Severe Pulmonic Stenosis with IntactVentricular Septum and Right

Aortic ArchBy WALTER J. GAMBLE, M.D., AND ALEXANDER S. NADAS, M.D.

IT HAS BEEN assumed that patients withpulmonic stenosis and a right aortic arch

practically always have a ventricular septaldefect as well. As a matter of fact, in thedifferential diagnosis of pulmonic stenosis andright-to-left shunt, the presence of a rightaortic arch often was taken as conclusiveevidence of a tetralogy of Fallot-type lesion. -3A review of the literature revealed among

several hundred, one patient with the pre-sumptive diagnosis of pure pulmonic steno-sis, who had a right aortic arch.4' 5 This diag-nosis was not made preoperatively, and theevidence is exclusively a surgical one, basedon the findings at closed valvulotomy, and ona favorable postoperative course. Taussig re-ported having seen one other patient withvalvular pulmonarv stenosis, intact ventricularseptum, and a right aortic arch, but did notgive details."*The three cases to be reported have been

studied and operated upon at the Children'sHospital Medical Center between January1960 and June 1963. Conclusive evidence ispresented that all three had severe pulmonicstenosis, a right aortic arch, and intact ven-tricular septum.

Case ReportsCase 1A heart murmur was discovered in this

From the Sharon Cardiovascular Unit of the Chil-dren's Hospital Medical Center and the Departmentof Pediatrics, Harvard Medical School, Boston, Mas-sachusetts.

Supported in part by Grant HTS 5310 from theNational Heart Institute, U. S. Public Health Service.

*Since this article was submitted for publication,J. L. Bressie has reported a well documented casewith pulmonary stenosis, right aortic arch, and intactventricular septum. No right-to-left shunt was dem-onstrated. Brit. Heart J. 36: 154, 1964.

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boy (R.M.) at 2 months of age, when he hadpneumonia. At 24 months of age, when first seenat the Children's Hospital Medical Center, hehad a grade V, long systolic, ejection murmur.His electrocardiogram showed an axis of +1200with right ventricular hypertrophy. Cardiac flu-oroscopy showed the heart to be of normal size,but the configuration was consistent with con-centric right ventricular hypertrophy. The aorticarch was noted to be on the right, and an obliquefilling defect in the esophagus was interpreted asrepresenting an anomalous left subclavian ar-tery passing from the right, behind the esophagusupwards to the left (fig. 1A).By 6 years of age, a slight decrease in exercise

tolerance was reported, and slight cyanosis andclubbing were noted.

At 11 years of age, there were definite cyanosisand clubbing. A precordial bulge was noted. Thesecond heart sound was single and moderatelydecreased in intensity. A grade III, long, ejectionmurmur was heard loudest at the third left inter-space, and was transmitted to the entire; pre-cordium. The electrocardiogram showed an axisof + 1150, "P pulmonale," and severe right ven-tricular hypertrophy (fig. 2, top). A chest x-rayshowed possible slight cardiac enlargement (fig.3A). The main pulmonary artery region was con-cave.

Cardiac catheterization was carried out at11 2/12 years (table 1) and showed infundibularpulmonic stenosis. Right ventricular pressure ex-ceeded systemic pressure. A right-to-left shuntwas demonstrated at the atrial level. No shunt atthe ventricular level could be demonstrated bymeans of oxygen saturation data, cineangiograms,nor by dye-dilution curves, with injection of indo-cyanine green into the right ventricle and sam-pling from the brachial artery.

At 11 6/12 years, during total cardiopulmonarybypass, a patent foramen ovale was sutured.Through a ventriculotomy, the infundibular ste-nosis, composed of fibromuscular tissue and dia-phragmatic in nature with two small holes 3 and4 mm. in diameter, respectively, was resected.The pulmonary valve was not stenotic. No ven-tricular septal defect was noted. The patient re-quired digitalis postoperatively, and made an un-

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PULMONIC STENOSIS

Figure 1Vascuilar anomaly as demonstrated by bariuim stwallow, showinig filling defect caiused bul aber-rant left sutbelavian artery posterior to esophaguis. A. Case 1. B. Case 2.

eventful recovery. He has been asvymptomaticsince surgery.

At 14 4/12, (23 years after operation) he wasrestudied by cardiac catheterization. Ausculta-tion revealed no significant murmurs. The heartsounds were of normal intensity. The secondheart sound demnonstr-ated a normal variationi insplitting with respiration. Right ventricular sys-tolic pressur-e was 22 mm. Hg. No significantpressure gradient was fouind across the right ven-tricular outflow and pulmonary valve regions. Noshunting of blood couild be detected by oxygensaturation data, cineangiography, or hydrogenappearance times.

Case 2A murmuir and enilarged heart were discovered

on this patient (P.R.) at 10 weeks of age, whenshe was hospitalized for croup and pneumonia. Agrade III to IV systolic murmur was heard wvhenshe was first seen at the Children's Hospital Med-ical Center at 2 2/12 years. No evidence ofcyanosis was noted. An electrocardiogram showedan axis of +70°, rSR' pattern in the riglht pre-cordial leads, and voltages within normal limits.Cardiac fluoroscopy showed considerable enlarge-ment, xvith right ventricuilar configuration. Anaberrant left subclavian artery was noted (fig.iB).By 6 9/12 years, slight decrease in. exercise

tolerance was reported. At 9 3/12 years "P pul-monale" and definite right ventricular hyper-trophy appeared.Circelation, Volume XXXII, Julo! 1965

At 10 9/12 years she wvas fouind to have a sin-gle second heart sounid aind a grade IV, long, sys-tolic murmur at the second left interspace. Thiswas thouight to be pansystolic at the lower leftsternal border. Cardiac catheterizationi (table 1)demonstrated severe inftundibuilar pulmonic steno-sis anid a right aortic arch. No evidence of a veni-tricular septal defect cou-ld be found by oxygenisaturation data nior by cineangiography with mul-tiple injection.s into the left anid right ventricles.Right ventricuilar systolic pressuire ranged from175 to 225 imn. Hg systolic, well above systemicarterial pressture. There was a 45 to 50 mnmi. rise inpressure following prematur-e beats. Peripheralarterial blood wvas ftully satuirated.

At 11 8/12 years of age, wvith slightly increas-inig exercise intoleranice, she vas stubjected tosurgery. Slight, but definite tricuispid regurgita-tioIn was noted. During total cardiopulinonary by-pass with cold arrest, marked infundibuilar ob-struction consisting of fibromuscular tissue wasfoLund through a ventriculotomy and was excised.No valvular stenosis was noted. A careful in-spection of the ventricuilar septum, especially fromthe crista region xvell down to the tricuispid valve,revealed nio septal defect. The aorta was notedto be on the right. Her postoperative recovervwas uineven-tful. Six months later there was partialregression of the right ventricular hypertrophy byelectrocardiogram, with disappearance, of the "Ppulmonale." A chest x-ray showed reductioni inthe heairt size. Twelve months postoperatively theseconid heart soind xvas noted to show normal

11.5

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splitting. A grade II ejection murmur was heardat the second left intercostal space, and a gradeIII protodiastolic blow of pulmonic regurgitationwas heard at the lower left sternal border. Herexercise tolerance returned to normal. She nolonger fatigues easily.

Case 3

Shortly after birth this infant (R.N.) becameashen and cyanotic. No murmur was found,and chest x-ray was reported as normal. He wastransferred to the Children's Hospital MedicalCenter at 10 days of age. He was noted to beacyanotic at rest, but became dusky and bluewhen feeding. A tapping right ventricular im-pulse was felt. The second heart sound wasthought to be single. An inconstant click washeard at the lower left sternal border. A gradeII to III ejection murmur was heard at the secondand third intercostal spaces and transmitted downto the lower left sternal border. An electrocardio-gram, at 11 days of age, showed an axis of +1350and right ventricular hypertrophy, probably ab-normal for age. Cardiac fluoroscopy at 4 weeksshowed moderate cardiac enlargement, with rightventricular configuration (fig. 2C). The aorticarch was noted to be on the right, and the pul-monary vasculature was slightly decreased. Earoximetry showed 79 per cent saturation at rest,70 per cent on crying. At 632" months rather suddendeterioration, including increased cyanosis, dif-ficulty in feeding, and decreased exercise toler-ance, was noted. Examination disclosed markedcardiac enlargement, with a right ventricular im-pulse. The second sound was felt to be single. Agrade III ejection murmur was heard. The liverwas down 3T cm. An electrocardiogram showed amean frontal plane axis of +1350, increasedright ventricular hypertrophy, and "P pulmonale"(fig. 2, bottom).Cardiac catheterization (table 1) was carried

out at 7 months. This suggested severe infundib-ular and valvular pulmonic stenosis with rightventricular systolic pressure well above systemiclevel, ranging from 140 to 160 mm. Hg. Oneand a quarter hours after intravenous digoxin theright ventricular systolic pressure was 210 mm.Hg. A right-to-left shunt was demonstrated at theatrial level by oxygen saturation data, ascorbicacid appearance times, and by selective cineangi-ography. No shunt could be demonstrated by thelatter two methods at the ventricular level. Cine-angiograms also suggested peripheral pulmonicstenosis at the junction of the right and mainpulmonary arteries.

Three days later the patient underwent trans-ventricular valvulotomy. The main pulmonary ar-tery was too small (4 to 5 mm. diameter) to per-mit a transarterial approach to the valve. The

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postoperative course was uneventful. He wasconitinued on digitalis. Two months postoperative-ly he was nuch more active, was feeding well,aind was virtually acyanotic. A gralde III to IVlong, systolic, ejection murmur xvas present, max-imum at the second left intercostal space, trans-

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mitted to the neck, lower left sternal border,and back. There was a suggestion of a faint,early protodiastolic blow. The second heart soundcontinued to be sinigle, and the heavy right ven-tricular impulse was still present. The electro-cardiogram showed some decrease in right ven-

Figure 3Preoperative x-rays of patients. A. Case 1, age 11 7/12 years. B. Case 2, 10 6112 years. C.Case 3, age 7/12 years.

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GAMBLE, NADAS

tricular potentials, and "P pulmonale" persisted.The chest x-ray showed the heart to be slightlylarger than preoperatively. Oxygen saturation, byear oximetry, had increased from 58 per cent be-fore operation to 86 per cent.

Discussion

There is nothing unusual in the family andprenatal history of the first and second pa-tients. The gestation of the third patient wascomplicated by bleeding during the secondmonth, and the mother was treated with oralnorethindrone, 5 mg. twice a day for 15 days.

There is a striking similarity between cases1 and 2. The same vascular anomaly wasnoted in both (fig. 1), suggesting an identicalembryologic mechanism in these two patients.The stenosis was proved to be infundibular,and was described as diaphragmatic at sur-gery in case 1, and appeared to be the sameby angiography in case 2. Because of the"blind" surgical approach, and less than com-pletely satisfactory angiograms, no definite lo-calization of the stenosis is possible in case3. The surgeon had the impression that theobstruction was at the valve level.

After surgery all three patients showedstriking clinical improvement. A virtual dis-appearance of the pressure gradient acrossthe right ventricular outflow tract was dem-onstrated at catheterization in patient 1 (ta-ble 1), a satisfactory drop in pressure inpatient 2 was implied in the postoperativeelectrocardiographic changes. Not enoughtime has elapsed to assess patient 3 in thisrespect.The clinical picture, particularly the ap-

preciable cardiomegaly (fig. 3) and the elec-trocardiogram (fig. 2), as well as the rightventricular pressure well above systemic ar-terial level, suggested preoperatively thepresence of pulmonic stenosis with an intactventricular septum. On the other hand, thediagnosis of pulmonary stenosis with ventricu-lar septal defect was implied by the findingof a right aortic arch. The murmurs were alldescribed as long ejection murmurs, maximalat the second (and third) left intercostalspace at the left sternal border. Cases 1 and2, in addition, also had pansystolic munnurs

at the lower left sternal border which hasbeen thought to reflect tricuspid regurgitation.At catheterization the behavior of the right

ventricular pressure pulse (absence of notchon upstroke) particularly after premature con-tractions was in favor of an intact ventricularseptum,8 while the deep peripheral cyano-sis and the finding of infundibular stenosisfavored the presence of a ventricular septaldefect. Indicator-dilution curves and cinean-giograms were the principal means by whichthe presence of a ventricular septal defect wasexcluded preoperatively. Surgical observa-tions and postoperative course substantiatedthe clinical assessment.The conclusion to be drawn from these

three cases is simply that infundibular andpossibly valvular pulmonic stenosis with anintact ventricular septum and a right aorticarch does indeed exist, although obviously itis very rare (less than 1 per cent of patientswith pure pulmonic stenosis). If clinical andphysiologic evidences point to the diagnosisof pulmonic stenosis with an intact ventricu-lar septum, the presence of a right aorticarch should not negate this conclusion. Ob-viously more than average care should betaken in these instances to exclude the pres-ence of a ventricular septal defect by meansof indicator-dilution curves and selective an-giograms.

SummaryThree cases with pulmonary stenosis, intact

ventricular septum, and right aortic arch arepresented, along with physiologic data. Twocases had infundibular stenosis and, in addi-tion, an aberrant left subelavian artery. Thethird case was thought to have valvular ste-nosis. All three patients have been successful-ly operated upon and show good clinical re-sults.

AcknowledgmentWe wish to thank Dr. Anna J. Hauck and Dr.

Robert E. Gross for making material available.

References1. WOOD, P.: Diseases of the Heart and Circulation.

London, Eyre and Spottiswoode, 1956. p. 343.Circulation, Volume XXXII, July 1965

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2. KEITH, J.: Heart Disease in Infancy and Child-hood. New York, Macmillan Company, 1958,p. 367.

3. NADAS, A. S.: Pediatric Cardiology. Philadelphia,W. B. Saunders Company, 1957, p. 451.

4. BROCK, R. C., AND CAMPBELL, M.: Valvulotomyfor pulmonary valvular stenosis. Brit. Heart J.12: 377, 1950.

5. CAMPBELL, M.: Simple pulmonary stenosis: Pul-monary valvular stenosis with a closed ven-tricular septum. Brit. Heart J. 16: 273, 1954.

6. TAUSsIG, H. B.: Congenital Malformations of theHeart, Vol. II. Cambridge, Harvard UniversityPress, 1960, p. 751.

7. HAMILTON, W. J., BOYD, J. D., AND MOSSMAN,H. W.: Human Embryology. Baltimore, Wil-liams & Wilkins, 1962.

8. HOFFMAN, J. I. E., RUDOLPH, A. M., NADAS, A. S.,AND GROSs, R. E.: Pulmonic stenosis, ventricu-lar septal defect, and right ventricular pressureabove systemic level. Circulation 22: 405,1960.

Stephen Hales 1677-1761Stephen Hales, the clergyman of Teddington (1677-1761), who was particularly

interested in plant physiology, made significant observations on the exchange betweenair and blood. Lavoisier later said that he was "the first who examined the problemfrom the quantitative point of view; he developed several devices, simple and easyto handle, in order to measure exactly the volume of air." In Vol. I of his StaticalEssays (1731), Hales described the apparatus making it possible to "take an estimateof the quantity of Air absorbed or fixed or generated by the breath of living animals."In Experiment CVII he measured the amount of air absorbed by breathing, and inExperiment CX he defined the site of absorption, marveling at the vast expanse of thealveolo-capillary membrane:

but some of the elasticity of air which is inspired is destroyed and that chieflyamong the vesicles . . . whence probably . . . acid spirits . . are conveyed in theblood which we see by an admirable contrivance spread into a vast expanse com-mensurate to a large surface of air from which it is parted by very thin partitions; sovery thin as thereby probably to admit the blood and air particles . . . within the reachof each other's attraction, whereby a continued succession of fresh air must be absorbedby the blood."-ANDRE COURNAND, M.D. Circulation of the Blood. Edited by Alfred P.Fishman, M.D., and Dickinson W. Richards, M.D., New York, Oxford University Press,1964, p. 38.

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WALTER J. GAMBLE and ALEXANDER S. NADASSevere Pulmonic Stenosis with Intact Ventricular Septum and Right Aortic Arch

Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 1965 American Heart Association, Inc. All rights reserved.

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