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Repair of double-outlet right ventricle
Repair of double-outlet right ventricle The results of repair of double outlet right ventricle in 26 children are reported. Eighteen of these patients had subaortic ventricular septal defect (VSD), without pulmonary stenosis (PS) in 5 and with PS in 13. Another 8 patients had subpulmonic VSD, without PS in 6 and with PS in 2. All had concordant atrioventricular relationships. There were six early deaths and two late deaths of the 26 patients in our study. Of these, 5 patients had severe associated cardiac abnormalities, and 2 who died early had had an incision in the systemic (right) ventricle.
J. C. Harvey, H. M. Sondheimer, W. G. Williams, P. M. Olley, and G. A. Trusler, Toronto, Ontario, Canada
T
A he most useful surgical definition of double-outlet right ventricle (DORV) is that proposed by Lev and associates,1 who described a spectrum of anomalies in which both great vessels arise predominantly from the morphologic right ventricle. This does not exclude defects with mitral-semilunar valve continuity. Surgeons have accepted this definition because the approach to repair of these defects depends upon the relationships of the great vessels to the ventricular septal defect (VSD), without regard to the presence or absence of conal tissue.2 The present study reports our experience with total correction of DORV. Clinical material From 1961 to May, 1976, we repaired DORV in 26 children, all of whom had concordant atrioventricular relationships. We divided the patients into two groups: Group I comprised 18 patients with subaortic VSD (Table I) and Group II comprised 8 with subpulmonic VSD (Table II). The groups were subdivided to denote (a) absence or (b) presence of pulmonary stenosis (PS). Group la: Subaortic VSD without PS (5 patients). The patients were aged 21 months to 5 years at the time of repair. They had viscero-atrial situs solitus, d-loop, and right-sided aorta (S,D,D in the symbolic terminolFrom the Departments of Surgery (Cardiovascular Division) and Cardiology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Received for publication Sept. 23, 1976. Accepted for publication Nov. 18, 1976. Address for reprints: Dr. J. C. Harvey, Department of Surgery, Mount Sinai Hospital, 600 University Avenue, Toronto, Ontario, Canada.
ogy of Van Praagh and associates3). All were small for their ages and had symptoms of pulmonary overcirculation. Pansystolic murmurs were audible at the left sternal border, and thrills were palpable at the third and fourth intercostal spaces. The electrocardiogram (ECG) evidenced right ventricular hypertrophy (and left ventricular hypertrophy in 2 cases), and chest roentgenograms revealed cardiac enlargement with pulmonary plethora. Pressures in the right ventricle and pulmonary artery were at the systemic level at catheterization, but pulmonary vascular resistance was not severly elevated at the time of repair. The pulmonary artery had been banded in 3 patients (Nos. 2 to 4, Table I), at the age of 3 months in 2 and at 4 months in one. Group lb: Subaortic VSD with PS (13 patients). One of these patients (No. 6) had mirror-image dextrocardia: situs inversus, 1-loop, and left-sided aorta (I,L,L). The other 12 had a defect of the S,D,D type. History and physical findings were similar to those in children with tetralogy of Fallot.4 Before shunting, the ECG had indicated right ventricular hypertrophy and chest roentgenograms had shown pulmonary oligemia. At cardiac catheterization, right ventricular and left ventricular pressures were equal in 12 patients, and left ventricular pressure exceeded right ventricular and systemic pressures in one (patient No. 8, who had a restrictive VSD). There was a gradient between the right ventricle and pulmonary artery in all cases. Only cineangiography distinguished these patients from those with tetralogy of Fallot. Five patients had had palliative systemic-pulmonary shunts: a BlalockTaussig shunt in 3 (Patients 6 , 1 1 , and 13, at ages 6, 2, and 5 years, respectively), a Glenn procedure in one (No. 16, at 4 years), and a Potts anastomosis in one (No. 15, at 13 months). 61 1
The Journal of Thoracic and Cardiovascular Surgery
6 1 2 Harvey et al.
Table I Patient No.
Sex
Correction Previous
surgery
Date
Group la: Subaortic VSD without PS March, 1966 1 F None 2 3 4 5
M M F F
PA band (April, 1970) PA band (July, 1968) PA band (Jan., 1969) None
Grouplb: Subaortic VSD with PS 6 F Blalock-Taussig shunt (1961) 7 F None 8 M None 9 10 11
F M F
12 13
F M
14 15 16
17 18
June, 1972 March, 1973 Nov., 1973 April, 1976
Age (yr.) 2% 2'/2 41/2
5 1%
June, 1968
12
Oct., 1969 Dec, 1969
13 3'/2
Associated cardiac problems
Result
Cleft mitral valve discovered at necropsy None ASD Mild mitral regurgitation None
Complete heart block; low cardiac output; died within 24 hr. No cardiac symptoms No cardiac symptoms No cardiac symptoms Hemodynamically well, but severe diffuse brain damage caused by hypoxia
Mirror-image dextrocardia (I,L,L) Left SVC Restrictive VSD
No cardiac symptoms
Sept., 1970 Nov., 1972 May, 1973
6 8 7
May, 1973 Oct., 1973
7 10
ASD; cleft mitral valve None
F M M
None None Blalock-Taussig shunt (1967) None Blalock-Taussig shunt (1968) None Potts shunt (1958) Glenn shunt (1968)
Feb., 1974 June, 1974 June, 1974
4V2 17 9
M F
None None
Nov., 1975 Feb., 1976
15 5%
Absent IVC; bilateral SVC None ASD; severe tricuspid regurgitation and SVC obstruction ASD; PAPVD None
None None None
Died suddenly on Day 3 Residual PS with some pulmonary insufficiency No cardiac symptoms No cardiac symptoms Symptomatically well, but moderate mitral regurgitation No cardiac symptoms No cardiac symptoms Well except for easy fatigability Died lyr. later, 4 days after reoperation to take down Glenn shunt and correct regurgitation No cardiac symptoms No cardiac symptoms
Legend: VSD, Ventricular septal defect. PS, Pulmonary stenosis. PA, Pulmonary artery. ASD, Atrial septal defect. SVC, Superior vena cava. IVC, Inferior vena cava. PAPVD, Partial anomalous pulmonary venous drainage.
Group Ha: Subpulmonary VSD without PS (6 patients). The defect was situs solitus, with the aorta anterior and to the right (S,D,D). In all 6 patients, cyanosis, congestive heart failure, and pulmonary overcirculation had developed in early infancy. Roentgenography revealed pulmonary plethora and enlargement of the heart and pulmonary arteries, and cineangiography was necessary to establish the diagnosis. Pulmonary artery banding had been done in 5 cases, before one month of age in 3 (Nos. 20 to 22, Table II), with simultaneous atrial septal shift (Edwards5 procedure) to improve systemic arterial oxygen saturation in 2 (Nos. 20 and 21) and with coarctectomy in 2 (Nos. 23 and 24) at 2 and 3 months of age, respectively. In the sixth child (No. 19), no palliative procedures were performed before attempted repair at the age of 17 months. Group lib: Subpulmonary VSD with PS (2 patients). One patient (No. 26) had situs solitus, d-loop,
and left-sided aorta (S,D,L); in the other (No. 25) the malformation was of S,D,D type. Both had congestive heart failure and cyanosis in the first month of life, for which they required treatment with digitalis and diuretics. Catheterization demonstrated equal pressures in the right and left ventricles and the aorta. No pulmonary artery pressure was obtainable in one child (No. 25), and there was only a small gradient (20 mm. Hg) across the pulmonary valve in the other. Cineangiography confirmed the diagnosis. The child with severe PS had had a palliative Glenn anastomosis at the age of 8 months. The other child had been treated only medically until repair was undertaken at the age of 13 months. Results of repair Group la: Subaortic VSD without PS. The operative procedure consisted of creation of a tunnel graft from the VSD to the aorta and repair of associated
Volume 73 Number 4 April, 1977
Double-outlet right ventricle
61 3
Table I I Patient No.
Sex
Correction Previous surgery
Date
Age (yr.)
Associated cardiac problems
Group Ila: Subpulmonic VSD without PS 19 M None Feb., 1961
l'/2
20
M
March, 1970
4
Unsuspected mitral stenosis; small LV Restricted VSD
21
F
June, 1973
3'/2
ASD
Feb., 1974 Nov., 1975
4
March, 1976
m
ASD Recurrent coarctation of aorta, small RV Recurrent coarctation of aorta
22 M 23 F 24
M
PA band and Edwards atrial septal shift (1966) PA band and Edwards atrial septal shift (1970) PA band (1969) Coarctectomy and PA band (1971) Coarctectomy and PA band (1974)
Group lib: Subpulmonic VSD with PS 25 M Glenn shunt (1967) Dec, 1974 26 F
None
May, 1976
4
8 1
Unsuspected straddling mitral valve Restrictive VSD
Result Died on day of operation Not permitted competitive sports, but otherwise unrestricted activity Reoperation, 1974; progressive myocardial failure and cardiac arrhythmias; died 2 yr. later No cardiac symptoms Died in operating room Died on day of operation
Low cardiac output; died on Day 3 No cardiac symptoms
Legend: VSD, Ventricular septal defect. PS, Pulmonary stenosis. PA, Pulmonary artery. LV, Left ventricle. ASD, Atrial septal defect. RV, Right ventricle.
cardiac defects. In Cases 2 to 4, the pulmonary artery band was taken down first. One child (No. 1, the first for whom we attempted repair of this defect) had complete heart block postoperatively, and we could not restore cardiac output; necropsy revealed an unsuspected cleft mitral valve. All 4 survivors are hemodynamically well 3 months to 4 years since the reparative operation. However, patient No. 5 has severe diffuse brain damage as a consequence of cardiac arrest due to obstruction of an endotracheal tube. Group lb: Subaortic VSD with PS. Surgical correction, performed between the ages of 3V2 and 17 years, consisted of infundibular resection, tunneling the left ventricular outflow through the VSD into the aorta, and insertion of a pericardial patch to relieve pulmonary outflow-tract obstruction. A pericardial cusp6 was attached to the patch in 3 cases. The defect was enlarged before the tunnel was closed in the patient with a restrictive VSD (No. 8). Systemicpulmonary shunts were repaired in 5 cases, but a Glenn anastomosis was left intact in one other (No. 16). There was one early death (Patient 7), caused by sudden cardiac arrest, and one late death. The latter patient (No. 16), who had a Glenn anastomosis, had severe tricuspid regurgitation and superior vena cava (SVC) obstruction. One year after the first repair, the anastomosis was detached, the SVC was reconstructed, a Dacron graft was used to connect the main pulmo-
nary artery to the right pulmonary artery, and tricuspid annuloplasty was performed. Arrhythmias developed postoperatively and cardiac output remained low. He died 4 days after the operation. The 11 survivors are now 4 months to 8 years of age. Nine enjoy unrestricted activity and, although the other 2 (Nos. 8 and 14) cannot take part in competitive sports, they are encouraged to participate in other normal activities. Group Ha: Subpulmonic VSD without PS. In 3 of these children (Nos. 20, 23, and 24, Table II) after debanding of the pulmonary artery, the defect was repaired by patch closure of the VSD to establish left ventricular-pulmonary arterial continuity and intraatrial transposition of venous return (Mustard7 operation). The VSD was closed via an atriotomy in Patient 20 at 4 years of age, and he is well 6 years later. The defect was repaired through an incision in the right (systemic) ventricle before intra-atrial transposition of venous return in Patients 23 and 24 (aged 4 and \l/i years, respectively). These 2 children had previously undergone coarctectomy and were thought to have mild residual coarctation. Their cardiac output remained low postoperatively, and both died within 24 hours. At necropsy, Patient 23 was thought to have a hypoplastic right ventricle. In Patients 19, 21, and 22, left ventricular-aortic continuity was established with an intraventricular conduit, after debanding of the pulmonary artery in the latter 2 cases. Patient 19, the first in
6 1 4 Harvey et al.
whom we attempted this procedure, was 17 months old at the time of operation. Cardiac output remained low, and he died that day; necropsy revealed unsuspected mitral stenosis and a small left ventricle. Patient 21 required reoperation one year later to relieve obstruction to aortic outflow by the tunnel graft; she had progressive myocardial failure and uncontrollable cardiac arrhythmias, and she died 2 years after reoperation. Patient 22 is well 2l/i years later. Group lib: Subpulmonic VSD with PS. Patient 25, in whom the PS was severe, had had a palliative Glenn anastomosis at 8 months of age. Repair was attempted when he was 8 years old: An intraventricular tunnel graft was inserted to establish left ventricularaortic continuity. A valved external conduit was inserted between the right ventricle and the left pulmonary artery, the Glenn anastomosis being left intact. Severe mitral regurgitation postoperatively necessitated a return to cardiopulmonary bypass for replacement of a previously unsuspected straddling mitral valve. He died 3 days later with complete heart block, low cardiac output, and pulmonary insufficiency. Patient 26 had minimal subvalvular PS. At operation, when she was 13 months old, fibromuscular obstructive tissue was resected from the pulmonary outflow tract and, via an atriotomy, the VSD was patched to establish left ventricular-pulmonary arterial continuity. Finally, the atrial septum was resected, and venous return was transposed in a Mustard operation. She is now clinically well. Discussion DORV with subaortic VSD, with or without PS, can be corrected with little risk of complications and a high probability of good results.8' 9 Our experience in 18 cases includes only two early deaths (among our first 3 patients) and one late death. Since late in 1969, we have corrected this defect in 15 patients, all of whom are still alive: Twelve enjoy unrestricted activity, 2 participate in all activities except competitive sports, and one child had severe brain damage in a probably preventable mishap associated with respiratory care. Our results for total correction of DORV with subpulmonic VSD have not been as satisfactory. Many of our 8 patients had serious associated anomalies, including coarctation of the aorta, mitral valve abnormalities, and hypoplastic ventricles. Previous palliative surgery had been required in 6 patients—pulmonary artery banding in 5 (with an Edwards atrial septal shift in 2) and a Glenn anastomosis in one. The operative procedure is prolonged by the need to deal with previous banding and mitral valve anomalies, and results are
The Journal of Thoracic and Cardiovascular Surgery
further compromised by problems not easily treated simultaneously (such as recoarctation). Four of the 8 patients died the day of operation: In 2 of them, the systemic (right) ventricle had been incised for VSD repair in conjunction with a Mustard operation, and the other 2 had mitral valve anomalies. None of the 4 early survivors had major associated anomalies, and repair was accomplished without incising the systemic ventricle: In 2, closure of the VSD with a tunnel graft established left ventricular-aortic continuity; in the other 2, patch closure established left ventricularpulmonary arterial continuity via an atriotomy, and repair was completed by a Mustard operation. One early survivor required reoperation for left ventricular outflow obstruction by the intraventricular tunnel graft; she had progressive myocardial failure and died within 2 years of the second operation. Left ventricular outflow obstruction as a complication of intraventricular tunnel grafting occurred in another patient (not reported in this series) whose repair was performed elsewhere. At reoperation, our observations were similar to those in our Case 21. The other survivor of the intraventricular tunnel-graft repair is well 2V4 years later. The 2 early survivors in whom the ventricle was not incised are still well, 4 months and 6 years after operation. Our experience shows that successful repair of DORV with subpulmonic VSD is possible, particularly in patients with no other severe anomalies. Incisions in what is to become the systemic ventricle should be avoided. The VSD should be assessed through an atrial incision and, if possible, should be repaired through an atriotomy. In most cases this repair will tunnel the left ventricular outflow to the pulmonary artery, and a Mustard operation will be necessary to transpose venous return. If a VSD cannot be repaired through the atrium and it appears the left ventricular-aortic continuity can be established through graft closure of the VSD, a right ventriculotomy should be done. However, left ventricular outflow obstruction is a hazard of this type of repair. The tunnel must be ample, and it should be sutured some distance from the aortic valve ring. In cases of severe subvalvular PS, a valved external conduit from the right ventricle to the main pulmonary artery10 can be used to complete the repair. Pacifico and associates11 suggested that a better approach to repair of subpulmonic VSD in cases of DORV may be via a left ventriculotomy, the repair being completed with a valved external conduit from the left ventricle to the pulmonary artery, followed by interatrial transposition of venous return. This approach, which avoids incising the systemic ventricle, could be used for patients whose
Volume 73 Number 4 April, 1977
Double-outlet right ventricle
anatomy is unfavorable for either creation of left ventricular-aortic continuity or transatrial repair of the VSD. Summary We repaired DORV in 26 patients. Group I comprised 18 patients with subaortic VSD, without PS in 5 and with PS in 13. Group II comprised 8 patients with subpulmonic VSD, without PS in 6 and with PS in 2. All had concordant atrioventricular relationships. In Group I there were two early deaths and one late death; there were no early deaths among the 15 most recent cases. In Group II there were four early deaths and one late death; all 5 patients had severe associated cardiac abnormalities, and the 2 who died early had had an incision in the systemic (right) ventricle. The Medical Publications Department, The Hospital for Sick Children, Toronto, Ontario, Canada, assisted in the preparation of this manuscript. REFERENCES 1 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. 2 Stewart, S.: Double-Outlet Right Ventricle: A Collective Review With a Surgical Viewpoint, J. THORAC. CARDIOVASC. SURG. 71: 355,
1976.
3 Van Praagh, R., Perez-Trevino, C , Reynolds, J. L., Moes, C. A. F., Keith, J. D., Roy, D. L., Belcourt, C , Weinberg, P. M., and Parisi, L. F.: Double Outlet Right Ventricle(S,D,L)WithSubaortic Ventricular Septal Defect
615
and Pulmonary Stenosis: Report of Six Cases, Am. J. Cardiol. 35: 42, 1975. 4 Sondheimer, H. M., Freedom, R. M., and Olley, P. M.: The Natural History of Double Outlet Right Ventricle (Abst.), Pediatr. Res. 10: 317, 1976. 5 Edwards, W. S., Bargeron, L. M., Jr., and Lyons, C : Reposition of Right Pulmonary Veins in Transposition of Great Vessels, J.A.M.A. 188: 522, 1964. 6 Trusler, G. A., Iyengar, S. R., and Mustard, W. T.: Reconstruction of the Pulmonary Valve and Outflow Tract: A Report of 25 Cases, J. THORAC. CARDIOVASC. SURG. 65: 245,
1973.
7 Mustard, W. T.: Successful Two-Stage Correction of Transposition of the Great Vessels, Surgery 55: 469, 1964. 8 Gomes, M. M. R., Weidman, W. H., McGoon, D. C , and Danielson, G. K.: Double-Outlet Right Ventricle With Pulmonic Stenosis: Surgical Considerations and Results of Operation, Circulation 43: 889, 1971. 9 Gomes, M. M. R., Weidman, W. H., McGoon, D. C , and Danielson, G. K.: Double-Outlet Right Ventricle Without Pulmonic Stenosis: Surgical Considerations and Results of Operation, Circulation 43, 44: 131, 1971 (Suppl. I). 10 McGoon, D. C , Wallace, R. B., and Danielson, G. K.: The Rastelli Operation: Its Indications and Results, J. THORAC. CARDIOVASC. SURG. 65: 65, 1973.
11 Pacifico, A. D., Kirklin, J. W., and Bargeron, L. M., Jr.: Complex Congenital Malformations: Surgical Treatment of Double-Outlet Right Ventricle and Double-Outlet Left Ventricle, in Kirklin, J. W., editor: Advances in Cardiovascular Surgery, New York and London, 1973, Grune & Stratton, Inc., pp. 57-76.
J. C. Harvey, H. M. Sondheimer, W. G. Williams, P. M. Olley, and G. A. Trusler, Toronto, Ontario, Canada
T
A he most useful surgical definition of double-outlet right ventricle (DORV) is that proposed by Lev and associates,1 who described a spectrum of anomalies in which both great vessels arise predominantly from the morphologic right ventricle. This does not exclude defects with mitral-semilunar valve continuity. Surgeons have accepted this definition because the approach to repair of these defects depends upon the relationships of the great vessels to the ventricular septal defect (VSD), without regard to the presence or absence of conal tissue.2 The present study reports our experience with total correction of DORV. Clinical material From 1961 to May, 1976, we repaired DORV in 26 children, all of whom had concordant atrioventricular relationships. We divided the patients into two groups: Group I comprised 18 patients with subaortic VSD (Table I) and Group II comprised 8 with subpulmonic VSD (Table II). The groups were subdivided to denote (a) absence or (b) presence of pulmonary stenosis (PS). Group la: Subaortic VSD without PS (5 patients). The patients were aged 21 months to 5 years at the time of repair. They had viscero-atrial situs solitus, d-loop, and right-sided aorta (S,D,D in the symbolic terminolFrom the Departments of Surgery (Cardiovascular Division) and Cardiology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Received for publication Sept. 23, 1976. Accepted for publication Nov. 18, 1976. Address for reprints: Dr. J. C. Harvey, Department of Surgery, Mount Sinai Hospital, 600 University Avenue, Toronto, Ontario, Canada.
ogy of Van Praagh and associates3). All were small for their ages and had symptoms of pulmonary overcirculation. Pansystolic murmurs were audible at the left sternal border, and thrills were palpable at the third and fourth intercostal spaces. The electrocardiogram (ECG) evidenced right ventricular hypertrophy (and left ventricular hypertrophy in 2 cases), and chest roentgenograms revealed cardiac enlargement with pulmonary plethora. Pressures in the right ventricle and pulmonary artery were at the systemic level at catheterization, but pulmonary vascular resistance was not severly elevated at the time of repair. The pulmonary artery had been banded in 3 patients (Nos. 2 to 4, Table I), at the age of 3 months in 2 and at 4 months in one. Group lb: Subaortic VSD with PS (13 patients). One of these patients (No. 6) had mirror-image dextrocardia: situs inversus, 1-loop, and left-sided aorta (I,L,L). The other 12 had a defect of the S,D,D type. History and physical findings were similar to those in children with tetralogy of Fallot.4 Before shunting, the ECG had indicated right ventricular hypertrophy and chest roentgenograms had shown pulmonary oligemia. At cardiac catheterization, right ventricular and left ventricular pressures were equal in 12 patients, and left ventricular pressure exceeded right ventricular and systemic pressures in one (patient No. 8, who had a restrictive VSD). There was a gradient between the right ventricle and pulmonary artery in all cases. Only cineangiography distinguished these patients from those with tetralogy of Fallot. Five patients had had palliative systemic-pulmonary shunts: a BlalockTaussig shunt in 3 (Patients 6 , 1 1 , and 13, at ages 6, 2, and 5 years, respectively), a Glenn procedure in one (No. 16, at 4 years), and a Potts anastomosis in one (No. 15, at 13 months). 61 1
The Journal of Thoracic and Cardiovascular Surgery
6 1 2 Harvey et al.
Table I Patient No.
Sex
Correction Previous
surgery
Date
Group la: Subaortic VSD without PS March, 1966 1 F None 2 3 4 5
M M F F
PA band (April, 1970) PA band (July, 1968) PA band (Jan., 1969) None
Grouplb: Subaortic VSD with PS 6 F Blalock-Taussig shunt (1961) 7 F None 8 M None 9 10 11
F M F
12 13
F M
14 15 16
17 18
June, 1972 March, 1973 Nov., 1973 April, 1976
Age (yr.) 2% 2'/2 41/2
5 1%
June, 1968
12
Oct., 1969 Dec, 1969
13 3'/2
Associated cardiac problems
Result
Cleft mitral valve discovered at necropsy None ASD Mild mitral regurgitation None
Complete heart block; low cardiac output; died within 24 hr. No cardiac symptoms No cardiac symptoms No cardiac symptoms Hemodynamically well, but severe diffuse brain damage caused by hypoxia
Mirror-image dextrocardia (I,L,L) Left SVC Restrictive VSD
No cardiac symptoms
Sept., 1970 Nov., 1972 May, 1973
6 8 7
May, 1973 Oct., 1973
7 10
ASD; cleft mitral valve None
F M M
None None Blalock-Taussig shunt (1967) None Blalock-Taussig shunt (1968) None Potts shunt (1958) Glenn shunt (1968)
Feb., 1974 June, 1974 June, 1974
4V2 17 9
M F
None None
Nov., 1975 Feb., 1976
15 5%
Absent IVC; bilateral SVC None ASD; severe tricuspid regurgitation and SVC obstruction ASD; PAPVD None
None None None
Died suddenly on Day 3 Residual PS with some pulmonary insufficiency No cardiac symptoms No cardiac symptoms Symptomatically well, but moderate mitral regurgitation No cardiac symptoms No cardiac symptoms Well except for easy fatigability Died lyr. later, 4 days after reoperation to take down Glenn shunt and correct regurgitation No cardiac symptoms No cardiac symptoms
Legend: VSD, Ventricular septal defect. PS, Pulmonary stenosis. PA, Pulmonary artery. ASD, Atrial septal defect. SVC, Superior vena cava. IVC, Inferior vena cava. PAPVD, Partial anomalous pulmonary venous drainage.
Group Ha: Subpulmonary VSD without PS (6 patients). The defect was situs solitus, with the aorta anterior and to the right (S,D,D). In all 6 patients, cyanosis, congestive heart failure, and pulmonary overcirculation had developed in early infancy. Roentgenography revealed pulmonary plethora and enlargement of the heart and pulmonary arteries, and cineangiography was necessary to establish the diagnosis. Pulmonary artery banding had been done in 5 cases, before one month of age in 3 (Nos. 20 to 22, Table II), with simultaneous atrial septal shift (Edwards5 procedure) to improve systemic arterial oxygen saturation in 2 (Nos. 20 and 21) and with coarctectomy in 2 (Nos. 23 and 24) at 2 and 3 months of age, respectively. In the sixth child (No. 19), no palliative procedures were performed before attempted repair at the age of 17 months. Group lib: Subpulmonary VSD with PS (2 patients). One patient (No. 26) had situs solitus, d-loop,
and left-sided aorta (S,D,L); in the other (No. 25) the malformation was of S,D,D type. Both had congestive heart failure and cyanosis in the first month of life, for which they required treatment with digitalis and diuretics. Catheterization demonstrated equal pressures in the right and left ventricles and the aorta. No pulmonary artery pressure was obtainable in one child (No. 25), and there was only a small gradient (20 mm. Hg) across the pulmonary valve in the other. Cineangiography confirmed the diagnosis. The child with severe PS had had a palliative Glenn anastomosis at the age of 8 months. The other child had been treated only medically until repair was undertaken at the age of 13 months. Results of repair Group la: Subaortic VSD without PS. The operative procedure consisted of creation of a tunnel graft from the VSD to the aorta and repair of associated
Volume 73 Number 4 April, 1977
Double-outlet right ventricle
61 3
Table I I Patient No.
Sex
Correction Previous surgery
Date
Age (yr.)
Associated cardiac problems
Group Ila: Subpulmonic VSD without PS 19 M None Feb., 1961
l'/2
20
M
March, 1970
4
Unsuspected mitral stenosis; small LV Restricted VSD
21
F
June, 1973
3'/2
ASD
Feb., 1974 Nov., 1975
4
March, 1976
m
ASD Recurrent coarctation of aorta, small RV Recurrent coarctation of aorta
22 M 23 F 24
M
PA band and Edwards atrial septal shift (1966) PA band and Edwards atrial septal shift (1970) PA band (1969) Coarctectomy and PA band (1971) Coarctectomy and PA band (1974)
Group lib: Subpulmonic VSD with PS 25 M Glenn shunt (1967) Dec, 1974 26 F
None
May, 1976
4
8 1
Unsuspected straddling mitral valve Restrictive VSD
Result Died on day of operation Not permitted competitive sports, but otherwise unrestricted activity Reoperation, 1974; progressive myocardial failure and cardiac arrhythmias; died 2 yr. later No cardiac symptoms Died in operating room Died on day of operation
Low cardiac output; died on Day 3 No cardiac symptoms
Legend: VSD, Ventricular septal defect. PS, Pulmonary stenosis. PA, Pulmonary artery. LV, Left ventricle. ASD, Atrial septal defect. RV, Right ventricle.
cardiac defects. In Cases 2 to 4, the pulmonary artery band was taken down first. One child (No. 1, the first for whom we attempted repair of this defect) had complete heart block postoperatively, and we could not restore cardiac output; necropsy revealed an unsuspected cleft mitral valve. All 4 survivors are hemodynamically well 3 months to 4 years since the reparative operation. However, patient No. 5 has severe diffuse brain damage as a consequence of cardiac arrest due to obstruction of an endotracheal tube. Group lb: Subaortic VSD with PS. Surgical correction, performed between the ages of 3V2 and 17 years, consisted of infundibular resection, tunneling the left ventricular outflow through the VSD into the aorta, and insertion of a pericardial patch to relieve pulmonary outflow-tract obstruction. A pericardial cusp6 was attached to the patch in 3 cases. The defect was enlarged before the tunnel was closed in the patient with a restrictive VSD (No. 8). Systemicpulmonary shunts were repaired in 5 cases, but a Glenn anastomosis was left intact in one other (No. 16). There was one early death (Patient 7), caused by sudden cardiac arrest, and one late death. The latter patient (No. 16), who had a Glenn anastomosis, had severe tricuspid regurgitation and superior vena cava (SVC) obstruction. One year after the first repair, the anastomosis was detached, the SVC was reconstructed, a Dacron graft was used to connect the main pulmo-
nary artery to the right pulmonary artery, and tricuspid annuloplasty was performed. Arrhythmias developed postoperatively and cardiac output remained low. He died 4 days after the operation. The 11 survivors are now 4 months to 8 years of age. Nine enjoy unrestricted activity and, although the other 2 (Nos. 8 and 14) cannot take part in competitive sports, they are encouraged to participate in other normal activities. Group Ha: Subpulmonic VSD without PS. In 3 of these children (Nos. 20, 23, and 24, Table II) after debanding of the pulmonary artery, the defect was repaired by patch closure of the VSD to establish left ventricular-pulmonary arterial continuity and intraatrial transposition of venous return (Mustard7 operation). The VSD was closed via an atriotomy in Patient 20 at 4 years of age, and he is well 6 years later. The defect was repaired through an incision in the right (systemic) ventricle before intra-atrial transposition of venous return in Patients 23 and 24 (aged 4 and \l/i years, respectively). These 2 children had previously undergone coarctectomy and were thought to have mild residual coarctation. Their cardiac output remained low postoperatively, and both died within 24 hours. At necropsy, Patient 23 was thought to have a hypoplastic right ventricle. In Patients 19, 21, and 22, left ventricular-aortic continuity was established with an intraventricular conduit, after debanding of the pulmonary artery in the latter 2 cases. Patient 19, the first in
6 1 4 Harvey et al.
whom we attempted this procedure, was 17 months old at the time of operation. Cardiac output remained low, and he died that day; necropsy revealed unsuspected mitral stenosis and a small left ventricle. Patient 21 required reoperation one year later to relieve obstruction to aortic outflow by the tunnel graft; she had progressive myocardial failure and uncontrollable cardiac arrhythmias, and she died 2 years after reoperation. Patient 22 is well 2l/i years later. Group lib: Subpulmonic VSD with PS. Patient 25, in whom the PS was severe, had had a palliative Glenn anastomosis at 8 months of age. Repair was attempted when he was 8 years old: An intraventricular tunnel graft was inserted to establish left ventricularaortic continuity. A valved external conduit was inserted between the right ventricle and the left pulmonary artery, the Glenn anastomosis being left intact. Severe mitral regurgitation postoperatively necessitated a return to cardiopulmonary bypass for replacement of a previously unsuspected straddling mitral valve. He died 3 days later with complete heart block, low cardiac output, and pulmonary insufficiency. Patient 26 had minimal subvalvular PS. At operation, when she was 13 months old, fibromuscular obstructive tissue was resected from the pulmonary outflow tract and, via an atriotomy, the VSD was patched to establish left ventricular-pulmonary arterial continuity. Finally, the atrial septum was resected, and venous return was transposed in a Mustard operation. She is now clinically well. Discussion DORV with subaortic VSD, with or without PS, can be corrected with little risk of complications and a high probability of good results.8' 9 Our experience in 18 cases includes only two early deaths (among our first 3 patients) and one late death. Since late in 1969, we have corrected this defect in 15 patients, all of whom are still alive: Twelve enjoy unrestricted activity, 2 participate in all activities except competitive sports, and one child had severe brain damage in a probably preventable mishap associated with respiratory care. Our results for total correction of DORV with subpulmonic VSD have not been as satisfactory. Many of our 8 patients had serious associated anomalies, including coarctation of the aorta, mitral valve abnormalities, and hypoplastic ventricles. Previous palliative surgery had been required in 6 patients—pulmonary artery banding in 5 (with an Edwards atrial septal shift in 2) and a Glenn anastomosis in one. The operative procedure is prolonged by the need to deal with previous banding and mitral valve anomalies, and results are
The Journal of Thoracic and Cardiovascular Surgery
further compromised by problems not easily treated simultaneously (such as recoarctation). Four of the 8 patients died the day of operation: In 2 of them, the systemic (right) ventricle had been incised for VSD repair in conjunction with a Mustard operation, and the other 2 had mitral valve anomalies. None of the 4 early survivors had major associated anomalies, and repair was accomplished without incising the systemic ventricle: In 2, closure of the VSD with a tunnel graft established left ventricular-aortic continuity; in the other 2, patch closure established left ventricularpulmonary arterial continuity via an atriotomy, and repair was completed by a Mustard operation. One early survivor required reoperation for left ventricular outflow obstruction by the intraventricular tunnel graft; she had progressive myocardial failure and died within 2 years of the second operation. Left ventricular outflow obstruction as a complication of intraventricular tunnel grafting occurred in another patient (not reported in this series) whose repair was performed elsewhere. At reoperation, our observations were similar to those in our Case 21. The other survivor of the intraventricular tunnel-graft repair is well 2V4 years later. The 2 early survivors in whom the ventricle was not incised are still well, 4 months and 6 years after operation. Our experience shows that successful repair of DORV with subpulmonic VSD is possible, particularly in patients with no other severe anomalies. Incisions in what is to become the systemic ventricle should be avoided. The VSD should be assessed through an atrial incision and, if possible, should be repaired through an atriotomy. In most cases this repair will tunnel the left ventricular outflow to the pulmonary artery, and a Mustard operation will be necessary to transpose venous return. If a VSD cannot be repaired through the atrium and it appears the left ventricular-aortic continuity can be established through graft closure of the VSD, a right ventriculotomy should be done. However, left ventricular outflow obstruction is a hazard of this type of repair. The tunnel must be ample, and it should be sutured some distance from the aortic valve ring. In cases of severe subvalvular PS, a valved external conduit from the right ventricle to the main pulmonary artery10 can be used to complete the repair. Pacifico and associates11 suggested that a better approach to repair of subpulmonic VSD in cases of DORV may be via a left ventriculotomy, the repair being completed with a valved external conduit from the left ventricle to the pulmonary artery, followed by interatrial transposition of venous return. This approach, which avoids incising the systemic ventricle, could be used for patients whose
Volume 73 Number 4 April, 1977
Double-outlet right ventricle
anatomy is unfavorable for either creation of left ventricular-aortic continuity or transatrial repair of the VSD. Summary We repaired DORV in 26 patients. Group I comprised 18 patients with subaortic VSD, without PS in 5 and with PS in 13. Group II comprised 8 patients with subpulmonic VSD, without PS in 6 and with PS in 2. All had concordant atrioventricular relationships. In Group I there were two early deaths and one late death; there were no early deaths among the 15 most recent cases. In Group II there were four early deaths and one late death; all 5 patients had severe associated cardiac abnormalities, and the 2 who died early had had an incision in the systemic (right) ventricle. The Medical Publications Department, The Hospital for Sick Children, Toronto, Ontario, Canada, assisted in the preparation of this manuscript. REFERENCES 1 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. 2 Stewart, S.: Double-Outlet Right Ventricle: A Collective Review With a Surgical Viewpoint, J. THORAC. CARDIOVASC. SURG. 71: 355,
1976.
3 Van Praagh, R., Perez-Trevino, C , Reynolds, J. L., Moes, C. A. F., Keith, J. D., Roy, D. L., Belcourt, C , Weinberg, P. M., and Parisi, L. F.: Double Outlet Right Ventricle(S,D,L)WithSubaortic Ventricular Septal Defect
615
and Pulmonary Stenosis: Report of Six Cases, Am. J. Cardiol. 35: 42, 1975. 4 Sondheimer, H. M., Freedom, R. M., and Olley, P. M.: The Natural History of Double Outlet Right Ventricle (Abst.), Pediatr. Res. 10: 317, 1976. 5 Edwards, W. S., Bargeron, L. M., Jr., and Lyons, C : Reposition of Right Pulmonary Veins in Transposition of Great Vessels, J.A.M.A. 188: 522, 1964. 6 Trusler, G. A., Iyengar, S. R., and Mustard, W. T.: Reconstruction of the Pulmonary Valve and Outflow Tract: A Report of 25 Cases, J. THORAC. CARDIOVASC. SURG. 65: 245,
1973.
7 Mustard, W. T.: Successful Two-Stage Correction of Transposition of the Great Vessels, Surgery 55: 469, 1964. 8 Gomes, M. M. R., Weidman, W. H., McGoon, D. C , and Danielson, G. K.: Double-Outlet Right Ventricle With Pulmonic Stenosis: Surgical Considerations and Results of Operation, Circulation 43: 889, 1971. 9 Gomes, M. M. R., Weidman, W. H., McGoon, D. C , and Danielson, G. K.: Double-Outlet Right Ventricle Without Pulmonic Stenosis: Surgical Considerations and Results of Operation, Circulation 43, 44: 131, 1971 (Suppl. I). 10 McGoon, D. C , Wallace, R. B., and Danielson, G. K.: The Rastelli Operation: Its Indications and Results, J. THORAC. CARDIOVASC. SURG. 65: 65, 1973.
11 Pacifico, A. D., Kirklin, J. W., and Bargeron, L. M., Jr.: Complex Congenital Malformations: Surgical Treatment of Double-Outlet Right Ventricle and Double-Outlet Left Ventricle, in Kirklin, J. W., editor: Advances in Cardiovascular Surgery, New York and London, 1973, Grune & Stratton, Inc., pp. 57-76.