Diagnosis of Congenital Aortic Septal Defects

Description of Two Cases and Special Emphasis on a NewMethod which Allows an Accurate Diagnosis by Means

of Cardiac CatheterizationBy H. A. H. D'HEER, M.D. AND C. L. C. VAN NIEUWENHUIZEN, M.D.

Two cases of aortic septal defect, diagnosed in life by means of cardiac catheterization, are de-scribed. An explanation is given of a method, used in these cases, whereby a correct diagnosiscould be made before operation. Other methods for diagnosis and differential diagnosis are dis-cussed.

AMONG congenital cardiac anomalieswhich are now amenable to cardiacsurgery, none is more difficult to diag-

nose than aortic septal defect. All authors em-phasize the difficulty of making a correct diag-nosis before operation, and most of the caseswhich have been described were at first diag-nosed as patent ductus, ventricular septaldefect, truncus arteriosus or other anomalies.Among 34 cases described in the literature, 17were diagnosed at necropsy and 12 during op-eration for patent ductus. On only five caseswas the correct diagnosis made before opera-tion: by retrograde aortography in three andby catheterization in two cases.

In our two cases, the diagnosis was madewith certainty by the interpretation of thevarious positions of the catheter during cardiaccatheterization.

Since the application of surgery for the reliefof these anomalies depends upon correct diag-nosis, we will, after a short description of ourtwo cases, review the various methods of mak-ing a diagnosis and discuss the differentialdiagnosis.

CASE REPORTSCase 1. H. D., a 7 year old girl, was admitted

to our Cardiologic Department on March 13, 1953with the diagnosis of possible atypical patentductus. Cardiac anomality was first recognizedwhen she was 3 months old.

Her complaints were fatigue, exertional dyspneaFrom the Cardiologic Section of the department

for Cardiac Surgery. St. Anthonius Ziekenhuis,Utrecht, Netherlands.

and palpitation. There was no cyanosis and she didnot squat. There were also complaints of coughingand a history of frequent bronchitis. On physicalexamination, we found a child with a reddish color,but no cyanosis, and no clubbing of the fingers waspresent. The blood pressure was 105/70 mm. Hg.A harsh systolic murmur was present in the secondto the fourth intercostal space along the left sternalborder, beneath the manubrium and in the secondand third right intercostal spaces. The secondpulmonic sound was extremely loud.The hemoglobin was 78 per cent, and the red

blood cell count was 3,820,000. The electrocardio-gram showed evidences of left ventricular hyper-trophy with strain. On x-ray examination there wasno hilar dance, but an enormous enlargment ofthe pulmonary artery and great pulmonary engorge-ment was evident. The heart was enlarged to theright and to the left.

Catheterization was performed for the firsttime on March 17, 1953. At this time it was possibleto pass the catheter into the aorta (fig. 1). Thepressures were: aorta 100/55 mm. Hg, mean 72;pulmonary artery 100/65, mean 77; right ventricle90/0, mean 45 mm. Hg. There were no differencesbetween the pressure curves of the aorta and pul-monary artery. The blood samples showed a slightbidirectional shunt between aorta and pulmonaryartery.

In considering the pathway taken by the catheter,it was evident that it did not enter the aorta througha patent ductus, because its position was too farmedian, and it was lying in the ascending aorta,the tip not reaching the site in the arch at whichthe ductus arteriosus enters the aorta.

There remained, therefore, two possible routesthat the catheter may have followed: an aorticseptal defect or a high ventricular septal defect.To secure more information we decided to performa second catheterization, and to take as manyblood samples as possible in the pulmonary arteryand in the right ventricle. At the second catheteriza-

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D'HEER AND VAN NIEUWENHUIZEN

FIG. 1. Case 1. Catheter is in the aorta, havingreached it via right ventricle and pulmonary artery.

FIG. 2. Case 1. Catheter, via right ventricle and

pulmonary artery, passed through an aortic septal

.1Y

defect into the ascending aorta toward the aortic

valves.

tion (March 26, 1953), however, it was possible

again to guide the catheter into the aorta, but on

this occasion downward into the ascending aorta

toward the aortic valves rather than upward into

the ascending aorta toward the arch (fig. 2). Through

these observations, a high ventricular septal defect

was excluded, and when the films showing the two

catheter positions were superposed, it could be

seen that a large defect above the aortic valves

must be present (fig. 3)

The child was operated upon on April 24, 1953

by Prof. Dr. A. G. Brom and Dr. A. L. E. Schaepkens

van Riempst. A large defect with a diameter of 5 cm.

between the large pulmonary artery and the aortaabove the semilunar valves was found. The defectwas partially closed by a large ligature. The post-

operative course was uneventful.

Case 2. C. E.,7 a 6 year old girl, was admitted to

the hospital on March 29, 1954. Heart disease was

observed immediately after birth by the physician.When she was three months old, she became cy-

anosed for the first time. At the time of admission

she tired very quickly, had dyspnea and cyanosis

and squatted. She had also frequently bronchitis.

On physical examination acrocyanosis and slight

clubbing were observed. The blood pressure was

110/SO mm. Hg. A systolic murmur varying from

FIG. 3. Case 1. Superposition of the two catheterpositions in figures 1 and 2. The defect must be situ-ated in the aortic septum at a certain distance abovethe aortic valves.

FIG. 4. Case 2. Catheter is in the aorta, havingreached this vessel via right ventricle and pulmona~ryarters..

FIGS 5 C.e2. Pressure recording while with-drawing the catheter from aorta into pulmonaryarteranhdputshing it into a "campillary." Note, thereis no ventriculair pressure pattern between aorta andpulmonary artery lpressures, and the two pressuresare equal

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DIAGNOSIS OF CONGENITAL AORTIC SEGPTAL DEFECTS

FIG. 6. Case 2. Catheter, via right ventricle andpulmonary artery, passed through an aortic septaldefect into the ascending aorta toward the aorticvalves.

FIG. 7. Case 2. Same catheter position as in figure6, lateral view.

FIG. 8. Case 2. Superposition of the two catheterpositions in figures 4 and 6. Here again there must bea defect in the aortic septum at some distance abovethe aortic valves.

slight to harsh was present over the pulmonaryarea. The second pulmonic sound was very loud.

Hemoglobin was 110 per cent with 5,406,000 redblood cells. X-ray films showed a somewhat dilated

heart, especially the right atrium and the leftventricle. There was pulmonary engorgement, butno hilar dance. The electrocardiogram showedright ventricular hypertrophy.

Heart catheterization was performed on April4, 1954. The pressure in the pulmonary artery was110/55, the mean being 85 mm. Hg. Then we wereable to put the catheter into the carotid artery(fig. 4). While withdrawing the catheter from theaorta, we could push it into the pulmonary artery"capillary," without obtaining a ventricular pressurerecording between aorta and pulmonary artery(fig. 5).The possibility of an aortic septal defect was then

thought of and a second attempt was made to enterthe aorta via the defect; and, by manipulatingthe catheter, to direct it into the ascending aortatoward the aortic valves. This was accomplished(figs. 6 and 7). Again, superposition of the two x-rayfilms (fig. 8) demonstrated with absolute certaintythe presence of an aortic septal defect situatedabove the aortic valves.

COMMENTOn embryologic grounds, aortic septal defect

lies close to truncus arteriosus and the differ-ence is only a matter of gradation. Someauthors have designated aortic septal defect aspartial truncus. Clinically, aortic septal defectresembles patent ductus and, especially, patentductus with pulmonary hypertension. It is alsofrequently mistaken for a high ventricularseptal defect.

Diagnosis of Aortic Septal Defect

(1) Clinically, this condition is a possibility,and we must have this diagnosis in mind,therefore, in any case with a continuous orsystolic and diastolic murmur and right ven-tricular hypertrophy in the electrocardiogram.On x-ray films, there is an enlarged heart and avery large pulmonary artery.

(2) In cardiac catheterization, the impossi-bility of catheterizing the aorta when a patentductus is suspected, always suggests the pos-sibility of an aortic septal defect. When acatheter can be passed into the aorta, the fol-lowing possibilities exist: ventricular septaldefect or overriding aorta, patent ductus,truncus arteriosus and transposition of thegreat vessels.The difference between ventricular septal

defect and aortic septal defect is very difficult

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D'HEER AND VAN NIEUWENHUIZEN

to find out by location of the catheter in theaorta only. However, it may be possible inaortic septal defect, while withdrawing thecatheter out of the aorta, to see that the aorticpressure changes into the lower pressure of thepulmonary artery. When, in aortic septal de-fect, the pressures in aorta and pulmonaryartery are equal, it may be possible to with-draw the catheter out of the aorta and push itimmediately into the "capillaries" of the pul-monary artery, without obtaining a ventricularpressure pattern between the aorta and pul-monary artery pressures (fig. 5).

Patent ductus is easier to distinguish fromaortic septal defect. When the catheter entersthe aorta via a patent ductus, it nearly alwaysgoes into the descending aorta, without as-cending in the arch. In the occasional instancewhen it starts toward the arch, almost alwaysit will enter the left carotid artery and not theright. Through an aortic septal defect or anoverriding aorta, the catheter follows the archand in that way enters the descending part, orit ascends in any of the carotid or subclavianarteries.

Differentiation of aortic septal defect andtruncus arteriosus is often very difficult. Ondetermining the oxygen content of variousblood samples, both anomalies can give exactlythe same picture. Only the method describedin this paper, by which catheters are made totake two directions after entering the aorta,can give a complete differentiation.

Transposition of the great vessels gives atotally different clinical picture since it is notpossible to place a catheter in the pulmonaryartery.While determination of the oxygen content

of blood samples, obtained from the pulmonaryartery and right ventricle, may aid in differ-entiating these anomalies and, particularly, inexcluding a ventricular septal defect (or anoverriding aorta), the only absolutely suremethod of differentiation is to pass the catheterinto the aorta via the aortic septal defect andphotograph it first, after it has been directedtoward the arch and again, after it has beendirected toward the aortic valves. X-ray films,thus obtained, will show clearly that the defectis situated in the ascending aorta and at a

certain distance above the aortic valves (figs. 3and 8).

(3) The oxygen content of blood of right brachialartery and femoral artery on effort can also givemuch information. This can be used in cases inwhich a shunt into the pulmonary artery and apulmonary hypertension are present. On effortthe blood of the femoral artery will be more de-saturated than that of the brachial artery inthe presence of a patent ductus with reversedshunt. However, when there is an aortic septaldefect, the desaturation of the blood in the twoarteries will be the same, since the shunt willlie proximal to the mouth of the innominateartery. Nearly the same information can bereached by obtaining simultaneous oxymetricreadings from both ears, during effort, by meansof a two-ear oxymeter.

(4) Retrograde aortography is also a goodmethod for making a correct diagnosis of aorticseptal defect. Since in our cases the diagnosiswas confirmed by catheterization, aortographywas not necessary, and we have had no experi-ence with this method in the diagnosis of aorticseptal defect.

SUMMARYTwo cases of aortic septal defect are de-

scribed. The correct diagnosis could be madeonly by catheterization of the heart. After thecatheter was put into the aorta via the aorticseptal defect, it was advanced first in a forwarddirection toward the aortic arch and carotidartery and then in a backward directionthrough the ascending aorta toward the aorticvalves. By superposition of the x-ray films,showing the two catheter positions, a defect inthe ascending aorta, at some distance abovethe aortic valves, was clearly demonstrated.

Various methods for making a correct diag-nosis of aortic septal defect are discussed. It ispointed out that cardiac catheterization yieldsthe most important diagnostic information.This consists mainly in the findings discussedin the preceding paragraph, but also in theoxygen content of blood samples obtained fromdifferent chambers and vessels. When greatpulmonary hypertension exists, only themethod described in the above paragraphinsures a correct diagnosis.

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DIAGNOSIS OF CONGENITAL AORTIC SEPTAL DEFECTS

SUMMARIO IN INTERLINGUAEs describite duo casos de defecto aortico-

septal. Le correcte diagnose esseva possibilesolmente per catheterisation cardiac. Post quele catheter esseva inserite in le aorta via le de-fecto aortico-septal, illo esseva movite (1) inavante verso le arco aortic e le arteria carotidee (2) in retro a transverso le aorta ascendentein le direction del valvulas aortic. Per superim-poner le pelliculas roentgenographic del duopositiones del catheter, il esseva possibile de-monstrar clarmente le presentia de un defecto inle aorta ascendente alique supra le valvulasaortic.Es discutite varie methodos pro le correcte

diagnose de defectos aortico-septal. Nos signalaque catheterisation cardiac provide le plusimportante information diagnostic. Isto con-siste principalmente in le supra-discutite cons-tatationes sed etiam in datos in re le contentode oxygeno in specimens de sanguine obteniteab varie cameras e vasos. In casos de grandehypertension pulmonar, solmente le supra-describite methodo (vide paragrapho 1) asse-cura un correcte diagnose.

REFERENCES'BROWN, J. W.: Congenital Heart Disease. London,

Staples, 1950.2 SCOTT, H. W. AND SABISTON, D. C.: Surgical

treatment for congenital aortico-pulmonaryfistula. J. Thoracic Surg. 25: 26, 1953.

GASUL, B. M., FELL, E. H. AND CASAS, R.: Thediagnosis of aortic septal defects by retrogradeaortography. Circulation 4: 251, 1951.

4 SPENCER, H. AND DWORKEN, H. J.: Congenitalaortic septal defect with communication be-tween the aorta and the pulmonary artery.Circulation 2: 880, 1950.

5DADDS, J. H. AND HOYLE, C.: Congenital aorticseptal defect. Brit. Heart J. 11: 390, 1949.

6 DOWNING, D. F.: Congenital aortic septal defect.Am. Heart J. 40: 285, 1950.

7 ERF, L. A., FOLDES, J., PICCIONE, F. V. ANDWAGNER, F. B.: Pulmonary hemangioma withpulmonary-aortic septal defect. Am. Heart J.38: 766, 1949.

8 MYERS, G. S., SCANNEL, J. G., WYMAN, S. M.,DIMOND, E. G. AND HURST, F. W.: Atypicalpatent ductus arteriosus with absence of theusual aortico-pulmonary pressure gradient andof the characteristic murmur. Am. Heart J.41: 819, 1951.

9 DOWNING, D. F., BAILEY, C. P., AMANIGLIA, R.AND GOLDBERG, H.: Defect of the aorticseptum. Am. Heart J. 45: 305, 1953.

10 GROSS, R. E.: Surgical closure of an aortic septaldefect. Circulation 5: 858, 1952.

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H. A. H. D'HEER and C. L. C. VAN NIEUWENHUIZENMeans of Cardiac Catheterization

Special Emphasis on a New Method which Allows an Accurate Diagnosis by Diagnosis of Congenital Aortic Septal Defects: Description of Two Cases and

Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 1956 American Heart Association, Inc. All rights reserved.

75231is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TXCirculation

doi: 10.1161/01.CIR.13.1.581956;13:58-62Circulation. 

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