Evaluation of the Damus-Kaye-Stansel Operation in Infancy Duccio C. di Carlo, MD, Roberto M. Di Donato, MD, Adriano Carotti, MD, Luigi Ballerini, MD, and Carlo Marcelletti, MD Dipartimento Medico-Chirurgico di Cardiologia Pediatrica, Ospedale Bambino Gesu, Roma, Italia
Thirteen patients, 12 of whom younger than 2 years, underwent a Damus-Kaye-Stansel procedure for complete transposition of the great arteries, ventricular septal defect, or double-outlet right ventricle and subpulmonary ventricular septal defect. In 6 patients, associated cardiac anomalies caused systemic flow obstruction. There were six hospital deaths (mortality rate, 42%).In a mean follow-up period of 57 months, 5 of 7 survivors required relief of right ventricular hypertension through conduit replacement or enlargement (4 patients) or conduit valve balloon dilation (1patient). The aortic valve became regurgitant in 2 patients in whom it had been left in potential connection with the right ventricle. One patient has moderate pulmonary valve regurgitation. The main advantage of the Damus-Kaye-Stansel procedure is
that it avoids coronary relocation; also, the spatial rdationship of the great arteries and the coronary anatomy do not affect its feasibility. One drawback is the need for a conduit in infancy. Our present indication for DamusKaye-Stansel procedure is confined to double-outlet right ventricle with subpulmonary ventricular septal defect; 5 of 6 patients survived repair in this series. Possible indications are for patients with associated subaortic obstruction or unusual coronary arrangements. Fresh1 or cryopreserved homografts as extracardiac conduits and primary closure of the subaortic area may reduce the need for reoperation after Damus-Kaye-Stansel proicedure.
T
with VSD, and double-outlet right ventricle with s u b p l monary VSD treated by the Damus-Kaye-Stansel (DIG) procedure. Their results indicated a high mortality rate in patients less than 18 months of age. We report on a smaller sample of infants and children treated with the same procedure to highlight the effect of age on immediate survival.
he advantage of anatomically corrective procedures [l-51 for patients with complete transposition of the great arteries and ventricular septal defect (VSD) is generally accepted. Early and midterm results of intraatrial repair and VSD closure are, in fact, unsatisfactory [4-61. Since its introduction in 1976 [l], the arterial switch operation has been widely accepted as the procedure of choice for anatomical correction. For patients with associated left ventricular outflow tract obstruction, the Rastelli [7] and REV [8] procedures are also important in the surgical repertoire.
For editorial comment, see page 1033. Fewer reports have appeared on the procedure advocated separately by Damus, Kaye, and Stansel [9-121: essentially an arterial switch operation without coronary reimplantation. The pulmonary trunk is anastomosed end-to-side to the ascending aorta in order to constitute an outlet from the left ventricle, and continuity between the right ventricle and the bifurcation of the pulmonary trunk is established by an extracardiac valved conduit. Part of this procedure has been successfully applied as a means to bypass subaortic obstruction in certain types of single ventricle [13, 141. The Mayo Clinic group [15] has published the largest series of patients with simple transposition, transposition Accepted for publication June 19, 1991. Address reprint requests to Dr di Carlo, Ospedale Pediatric0 Bambino Gesu, Piazza S . Onofrio 4, 00165 Roma, Italia.
0 1991 by The Society of Thoracic Surgeons
(Ann Tkorac Surg 1991;52:1148-53)
Material and Methods
Patients In a 7-year period we operated on 13 patients. Seven of them had complete transposition and VSD, and 6 had double-outlet right ventricle with subpulmonary VSD (Taussig-Bing anomaly). Mean age and weight at operation were 8.2 months (range, 12 days to 39 months) and 5.2 kg (range, 3 to 12.5 kg), respectively. In 6 patients, associated anomalies resulted in obstriiction of systemic flow. Aortic arch interruption was present in 2 patients and coarctation in 2 others; 2 patients had subaortic obstruction, in 1 associated with hypoplastic aortic arch and coarctation. Previous palliative procedures are indicated in Table 1.
Surgical Technique Standard hypothermic cardiopulmonary bypass with crystalloid cardioplegia was used in 9 patients; in 4 others, deep hypothermia and circulatory arrest were employed. The aorta was dissected from the pulmonary trunk and cannulated as far distally as possible. Each caval vein was cannulated separately through the atrium. A single atrial 0003-4975/91/$350
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Table 1. Clinical Characteristics and Surgical Results ~~
Age at
Mode of Aortic Valve
Patient Operation Weight Associated No (mo) Diagnosis (kg) Malformations
Previous Operations
VSD VSD
AAI
AAI repair
...
...
SubAo St
...
...
PAB ... Subcl flap
Separate patch
. . .
VSD patch VSD patch VSD patch ...
Alive
1 2 3 4 5 6 7 8 9 10 11 12 13
0.5 3 5 6 7 7 8 2 3 7 8 11 42
TGA, TGA, TGA, TGA, TGA, TGA, TGA,
VSD VSD VSD
VSD VSD TB TB TB TB TB TB
3.5 4.3 4.5 4.2 5.6 4.5 5.4
3 4.3 6.2 4.3 5.8 12.5
LSVC CoAo
... CoAo, SubAo St ... AAI
... ...
CoAo
...
... AAI repair, PAB
Closure
Outcome
VSD patch VSD patch VSD patch Separate patch
Separate patch
Leaflet suture A sept, PAB Leaflet suture Subcl flap, PAB VSD patch . . .
Follow-up
Semilunar
Re-
Valve
(mo)
operation
Died
... 30 60
... No Yes
...
Alive
... ...
Alive Died
. . .
Died Died
...
...
...
Alive
73 54 86 87 9
Yes Yes BD; yes BD No
Alive Alive
...
...
...
Died Died
Alive
Fate
...
A1 A1 PI
...
BD = balloon dilation of conduit valve; CoAo = AAI = aortic arch interruption; A1 = aortic insufficiency; A sept = atrial septectomy; PAB = pulmonary artery banding; PI = pulmonary valve insufficiency; SubAo St coarctation of the aorta; LSVC = left superior vena cava; TGA = transposition of TB = Taussig-Bing type of double-outlet right ventricle; = subaortic stenosis; Subcl flap = subclavian flap aortoplasty; VSD = ventricular septal defect. the great arteries;
cannula was placed if circulatory arrest was planned. Bypass time averaged 155 minutes (range, 127 to 217 minutes) and aortic cross-clamp time, 77 minutes (range, 49 to 143 minutes). During the cooling period, the pulmonary trunk was transected close to its bifurcation or at the site of previous banding; the proximal stump was tailored obliquely to face the ascending aorta. We introduced two modifications of the original technique: first, the aortic pathway was routinely occluded (except in 1 patient whose aortic valve was left open and connected with the right ventricle). Second, after the aorta-pulmonary artery anastomosis was completed (Fig lA), the pulmonary confluence was closed either directly or with a pericardial patch, the arteriotomy was extended to the main right pulmonary branch (Fig lB), and the valved conduit was anastomosed to it (Fig 1C). This maneuver allows the conduit to be positioned to the right of the ascending aorta and the aorta-pulmonary anastomosis to lie posterior and to the left of the ascending aortic segment, so that it is more accessible for control of hemostasis. Various techniques were employed for the first described modification (Table 1). In the last 6 patients, the VSD patch was extended to the subaortic region, thereby placing the semilunar valve in communication with the left ventricle in a similar fashion to the Rastelli procedure (Fig 2). A wide-open subaortic tunnel is not necessary, as the left ventricle has an unobstructed outlet through the pulmonary trunk. Heterograft valved conduits, size 12 or 14, were used to complete the operation. In 1 patient, a hypoplastic aortic arch associated with coarctation required pericardial patch reconstruction extended beyond the coarctation site.
Results Six patients did not survive the immediate postoperative period (early mortality rate, 42%). Of 6 patients with double-outlet right ventricle, 5 survived. One 12-day-old baby in desperate condition was operated on 24 hours after repair of concomitant interruption of the aortic arch; the pulmonary trunk had not been banded in this patient. One patient died of respiratory insufficiency after massive blood transfusion in the operating room. The pulmonary trunk was paper-thin after pulmonary artery banding and profuse bleeding occurred from the aorta-pulmonary anastomosis. Acute myocardial failure followed an untimely attempt at extubation of another, apparently stable, child. In another patient, coronary air embolism occurred before the institution of cardiopulmonary bypass; this was the only patient in the sample whose left ventricular systolic pressure was less than systemic (ratio, 0.68) despite the presence of a ventricular septal defect. Two deaths followed Staphylococcus aureus septicemia. In 1 patient endocarditis occurred after an otherwise uneventful postoperative recovery. Mediastinitis developed in the other patient after urgent DKS procedure; his condition deteriorated after cardiac catheterization complicated by iliac vein thrombosis. In retrospect, technical mistakes or errors of judgment resulted in the death of three patients. The role of an inadequately prepared left ventricle in causing a fatal outcome in 1 patient is debatable. Follow-up to date averages 57 months (range, 9 to 87 months). No patient died in this period. Of the 7 survivors, 4 required further surgical treatment. The right ventricle-pulmonary artery conduit became stenotic and was replaced with an aortic homograft (2 patients) or
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CARL0 ET AL DAMUS OPERATION IN INFANTS
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Ann Thorac Surg 1991;52:1148-53
Fig I. Modified Damus-Kaye-Stansel procedure. (See text for explanation.)
enlarged with a pericardial roof (1 patient) at 6, 7, and 3 years after the DKS procedure. One patient required three reoperations because of conduit stenosis and native aortic valve regurgitation. An aortic homograft was implanted at the second procedure but had to be replaced after 8 months owing to angulation under the sternum. Conduit valve stenosis was identified as the main cause of right ventricle-pulmonary artery gradient and was treated by balloon dilation in 2 patients: in both the gradient was partially relieved (from 75 to 41 mm Hg and from 45 to 25 mm Hg). In 1 of these patients the conduit was replaced 2% years later, whereas the other continues to do well, but has moderate right ventricular hypertension and dysfunction. Finally, 2 patients, both operated on in the last 2 years, are well with no evidence of right ventricular outflow tract obstruction. On the whole, 5 of 7 early survivors needed some type of right ventricular decompression.
Semilunar valve regurgitation was found in 3 patients: in 1 there was moderate pulmonary valve insufficiency, and in 2 there was aortic insufficiency. In the latter 2 patients the aortic valve was left anatomically connected to the right ventricle, with sutured leaflets in 1. This patient required reoperation for repeat suture of the aortic valve leaflets. One other patient has pulmonary insufficiency; despite this problem and associated right ventricular outflow tract obstruction he is currently asymptomatic. Pulmonary artery banding had previously been performed in this patient. The angiographic and crosssectional echocardiographic appearance of the left and right outflow tracts after the DKS procedure are shown in Figures 3 and 4. Figure 5 shows the left ventriculograni of a patient in whom a double outlet from the left ventricle was surgically created. With this technique a wash-out effect of the sinuses of Valsalva and coronary ostia is obtained through the natural pathway.
DI CARLO ET AL DAMUS OPERATION IN INFANTS
A n n Thorac Surg 1991;52:114&53
1151
observed after arterial switch operation [>5] and palliative pulmonary-ascending aorta anastomosis [ 17, 181. This complication occurred in 1 of the patients in our sample (14% of early survivors). Its cause is unclear; if the
Fig 2. "Limited connection" tt,chnique: the rrght ventricle-yulmonary artery conduit is omitted for clarity of illusfration. (See text for explanation.) (Ao = aorta; LV = kft ventricle; PA = pulnionary artery; RV = right ventricle.)
A
Comment In this small sample the hospital mortality was high, reflecting the complexity of the repair during the learning curve. These statistics are similar to the first results of the arterial switch operation. The early mortality in infants younger than 18 months was 50%; the Mayo Clinic group obtained similar results ( p = 0.77, not significant). Low age and weight in our experience did not preclude surgical success, but the use of a prosthetic conduit for right ventricle-pulmonary artery connection substantially contributed to morbidity and, in some cases, mortality. From this point of view the procedure must be considered no more than palliative. The negative impact of residual defects such as pulmonary outflow tract stenosis, pulmonary insufficiency, or a combination of the two on late survival must be kept in mind. In this sample, all patients with a longer follow-up interval than 36 months had to undergo further operations. Some of them still have mild or moderate right ventricular outflow tract pressure gradients and right ventricular dysfunction. In most reoperations fresh or cryopreserved homografts replaced the original conduits; in an "adult" size, these may offer a satisfactory performance for an indefinite number of years [16]. Another cause of reoperation was aortic incompetence, which occurred when this valve was left communicating with the right ventricle. Because of the peculiar physiology of the DKS procedure, aortic insufficiency results in varying degrees Of left-to-right shunt and poorly tolerated [ 171. The native Pulmonary valve, as in the Jatene operation (arterial switch), will always work under systemic pressure load. Late development of regurgitation has been
B
Fig 3 . Angiographic (A)and cross-sectional echocardio~ruphic( B ) appeararice of the right ventricular outlet after the Damus-KayeStaiisel procedure; the black asterisk marks the conduit valve, and the white asterisks mark the tnairi pulmonary branches. (C = con duit; RA = righf atrium; RV = right rientricle.)
1152
CARLO ET AL DAMUS OPERATION IN INFANTS DI
Ann Thorac Surg 1991;52:114.%53
valve were intrinsically unable to function as the aortic valve, the incidence of regurgitation should be similar for the two arterial switch procedures.
Fig 5. Leff ventricular contrast injection; in this putient the subao.rtic region was excluded from the right ventricle by extension of the venfricular septal defect patch. A double-outlet left ventricle is demonstrated (arrows).
A
B
FIg 4. Angiographic (A) and cross-sectional echocardiographic f B ) appearance of the leff ventricular outlet after Damus-Kay-Stansel procedure. (A = ascending aorta; LV = left ventricle; P = pulmonary trunk.)
The presence of a pulmonary band, in our experience, complicated the procedure by producing scarring and shortening of the pulmonary trunk. Hemostasis of the aorta-pulmonary anastomosis was time-consuming. We now believe that, with the exception of newborns with associated aortic arch interruption, banding should not be performed if a DKS procedure is planned. Our current indications for this operation concentrate on the Taussig-Bing anomaly, in which our experience and that of other groups is favorable. Thirteen of 14 reported patients, ours included, survived [17, 19-23]. Patients with complete transposition and VSD forrn a fairly homogeneous population in which a Jatene procedure may now be safely performed. Nevertheless, select patients might be, in our view, considered for a DKS procedure. The association of subaortic obstruction ma.kes these patients less than ideal candidates for the Jatene procedure [23, 241. The association of aortic coarctation might suggest the possibility of subaortic stenosis, even in the absence of pressure gradients; this is often the case in a double-outlet right ventricle of the Taussig-Bing type [25]. One should also be aware of particular arrangements of the coronary system that would require complex repairs [25, 261 or contraindicate the Jatene operation [27]. This is true for the intramural course of a major coronary vessel [26]. In conclusion, the DKS operation permits an anatomical repair of transposition defects. It is always feasible, independent of great vessel position or coronary arrangement; furthermore, coronary artery manipulation is avoided. Its drawbacks are the need for ventriculotomy and subsequent conduit replacement(s). The use of fresh or cryopreserved homografts during the initial procedure would respond in part to this consideration. Immediate aortic valve occlusion or "limited connection'' with the systemic ventricle should also help reduce the number of reoperations required by this procedure.
Ann Thorac Surg 1991;52:1148-53
We are grateful to Ms Donata Piccioli for the art work and to MS Clarissa Botsman for editing the manuscript.
References 1. Jatene AD, Fontes VF, Paulista PP, et al. Anatomic correction
of transposition of the great vessels. J Thorac Cardiovasc Surg 1976;72:36&70. 2. Yacoub MH. The case for anatomic correction of transposition of the great arteries. J Thorac Cardiovasc Surg 1979;78: L6. 3. Williams WG, Freedom RM, Culham G, et al. Early experience with arterial repair of transposition. Ann Thorac Surg 1981;32:8-15. 4. Stark J. Transposition of the great arteries: which operation? Ann Thorac Surg 1984;38:429-31. 5. Quaegebeur JM, Rohmer JM, Ottenkamp J. The arterial
switch operation. An eight year experience. J Thorac Cardiovasc Surg 1986;92:361-84. 6. Penkoske PA, Westerman GR, Marx GR, et al. Transposition of the great arteries and ventricular septal defect: results with the Senning operation and closure of ventricular septal defect in infancy. Ann Thorac Surg 1983;36:281-8. 7. Marcelletti C, Mair DD, McGoon DC, Wallace RB, Danielson GK. The Rastelli operation for transposition of the great arteries. Early and late results. J Thorac Cardiovasc Surg 1976;72:427-34. 8. Lecompte Y, Neveux JY, Leca F, et al. Reconstruction of the
pulmonary outflow tract without prosthetic conduit. J Thorac Cardiovasc Surg 1982;84:727-33. 9. Damus PS, Thomson NB, McLoughlin TG. Arterial repair without coronary relocation for transposition of the great arteries with ventricular septal defect. Report of a case. J Thorac Cardiovasc Surg 1982;83:316-8. 10. Kaye MP. Anatomic correction of transposition of great arteries. Mayo Clin Proc 1975;50:63&40. 11. Stansel HC Jr. A new operation for d-loop transposition of the great vessels. Ann Thorac Surg 1975;19:565-7. 12. Danielson GK, Tabry IF, Mair DD, Fulton RE. Great vessel switch operation without coronary relocation for transposition of the great arteries. Mayo Clin Proc 1978;53:67582. 13. Lin AE, Laks H, Barber G, Chin AJ, Williams RG. Subaortic obstruction in complex congenital heart disease: management by proximal pulmonary artery to ascending aorta end to side anastomosis. J Am Coll Cardiol 1986;7617-24. 14. Di Donato RM, di Carlo DC, Giannico S, Marcelletti C. Palliation of complex anomalies with subaortic obstruction: new operative approach. J Am Coll Cardiol 1989;13:40&12.
DI CARL0 ET AL DAMUS OPERATION IN INFANTS
1153
15. Ceithaml EL, Puga FJ, Danielson GK, McGoon DC, Ritter
DG. Results of the Damus-Stansel-Kaye procedure for transposition of the great arteries and for double-outlet right ventricle with subpulmonary ventricular septal defect. Ann Thorac Surg 1984;33:43>7. 16. di Carlo DC, de Leva1 MR, Stark J. "Fresh," antibiotic sterilised aortic homografts in extracardiac valved conduits. Long-term results. Thorac Cardiovasc Surg 1984;32:1M. 17. DeLeon SY, Idriss FS, Ilbawi MN, et al. The Damus-KayeStansel procedure: should the aortic valve or subaortic region be closed? J Thorac Cardiovasc Surg 1986;91:747-53. 18. Chin AJ, Barber G, Helton JG, et al. Fate of the pulmonic valve after proximal pulmonary artery-to-ascending aorta anastomosis for aortic outflow tract obstruction. Am J Cardiol 1988;62:4358. 19. Smith EEJ, Pucci JJ, Walesby RK, Oakley CM, Sapsford RN.
A new technique for correction of the Taussig-Bing anomaly. J Thorac Cardiovasc Surg 1982;83:9014. 20. Crupi G, Parenzan L. Arterial repair without coronary relocation for double-outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly). [Letter]. J Thorac Cardiovasc Surg 1983;85:80@1. 21. Glaser J, Castaneda AR. A new variation of the DamusStansel-Kaye procedure for correction of the Taussig-Bing anomaly [Letter]. J Thorac Cardiovasc Surg 1983;86:157. 22. Binet JP, Lacour-Gayet F, Conso JF, Dupuis C, Bruniaux J. Complete repair of the Taussig-Bing type of double-outlet right ventricle using the arterial switch operation without coronary translocation. J Thorac Cardiovasc Surg 1983;85: 272-5. 23. Kanter KR, Anderson RH, Lincoln C, Rigby ML, Shine-
bourne EA. Anatomic correction for complete transposition and double-outlet right ventricle. J Thorac Cardiovasc Surg 1985;90: 690-9. 24. Boyadjiev K, Ho SY, Anderson RH, Lincoln C. The potential
for subpulmonary obstruction in complete transposition after the arterial switch procedure. An anatomic study. Eur J Cardiothorac Surg 1990;4:214-8. 25. Quaegebeur JM. The arterial switch operation. Rationale, results, perspectives [Thesis]. Leiden, 1986. 26. Gittenberger-de Groot AC, Sauer U, Quaegebeur JM. Aortic intramural coronary artery in three hearts with transposition of the great arteries. 1986;91:56&71. 27. Di Donato RM, Wernovsky G, Walsh EP, et al. Results of the arterial switch operation for transposition of the great arteries and ventricular septal defect. Circulation 1989;80:1689-705.
Thirteen patients, 12 of whom younger than 2 years, underwent a Damus-Kaye-Stansel procedure for complete transposition of the great arteries, ventricular septal defect, or double-outlet right ventricle and subpulmonary ventricular septal defect. In 6 patients, associated cardiac anomalies caused systemic flow obstruction. There were six hospital deaths (mortality rate, 42%).In a mean follow-up period of 57 months, 5 of 7 survivors required relief of right ventricular hypertension through conduit replacement or enlargement (4 patients) or conduit valve balloon dilation (1patient). The aortic valve became regurgitant in 2 patients in whom it had been left in potential connection with the right ventricle. One patient has moderate pulmonary valve regurgitation. The main advantage of the Damus-Kaye-Stansel procedure is
that it avoids coronary relocation; also, the spatial rdationship of the great arteries and the coronary anatomy do not affect its feasibility. One drawback is the need for a conduit in infancy. Our present indication for DamusKaye-Stansel procedure is confined to double-outlet right ventricle with subpulmonary ventricular septal defect; 5 of 6 patients survived repair in this series. Possible indications are for patients with associated subaortic obstruction or unusual coronary arrangements. Fresh1 or cryopreserved homografts as extracardiac conduits and primary closure of the subaortic area may reduce the need for reoperation after Damus-Kaye-Stansel proicedure.
T
with VSD, and double-outlet right ventricle with s u b p l monary VSD treated by the Damus-Kaye-Stansel (DIG) procedure. Their results indicated a high mortality rate in patients less than 18 months of age. We report on a smaller sample of infants and children treated with the same procedure to highlight the effect of age on immediate survival.
he advantage of anatomically corrective procedures [l-51 for patients with complete transposition of the great arteries and ventricular septal defect (VSD) is generally accepted. Early and midterm results of intraatrial repair and VSD closure are, in fact, unsatisfactory [4-61. Since its introduction in 1976 [l], the arterial switch operation has been widely accepted as the procedure of choice for anatomical correction. For patients with associated left ventricular outflow tract obstruction, the Rastelli [7] and REV [8] procedures are also important in the surgical repertoire.
For editorial comment, see page 1033. Fewer reports have appeared on the procedure advocated separately by Damus, Kaye, and Stansel [9-121: essentially an arterial switch operation without coronary reimplantation. The pulmonary trunk is anastomosed end-to-side to the ascending aorta in order to constitute an outlet from the left ventricle, and continuity between the right ventricle and the bifurcation of the pulmonary trunk is established by an extracardiac valved conduit. Part of this procedure has been successfully applied as a means to bypass subaortic obstruction in certain types of single ventricle [13, 141. The Mayo Clinic group [15] has published the largest series of patients with simple transposition, transposition Accepted for publication June 19, 1991. Address reprint requests to Dr di Carlo, Ospedale Pediatric0 Bambino Gesu, Piazza S . Onofrio 4, 00165 Roma, Italia.
0 1991 by The Society of Thoracic Surgeons
(Ann Tkorac Surg 1991;52:1148-53)
Material and Methods
Patients In a 7-year period we operated on 13 patients. Seven of them had complete transposition and VSD, and 6 had double-outlet right ventricle with subpulmonary VSD (Taussig-Bing anomaly). Mean age and weight at operation were 8.2 months (range, 12 days to 39 months) and 5.2 kg (range, 3 to 12.5 kg), respectively. In 6 patients, associated anomalies resulted in obstriiction of systemic flow. Aortic arch interruption was present in 2 patients and coarctation in 2 others; 2 patients had subaortic obstruction, in 1 associated with hypoplastic aortic arch and coarctation. Previous palliative procedures are indicated in Table 1.
Surgical Technique Standard hypothermic cardiopulmonary bypass with crystalloid cardioplegia was used in 9 patients; in 4 others, deep hypothermia and circulatory arrest were employed. The aorta was dissected from the pulmonary trunk and cannulated as far distally as possible. Each caval vein was cannulated separately through the atrium. A single atrial 0003-4975/91/$350
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Table 1. Clinical Characteristics and Surgical Results ~~
Age at
Mode of Aortic Valve
Patient Operation Weight Associated No (mo) Diagnosis (kg) Malformations
Previous Operations
VSD VSD
AAI
AAI repair
...
...
SubAo St
...
...
PAB ... Subcl flap
Separate patch
. . .
VSD patch VSD patch VSD patch ...
Alive
1 2 3 4 5 6 7 8 9 10 11 12 13
0.5 3 5 6 7 7 8 2 3 7 8 11 42
TGA, TGA, TGA, TGA, TGA, TGA, TGA,
VSD VSD VSD
VSD VSD TB TB TB TB TB TB
3.5 4.3 4.5 4.2 5.6 4.5 5.4
3 4.3 6.2 4.3 5.8 12.5
LSVC CoAo
... CoAo, SubAo St ... AAI
... ...
CoAo
...
... AAI repair, PAB
Closure
Outcome
VSD patch VSD patch VSD patch Separate patch
Separate patch
Leaflet suture A sept, PAB Leaflet suture Subcl flap, PAB VSD patch . . .
Follow-up
Semilunar
Re-
Valve
(mo)
operation
Died
... 30 60
... No Yes
...
Alive
... ...
Alive Died
. . .
Died Died
...
...
...
Alive
73 54 86 87 9
Yes Yes BD; yes BD No
Alive Alive
...
...
...
Died Died
Alive
Fate
...
A1 A1 PI
...
BD = balloon dilation of conduit valve; CoAo = AAI = aortic arch interruption; A1 = aortic insufficiency; A sept = atrial septectomy; PAB = pulmonary artery banding; PI = pulmonary valve insufficiency; SubAo St coarctation of the aorta; LSVC = left superior vena cava; TGA = transposition of TB = Taussig-Bing type of double-outlet right ventricle; = subaortic stenosis; Subcl flap = subclavian flap aortoplasty; VSD = ventricular septal defect. the great arteries;
cannula was placed if circulatory arrest was planned. Bypass time averaged 155 minutes (range, 127 to 217 minutes) and aortic cross-clamp time, 77 minutes (range, 49 to 143 minutes). During the cooling period, the pulmonary trunk was transected close to its bifurcation or at the site of previous banding; the proximal stump was tailored obliquely to face the ascending aorta. We introduced two modifications of the original technique: first, the aortic pathway was routinely occluded (except in 1 patient whose aortic valve was left open and connected with the right ventricle). Second, after the aorta-pulmonary artery anastomosis was completed (Fig lA), the pulmonary confluence was closed either directly or with a pericardial patch, the arteriotomy was extended to the main right pulmonary branch (Fig lB), and the valved conduit was anastomosed to it (Fig 1C). This maneuver allows the conduit to be positioned to the right of the ascending aorta and the aorta-pulmonary anastomosis to lie posterior and to the left of the ascending aortic segment, so that it is more accessible for control of hemostasis. Various techniques were employed for the first described modification (Table 1). In the last 6 patients, the VSD patch was extended to the subaortic region, thereby placing the semilunar valve in communication with the left ventricle in a similar fashion to the Rastelli procedure (Fig 2). A wide-open subaortic tunnel is not necessary, as the left ventricle has an unobstructed outlet through the pulmonary trunk. Heterograft valved conduits, size 12 or 14, were used to complete the operation. In 1 patient, a hypoplastic aortic arch associated with coarctation required pericardial patch reconstruction extended beyond the coarctation site.
Results Six patients did not survive the immediate postoperative period (early mortality rate, 42%). Of 6 patients with double-outlet right ventricle, 5 survived. One 12-day-old baby in desperate condition was operated on 24 hours after repair of concomitant interruption of the aortic arch; the pulmonary trunk had not been banded in this patient. One patient died of respiratory insufficiency after massive blood transfusion in the operating room. The pulmonary trunk was paper-thin after pulmonary artery banding and profuse bleeding occurred from the aorta-pulmonary anastomosis. Acute myocardial failure followed an untimely attempt at extubation of another, apparently stable, child. In another patient, coronary air embolism occurred before the institution of cardiopulmonary bypass; this was the only patient in the sample whose left ventricular systolic pressure was less than systemic (ratio, 0.68) despite the presence of a ventricular septal defect. Two deaths followed Staphylococcus aureus septicemia. In 1 patient endocarditis occurred after an otherwise uneventful postoperative recovery. Mediastinitis developed in the other patient after urgent DKS procedure; his condition deteriorated after cardiac catheterization complicated by iliac vein thrombosis. In retrospect, technical mistakes or errors of judgment resulted in the death of three patients. The role of an inadequately prepared left ventricle in causing a fatal outcome in 1 patient is debatable. Follow-up to date averages 57 months (range, 9 to 87 months). No patient died in this period. Of the 7 survivors, 4 required further surgical treatment. The right ventricle-pulmonary artery conduit became stenotic and was replaced with an aortic homograft (2 patients) or
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Fig I. Modified Damus-Kaye-Stansel procedure. (See text for explanation.)
enlarged with a pericardial roof (1 patient) at 6, 7, and 3 years after the DKS procedure. One patient required three reoperations because of conduit stenosis and native aortic valve regurgitation. An aortic homograft was implanted at the second procedure but had to be replaced after 8 months owing to angulation under the sternum. Conduit valve stenosis was identified as the main cause of right ventricle-pulmonary artery gradient and was treated by balloon dilation in 2 patients: in both the gradient was partially relieved (from 75 to 41 mm Hg and from 45 to 25 mm Hg). In 1 of these patients the conduit was replaced 2% years later, whereas the other continues to do well, but has moderate right ventricular hypertension and dysfunction. Finally, 2 patients, both operated on in the last 2 years, are well with no evidence of right ventricular outflow tract obstruction. On the whole, 5 of 7 early survivors needed some type of right ventricular decompression.
Semilunar valve regurgitation was found in 3 patients: in 1 there was moderate pulmonary valve insufficiency, and in 2 there was aortic insufficiency. In the latter 2 patients the aortic valve was left anatomically connected to the right ventricle, with sutured leaflets in 1. This patient required reoperation for repeat suture of the aortic valve leaflets. One other patient has pulmonary insufficiency; despite this problem and associated right ventricular outflow tract obstruction he is currently asymptomatic. Pulmonary artery banding had previously been performed in this patient. The angiographic and crosssectional echocardiographic appearance of the left and right outflow tracts after the DKS procedure are shown in Figures 3 and 4. Figure 5 shows the left ventriculograni of a patient in whom a double outlet from the left ventricle was surgically created. With this technique a wash-out effect of the sinuses of Valsalva and coronary ostia is obtained through the natural pathway.
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observed after arterial switch operation [>5] and palliative pulmonary-ascending aorta anastomosis [ 17, 181. This complication occurred in 1 of the patients in our sample (14% of early survivors). Its cause is unclear; if the
Fig 2. "Limited connection" tt,chnique: the rrght ventricle-yulmonary artery conduit is omitted for clarity of illusfration. (See text for explanation.) (Ao = aorta; LV = kft ventricle; PA = pulnionary artery; RV = right ventricle.)
A
Comment In this small sample the hospital mortality was high, reflecting the complexity of the repair during the learning curve. These statistics are similar to the first results of the arterial switch operation. The early mortality in infants younger than 18 months was 50%; the Mayo Clinic group obtained similar results ( p = 0.77, not significant). Low age and weight in our experience did not preclude surgical success, but the use of a prosthetic conduit for right ventricle-pulmonary artery connection substantially contributed to morbidity and, in some cases, mortality. From this point of view the procedure must be considered no more than palliative. The negative impact of residual defects such as pulmonary outflow tract stenosis, pulmonary insufficiency, or a combination of the two on late survival must be kept in mind. In this sample, all patients with a longer follow-up interval than 36 months had to undergo further operations. Some of them still have mild or moderate right ventricular outflow tract pressure gradients and right ventricular dysfunction. In most reoperations fresh or cryopreserved homografts replaced the original conduits; in an "adult" size, these may offer a satisfactory performance for an indefinite number of years [16]. Another cause of reoperation was aortic incompetence, which occurred when this valve was left communicating with the right ventricle. Because of the peculiar physiology of the DKS procedure, aortic insufficiency results in varying degrees Of left-to-right shunt and poorly tolerated [ 171. The native Pulmonary valve, as in the Jatene operation (arterial switch), will always work under systemic pressure load. Late development of regurgitation has been
B
Fig 3 . Angiographic (A)and cross-sectional echocardio~ruphic( B ) appeararice of the right ventricular outlet after the Damus-KayeStaiisel procedure; the black asterisk marks the conduit valve, and the white asterisks mark the tnairi pulmonary branches. (C = con duit; RA = righf atrium; RV = right rientricle.)
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valve were intrinsically unable to function as the aortic valve, the incidence of regurgitation should be similar for the two arterial switch procedures.
Fig 5. Leff ventricular contrast injection; in this putient the subao.rtic region was excluded from the right ventricle by extension of the venfricular septal defect patch. A double-outlet left ventricle is demonstrated (arrows).
A
B
FIg 4. Angiographic (A) and cross-sectional echocardiographic f B ) appearance of the leff ventricular outlet after Damus-Kay-Stansel procedure. (A = ascending aorta; LV = left ventricle; P = pulmonary trunk.)
The presence of a pulmonary band, in our experience, complicated the procedure by producing scarring and shortening of the pulmonary trunk. Hemostasis of the aorta-pulmonary anastomosis was time-consuming. We now believe that, with the exception of newborns with associated aortic arch interruption, banding should not be performed if a DKS procedure is planned. Our current indications for this operation concentrate on the Taussig-Bing anomaly, in which our experience and that of other groups is favorable. Thirteen of 14 reported patients, ours included, survived [17, 19-23]. Patients with complete transposition and VSD forrn a fairly homogeneous population in which a Jatene procedure may now be safely performed. Nevertheless, select patients might be, in our view, considered for a DKS procedure. The association of subaortic obstruction ma.kes these patients less than ideal candidates for the Jatene procedure [23, 241. The association of aortic coarctation might suggest the possibility of subaortic stenosis, even in the absence of pressure gradients; this is often the case in a double-outlet right ventricle of the Taussig-Bing type [25]. One should also be aware of particular arrangements of the coronary system that would require complex repairs [25, 261 or contraindicate the Jatene operation [27]. This is true for the intramural course of a major coronary vessel [26]. In conclusion, the DKS operation permits an anatomical repair of transposition defects. It is always feasible, independent of great vessel position or coronary arrangement; furthermore, coronary artery manipulation is avoided. Its drawbacks are the need for ventriculotomy and subsequent conduit replacement(s). The use of fresh or cryopreserved homografts during the initial procedure would respond in part to this consideration. Immediate aortic valve occlusion or "limited connection'' with the systemic ventricle should also help reduce the number of reoperations required by this procedure.
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We are grateful to Ms Donata Piccioli for the art work and to MS Clarissa Botsman for editing the manuscript.
References 1. Jatene AD, Fontes VF, Paulista PP, et al. Anatomic correction
of transposition of the great vessels. J Thorac Cardiovasc Surg 1976;72:36&70. 2. Yacoub MH. The case for anatomic correction of transposition of the great arteries. J Thorac Cardiovasc Surg 1979;78: L6. 3. Williams WG, Freedom RM, Culham G, et al. Early experience with arterial repair of transposition. Ann Thorac Surg 1981;32:8-15. 4. Stark J. Transposition of the great arteries: which operation? Ann Thorac Surg 1984;38:429-31. 5. Quaegebeur JM, Rohmer JM, Ottenkamp J. The arterial
switch operation. An eight year experience. J Thorac Cardiovasc Surg 1986;92:361-84. 6. Penkoske PA, Westerman GR, Marx GR, et al. Transposition of the great arteries and ventricular septal defect: results with the Senning operation and closure of ventricular septal defect in infancy. Ann Thorac Surg 1983;36:281-8. 7. Marcelletti C, Mair DD, McGoon DC, Wallace RB, Danielson GK. The Rastelli operation for transposition of the great arteries. Early and late results. J Thorac Cardiovasc Surg 1976;72:427-34. 8. Lecompte Y, Neveux JY, Leca F, et al. Reconstruction of the
pulmonary outflow tract without prosthetic conduit. J Thorac Cardiovasc Surg 1982;84:727-33. 9. Damus PS, Thomson NB, McLoughlin TG. Arterial repair without coronary relocation for transposition of the great arteries with ventricular septal defect. Report of a case. J Thorac Cardiovasc Surg 1982;83:316-8. 10. Kaye MP. Anatomic correction of transposition of great arteries. Mayo Clin Proc 1975;50:63&40. 11. Stansel HC Jr. A new operation for d-loop transposition of the great vessels. Ann Thorac Surg 1975;19:565-7. 12. Danielson GK, Tabry IF, Mair DD, Fulton RE. Great vessel switch operation without coronary relocation for transposition of the great arteries. Mayo Clin Proc 1978;53:67582. 13. Lin AE, Laks H, Barber G, Chin AJ, Williams RG. Subaortic obstruction in complex congenital heart disease: management by proximal pulmonary artery to ascending aorta end to side anastomosis. J Am Coll Cardiol 1986;7617-24. 14. Di Donato RM, di Carlo DC, Giannico S, Marcelletti C. Palliation of complex anomalies with subaortic obstruction: new operative approach. J Am Coll Cardiol 1989;13:40&12.
DI CARL0 ET AL DAMUS OPERATION IN INFANTS
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15. Ceithaml EL, Puga FJ, Danielson GK, McGoon DC, Ritter
DG. Results of the Damus-Stansel-Kaye procedure for transposition of the great arteries and for double-outlet right ventricle with subpulmonary ventricular septal defect. Ann Thorac Surg 1984;33:43>7. 16. di Carlo DC, de Leva1 MR, Stark J. "Fresh," antibiotic sterilised aortic homografts in extracardiac valved conduits. Long-term results. Thorac Cardiovasc Surg 1984;32:1M. 17. DeLeon SY, Idriss FS, Ilbawi MN, et al. The Damus-KayeStansel procedure: should the aortic valve or subaortic region be closed? J Thorac Cardiovasc Surg 1986;91:747-53. 18. Chin AJ, Barber G, Helton JG, et al. Fate of the pulmonic valve after proximal pulmonary artery-to-ascending aorta anastomosis for aortic outflow tract obstruction. Am J Cardiol 1988;62:4358. 19. Smith EEJ, Pucci JJ, Walesby RK, Oakley CM, Sapsford RN.
A new technique for correction of the Taussig-Bing anomaly. J Thorac Cardiovasc Surg 1982;83:9014. 20. Crupi G, Parenzan L. Arterial repair without coronary relocation for double-outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly). [Letter]. J Thorac Cardiovasc Surg 1983;85:80@1. 21. Glaser J, Castaneda AR. A new variation of the DamusStansel-Kaye procedure for correction of the Taussig-Bing anomaly [Letter]. J Thorac Cardiovasc Surg 1983;86:157. 22. Binet JP, Lacour-Gayet F, Conso JF, Dupuis C, Bruniaux J. Complete repair of the Taussig-Bing type of double-outlet right ventricle using the arterial switch operation without coronary translocation. J Thorac Cardiovasc Surg 1983;85: 272-5. 23. Kanter KR, Anderson RH, Lincoln C, Rigby ML, Shine-
bourne EA. Anatomic correction for complete transposition and double-outlet right ventricle. J Thorac Cardiovasc Surg 1985;90: 690-9. 24. Boyadjiev K, Ho SY, Anderson RH, Lincoln C. The potential
for subpulmonary obstruction in complete transposition after the arterial switch procedure. An anatomic study. Eur J Cardiothorac Surg 1990;4:214-8. 25. Quaegebeur JM. The arterial switch operation. Rationale, results, perspectives [Thesis]. Leiden, 1986. 26. Gittenberger-de Groot AC, Sauer U, Quaegebeur JM. Aortic intramural coronary artery in three hearts with transposition of the great arteries. 1986;91:56&71. 27. Di Donato RM, Wernovsky G, Walsh EP, et al. Results of the arterial switch operation for transposition of the great arteries and ventricular septal defect. Circulation 1989;80:1689-705.
