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Surgical repair of single ventricle
Surgical repair of single ventricle Richard N. Edie, M.D. (by invitation), Kent Ellis, M.D. (by invitation), Welton M. Gersony, M.D. (by invitation), Ehud Krongrad, M.D. (by invitation), Frederick O. Bowman, i-; M.D., and James R. Maim, M.D., New York, N. Y.
fir years, physicians and anatomists have been interested in the heart that has one functioning ventricle. Various terms have been suggested for this entity including single ventricle, 1 , 2 common ventricle." 4 double-inlet left ventricle," cor biatriatum triloculate," and primitive ventricle.' In this communication, the term "single ventricle" is utilized, as suggested by Van Praagh,' and is defined as that congenital cardiac anomaly in which a common or separate atrioventricular valve(s) opens into a ventricular chamber from which both great arterial trunks emerge. 1, 3, 8 An outlet chamber.>" or infundibulum, mayor may not be present and give rise to the origin of either of the great arteries. This definition excludes the entities of mitral and tricuspid atresia. Classification
Four patients, aged 6, 7, 13, and 21 years, with single ventricle and two atrioventricular valves have undergone successful surgical repair. Total correction was possible in all 4 primarily because the ventricular anatomy permitted the construction of a prosthetic septum, resulting in two functioning ventricles. According to Van Praagh's' classificaFrom the Departments of Surgery, Pediatrics, and Radiology, Columbia University, College of Physicians and Surgeons, and the Surgical, Pediatric and Radiology Services of the Presbyterian Hospital and Babies Hospital, New York, N. Y. 10032. Read at the Fifty-third Annual Meeting of The American Association for Thoracic Surgery, Dallas, Texas, April 16, 17, and 18, 1973.
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tion (Fig. 1), type A is the classic single ventricle with a rudimentary outlet chamber. In this type, the single ventricle is composed morphologically of left ventricular sinus myocardium which arises from the ventricular portion of the bulboventricular loop. The outflow chamber consists of right ventricular myocardium which originates from the arterial or distal portion of the bulbus cordis. Type B is very rare; the ventricle is formed entirely from right ventricular sinus myocardium. Type C refers to a common ventricular chamber composed of approximately equal parts of both right and left ventricular sinus myocardium with a rudimentary ventricular septum at the apex of the heart. In type D, there is a primitive ventricle formed completely from undeveloped ventricular myocardium. The ventricular types are further subclassified according to the relationship between the great arteries, the direction of the bulboventricular loop, and the position of both the atria and viscera. This paper, however, will focus primarily on the ventricular anatomy and will comment on the associated abnormalities only as they are pertinent. Clinical material
Two of the 4 patients conformed to the type A and 2 to the type C classification described above. Both type A patients (Fig. 2) had separate atrioventricular valves which entered into a single anatomic left ventricle. Each had an underdeveloped
Volume 66
Surgical repair of single ventricle
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LV
ABSENT RV. SINUS
ABSENT OR RlVIMENTARY VENTRICULAR SEPTUM
ABSENT LV SINUS
ABSENT RV." L V SINUSES VENTRICULAR SEPTUM
e
Fig. 1. An illustration of the clas sification of ventricular types A through D according to Van Praagh.' The cross-hatched portion represents right ventri cul ar (RY.) myocardium, while the smooth print represents the left ventricular (L.V.) myocardium. A rudimentary right ventricular infundibulum (RY . Inj.) is seen in ventricular type A.
right ventricular infundibulum which led to an obstructed outflow tract with normal great vessel relationships. The type C patients (Fig. 3) had a single ventricle composed of almost equal portions of right and left ventricular myocardium. The two atrioventricular valves entered into either side of this single chamber, which had a rudimentary septum at its apex . One type C patient had associated D transposition of the great vessels. All 4 patients had dextrorotation of the bulboventricular loop and situs solitus of the atria and viscera. Each of the patients presented initially with cyanosis, and 1 had congestive heart failure. Two had undergone previous palliative systemic-pulmonary shunts, whereas a third who originally displayed hypertension and increased pulmonary blood flow, had been treated by pulmonary artery banding. At the time of total correction, exercise intolerance and cyanosis had progressed in all of the patients. The clinical information, cardiac cathe-
Fig. 2. An illustration of type A single ventricle with normal relationship of the great vessels. A single ana tom ic left ventricle, into which enter two separate atrioventricular valves. is seen. The rudimentary right ven tricular infund ibulum leads to a narrow pulmon ary outflow trac t.
terization data (Table I), and angiocardiograms (Figs. 4 to 6) were consistent with the basic diagnosis of single ventricle and pulmonary outflow tract obstruction. However, in no case could this diagnosis be made a certainty before operation. The possibility of a large ventricular septal defect in the context of the various diagnoses listed in Table I could not be ruled out by the data. Thus although single ventricle, with all of its surgical implications, was a diagnostic consideration, in each instance a corrective operative procedure was undertaken. Operative technique
At operation each of the 4 patients had an anatomic single ventricle with complete
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Table I. Preoperative data
Patient
Age (yr.)
Ventricular type
Preoperative diagnosis
E.l. D.M.
21 13
A A
Tetralogy of Fallot with left Blalock shunt Tetralogy of Fallot with right Blalock shunt
I.M.
6
C
H.W.
7Jh
C
YSD, double-outlet right ventricle, TGV, pulmonic stenosis, secondary to a PA band VSD, double-outlet right ventricle, pulmonic stenosis
Legend: EeG, Electrocardiogram. SV, Single ventricle. PA, Pulmonary artery. VSD, Ventricular septal defect. TGV. Trail RBBB, Right bundle branch block. LVH, Left ventricular hypertrophy.
Table II. Postoperative data Patient
Age (yr.)
Ventricular type
s.r.
21
A
D.M.
13
A
1. M.
6
C
H.W.
C
Operation
Ligation of left Blalock shunt; infundibular resection; excision of pulmonic valve; closure of secundum ASD; reconstruction of ventricular septum Ligation of right Blalock shunt; infundibular resection; pulmonic valvulotomy; insertion of pulmonary outflow patch; reconstruction of ventricular septum Infundibular resection (subaortic stenosis); PA angioplasty; insertion of interatrial baffle; reconstruction of ventricular septum Infundibular resection; pulmonic valvulotomy; reconstruction of ventricular septum
Legend: RV, Right ventricle. LV. Left ventricle. PA, Pulmonary artery. ASD, Atrial septal defect. AV, Atrioventricular.
absence of the ventricular septum and two normal atrioventricular valves with separate papillary muscle attachments. As expected, 3 patients had infundibular and valvular pulmonic obstruction. The 1 patient with transposition of the great vessels had a competent pulmonary artery band in place. The risk of injury to the bundle of His is increased during the construction of a ventricular septum in single ventricle, since variations in the usual position of this structure may be expected. Atrioventricular dissociation developed in 2 patients, resulting in permanent heart block in 1 of them. In the most recently treated patient, a method of electrical recording of the conduction system" was helpful in locating the bundle of His (Fig. 7). The bundle was found to enter the single ventricle beneath the septal
leaflet of the tricuspid valve, as in the normal heart, and then to extend distally along the posterior wall between the atrioventricular valves. A Teflon prosthesis was used for the construction of the new ventricular septum (Fig. 8). The initial sutures were inserted along the posterior wall between the atrioventricular valves; when feasible, the annulus of the septal leaflet of the tricuspid valve was used as well. The sutures were continued superiorly between the semilunar valves, then inferiorly toward the apex, and into the rudimentary septum when present. Anteriorly, it was necessary to insert the sutures from the outside of the heart in order to use the entire wall thickness for stable fixation. Relief of the muscular infundibular ob-
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Aortic 0, saturation (per cent)
SV pressure (mm. Hg)
PA pressure (mm. Hg)
RSR, RAD, RVH RSR with intra-atrial block, RAD, RVH, incomplete RBBB RSR, RAD, RVH, LVH
74 77
105/7 125/2
15
80
112/15
18/7
RSR, RAD, RVH
89
105/9
19/12 14
Symptoms
ECG
Cyanosis, exercise intolerance Cyanosis, syncope, dyspnea
Cyanosis, syncope, exercise intolerance Cyanosis, exercise intolerance position of great
vessels. RSR,
RV pressure (mill. Hg]
36/9
31/18
Regular sinus
rhythm.
RAD,
axis
Right
deviation.
RVH,
Right
ventricular
IT
hypertrophy.
LV pressure (mill. Hg)
PA pressure (mm. Hg)
Aortic 0, sail/ration (per cent)
Cardiac index (L./min./sq.M.)
10117
35/10
94
3.8
28/16
96
94/10
2.1 (during AV dissociation)
65/4
110/17
(anatomic LV)
(anatomic RV)
27/10
90/15
37/25
struction and pulmonic valvular stenosis was considered to be essential to the satisfactory total correction. A Mustard procedure and pulmonary artery angioplasty were performed on the patient who had transposition and a pulmonary artery band.
Operative results The early postoperative period was marked by the development of congestive heart failure in 3 patients, caused by transient cardiac arrhythmias in 2. The third patient with cardiac decompensation had a residual ventricular shunt which required reoperation. At the second operation, the superior aspect of the new septum was found to be detached from the aortic root, necessitating the insertion of a longer prosthesis to accommodate the overriding aorta.
94
3.4
93
3.6
This patient also had persistent heart block which necessitated the insertion of a permanent pacemaker. The fourth patient had an uneventful recovery. Cardiac catheterization and angiocardiography were performed on the 4 patients 1 week to 5 months after operation. The data are shown in Table II. A normal cardiac output was documented in 3 patients, but it was decreased in the 1 patient who was studied during transient atrioventricular dissociation. All patients had normal arterial oxygen saturations, satisfactory pulmonary ventricular pressures, and no significant obstruction to the right ventricular outflow tract. One patient had a trivial residual leftto-right ventricular shunt. The postoperative angiocardiograms clearly illustrated the satisfactory construction of a prosthetic sep-
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Fig. 3. An illustration of type C single ventricle with transposition of the great vessels and previous pulmonary artery banding. The separate atrioventricular valves attach to different sets of pap illary muscles on either side of the ventricular chamber. The chamber has a rundiment ary septum at its apex. An undeveloped infundibulum is seen in the subaortic area.
tum with the creation of two functioning separate ventricles (Figs. 9 to 12). After 1 to .11 years of follow-up, the 4 patients are leading active lives. Discussion Correction of single ventricle has rarely been attempted and has met with virtually no success. The reasons for failure are multiple. Aside from the obvious difficult technical aspects of prosthetic division of the ventricle, a high percentage of patients have associated anomalies (e .g, transposition of the great vessels) 1 , 1, S which add significantly to operative risks. Furthermore, even when anatomic repair is achieved, the question has remained unanswered whether
ventricles can function properly with an entirely prosthetic septum. In addition, the conduction system in single ventricle is poorly understood, raising the specter of permanent heart block. Until recently , no method for His bundle delineation has been available. Finally, despite refinement of biplane angiocardiography, it is often difficult to establish an accurate preoperative anatomic diagnosis. Since recent developments in surgical techniques allow contemplation of repair in selected cases of single ventricle, careful definition of the exact anatomy of the various types becomes important. It is critical to identify the number and position of the atrioventricular valves, the relationship of the great vessels, and the nature and severity of pulmonary outflow tract obstruction. If two atrioventricular valves are present , total correction of single ventricle is feasible. At operation, it is of utmost importance to define and avoid the conduction system, for arrhythmias in this group of patients may cause major postoperative complications. Second , it is necessary to obtain stable fixation for the prosthetic septum, often a difficult technical task . Third, the relief of pulmonary outflow tract obstruction and complete correction of associated anomalies (e.g., transposition of great vessels by Mustard procedure) are essential to successful total repair. If the complexity and anatomy of the defect are not amenable to total correction, an appropriate palliative procedure should be performed. The results of repair of single ventricle reported in this paper are encouraging, since it has now been established that correction can be achieved without permanent injury to the conduction system and that two ventricles separated by prosthetic material can function independently in an effective manner.
Summary Four patients with single ventricle and two atrioventricular valves have undergone total correction. Postoperative cardiac
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Fig. 4. Preoperative anteroposterior (left) and lateral (right) angiocardiograms in type A single ventricle with normal relationship of the great vessels. The catheter is through the tricuspid valve (TY.) with the injection into a large, poorly defined single ventricle (SV) which gives origin to both the aorta (Aa ) and the narrow pulmonary outflow tract. The lateral view shows both the mitral (MY,) and tricuspid valves (TY.) entering the single ventricle with an anterior infundibulum (/) leading to the pulmonary outflow tract.
Fig. S. Preoperative anteroposterior angiocardiograms in a type C single ventricle with D transposition of the great vessels and a previous pulmonary artery banding (Pa band). The catheter is across the tricuspid valve, and two separate injections reveal the anterior trabeculated portion of the single ventricle (SV) on the left and the posterior, smooth-walled portion of the single ventricle on the right. Both portions communicate with the transposed aorta (Ao) and pulmonary artery (P A). A muscular band separates the great vessels, and the semilunar valves are side by side.
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Fig. 6. Preoperative anteroposterior (left) and lateral (right) angiocardiograms in the same patient with type C single ventricle and transposition. The lateral projection illustrates the single ventricle (SV) and the anterior infundibulum (I ), which leads to a narrow outflow tract creating subaortic stenosis. The poststenotic dilatation of the pulmonary artery (PA) is seen distal to the band (Pa bandy.
Fig. 7. An illustration of the intraventricular location of the bundle of His (++) in I patient with single ventricle. The electrical probe used for the operative recordings is seen.
Fig. 8. An illustration of the operative technique used for reconstruction of the ventricular septum demonstrates the prosthesis separating the two atrioventricular valve mechanisms. The anterior sutures have been inserted from the outside of the heart through the entire wall thickness.
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Fig. 9. A comparison between preoperative (leit) and postoperative [right ) anteroposterior angiocardiograms in I of the type A patients. The preoperative single ventricle (SV) is compared with the new postoperative right ventricle (RY.) which gives origin to the reconstructed pulmonary outflow tract and pulmonary artery (PA) .
Fig. 10. A comparison between preoperative (left) and postoperative (right) lateral angiecardiograms in the same type A patient. The preoperative single ventricle (SV) is compared with the new postoperative right ventricle (RY.), of which the infundibulum is an appendage. The site of the reconstructed septum is seen.
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Fig. 11. A comparison of preoperative (left) and postoperative (right) anteroposterior angiocardiograms in a type C patient with transposition. The preoperative trabeculated portion of the single ventricle (SV) which communicates with the aorta (Ao) and pulmonary artery (PA) is compared with the postoperative study, which shows the new posterior, smooth-walled left ventricle (LV) which gives origin only to the debanded pulmonary artery (P A).
Fig. 12. A comparison of postoperative lateral angiocardiograms in the same type C patient with single ventricle and transposition. The individual injections into the new right (RV) and left (LV) ventricles illustrate the site of the reconstructed septum and the separate origins of the aorta (Ao) and pulmonary artery (PA).
Surgery
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catheterization data and angiocardiography indicated satisfactory hemodynamic and anatomic results. The patients were acyanotic and have displayed marked clinical improvement I to 11 years after operation. REFERENCES Van Praagh, R., Ongley, P. A, and Swan, H. J. C.: Anatomic Types of Single or Common Ventricle in Man, Am. J. Cardio\. 13: 367, 1964. 2 Van Praagh, R., Van Praagh, S., Vlad, P., and Keith, J. D.: Diagnosis of Single or Common Ventricle, Am, J, Cardio\. 15: 345, 1965. 3 Elliott, L. P., and Morgan, A. D.: Common Ventricles, ill Moss, A. 1., and Adams, F. H., editors: Heart Disease in Infants, Children and Adolescents, chap. 28, Baltimore, 1968, The Williams & Wilkins Company, p. 589, 4 Rahimtoola, S. H., Ongley, R. A, and Swan, H. T. C.: The Hemodynamics of Common (or Single) Ventricle, Circulation 34: 14, 1966. 5 de la Cruz, M. V., and Miller, B. L.: Double Inlet Left Ventricle, Circulation 37: 249, 1968, 6 Elliott, L. P., Amplatz, K., Anderson, R. c., and Edwards, J. E.: Cor Triloculare Biatriatum With Pulmonary Stenosis and Normally Related Great Vessels, Am. J. Cardio\. 11: 469, 1963. 7 Lev, M., Liberthson, R. R., Kirkpatrick, J. R., Eckner, F. A 0., and Arcilla, R. A: Single (Primitive) Ventricle, Circulation 39: 577, 1969. 8 Keith, J. D., Rowe, R. D., and Vlad, P.: Heart Disease in Infancy and Childhood, chap, 23, New York, 1958, Macmillan Publishing Co., Inc., p. 745. 9 Kaiser, G. A, Waldo, A L., Beach, P. M., Bowman, F. 0., Jr., Hoffman, B. F., and Maim, J. R.: Specialized Cardiac Conduction System: Improved Electrophysiologic Identification Techniques at Surgery, Arch. Surg. 101: 673, 1970. 10 Sakakibara, S., Tominaga, S., Imai, Y., Vehara, K., and Matsumuro, M.: Successful Total Correction of Common Ventricle, Chest 61: 192, 1972, 11 Seki, S., Tsakiris, A G., Mair, D. D" and McGoon, D. C.: Radical Correction of Single Ventricle in an Experimental Model, Ann. Surg, 176: 748, 1972. 12 Morgan, A D., Krovetz, L. J., Bartley, T. D., Green, J. R., Shanklin, D. R., Wheat, M. W., Jr., and Schiebler, G. L.: Clinical Features of Single Ventricle With Congenitally Corrected Transposition, Am. 1. Cardio\. 17: 379, 1966.
13 Hallermann, F. J., Davis, G. D., Ritter, D. G., and Kincaid, O. W.: Roentgenographic Features of Common Ventricle, Radiology 87: 409, 1966. 14 Hallermann, F. J., Kincaid, O. W., Ritter, D. G., Ongley, P. A, and Titus, J. L.: Angiocardiographic and Anatomic Findings in Origin of Both Great Arteries From the Right Ventricle, Am. J. Roentgeno\. Radium. Ther. Nuc\. Med. 109: 51,1970. 15 Anselmi, G., Armas, S. M., de la Cruz, M. V., dePisani, F., and Blanco, P.: Diagnosis and Classification of Single Ventricle, Am. J. Cardio\. 21: 813, 1968. 16 Edwards, J. E.: Congenital Malformations of the Heart and Great Vessels, ill Gould, S. E., editor: Pathology of the Heart, Springfield, III., 1960, Charles C Thomas, Publisher, p. 260. 17 Klaus, A. P., Smith, R. B., Schneider, A. B., and Parker, B. M.: Single Ventricle With Normal Relationship of the Great Vessels and Pulmonic Stenosis, Am. Heart J. 78: 530, 1969. 18 Morris, E. W. T.: The Interventricular Septum, Thorax 12: 304, 1957. 19 Davachi, F., and Moller, J. H.: The Electrocardiogram and Vectorcardiogram in Single Ventricle, Am. J. Cardio\. 23: 19, 1969.
Discussion DR. GORDON K. DANIELSON Rochester, Minn.
I would like to compliment Dr. Edie on his fine presentation and excellent results with repair of common ventricle. One year ago we encountered an interesting variant of common ventricle which illustrates other possibilities for repair of this anomaly. [Slide] A 22-year-old man from Italy was studied and found to have a common atrium, common ventricle, atrioventricular canal-type mitral and tricuspid valve structures with massive incompetence of both valves, and absence of the coronary sinus. The cardiac index was only 1.1 L. per minute per square meter. The chest x-ray film showed gross cardiomegaly and filling of all four chambers of the heart from an injection in the single ventricle. The only hint of where the septum should have been was a slight ridge on the caudal aspect of the ventriculogram. [Slide] Repair was accomplished by opening the right atrium and excising the attenuated mitral and tricuspid tissue. This left us with essentially a one-chambered heart. A large Dacron patch was then sewn into the apex of the ventricle and carried up to the level of the atrioventricular valve annuli. Two M-32 Braunwald-Cutter valves
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at.
Thoracic and Cardiovascular Surgery
were inserted into the mitral and tricuspid areas, and the patch was carried cephalad to close the atrial septal defect. There was no heart block. [Slide] He had an uneventful convalescence and was restudied prior to discharge. The left ventriculogram showed prompt ejection of dye out into the aorta. The cardiac index had increased from 1.1 to 2.2 L. per minute. The left ventricular end-diastolic pressure fell from 23 to 7 mm. Hg and the right ventricular systolic pressure from 76 to 48 mm. Hg. Several points are illustrated. The bundle of His locator, described by Dr. Edie, was useful in tracing the conduction bundle in this patient, particularly since there was no coronary sinus to furnish an anatomic landmark. In some patients, particularly those with large hearts, it should be possible to repair the defects entirely through the right atrium, as with uncomplicated complete atrioventricular canal. Finally, one of the criteria that has thus far been a requisite for correction of common ventricle has been the presence of two atrioventricular valves. This case suggests that the presence of a single atrioventricular valve may not be a
contraindication, at least in older children and young adults, as the valve can be excised and replaced with two prostheses. DR. EDIE (Closing) My thanks to Dr. Danielson for his comments. We have not attempted total correction in patients with single ventricle and a common atrioventricular valve, and we commend him for the success with this difficult case. We have used electrical recording to locate the bundle of His and define its relationship to the reconstructed ventricular septum. The slide [slide] illustrates such a recording. The A spike on the electrogram is comparable to the P wave on the electrocardiogram, and the B spike with the QRS complex. The bundle of His occurs in the isoelectric portion of the electrocardiogram between the P wave and the QRS complex and is clearly seen on the electrogram between the A and B spikes. After using this technique in 2 patients in the current series, we believe it was of assistance in avoiding the conduction system during the surgical repair.
fir years, physicians and anatomists have been interested in the heart that has one functioning ventricle. Various terms have been suggested for this entity including single ventricle, 1 , 2 common ventricle." 4 double-inlet left ventricle," cor biatriatum triloculate," and primitive ventricle.' In this communication, the term "single ventricle" is utilized, as suggested by Van Praagh,' and is defined as that congenital cardiac anomaly in which a common or separate atrioventricular valve(s) opens into a ventricular chamber from which both great arterial trunks emerge. 1, 3, 8 An outlet chamber.>" or infundibulum, mayor may not be present and give rise to the origin of either of the great arteries. This definition excludes the entities of mitral and tricuspid atresia. Classification
Four patients, aged 6, 7, 13, and 21 years, with single ventricle and two atrioventricular valves have undergone successful surgical repair. Total correction was possible in all 4 primarily because the ventricular anatomy permitted the construction of a prosthetic septum, resulting in two functioning ventricles. According to Van Praagh's' classificaFrom the Departments of Surgery, Pediatrics, and Radiology, Columbia University, College of Physicians and Surgeons, and the Surgical, Pediatric and Radiology Services of the Presbyterian Hospital and Babies Hospital, New York, N. Y. 10032. Read at the Fifty-third Annual Meeting of The American Association for Thoracic Surgery, Dallas, Texas, April 16, 17, and 18, 1973.
350
tion (Fig. 1), type A is the classic single ventricle with a rudimentary outlet chamber. In this type, the single ventricle is composed morphologically of left ventricular sinus myocardium which arises from the ventricular portion of the bulboventricular loop. The outflow chamber consists of right ventricular myocardium which originates from the arterial or distal portion of the bulbus cordis. Type B is very rare; the ventricle is formed entirely from right ventricular sinus myocardium. Type C refers to a common ventricular chamber composed of approximately equal parts of both right and left ventricular sinus myocardium with a rudimentary ventricular septum at the apex of the heart. In type D, there is a primitive ventricle formed completely from undeveloped ventricular myocardium. The ventricular types are further subclassified according to the relationship between the great arteries, the direction of the bulboventricular loop, and the position of both the atria and viscera. This paper, however, will focus primarily on the ventricular anatomy and will comment on the associated abnormalities only as they are pertinent. Clinical material
Two of the 4 patients conformed to the type A and 2 to the type C classification described above. Both type A patients (Fig. 2) had separate atrioventricular valves which entered into a single anatomic left ventricle. Each had an underdeveloped
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Surgical repair of single ventricle
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September. 1973
LV
ABSENT RV. SINUS
ABSENT OR RlVIMENTARY VENTRICULAR SEPTUM
ABSENT LV SINUS
ABSENT RV." L V SINUSES VENTRICULAR SEPTUM
e
Fig. 1. An illustration of the clas sification of ventricular types A through D according to Van Praagh.' The cross-hatched portion represents right ventri cul ar (RY.) myocardium, while the smooth print represents the left ventricular (L.V.) myocardium. A rudimentary right ventricular infundibulum (RY . Inj.) is seen in ventricular type A.
right ventricular infundibulum which led to an obstructed outflow tract with normal great vessel relationships. The type C patients (Fig. 3) had a single ventricle composed of almost equal portions of right and left ventricular myocardium. The two atrioventricular valves entered into either side of this single chamber, which had a rudimentary septum at its apex . One type C patient had associated D transposition of the great vessels. All 4 patients had dextrorotation of the bulboventricular loop and situs solitus of the atria and viscera. Each of the patients presented initially with cyanosis, and 1 had congestive heart failure. Two had undergone previous palliative systemic-pulmonary shunts, whereas a third who originally displayed hypertension and increased pulmonary blood flow, had been treated by pulmonary artery banding. At the time of total correction, exercise intolerance and cyanosis had progressed in all of the patients. The clinical information, cardiac cathe-
Fig. 2. An illustration of type A single ventricle with normal relationship of the great vessels. A single ana tom ic left ventricle, into which enter two separate atrioventricular valves. is seen. The rudimentary right ven tricular infund ibulum leads to a narrow pulmon ary outflow trac t.
terization data (Table I), and angiocardiograms (Figs. 4 to 6) were consistent with the basic diagnosis of single ventricle and pulmonary outflow tract obstruction. However, in no case could this diagnosis be made a certainty before operation. The possibility of a large ventricular septal defect in the context of the various diagnoses listed in Table I could not be ruled out by the data. Thus although single ventricle, with all of its surgical implications, was a diagnostic consideration, in each instance a corrective operative procedure was undertaken. Operative technique
At operation each of the 4 patients had an anatomic single ventricle with complete
The Journal of
352
Edie et at.
Thoracic and Cardiavascular Surgery
Table I. Preoperative data
Patient
Age (yr.)
Ventricular type
Preoperative diagnosis
E.l. D.M.
21 13
A A
Tetralogy of Fallot with left Blalock shunt Tetralogy of Fallot with right Blalock shunt
I.M.
6
C
H.W.
7Jh
C
YSD, double-outlet right ventricle, TGV, pulmonic stenosis, secondary to a PA band VSD, double-outlet right ventricle, pulmonic stenosis
Legend: EeG, Electrocardiogram. SV, Single ventricle. PA, Pulmonary artery. VSD, Ventricular septal defect. TGV. Trail RBBB, Right bundle branch block. LVH, Left ventricular hypertrophy.
Table II. Postoperative data Patient
Age (yr.)
Ventricular type
s.r.
21
A
D.M.
13
A
1. M.
6
C
H.W.
C
Operation
Ligation of left Blalock shunt; infundibular resection; excision of pulmonic valve; closure of secundum ASD; reconstruction of ventricular septum Ligation of right Blalock shunt; infundibular resection; pulmonic valvulotomy; insertion of pulmonary outflow patch; reconstruction of ventricular septum Infundibular resection (subaortic stenosis); PA angioplasty; insertion of interatrial baffle; reconstruction of ventricular septum Infundibular resection; pulmonic valvulotomy; reconstruction of ventricular septum
Legend: RV, Right ventricle. LV. Left ventricle. PA, Pulmonary artery. ASD, Atrial septal defect. AV, Atrioventricular.
absence of the ventricular septum and two normal atrioventricular valves with separate papillary muscle attachments. As expected, 3 patients had infundibular and valvular pulmonic obstruction. The 1 patient with transposition of the great vessels had a competent pulmonary artery band in place. The risk of injury to the bundle of His is increased during the construction of a ventricular septum in single ventricle, since variations in the usual position of this structure may be expected. Atrioventricular dissociation developed in 2 patients, resulting in permanent heart block in 1 of them. In the most recently treated patient, a method of electrical recording of the conduction system" was helpful in locating the bundle of His (Fig. 7). The bundle was found to enter the single ventricle beneath the septal
leaflet of the tricuspid valve, as in the normal heart, and then to extend distally along the posterior wall between the atrioventricular valves. A Teflon prosthesis was used for the construction of the new ventricular septum (Fig. 8). The initial sutures were inserted along the posterior wall between the atrioventricular valves; when feasible, the annulus of the septal leaflet of the tricuspid valve was used as well. The sutures were continued superiorly between the semilunar valves, then inferiorly toward the apex, and into the rudimentary septum when present. Anteriorly, it was necessary to insert the sutures from the outside of the heart in order to use the entire wall thickness for stable fixation. Relief of the muscular infundibular ob-
Volume 66 Number 3
Surgical repair of single ventricle
353
September, 1973
Aortic 0, saturation (per cent)
SV pressure (mm. Hg)
PA pressure (mm. Hg)
RSR, RAD, RVH RSR with intra-atrial block, RAD, RVH, incomplete RBBB RSR, RAD, RVH, LVH
74 77
105/7 125/2
15
80
112/15
18/7
RSR, RAD, RVH
89
105/9
19/12 14
Symptoms
ECG
Cyanosis, exercise intolerance Cyanosis, syncope, dyspnea
Cyanosis, syncope, exercise intolerance Cyanosis, exercise intolerance position of great
vessels. RSR,
RV pressure (mill. Hg]
36/9
31/18
Regular sinus
rhythm.
RAD,
axis
Right
deviation.
RVH,
Right
ventricular
IT
hypertrophy.
LV pressure (mill. Hg)
PA pressure (mm. Hg)
Aortic 0, sail/ration (per cent)
Cardiac index (L./min./sq.M.)
10117
35/10
94
3.8
28/16
96
94/10
2.1 (during AV dissociation)
65/4
110/17
(anatomic LV)
(anatomic RV)
27/10
90/15
37/25
struction and pulmonic valvular stenosis was considered to be essential to the satisfactory total correction. A Mustard procedure and pulmonary artery angioplasty were performed on the patient who had transposition and a pulmonary artery band.
Operative results The early postoperative period was marked by the development of congestive heart failure in 3 patients, caused by transient cardiac arrhythmias in 2. The third patient with cardiac decompensation had a residual ventricular shunt which required reoperation. At the second operation, the superior aspect of the new septum was found to be detached from the aortic root, necessitating the insertion of a longer prosthesis to accommodate the overriding aorta.
94
3.4
93
3.6
This patient also had persistent heart block which necessitated the insertion of a permanent pacemaker. The fourth patient had an uneventful recovery. Cardiac catheterization and angiocardiography were performed on the 4 patients 1 week to 5 months after operation. The data are shown in Table II. A normal cardiac output was documented in 3 patients, but it was decreased in the 1 patient who was studied during transient atrioventricular dissociation. All patients had normal arterial oxygen saturations, satisfactory pulmonary ventricular pressures, and no significant obstruction to the right ventricular outflow tract. One patient had a trivial residual leftto-right ventricular shunt. The postoperative angiocardiograms clearly illustrated the satisfactory construction of a prosthetic sep-
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Fig. 3. An illustration of type C single ventricle with transposition of the great vessels and previous pulmonary artery banding. The separate atrioventricular valves attach to different sets of pap illary muscles on either side of the ventricular chamber. The chamber has a rundiment ary septum at its apex. An undeveloped infundibulum is seen in the subaortic area.
tum with the creation of two functioning separate ventricles (Figs. 9 to 12). After 1 to .11 years of follow-up, the 4 patients are leading active lives. Discussion Correction of single ventricle has rarely been attempted and has met with virtually no success. The reasons for failure are multiple. Aside from the obvious difficult technical aspects of prosthetic division of the ventricle, a high percentage of patients have associated anomalies (e .g, transposition of the great vessels) 1 , 1, S which add significantly to operative risks. Furthermore, even when anatomic repair is achieved, the question has remained unanswered whether
ventricles can function properly with an entirely prosthetic septum. In addition, the conduction system in single ventricle is poorly understood, raising the specter of permanent heart block. Until recently , no method for His bundle delineation has been available. Finally, despite refinement of biplane angiocardiography, it is often difficult to establish an accurate preoperative anatomic diagnosis. Since recent developments in surgical techniques allow contemplation of repair in selected cases of single ventricle, careful definition of the exact anatomy of the various types becomes important. It is critical to identify the number and position of the atrioventricular valves, the relationship of the great vessels, and the nature and severity of pulmonary outflow tract obstruction. If two atrioventricular valves are present , total correction of single ventricle is feasible. At operation, it is of utmost importance to define and avoid the conduction system, for arrhythmias in this group of patients may cause major postoperative complications. Second , it is necessary to obtain stable fixation for the prosthetic septum, often a difficult technical task . Third, the relief of pulmonary outflow tract obstruction and complete correction of associated anomalies (e.g., transposition of great vessels by Mustard procedure) are essential to successful total repair. If the complexity and anatomy of the defect are not amenable to total correction, an appropriate palliative procedure should be performed. The results of repair of single ventricle reported in this paper are encouraging, since it has now been established that correction can be achieved without permanent injury to the conduction system and that two ventricles separated by prosthetic material can function independently in an effective manner.
Summary Four patients with single ventricle and two atrioventricular valves have undergone total correction. Postoperative cardiac
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Fig. 4. Preoperative anteroposterior (left) and lateral (right) angiocardiograms in type A single ventricle with normal relationship of the great vessels. The catheter is through the tricuspid valve (TY.) with the injection into a large, poorly defined single ventricle (SV) which gives origin to both the aorta (Aa ) and the narrow pulmonary outflow tract. The lateral view shows both the mitral (MY,) and tricuspid valves (TY.) entering the single ventricle with an anterior infundibulum (/) leading to the pulmonary outflow tract.
Fig. S. Preoperative anteroposterior angiocardiograms in a type C single ventricle with D transposition of the great vessels and a previous pulmonary artery banding (Pa band). The catheter is across the tricuspid valve, and two separate injections reveal the anterior trabeculated portion of the single ventricle (SV) on the left and the posterior, smooth-walled portion of the single ventricle on the right. Both portions communicate with the transposed aorta (Ao) and pulmonary artery (P A). A muscular band separates the great vessels, and the semilunar valves are side by side.
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Fig. 6. Preoperative anteroposterior (left) and lateral (right) angiocardiograms in the same patient with type C single ventricle and transposition. The lateral projection illustrates the single ventricle (SV) and the anterior infundibulum (I ), which leads to a narrow outflow tract creating subaortic stenosis. The poststenotic dilatation of the pulmonary artery (PA) is seen distal to the band (Pa bandy.
Fig. 7. An illustration of the intraventricular location of the bundle of His (++) in I patient with single ventricle. The electrical probe used for the operative recordings is seen.
Fig. 8. An illustration of the operative technique used for reconstruction of the ventricular septum demonstrates the prosthesis separating the two atrioventricular valve mechanisms. The anterior sutures have been inserted from the outside of the heart through the entire wall thickness.
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Fig. 9. A comparison between preoperative (leit) and postoperative [right ) anteroposterior angiocardiograms in I of the type A patients. The preoperative single ventricle (SV) is compared with the new postoperative right ventricle (RY.) which gives origin to the reconstructed pulmonary outflow tract and pulmonary artery (PA) .
Fig. 10. A comparison between preoperative (left) and postoperative (right) lateral angiecardiograms in the same type A patient. The preoperative single ventricle (SV) is compared with the new postoperative right ventricle (RY.), of which the infundibulum is an appendage. The site of the reconstructed septum is seen.
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Fig. 11. A comparison of preoperative (left) and postoperative (right) anteroposterior angiocardiograms in a type C patient with transposition. The preoperative trabeculated portion of the single ventricle (SV) which communicates with the aorta (Ao) and pulmonary artery (PA) is compared with the postoperative study, which shows the new posterior, smooth-walled left ventricle (LV) which gives origin only to the debanded pulmonary artery (P A).
Fig. 12. A comparison of postoperative lateral angiocardiograms in the same type C patient with single ventricle and transposition. The individual injections into the new right (RV) and left (LV) ventricles illustrate the site of the reconstructed septum and the separate origins of the aorta (Ao) and pulmonary artery (PA).
Surgery
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catheterization data and angiocardiography indicated satisfactory hemodynamic and anatomic results. The patients were acyanotic and have displayed marked clinical improvement I to 11 years after operation. REFERENCES Van Praagh, R., Ongley, P. A, and Swan, H. J. C.: Anatomic Types of Single or Common Ventricle in Man, Am. J. Cardio\. 13: 367, 1964. 2 Van Praagh, R., Van Praagh, S., Vlad, P., and Keith, J. D.: Diagnosis of Single or Common Ventricle, Am, J, Cardio\. 15: 345, 1965. 3 Elliott, L. P., and Morgan, A. D.: Common Ventricles, ill Moss, A. 1., and Adams, F. H., editors: Heart Disease in Infants, Children and Adolescents, chap. 28, Baltimore, 1968, The Williams & Wilkins Company, p. 589, 4 Rahimtoola, S. H., Ongley, R. A, and Swan, H. T. C.: The Hemodynamics of Common (or Single) Ventricle, Circulation 34: 14, 1966. 5 de la Cruz, M. V., and Miller, B. L.: Double Inlet Left Ventricle, Circulation 37: 249, 1968, 6 Elliott, L. P., Amplatz, K., Anderson, R. c., and Edwards, J. E.: Cor Triloculare Biatriatum With Pulmonary Stenosis and Normally Related Great Vessels, Am. J. Cardio\. 11: 469, 1963. 7 Lev, M., Liberthson, R. R., Kirkpatrick, J. R., Eckner, F. A 0., and Arcilla, R. A: Single (Primitive) Ventricle, Circulation 39: 577, 1969. 8 Keith, J. D., Rowe, R. D., and Vlad, P.: Heart Disease in Infancy and Childhood, chap, 23, New York, 1958, Macmillan Publishing Co., Inc., p. 745. 9 Kaiser, G. A, Waldo, A L., Beach, P. M., Bowman, F. 0., Jr., Hoffman, B. F., and Maim, J. R.: Specialized Cardiac Conduction System: Improved Electrophysiologic Identification Techniques at Surgery, Arch. Surg. 101: 673, 1970. 10 Sakakibara, S., Tominaga, S., Imai, Y., Vehara, K., and Matsumuro, M.: Successful Total Correction of Common Ventricle, Chest 61: 192, 1972, 11 Seki, S., Tsakiris, A G., Mair, D. D" and McGoon, D. C.: Radical Correction of Single Ventricle in an Experimental Model, Ann. Surg, 176: 748, 1972. 12 Morgan, A D., Krovetz, L. J., Bartley, T. D., Green, J. R., Shanklin, D. R., Wheat, M. W., Jr., and Schiebler, G. L.: Clinical Features of Single Ventricle With Congenitally Corrected Transposition, Am. 1. Cardio\. 17: 379, 1966.
13 Hallermann, F. J., Davis, G. D., Ritter, D. G., and Kincaid, O. W.: Roentgenographic Features of Common Ventricle, Radiology 87: 409, 1966. 14 Hallermann, F. J., Kincaid, O. W., Ritter, D. G., Ongley, P. A, and Titus, J. L.: Angiocardiographic and Anatomic Findings in Origin of Both Great Arteries From the Right Ventricle, Am. J. Roentgeno\. Radium. Ther. Nuc\. Med. 109: 51,1970. 15 Anselmi, G., Armas, S. M., de la Cruz, M. V., dePisani, F., and Blanco, P.: Diagnosis and Classification of Single Ventricle, Am. J. Cardio\. 21: 813, 1968. 16 Edwards, J. E.: Congenital Malformations of the Heart and Great Vessels, ill Gould, S. E., editor: Pathology of the Heart, Springfield, III., 1960, Charles C Thomas, Publisher, p. 260. 17 Klaus, A. P., Smith, R. B., Schneider, A. B., and Parker, B. M.: Single Ventricle With Normal Relationship of the Great Vessels and Pulmonic Stenosis, Am. Heart J. 78: 530, 1969. 18 Morris, E. W. T.: The Interventricular Septum, Thorax 12: 304, 1957. 19 Davachi, F., and Moller, J. H.: The Electrocardiogram and Vectorcardiogram in Single Ventricle, Am. J. Cardio\. 23: 19, 1969.
Discussion DR. GORDON K. DANIELSON Rochester, Minn.
I would like to compliment Dr. Edie on his fine presentation and excellent results with repair of common ventricle. One year ago we encountered an interesting variant of common ventricle which illustrates other possibilities for repair of this anomaly. [Slide] A 22-year-old man from Italy was studied and found to have a common atrium, common ventricle, atrioventricular canal-type mitral and tricuspid valve structures with massive incompetence of both valves, and absence of the coronary sinus. The cardiac index was only 1.1 L. per minute per square meter. The chest x-ray film showed gross cardiomegaly and filling of all four chambers of the heart from an injection in the single ventricle. The only hint of where the septum should have been was a slight ridge on the caudal aspect of the ventriculogram. [Slide] Repair was accomplished by opening the right atrium and excising the attenuated mitral and tricuspid tissue. This left us with essentially a one-chambered heart. A large Dacron patch was then sewn into the apex of the ventricle and carried up to the level of the atrioventricular valve annuli. Two M-32 Braunwald-Cutter valves
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were inserted into the mitral and tricuspid areas, and the patch was carried cephalad to close the atrial septal defect. There was no heart block. [Slide] He had an uneventful convalescence and was restudied prior to discharge. The left ventriculogram showed prompt ejection of dye out into the aorta. The cardiac index had increased from 1.1 to 2.2 L. per minute. The left ventricular end-diastolic pressure fell from 23 to 7 mm. Hg and the right ventricular systolic pressure from 76 to 48 mm. Hg. Several points are illustrated. The bundle of His locator, described by Dr. Edie, was useful in tracing the conduction bundle in this patient, particularly since there was no coronary sinus to furnish an anatomic landmark. In some patients, particularly those with large hearts, it should be possible to repair the defects entirely through the right atrium, as with uncomplicated complete atrioventricular canal. Finally, one of the criteria that has thus far been a requisite for correction of common ventricle has been the presence of two atrioventricular valves. This case suggests that the presence of a single atrioventricular valve may not be a
contraindication, at least in older children and young adults, as the valve can be excised and replaced with two prostheses. DR. EDIE (Closing) My thanks to Dr. Danielson for his comments. We have not attempted total correction in patients with single ventricle and a common atrioventricular valve, and we commend him for the success with this difficult case. We have used electrical recording to locate the bundle of His and define its relationship to the reconstructed ventricular septum. The slide [slide] illustrates such a recording. The A spike on the electrogram is comparable to the P wave on the electrocardiogram, and the B spike with the QRS complex. The bundle of His occurs in the isoelectric portion of the electrocardiogram between the P wave and the QRS complex and is clearly seen on the electrogram between the A and B spikes. After using this technique in 2 patients in the current series, we believe it was of assistance in avoiding the conduction system during the surgical repair.