Aorto-left ventricular tunnel Clinical.features and ... · Severe aortic regurgitation is well tolerated in the young, andwith the current uncertainty about the long-termfunction

British HeartJournal, I974, 36, 32I-328.

Aorto-left ventricular tunnelClinical.features and surgical management

Jane Somerville, Terence English, and Donald N. RossFrom Cardiothoracic Institute and National Heart Hospital, London

Three patients aged 5 to 7years with aorto-left ventricular tunnel had successful surgical closure, and afourthpatient aged I6 years with bad postoperative aortic regurgitation is described. The diagnosis should be con-

sidered in a child presenting with the features suggestive of severe aortic regurgitation. Early operation isrecommended before the aortic annulus becomes too dilated.

Aorto-left ventricular tunnel is an uncommon con-dition which presents as apparent gross aorticregurgitation in childhood (Levy et al., I963).Severe aortic regurgitation is well tolerated in theyoung, and with the current uncertainty about thelong-term function of any valve replacement one isreluctant to replace the aortic valve prematurelyin a child or adolescent. However, the aorto-leftventricular tunnel may be 'cured' without replace-ment of the aortic valve providing surgery is per-formed before the valve cusps and annulus havebecome distorted (Roberts and Morrow, I965). It is,therefore, important to distinguish patients with atunnel from those with primary valvar regurgita-tion in order that appropriate management can bedecided. The present report is concerned with theclinical and surgical management of three patientswith proven aorto-left ventricular tunnel seen in theNational Heart Hospital in the past four years, andthe clinical data on a fourth patient operated onelsewhere are mentioned.

FindingsPresentationThere were two boys and one girl. Heart murmurs wererecognized early in all, on day one by the general prac-titioner who delivered the child and at 3 weeks and 2years in the others. The features suggesting aortic re-gurgitation were obvious in the first two by the age ofi year, and the child whose heart abnormality wasrecognized at the age of 2 was diagnosed initially as a

persistent duct.Two children had recurrent bronchitis in the first

three years and two had increasing effort dyspnoea bythe age of 6 years. Consultation was sought in two be-

Received 24 September I973.

cause the mothers noted the vigorous chest pulsation.No patient had angina.

Clinical examinationThe three children were of normal build, and were re-ferred to the National Heart Hospital at 5, 6, and 7years. All were referred for surgical treatment for sup-posed severe aortic valve disease.

Visible vigorous carotid pulses and a hyperdynamicenlarged left ventricle were constant features. The bloodpressure varied from 140/50 to I50/IO mmHg. A diastolicthrill in the second and third left interspace was noted intwo patients and was also evident to the right of thesternum in one and in the suprasternal notch in another.One patient had an intense systolic thrill while the othershad a short murmur in systole only. All had a loudejection sound at the apex and along the left sternalborder. Aortic valve closure was always clear and thedicrotic notch was obvious on the carotid trace in allthree patients (Fig. i). Pulsation to the right of thesternum was visible and palpable in all three.

Chest radiographyThere was obvious dilatation of the ascending aorta inall patients, and this had been present since infancy intwo (Fig. 2a, b). The left ventricle was enlarged in all,and the 7-year-old boy, who was breathless, hadprominent upper lobe pulmonary veins suggesting pul-monary venous congestion. Two patients had a visiblebulge in the region of the infundibulum below the mainpulmonary artery shadow which was not obvious on allfilms but could be seen on screening to be pulsatingvigorously particularly when the patient was turned intothe right anterior oblique.

ElectrocardiogramsAll had significant increase in left ventricular voltagesuch as would be expected in long-standing aortic re-gurgitation.

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FIG I Phonocardiogram from 8-year-old boy with aorto-left ventricular tunnel showing anintense immediate diastolic murmur (Edm) and a short ejection systolic murmur (Esm) at theleft sternal edge. The dicrotic notch (dn) is obvious on the carotid tracing. An a wave (a) hasbeen recorded on the apex cardiogram taken from the left ventricle.

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(a) (b)F I G 2 Chest radiographsfrom a patient with aorto-left ventricular tunnel. (a) Age 4 weeks show-ing cardiac enlargement and prominence of the ascending aorta which could be confused withthymus gland. (b) Age 6 years showing unusual prominence of the ascending aorta and a bulgebelow the pulmonary area on the left border due to the aneurysmal supracardiac part of the tunnel.

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Aorto-left ventricular tunnel 323

(a) (b)FIG 3 Biplane Elema aortograms from 7-year-old girl with aorto-left ventricular tunnel. (a)Anteroposterior view showing free regurgitation into the left ventricle mainly through the rightside of the aortic valve. The aortic valve ring is dilated and there is some central regurgitationas well. (b) Lateral view. The right anterior aortic sinus is grossly dilated and distorted and thetunnel contributes to the abnormal appearance. Central regurgitation through the aortic valvering is present.

FIG 4 The appearance of the heart and aortic root at operation for aorto-left ventriculartunnel as viewed through a median sternotomy. The dilated and rotated right aortic sinus. Theright coronary artery was 'absent'. The ascending aorta is also dilated.



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InvestigationRight heart catheterization was normal in all. Gradientsof 5 mmHg and I8 mnmHg were recorded across theaortic valve in two and there was no gradient in the other.The striking features were seen on aortography. Two

had aortograms using Elema still films and cineangio-grams were available in two. One patient had both in-vestigations. There was dilatation ofthe right aortic sinuswhich looked abnormal in both planes (Fig. 3). The aorticvalve ring was large in all and aortic regurgitation wasfree. As the reflux of contrast medium was severe it wasdifficult to localize its origin, but in two there was theappearance of a short channel, seen on the lateral view,which appeared to originate from the abnormal rightaortic sinus of Valsalva.

Surgical techniqueIn each patient a median stemotomy and normothermicbypass were used. The external appearance showed un-usual dilatation of the ascending aorta and aortic rootwith a particular localized large bulge anteriorly in theregion of the right aortic sinus which extended over theright ventricular outflow tract or base of the main pul-monary artery (Fig. 4). There was an intense 'to andfro' purring in this, and firm pressure on it abolished thethrill and caused the systolic pressure to rise.On opening the aorta, the aortic valve was seen to be

of tricuspid configuration in all and rotated anticlockwiseas viewed from above so that the commissure betweenthe right and non-coronary cusps was in the sagittalplane. There was thickening of the right and non-coronary cusps, and in one patient there were lacunae atone commissural edge of a deep left coronary cusp. Oneboy had a thick abnormal valve oftricuspid configurationwith minor fusion of the points of commissural attach-ment only. One patient had an absent right coronaryartery and in the others both orifices were normal andseparate from the tunnel enabling coronary perfusion tobe established. Two patients had no coronary perfusion.

In each patient there was an opening at the base of theright aortic sinus (Fig. 5) separate from and adjacent tothe right coronary artery orifice when present. This de-fect, about I cm in diameter, led into an endothelium-lined tunnel which merged into, a dilated ampullabeyond. This in turn opened by a slit-like orifice into theleft ventricular outflow. A sound was placed in the rightcoronary artery to protect it and in one patient theampulla was filled with gel foam and the orifice in theright sinus was patched with pericardium; the leftventricular opening was closed with multiple interruptedooo sutures. In the other two patients repair consisted ofobliteration of the tunnel and closure of the aortic andventricular openings by direct suture. The bypass timewas 3I to 50 minutes and the heart in each patient cameoff bypass without difficulty.

Postoperative courseIn one boy, aged 8, an immediate diastolic murmur wasnoted on the second postoperative day and though he lefthospital with a normal pulse and blood pressure he hadsigns of a moderate aortic regurgitation I8 months later.

Reinvestigation confirmed this to be occurring throughthe centre of the valve. It was decided not to reoperatebecause the heart was smaller than before operation, thepatient was asymptomatic, and valve replacement mighthave been necessary.The two other children, now aged 8 and 9 years, re-

main well, with normal sized hearts and regression of theelectrocardiographic evidence of left ventricular hyper-trophy. One has a soft immediate diastolic murmurnoted two years later.

DiscussionAorto-left ventricular tunnel is a rare anomaly anduntil now only I4 patients have been reported sinceLevy and his colleagues (I963) drew attention tothe condition. As well as these three patients, anothergirl aged i6 with florid aortic regurgitation wasdiagnosed by Dr. Paul Wood as Marfan's syndromeaffecting the right aortic sinus (Fig. 6) and operatedupon elsewhere by Mr. W. Cleland in I962. It wasclear that she had a tunnel which was successfullyclosed, but she died 8 years later and after 2 furtheroperations for severe central aortic regurgitation dueto annular dilatation. Our first patient, in spite ofhis short thick set stature, was thought to have aMarfan aortic pathology because of the severedilatation. Histology of the ascending aorta taken atthe time of operation was normal and his tunnel wassuccessfully closed. The next two patients werecorrectly diagnosed before operation.The diagnosis of a tunnel should be considered in

any infant or young child presenting with signs sug-gesting gross aortic regurgitation; the earlier the ageat which the signs are found the more likely is thisto be the diagnosis, particularly if the murmur isnoted to be loud in the first few months of life. Adiastolic murmur louder than the systolic, and thepresence of a diastolic thrill of maximum intensityat right or left sternal edge are additional featuressuggesting the diagnosis; loud aortic valve closureis evidence of good cusp tissue. Congenital valvarlesions are rarely the cause offree aortic regurgitationunless complicated by infection or rupture and theseevents are unusual in early childhood.Another clinical feature that supports the diag-

nosis of an aorto-left ventricular tunnel is a distinctdicrotic notch in the presence of apparent freeaortic regurgitation, as this is not present in re-gurgitation of valvar origin (personal observation,Dr. Richard Sutton).

Routine chest radiography shows not only re-markable dilatation of the ascending aorta which isseen with other forms of aortic regurgitation but alsodisproportionate dilatation of the right aortic sinuswhich was on occasions visible on the straight chestradiograph. The bulge was not constant and its ap-



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FIG 5 The surgeon's view of the aortic valve (A V)and aortic ostium of the tunnel (T) to the left as seenfrom above through the opened ascending aorta. Theright coronary orifice was absent in the sinus (RS). Theaortic valve had three large cusps with slightly rollededges.

pearance presumably depends on the phase of thecardiac cycle and slight rotation of patient. For thisreason it is recommended that any child with a sus-

pectedtunnelshould be carefullyscreened and turnedto see if this vigorous pulsating bulge can be seen

just below the pulmonary artery shadow. The dilata-tion of the ascending aorta may be present in theneonate and has been confirmed at necropsy (Morton,Murtagh, and O'Hara, I969) and a personally ob-served (J.S.) io-day-old child operated on in anotherunit. This may be confused with an enlarged thymuswhich is commonly seen at this age.Another condition which may present in infancy

with a loud diastolic or 'to-and-fro' murmur is

Fallot's tetralogy with 'absent' pulmonary valve(Osman, Meng, and Girdany, I969). The rightventricular hypertrophy, normal pulse pressure, andabsence of dilatation of the ascending aorta shoulddistinguish the conditions. There may also be diffi-culty in differentiating a ruptured sinus of Valsalvaaneurysm, and even a ventricular septal defect with

FIG 6 Aortogram (AP view) from a i6-year-old girlwith a proven aorto-left ventricular tunnel. The hugedilatation of the aortic sinus is shown on the left cardiacborder.

pronounced aortic regurgitation. However, in ourexperience, patients with a ruptured sinus aneurysmdo not present so early unless there is associatedbacterial endocarditis; this, however, does not occurin infancy and the regurgitation is of acute onsetlater in life. It is likely that the 8-hour-old infantreported by Morton et al. (I969) as having an aneu-rysm of the ventricular septum had in fact an aorto-left ventricular tunnel with aortic valve stenosis. Thetrue anatomical diagnosis of ruptured sinus intoleft ventricle must be extremely rare.

Aortography is necessary to confirm the diag-nosis. The upper part of the right aortic sinus isconstantly large, eccentrically deformed, and pro-jects anteriorly. This should suggest the trueanatomical diagnosis. It is more pronounced butsimilar to the appearance seen in ventricular septaldefect with aortic regurgitation. The intracardiacpart of the tunnel itself may be difficult to define,but, in two, a short localized jet was visible emergingfrom the abnormal right anterior sinus. There wasobvious associated central aortic valve regurgitationin Case i, caused by ring dilatation.

Apart from minor variations, the anatomy hasbeen strikingly similar in each instance. The tunnel



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arises from an aortic ostium high within the rightsinus and about level with the right coronary ostium.Dilatation of the right aortic sinus is always present.Levy and his associates (i963) described the originof the tunnel above the right coronary orifice as oneof the anatomical features which distinguished atunnel from a right coronary sinus aneurysm rup-turing into left ventricle. However, Bove andSchwartz (I967) observed that the relation betweenthe openings of the tunnel and the coronary arterywere not constant, and agreed with Roberts andMorrow (I965) that the value of this criterion fordistinguishing the two conditions was limited. Whenthe right coronary ostium is absent, as in one of ourpatients, this adds to the difficulty.

Inspection beyond the opening shows there is atrue tunnel leading from it, and this supracardiacpart of the tunnel may be aneurysmally dilated.From this, it passes as a wide ampulla-like structurethrough that part of the ventricular septum whichforms the posterior wall of the right ventricularinfundibulum. In so doing it bypasses the aorticvalve and enters the aortic vestibule beneath theintercoronary commissure.Levy et al. (I963) also suggested that aneurysmal

dilatation of the tunnel might be an acquired phe-nomenon. However, there does not appear to be anycorrelation between the presence of such dilatationand the age of the patient. It was seen in a i6-month-old boy (Cooley, Harris, and Rodin, I965) and ispresent in the newborn, so it is likely to be part ofthe developmental anomaly or that the aorta dilatedin fetal life.

In 4 of the 14 patients previously reported,gradients have been demonstrated across the out-flow tract of the right ventricle. It is presumed thatthe gradient results from compression of theposterolateral wall of the infundibulum by thetunnel as it passes through the ventricular septum.This area is well cephalad to the conduction path-ways, and dysrhythmias have not been a feature ofthe condition which is unlike ruptured aneurysm ofthe sinus of Valsalva. It is of interest that twopatients have been reported as having rupturedaortic sinus into left ventricle (Scott, Collins, andSinclair-Smith, I964; Morton et al., I969) andclearly in retrospect had aorto-left ventriculartunnels with the same anatomical features as theother described cases. It is likely that Tasaka et al.(I960) and Warthen (I949) also were describing atunnel and not a ruptured sinus aneurysm.Levy et al. (I963) presented angiographic evidence

that blood flows through the tunnel in systole as wellas during diastole, which is confirmed by cine-angiography in our two patients. This may contributeto the coarse systolic bruits noted in some patients.

In our cases digital compression of the tunnel atoperation regularly obliterated the continuous thrillpalpable over it. At the same time, the systolicpressure rose and the heart rate slowed as withother fistulous communications.

Associated aortic valve abnormalities have beenfound. These may be acquired from free aorticregurgitation stretching the cusps and valve ring asin two of our patients where the cusps were rolledand thickened, or congenital such as a bicuspidaortic valve (Roberts and Morrow, I965) or aorticvalve stenosis which caused the patient's death afterclosure of the tunnel (Cooley et al., I965). Becauseof these abnormalities the patients should be pro-tected from bacterial endocarditis throughout lifeeven in the absence of any haemodynamic dys-function after successful operation.Another feature noted is the anticlockwise

rotation of the aortic valve when viewed fromabove, so the right coronary sinus is directedanteriorly and to the left. This is similar to theappearance of the aortic root in patients with ven-tricular septal defect and aortic regurgitation (Somer-ville, Brandao, and Ross, 1970) and suggests apossible common developmental origin. The angio-cardiographic appearance of the aortic root in thelateral view is also similar in the two conditions, butthe sinus dilatation is greater in the patients with atunnel. Ventricular septal defect with aortic re-gurgitation probably results from a disturbance in theincorporation of the proximal bulbus cordis into theventricle. The bulbus forms not only part of thedistal right and left ventricle (infundibulum andvestibule) and the membranous septum but also thesemilunar valves and roots of the great vessels andsinuses. The tissue in the sinuses of Valsalva isrelatively free of elastic tissue but distal to thebulbar root is the divided truncus and its thickelastic walls. We suggest that the tunnel arisesat the junction of the distal bulbus where it joins thedividing truncus and results from a defect in thatarea. The re-entry into the left ventricle is difficultto explain. It may be as a result of ischaemic com-pression necrosis in the fetus, but the smooth ap-pearance of the endothelialized ampulla is againstthis.

It is of interest that when associated abnormalitieshave been described with a tunnel they are ones thatarise from defective bulbus cordis development,such as pulmonary and aortic valve stenosis. Levyet al. (I963) suggested that the tunnel representedan anomalous coronary artery and that its subse-quent dilatation was an acquired phenomenon. Mor-gan and Mazur (I963) considered that the aorto-leftventricular communication might result from pri-mary separation of the aortic annulus from the



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fibrous skeleton of the heart, while Cooley et al.(I965) believed, on the basis of histological evidence,that the condition was related to Marfan's disease.However, none of these theories accounts for all thepatients described and no other case had histologi-cal changes of Marfan's disease. We think that adisturbance in development and incorporation ofdistal bulbus cordis is more likely though it is notclear how the actual tunnel is formed.

Only five successful operations for this conditionhave previously been reported (Levy et al., I963;Bove and Schwartz, I967; Bernhard, Plauth, andFyler, I970; Fishbone, DeLeuchtenberg, and Stan-sel, I97I). These authors' patients were aged 2 to IIyears. In each instance the only procedure per-formed was closure of the aortic ostium of the tun-nel with or without obliteration of the tunnel. Asin congenital aortic valve stenosis, aorto-left ven-tricular tunnel has a strong male preponderance.Only two female cases have previously been de-scribed and now a further two are added.The importance of distinguishing aorto-left

ventricular tunnel from congenital valvar regurgi-tation lies in the different indications for operationthat exist in the two conditions. In children withvalvar regurgitation, valve replacement is nearlyalways necessary to correct the lesion andoperation should in our opinion be deferred for aslong as is reasonably possible. Conversely, inpatients with aorto-left ventricular tunnel, closureof the tunnel in early childhood is advisable beforesevere dilatation of the aortic annulus has causedsecondary changes in the basically normal aorticvalve. Ideally this should be before 5 years. This issuggested by our patient aged 8 who had persistentcentral aortic regurgitation after the tunnel wasclosed which might not have occurred if it hadbeen corrected earlier. The lesson learnt from thei6-year-old girl, cited earlier, supports this view. Inthe other two cases operated on earlier, good aorticvalve function was evident after closure of the tun-nel and confirmed by postoperative aortography.

This lesion may cause heart failure in infancy anddemand emergency correction at this time. Moreprobably it will present in an asymptomatic childover I year with cardiomegaly and severe aortic re-gurgitation. Tunnel should be the first diagnosis insuch a patient and should be confirmed by aorto-graphy. Early closure of the tunnel should obviatethe need for aortic valve replacement which mayarise if the child is allowed to wait until seriouslysymptomatic or until the second decade.

We are grateful to Professor Reginald Hudson and Dr.Ariela Pomerance for reporting on the histology of the

aortic wall and to Dr. Aubrey Leatham for permissionto include the data on his patient. We thank Dr. RichardSutton for the phonocardiogram reproduced in Fig. i.

ReferencesBernhard, W. F., Plauth, W., and Fyler, D. (1970). Unusual

abnormalities of the aortic root or valve necessitatingsurgical correction in early childhood. New EnglandJournalof Medicine, 282, 68.

Bove, K. E., and Schwartz, D. C. (I967). Aortico-left ven-tricular tunnel. A new concept. American Jtournal ofCardiology, I9, 696.

Cooley, R. N., Harris, L. C., and Rodin, A. E. (I965). Ab-normal communication between the aorta and left ven-tricle. Aortico-left ventricular tunnel. Circulation, 31,564.

Fishbone, G., DeLeuchtenberg, N., and Stansel, H. C. (I97I).Aortico-left ventricular tunnel. Radiology, 98, 579.

Levy, M. J., Lillehei, C. W., Anderson, R. C., Amplatz, K.,and Edwards, J. E. (I963). Aortico-left ventricular tunnel.Circulation, 27, 84I.

Morgan, R. I., and Mazur, J. H. (I963). Congenital aneurysmof aortic root with fistula to left ventricle. A case reportwith autopsy findings. Circulation, 28, 589.

Morton, P., Murtagh, J. G., and O'Hara, M. D. (I969).Aneurysm of interventricular septum with aortic valve mal-formation in an infant. British Heart Journal, 3I, 807.

Osman, M. Z., Meng, C. C. L., and Girdany, B. R. (I969).Congenital absence of the pulmonary valve: report of eightcases with review of the literature. American Journal ofRoentgenology, io6, 58.

Roberts, W. C., and Morrow, A. G. (i965). Aortico-leftventricular tunnel. A cause of massive aortic regurgitationand of intra-cardiac aneurysm. American Jtournal ofMedicine, 39, 662.

Scott, H. W., Collins, H. A., and Sinclair-Smith, B. (I964).Surgical repair of congenital aneurysm ofthe right coronarysinus of Valsalva with rupture into the left ventricle.journal of Cardiovascular Surgery, 5, 23I.

Somerville, J., Brandao, A., and Ross, D. N. (I970). Aorticregurgitation with ventricular septal defect: surgicalmanagement and clinical features. Circulation, 41, 317.

Tasaka, S., Yoshitoshi, Y., Seki, K., Koide, K., Ogata, E., andNakamura, K. (I960). Congenital aneurysm of the rightcoronary sinus of Valsalva with rupture into the left ven-tricle. J7apanese Heart Journal, I, I06.

Warthen, R. 0. (1949). Congenital aneurysm of right anteriorsinus of Valsalva (interventricular aneurysm) with spon-taneous rupture into the left ventricle. American HeartJournal, 37, 975.

Requests for reprints to Dr. Jane Somerville, Cardio-thoracic Institute, 2 Beaumont Street, London WiN2DX.

AddendumSince this paper was submitted a further patient, a managed 26 years, has been operated on for this condition.After the aorta was opened to confirm the diagnosis, theampulla was opened; this was lined by endothelium andwas structurally similar to a dilated aortic sinus ofValsalva. There were two openings from this, one abovethe right coronary cusp opening into the ascending aortaand the lower one obliquely below the right coronary cusp



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in the left ventricle. Thus, there was a deficiency aboveand below the base of the cusp enclosed by the dilatedampullary structure. In addition to this and opposite thelower opening was a jet lesion in the non-coronary cuspwith a perforation; there was no history to suggest bac-terial endocarditis, though six months earlier he had ex-

perienced sudden palpitation and his friends commentedon the purring in his chest probably related to the smallperforation of the cusp. In addition, the anterior cusp ofthe mitral valve was a thin, billowing, sail-like structurewith a web of tissue between the chordae. The cusp wasplicated.

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Documents

Aorto-left ventricular tunnel Clinical.features and ... · Severe aortic regurgitation is well tolerated in the young, andwith the current uncertainty about the long-termfunction