Experience Surgical Treatment Patients with Common Atrio … › content › heartjnl › 29 › 1 › 96.full.pdf · Surgical Treatment ofCommonAtrio-ventricular Canal S;rt.F; (-wePVE

Brit. Heart J., 1967, 29, 96.

Experience in the Surgical Treatment of Patientswith Common Atrio-ventricular Canal

V. I. BOURAKOVSKI, V. A. BUKHARIN, AND F. N. ROMASHOV

From the Institute of Cardiovascular Surgery, Academy of Medical Sciences of the U.S.S.R. (Moscow), U.S.S.R.

The problem of surgical treatment for patientswith common atrio-ventricular canal cannot beconsidered as solved, because of difficulties indiagnosis and complications during surgical inter-vention and in the post-operative period. Thecardinal clinical manifestations of this anomaly havebeen studied and principles of surgical treatmenthave been worked out (Lillehei et al., 1957;McGoon, DuShane, and Kirklin, 1959; Johanssonand Senning, 1959).

In the Soviet Union, investigations are carried outin clinics under N. M. Amosov, A. A. Vishnevski,S. A. Kolesnikov, P. A. Kouprianov, B. V. Petrovski,and others, to overcome the problems connectedwith diagnosis and surgical treatment of this kind ofcardiac defect. (Various works by these authors arelisted in the References.)We have operated on 21 patients with various

forms of common atrio-ventricular canal (by theend of 1963, 400 operations for congenital heartdisease had been carried out on the open heart underconditions of artificial blood circulation, using hypo-thermia and deep hypothermia).

ANATOMICAL TYPEs OF THE DEFECTThere were 11 patients with an incomplete form

of A-V canal: 8 of them were found to have acombination of primary atrial septal defect withcomplete splitting of the septal cusp of the mitralvalve. The primary atrial septal defect wasgenerally 2-5 to 3 cm. in diameter. One patient hadthe primary defect combined with splitting of theseptal cusp of the tricuspid valve: the site of thesplit was where the valve was attached to theseptum, and the small opening led to the leftventricle. Thus, the patient had a left-to-rightshunt of blood in addition to tricuspid regurgitation.

Received July 15, 1965.96

Diagrams illustrating the partial form of commonatrio-ventricular canal are shown in Fig. 1.We have observed considerable diversity in the

anatomical picture among a further 10 patients,operated on for the complete form of this defect.This has allowed us to classify our patients into thefollowing three groups.

(1) A common A-V canal with clear halves of theseptal cusps of the mitral and tricuspid valves, witha large ventricular septal defect, and a primary atrialseptal defect (one case). The split undevelopedcusps of the valves hang like a tent over the muscularedge of the large ventricular septal defect (Fig. 2).

(2) A common A-V canal with well-developedventral and dorsal parts ofthe septal cusps connectedat their lower surface with the edge of the inter-ventricular septum. The edges of both halves ofthe cusps are not connected, and the ventricularseptal defect is a kind of opening, sometimes arather deep one. Over this opening is the primaryatrial septal defect (Fig. 3). This form is thecommonest, and we have observed it in 8 patients.

(3) A common A-V canal without any interatrialseptum (Fig. 4). This form has been seen in onepatient. The disorder in the development of theinterventricular septum and the valve apparatus inthis case was identical with the changes that tookplace in cases in the first group.

Sometimes patients having a common A-V canalare found to have stenosis of the pulmonary arteryor aorta, a persistent ductus, and other malforma-tions of the heart and cardinal vessels. This was soin 5 patients, who were not analysed in this study.

HRMODYNAMIC CHANGES IN THE DEFECTThe hemodynamic disorders of the common

atrio-ventricular canal are caused by septal defectsand regurgitation at the atrio-ventricular valves.

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Surgical Treatment of Common Atrio-ventricular Canal

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(-wePVE s Je - SFIG. 1.-Diagrams illustrating the partial form of the commonA-V canal. Left, splitting of the mitral cusp. Right, splitting

of the medial cusp of the tricuspid valve.

FIG. 3.-The complete form with a small ventricular septaldefect.

The splitting of the mitral valve leads to regurgita-tion of blood into the left atrium, and so to an in-crease in the left-to-right shunt through the atrialseptal defect. In patients with the complete formthere is also a simultaneous shunt of blood throughthe ventricular septal defect. The shunting of alarge quantity of arterial blood to the venous sidecauses, in many cases from the earliest years, severecardiac dilatation, the appearance of pulmonaryhypertension, and insufficiency of blood circulation.The most serious disorders have been observed inpatients in the first and third groups, with the com-plete form of defect. Other patients have similardisorders of circulation but to a lesser degree.The presence of atrial septal defects and regurgita-

tion through the valves distorts the dynamics ofcirculation. First, the cycle of atrial work is dis-torted, and this is reflected in records of atrial andpulmonary capillary pressures; the point "a" is veryhigh in some cases, and at the same time the wave ofregurgitation is often difficult to determine.7

FIG. 2.-The complete form with a large ventricularseptal defect.

_

FIG. 4.-The complete form without any interatrialseptum, combined with a large ventricular septal

defect.

By analogy with the effect of acquired mitralregurgitation on the recorded curves of pressure inthe pulmonary capillaries and left atrium, the pre-sence of regurgitation waves could be expected incases with the common A-V canal. We have seenthis in isolated cases, but generally the wave ofregurgitation was not clearly shown or not shown atall. Wakai and Edwards (1956), among others,point out the rarity of such a sign.The curves of pressure (Fig. 5) recorded in the

atria in patients with the complete form of thecommon A-V canal indicated the regurgitationwave only in the first case (a). In two others(b and c) it was not present. Its rarity can beexplained by the complicated anatomical inter-relations in cases with common A-V canal.

DIAGNOSIS AND CLINICAL FEATURESPatients with a common A-V canal represent one

of the most serious groups among all those with

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Bourakovski, Bukharin, and Romashov

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FIG. 5.-Pressure curves in the atria of patients with the complete form of the cardiac defect.

congenital cardiac defects. In the incomplete formthe clinical picture is not so serious, though wecannot show an exact parallelism between the natureof the anatomical disorders and the clinical andradiological picture. We have not observed anyprevalence in either sex among our patients. Themajority were no older than 10 years and only onewith the complete form was 15 years old. In almostall, the cardiac defect was diagnosed immediatelyafter birth or in the first months of life, and intwo-thirds there were attacks of pneumonia andpulmonary congestion.

All the patients were short of breath. Somechildren with the complete form had a rapid rate ofbreathing, up to 40 a minute, even at rest. Owingto the insufficiency of the blood flow to the systemicvessels, children were physically backward andgenerally inactive. Persistent slight cyanosis of thelips was observed in one patient with the completeform with a large ventricular septal defect. In twoothers with pulmonary hypertension, slight cyanosisappeared only during physical effort.A chest deformity, "a cardiac hump", was found

in 12 patients. The independent systolic murmurat the cardiac apex, which was caused by the mitralregurgitation, and the second systolic sound in thesecond left intercostal space were heard in all cases.Its intensity was not usually great, and it was causedby the large volume of blood passing through thepulmonary valve ring. Besides this, in two-thirdsof our patients the diastolic murmur was loudestover the xiphisternum, and in 4 over the pulmonaryartery. As a rule, the second sound over thepulmonary artery became louder, and in half thepatients it was split.

Arterial hypotonia was present in halfour patients,and various degrees of liver enlargement werefound in two-thirds of them.

Electrocardiographic Examination. This is veryimportant in the diagnosis of common A-V canal.

The changes in the cardiogram are caused by mitralregurgitation and the overcharge of the rightventricle and atrium.The discovery of right bundle-branch block with

clearly pronounced overcharge of the right ventricle,the deceleration of excitement of the myocardium ofthe right ventricle, combined with the overchargeand hypertrophy of the myocardium of the leftventricle, and left axis deviation-all these are typicalof the common A-V canal. Right axis deviationwas noted only in 2 patients with the incompleteform: both were thought to have slight mitralregurgitation. In general, in patients with the in-complete form, the symptoms of overcharge of theright side of the heart are dominant, though this isnot always the case.

Half the patients had prolonged atrio-ventricularconductivity (P-R from 0-20 to 0-24 sec.) because ofclearly pronounced overcharge of the atria and theirdilatation. This prolonged conductivity can also beexplained by the anatomical peculiarities of thedefect.

X-ray Investigations. The serious hemodynamicdisturbances give rise to a typical radiologicalpicture, even in earliest years. The heart is con-siderably enlarged because of the acute dilatation ofall the cardiac chambers (Fig. 6).

Investigation in all three projections allows us todetermine the dilatation of both atria, expecially theright, and pronounced enlargement ofboth ventricles.Functional evidence of mitral regurgitation (yoke-like movements of the left atrium and systolicdisplacement of barium in the aesophagus) was notfound in all cases.The pulmonary trunk and its branches were very

dilated and pulsatile because of a large volumecombined with the mitral regurgitation. The pul-monary picture was intensified in all cases by thefilling of both arterial and venous channels.

It is interesting to note that in 10 cases there were

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also pleural lines-replete lymphatic vessels due tovenous stagnation in the pulmonary circulation.

Naturally, the pulmonary picture changes as pul-monary hypertension, due to sclerosis of thepulmonary vessels, develops. The "amputated"sharp dilated pulmonary vessels are clearly seen. Inthe peripheral sections the impoverishment of thepulmonary picture is shown.

Cardiac catheterization allows us to make a finaldiagnosis. The data obtained by catheterization ofchambers of the heart and vessels are indicated inthe Table. In more than half the patients thecatheterpassed to the left atrium and it was located low overthe A-V valves, probably because of the primaryatrial septal defect.Ten patients had not only an increase in the per-

centage oxygen saturation in the right atrium,but also a similar increase in the right ventricle. Wehave seen this mainly in patients with the completeform, with the blood shunt at the level of both atriaand ventricles.

THE TECHNIQUE OF THE OPERATIONMost patients have been operated on with an artificial

circulation through the trans-sternal access, and the rightantero-lateral approach has been used only twice.

In 9 patients the operation was performed on thenon-contracting heart. In 2 of these, deep commonhypothermia (10°-12°C.) was used, with the blood

FIG. 6.-Radiograph of patient 4 yearsold, with the incomplete form of the cardiac

defect.

circulation arrested. In 2 others, local hypothermia up

to 10°C. was used, with the aorta blocked (clamped), in2 others, with cardioplegia, by citrate ofpotassium, and in3, we operated on the fibrillating heart with the coronaryblood flow continued with an artificial blood circulationwith moderate hypothermia.

In all other cases the correction of the cardiac defectwas done on the contracting heart with extracorporealblood circulation carried out at the normal body tempera-ture of the patient. We conclude that this method is thebest for cases having a common atrio-ventricular canal,because during the process of stitching the edges ofthe defect and tying them it is possible to monitorthe electrocardiogram. We had to abandon all the

BLEPRESSURE AND OXYGEN SATURATION IN CAVITIES OF HEART AND VESSELS

Patient No. Age(yr.)

Complete Form1 132 103 54 75 76 97 118 159 510 8

Incomplete Form11 812 1113 414 615 916 717 818 619 1420 721 13

Data obtained at catheterization

Pressure (mm. Hg) Oxygenation of blood(percentage)

Brachial Right Pulmonary Superior Pulmonaryartery ventricle artery vena cava artery

I.

105/60120/7590/5480/50117/87120/9090/8090/70120/9075/50

120/84112/7593/6896/6690/60120/70105/7080/55100/55100/30108/72

30/054/070/035/045/060/10102/366/096/060/0

34/037/054/333/338/069/045/347/030/037/030/3

29/9

60/27

33/1260/3087

50/2587/4560/30

34/1530/1554/2430/18

36/2045/1544/2230/1522/730/15

76705274757856776567

77666972

578271656662

94938797938782928188

94829186

859387928177

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SX C, MC-

FIG. 7.-Diagram showing the correction of the incomplete form of thedefect.

A B C

FIG. 8.-Operation for the complete form with a small ventricular septal defect (see text).

FIG. 9.-Methods for treating the complete form with a large ventricular septal defect.

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methods of cardioplegia, as it was impossible to watchfor the moment when complete heart block occurred andalso to prevent cardiovascular insufficiency in the periodof restoration. One patient, who was operated on usingcardioplegia by citrate of potassium, was lost because ofthis complication. Two patients who were operated onunder deep hypothermia died because of the acutecardiac insufficiency. All these patients were sufferingfrom a very serious circulatory disturbance, due to theirhaving the complete form of the common A-V canal.We have observed complete heart block in 4 out of 9patients: in all these the cardiac defect was closed on thenon-contracting heart. On the other hand, only one of12 patients who had the correction of defect done on thecontracting heart had this complication. It should benoted that in one other patient in this group completeheart block developed at the moment when the stitch inthe low back part of the defect was tied. After im-mediate removal of the stitch, normal cardiac contract-tions were restored.For the incomplete form of the defect, correction was

carried out by putting in interrupted stitches at theedges of the split valve cusps. We began with the distaledge of the valve and then continued to the interven-tricular septum. The atrial septal defect was closedwith a teflon patch (Fig. 7).

For the complete form ofthe defect (second anatomicalgroup) the best method of operation is the one proposedby Johansson and Senning (1959) (Fig. 8). Using thismethod we operated on 6 patients, and 4 recovered.This operation consists in putting a mattress stitch in thethick part of the interventricular septum, in the zonewhere the defect is. After the stitch is tied, the ventricularseptal defect can be closed and the bases of the split cuspcan be drawn together. The split of the cusp is dealtwith after this by putting in interrupted stitches, and theprimary atrial septal defect is closed with a patch.

In the case of the first and third anatomical varieties(Fig. 9) more complicated surgical intervention shouldbe carried out.

RESULTS

Of 11 patients operated on for the incompleteform of the cardiac defect, only 2 have died (bothhad the combination of the primary defect with asplit cusp of the mitral valve). One girl died whenher heart stopped on the third day after the opera-tion, due to cardiac insufficiency. The death of thesecond child was due to cardiac insufficiency,hypoxia, and secondary hemorrhage.Of 10 patients having the complete form of the

defect, 6 died. Two, who belonged to the first andthird anatomical groups, were suffering from seriouspulmonary hypertension, and one of them also hadpronounced cardiac insufficiency. Both died fromacute cardiac insufficiency (for all of them we usedvarious methods of cardioplegia). One otherpatient died because of cardiac failure in the fourthmonth after operation.

Persistent complete heart block was observed in 3of the patients who recovered. In one, sinus rhythmwas spontaneously restored on the 12th day after theoperation: up to then she had an artificial rhythmproduced by electrostimulation. Two others stillhave complete heart block. In one, the electro-stimulation was continued for 23 days, and in theother for 17 days. At present all the patients feelwell in spite of having complete heart block, and wehave not observed any symptoms of cardiac in-sufficiency.

Eleven patients were examined later, at from 6months to 3 years. The results in 6 of them afterthe correction of the incomplete form of the defectcan be considered good, and they now have no shuntat all. The pulmonary picture became normal, theheart size significantly decreased (Fig. 10), and thesigns of overcharge of the right side of the heart

I . t

FIG. 1O.-Radiographs of a patient before, and 1 year after, the operation for the incomplete form of thecardiac defect.

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disappeared. In only a few patients could the slightsystolic murmur at the cardiac apex be heard, per-haps because of cicatricial deformation of the mitralvalve. There were no symptoms of mitral regurgita-tion.The results obtained by correcting the complete

form of the defect were not nearly so good. Evenwhen the operation resulted in some improvementin the condition of the patients, there were stillgenerally rather clearly pronounced circulatory dis-turbances, basically due to mitral regurgitation.The intensity of the systolic murmur at the apexstill remained to a considerable extent. The signsof overcharge of the left side of the heart were clearlyseen during the radiological examination.The improvements in the circulation were noted in

the radiographs six months after correction of thecomplete form of the common A-V canal. A pro-nounced systolic murmur was still heard at thecardiac apex of these patients, but the pressure inthe right ventricle decreased from 60 to 30 mm. Hg,and the left-to-right shunt was abolished.

CONCLUSIONWe have studied and operated on 21 patients with

a common atrio-ventricular canal. The investiga-tions showed the diversity of forms of the cardiacdefect, especially among patients with the completetype.Heemodynamic disorders were serious in all the

patients, and included the shunting of blood fromthe left to the right atrium and from the left ventricleto the right side of the heart, and, in addition, mitralregurgitation. These disorders were particularlyserious in the patients with either (a) a common A-Vcanal with clear halves of the septal cusps of themitral and tricuspid valves, with a large ventricularseptal defect, and a primary atrial septal defect, or(b), a common A-V canal without any interatrialseptum.The clinical and radiological picture of the cardiac

defect as well as the cardiographic data are patho-gnomonic.

It is best to operate on the contracting heart withan artificial blood circulation at normal bodytemperature. The operation for the patients withthe incomplete form consists in repair of the splitcusps of the mitral valve and in closing the primaryatrial septal defect with a teflon patch. In thosewith a common A-V canal with well-developedventral and dorsal parts of the septal cusps con-nected at their lower surface with the edge of the

interventricular septum, the ventricular septal defectshould be closed with the help of the stitch sug-gested by Johansson and Senning (1959). In theanatomical varieties (a)and (b) (above) the ventricularseptal defect should be closed with a separate patch.The later results in cases with the incomplete

form of cardiac defect are good, the patients be-coming normal healthy people. In those with thecomplete form of the defect the results are not sogood because we have been unable to cure the mitralregurgitation.

For a number of patients in the last group we stillhave to search for new methods of closing theventricular septal defect and correcting the mitralregurgitation.

REFERENCESAmosov, N. M., Lissov, J. L., Sidarenko, L. N., and Treshin-

ski, A. M. (1962). Cardiac operations using artificialblood circulation. Medgiz, Kiev.

Bourakovski, V. I., Bukharin, V. A., Romashov, F. N., andSu-Chzhun, Huan (1963). Classification, clinics,diagnostics and surgical treatment of the complete formof the atrio-ventricular canal. Vestnik Academy ofMedical Science, U.S.S.R., 9, 32.

-, and Romashov, F. N. (1962). Indications for theoperation and choosing the methods in cases with theinteratrial septal defect. Chest surgery, No. 1, 3.

Johansson, L., and Senning, A. (1959). Surgical correctionof atrial septal defects. Acta chir. scand., Suppl. 245,p. 283.

Kolesnikov, S. A. (1961). Operations on the open heart underconditions of artificial blood circulation. Reports of theChest Surgery Institute, VII. Medgiz, Kiev.

Kuprianov, P. A. (1962). The blood circulation in surgicaltreatment of the heart and main vessels. Medgiz, Kiev.

Lillehei, C. W., Warden, H. E., DeWall, R. A., Stanley, P.,and Varco, R. L. (1957). Cardiopulmonary by-pass insurgical treatment of congenital or acquired cardiacdisease. Use in 305 patients. Arch. Surg., 75, 928.

McGoon, D. C., DuShane, J. W., and Kirklin, J. W. (1959).The surgical treatment of endocardial cushion defects.Surgery, 46, 185.

Petrovski, B. V., Soloviev, G. M., Bunatjn, A. A., Chodas,M. J., Osipov, V. P., Zargarli, F. I., and Shumakov, V. I.(1962). Hypothermal perfusion during the operationson the open heart. Report at the conference of thecardiovascular surgery link, Moscow, December 1962.

Romashov, F. N. (1963). Defects of interatrial septum andtheir surgical treatment. Khirurgiya (Mosk.), 39, No. 2,p. 70. [In Russian-Eng. Summary.]

Scott, L. P., Hauck, A. J., Nadas, A. S., and Gross, R. E.(1962). Endocardial cushion defect. Preoperative andpostoperative survey. Circulation, 26, 218.

Vishnevski, A. A., and Galankin, N. K. (1962). Congenitalcardiac defects and defects of big vessels. Medgiz, Kiev.

Wakai, C. S., and Edwards, J. E. (1956). Developmental andpathologic considerations in persistent common atrio-ventricular canal. Proc. Mayo Clin., 31, 487.

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Experience Surgical Treatment Patients with Common Atrio … › content › heartjnl › 29 › 1 › 96.full.pdf · Surgical Treatment ofCommonAtrio-ventricular Canal S;rt.F; (-wePVE