofthese lesions are multiple, however, and theymay be located at various points in the pul-monary artery. Furthermore, the Skodaictheory of the origin of the adult variety ofcoarctation of the aorta has been attacked forcompelling reasons by Edwards and associates,21and the possibility of such a process being thecause of a stenosis of the pulmonary artery isonly conjectural. Oppenheimer6 referred to thelesions as "partial atresia of the main branchesof the pulmonary artery." This is a poor termbecause atresia means "without a lumen,"hence it cannot be partial. Considering the factthat such areas of narrowing may occur atmany sites in the pulmonary artery distal tothe valve, that they may be multiple, and thattheir exact origin is still unknown, it might bebest to refer to them as "stenoses of the pul-monary artery" specifying the area involved,i.e., "stenosis of the main pulmonary artery,stenosis of the right or left pulmonary artery,etc."

Arvidsson and co-workers'2 suggested thatthis anomaly may be the cause of hypertensionin the main pulmonary artery. The fact thatin 2 of their patients with multiple stenoses noother anomaly was demonstrated suggests thatthis may be the case; but such a relationship isby no means certain. It should be pointed outthat abundant clinical and experimental evi-dence is available to show that even totalocclusion of 1 main pulmonary artery does notincrease the resistance enough to elevate pulmo-nary arterial pressure if the flow is not increasedand if the pulmonary vascular bed is normal.Localized narrowing would be even less likely tocause pressure rise per se, unless both brancheswere involved. Furthermore, in 2 cases of the 3reported here, in which pulmonary hyperten-sion was present, there existed another congen-ital defect (anomalous venous return and ven-

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STENOSIS OF PULMONARY ARTERY BRANCH

tricular septal defect) which in itself may beassociated with pulmonary hypertension.'5The physiologic effects of stenosis of a main

branch of the pulmonary artery are not readilyapparent unless a relationship to pulmonaryhypertension can be demonstrated. No meas-urements of relative blood flow to the lungshave been made, although on theoretic groundsone might expect some changes from thenormal.

In one of the reported cases the stenotic areawas dilated surgically7 and clinical improve-ment was thought to have taken place.Whether surgical intervention for the solepurpose of dilating a localized stenosis of apulmonary arterial branch is justified, cannotbe answered on the basis of information athand. One is inclined, however, to lean towardthe view that this is a benign, clinically insig-nificant lesion that would not justify thoracot-omy under ordinary circumstances.

Unfortunately, there are no distinctiveclinical signs of the presence of stenosis of themain branches of the pulmonary artery, unlessa continuous murmur be present. In order todetect such lesions during cardiac catheteriza-tion, both branches of the pulmonary arteryshould be entered and withdrawal tracings re-corded from each branch. Since continuousmurmurs are produced by this lesion only whenthe degree of stenosis is severe, one mightexpect the murmur to be extinguished or sud-denly changed in intensity when the catheter ispassed through the area of narrowing.The most apparent significance of stenosis of

a main branch of the pulmonary artery lies inits relationship to the origin of continuousmurmurs. Continuous, ductus-like murmurshave been observed in 3 cases: in 1 patient ofArvidsson12 and in 2 reported here. The locationof the continuous murmur over the thorax atthe site of the stenosis and the appearance ofthe phonocardiogram suggest strongly that themurmur originated at the stenosis. It wasthought, however, that in order to prove thisrelationship beyond doubt, an examination ofthe nature of the pressure gradient across thestenotic areas should be made and an experi-mental reproduction of such a murmur wouldhave to be accomplished.

Several conditions must be fulfilled for acontinuous murmur of this sort to be produced.There must be narrowing of the blood channeland sufficient flow through the constricted areato produce turbulence during both systole anddiastole. In order to have flow in diastole ofsufficient volume and velocity to produce thisturbulence, a reservoir of blood under pressuremust exist proximally to the constriction. Theseconditions are met in the case of patent ductusarteriosus, peripheral and pulmonary arterio-venous fistulas, and in some of the moreunusual arteriovenous communications. Theyare also met in the case of stenosis of a pul-monary artery branch where the proximalpulmonary arterial bed acts as the reservoirunder pressure.The presence of a sufficient volume and

velocity of blood flow to produce turbulencecan best be demonstrated by showing a pressuregradient between the areas proximal and distalto the narrowed area. Such a gradient can easilybe demonstrated, both in systole and diastole,between the aorta and the pulmonary artery ina typical case of patent ductus arteriosus withcontinuous murmur. Furthermore, it has beenshown that when pulmonary hypertensiondevelops,- leading to an obliteration of thegradient in diastole or in both systole and dias-tole, there is disappearance of the diastoliccomponent of the murmur in the first instanceand total disappearance of the murmur in thesecond.24 Figure 4 shows the gradients of pres-sure for 1 cardiac cycle across the areas ofstenosis in the 3 patients presented in this re-port. The pressure gradients were constructedfrom an analysis of the pressure curves ob-tained proximally and distally to the area ofpressure drop. Significantly, the 2 patients withcontinuous murmurs showed a gradientthroughout the entire cycle, whereas the thirdpatient, in whom only a systolic murmur wasobserved, showed a pressure gradient only insystole. This is offered as evidence that thecontinuous murmur heard in 2 of our casesoriginated at the site and was caused by thestenosis of the pulmonary artery. It also ex-plains why such a murmur is present in somecases only.

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ELDRIDGE, SELZER, AND HULTGREN

Ms

20-

CASE

0SECONDS t

QRs

.20 .40 0 .20 .40 hQGA ORS

CASE 2

14|0 <

0 .20 .40 0 .20 .40 0SECONDSt t

asmsas

50CASE 3

to3

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6 io 4o d io 4o 6SECONDSt f- GAC GSS

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FIG. 4. Graph of pulmonary artery pressuresproximal (upper curve in each case) and distal (lowercurve in each case) to area of stenosis, showing pres-sure gradients across the stenosis during the heartcycle.

EXPERIMENTAL STUDYThe experimental part of our study was performed

in acute experiments on dogs anesthetized with intra-venous pentobarbital. A special constricting clamp22was placed around a large branch of the pulmonaryartery and graded constriction applied. Auscultationof the constricted area revealed only a faint systolicmurmur. In the next experiment the constrictingclamp was placed around the thoracic aorta andgraded constriction applied. One could observe andregister phonocardiographically a soft systolic mur-mur with mild constriction, a loud systolic murmurwith more severe constriction, then a continuousmurmur, first with a faint diastolic phase, and uponfurther constriction a loud murmur throughout thecardiac cycle. A similar series of experiments, per-formed independently, was recently reported byMyers and associates.23 In the final experiment itwas thought that if a branch of the pulmonary ar-tery could be constricted and the flow through itincreased, a situation similar to that in the thoracicaorta would arise. In this experiment the clamp wasplaced around the superior branch of the left pul-

monary artery (the main branch in the dog is tooshort before its bifurcation to place a clamp aroundit) and graded constriction was applied after com-pletely clamping the right pulmonary artery. Inthis experiment mild constriction of the left pul-monary artery produced a systolic murmur and moresevere constriction produced a continuous murmursimilar to that of aortic constriction (fig. 5).The experimental reproduction of a continuous

murmur at the site of constriction of a branch of thepulmonary artery fully confirms the clinical impres-sion that the murmurs observed in 2 of our patientswere due to the stenosis of the pulmonary artery.

The fact that stenosis of the left branch ofthe pulmonary artery may produce a murmurindistinguishable in quality and location fromthat of a typical case of patent ductus arteriosusis of a considerable practical importance. Thishas been dramatically demonstrated in ourcase 2, in whom a thoracotomy was performedunder the mistaken diagnosis of patent ductusarteriosus. Presently, surgical treatment ofpatent ductus arteriosus has reached such adegree of safety that in most quarters all casesare being operated on regardless of whetheror not the lesions are dynamically significant.Furthermore, the presence of a typical con-tinuous murmur is usually considered pathog-nomonic for this lesion, so that few casesundergo special procedures to confirm thediagnosis. The possibility of other conditionscausing continuous murmurs over the thorax

I. MILDCONSTRICTIONSYSTOLICMURMUR

2.MODERATECONSTRICTION

CONTINUOUS ;MURMUR WITHaSYSTOLIC.1ACCENTUATION

3. MORE LAM:1MCONSTRICTIONTHAN IN 2.

CONTINUOUSrn iiMURMUR

FIG. 5. Phonocardiographic record of murmursproduced during experimental constriction of dogpulmonary artery.

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It

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STENOSIS OF PULMONARY ARTERY BRANCH

should constantly be kept in mind. A reviewv ofsuch lesions has been recently published byDavis and associates.25 To the list of such con-ditions should now be added stenosis of a mainbranch of the pulmonary artery.

SUMMARYThree patients, all with other congenital

cardiac malformations, were shown by cardiaccatheterization to have stenosis of a mainbranch of the pulmonary artery. Two of the 3patients exhibited continuous ductus-like mur-murs heard at the chest wall over the site of thestenosis. The origin of the murmur at the site ofthe constriction was proved by the demonstra-tion of pressure gradients throughout thecardiac cycle at the site of constriction and bythe experimental production of continuousmurmurs in dogs in which partial constrictionof a pulmonary arterial branch was accom-plished. Conditions necessary for the produc-tion of such murmurs are discussed. Theimportance of these observations in the dif-ferential diagnosis of congenital heart disease isemphasized and demonstrated by the fact thatone of the patients underwent thoracotomyunder the mistaken diagnosis of patent ductusarteriosus.

SUMMARIO IN INTERLINGUAIn tres patientes, catheterisation cardiac

serviva a monstrar le presentia de un stenosisdel branca major del arteria pulmonar. Inomnes altere congenite malformationes car-diac esseva presente. Duo del tres patientesexhibiva continue murmures, simile al mur-mure de patente ducto arteriose, al parietethoracic supra le sito del stenosis. Le originedel murmure al sito del constriction essevaprovate per le demonstration de gradientes depression durante le integre cyclo cardiac alsito del constriction e per le production ex-perimental de continue murmures in canes inque constriction partial del branca pulmono-arterial habeva essite effectuate. Es discutitele conditiones necessari pro le production detal murmures.Es sublineate le importantia de iste obser-

vationes in le diagnose differential de con-genite morbo cardiac. Un illustration multo

significative se vide in le facto que un del pa-tientes hic reportate esseva subjicite a thora-cotomia in consequentia del diagnose erroneede patente ducto arteriose.

ADDENDUMSince this paper was submitted for publication, 2

additional patients with stenosis of a branch of thepulmonary artery have been studied in this labora-tory.

Case 4. A 4-year-old girl without symptoms inwhom a heart murmur had first been heard at theage of 9 months. Positive physical findings includeda prominent apex impulse, a loud high-pitched apicalsystolic murmur, and in the aortic area a systolicmurmur of lower frequency, which was well trans-mitted to the neck.The electrocardiogram showed high voltage com-

plexes over V4 to V6 and was thought to be suggestiveof left ventricular hypertrophy. X-rays were inter-preted as showing moderate left ventricular enlarge-ment, slight right ventricular enlargement, sandprominent proximal pulmonary arteries.

Cardiac catheterization revealed no intracardiacshunts. The arterial oxygen saturation was normal.Pulmonary artery pressure tracings, however, re-vealed a sharp pressure drop in the right pulmonaryartery distal to the bifurcation of the main pulmo-nary artery. The following measurements were ob-tained: right ventricle 38/0 mm. Hg; main pulmo-nary artery 36/16 mm. Hg; left pulmonary artery34/15 mm. Hg; right pulmonary artery 24/14 mm.Hg. Analysis of the pressure tracings revealed agradient only during systole.The diagnosis was aortic stenosis plus stenosis of

the right pulmonary artery.Case 5. A 51 -year-old girl without symptoms in

whom a cardiac murmur was noted at the age of 2weeks. There was a slight left parasternal lift. Athrill was noted in the left second and third inter-costal spaces parasternally. A moderately loud holo-systolic murmur was heard in the same area. Inaddition a louder systolic ejection murmur was heardin the midsternal line at the level of the secondintercostal space. This murmur began after the firstsound, reached its peak intensity just before mid-systole and decreased in intensity during late systole.The electrocardiogram was normal. Cardiac

catheterization revealed a rise of 1.0 ml. per 100 ml.in oxygen content in the right ventricle, indicatingthe presence of an interventricular septal defect.There were sharp pressure drops in both right andleft pulmonary arteries at their juncture with themain pulmonary artery. The pressures were: rightventricle 28/1 mm. Hg; main pulmonary artery28/8 mm. Hg; left pulmonary artery 19/8 mm. Hg;right pulmonary artery 20/7 mm. Hg. Analysis ofthe pressure tracings again revealed pressure gradi-ents only in systole. The maximum gradient on the

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ELDRIDGE, SELZER, AND HULTGREN

right side occurred 0.18 to 0.22 second after theQRS complex. This corresponded very closely intime to the point of maximum intensity of the mur-mur recorded phonocardiographically over the mid-line at the level of the second intercostal space.

This is the only patient of this series who exhibitedbilateral stenosis of the pulmonary artery branches.Comment. Several additional reports of this condi-

tion have come to our attention. Powell and Hiller26described a 5-year-old child in whom were foundpressure gradients between the main pulmonaryartery and its branches, as well as angiographic evi-dence of stenosis of both branches at the bifurcationof the main pulmonary artery. This patient exhibiteda continuous murmur in the pulmonic area. Hodges27discussed the catheterization findings of a patientwho had low pressures in both pulmonary arterybranches in addition to a pulmonic valve stenosis.Coles and Walker28 report a child, 26 months old,in whom angiography demonstrated a narrowing ofthe initial portion of each pulmonary artery branch,and in whom cardiac catheterization gave evidenceof pulmonary branch stenosis as well as pulmonaryvalve stenosis. Figley29 reported a patient withstenosis of the pulmonary artery branches at 3 sites.These were shown by angiography and pressuregradients were found at cardiac catheterization.

Nineteen additional cases have been studied butnot reported by 4 other workers, T. Schnabel, B.Jonsson, A. Leatham, and M. Figley. In several ofthese other cardiac malformations were also present.Three of these cases exhibited continuous murmurs.

It becomes apparent from the number of patientsdiscovered by these few investigators over a fairlyshort period of time that stenosis of the pulmonarybranches is not a rare condition. The awareness ofits existence will probably lead to many more suchcases being discovered.

REFERENCES1 BREDT, H.: D)ie AMissbildungen des menschlichen

Herzens. Ergebn. d. alg. Path. u. path. Anat.30: 77, 1936.

2 MONCKEIBERG, G.: Henke-Lubarsch: Handbuchder Spez path. Anatomie u. Histologie, Vol. 2,Herz und Gefasse, Berlin, Julius Springer, 1924.

3SCHWALBE, E.: Morphologie der Missbildungen,Part III, Jena, Gustav Fischer, 1909, p. 426.

4 FURST, L.: Gerhardt's Handb. d. Kinderkrank-heiten, 3, II, Tuebingen, 1878, p. 583.

5 MAUGAR: R6&. p6riod. Soc. de M&d. d. Paris 13:74, 1802.

6 OPPENHEIMER, E.: Partial atresia of the mainbranches of the pulmonary artery occurring ininfancy and accompanied by calcification of thepulmonary artery and aorta. Bull. Johns Hop-kins Hosp. 63: 261, 1938.

ScHUMAKER, H., AND LURIE, P.: Pulmonaryvalvulotomy. J. Thoracic Surg. 25: 173, 1953.

8 S0NDERGAARD, T.: Coaretation of the pulmonaryartery. Danish Med. Bull. 1: 46, 1954.

9 KJELLBERG, S., MANNHEIMER, E., RUDHE, U.,AND JONSSON, B.: Diagnosis of congenital heartdisease. Chicago, The Year Book Publishers,Inc., 1955.

10 MULLER, T.: A case of peripheral pulmonarystenosis. Acta paediat. 42: 390, 1953.

ARVIDSSON, H.: Paper read at the meeting ofSvensk Forening For Medicinsk Radiologi.September 25, 1954.

12 -, KARNELL, J., AND M6LLER, T.: Multiple steno-ses of the pulmonary arteries associated withpulmonary hypertension, diagnosed by selectiveangiocardiography. Acta radiol. 44: 209, 1955.

13 BOUCHARD, F., AND CORNU, C.: Etude des courbesde pressions ventriculaire droit et art6riellepulmonaire dan les retrecissements pulnio-naires. Arch mal. coeur 47: 417, 1954.

14 SOBIN, S., CARSON, M., JOHNSON, I., AND BAKER,C.: Pulmonary stenosis with intact ventricularseptum: Isolated valvular stenosis and valvularstenosis associated with interatrial shunt. Am.Heart J. 48: 416, 1954.

15 KEITH, I., ROWE, R., VLAD, P., AND O'HANLEY, J.:Complete anomalous pulmonary venous drain-age. Am. J. Med. 16: 23, 1954.

16 DIMOND, E., AND JONES, T.: Pulmonary arterythrombosis simulating pulmonary valve steno-sis with patent foramen ovale. Am. Heart J.47: 105, 1954.

17 M0LLER, P.: Studien ilber embolische und autoch-thone thrombose in der arteria pulmonalis.Beitr. path. Anat. 71: 27, 1922.

18 LAUFER, S., AND GRAY, I.: Organized thrombusoccluding a main pulmonary artery: Report oftwo cases. New England J. Med. 254: 893, 1956.

19 SAPHIR, 0.: Bands and ridges in the pulmonaryartery. Their relation to Ayerza's disease. Arch.Path. 14: 10, 1932.

20 CRAIGIE, D.: Instance of obliteration of the aortabeyond the arch illustrated by similar cases andobservations. Edinburgh M. Surg. J. 56: 427,1841.

21 EDWARDS, J., CHRISTENSEN, N., CLAGETT, T.,AND McDONALD, J.: Pathologic considerationsin coaretation of the aorta. Proc. Staff Meet.,Mayo Clinic 28: 324, 1948.

22 SELZER, A., LEE, R., GOGGANS, W., AND GERBODE,F.: The effect of ouabain upon the maximumperformance of the normal dog's heart. StanfordM. Bull. 11: 253, 1953.

23 MYERS, J., MURDAUGH, H., MCINTOSH, H., ANDBLAISDELL, R.: Observations on continuousmurmurs over partially obstructed arteries.Arch. Int. Med. 97: 726, 1956.

24 HULTGREN, H., SELZER, A., PURDY, A., HOLMAN,E., AND GERBODE, F.: The syndrome of patent

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STENOSIS OF PULMONARY ARTERY BRANCH

ductus arteriosus with pulmonary hyperten-sion. Circulation 8: 15, 1953.

25 DAVIS, C., JR., DILLON, R., FELL, E., AND GASUL,B.: Anomalous coronary artery simulatingpatent ductus arteriosus. J. A. M. A. 160: 1047,1956.

26 POWELL, M., AND HILLER, H.: Pulmonary coarc-tation. M. J. Australia 1: 272, 1955.

27HODGES, F.: Presented at Panel Discussion onCardiovascular Diseases. Meeting of AmericanMedical Association, Atlantic City, New Jersey,June 8, 1955.

28 COLES, J., AND WALKER, W.: Coarctation of thePulmonary Artery. Am. Heart J. 52: 469, 1956.

29 FIGLEY, M.: The expanding scope of cardiovascu-lar radiology. Am. J. Roentgenol. 76: 721, 1956.

9.Campbell, M., and Baylis, J. H.: The Course and Prognosis of Coarctation of the Aorta. Brit.Heart J. 18: 475 (Oct.), 1956.This paper attempts to determine the course and prognosis of coarctation of the aorta, based

upon a study of 130 patients. Sixty per cent were first seen when under 20 years and only 14 percent when over 30. Eighty patients have been followed 5 years or more. Twenty-eight were seenin the first decade. Three patients died, 1 with recurrent bouts of failure, 1 of aortic stenosis andrheumatic heart disease, and 1 of failure after being cured of bacterial endocarditis. The livingpatients were regarded as normal by their parents. Fifty were seen in the second decade. Onedied, probably of heart failure. The clinical course was usually uneventful in this decade althoughthe blood pressure rose slowly. Thirty-seven were seen in the third decade. The blood pressurewas now stabile. Five died, 1 of ruptured aorta, 1 of cerebral hemorrhage, and 3 of congestivefailure. Seventeen were seen in the fourth decade. Two died, 1 with aortic regurgitation and 1with aortic stenosis. Only a few were seen in the fifth and sixth decades.About 25 per cent had aortic regurgitation and 5 per cent aortic stenosis. Large hearts and elec-

trocardiographic evidence of left ventricular strain were uncommon in the absence of valvulardisease. Congestive heart failure was the commonest cause of death. Below the age of 30, deathwas more commonly due to aortic rupture, bacterial endocarditis (with or without aortitis), andintracranial hemorrhage.

Because of the possibility of sudden and unexpected death, operation was advised for mostchildren. Aortic regurgitation was an added reason for urging operation.

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FREDERIC ELDRIDGE, ARTHUR SELZER and HERBERT HULTGRENContinuous Murmurs over the Chest

Stenosis of a Branch of the Pulmonary Artery: An Additional Cause of

Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 1957 American Heart Association, Inc. All rights reserved.

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