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The surgical anatomy of truncus arteriosus communis
Volume 67, Number 4
The Journal of
April
1974
THORACIC AND
CARDIOVASCULAR SURGERY The surgical anatomy of truncus arteriosus communis ln a classical work, Collett and Edwards" classified truncus according to the manner of origin of the pulmonary tree. We believe that, from the surgical standpoint, more factors should be taken into account in evaluating each heart. Clinical, catheterization, and angiographic examination should be used to clarify the site of origin of the truncus, the amount of pulmonary flow, the architecture of the truncal valve and truncal vascular tree, the coronary circulation, the size of the ventricular septal defect, and the presence or absence of associated cardiac and extracardiac abnormalities. All these factors may have a bearing on the suitability of a candidate for complete correction, or they may influence the prognosis of surgical therapy.
Saroja Bharati, M.D., Hugh A. McAllister, Jr., M.D., Glenn C. Rosenquist, M.D., Robert A. Miller, M.D., Constantine J. Tatooles, M.D., and Maurice Lev, M.D., * Chicago, Ill., Baltimore, Md., Washington, D. C., and London, England
From the Congenital Heart Disease Research and Training Center, Hektoen Institute for Medical Research; the Departments of Pathology-Northwestern University Medical School; Pritzker School of Medicine, University of Chicago; The Chicago Medical School, University of Health Sciences; and Loyola University, Stritch School of Medicine; Departments of Pathology and Medicine, Abraham Lincoln School of Medicine, University of Illinois; Pediatric Cardiology and Thoracic Surgery Departments, Cook County Hospital, Chicago, Ill.; Departments of Pediatrics and Pathology, The Johns Hopkins Hospital. Baltimore, Md.; the Armed Forces Institute of Pathology, Washington, D. C.; and the Hospital for Sick Children, London, England. Aided by Grant HL 07605-12 from the National Institutes of Health, National Heart and Lung Institute, Bethesda, Md. The opinions or assertations contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense. Received for publication Nov. 29, 1973. Address for reprints: Dr. M. Lev, 637 S. Wood Street, Chicago, Ill. 60612. 'Career Investigator and Educator, Chicago Heart Association.
Truncus arteriosus communis is today amenable to corrective surgery.':" It is therefore necessary to re-evaluate the pathological anatomy of this condition to meet the surgeon's needs. Truncus arteriosus communis is that condition in which only one arterial trunk emerges from the heart. This trunk gives rise to the coronary, the systemic, and the pulmonary circulations. It is to be differentiated from pseudotruncus and from truncus solitarius pulmonalis. In both of these conditions, an aortic and a pulmonary trunk emerge from the heart; however, in the former there is pulmonary atresia, while in the latter there is aortic atresia. We have examined 180 hearts with truncus communis as defined above. Eighty501
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the status of the spleen, and (12) the status of the ductus. Findings
Fig. 1. Truncus arteriosus communis. Usual architecture of the musculature of the outflow tract of the right ventricl e. Note the thickened truncal valve, which was insufficient. S" Superior prong of the septal band. S" Inferior prong of the
septal band. P, Parietal band. T, Truncus. B, Banding procedure. V, Ventri cular septal defect.
three hearts were supplied by the Congenital Heart Disea se Research and Training Center, 66 by the Armed Forces Institute of Pathology, 17 by the Hospital for Sick Children, London, England, and 14 by the Helen B. Taussig Children's Cardiac Center, Th e Johns Hopkins Hospital. These form the basis of the present report on the surgical anatomy. Method of examination These hearts were examined according to the following factors: (I) the site of origin of the truncus, (2) the mann er of origin of the pulmonary tre e," ( 3 ) the amount of pulmonary flow, (4) the nature of the truncus valve, (5) the presence or absence of truncal stenosis or insufficienc y, (6 ) the qu estion of hypoplasia of the aortic tree , (7) the coronary distribution, (8) the presence or absence of ventricular septal defect stenosis, (9) associated cardiac abnormalities, (10 ) associated extracardiac abnormalities, (II)
The complex formed by truncus communis. In the specimens that we examined, the heart was usually enlarged. The apex was form ed by the left or both ventricle s. Hyp ertrophy of the right atrium and ventricle was present in each case , with increased hemodynamic changes in the tricuspid valve. "Mitralization" of the tricuspid valve (that is, a tendency of the leaflets to be altered to bicuspidali ty) was noted in 46 cases. In specimens in which pulmonary flow had been increased, the left atrium and left ventricle were hypertrophied and enlarged , and there were increased hemodynamic changes in the mitral valve. The ventricular septal defect was usually large and situated in the anterior (conal) septum, that is, anterior to the pars membranacea, involving the membrane in part. The architecture of the musculature of the right ventricle in all but 5 cases was as follows: The septal band extended to the base and bifurcated into two prongs, usually a more robust superior prong and a smaller inferior prong (Fig. I ) . Occasionally, the two prongs were of equal size. Between these prongs lay the ventricular septal defect. Th e parietal band group extended downward over the parietal wall in close relationship to the anterior leaflet of the tricuspid valve. In some case s it did not meet the septal band, whereas in most others it established only a tenuous connection. In thos e hearts in which the inferior prong of the septal band was more robust, the connection was more extensive. Thus either no arch was form ed by the septal or parietal bands or else the arch was markedly excavated. In the 5 exceptional cases , the septal and parietal bands formed a distinct arch and the ventricula r septal defect lay beneath it (Fig. 2) . Factorial analysis Site of origin of the truncus. The truncus arose mostly from the right ventricle in 117
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Fig. 2. Atypical truncus arteriosus communis. The truncus emerges completely from the right ventricle. A, The septal and parietal bands form a distinct arch beneath it. B, The right pulmonary artery emerges from the innominate and C, the left pulmonary artery from the ductus. S, Septal band. RP, Right pulmonary artery. I, Innominate artery. LP, Left pulmonary artery a continuation of the ductus going to the left lung. For other abbreviations, see Fig. 1.
cases (66.5 per cent) and entirely from the right ventricle in 25 cases (14.2 per cent). The truncus straddled the ventricular septum, emerging equally from both ventricles, in 29 cases (16.5 per cent), whereas it arose mostly from the left ventricle in 5 cases (2.8 per cent). In 2 hearts the origin could not be ascertained due to total correction. In I case the truncus arose from a single ventri-
cleo In another, in which there was tricuspid atresia, it arose completely from the left ventricle. The manner of origin of the pulmonary tree. There were 98 Type I cases (57.6 per cent), 58 Type II cases (34.1 per cent), 10 Type III cases (5.9 per cent), and 4 Type IV cases (2.4 per cent), according to the classification of Collett and Edwards."
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Table
I.
Case No.
Patterns
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Right pulmonary blood supply
Left pulmonary blood supply
5
Right pulmonary artery absent; right lung supplied by bronchials Right pulmonary artery and vein absent; right lung absent Right pulmonary artery a continuation of right ductus (right aortic arch) Right pulmonary artery from innominate artery Right pulmonary artery from truncus
6
Right pulmonary artery from truncus
7
Right pulmonary artery from truncus
8
Right pulmonary artery from truncus
9
Ma in pulmonary artery from anterior part of truncus Right pulmonary artery absent; supplied by bronchials
2 3
4
10
Left pulmonary artery from transverse aorta (by way of ductus?) Left pulmonary artery and vein absent; left lung absent Left pulmonary artery from truncus Left pulmonary artery a continuation of left ductus Left pulmonary artery from transverse aorta adjacent to obliterated ductus arteriosus Left pulmonary artery a continuation of left ductus Left pulmonary artery a continuation of left ductus Left pulmonary artery absent; lung supplied by bronchials
Left pulmonary artery from truncus
Fig. 3. Truncus arteriosus communis, Type I (window type) . RA , Right aortic arch. PT, Pulmonary trunk.
Type I. The pulmonary trunk was usually very short and, with the exception of 4 specimens, low in origin. The shortness of .the pulmonary trunk made a distinction between Type I and Type II difficult. In I heart the main pulmonary trunk was confluent with the posterior sinus of Valsalva. Also, there were 6 cases which may be con-
Fig. 4. Truncus arteriosus communis, Type II , with the left pulmonary artery arising above the right. LP, Left pulmonary artery. For other abbre viations, see Figs. I to 3.
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Fig. S. Truncus arteriosus communis, Type III, with narrowing of right pulmonary artery. RC, Right coronary ostium. LC, Left coronary ostium. RV, Right ventricle. For other abbreviations, see Figs. 1 to 3.
sidered a subgroup of this type. In these hearts, a septum hung down between the pulmonary trunk and aortic portion of the truncus . This might be called the "window" type of truncus (Fig. 3). Type II. The two pulmonary arteries arose close to each other posteriorly, close to the truncal valve, or posteriorly and to the left. In 7 cases, the left artery arose above the right (Fig. 4). In 4 cases, the two pulmonary arteries arose high above the truncal valve. Type II I. In 4 specimens the pulmonary arteries arose just above the truncal valve, and in 4 others they were higher (Fig. 5). In 1 specimen only the right pulmonary artery was high, while in another, with agenesis of the right lung, the left pulmonary artery arose high above the truncal valve on the left side. In addition to the above distinct types, there were 10 cases which could not be categorized (Table I) (Fig. 2) . In 8 of these cases the blood supply of one lung was from
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Fig. 6. Truncus arteriosus communis. Main pulmonary trunk arising from the anterior part of the truncus. RV, right ventricle. For other abbreviations, see Figs. I to 3.
the truncus, while that of the other lung was from a ductus, an innominate vessel, or the bronchial circulation. In the ninth case, the pulmonary trunk arose from the anterior part of the truncus (Fig. 6) . In the tenth case both lungs were absent, and hence the pulmonary arteries and veins were absent . Amount of pulmonary ftow. As gauged by the size of the left atrium and ventricle, the pulmonary flow had been increased in 137 cases (86.2 per cent), decreased in 18 cases (11.3 per cent), and normal in 4 cases (2 .5 per cent). In 21 specimens no statement could be made. The truncus valve. The truncus valve was tricuspid in 121 cases (68 per cent), although in 3 hearts the cusps were incompletely divided . The valve was quadricuspid in 45 specimens (25 .3 per cent), although incompletely divided in 9. It was bicuspid in 12 (6.7 per cent) and pentacuspid in I case. The valve could not be interpreted in another heart due to sectioning. The bicuspid and quadricuspid valves were more thickened and nodular than the tricuspid valves. The valve was markedly thickened even in infancy in many cases, and there did not seem to be any relationship between the
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Fig, 8. Truncus arteriosus communis, Type I. Origin of the left coronary from the pulmonary trunk. For abbreviations, see Figs. I to 5. Fig. 7. Truncus arteriosus communis, Type I, with truncus stenosis and possible ventricular septal defect stenosis. The pulmonary trunk arises in a sinus of Valsalv a. Note the poststenotic dilatation. For abbreviations, see Figs. I to 5.
thickening of the valve and the age of the patient. In specimens with multiple extracardiac congenital malformations, the valve often was thickened. Truncal stenosis and insufficiency. Truncal stenosis was noted in 10 cases (5 .6 per cent) (Fig. 7) , and there was anatomic evidence of truncal insufficiency in 4 (2.2 per cent) (Fig. 1) . Hypoplasia of the aorta in truncus arteriosus. In this anomaly, the truncus gives rise to a hypoplastic aorta, with either moderate (1 case) or severe (4 cases) fetal coarctation, atresia of the aortic arch (1 case), or complete interruption of the aortic arch (20 cases, 11. I per cent). The coronary circulation. There was 11 cases of single coronary artery (in 6 the artery arose above the sinus of Valsalva) , 14 cases of origin of both coronary arteries above the sinus of Valsalva , 42 cases of high origin of the left coronary artery alone ( Fig.
5), and 5 cases of high origin of the right coronary artery alone. The coronary distribution was studied in 83 hearts. Regardless of whether the valve was bicuspid, tricuspid , or quadricuspid, the left coronary art ery originated from a cusp situated posteriorly. The artery curved widely around the base of the truncus to the left, below (caudal to) the pulmonary arteries. In many cases, it coursed along the anterior wall of the left ventricle to the left of the anterior interventricular groove, while the anterior descending artery extended to the left of the groove, reaching the latter only in its distal course. The right coronary art ery usually arose from a cusp situated anteriorly, anteriorly and to the right, or anteriorly and to the left. It gave off robust branches to the anterior wall of the right ventricle; these vessels were usually larger than normal, sometimes larger than the anterior descending coronary artery . There were 6 specimens worthy of note. In 1 case, the left coronary artery arose above the sinus of Valsalva , while the common pulmonary trunk arose in the sinus
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(Fig. 7). In 4 other cases the origin of the left coronary was from the main pulmonary trunk, about I em. above the truncal valve (Fig. 8), In the sixth, two left coronary arteries arose from the main pulmonary trunk. Ventricular septal defect stenosis. The ventricular septal defect was stenotic in 6 cases (3,3 per cent) and absent in I case. Associated cardiac abnormalities. These anomalies are given in Table II. The following were common: right aortic arch, 34 cases (18.9 per cent); abnormal brachiocephalic vessels, 24 cases (13.3 per cent); atrial septal defect, fossa ovalis type, 36 cases (20.0 per cent); mild tricuspid stenosis, I 1 cases (6, I per cent); and entrance of the left superior vena cava into the coronary sinus, 8 cases (4.4 per cent). Associated extra cardiac abnormalities and the status of the spleen. Ninety-five specimens (67.4 per cent) had normal situs, a normal (89) or accessory (6) spleen, and no extracardiac abnormalities, Among these there was 1 case of dextroversion and 1 case of mixed dextrocardia. On the other hand, 42 specimens (29.8 per cent) had multiple extracardiac abnormalities with a normal (38) or accessory (4) spleen. There was 1 example each of the following combinations: isolated levocardia with partial visceral heterotaxy, multiple extracardiac congenital abnormalities, and a normal spleen; normal situs, multiple congenital abnormalities, and a multilobated spleen; dextrocardia with complete situs inversus, multiple extracardiac congenital abnormalities, and asplenia; and dextrocardia with partial situs inversus, multiple congenital abnormalities, and asplenia. Information was not available in the remaining cases. Status of the ductus arteriosus. The ductus was closed in 24 cases (13.3 per cent), absent in 40 (22.2 per cent), closing in 2, and patent in 30 cases (16.7 per cent). The status of the ductus could not be ascertained in the other specimens. Age and sex. The specimens were obtained from 90 male and 87 female subjects. No information was available in the re-
Table II. Associated cardiac abnormalities Abnormality Right aortic arch Aberrant origin of right subclavian Aberrant origin of left subclavian Origin of left subclavian from ductus Origin of right subclavian from descending 6 aorta 1 Common brachiocephalic trunk Aberrant left subclavian posterior to esophagus Aberrant right subclavian posterior to esophagus Aberrant origin of left vertebral from 2 aortic arch 2 Left innominate 1 Aberrant origin of brachiocephalic vessel 36 Atrial septal defect 1 Tricuspid insufficiency 11 Tricuspid stenosis 1 Almost complete absence of atrial septum Left superior vena cava emptying into 8 coronary sinus 1 Coronary sinus opening to both atria Total anomalous pulmonary venous 2 drainage 3 Common atrioventricular orifice Dextrocardia (type undetermined) with common atrioventricular orifice Dextrocardia with atria inverted, ventricles pivoted, and atrial septal defect; common atrioventricular orifice; bilateral superior vena cava; total anomalous pulmonary venous drainage into abdomen; absent coronary sinus with asplenia and partial situs inversus Mixed dextrocardia with total anomalous pulmonary venous drainage into azygos Dextroversion Mesocardia Tricuspid atresia Mitral atresia Single ventricle with straddling conus Abnormal mitral valve Straddling tricuspid value with ventricular septal defect (common atrioventricular 3 orifice) 1 Adult coarctation 1 Aneurysm of the fossa ovalis Premature narrowing of foramen ovale with absent ventricular septal defect and hypoplastic mitral valve Partial anomalous pulmonary venous drainage with sinus venosus and atrial septal defect Diverticulum of right ventricle
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maining cases. In the 177 cases in which the age was known, the range was from stillbirth to 19 years, with a mean of 6 months, 11 days.
Discussion Truncus communis has been well studied in the literature. 1-4'1 The modern work on this subject begins with the classical study by Collett and Edwards." These authors laid their main stress on the manner of origin of the pulmonary trunk or arteries. In their Type I, a main pulmonary trunk was present. In Type II the trunk was absent, and two pulmonary arteries emerged from the posterior or posteroleft aspect of the truncus close to each other. In Type III the pulmonary arteries arose laterally, and in Type IV the pulmonary trunk and arteries were absent, and the bronchial arteries supplied the lungs. All these types had subtypes related to other factors which we have discussed. The state of the pulmonary tree as depicted by these authors is obviously very important in total correction. However, we must emphasize that in our material there is no sharp line of demarcation between Types I and II. In most of our cases the main trunk in Type I was very short, so that for practical purposes these specimens could just as well be designated Type II. We further believe that other factors must be considered in evaluating candidates for total correction. The first of these is the origin of the truncus. In the majority of our series, the truncus arose mostly from the right ventricle. This fact might be of importance in the surgical transfer of the aorta into the left ventricle. We H have called attention to a possible hazard in the creation of subaortic stenosis in double-outlet right ventricle with a subaortic ventricular septal defect. A similar hazard might have to be entertained here. In this context, the question of stenosis of the left ventricular outflow tract at the ventricular septal defect cannot be overlooked. The necessity of surgical enlargement of the ventricular septal defect must be evaluated.
The nature of the truncus valve may likewise have some importance in surgery. Although it is most often tricuspid, a sizeable number of cases have bicuspid or quadricuspid valves. All three types of valves, but especially the latter two, tend to become thickened and are in some cases insufficient or stenotic. The immediate effect of stenosis at the time of surgery, as well as the longterm postsurgical effects of these thickened valves, must be taken into account by the surgeon. A sizeable number of cases in our series have associated extracardiac abnormalities similar to those found in trisomy 18 and 13 to 15. However, chromosomal studies were not done. The question of the immediate and long-term surgical prognosis of these cases must be evaluated. The surgical importance of increased or decreased pulmonary flow is obvious. Likewise, the coronary circulation is of importance to the surgeon. Origin of the left coronary artery from the posterior aspect of the truncus, close to the origin of the pulmonary tree, may pose certain surgical problems. In 1 case in our collection, the surgical sutures used to remove the pulmonary tree from the truncus were directly adjacent to the mouth of the left coronary artery. Another possible source of difficulty is the branches of the right circumflex that extend over the anterior wall of the right ventricle. If they are markedly enlarged, they may be of some importance in the ventriculotomy. Hypoplasia of the aorta in truncus should be mentioned briefly. Although in general this anomaly does not permit long survival, there is a case in the literature of survival to adult life H and another case of total correction." The frequent presence of a right aortic arch and other brachiocephalic vascular anomalies, an atrial septal defect, a left superior vena cava entering the coronary sinus, as well as the rare association with other cardiac complexes, deserves attention. The 10 cases in which the blood supply to the lungs came from two sources are of note. In some, one lung was supplied by a pul-
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monary artery arising from the truncus, from the ductus, from the innominate artery, while the other was supplied by the bronchial or an opposite ductus. Collett and Edwards" believed that "truncus" is an improper designation for cases in which a ductus gives rise to a pulmonary artery, They felt there was a possibility that a main pulmonary trunk might have been present but had disappeared. Nevertheless, we feel that, anatomically, it is better to call all these cases atypical truncus communis. We wish to thank the staff of the Thoracic Unit, The Hospital for Sick Children, Great Ormond Street, London, for permission to review and publish their specimens. Acknowledgement is also made to Florence Kotal for her help in the preparation of the manuscript.
REFERENCES
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McGoon, D. C., Rastelli, G. C., and Ongley, P. A.: An Operation for the Correction of Truncus Arteriosus, J. A. M. A. 205: 69, 1968. Weldon, C. S., and Cameron, J. L.: Correction of Persistent Truncus Arteriosus, J. Cardiovase. Surg. 9: 463, 1968. Wallace, R. B., Rastelli, G. C., Ongley, P. A., Titus, J. L., and McGoon, D. C.: Complete Repair of Truncus Arteriosus Defects, J. THoRAc. CARDIOVASC. SURG. 57: 95, 1969. Rogers, M. A., Winship, W. S., and Coleman, A. J.: Correction of Persistent Truncus Arteriosus, Thorax 26: 115, 1971. Brawley, R. K., Gardner, T. J., Donahoo, J. S., Neill, C. A., Rowe, R. D., and Gott, V. L.: Late Results After Right Ventricular Outflow Tract Reconstruction With Aortic Root Homograft, J. THORAC. CARDIOVASC. SURG. 64: 314, 1972. McGoon, D. C., Wallace, R. B., and Danielson, G. K.: Homografts in Reconstruction of Congenital Cardiac Anomalies: Expanded Operability in Complex Congenital Heart Disease, Mayo Clin. Proc. 47: 101, 1972. McGoon, D. C.: Advances in Surgical Treatment of Congenital Heart Disease, Proc. Inst. Med. Chic. 29: 97, 1972. Gomes, M. M. R., and McGoon, D. C.: Truncus Arteriosis With Interruption of the Aortic Arch: Report of a Case Successfully Repaired, Mayo Clin. Proc. 46: 40, 1971. Collett, R. W., and Edwards, J. E.: Persistent Truncus Arteriosus: A Classification According to Anatomic Types, Surg. Clin. North Am. 29: 1245, 1949.
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17 Smith, G. W., Thompson, W. M., Jr., and Dammann, J. F., Jr.: Use of the Pulmonary Artery Banding Procedure in Treating Type II Truncus Arteriosus, Circulation 29: 108, 1964 (Supp!. I). 18 Dunkin, D. E.: Persistent Truncus Arteriosus With Dextropositioned Arch and Widely Divergent Origins of the Pulmonary Arteries: Case Report, J. Am. Osteopath. Assoc. 63: 558, 1964. 19 McCue, C. M., Lester, R. G., Bosher, L. H., Jr., and Mauck, H. P., Jr.: Persistent Truncus Arteriosus: A Clinical Correlation With the Pathologic Anatomy, Chest 46: 507, 1964. 20 Steiner, P., and Finegold, M. J.: Truncus Arteriosus With Atresia of the Aortic Arch, Arch. Patho!' 79: 518, 1965. 21 Van Praagh, R., and Van Praagh, S.: The Anatomy of Common Aorticopulmonary Trunk (Truncus Arteriosus Communis) and Its Embryologic Implications: A Study of 57 Necropsy Cases, Am. J. Cardio!. 16: 406, 1965. 22 Morgan, A. D., Brinsfield, D., and Edwards, F. K.: Persistent Truncus Arteriosus: An Unusual Variant With Atresia of the Aortic Arch, Am. J. Dis. Child. 109: 74, 1965. 23 Gasul, B. M., Arcilla, R. A., and Lev, M.: Heart Disease in Children, Philadelphia, 1966, 1. B. Lippincott Company, chap. 24, p. 617. 24 Hicken, P., Evans, D., and Heath, D.: Persistent Truncus Arteriosus With Survival to the Age of 38 Years, Br. Heart 1. 28: 284, 1966. 25 Silverman, J. J., and Scheinesson, G. P.: Per-
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Arteriosus Malformation in a Human Embryo, Am. J. Anal. 134: 167, 1972. Oldham, H. N., Jr., Kakos, G. S., Jarmakani, M. M., and Sabiston, D. S.: Pulmonary Artery Banding in Infants With Complex Heart Defects, Ann. Thorac. Surg. 13: 342, 1972. Morgan, J. R., Kearney, D. 1., and Hagan, A. D.: Type I Truncus Arteriosus in an Adult, Chest 62: 501, 1972. Billig, D. M., Kreidberg, M. B., Chernoff, H. L., and Ali Khan, M. A: Systemic to Pulmonary Anastomosis in Truncus Arteriosus With Reduced Pulmonary Flow, Am. J. Cardio!. 30: 288, 1972. Klinkhamer, A. c, and Verheught, A P. M.: Persistent Truncus Arteriosus and Pseudotruncus Arteriosus, Radio!. Clin. Bio!. 41: 36, 1972. Nadal-Ginard, B., Malpartida, F., and EspinoVela, 1.: Ventricular Inversion With Common Truncus, Arch. Inst. Cardio!. Mex. 42: 181, 1972. Testelli, M. R.: Truncus Arteriosus Communis With Interruption of the Aortic Arch: Report of a Case With Survival to Adult Age, Arch. Inst. Cardio!. Mex. 42: 122, 1972. Sundararajan, V., and MoIthan, M. E.: Truncus Arteriosus (Type II) Associated With Interruption of the Aortic Arch (Type B): Case Report of a 3-Year-Old Child, Am. J. Dis. Child. 123: 494, 1972. Farnsworth, P. B., Lefkowitz, M., Shehadi, W., and Chan, T. T.: Spontaneous Rupture of Fibrous Diverticulum of the Right Ventricle: Occurrence in an Infant With Persistent Truncus Arteriosus, Am. J. Dis. Child. 123: 248, 1972. Lev, M., Bharati, S., Meng, C. C. L., Liberthson, R. R., Paul, M. H., and Idriss, F.: A Concept of Double-Outlet Right Ventricle, J. THORAC. CARDIOVASC. SURG. 64: 271, 1972.
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THORACIC AND
CARDIOVASCULAR SURGERY The surgical anatomy of truncus arteriosus communis ln a classical work, Collett and Edwards" classified truncus according to the manner of origin of the pulmonary tree. We believe that, from the surgical standpoint, more factors should be taken into account in evaluating each heart. Clinical, catheterization, and angiographic examination should be used to clarify the site of origin of the truncus, the amount of pulmonary flow, the architecture of the truncal valve and truncal vascular tree, the coronary circulation, the size of the ventricular septal defect, and the presence or absence of associated cardiac and extracardiac abnormalities. All these factors may have a bearing on the suitability of a candidate for complete correction, or they may influence the prognosis of surgical therapy.
Saroja Bharati, M.D., Hugh A. McAllister, Jr., M.D., Glenn C. Rosenquist, M.D., Robert A. Miller, M.D., Constantine J. Tatooles, M.D., and Maurice Lev, M.D., * Chicago, Ill., Baltimore, Md., Washington, D. C., and London, England
From the Congenital Heart Disease Research and Training Center, Hektoen Institute for Medical Research; the Departments of Pathology-Northwestern University Medical School; Pritzker School of Medicine, University of Chicago; The Chicago Medical School, University of Health Sciences; and Loyola University, Stritch School of Medicine; Departments of Pathology and Medicine, Abraham Lincoln School of Medicine, University of Illinois; Pediatric Cardiology and Thoracic Surgery Departments, Cook County Hospital, Chicago, Ill.; Departments of Pediatrics and Pathology, The Johns Hopkins Hospital. Baltimore, Md.; the Armed Forces Institute of Pathology, Washington, D. C.; and the Hospital for Sick Children, London, England. Aided by Grant HL 07605-12 from the National Institutes of Health, National Heart and Lung Institute, Bethesda, Md. The opinions or assertations contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense. Received for publication Nov. 29, 1973. Address for reprints: Dr. M. Lev, 637 S. Wood Street, Chicago, Ill. 60612. 'Career Investigator and Educator, Chicago Heart Association.
Truncus arteriosus communis is today amenable to corrective surgery.':" It is therefore necessary to re-evaluate the pathological anatomy of this condition to meet the surgeon's needs. Truncus arteriosus communis is that condition in which only one arterial trunk emerges from the heart. This trunk gives rise to the coronary, the systemic, and the pulmonary circulations. It is to be differentiated from pseudotruncus and from truncus solitarius pulmonalis. In both of these conditions, an aortic and a pulmonary trunk emerge from the heart; however, in the former there is pulmonary atresia, while in the latter there is aortic atresia. We have examined 180 hearts with truncus communis as defined above. Eighty501
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the status of the spleen, and (12) the status of the ductus. Findings
Fig. 1. Truncus arteriosus communis. Usual architecture of the musculature of the outflow tract of the right ventricl e. Note the thickened truncal valve, which was insufficient. S" Superior prong of the septal band. S" Inferior prong of the
septal band. P, Parietal band. T, Truncus. B, Banding procedure. V, Ventri cular septal defect.
three hearts were supplied by the Congenital Heart Disea se Research and Training Center, 66 by the Armed Forces Institute of Pathology, 17 by the Hospital for Sick Children, London, England, and 14 by the Helen B. Taussig Children's Cardiac Center, Th e Johns Hopkins Hospital. These form the basis of the present report on the surgical anatomy. Method of examination These hearts were examined according to the following factors: (I) the site of origin of the truncus, (2) the mann er of origin of the pulmonary tre e," ( 3 ) the amount of pulmonary flow, (4) the nature of the truncus valve, (5) the presence or absence of truncal stenosis or insufficienc y, (6 ) the qu estion of hypoplasia of the aortic tree , (7) the coronary distribution, (8) the presence or absence of ventricular septal defect stenosis, (9) associated cardiac abnormalities, (10 ) associated extracardiac abnormalities, (II)
The complex formed by truncus communis. In the specimens that we examined, the heart was usually enlarged. The apex was form ed by the left or both ventricle s. Hyp ertrophy of the right atrium and ventricle was present in each case , with increased hemodynamic changes in the tricuspid valve. "Mitralization" of the tricuspid valve (that is, a tendency of the leaflets to be altered to bicuspidali ty) was noted in 46 cases. In specimens in which pulmonary flow had been increased, the left atrium and left ventricle were hypertrophied and enlarged , and there were increased hemodynamic changes in the mitral valve. The ventricular septal defect was usually large and situated in the anterior (conal) septum, that is, anterior to the pars membranacea, involving the membrane in part. The architecture of the musculature of the right ventricle in all but 5 cases was as follows: The septal band extended to the base and bifurcated into two prongs, usually a more robust superior prong and a smaller inferior prong (Fig. I ) . Occasionally, the two prongs were of equal size. Between these prongs lay the ventricular septal defect. Th e parietal band group extended downward over the parietal wall in close relationship to the anterior leaflet of the tricuspid valve. In some case s it did not meet the septal band, whereas in most others it established only a tenuous connection. In thos e hearts in which the inferior prong of the septal band was more robust, the connection was more extensive. Thus either no arch was form ed by the septal or parietal bands or else the arch was markedly excavated. In the 5 exceptional cases , the septal and parietal bands formed a distinct arch and the ventricula r septal defect lay beneath it (Fig. 2) . Factorial analysis Site of origin of the truncus. The truncus arose mostly from the right ventricle in 117
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Fig. 2. Atypical truncus arteriosus communis. The truncus emerges completely from the right ventricle. A, The septal and parietal bands form a distinct arch beneath it. B, The right pulmonary artery emerges from the innominate and C, the left pulmonary artery from the ductus. S, Septal band. RP, Right pulmonary artery. I, Innominate artery. LP, Left pulmonary artery a continuation of the ductus going to the left lung. For other abbreviations, see Fig. 1.
cases (66.5 per cent) and entirely from the right ventricle in 25 cases (14.2 per cent). The truncus straddled the ventricular septum, emerging equally from both ventricles, in 29 cases (16.5 per cent), whereas it arose mostly from the left ventricle in 5 cases (2.8 per cent). In 2 hearts the origin could not be ascertained due to total correction. In I case the truncus arose from a single ventri-
cleo In another, in which there was tricuspid atresia, it arose completely from the left ventricle. The manner of origin of the pulmonary tree. There were 98 Type I cases (57.6 per cent), 58 Type II cases (34.1 per cent), 10 Type III cases (5.9 per cent), and 4 Type IV cases (2.4 per cent), according to the classification of Collett and Edwards."
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I.
Case No.
Patterns
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Right pulmonary blood supply
Left pulmonary blood supply
5
Right pulmonary artery absent; right lung supplied by bronchials Right pulmonary artery and vein absent; right lung absent Right pulmonary artery a continuation of right ductus (right aortic arch) Right pulmonary artery from innominate artery Right pulmonary artery from truncus
6
Right pulmonary artery from truncus
7
Right pulmonary artery from truncus
8
Right pulmonary artery from truncus
9
Ma in pulmonary artery from anterior part of truncus Right pulmonary artery absent; supplied by bronchials
2 3
4
10
Left pulmonary artery from transverse aorta (by way of ductus?) Left pulmonary artery and vein absent; left lung absent Left pulmonary artery from truncus Left pulmonary artery a continuation of left ductus Left pulmonary artery from transverse aorta adjacent to obliterated ductus arteriosus Left pulmonary artery a continuation of left ductus Left pulmonary artery a continuation of left ductus Left pulmonary artery absent; lung supplied by bronchials
Left pulmonary artery from truncus
Fig. 3. Truncus arteriosus communis, Type I (window type) . RA , Right aortic arch. PT, Pulmonary trunk.
Type I. The pulmonary trunk was usually very short and, with the exception of 4 specimens, low in origin. The shortness of .the pulmonary trunk made a distinction between Type I and Type II difficult. In I heart the main pulmonary trunk was confluent with the posterior sinus of Valsalva. Also, there were 6 cases which may be con-
Fig. 4. Truncus arteriosus communis, Type II , with the left pulmonary artery arising above the right. LP, Left pulmonary artery. For other abbre viations, see Figs. I to 3.
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Fig. S. Truncus arteriosus communis, Type III, with narrowing of right pulmonary artery. RC, Right coronary ostium. LC, Left coronary ostium. RV, Right ventricle. For other abbreviations, see Figs. 1 to 3.
sidered a subgroup of this type. In these hearts, a septum hung down between the pulmonary trunk and aortic portion of the truncus . This might be called the "window" type of truncus (Fig. 3). Type II. The two pulmonary arteries arose close to each other posteriorly, close to the truncal valve, or posteriorly and to the left. In 7 cases, the left artery arose above the right (Fig. 4). In 4 cases, the two pulmonary arteries arose high above the truncal valve. Type II I. In 4 specimens the pulmonary arteries arose just above the truncal valve, and in 4 others they were higher (Fig. 5). In 1 specimen only the right pulmonary artery was high, while in another, with agenesis of the right lung, the left pulmonary artery arose high above the truncal valve on the left side. In addition to the above distinct types, there were 10 cases which could not be categorized (Table I) (Fig. 2) . In 8 of these cases the blood supply of one lung was from
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Fig. 6. Truncus arteriosus communis. Main pulmonary trunk arising from the anterior part of the truncus. RV, right ventricle. For other abbreviations, see Figs. I to 3.
the truncus, while that of the other lung was from a ductus, an innominate vessel, or the bronchial circulation. In the ninth case, the pulmonary trunk arose from the anterior part of the truncus (Fig. 6) . In the tenth case both lungs were absent, and hence the pulmonary arteries and veins were absent . Amount of pulmonary ftow. As gauged by the size of the left atrium and ventricle, the pulmonary flow had been increased in 137 cases (86.2 per cent), decreased in 18 cases (11.3 per cent), and normal in 4 cases (2 .5 per cent). In 21 specimens no statement could be made. The truncus valve. The truncus valve was tricuspid in 121 cases (68 per cent), although in 3 hearts the cusps were incompletely divided . The valve was quadricuspid in 45 specimens (25 .3 per cent), although incompletely divided in 9. It was bicuspid in 12 (6.7 per cent) and pentacuspid in I case. The valve could not be interpreted in another heart due to sectioning. The bicuspid and quadricuspid valves were more thickened and nodular than the tricuspid valves. The valve was markedly thickened even in infancy in many cases, and there did not seem to be any relationship between the
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Fig, 8. Truncus arteriosus communis, Type I. Origin of the left coronary from the pulmonary trunk. For abbreviations, see Figs. I to 5. Fig. 7. Truncus arteriosus communis, Type I, with truncus stenosis and possible ventricular septal defect stenosis. The pulmonary trunk arises in a sinus of Valsalv a. Note the poststenotic dilatation. For abbreviations, see Figs. I to 5.
thickening of the valve and the age of the patient. In specimens with multiple extracardiac congenital malformations, the valve often was thickened. Truncal stenosis and insufficiency. Truncal stenosis was noted in 10 cases (5 .6 per cent) (Fig. 7) , and there was anatomic evidence of truncal insufficiency in 4 (2.2 per cent) (Fig. 1) . Hypoplasia of the aorta in truncus arteriosus. In this anomaly, the truncus gives rise to a hypoplastic aorta, with either moderate (1 case) or severe (4 cases) fetal coarctation, atresia of the aortic arch (1 case), or complete interruption of the aortic arch (20 cases, 11. I per cent). The coronary circulation. There was 11 cases of single coronary artery (in 6 the artery arose above the sinus of Valsalva) , 14 cases of origin of both coronary arteries above the sinus of Valsalva , 42 cases of high origin of the left coronary artery alone ( Fig.
5), and 5 cases of high origin of the right coronary artery alone. The coronary distribution was studied in 83 hearts. Regardless of whether the valve was bicuspid, tricuspid , or quadricuspid, the left coronary art ery originated from a cusp situated posteriorly. The artery curved widely around the base of the truncus to the left, below (caudal to) the pulmonary arteries. In many cases, it coursed along the anterior wall of the left ventricle to the left of the anterior interventricular groove, while the anterior descending artery extended to the left of the groove, reaching the latter only in its distal course. The right coronary art ery usually arose from a cusp situated anteriorly, anteriorly and to the right, or anteriorly and to the left. It gave off robust branches to the anterior wall of the right ventricle; these vessels were usually larger than normal, sometimes larger than the anterior descending coronary artery . There were 6 specimens worthy of note. In 1 case, the left coronary artery arose above the sinus of Valsalva , while the common pulmonary trunk arose in the sinus
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(Fig. 7). In 4 other cases the origin of the left coronary was from the main pulmonary trunk, about I em. above the truncal valve (Fig. 8), In the sixth, two left coronary arteries arose from the main pulmonary trunk. Ventricular septal defect stenosis. The ventricular septal defect was stenotic in 6 cases (3,3 per cent) and absent in I case. Associated cardiac abnormalities. These anomalies are given in Table II. The following were common: right aortic arch, 34 cases (18.9 per cent); abnormal brachiocephalic vessels, 24 cases (13.3 per cent); atrial septal defect, fossa ovalis type, 36 cases (20.0 per cent); mild tricuspid stenosis, I 1 cases (6, I per cent); and entrance of the left superior vena cava into the coronary sinus, 8 cases (4.4 per cent). Associated extra cardiac abnormalities and the status of the spleen. Ninety-five specimens (67.4 per cent) had normal situs, a normal (89) or accessory (6) spleen, and no extracardiac abnormalities, Among these there was 1 case of dextroversion and 1 case of mixed dextrocardia. On the other hand, 42 specimens (29.8 per cent) had multiple extracardiac abnormalities with a normal (38) or accessory (4) spleen. There was 1 example each of the following combinations: isolated levocardia with partial visceral heterotaxy, multiple extracardiac congenital abnormalities, and a normal spleen; normal situs, multiple congenital abnormalities, and a multilobated spleen; dextrocardia with complete situs inversus, multiple extracardiac congenital abnormalities, and asplenia; and dextrocardia with partial situs inversus, multiple congenital abnormalities, and asplenia. Information was not available in the remaining cases. Status of the ductus arteriosus. The ductus was closed in 24 cases (13.3 per cent), absent in 40 (22.2 per cent), closing in 2, and patent in 30 cases (16.7 per cent). The status of the ductus could not be ascertained in the other specimens. Age and sex. The specimens were obtained from 90 male and 87 female subjects. No information was available in the re-
Table II. Associated cardiac abnormalities Abnormality Right aortic arch Aberrant origin of right subclavian Aberrant origin of left subclavian Origin of left subclavian from ductus Origin of right subclavian from descending 6 aorta 1 Common brachiocephalic trunk Aberrant left subclavian posterior to esophagus Aberrant right subclavian posterior to esophagus Aberrant origin of left vertebral from 2 aortic arch 2 Left innominate 1 Aberrant origin of brachiocephalic vessel 36 Atrial septal defect 1 Tricuspid insufficiency 11 Tricuspid stenosis 1 Almost complete absence of atrial septum Left superior vena cava emptying into 8 coronary sinus 1 Coronary sinus opening to both atria Total anomalous pulmonary venous 2 drainage 3 Common atrioventricular orifice Dextrocardia (type undetermined) with common atrioventricular orifice Dextrocardia with atria inverted, ventricles pivoted, and atrial septal defect; common atrioventricular orifice; bilateral superior vena cava; total anomalous pulmonary venous drainage into abdomen; absent coronary sinus with asplenia and partial situs inversus Mixed dextrocardia with total anomalous pulmonary venous drainage into azygos Dextroversion Mesocardia Tricuspid atresia Mitral atresia Single ventricle with straddling conus Abnormal mitral valve Straddling tricuspid value with ventricular septal defect (common atrioventricular 3 orifice) 1 Adult coarctation 1 Aneurysm of the fossa ovalis Premature narrowing of foramen ovale with absent ventricular septal defect and hypoplastic mitral valve Partial anomalous pulmonary venous drainage with sinus venosus and atrial septal defect Diverticulum of right ventricle
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maining cases. In the 177 cases in which the age was known, the range was from stillbirth to 19 years, with a mean of 6 months, 11 days.
Discussion Truncus communis has been well studied in the literature. 1-4'1 The modern work on this subject begins with the classical study by Collett and Edwards." These authors laid their main stress on the manner of origin of the pulmonary trunk or arteries. In their Type I, a main pulmonary trunk was present. In Type II the trunk was absent, and two pulmonary arteries emerged from the posterior or posteroleft aspect of the truncus close to each other. In Type III the pulmonary arteries arose laterally, and in Type IV the pulmonary trunk and arteries were absent, and the bronchial arteries supplied the lungs. All these types had subtypes related to other factors which we have discussed. The state of the pulmonary tree as depicted by these authors is obviously very important in total correction. However, we must emphasize that in our material there is no sharp line of demarcation between Types I and II. In most of our cases the main trunk in Type I was very short, so that for practical purposes these specimens could just as well be designated Type II. We further believe that other factors must be considered in evaluating candidates for total correction. The first of these is the origin of the truncus. In the majority of our series, the truncus arose mostly from the right ventricle. This fact might be of importance in the surgical transfer of the aorta into the left ventricle. We H have called attention to a possible hazard in the creation of subaortic stenosis in double-outlet right ventricle with a subaortic ventricular septal defect. A similar hazard might have to be entertained here. In this context, the question of stenosis of the left ventricular outflow tract at the ventricular septal defect cannot be overlooked. The necessity of surgical enlargement of the ventricular septal defect must be evaluated.
The nature of the truncus valve may likewise have some importance in surgery. Although it is most often tricuspid, a sizeable number of cases have bicuspid or quadricuspid valves. All three types of valves, but especially the latter two, tend to become thickened and are in some cases insufficient or stenotic. The immediate effect of stenosis at the time of surgery, as well as the longterm postsurgical effects of these thickened valves, must be taken into account by the surgeon. A sizeable number of cases in our series have associated extracardiac abnormalities similar to those found in trisomy 18 and 13 to 15. However, chromosomal studies were not done. The question of the immediate and long-term surgical prognosis of these cases must be evaluated. The surgical importance of increased or decreased pulmonary flow is obvious. Likewise, the coronary circulation is of importance to the surgeon. Origin of the left coronary artery from the posterior aspect of the truncus, close to the origin of the pulmonary tree, may pose certain surgical problems. In 1 case in our collection, the surgical sutures used to remove the pulmonary tree from the truncus were directly adjacent to the mouth of the left coronary artery. Another possible source of difficulty is the branches of the right circumflex that extend over the anterior wall of the right ventricle. If they are markedly enlarged, they may be of some importance in the ventriculotomy. Hypoplasia of the aorta in truncus should be mentioned briefly. Although in general this anomaly does not permit long survival, there is a case in the literature of survival to adult life H and another case of total correction." The frequent presence of a right aortic arch and other brachiocephalic vascular anomalies, an atrial septal defect, a left superior vena cava entering the coronary sinus, as well as the rare association with other cardiac complexes, deserves attention. The 10 cases in which the blood supply to the lungs came from two sources are of note. In some, one lung was supplied by a pul-
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monary artery arising from the truncus, from the ductus, from the innominate artery, while the other was supplied by the bronchial or an opposite ductus. Collett and Edwards" believed that "truncus" is an improper designation for cases in which a ductus gives rise to a pulmonary artery, They felt there was a possibility that a main pulmonary trunk might have been present but had disappeared. Nevertheless, we feel that, anatomically, it is better to call all these cases atypical truncus communis. We wish to thank the staff of the Thoracic Unit, The Hospital for Sick Children, Great Ormond Street, London, for permission to review and publish their specimens. Acknowledgement is also made to Florence Kotal for her help in the preparation of the manuscript.
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