Ventricular septal defect and aortic regurgitation · The association of ventricular septal defect...

Thorax (1969), 24, 257.

Ventricular septal defect and aortic regurgitationK. A. HALLIDIE-SMITH, E. G. J. OLSEN', C. M. OAKLEY2,

J. F. GOODWIN, AND W. P. CLELAND

From the Unit of Clinical Cardiology, the Unit of Cardiothoracic Surgery, and theDepartment of Morbid Anatomy, Royal Postgraduate Medical School, London W.12

We have reviewed our experience of 29 patients with the association of ventricular septal defectand aortic regurgitation, 27 of whom have been treated surgically. Our present approach to thesurgical management is outlined and the embryology and pathological anatomy are reviewed.The most common cause of aortic regurgitation was a prolapsed aortic valve cusp, which occurredin 19 patients. Aneurysmal dilatation of an aortic sinus accounted for the regurgitation in threepatients, and two patients had both abnormalities. In two patients no cause for the aorticregurgitation was found. Those patients with aortic cusp prolapse commonly had -large ventricularseptal defects, of which nearly a third were supracristal. Because of cusp prolapse into the ven-tricular septal defect, and sometimes into the right ventricular outflow tract, the findings at cardiaccatheterization often suggested erroneously that the ventricular septal defect was small and thatinfundibular obstruction was present. Characteristic angiographic features have been demonstrated,in particular the deformed and prolapsed aortic cusp. The various techniques of surgical correctionwhich were used are described, the majority of patients having had a patch closure of the ven-tricular septal defect and repair of the aortic valve. Some residual aortic regurgitation was usual,but it was improved or minimized in 18 of the 23 survivors, and the more recent results have beenparticularly encouraging. Closure of the ventricular septal defect alone relieved heart failure in a

3-year-old child. There were four operative deaths (15% mortality) in patients with severe aorticregurgitation, three of whom were in heart failure before operation. The medical course wascommonly of slowly increasing severity of the aortic regurgitation with progression to heartfailure. However, the individual course was unpredictable and three children developed heartfailure within a year of the onset of their aortic regurgitation. Infective endocarditis occurred ina quarter of the patients and usually followed the development of the aortic regurgitation. Despitethe difficulties of surgical management it is felt that the unpredictable prognosis, high operativerisk once cardiac failure has supervened, and the hazard of infective endocarditis make earlyoperation desirable.

The association of ventricular septal defect andaortic regurgitation was first described by Laubryand, Pezzi in 1921. Since then, although onlyapproximately 150 cases have been reported in theliterature, the development of aortic regurgitationhas come to be recognized as one of the morecommon and the most serious of the complica-tions of ventricular septal defect.

This combination of defects has a poor medicalprognosis, and so with improving surgical tech-niques total correction is being more widely advo-cated. Surgical correction is not always easy, andsuccess depends largely on a comprehensive know-ledge of the anatomy and function of the aorticvalve.1'On a nt ftom the British Heart Fondation2Supported by the BrIth Heart Foundation

THE PATIENTS STUDIED

Between 1958 and 1967 we investigated 29 patientswith ventricular septal defect and aortic regurgita-tion, 27 of whom had been surgically corrected.This is the largest series so far reported. Thisgroup represents 12% of our series of 229 surgi-cally treated ventricular septal defects. Since themajority of the patients are specially referred thisfigure may give a falsely high percentage of inci-dence.Our patients consisted of 17 males and 12

females, between 2 and 37 years of age at thetime of investigation, and 3 and 38 years at thetime of operation, when 15 patients were less than16 years.

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258 K. A. Hallidie-Smith, E. G. J. Olsen, C. M. Oakley, J. F. Goodwin, and W. P. Cleland

HISTORY The early history of the adult patientswas difficult to elicit. Nine of the 15 childrengave a history of failure to thrive in infancy;13 of the total number of patients complained ofsome limitation of effort tolerance, and six devel-oped signs of heart failure.

In 20 of the 29 patients a heart murmur washeard in early infancy, and in a further eight itwas first noted between the ages of 3 and 8 years,usually at a school medical examination. Theremaining patient, a West Indian, was neverexamined in childhood.The initial murmur was known to have been

solely systolic in 19 patients. The time, of appear-ance of the diastolic murmur was well documentedin 11 children, and varied between 2 and 12 years.In eight other children the exact time of appear-ance of the diastolic murmur was uncertain, butall of them were between 2 and 13 years whenit was first noted. The remaining patients pre-sented in adult life with an established diastolicmurmur.

Seven of our patients (24%) provided a clearhistory of subacute infective endocarditis, but innone was it certain that the infection precededthe aortic regurgitation. The aortic regurgitation,however, preceded the subacute infective endo-carditis in three patients.

PHYSICAL SIGNS Nine of the 15 children wereundersized, particularly in regard to weight.Bulging of the chest, particularly of the left side,was common.

In all patients the pulses were described asjerky or ill-sustained. In 17 patients the diastolicarterial blood pressure was below 40 mm. Hg,and in 15 patients the arterial pulse pressure was60 mm. or more.A left ventricular impulse was noted in all the

patients, and some degree of right ventricularhypertrophy in 14. A systolic thrill at the leftsternal edge was common. Both the systolic anddiastolic murmurs were commonly described asmaximal at the lower left sternal edge, but thepulmonary area was thought to be the site ofmaximum intensity in a few. Three patients withright ventricular infundibular stenosis had loudsystolic murmurs in the pulmonary area.The murmurs were variously described, the

description varying not only between differentobservers but between different examinations bythe same observer.Most often it was thought that the systolic

murmur filled systole, stopping at the secondsound and followed by a decrescendo early dia-

stolic murmur. Sometimes the murmurs werethought to be truly continuous, the diastolicmurmur following the systolic murmur withoutintermission. The majority of patients had a thirdheart sound and a short apical mid-diastolicmurmur.The aortic regurgitation was assessed as mild

in 5, moderate in 13, and severe in 11 on thebasis of the clinical findings, electrocardiogram,and chest radiograph.The most difficult differential diagnosis of

ventricular septal defect with aortic regurgitationwas from a sinus of Valsalva fistula into the rightventricle. Other differential diagnoses consideredin some patients, particularly early in our experi-ence, were patent ductus arteriosus, aorto-pulmonary window, coronary artery fistula, andisolated aortic valve disease. Three patients hadhad a previous thoracotomy elsewhere for asupposed patent ductus arteriosus.

PHONOCARDIOGRAPHY Recordings wore carriedout in all the patients. They confirmed that themost common murmurs were as described clini-cally but illustrated the similarity of the murmursin some patients to those found in sinus of Val-salva fistula, patent ductus arteriosus, and aorticvalve disease (Fig. 1). The second sound waswidely split in the presence of infundibular pul-monary stenosis.

ELECTAOCARDIOGRAPHY All the records showedsome evidence of left ventricular hypertrophy,ranging from slight in 12 to severe in five. Therewas unequivocal evidence of biventricular hyper-trophy in the three children who had infundibularpulmonary stenosis requiring resection. Twopatients had incomplete right bundle-branch blockwhile three, all with severe aortic regurgitation,showed evidence of left atrial hypertrophy. Thefrontal plane axis varied from +300 to +70° inall but twio patients, who had left axis deviationof -30° and - 40° respectively, which was un-related to the severity of the aortic regurgitationor to the site or size of the ventricular septaldefect. Several patients showed progressive leftventricular hypertrophy, coinciding with progres-sion of the aortic regurgitation (Fig. 2).RADIOGRAPHY There was some left ventricularenlargement in all patients, and the cardiothoracicratio was over 50% in all save two. Right-sidedenlargement was noted in 16 patients, includingthe six in heart failure and three with a systolicright ventricular outflow tract gradient of over50 mm. Hg. Ten patients showed some left atrial



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FIG. 1. Phonocardiograins of three patients with ventricular septal defect andaortic regurgitation compared with those of rheumatic aortic regurgitation, sinusof Valsalva fistula from aorta to right ventricle, and patent ductus arteriosusrespectively. A.1 = aortic regurgitation ; V.S.D. = ventricular septal defect;P.D.A. = patent ductus arteriosus; P.A.=pulmonary area; A.A.=aortic area;H.F. = high frequency; L.S.E. = left sternal edge; M.A.=mitral area.

enlargement, while right atrial enlargement wasnoted in the patients in heart failure. The mainpulmonary artery was often prominent. The lungfields were assessed as slight or moderately over-filled in all but five patients. Serial increase inheart size correlated with the speed of progressionof the aortic regurgitation (Fig. 3a, b). The degreeof left ventricular enlargement sometimesappeared disproportionately great in regard to theseverity of the aortic regurgitation. This could beaccounted for by the additional volume load onthe left ventricle imposed by the presence of theventricular septal defect.

CARDIAC CATHETERIZATION Right heart catheter-ization was carried out in all the patients (Table).There were certain characteristics of the group

as a whole (Figs 4 and 5). A systolic pressuregradient below the pulmonary valve or high inthe body of the right ventricle was common, andwas 20 mm. Hg or more in 12 patients. Threeof these patients had infundibular hypertrophyrequiring resection, but in the remaining nine the

gradient was abolished after correction of theventricular septal defect and aortic regurgitation,without infundibular resection.

Despite the fact that most of the ventricularseptal defects were of a size that an elevated pul-monary artery pressure or one balanced with thesystemic arterial pressure would be expected, infact there was only one patient in whom the pul-monary artery pressure was more than moderatelyelevated. This patient was the only one who hada raised pulmonary vascular resistance (10 units).The other patients had normal or slight to moder-ate elevation of the pulmonary artery pressureand normal pulmonary vascular resistances.The majority of patients had small left-to-right

shunts with a ratio of pulmonary to systemic flowof less 'than 2: 1. However, six patients with apulmonary artery systolic pressure of 40 mm. orless had large left-to-right shunts with a ratio ofpulmonary to systemic flow of around 3:1. Theleft atrial mean pressure was normal except inthose patients in heart failure, in whom it waselevated to between 15 and 20 mm. Hg. The

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FIG. 2. Case 14. Serial electrocardiograms (a) at age 11 months withphysical signs of a large septal ventricular defect, showing right ventri-cular hypertrophy; (b) two months after onset of aortic regurgitation, atage 5 years, showing left ventricular hypertrophy developing; (c) sevenmonths later, demonstrating rapidly progressive left ventricularhypertrophy.

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FIG. 3. Case 5. Postero-anterior chest radiographs (a) several months after thedevelopment of aortic regurgitation; (b) 15 months later, demonstrating increasingleft ventricular enlargement.



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FIG. 5. Withdrawal tracings from pul-monary artery (P.A.) to right ventricle(R.V.) in two patients with ventricularseptal defect and aortic regurgitation,showing (above) gradients due to a

prolapsed cusp obstructing the rightventricular outflow tract, and (below)infundibular stenosis.

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ANGIOCARDIOGRAPHY Aortography was carriedout in 24 patients by the retrograde Seldingertechnique (Seldinger, 1953), using cine angio-

graphy in 13. Six of these patients had left ventri-cular angiocardiograms and four had right ventri-cular angiocardiograms. The aortic regurgitationwas assessed as severe in nine, moderate in 10,slight in four, and trivial in one, agreeing wellwith the clinical grading.

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The left ventricle was assessed as enlarged inall except two, and there was dilatation of theascending aorta in all with marked systolic expan-sion. There was right ventricular enlargement inthose patients in heart failure or with a significantright ventricular outflow tract pressure gradient.

Characteristically (Fig. 6a-c) there was a systolicshunt from the left ventricle to the right ventriclethrough the ventricular septal defect or directly

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from the aorta to the right ventricle through asinus of Valsalva fistula, with early opacificationof the right ventricular outflow tract. In diastolethe regurgitant jet of contrast from the aorta splitup, one stream going directly to fill the left ven-tricle and the other passing to the right ventriclethrough the ventricular septal defect or directlythrough a sinus of Valsalva fistula.A prolapsed aortic valve cusp, usually the right

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FIG. 6. (a) Systolic and (b) diastolic frames of lateralaortogram of patient with a supracristal ventricularseptal defect and prolapsed right. coronary cusp andsinus. (c) The frames are superimposed diagrammatically,demonstrating the characteristic movement of the aorticroot, deformity of the cusp and sinus, retention of contrastmedium in the affected cusp and sinus, and contrastmnedium passing from the aorta to the left and rightventricles in diastole.--, svstole diastole.

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coronary, which was the cause of the aortic re-gurgitation in the majority of our patients, gavecertain characteristic appearances (Figs 6 and 7).In diastole there was a berry-shaped or 'squaredoff' appearance to the affected cusp and sinus.In systole the cusp appeared more deformed andtended to retain contrast medium. The redundantcusp often showed a double contour, and thedeformed cusp and sinus could be seen bulginginto the right ventricular outflow tract. Anexaggeration of normal aortic root movementwas noted (Fig. 6).Aortograms of the patients with sinus of Val-

salva aneurysms and fistulae showed the affectedsinus to be dilated both in systole and diastole(Fig. 8) with no obvious abnormality of the aorticvalve cusps.

In the absence of a deformed cusp, it was notalways possible to distinguish by angiocardio-graphy between a sinus of Valsalva fistula intothe right ventricle and a ventricular septal defect(particularly a supracristal one) with aortic re-gurgitation due to an incompetent valve. It ispossible that left ventricular cine angiocardio-graphy with careful differentiation betweensystolic and diastolic shunts could help to eluci-date this further.

SURGICAL MANAGEMENT The type of surgicalmanagement is largely dictated by the severity ofthe aortic regurgitation. If the latter is minimalit is unlikely that any special measures will berequired, either to handle the perfusion or to dealwith the aortic valve. In earlier patients it washoped that repair of the ventricular septal defectwould lead to a reduction or even abolition ofthe aortic leak, but these hopes were illfounded.However, with few exceptions further progressof the aortic regurgitation has been halted.Possibly, however, closure of the ventricular septaldefect alone may reduce the risk of infection.

In patients with moderate or severe aorticregurgitation, aortic cross-clamping will be re-quired if an adequate body perfusion is to becarried out and if the operative field is to be keptrelatively dry. In these patients the aorta shouldbe opened in order that the valve can be carefullyinspected both from above and from below, and

FIG. 7. Lateral aortogram of a 3-year-old child withventricular septal defect and prolapsed right coronarycusp, demonstrating particularly well the deformedcusp and sinus. (a) early systole; (b) late diastole;(c) early diastole; (d) late diastole.

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(b)FIG. 8. Lateral aortograms of a patient with sinus ofValsalva fistula from right aortic sinus to right ventricleto show characteristic deformity of sinus (a) in systoleand (b) in diastole, aortic regurgitation, and opacificationof the~right ventricle.

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so that the coronary vessels can be cannulatedand perfused.The valve should be very carefully inspected

in order to determine the exact cause ofregurgitation. Some valves are capable of recon-struction or repair, whereas others are so grosslydeformed that replacement with a hom-ograft ora mechanical prosthesis is required.The following techniques have been used in the

patients recorded here:(a) Simple repair of a ruptured sinus of Valsalva

or perforated cusp: in these patients the cuspsthemselves are reasonably normal, and meet cen-trally so that reinforced sutures of the perforationresult in a competent valve.Sometimes the aortic annulus is dilated as a

result of the regurgitation, so that the cusps donot meet centrally. In such cases the cusps maybe widened by suturing a strip of fascia lata totheir free edges or the valve may require replace-ment.

(b) The comm-onest abnormality is the pro-lapsed and grossly redundant right coronary cusp,often associated with enlargement of the relevantsinus of Valsalva. In such cases it is often difficultto determine the precise position of the aorticannulus into which the stitches for closing theventricular septal defect should be inserted. Anattempt should be made to oorrect the regurgita-tion by shortening the cusp and elevating itslightly by reinforced stitches placed in each com-missure. This procedure may have to be accom-panied by cusp extension with fascia lata.

(c) Occasionally regurgitation has been due tolack of support beneath the right coronary/non-coronary commissure, allowing the adjacentmargins of the cusps to prolapse. Elevation andfixation of this commissure a few millimetreshigher up on the aortic wall may result in com-petence.

(d) Reconstruction of the whole valve orreplacement with a homograft or a mechanicalprosthesis will be required in those patients whererepair fails to produce competence. In such casesthe redundant right coronary cusp can be partiallyretracted and sutured to the margins of the ventri-cular defect.

OPERATIVE FINDINGS (Table) The majority of theventricular septal defects were large, 15 being over1 cm./m.2 B.S.A. Seven patients (26%) had supra-cristal defects, an incidence over eight times thatin the remainder of our series of surgicallycorrected ventricular septal defects.

The regurgitation was thought to be due to aprolapsed cusp in 19 patients, a deformed cuspin one, perforated cusps in two (with a partiallyprolapsed cusp in one), and a sinus of Valsalvaaneurysm in three, while in two patients (in whomaortotomy was not carried out) there was noobvious cause for the aortic regurgitation. In twopatients a prolapsed or distorted cusp was asso-ciated with a sinus of Valsalva defect, and fourof the five patients with an aneurysim of the sinusof Valsalva had fist.ulae into the right ventricle.The deformed cusp was the right coronary in

19 patients and the non-coronary cusp in one.Often the portion of the non-coronary cuspadjacent to the affected right coronary cusp wasalso abnormal. The affected cusp was usuallylarger than normal, being patulous and thinnedout with a long leading edge. There were oftenareas of thickening in such cusps, either adjacentto the non-coronary cusp or in the most depen-dent inferior portion of the prolapsing cusp, andsometimes small perforations in the thinned outbase. The affected cusp was often prolapsing intoor through the ventricular septal defect towardsthe right ventricular outflow tract, thus partiallyoccluding the ventricular septal defect andreducing its effective size. In two patients part ofthe right coronary cusp and a smaller portion ofthe adjacent non-coronary cusps were fibroticand calcified without being obviously prolapsed.In one young child the right coronary cusp wasshortened, thickened, and thus deficient andincompetent.The majority of the ventricular septal defects

in which there was prolapse of an aortic valvecusp were either supracristal or high infracristal;that is, the defect was sited immediately below theaortic ring, often at the commissure between theright coronary and non-coronary cusp. There wasthus an area of aortic ring unsupported by septum,predisposing towards prolapse of the aorticannulus, cusp or sinus.

Three patients had infundibular right ventri-cular hy,pertrophy requiring resection. One patienthad an associated atrial septal defect which wasclosed, and discrete subvalvar aortic stenosiswhich was resected. The aortic regurgitation inthis patient (case 24) appeared to be due to cuspperforations. The right coronary cusp was notprolapsed at surgery but appeared large and lax.The angiographic findings suggested that whenthe heart was beating the cusp was blown out andprolapsing through the defect.The affected cusp was repaired or hitched up

in 12 patients (Fig. 9), a Starr-Edwards prosthesiswas inserted in one patient, a homograft in a



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(b) (c)FIG. 9. (a) Heart of a 15-year. old boy opened from the left ventricular aspect, showing grossly thickened andredundant right coronary cusp and sinus (the leading edge has been reefed and the commissures stitched)prolapsing into a 4 x 2 5 cm. infracristal ventricular septal defect. Lateral aortograms of the same patient(b) in systole and (c) in diastole.



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second, and a fascia lata repair was carried outin a third. In eight patients no attempt was madeto deal with the aortic regurgitation. The sinus ofValsalva fistulae were closed, and the ventricularseptal defects were closed by direct suture or witha patch.

RESULTS OF SURGERY Four patients (17%) didnot survive operation, but three of these patientswere in heart failure, and the fourth, with severeaortic regurgitation, was operated on very early inthe series. Of the 23 survivors, the residual aorticregurgitation was thought to be severe in 4,moderate in 9, slight or trivial in 7, and absent in3. The most effective repairs were made in thosepatients in whom the aortic regurgitation was notsevere and who have been operated on morerecently. Closure of the ventricular septal defectalone appeared sufficient to halt the progressionof the aortic regurgitation and to relieve heartfailure in a child of 3 years.

POST-MORTEM FINDINGS Two hearts were avail-able for pathological examination. The first (Fig.9a) was from a 15-year-old boy, who was inheart failure at the time of operation. His heartweighed 920 g. with dilatation and hypertrophyof all chambers.

There was an infracristal defect, 3x2 mm. insize, situated immediately below the aortic ring,centred between the right coronary and non-coronary cusps. The right coronary cusp was un-supported and prolapsing through the defect. Itwas very lax and redundant, measuring 32 mm.along its leading edge with a depth of 20 mm.The most dependent part of the cusp was parti-cularly thickened. The adjacent sinus was alsodilated and incorporated into the cusp. The non-coronary cusp was also deformed, measuring 22x14 mm. The right ventricular outflow tractappeared normal.

Histology of the right coronary cusp showedgross thickening by hyalinized collagen.The leading edge of the right coronary cusp

had been shortened by reefing and the cusphitched up by stitching the commissure betweenthe right coronary and non-coronary cusps.The second patient (Fig. 10), an 8-year-old boy,

was also in heart failure at the time of operation.The anatomy was similar to that described above,but the affected cusps were not so grossly de-formed. It can be seen how the right coronarycusp has been hitched up and the adjoining com-missure stitched at the base, using a Teflonpledget. T;he ventricular septal defect was patchedwith Teflon.

FIG. 10. Heart of an 8-year-old boy opened from theleft ventricular aspect to show a 2 x 1-5 cm. infracristalventricular septal defect, which has been patched, and athickened right coronary cusp hitched up at the commis-sures over Teflon pledgets. (A block has been takenfor histology.)

MEDICAL PROGNOSIS Even though not all ourpatients were under continuous medical super-vision, certain salient features emerge. Thus, themajority of patients apparently slowly developedprogressive aortic regurgitation with increasingleft ventricular hypertrophy and eventual develop-ment of left ventricular failure.

In three of our patients, in whom the onset ofaortic regurgitation was well documented, itsprogression was extremely rapid, heart failuresu,pervening in less than a year. Six patients werein heart failure at the time of operation.

In one patient, assessed now over a period of12 years, there appears to have been no obviousincrease in aortic regurgitation or heart size.As previously mentioned, patients with this

combination of defects appeared particularlyprone to develop infective endocarditis, and thisfurther prejudiced their prognosis.

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DISCUSSION

PATHOLOGICAL ANATOMY The ventricular septaldefect has been classified by Edwards (1960) asfollows:

1. Defects related to the ventricular outflowtracts

2. Defects related to the ventricular inflowtracts

3. Defects common to both outflow and inflowtracts

4. Left ventricular-right atrial communication.The defects related to the ventricular outflowtracts have been further subdivided into thosesituated postero-inferiorly to the crista supraventricularis and those lying antero-superior tothis anatomical landmark. The last mentioned isthe less common variety. Becu, Fontana,DuShane, Kirklin, Burchell, and Edwards (1956)found that of 50 hearts with ventricular septaldefect 40 showed the defect in the outflow por-tion of the ventricular septum. In 35 of these thedefect lay postero-inferiorly to the crista, whileonly in four was the defect antero-superior.The ventricular septal defects associated with

aortic regurgitation are situated between the rightand non-coronary cusps. The anatomical relationshave been described by Nadas, Thilenius,LaFarge, and Hauck (1964). Seven of his cases

came to necropsy and were divided into twogroups. The first group consisted of four hearts,and then the defect involved the membranous por-

tion of the septum mainly, but also a small portionof the bulbar musculature. Inferiorly, the defectwas bounded by a ridige of the main ventricularmuscular septum, and superiorly by the commis-sure between the right and non-coronary

(posterior) aortic valve cusps. Both the hearts inour series which were available for post-mortemexamination fit the description of Nadas' group 1.The remaining hearts fit into group 2, and theventricular septal defect was situated just belowthe commissure between the right and non-

coronary aortic cusps involving the anterior halfof the membranous septum and also a portionof the anterior bulibar muscular septum.The anatomy of the root of the aorta has been

described by Edwards and Burchell (1957). Eachaortic sinus is subdivided into three more or lessequal parts and inferiorly, all parts of the rightaortic sinus lying in close relation to the ventri-cular septum. The muscular portion is constantbeneath the central and the left parts, but beneaththe posterior part of the right aortic sinus eithermuscular or membranous portions of the ventri-

cular septum may be present. The right part ofthe posterior aortic sinus is related inferiorlyeither to the muscular or membranous portions ofthe interventricular septum.

Over-riding of the aorta is usually present, andwas estimated by Nadas et al. (1964) to be about30%. The aorta is not in contact with the ventri-cular septal defect at the site of the defect (Ellis,Ongley, and Kirklin, 1963). Slight hypertrophy ofthe crista supra ventricularis was present in ourtwo necropsied cases, but this can be variable andneed not necessarily be present.The site of the ventricular septal defect is

imp.ortant, and experimental high ventricularseptal defects in newborn chicks demonstratedprolapse of the right coronary cusps (de la Cruz,cited by Nadas et al., 1964). The close relation-ship between the right ventricular outflow tractand the ventricular septum is well described byEdwards and Burchell (1957). The cause of theprolapse of the aortic cusp and its occurrence insome high ventricular septal defects but not inothers, even though the anatomical position maybe similar, is unknown.The anatomy and embryology of septal defects

in the membranous and bulbar areas of theanterior bulbar septum have been described byGrant, Downey, and MacMahon (1961). Bycareful dissection they were able to show thenormal anatomy of the bulbar muscle. This con-sisted of two muscular layers: the superficiallayer is made up of three components-the onedeep layer which originates from the membranousseptum and also from the aortico-pulmonarytendon. This tendon is a fibrous ring at the rootof the aorta which partly arises from the mem-branous septum and from the right trigone of theleft ventricle. It meets a similar fibrous ring at theroot of the pulmo.nary artery and is inserted inthe left trigone. In the light of the above descrip-tion the defects were subdivided as follows:

(1) defects of the atrioventricular ring; (2)defects in the main body; (3) bulbar defects.The particular location of bulbar septal defects

is governed by the missing su.perficial bulbarmuscle components, whilst a missing deeper oom-ponent appears to determine the size of the ventri-cular septal defect. This last component is some-times difficult to evaluate because only part of thismuscle may be missing. The deep bulbar com-ponent is inserted into the aortioo-pulmonary ten-don at the root of the aorta near the aortic cusps.If this muscle layer is absent (and in the absenceof bulbar muscle no left ventricular tissue cangrow) the anterior lip of the aorta will have no



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attachment to the left ventricle. This then partlyexplains why some of the aortic valves may beless supported and also the over-riding of the aortaitself.

In our own case two sections were taken show-ing the root of the aorta, the prolapsed aorticvalve, and the underlying muscular portion of theseptum. In each there was some definite musclebeneath the aortic valve and, as has been pointedout, unless careful dissection is carried outevaluation of this is indeed very difficult. Controlspecimens from a case of tetralogy of Fallotshowed no discernible difference histologically.We have confirmed the observation of Nadas

et al. (1964) that the signs of aortic regurgitationcomplicating those of a ventricular septal defectdevelop after the first year of life, thus addingweight to the supposition that this is an acquiredcomplication.We have also confirmed the findings of Keck,

Ongley, Kincaid, and Swan (1963), Scott,McGuire, Kaplan, Fowler, Green, Gordon,Shabetai, and Davolos (1958), Nadas et al. (1964),and Plauth, Braunwald, Rockoff, Mason, andMorrow (1965) that the most common cause ofthe aortic regurgitation is a deformed aortic valvecusp, usually the right coronary, sometimes withpartial involvement of the adjacent non-coronarycusp. The affected cusp is deformed in severalways, but most commonly prolapses into orthrough the ventricular septal defect.The production of aortic regurgitation in sinus

of Valsalva aneurysms has been ascribed by Lon-don and London (1961) to progressive dilatation ofthe sinus with eventual distortion of the relevantvalve cusp. It seems likely that this mechanismaccounted for or contributed to the aorticregurgitation in our six patients with sinus ofValsalva fistulae or aneurysms.A characteristic of the ventricular septal defects

in which prolapse of an aortic cusp had occurredis their high position immediately under the aorticvalve ring, thus leaving an unsupported area wherethere was no continuity between aorta and ventri-cular septum, as noted by Ellis et al. (1963). Ahigh incidence of supracristal defects has alsobeen noted by others (Ellis et al., 1963 ; Plauthet al., 1965). These authors also noted the ventri-cular septal defects to be of small size, a findingwhich we were unable to confirm. Indeed themajority (17) of our ventricular septal defects witha prolapsed cusp were large.

Infundibular pulmonary stenosis with thisassociation of defects has already been described

(Collins, East, Godfrey, Harris, and Oram, 1958;Nellen, Schrire, and Vogelpoel, 1959; Nadaset al., 1964; Plauth et al., 1965; Carlsson, Hart-mann, and Kissane, 1965). We were able todemonstrate this in three patients, but found thatan infundibular gradient or gradient within theright ventricular cavity was common. Our impres-sion is that hypertrophy of the crista (Ellis et al.,1963) is uncommon, and that the gradient isusually abolished after repair of the ventricularseptal defect and aortic regurgitation. It wouldseem likely, therefore, that at least some of thegradient can be accounted for by prolapse of theaortic cusp (Nadas et al., 1964) through the defectinto the right ventricular outflow tract. Ourhaemodynamic data demonstrating small left-to-right shunts with normal pulmonary artery pres-sures and a large ventricular septal defect can beexplained by prolapse of the cusp or sinus, orsometimes of the unsupported aortic annulus intothe defect (Plauth et al., 1965), thereby reducingits effective size. This situation was suspected insome of our patients at angiocardiography anddemonstrated clearly at surgery. The finding of alarge left-to-right shunt with normal pulmonaryartery pressures and a large ventricular septaldefect can be explained by the fact that the effec-tive size of the ventricular septal defect is reducedby the prolapsed cusp partially occluding it, whilea diastolic as well as a systolic left-to-right shuntis made possible from aorta to right ventricle viathe incompetent aortic valve and ventricularseptal defect.

If the ventricular septal defect is supracristal,it is easy to visualize how the right or non-coronary cusp, sinus, and even the aortic ring canprolapse downwards into the defect. Figure 11shows the heart of a 3-year-old boy with a supra-cristal ventricular septal defect showing how theright coronary cusp is deformed and enlarged,already potentially falling through the defect,though there was no clinical evidence of aorticregurgitation.The right and non-coronary cusps in an infra-

cristal defect look directly into the ventricularseptal defect when it is situated immediately underthe aortic ring. It is possible that a jet of bloodimpinges on the base of the cusp, causing local-ized thickening. It will then tend to elongate andwith each successive cardiac cycle to be drawndown through the open septum, often resulting inelongation of the relevant sinus.The difficulties of differential diagnosis are

shown by Morgan and Burchell (1950), Scott

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FIG. 11. Heart of a 3-year-old boy opened from theleft ventricular aspect to show a supracristal ventricularseptal defect and a deformed right coronary cusp over-

lying the defect.

et al. (1958), Winchell and Bashour (1956), Clay-pool, Ruth, and Lin (1957), and Denton andPappas (1958). Gross (1952) noted that an

erroneous clinical diagnosis of pat-nt ductusarteriosus had resulted in needless thoracotomies.More recently, increasing awareness of the com-bination of ventricular septal defect and aorticregurgitation has made us more appreciative ofclues in its diagnosis. Patent ductus arteriosus canbe excluded clinically, mainly by the site of in-tensity of the murmurs which are maximal at thelower left sternal edge and the disproportionateleft ventricular enlargement both clinically andradiologically.We have noted a high incidence of infective

endocarditis in our patients as compared with thelow incidence in isolated ventricular septal defects(Shah, Singh, Rose, and Keith. 1966). and we con-

firmed the findings of Nadas et al. (1964) thatthe infection often occurs after the developmentof aortic regurgitation. It was not confined tothose who had severe aortic regurgitation.Possibly the jet lesion from the ventricular septaldefect renders the prolapsed cusp of the aorticvalve more vulnerable to infection.We have also confirmed in general the progres-

sive nature of the aortic regurgitation in ourpatients (Nadas et al., 1964; Plauth et al., 1965)with slow or rapid progression to left ventricularfailure. The course of an individual patientappeared to be unpredictable, however.

Recent surgical procedures have given en-couraging results, and with the known poormedical prognosis and high incidence of infection,we now feel that surgery should be offered tothese patients on first diagnosis.

We are most grateful to Professor R. E. Steinerfor radiological advice and co-operation, and toProfessor H. H. Bentall for kindly allowing us toquote his surgical results and for advice. Mr. GeraldRainbow supervised the technical aspects of thehaemodynamic studies, and we are indebted to himfor his expert assistance.

REFERENCESBecu, L. M., Fontana, R. S., DuShane, J. W., Kirklin, J. W., Burchell

H. B., and Edwards, J. E. (1956). Anatomic and pathologicstudies in ventricular septal defect. Circulation, 14, 349.

Carlsson, E., Hartmann, A. F. Jr., and Kissane, J. M. (1965). Ventricu-lar septal defect with prolapsed aortic valve and outflow tractobstruction. Acta Radiol. (Diagn.), 3, 554.

Claypool, J. G., Ruth, W., and Lin, T. K. (1957). Ventricular septaldefect with aortic incompetence simulating patent ductusarteriosus. Amer. Heart J., 54, 788.

Collins, D. M., East, T., Godfrey, M. P., Harris, P., and Oram, S.(1958). Ventricular septal defect with pulmonary stenosis andaortic regurgitation. Brit. Heart J., 20, 363.

de la Cruz, M. V. (1964). (Cited by Nadas, A. S., et al., 1964, personalcommunication.) Vide infra.

Denton, C., and Pappas, E. G. (1958). Ventricular septal defect andaortic insufficiency: report of three cases. Amer. J. Cardiol., 2,554.

Edwards, J. E. (1960). In Pathology of the Heart. Ed. Gould, S. E.Charles C. Thomas, Springfield, Illinois.and Burchell, H. B. (1957). The pathological anatomy ofdeficiencies between the aortic root and the heart, includingaortic sinus aneurysms. Thorax, 12, 125.

Ellis, F. H., Ongley, P. A., and Kirklin, J. W. (1963). Ventricularseptal defect with aortic valvular incompetence. Surgical con-siderations. Circulation, 27, 789.

Grant, R. P., Downey, F. M., and MacMahon, H. (1961). Thearchitecture of the right ventricular outflow tract in the normalhuman heart and in the presence of ventricular septal defects.Ibid., 24, 223.

Gross, R. E. (1952). The patent ductus arteriosus. Observations ondiagnosis and therapy in 525 surgically treated cases. Amer. J.Med., 12,472.

Keck, E. W. O., Ongley, P. A., Kincaid, 0. W., and Swan, H. J. C.(1963). Ventricular septal defect with aortic insufficiency. Aclinical and hemodynamic study of 18 proved cases. Circulation,27, 203.

London, S. B., and London, R. E. (1961). Production of aortic re-gurgitation by unperforated aneurysm of the sinus of Valsalva.Ibid., 24, 1403



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Laubry, C., and Pezzi, C. (1921). Traite des Maladies Congenitalesdu Cawr. Bailliere, Paris.

Morgan, E. H., and Burchell, H. B. (1950). Symposium on cardiaccatheterization; ventricular septal defect simulating patent ductusarteriosus. Proc. Mayo Clin., 25, 69.

Nadas, A. S., Thilenius, 0. G., LaFarge, C. G., and Hauck, A. J.(1964). Ventricular septal defect with aortic regurgitation.Medical and pathologic aspects. Circulation, 29, 862.

Nellen, M., Schrire, V., and Vogelpoel, L. (1959). Ventricular septaldefect with aortic incompetence. S. Afr. med. J., 33, 91.

Plauth, W. H., Jr., Braunwald, E., Rockoff, S. D., Mason, D. T., andMorrow, A. G. (1965). Ventricular septal defect and aorticregurgitation. Clinical, hemodynamic and surgical considerations.Amer. J. Med., 39, 552.

Scott, R. C., McGuire, J., Kaplan, S., Fowler, N. O., Green, R. S.,Gordon, L. Z., Shabetai, R., and Davolos, D. D. (1958). Thesyndrome of ventricular septal defect with aortic insufficiency.Amer. J. Cardiol., 2, 530.

Seldinger, S. I. (1953). Catheter replacement of the needle in per-cutaneous arteriography; a new technique. Acta Radiol. (Stockh.),39, 368.

Shah, P., Singh, W. S. A., Rose, V., and Keith, J. D. (1966). Incidenceof bacterial endocarditis in ventricular septal defects. Circulation,34, 127.

Winchell, P., and Bashour, F. (1956). Ventricular septal defect withaortic incompetence simulating patent ductus arteriosus. Amer.J. Med., 20, 361.

ADDENDUM

Longer follow-up of patients who have had repairprocedures has shown that aortic regurgitationmay reappear or progress. However, we regard thetreatment as surgical, while realizing that the idealoperative procedure has not yet been devised.Homograft replacement, the most appealing pro-

cedure, may be impossible in children because ofthe small aortic valve ring; similarly, a prosthesismay be impractical and is undesirable in this age

group. We would then regard a repair procedureas being indicated initially in a child with more

than slight aortic regurgitation, envisaging homo-graft replacement as a definitive procedure wherenecessary.

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