Damus-Kaye-Stansel Procedure: Midterm Follow-up and Technical Considerations

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1 Damus-Kaye-Stansel Procedure: Midterm Follow-up and Technical Considerations Thomas L. Carter, MD, Richard D. Mainwaring, MD, and John J. Lamberti, MD Division of Cardiac Surgery, Children's Hospital and Health Center, San Diego, California The Damus-Kaye-Stansel operation is useful in the management of complex congenital heart defects. We reviewed our experience with 23 patients who underwent a Damus-Kaye-Stansel procedure. The anastomotic technique was individualized depending on the anatomy. The aortic and pulmonary artery incisions were carried into the sinuses of Valsalva in 9 patients, the aorta was transected in 11 patients, and a patch was used to augment the anastomosis in 13 patients. Concurrent procedures included a operation (n = 9, mortality = 0), right ventricle-pulmonary artery conduit (n = 5, mortal- ity = 0), bidirectional Glenn procedure (n = 6, mortality = 1), and central aortopulmonary (n = 3, mortality = 2; emergency = 1). Survival is 87% with a median follow-up of 7 years (range, 2 months to 9.2 years). Four patients underwent late revision of the Damns-Kaye Stansel connection. All survivors are asymptomatic. We conclude that the Damus-Kaye-Stansel connection provides excellent midterm results when the proximal anastomosis is adapted to the anatomy of the patient. (Ann Thorac Surg ) A n astom osis of the pulmonary artery to the aorta can provide an alternative pathway for systemic blood flow in patients with various forms of complex congenital heart disease. Damus [1], Kaye [2], and Stansel [3] originally described an end-to-side anastomosis (DKS operation) to achieve this goal in patients with dextrotransposition of the great arteries (n-tga). We [4] have previously reported several technical modifications of the DKS connection. This report summarizes the midterm follow-up of our experience with the DKS procedure since Material and Methods Patient Population We reviewed our recent experience with 23 consecutive patients who underwent a DKS anastomosis (Table 1). Thirteen patients had single ventricle with small-outlet chamber anatomy. Six patients had TGA with subaortic stenosis. Two patients had double-outlet right ventricle with a malposed aorta and subaortic stenosis. Two patients had variants of the hypoplastic left heart syndrome. Mean age at operation was 2.5 years (range, 12 days to 9.5 years). Prior surgical procedures included pulmonary artery banding (n = 22), repair of coarctation of the aorta (n = 11), ligation of patent ductus arteriosus (n = 10), and apical-aortic conduit (n = 1). Procedures performed concurrently with the DKS anastomosis included a operation (n = 9), procedure (right ventricle- Presented at the Poster Session of the Thirtieth Annual Meeting of The Society of Thoracic Surgeons, New Orleans, LA, Jan 31-Feb 2, Address reprint requests to Dr Lamberti, 3030 Children's Way, Suite 310, San Diego, CA by The Society of Thoracic Surgeons pulmonary artery conduit) (n = 5), bidirectional Glenn operation (n = 6), and central aortopulmonary (n = 5). Operative Technique Operations were generally performed using hypothermic cardiopulmonary bypass. The aorta was usually cannulated on the inner curvature of the arch. This allowed placement of the cross-clamp immediately proximal to the innominate artery, thus providing continuous perfusion of the arch vessels during the operation. Deep hypothermic circulatory arrest was used in small infants to provide access to the proximal aorta. Bicaval cannulation was typically performed. Blood cardioplegia and topical myocardial cooling were used in all instances. Myocardial temperature was continuously monitored. Cardioplegia was repeated at 20-minute intervals. Performance of the DKS anastomosis involved several techniques depending on the anatomy of each patient (Fig 1). The main pulmonary artery was divided either at its bifurcation or at the site of a previously placed band, when present. All scar tissue was resected. In patients with obscured coronary anatomy secondary to dense adhesions, the aorta was transected at a level above the sinuses of Valsalva. Construction of the aortopulmonary anastomosis was performed in a traditional end-to-side fashion in patients with sufficient length of pulmonary artery (n = 5). In the remaining patients (n = 18), a side-to-side anastomosis was created. If the pulmonary artery was particularly short, this anastomosis was performed at the level of the sinuses of Valsalva. A gusset was used to complete the anastomosis in patients with an intact aorta. If the aorta had previously been divided, the /94/$7.00

2 1604 CARTER ET AL Ann Thorac Surg Table 1. Summary of Data on Patients Undergoing Damus-Kaye-Stansel Procedure Patient No. Diagnosis Age at Operation (mo) Concurrent Operation Technical Modifications Outcome" DOLV, TGA, VSD, SAS TGA, VSD, overriding AV valve Dextrocardia, TA,TGA,VSD Single ventricle, TGA, VSD, SAS DILV, TGA, VSD,SAS DILV, TGA, VSD HLHS variant DILV, TGA, VSD HLHS variant DORV, TGA, VSD,SAS DORV,MGA, VSD,SAS DILV, TGA, VSD CommonAV canal, VSD DORV, absent ventricular septum Central aortopulmonary Central aortopulmonary Central aortopulmonary ; central aortopulmonary ; central aortopulmonary Divide aorta Divide aorta, patch Patch Divide aorta, patch Sinus of Valsalva incision, patch Patch Patch Patch Patch Patch 77 mo: replace conduit, patch of DKS anastomosis 86 mo: replace conduit 70 mo: replace conduit 9 mo: oversew tricuspid valve, pacemaker Operative death 29 mo: oversew native aortic valve, replace conduit; 78 mo: pacemaker; 80 mo: implantable cardioverter defibrillator 69 mo: pacemaker 4 mo: patch aortoplasty; 18 mo: bidirectional Glenn; 46 mo: Operative death Operative death 18 mo: oversew native aortic valve, patch of DKS anastomosis, replace conduit, resect SAS; 46 mo: replace conduit, neo-aortic valve annuloplasty 20 mo: 20 mo: : 21 mo: pacemaker 1 mo: pacemaker a When months are given, they are time since DKS procedure. AV = atrioventricular; DILV = double-inlet left ventricle; DKS = Damus-Kaye-Stansel: DOLV ~ double-outlet left ventricle; DORV = double-outlet right ventricle; HLHS = hypoplastic left heart syndrome; MGA = malposition of great arteries; SAS = subaortic stenosis; TA = tricuspid atresia; TGA = transposition of great arteries; VSD = ventricular septal defect.

3 Ann Thorac Surg CARTER ET AL 1605 l Fig 1. Modification of Damus-Kaye-Stansel procedure. (A) In patients with extensive mediastinal adhesions, the aorta and the pulmonary artery are transected at the same level, ie, the cephalad end of the aortic sinuses of Valsalva. (B) Incisions are made in the contiguous pulmonary and aortic walls, with special attention paid to the coronary ostia and the valve commissures. These walls are sewn together. (C) Use of a gusset in fashioning an end-to-side anastomosis allows unobstructed flow into the ascending aorta without distortion of the pulmonary annulus (which causes regurgitation). (D) The ascending aorta can be sutured to the new (bivalved) single artery (see B). (E) Original Damus concept for comparison: end-to-side anastomosis of the pulmonary artery to the aorta. (Reprinted from Waldman ]D, Lamberti JJ, George L, et al. Experience with the Damus procedure. Circulation 1988;78fSuppl 31:32 9, by permission of the American Heart Association.) distal aorta was sutured to the new "bivalved" artery created by the side-to-side anastomosis. Results Twenty (87%) of the 23 patients survived the operation. The actuarial survival is shown in Figure 2. There have been no late deaths. All survivors are asymptomatic (New York Heart Association class I or II) with a median follow-up of 7 years (range, 2 months to 9.2 years). There were three perioperative deaths (13%; 70% confidence intervals, 3% to 34%). Patient 8 was seen in cardiogenic shock secondary to single-ventricle anatomy with obstruction of the bulboventricular foramen. The patient was taken emergently to the operating room where defibrillation was necessary before the start of the procedure. After a DKS anastomosis with a concurrent aortopulmonary was performed, the patient could not be weaned from cardiopulmonary bypass. Patient 13 was seen at 5 weeks of age with double-inlet left ventricle, TGA, and ventricular septal defect. Pulmonary artery banding was performed. At 2 years of age, he underwent a DKS procedure with a concurrent aortopulmonary. After weaning from cardiopulmonary bypass, the patient had persistent low cardiac output, which could not be reversed, and he subsequently died. Patient 14 was seen with a variant of hypoplastic left heart syndrome. Initial treatment at another institution included Alive 60 (%) (17) (15) (13) (11) (11) (11) (9) (8) (1) Years Post-8urgery Fig 2. Actuarial survival of patients undergoing Damus-Kaue Stansel procedure. There were three operative deaths, and there have been no late deaths.

4 1606 CARTER ET AL Ann Thorac Surg pulmonary artery banding, repair of coarctation of the aorta, and ligation of a patent ductus arteriosus. When he was 14 months of age, a DKS connection with a bidirectional Glenn procedure was performed. The patient had myocardial dysfunction because of an inability to oxygenate and could not be weaned from cardiopulmonary bypass. Four patients have required late revision of the DKS connection. Patient 2 initially underwent a DKS anastomosis with a concurrent porcine-valved right ventriclepulmonary artery conduit for TGA, ventricular septal defect, and subaortic stenosis. Six years 5 months later, gradients of 60 mm Hg across the conduit and 10 mm Hg across the DKS connection were detected. Conduit replacement and patch of the DKS anastomosis were performed. The patient has subsequently done well without cardiac medications. Patient 9 underwent a DKS procedure at 2 years 7 months of age with a concurrent porcine valve right ventricle-pulmonary artery conduit. Two years 5 months later, the patient was seen with a febrile illness and presumed (culture-negative) bacterial endocarditis of the conduit valve. After antibiotic therapy, cardiac catheterization demonstrated valvar obstruction of the conduit and insufficiency of the native aortic valve. Intraoperative inspection of the aortic valve identified mild fibrosis without evidence of endocarditis. Changes in the conduit valve were consistent with endocarditis. The conduit was replaced and the aortic valve, oversewn. The patient is currently doing well without any cardiac medications. Patient 12 had a hypoplastic left heart syndrome variant. When he was 12 days of age, a DKS connection, an atrial septectomy, and a modified Blalock-Taussig were performed. Four months later, arch hypoplasia was detected, and bovine pericardial patch of the aortic arch and DKS anastomosis was performed. The patient underwent subsequent bidirectional Glenn (18 months) and fenestrated (46 months) procedures. He is currently doing well on a regimen of digoxin, spironolactone, and hydrochlorothiazide hydrochloride. Patient 15 had double-outlet right ventricle, TGA, ventricular septal defect, and subaortic stenosis. When he was 2 years 4 months of age, a DKS anastomosis was performed with a concurrent right ventricle-pulmonary artery conduit. Eighteen months later, the patient was noted to have subaortic stenosis (44 mm Hg gradient), obstruction of the conduit, and insufficiency of the native aortic valve. He underwent conduit replacement, resection of subaortic muscle, closure of the native aortic valve, and patch of the DKS connection. At 6 years 2 months of age, he was seen with repeat obstruction of the conduit, recurrent subaortic stenosis, and moderate insufficiency of the neo-aortic valve. Treatment consisted of replacement of the right ventriclepulmonary artery conduit, resection of discrete subaortic stenosis, and subcommissural suture annuloplasty of the neo-aortic valve. The patient is currently well on a regimen of digoxin, spironolactone, hydrochlorothiazide, and Vasotec (enalapril maleate). Several other patients required subsequent surgical interventions unrelated to the DKS anastomosis. Two patients outgrew the right ventricle-pulmonary artery conduit and underwent replacement. Completion procedures were performed in 2 patients. One patient, who was presumed to have tricuspid atresia, underwent a DKS anastomosis plus a procedure. Nine months later, during evaluation of new-onset sick sinus syndrome, a right-to-left was detected across a stenotic but patent tricuspid valve. Surgical closure was performed and a permanent pacemaker implanted. Four additional patients required permanent pacemaker implantation. Medical treatment of ventricular tachycardia resulted in severe bradycardia in 1 patient 78 months after a DKS connection with a procedure. An implantable cardioverter defibrillator and permanent pacemaker were implanted. Atrioventricular dissociation occurring in a patient prior to a DKS anastomosis with a procedure required permanent pacing 69 months later. Twenty months after a DKS anastomosis combined with a bidirectional Glenn procedure, another patient underwent a completion operation. Sick sinus syndrome developed 1 month later and necessitated placement of a permanent pacemaker. Complete heart block was noted in a patient soon after a DKS anastomosis and a bidirectional Glenn procedure that caused a complicated postoperative course requiring extracorporeal membrane oxygenation. A permanent pacemaker was implanted. Comment Damus [l] first proposed using an end-to-side pulmonary artery-aorta anastomosis in the treatment of D-TGA. Descriptions of the same connection were later published by Kaye [2] and Stansel [3]. Though development of the arterial switch procedure has reduced the number of candidates for the DKS procedure, the technique has been applied to patients with several forms of complex congenital heart disease in which there is obstruction to systemic blood flow. Candidates include patients with univentricular heart and a restrictive bulboventricular foramen and biventricular heart with TGA and subaortic stenosis. The DKS procedure is especially suitable for patients in whom the connection is planned because it avoids surgical myocardial trauma and relieves outflow tract obstruction, thus obviating further cardiac hypertrophy and diastolic dysfunction [5]. Intracardiac repair in patients born with TGA and subaortic stenosis is possible, although the risk is increased. When complex baffles are used for the intraventricular repair, anatomic repair with concomitant resection of obstructing muscle or tissue may not be as safe as the DKS anastomosis plus the procedure. Other options for the surgical relief of outflow obstruction have been espoused. Enlargement of the bulboventricular foramen entails the risk of complete heart block, difficulty in determining the extent of resection, myocardial dysfunction secondary to ventriculotomy, and the potential for recurrent obstruction. An apical-aortic conduit provides unobstructed outflow from the systemic

5 Ann Thorae Surg CARTER ET AL 1607 ventricle but has detrimental effects on ventricular function and may require subsequent replacement. In 1991, Karl and colleagues [6] suggested the arterial switch for the management of univentricular heart with subaortic stenosis. Early summaries of the DKS procedure reported mortality rates in the range of 50% [7, 8]. In our series, there were three operative deaths (13%) and no late deaths. The results are comparable to those reported by Huddleston and associates [9] (2/18,11%) and Gates et al [10] (3/29,10%). We believe current surgical techniques allow performance of a DKS procedure with acceptable mortality rates. The technique used in performing a pulmonary arteryaorta connection should be determined on an individualized basis with the goals of avoiding outflow tract gradients and distortion of the semilunar valves. We [4] have previously described several technical modifications that facilitate construction of the anastomosis (see Fig 1). Division of the ascending aorta in patients with extensive mediastinal adhesions helps to identify the coronary anatomy and improves access to the branch pulmonary arteries should these need to be repaired. Construction of the anastomosis is adapted to the length of the pulmonary artery. With sufficient length, an endto-side aortopulmonary connection can be fashioned. A side-to-side anastomosis, which can be performed as proximal as the sinuses of Valsalva, is applied in patients with shorter pulmonary arteries. Completion of the sideto-side anastomosis is accomplished in patients with an intact aorta by using a gusset. In patients in whom the aorta has been divided, the distal aorta is sutured to the new "bivalved" artery with tailoring as needed. The effect of the DKS anastomosis on the long-term function of the semilunar valves is unknown. Stansel [3] originally proposed patching the subaortic valve region in patients with elevated right ventricular pressures to prevent opening of the aortic valve during the cardiac cycle and development of aortic regurgitation. Conversely, Damus [11], Danielson [12], and their co-workers suggested leaving the aortic valve open to allow right-toleft ing to prevent acute right heart failure in cases of elevated outflow resistances. Aortic valve insufficiency developed in 2 patients in our series. In both, a DKS anastomosis was performed with a concurrent right ventricle-pulmonary artery conduit. This represents a 40% incidence (2/5) of aortic insufficiency in patients undergoing a DKS procedure with a repair in contrast to 0% (0/18) in patients with palliation of univentricular anatomy. Gates et al [10] reported using a DKS connection in 29 patients, 4 of whom had a concurrent right ventriclepulmonary artery conduit. Two (50%) of these 4 patients had development of major aortic insufficiency. In the other 2, the aortic valve or infundibular region was closed at the time of the original procedure. Major aortic insufficiency developed in only 1 (4%) of the 25 patients undergoing a DKS anastomosis as part of a univentricular repair. It developed immediately postoperatively and was presumed to result from preexisting aortic valve disease. DeLeon et al [13, 14] also noted development of substantial aortic insufficiency in patients undergoing combined DKS and procedures; it occurred in 2 (67%) of 3 patients in whom the aortic valve was left open and in none (0/4) in whom the aortic valve was closed. The development of aortic insufficiency occurs in a substantial number of patients having a DKS anastomosis with a concurrent right ventricle-pulmonary artery conduit. Closure of the aortic valve at the time of the initial procedure should be performed in this subset of patients. Late patch enlargement of the DKS connection was performed in 3 of our patients because of a gradient across the anastomosis. One patient had only a 10 mm Hg gradient that was treated with patch enlargement at the time of replacement of a conduit that the patient had outgrown. In another patient, who had a DKS anastomosis as palliation for a hypoplastic left heart syndrome variant, aortic arch hypoplasia that included the anastomosis subsequently developed. Patch aortopiasty was performed. The third patient had development of a major gradient across the aortic outflow tract; this gradient was mainly attributable to a subaortic muscle band. Reoperation for resection of the muscle band included patch enlargement to prevent anastomotic narrowing. These patients represent 30% (3/10) of our patients who did not have a gusset placed at the time of construction of the DKS anastomosis. Despite gradient development, we suspect that an anastomotic patch in these 3 patients at the time of the initial operation would have had little impact on the subsequent clinical course. The first patient required replacement of the right ventricle-pulmonary artery conduit. A 10 mm Hg outflow gradient, of itself, would have little clinical impact, though the natural history of the gradient is unpredictable. Aortic hypoplasia in patients with a hypoplastic left heart syndrome variant requires treatment of the entire aortic arch, something not accomplished with an anastomotic patch. Initial patch aortoplasty should be considered in this subset of patients. Finally, subaortic stenosis secondary to a muscle band would probably have occurred regardless of anastomotic patch incorporation. Nevertheless, these patients did show development of some degree of outflow tract obstruction. In contrast, anastomotic gradients did not develop in any of our patients with patch placement at the time of the initial procedure (0/13). Likewise, neither Gates et al [10] nor DeLeon et al [14] reported gradient development in their respective series. Both groups used a gusset in performing the DKS anastomosis. Incorporation of a patch should be strongly considered when a DKS anastomosis is being constructed. The indications for the DKS anastomosis in patients with anatomy suitable for biventricular repair are quite limited in We have not used the DKS anastomosis plus the technique since For patients with a univentricular heart and the potential for obstruction to systemic blood flow, we now recommend the DKS anastomosis be performed whenever the pulmonary artery is divided whether or not actual obstruction to systemic blood flow is present at rest. The natural history of the

6 1608 CARTER ET AL Ann Thorac Surg bulboventricular foramen or the subaortic region in the univentricular heart with TGA suggests that a prophylactic DKS anastomosis may be appropriate in patients without pulmonary stenosis undergoing the bidirectional Glenn or the procedure. In summary, the DKS anastomosis is an attractive option for providing unobstructed blood flow from the systemic ventricle in patients with complex congenital heart disease. We believe that technical modifications can be selectively applied to achieve stable semilunar valve function and excellent midterm results. References 1. Damus PS. Correspondence. Ann Thorac Surg 1975;20: Kaye MP. Anatomic correction of transposition of the great arteries. Mayo Clin Proc 1975;50: Stansel HC Jr. A new operation for d-loop transposition of the great vessels. Ann Thorac Surg 1975;19: Waldman JD, Lamberti JJ, George L, et al. Experience with Damus procedure. Circulation 1988;78(Suppl 3): Lamberti JJ, Mainwaring RD, Waldman JD, et al. The Damus- procedure. Ann Thorac Surg 1991;52: Karl TR, Watterson KG, Sano 5, Mee RBB. Operations for subaortic stenosis in univentricular hearts. Ann Thorac Surg 1991;52: 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;38: Di Carlo DC, Di Donato RM, Carotti A, Ballerini L, Marcelletti C. Evaluation of the Darnus-Kaye-Stansel operation in infancy. Ann Thorac Surg 1991;52: Huddleston CB, Canter CE, Spray TL. Damus-Kaye-Stansel with cavopulmonary connection for single ventricle and subaortic obstruction. Ann Thorac Surg 1993;55: Gates RN, Laks H, Elami A, et al. Damus-Stansel-Kaye procedure: current indications and results. Ann Thorac Surg 1993;56: Damus PS, Thomson NB [r, McLoughlin TG. Arterial repair without coronary relocation of complete transposition of the great vessels with ventricular septal defect. J Thorac Cardiovase Surg 1982;83: Danielson GK, Tabry IF, Mair DD, Fulton RE. Great-vessel switch operation without coronary relocation for transposition of great arteries. Mayo Clin Proc 1978;53: DeLeon SY, Idriss FS, Ilbawi MN, et al. The Damus-Stansel Kaye procedure. J Thorac Cardiovasc Surg 1986;91: DeLeon SY, Ilbawi MN, Tubeszewski K, Wilson WRJr, Idriss FS. The Damus-Stansel-Kaye procedure: anatomical determinants and modifications. Ann Thorac Surg 1991;52: The Society of Thoracic Surgeons: Thirty-first Annual Meeting Mark your calendars for the Thirty-first Annual Meeting of The Society of Thoracic Surgeons, which will be held at the Palm Springs Convention Center in Palm Springs, California, January 30-February 1, Members may register for the Scientific Sessions at no charge. There will be a $250 registration fee for nonmember physicians except for Scientific and Poster Session presenters and residents. Registration for the Postgraduate Course is separate from the Annual Meeting. There will be a $70 registration fee for attendees of the Postgraduate Program, which will be held Sunday, January 29. The Postgraduate Course will provide in-depth coverage of thoracic surgical topics selected to enhance and broaden the knowledge of practicing thoracic and cardiac surgeons. Advance registration forms, hotel reservation forms, and details regarding transportation arrangements, as well as the complete meeting program, will be mailed to Society members this fall (1994). Nonmembers wishing to receive information on attending the meeting should write to the Society's Secretary, Richard P. Anderson. Richard P. Anderson, MD Secretary The Society of Thoracic Surgeons 401 N Michigan Ave Chicago, IL (312)