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1689 Results of the Arterial Switch Operation for Transposition of the Great Arteries With Ventricular Septal Defect Surgical Considerations and Midterm Follow-up Data Roberto M. Di Donato, MD, Gil Wernovsky, MD, Edward P. Walsh, MD, Steven D. Colan, MD, Peter Lang, MD, David L. Wessel, MD, Richard A. Jonas, MD, John E. Mayer Jr., MD, and Aldo R. Castafneda, MD, PhD Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 Between January 1983 and December 1987, 62 patients underwent an arterial switch operation for transposition of the great arteries with ventricular septal defect or double outlet right or left ventricle. There were three hospital deaths (4.8%), and no deaths occurred in neonates (<1 month of age, n = 18). There were three late deaths, one due to coronary obstruction and two due to pulmonary vascular obstructive disease. One child has been lost to follow-up. We have prospectively evaluated the remaining 55 survivors by clinical evaluation, echocardiography, cardiac catheterization, ambulatory electrocardiographic monitoring, and limited electrophysiologic studies. The mean length of follow-up has been 27±16 months since surgery. One child has required reoperation for a residual ventricular septal defect; no child has undergone reoperation for supravalvar pulmonary or aortic stenosis. Aortic regurgitation was identified in 12 children (22%), which was mild in 11 and moderate in one. One child has asymptomatic occlusion of the left main coronary artery, one child has a tiny right coronary artery-to-pulmonary artery fistula, and one child has abnormal left ventricular wall motion according to follow-up angiography. No other abnormalities of systemic (left) ventricular function have been identified at late follow-up. In addition to the two late deaths due to pulmonary vascular obstructive disease, three children, all of whom were repaired at more than 6 months of age, have elevated pulmonary vascular resistance. Notable postoperative arrhythmias include complete heart block in four patients and nonsustained supraventricular or ventricular tachycardia early after surgery in eight patients (all resolved without medication at later follow-up). Only two patients have evidence of sinus node dysfunction and have not required treatment. The low hospital mortality and encouraging early follow-up data represent a significant improvement over atrial level repairs, supporting the arterial switch operation as the procedure of choice for children who have transposition of the great arteries with ventricular septal defect or double outlet ventricle. Because of the potential for the development of early pulmonary vascular obstructive disease in these patients, repair is recommended within the first 2 months of life. (Circulation 1989;80:1689-1705) recently as 25 years ago, a child born with transposition of the great arteries (TGA) had only a 10% chance of surviving to 1 year of age.' The pioneering work by Rashkind and A s From the Departments of Cardiology and Cardiothoracic Surgery, The Children's Hospital, and the Departments of Pediatrics and Surgery, Harvard Medical School, Boston, Massachusetts. Supported in part by grant HL-41786 from the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland. Address for correspondence: Gil Wernovsky, MD, Department of Cardiology, The Children's Hospital, 300 Longwood Avenue, Boston, MA 02115. Received May 31, 1989; revision accepted August 11, 1989. Miller2 for initial palliation, along with the innovative surgical repairs by Senning3 and Mustard,4 has dramatically improved the long-term prognosis for these children.5 Early surgical repair has been advocated to reduce long-term complications, specifically the development of pulmonary vascular obstructive disease6'7 and the long-term effects of See p 1912 chronic cyanosis.8 A recent report of the Mustard operation in neonates and infants has been particularly encouraging, with no deaths in 36 consecutive neonates with TGA and an intact ventricular septum (IVS).9 1690 Circulation Vol 80, No 6, December 1989 Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 Despite significant improvements in hospital mortality, long-term follow-up has revealed disturbing incidences of arrhythmia,10-15 tricuspid regurgitation,1416-1 8ventricular dysfunction,19-24 and sudden death5J3"18 after atrial level repair. Many of these late complications seem to be inherent to the intraatrial repair itself, particularly the extensive atrial incisions and suture lines and continued reliance on the right ventricle and tricuspid valve as the systemic ventricle and atrioventricular valve. The coexistence of a ventricular septal defect (VSD) with TGA adds to the above problems, including a higher surgical mortality,13,25-30 an increased risk of pulmonary vascular obstructive disease,6'3' and tricuspid regurgitation. Closure of a VSD combined with an atrial inversion repair frequently involves manipulation of the tricuspid (systemic atrioventricular) valve, which may be structurally abnormal,32-33 or occasionally a ventriculotomy in what will remain the systemic ventricle. For these reasons, the arterial switch operation (ASO) has been proposed as an alternative procedure for the treatment of TGA with VSD.29-30-34-39 Compared with atrial level repairs, the major potential advantages of the ASO are establishment of the left ventricle and mitral valve as the systemic ventricle and atrioventricular valve, and the maintenance of sinus node function by avoiding extensive atrial surgery. Although TGA with VSD was originally believed to be the only condition suitable for a primary ASO, many centers have also begun to successfully perform an ASO in neonates with TGA and IVS. However, when comparing earlier results of an ASO in patients with VSD to those with IVS, the association of a VSD with TGA has been considered to be an incremental risk factor for the ASO36,40 This report details our experience with the ASO for TGA with VSD, including double outlet right or left ventricle at The Children's Hospital in Boston. We have offered an ASO to all patients with TGA since 1983 and have continued prospective followup in all patients to serially and systematically evaluate anastomotic growth, ventricular function, and rhythm abnormalities. TABLE 1. Patient Population Characteristic Group 1 Group 2 Group 3 n 18 31 13 Age 9+8 days 4+3 mo 17+18 mo Weight (kg) 3.7+0.6 5.2+2.3 8.2+4.4 Balloon atrial septostomy 17 (94) 19 (61) 7 (54) Values are mean+SD when appropriate. Values in parentheses are percentages. Group 1, primary repair in neonates; group 2, primary repair in infants and children; group 3, repair after prior pulmonary artery banding. Methods Surgical Management The techniques used in our institution for the arterial switch portion of the procedure,44,45 as well as the VSD closure,46 have been reported previously. In two patients, excision of a small segment of the posterior commissure of the native aortic valve was necessary because of a paracommissural location of the coronary ostia. In 12 patients, coronary transfer involved intentional division and ligation of a small conal branch to avoid tension on the coronary anastomosis. Four patients required revision of the coronary anastomoses because of an inability to be weaned from cardiopulmonary bypass. One child with origin of the left circumflex coronary Patient Population From January 1983 through December 1987, 62 patients underwent combined ASO and VSD closure. Fifty-six (90%) had TGA with VSD, five (8%) had double outlet right ventricle, and one (2%) had double outlet left ventricle. There were 43 male and 19 female patients, and they have been divided into three groups (Table 1). In group 1, 18 patients underwent primary repair as neonates (range, 1-26 days; mean+SD, 9+8 days); in group 2, 31 patients had primary repair in infancy (range, 1.5-17.5 months; mean, 4+3 months); and in group 3, 13 patients had a secondary repair after pulmonary artery banding (range, 4-72 months; mean, 17 + 18 months). In group 1, 17 (94%) underwent balloon atrial septostomy, and eight (44%) also received prostaglandin El infusion before surgery for persistent cyanosis. In group 2, 19 (61%) underwent balloon atrial septostomy, and 21 children were older than 3 months of age, including six older than 6 months of age. Previous operations in this group included division of a vascular ring in one patient and aortic coarctation repair in another. Only seven of 13 (54%) patients in group 3 underwent balloon atrial septostomy. Ten patients had previously undergone a pulmonary artery banding combined with repair of obstructive lesions of the aorta (aortic coarctation in nine and interrupted aortic arch in one), whereas three patients had pulmonary artery banding alone. One of these had mild hypoplasia of the right ventricle and tricuspid valve. Other previous operations in this group included a Blalock-Hanlon atrial septectomy in two patients and a right Blalock-Taussig shunt in one. Additional cardiac and noncardiac diagnoses for the three groups are reported in Table 2. The location of the VSD is shown in Table 3 and was similar to other reported populations having TGA with VSD.28,41 The aorta was directly anterior to the pulmonary artery in 10 (16%), was anterior and rightward in 44 (71%), and anterior and slightly leftward in three (5%); the great vessels were side by side in five (8%). The coronary arterial branching patterns and their frequency are schematically illustrated in Figure 1 and are similar to other reported populations having TGA with VSD.42,43 Di Donato et al Arterial Switch Operation for TGA With VSD TABLE 2. Additional Diagnoses n Diagnoses Neuroblastoma 1 1 Hydronephrosis 6 Group 2 Subpulmonary stenosis 4 Dynamic 2 Atrioventricular valve tissue 3 Abnormal tricuspid valve 1 Straddling 1 Redundant tissue 1 Septal chordae 2 Left juxtaposition of the atrial appendages 1 Aortic coarctation 1 Vascular ring Dextrocardia 1 1 Left superior vena cava 1 Bicuspid pulmonary valve 1 Neuroblastoma 1 Multiple congenital anomalies Aortic coarctation 9 Group 3 1 Type A interrupted aortic arch Left juxtaposition of the atrial appendages 1 1 Redundant mitral valve tissue Group 1, primary repair in neonates; Group 2, primary repair in infants and children; Group 3, repair after prior pulmonary artery banding. Group Group 1 Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 from the right coronary artery and one child with a single right coronary artery required a second period of cardiopulmonary bypass and revision of the right coronary artery anastomosis to eliminate a kink in the circumflex artery. Two children with origin of the circumflex coronary from the right coronary artery required revision of the left anterior descending coronary anastomosis. One patient required a second period of cardiopulmonary bypass and circulatory arrest to close a residual VSD. In all cases, the neopulmonary reconstruction was accomplished during core rewarming. Anterior translocation of the pulmonary artery (Lecompte maneuver47) was performed in all but two patients (both with side-by-side great arteries). 1691 Cardiopulmonary bypass was conducted in three different ways depending on the date of operation and size of the patient. In 17 patients, who were operated upon early in our experience, the coronary and aortic anastomoses, as well as closure of the atrial defects and VSDs, were performed during one or two periods of deep hypothermic circulatory arrest totaling a mean of 83 14 minutes. In the patients with two periods of circulatory arrest, brief (approximately 10 minutes) periods of hypothermic reperfusion were interposed between the two periods of circulatory arrest. In 39 patients, the circulatory arrest time was limited to the atrial defect and VSD closure (mean, 46+22 minutes), whereas the ASO was performed under continuous low flow (50 ml/kg/min) hypothermic perfusion. In six patients, the entire procedure was performed under continuous low flow (50-75 ml/kg/min) cardiopulmonary bypass with profound hypothermia, including five patients with bicaval cannulation and a 17month-old child, without an atrial septal defect, who underwent patch closure of the VSD through the anterior semilunar valve. Details of cardiopulmonary bypass variables are shown in Table 4. The type and method of cardioplegia administration varied throughout the time period of the study. Crystalloid cardioplegia, 2.5% dextrose with 30 meq potassium chloride/l and 7 meq sodium bicarbonate/l or, more recently, Plegisol (Abbott) was used primarily. Two patients had oxygenated blood (100 ml per 1,000 ml of the dextrose cardioplegia) added to the solution. In 47 patients, a single dose (15-20 ml/kg) of cardioplegia was given after aortic cross-clamping. In 16 patients, two doses of cardioplegia were administered; one before VSD closure and one before the completion of the coronary and aortic anastomoses. The VSD was exposed through the right atrium in 48, through the anterior (native aortic) semilunar valve in six, through a combined (transatrial and transaortic) approach in four, through the native pulmonary valve in one, and through a right ventriculotomy in three (Table 3). The transpulmonary valve approach was adopted in one patient with a double outlet right ventricle and an L-malposed ± TABLE 3. Ventricular Septal Defect Anatomic Types and Surgical Approach Surgical approach VSD anatomic types Perimembranous Malalignment n % 25 22 5 5 40.2 35.5 8.1 8.1 6.5 1.6 100 Muscular Multiple 4 Atrioventricular canal type 1 Infundibular 62 Total VSD, ventricular septal defect. Right atrium 22 14 5 3 Aortic valve 3 3 Right atrium and aortic valve Pulmonary Right valve ventricle 2 1 2 1 1 3 2 4 48 6 4 1692 Circulation Vol 80, No 6, December 1989 1 4 A R+ L Usual Coronary Anatomy in TGA 40 (64.5%) LAD RCA. LAD Single Left Coronary Artery p R 2 LCx (3.2%) LCx RCA4 LCx 2 LAD 5 Circumflex Coronary from the Right Coronary Artery RCA 12 (19.4%) RCA Inverted Origin of the Coronary Arteries 2 (3.2%) LAD LCx LCx 3 6 RCA LAD Single Right Coronary Artery 4 4 (8.5%) (6.5%)RRCA CA L LAD Inverted Origin of the Coronaryand Artery ~~RightCircumflex (3.2%) ~~~~~~2 Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 ~LCxLC FIGURE 1. Distribution of coronary artery branching in 62 patients. TGA, transposition of the great arteries; LAD, left anterior descending coronary artery; LCx, left circumflex coronary artery; RCA, right coronary artery. aorta; the transventricular approach In five patients, the sternum was not closed primarily; the sternotomy was covered with a Silastic sheet without reapproximation of the bone edges followed by secondary sternal closure 2-26 days (median, 5 days) after surgery. These five patients included three of the four who required intraoperative revision of a coronary anastomosis and the patient who required a second period of cardiopulmonary bypass to close a residual VSD. All five patients had significant myocardial edema, three of whom had significant ventricular irritability during the initial attempt to close the sternum. The mean aortic cross-clamp time was 117 minutes for this group of children. was chosen in patient with overriding tricuspid valve, in one with right ventricular and tricuspid valve hypoplasia, and in one with the Taussig-Bing anomaly. Small VSDs were closed by suture alone in six neonates, and larger VSDs were closed by patch in the remaining 56 patients (five of whom had additional small muscular defects closed primarily with suture). An atrial septal defect was present in 55 patients and was closed primarily in 52 and with a patch in three. In addition to removal of the pulmonary artery band in 13 patients, associated procedures included right pulmonary artery plasty in four children (all after prior pulmonary artery banding), pulmonary ("neoaortic") valvotomy in one, resection of a subpulmonary (left ventricular outflow tract) fibrous ridge in one, reduction arterioplasty of a massively dilated main pulmonary artery that had been causing significant airway compression in one, division and reattachment of straddling tricuspid valve chordae in two, excision of redundant atrioventricular valve tissue in two (one mitral and one tricuspid), and patch reconstruction of the right ventricular outflow tract in one. one Postoperative Management Patients were weaned from cardiopulmonary bypass with inotropic support as dictated by heart rate, left and right atrial pressures, and the systolic blood pressure. In the latter half of our experience, patients were kept sedated with a continuous Fentanyl (10 ,ug/kg/min) infusion and neuromuscular blockade during the early (12-24 hours) postoperative period. Parenteral nutrition was initiated in most cases beginning on the first postoperative morning. Inotropic and TABLE 4. Perfusion Data Total time Patient groups n on cardiopulmonary bypass 163.4+41.4 170.5-+35.1 Aortic cross clamp (min) 91.6+14.9 99.0+21.0 102.2+11.7 Deep hypothermic circulatory arrest (min) 47.6+25.0 57.1+29.5 44.3+35.5 18 Group 1 31 Group 2 13 Group 3 173.2-+18.2 Total time on cardiopulmonary bypass includes circulatory arrest. Group 1, primary repair in neonates; Group 2, primary repair in infants and children; Group 3, repair after prior pulmonary artery banding. Di Donato et al Arterial Switch Operation for TGA With VSD 1693 Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 ventilatory support were withdrawn as permitted by clinical status. Digoxin and diuretics were routinely instituted on the first postoperative day and were usually continued after discharge. heart block was present. The child died suddenly 12 hours after surgery. An autopsy revealed a residual VSD, a restrictive left coronary anastomosis, and a subendocardial infarction of the anterior septum. Follow-up Protocol Studies at hospital discharge included 12-lead electrocardiography (ECG), 24-hour continuous ambulatory ECG monitoring, and two-dimensional echocardiography with Doppler, including analysis of load-independent indexes of left ventricular contractility.48,49 Outpatient follow-up included clinical evaluation and ECG at 6-month intervals, 24hour ambulatory electrocardiographic monitoring, and two-dimensional echocardiography with Doppler every 1-2 years. A complete hemodynamic and electrophysiologic evaluation was recommended within 1 year of surgery. The detailed methods of this protocol have been previously reported for patients having TGA with IVS.49,50 The methods used in our laboratory for quantifying valvar regurgitation by echocardiography with Doppler have also been previously reported.51 The great majority of patients in this study population were referred from and continue to be followed up at other institutions, which accounts for variable protocol compliance and intervals of follow-up. Only 10 of these children are primarily cared for at The Children's Hospital in Boston, although 10 additional patients did return for postoperative echocardiography and left ventricular function analysis, and five returned for cardiac catheterization. All of the referring cardiologists were contacted; the clinical records, ECGs, and angiograms (through February 1989) were reviewed by one of the authors (G.W.). Hospital Morbidity Among the 59 hospital survivors, the mean hospital stay was 18 ± 10 days (median, 16 days; range, 8-71 days). The duration of mechanical ventilation and intravenous inotropic support was 5±3 days. Eight reoperations were performed in seven patients: two for postoperative bleeding, five for delayed sternal closure, and one for a VSD that was believed before surgery to be hemodynamically insignificant and therefore was not initially repaired. This membranous VSD eventually required patch closure 37 days after the ASO because of congestive heart failure associated with a pulmonary-to-systemic flow ratio (Qp/Qs) of 3:1 at cardiac catheterization. Four patients required permanent pacemaker implantation for complete heart block. Two were neonates who had a small perimembranous VSD suture closed through the right atrium; one of these neonates required intraoperative revision of the left anterior descending coronary artery and was in normal sinus rhythm before his second period of cardiopulmonary bypass, suggesting that myocardial ischemia played a role in the development of heart block. The other two patients with complete heart block were infants who underwent VSD patch closure; one had an anteriorly malaligned VSD, and one had an atrioventricular canal-type VSD with a straddling tricuspid valve. In addition, one neonate developed complete left bundle branch block after single-suture closure of a small VSD. Two neonates had nonsustained supraventricular tachycardia, and one had accelerated junctional rhythm early after surgery while in the intensive care unit. Five children had seizure activity in the immediate postoperative period that was controlled with anticonvulsant medications. The mean circulatory arrest time in these five patients was 64 minutes (range, 41-89 minutes) compared with a mean of 50 minutes (range, 0-112 minutes) in the 54 survivors who did not have seizures. No other neurologic abnormalities such as abnormal movements, hemiparesis, or choreoathetosis were noted in the postoperative period. Hospital and Discharge Studies Two-dimensional echocardiography and Doppler. Early (days 1-3) postoperative echocardiograms were obtained in 35 patients. Ten studies were technically inadequate for assessment of left ventricular function, 15 patients had qualitatively normal left ventricular function, and 10 had left ventricular function abnormalities. One had marked hypokinesia of the interventricular septum and diaphragmatic free wall in the postoperative period that persisted for 12 days, gradually normalizing by the time of hospital discharge (day 33 after surgery). Results Hospital Mortality There were three hospital deaths (4.8%), all within 24 hours of surgery. The hospital mortality decreased from 18.2% (two of 11) before June 1985 to 2.0% (one of 51) after June 1985. There were no deaths in the neonatal group. The two deaths early in our experience were believed to be related to coronary compression by a dilated left pulmonary artery. This involved a single right coronary artery in a 1-month-old child with side-by-side great arteries in whom the Lecompte maneuver was not performed and the left anterior descending coronary in a 6-month-old child with the usual coronary anatomy and an anterior and rightward aorta. The third death occurred in a 5-month-old child with multiple VSDs, the usual pattern of coronary branching, and a previous coarctation repair and pulmonary artery banding. After the ASO, the pulmonary artery oxygen saturation was high, suggesting a significant residual VSD, and the pulmonary anastomosis was banded to facilitate withdrawal from cardiopulmonary bypass. Complete 1694 Circulation Vol 80, No 6, December 1989 Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 One patient developed severe biventricular dysfunction 16 days after surgery (despite a normal echocardiogram at 12 days), which resolved rapidly during the next 2 days with normal left ventricular function at the time of discharge. Eight additional patients manifested global left ventricular (n=5) or biventricular (n=3) dysfunction, which resolved by the time of discharge in six patients. Complete two-dimensional echocardiography with Doppler was performed on 55 (93%) patients before hospital discharge. All of these patients were taking digitalis and diuretics at the time of evaluation. A small, hemodynamically insignificant residual VSD was detected by pulsed or color Doppler in 23 patients, and a large, minimally restrictive VSD was imaged in one. Mild regurgitation of the neoaortic valve was noted in 12 patients (22%) with no instances of moderate or severe aortic regurgitation. Mild stenosis (.35 mm Hg) at the aortic anastomosis was found in three patients and at the pulmonary anastomosis in six, and moderate (45 mm Hg) supravalvar pulmonary stenosis was found in one. Regurgitation of the neopulmonary valve was mild in 14 patients and moderate in two. Mitral regurgitation was mild in eight patients and moderate in one; tricuspid regurgitation was mild in 14 and moderate in two. Systolic flattening of the interventricular septum, indicating right ventricular systolic hypertension,52 was present in 12 patients. Four patients were noted to have hypokinesia or dyskinesia of the basilar septum, and two had mild global dysfunction that could not be quantitatively assessed because of left ventricular distortion by residual right ventricular hypertension. In 14 patients without regional dysfunction who also had normal left ventricular configuration (i.e., no systolic or diastolic septal flattening), quantitative analysis of left ventricular performance was performed (Table 5). Fractional shortening (FS) was normal (>28%) in 11 patients and was below normal in three. Ventricular enddiastolic dimension, the rate-corrected velocity of shortening (VCFc), end-systolic stress (ESS, a quantitative measure of ventricular afterload48,49,53), and the ESS-VCFc relation (a load-independent index of contractility48,54) were normal in all patients (Figure 2). In contrast, the ESS-FS relation (a preloaddependent index of contractility48) was subnormal in five patients (Figure 3). This pattern of a low ESS-FS relation compared with the ESS-VCFc relation is found uniquely with low preload status.48,49,54 Thus, these subjects manifested normal afterload and contractility with reduced preload. Electrocardiography and ambulatory monitoring. Predischarge 12-lead ECGs from the 59 hospital survivors revealed normal sinus rhythm in 91 %. One patient had an ectopic atrial escape rhythm, and four were paced for complete heart block. Intraventricular conduction disturbances (usually right bundle branch block) were observed in 63% (Table 7). TABLE 5. Echocardiographic Measurements in Patients Evalu. ated at Hospital Discharge BSA EDD FS VCFc ESS (n2) (cm) (%) (circ/sec) (g/cm2) 0.27 1.83 43.7 1.57 13 0.36 2.21 33.5 1.13 27 0.24 2.10 31.0 1.14 23 0.46 2.95 33.9 1.10 29 0.29 1.97 36.5 1.26 22 0.27 2.29 37.1 1.33 37 0.26 2.38 34.9 1.29 22 0.29 1.90 37.8 1.35 16 0.25 1.83 26.2 1.18 28 0.28 2.09 27.8 1.20 37 0.26 2.20 34.1 1.21 21 0.22 1.62 33.3 1.33 20 0.29 2.45 26.3 0.96 43 0.19 2.19 30.1 1.11 33 33.3 1.23 27 4.6 0.14 8 BSA, body surface area; EDD, end-diastolic dimension; FS, fractional shortening; VCFc, rate-adjusted velocity of fiber shortening; ESS, end-systolic wall stress. Mean SD 0.28 0.06 2.14 0.32 Predischarge ambulatory electrocardiographic recordings were available for 49 patients (Table 8). Physiologic sinus rates were present in 86%, and 10% had some episodic sinus slowing with junctional escape rhythm. Atrial ectopy was frequently seen, but no sustained or symptomatic supraventricular tachycardias were recorded. The incidence of ventricular ectopy was high and included couplets and nonsustained ventricular tachycardia in 24%. The longest salvo of ventricular tachycardia was seven beats in duration. Because of the uncertain significance of this ventricular ectopic activity in the early postoperative period, suppressive drug therapy was not recommended, although such patients were followed up more closely with serial ambulatory recordings. Clinical Status Late mortality. Among the 59 hospital survivors, three late deaths have occurred. One child who had a primary ASO and VSD closure at 26 days of age had a large coronary artery from the right posterior sinus that gave rise to the right coronary, the left circumflex, and left anterior descending coronary arteries; only a small conal branch arose from the left anterior sinus. The right coronary anastomosis required intraoperative revision and two periods of cardiopulmonary bypass; placement of a Silastic sheet was necessary. The postoperative course was complicated by a sternal wound infection. After what appeared to be a stable recovery period, and after documentation of normal ventricular contractility and function by echocardiography at discharge, he was admitted 6 weeks later with the Di Donato et al Arterial Switch Operation for TGA With VSD A 1695 B I.D. 1.41 0 0) 0) 1.2 -_._ 0 0 IL 1.0 CD) L- -_ 0.8 o.6 %0.10 - 0 25 50 75 Meridional ESS (gm/cm2) 100 Meridional ESS (gm/cm2) Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 FIGURE 2. Plot ofthe relation ofrate-adjusted velocity ofcircumferential fiber shortening (VCFc) (Panel A) and percent fractional shortening (FS) (Panel B) to afterload (end-systolic wall stress, ESS) in 14 subjects at hospital discharge. The mean population regression line (solid line) and 95% confidence intervals (dashed lines) for nornal subjects in our laboratory are displayed. Normal-to-increased contractility (Panel A) was typical in these patients. The ESS-FS relation (Panel B) tended to be nornal or low, which is consistent with reduced preload. In three subjects, preload reduction was of sufficient magnitude to result in abnornal (<28%) FS. acute onset (<48 hours) of congestive heart failure. Two-dimensional echocardiography revealed global depression of myocardial function, and diffuse ischemic changes were seen on the electrocardiogram. He died within hours while awaiting cardiac catheterization. Autopsy revealed narrowing at the single right main coronary ostium, and histologic study of the left ventricle showed findings of nontransmural myocardial infarction. One child referred from another institution, who had previously undergone pulmonary artery banding at 6 months of age and a subsequent BlalockTaussig shunt at 8 months of age, developed progressive pulmonary vascular obstructive disease. At catheterization 2 days before the ASO and VSD closure (age, 19 months), the mean pulmonary artery pressure was 48 mm Hg with a calculated pulmonary vascular resistance of 6.2 Wood's units; immediately after surgery, the peak systolic pulmonary artery pressure was about 1/2 systemic pressure. Signs and symptoms of right heart failure developed 3 months after surgery, and cardiac catheterization revealed suprasystemic pulmonary artery pressure with minimal response to oxygen. The patient died at home 6 months after surgery. A third child referred from another institution had an ASO and VSD closure at 10 months of age after an atrial septectomy and an ineffective pulmonary artery banding (systolic pulmonary artery pressure, 82 mm Hg, pulmonary vascular resistance, 4.8 Wood's units). The ASO was complicated by complete heart block, and postoperative peak systolic pulmonary artery pressure was equal to systemic pressure. A permanent pacemaker was placed before hospital discharge, and the patient returned to his home country. Echocardiography performed 1 and 3 months after surgery suggested right ventricular hypertension with dimin- ished right ventricular function, presumably because of pulmonary vascular disease. The patient died suddenly 5 months after the ASO; the details of his death and status of his pacemaker are unknown; no autopsy was performed. Clinical follow-up. One child has been lost to follow-up out of the country. The remaining 55 survivors have been followed up for a mean of 27±16 months (range, 11-70 months) after surgery (mean absolute age, 33+22 months). One child with pulmonary vascular obstructive disease after primary repair at 6 months of age is mildly cyanotic because of a right-to-left interventricular shunt. No other patient has cardiovascular symptoms. Fortynine of 55 patients (89%) have normal (5th-95th percentile) height and weight. Ten children continue to take digitalis, and three remain on diuretic therapy, although none has clinical manifestations of congestive heart failure. Two children have been described by their referring physicians as having moderate developmental delay, and one child continues to take anticonvulsant medication. Although systolic murmurs at the base are a typical finding, only two patients have murmurs associated with a palpable thrill (grade 4); in one patient, this is due to a subaortic membrane, and in another, it is due to moderate supravalvar pulmonary stenosis. Although 12 patients have been shown to have aortic regurgitation by angiography or Doppler at later follow-up (see below), only three patients have audible diastolic murmurs of aortic regurgitation. No child has required reoperation for supravalvar pulmonary or aortic obstruction. One child required reoperation to close a large residual VSD. Hemodynamic Evaluation Of the 55 known late survivors, 44 (80%) have undergone cardiac catheterization: 15 of 17 (88%) in Circulation Vol 80, No 6, December 1989 1696 PEAK SYSTOLIC GRADIENT 45# 40 0* 35 . 30 0) m 25 E .b E 20 00 . 15 10 oh_ 5 n. U __ . _ :t o RV-PA (distal) -- LV-AO *sub-aortic membrane Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 FIGURE 3. Plot of the peak systolic ejection gradient (mm Hg) from right ventricle (RV) to distal pulmonary artery (PA) and left ventricle (LI) to ascending aorta (AO) measured at cardiac catheterization. The RV-PA gradient is a sum of the subvalvar, supravalvar, and branch PA gradients in each patient (see teext). The open circle represents subaortic obstruction in one patient. group 1, 21 of 28 (75%) in group 2, and eight of 10 (80%) in group 3. One child (group 2) had only an arterial study performed because of infrarenal obstruction of the inferior vena cava. The catheterizations were performed at a mean of 10.6+4.7 months after surgery (mean absolute age, 17 months). Great vessel anastomoses. The right ventricular to distal pulmonary artery peak systolic ejection gradient (PSEG) is shown in Figure 3. One patient had a moderate degree (45 mm Hg PSEG) of obstruction at the suture line, whereas the remaining 43 patients had PSEGs of 35 mm Hg or less. Multiple areas of potential obstruction were identified; one patient (with the Taussig-Bing anomaly) had primarily subvalvar narrowing (PSEG, 27 mm Hg), one had valvar obstruction (PSEG, 30 mm Hg), and five had branch (four right and one bilateral) pulmonary stenosis (>10 mm Hg PSEG). Most patients had small (5-15 mm Hg) gradients measured at the anastomosis. The PSEG from the left ventricle to ascending aorta is also shown in Figure 3. One patient had moderate (PSEG, 42 mm Hg) subvalvar obstruction due to a subaortic membrane, and one had moderate (PSEG, 38 mm Hg) obstruction at the aortic suture line. The remaining 42 patients had PSEGs of 20 mm Hg or less, and 29 (66%) of these had a PSEG of 5 mm Hg or less. An incidental finding of aortic coarctation with a PSEG of 15 mm Hg was found on pressure pullback measurements and angiography (Figure 4) in one patient with no arch obstruction identified after surgery, presumably because of late constriction of ductal tissue. Of the eight patients studied with preoperative arch obstruction, one had a residual PSEG of 20 mm Hg, whereas the remaining seven patients had PSEGs of 10 mm Hg or less. FIGURE 4. Lateral projection of ascending aortogram in a patient 15 months after neonatal repair (age, 9 days). No coarctation was identified before surgery. Note narrowing distal to the left subclavian artery, a 20-mm Hg peak systolic gradient was measured at this site. (Photograph courtesy of Dr. Michael Snyder.) Di Donato et al Arterial Switch Operation for TGA With VSD END-DIASTOLIC PRESSURE 12 0 1697 80 20 10 0* 0* 15. 0@ 60 8 E I E E 10[ 4: 0 0 0 Q 40 0: mm *00 41 20 5 .. ~0 U. an RV C -. LV *large residual VSD Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 FIGURE 5. Plot of right (RP) and left (LV) ventricular end-diastolic pressures measured at cardiac catheterization. The solid horizontal line represents the mean value. VSD, ventricular septal defect. Coronary anastomoses. The coronary anastomowell seen in all 44 children by aortic root angiography (n=43) or left ventriculography (n =43). One patient had complete occlusion of the left main coronary artery; the left anterior descending and left circumflex coronary arteries filled through collaterals from the right coronary artery. This patient was asymptomatic and had a normal ECG, cardiac index, and left ventricular end-diastolic pressure (LVEDP). One patient had a tiny fistula from the right coronary artery to the pulmonary artery without a significant left-to-right shunt. A small area of dyskinesis in the apex of the left ventricle was seen in one patient, who also had a normal ECG, cardiac index, and LVEDP. Oximetry and intracardiac shunting. The systemic oxygen saturation was 95% or more in all patients except one child with severe pulmonary vascular disease and a right-to-left shunt (arterial saturation, 90%) through a small residual VSD. Eight patients had trivial residual VSDs seen on left ventriculography, but the Qp/Qs was less than 1.5 in all. One patient had a large residual VSD (Qp/ Qs=2.4) that was successfully closed at reoperation. One patient has a residual atrial septal defect with QpIQs of 2.0, and another had a residual atrial septal defect seen on the pulmonary venous phase of the right ventriculogram with an insignificant (Qp/Qs=1.2) shunt. Ventricular function. The mean right ventricular end-diastolic pressure (RVEDP) was 5±2 mm Hg, whereas the mean LVEDP was 7+2 mm Hg (Figure 5). The patient with a large residual VSD had markedly elevated filling pressures (RVEDP and LVEDP, 16 mm Hg). Only three other patients had an LVEDP greater than 10 mm Hg, one of whom had ventricular pacing due to complete heart block and one who had moderate supravalvar aortic obstruction (38 mm Hg). The mean systemic carses were 0 rI 2 0 S 0 .rARiduni VSD FIGURE 6. Plot of the pulmonary vascular resistance (PVR) measured in Wood's units (wu) and the mean pulmonary artery pressure (PAp) measured at cardiac catheterization. Group I, primary repair in neonates; group II, primary repair in infants and children; group III, repair after prior pulmonary artery banding. The solid horizontal lines represent the mean values for each group; there were no significant differences detected between groups. The open circles represent a patient with a large residual ventricular septal defect (VSD). diac index was determined in 41 patients (by thermodilution in six and the Fick method in 35) and measured 4.1±+1.1 1/min/m2. Pulmonary hypertension. The mean pulmonary arteiy pressure and pulmonary vascular resistance was measured in 43 patients and is shown in Figure 6. In addition to the two patients who died within 6 months of surgery because of progressive pulmonary vascular disease (both in group 3), two additional patients (one in group 2 and one in group 3) had markedly elevated pulmonary vascular resistance. No patient who underwent repair during the neonatal period (group 1) had an elevated pulmonary vascular resistance. Semilunar and mitral valve regurgitation. Ascending aortograms were obtained in 43 patients. Catheter position precluded adequate evaluation of neoaortic valve competence in eight patients (one of whom had aortic regurgitation by clinical evaluation and echocardiography). Of the remaining 35 patients, eight (23%) had aortic regurgitation, including four of eight patients in group 3 who had previous pulmonary artery banding. The regurgitation was trivial or mild in seven patients and moderate in one. Left ventriculography in 43 patients revealed mild mitral regurgitation in two, one of whom had ventricular pacing for complete heart block. Late Follow-up Two-dimensional echocardiography and Doppler and ventricular function. Noninvasive studies were performed at The Children's Hospital in 20 patients at a mean of 20±18 months (range, 6-73 months) after surgery (mean absolute age, 23 months). Small 1698 Circulation Vol 80, No 6, December 1989 Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 FIGURE 7. Echocardiographic stop-frame of an apical twochamber view in a 2.2-yearold patient after neonatal (4 days) repair. An echogenic, dyskinetic segment in the basilar portion of the interventricular septum represents myocardial infarction (MI), thought to be the cause of this patient's complete heart block. LA, left atrium; LV left ventricle. residual VSDs were noted in two children. Mild aortic regurgitation was detected in four patients, all of whom had this finding at the time of hospital discharge and at cardiac catheterization. A mild gradient (<35 mm Hg) was present across the aortic anastomosis in three patients. One patient had a discrete subaortic membrane with a 50-60 mm Hg maximum instantaneous gradient (40 mm Hg PSEG at catheterization) that was not present at the time of discharge. The degree of obstruction had slowly progressed in the 2 years since surgery. There were four instances of mild (<35 mm Hg) and two of moderate (35 60 mm Hg) supravalvar pulmonary stenosis. Mild pulmonary regurgitation was found in six patients, mild tricuspid regurgitation in four, and mild mitral regurgitation in two. No patient had systolic flattening of the IVS, suggestive of significant right ventricular hypertension, including the two patients with moderate supravalvar pulmonic stenosis and the four who had systolic septal flattening at the time of discharge. Only one of the four patients with regional abnormalities at the time of discharge had late echocardiographic data available; this patient continues to show septal akinesis consistent with septal infaretion (Figure 7). This patient developed complete heart block after a second period of cardiopulmonary bypass to revise the left anterior descend- ing coronary anastomosis. Quantitative assessment of left ventricular performance could not be obtained in four additional patients because of poor echocardiographic windows but was obtained in the other 15 patients (Table 6). Left ventricular end-diastolic dimension and wall thickness, FS, VCFe, and ESS were normal in all. In addition, the ESS-VCFe and ESS-FS relations were normal and concordant in all, indicating normal contractility and normal preload status (Figure 8). Late (mean, 11±10 months; range, 5-56 months after surgery) echocardiograms were obtained at outside institutions in 16 additional patients, including seven of the 11 patients who had not had a hemodynamic evaluation by cardiac catheterization. A tiny residual VSD was noted in two patients and a residual atrial defect in one. Mild aortic regurgitation was noted in four patients, which was present at hospital discharge in all. No patient had significant (>35 mm Hg) supravalvar pulmonary stenosis, although one had a supravalvar aortic gradient of 38 mm Hg confirmed at catheterization. Mild tricuspid regurgitation was identified in two patients, and mild pulmonary regurgitation was seen in another two patients. Arrhythmia and conduction evaluation. On followup ECG (Table 7), sinus rhythm was preserved in 88% of patients. One had ectopic atrial escape, and Di Donato et al Arterial Switch Operation for TGA With VSD 1699 TABLE 6. Echocardiographic Measurements in Patients Evaluated at Follow-up Examinations BSA (i2) 0.80 0.67 0.89 0.46 0.58 0.61 0.44 0.49 0.44 0.52 0.47 0.53 0.45 0.56 0.46 EDD (cm) 4.05 3.55 2.30 2.49 3.26 2.76 2.45 3.09 2.85 2.83 2.92 2.50 2.48 2.74 2.37 EDWth (cm) 0.69 0.58 0.49 0.59 0.58 0.60 0.55 0.61 0.58 0.62 0.61 0.50 0.54 0.68 0.54 FS VCFc ESS (%) (circ/sec) (g/cm2) 35.3 37.2 31.4 42.6 34.4 34.4 38.8 32.0 36.8 43.8 35.6 42.0 35.9 39.8 43.0 1.05 1.15 0.94 1.30 0.98 1.18 1.12 1.02 1.07 1.12 1.01 1.22 1.05 1.11 1.27 52 44 51 23 51 31 34 48 39 18 33 33 34 27 18 Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 2.84 Mean 0.56 0.58 37.5 1.11 36 0.13 0.47 3.8 SD 0.05 0.10 11 Following examination was at Children's Hospital, Boston. BSA, body surface area; EDD, end-diastolic dimension; EDWth, end-diastolic wall thickness; FS, fractional shortening; VCFc, rate-adjusted velocity of fiber shortening; ESS, end-systolic wall stress. two had junctional escape rhythm. Of the patients with postoperative complete heart block, one died, and three had functioning ventricular pacemakers. No progression of atrioventricular or intraventricular conduction disturbances was noted. Follow-up ambulatory electrocardiographic recordings (Table 8) showed evidence of sinus node dysfunction in two asymptomatic patients who spent large portions of the monitoring period in junctional rhythm. Atrial and ventricular ectopy had largely resolved. No patient received specific antiarrhythmic medication at any time during the follow-up. Limited electrophysiologic data were collected from 18 patients at the time of their hemodynamic evaluation 7-21 months (mean, 10.3 months) after operation. Corrected sinus node recovery time was prolonged to 400 msec in one patient who had no other evidence of sinus node dysfunction on ECG or ambulatory recordings. Sinus node recovery time was normal for the remaining patients. The AH interval was normal for all. The HV interval was prolonged to 90 msec in one patient, without other clinical evidence of impaired atrioventricular conduction. Right ventricular apex activation time was B A a 0 0 0 I,) I._0 LL 0 .. Meridional ESS (gm/cm2) .. -- . . _ __,---2% Meridional ESS (gm/cm) FIGURE 8. Plot of the relation of rate-adjusted velocity of circumferentialfiber shortening (VCFc) (Panel A) and percent fractional shortening (FS) (Panel B) to afterload (end-systolic wall stress, ESS) in 15 patients at late (20+18 months) follow-up. The mean population regression line and 95% confidence intervals for nornal subjects in our laboratory are displayed as described in Figure 2. Normal contractility (Panel A) and FS (Panel B) were present in all patients. 1700 Circulation Vol 80, No 6, December 1989 TABLE 7. Early and Late Electrocardiographic Findings Hospital discharge 0.4+0.3 59 Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 Months after surgery (mean+SD) Patients (n) Basic rhythm (n) Normal sinus rhythm Ectopic atrial pacemaker Junctional rhythm Paced AV conduction (n) Normal First-degree AV block Second-degree AV block Third-degree AV block Ventricular conduction (n) Normal Complete RBBB Left anterior hemiblock Bifascicular block Complete LBBB Paced Values in parentheses are percentages. AV, atrioventricular; RBBB, right bundle branch block; hemiblock; LBBB, complete left bundle branch block. normal in all patients, including those with ECG patterns of right bundle branch block, suggesting only "peripheral" right ventricular conduction injury. Discussion The low surgical mortality rate, excellent clinical status of the survivors, low incidence of late reoperation, and the midterm follow-up data showing preservation of sinus rhythm and systemic ventricular function support our policy to perform an ASO 54 (91) 1 (2) 0 (0) 4 (7) Last follow-up 20.4+ 15 .9 52 46 1 2 3 (88) (2) (4) (6) 54 (91) 1 (2) 0 (0) 4 (7) 48 (92) 1 (2) 0 (0) 3 (6) (30) (45) (2) (14) (2) 19 (37) 21 (40) 1 (2) 7 (13) 1 (2) 3 (6) 18 27 1 8 1 4 (7) bifascicular block, complete RBBB+left anterior for patients having TGA with VSD or double outlet ventricle. For the purposes of this study, we have included all patients in whom surgical closure of a VSD was performed, regardless of preoperative determination of VSD size, pulmonary blood flow, or pulmonary arterial pressure. This is an operational definition; children with small restrictive VSDs in whom no surgical attempt is made to close the defect are considered in the group of patients having TGA with intact ventricular septum; seven such TABLE 8. Early and Late Ambulatory Electrocardiographic Monitoring Hospital discharge Months after surgery (mean+SD) Patients (n) Basic rhythm (n) Normal sinus rhythm Episodic sinus bradycardia with junctional escape Paced Atrial ectopy (n) None Atrial premature beats Nonsustained supraventricular tachycardia Ventricular ectopy (n) None Ventricular premature beats Couplets Nonsustained ventricular tachycardia Values in parentheses are percentages. 0.4+0.4 49 Last follow-up 12.0±9.0 28 42 (86) 26 (93) 5 (10) 2 (4) 2 (7) (0) 0 10 (20) 35 (71) 16 (57) 11 (39) 4 (8) 1 (4) 11 (22) 26 (54) 8 (16) 21 (75) 7 (25) 4 (8) 0 (0) 0 (0) Di Donato et al Arterial Switch Operation for TGA With VSD Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 children have been previously reported.50 The closure of a VSD of any size prolongs the ASO and adds to the potential risk of conduction system injury; thus, all children who had ASO and VSD closure are included in this report. Hospital Mortality In our initial experience with ASO and VSD closure (from January 1983 to June 1985), hospital mortality (18.2%) was similar to that previously reported with both Senning and Mustard operations combined with VSD closure.1325-28 However, in the second half of this experience (from June 1985 to December 1987), the early mortality after the ASO has been very low (2.0%), establishing a clear superiority over the atrial level repair for TGA with VSD. Furthermore, unlike the experience of others,36,37,40,55 our recent results with the ASO for TGA with VSD have been similar to the results for the ASO for TGA with intact ventricular septum.50 Therefore, the higher incidence of associated cardiac and noncardiac anomalies seen in patients with TGA with VSD36 may be a less significant risk factor than previously reported.30 The absence of hospital deaths in the neonatal group is consistent with the experience of others38,40,56 and is very encouraging. Early repair should decrease the incidence of pulmonary vascular disease and minimize the adverse effects of cyanosis or congestive heart failure. Early repair would also decrease the likelihood of spontaneous closure of small membranous or muscular VSDs; spontaneous VSD closure could reduce left ventricular pressure, thereby leading to a loss of adequate myocardial mass and potentially rendering the patient unsuitable for a primary ASO. We therefore recommend that patients having TGA with VSD undergo repair soon after the diagnosis is made, generally within the first 2 months of life. If surgery is to be delayed much beyond the immediate neonatal period, balloon atrial septostomy should be considered to allow for better intercirculatory mixing and to "decompress" the left atrium. Technical Considerations In most patients, the VSD was approached through the right atrium (75%), native aortic valve (10%), or both (6%). During an ASO, any surgical manipulation of the tricuspid or native aortic valve during VSD closure is of lesser concern than in the case of an intra-atrial operation because both valves will be in the low-pressure, pulmonary circulation after the ASO. On the other hand, the transpulmonary approach should probably be avoided to lessen the risk of damaging the future aortic valve. The transventricular approach is a useful alternative for VSD closure, although unnecessary in most occasions. Coronary translocation has been possible with all of the described branching patterns. When the 1701 circumflex coronary coronary artery arises from the right artery, the site of implantation in the neoaortic wall has been placed slightly more superiorly to avoid kinking of this vessel. The rarer variations (single coronary ostia and forms of "inverted" origin) occurred too infrequently in this series to allow for a formal statistical comparison of the relative risks of the various branching patterns. The recent use of deep hypothermic low flow (50 ml/kg/min) cardiopulmonary bypass has resulted in shorter periods of circulatory arrest. Although shorter periods of circulatory arrest may be beneficial to later central nervous system function, studies of neurologic sequelae after prolonged, continuous low flow cardiopulmonary bybass are lacking, and the issue of circulatory arrest and long-term neurologic outcome remains unresolved. Although the five patients in this report who had postoperative seizures had moderately long periods of circulatory arrest, the wide range of circulatory arrest times in this patient group and the relatively low incidence of postoperative seizures prohibit a reliable assessment regarding their etiology. Finally, a detailed evaluation of later development was not undertaken as part of this study; however, a formal assessment of developmental, cognitive, and neurologic sequelae of circulatory arrest and cardiopulmonary bypass is currently underway. Hospital Morbidity The average length of hospitalization was about 21/2 weeks, and reoperation and prolonged mechanical ventilation were the major causes of a prolonged stay in the intensive care unit. Twelve early reoperations, most notably delayed sternal closure and pacemaker implantation, were performed in 11 (19%) patients, which represents a higher incidence of early reoperation compared with atrial level repairs at our institution.28 Intraoperative revision of a coronary anastomosis or VSD patch led to extended periods of myocardial ischemia and cardiopulmonary bypass, which resulted in myocardial edema, necessitating delayed sternal closure in five patients. This was well tolerated, and in four of five patients, the secondary closure was within 5 days of the ASO. Five (8.0%) patients developed complete heart block, two of whom died (one shortly after surgery and one 5 months after surgery). A similar incidence (8.7%) of complete heart block was found in our institution with the Senning operation and VSD closure,28 which appears to be somewhat higher than in simple VSD closure.46,57,58 One welldescribed risk factor for the development of complete heart block is an inlet VSD with or without a straddling tricuspid valve,36 which was present in two of the children who developed complete heart block. Another possible explanation is an abnormal location of the bundle of His59 in children with TGA. Finally, complete heart block may occur after the ASO because of infarction of the basal portion 1702 Circulation Vol 80, No 6, December 1989 Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 of the interventricular septum as was suggested by the clinical course and postoperative echocardiogram in one child. Although the incidence of complete heart block may be somewhat higher, the incidence of lower grade atrioventricular conduction abnormalities (right bundle branch block, left anterior hemiblock, and bifascicular block) after the ASO with VSD closure appears to be similar to that reported for simple VSD closure.57,58,60 Ventricular dysfunction (as shown by twodimensional echocardiography) was a fairly common early finding and can be attributed to a variety of factors, including the effects of cardioplegia and aortic cross clamping, possible coronary insufficiency, and the effects of transfer of the systemic arterial load to the left ventricle that had previously supported the pulmonary circulation. The early postoperative echocardiographic studies were not performed on all patients, and clinical abnormalities were often the reason for obtaining these studies. This bias precludes an accurate assessment of the incidence of transient early postoperative abnormalities of left ventricular function in this patient group. Late Complications of the Arterial Switch Operation Aortic regurgitation is a common finding after arterial repair36'61-63 and was detected in this series by angiography or color Doppler examinations in a total of 12 of 55 late survivors (22%). No patient has been found to have the new appearance of aortic regurgitation or progression of its severity throughout follow-up. The severity of the aortic regurgitation was mild in most patients as determined by clinical evaluation, Doppler echocardiography, and angiography and appears at present to be of little hemodynamic significance. A recent report by Martin et a161 using quantitative radionuclide angiography showed similar findings. A prior pulmonary artery banding probably plays a role in the development of aortic regurgitation62 and should be avoided if possible in this patient group to reduce the potential for aortic regurgitation as well as pulmonary artery distortion. Aortic regurgitation has also occurred after a primary ASOS5 as well, and native pulmonary valve regurgitation has occasionally been noted before surgery.64 The ultimate impact of aortic regurgitation after the ASO on ventricular size and function remains unknown, and long-term, serial follow-up will be necessary. Coronary translocation remains the most technically challenging aspect of the ASO and may contribute significantly to perioperative mortality and morbidity. In this series, coronary obstruction was implicated in all three early deaths, but increased experience with coronary transfer has reduced the incidence of this complication. Because of the high incidence of complete right bundle branch block or bifascicular block after VSD closure, the ECG is of limited help in the postoperative period for diagnosing ischemia. Hemodynamic instability may be a marker for coronary compromise, and further investigation with echocardiography or angiography is indicated in these cases. Furthermore, the appearance of congestive heart failure in a previously well child after the ASO can be an indication of coronary compromise and should be investigated and treated promptly. The late implications of neonatal coronary translocation remain unknown. Asymptomatic coronary occlusion was previously documented in survivors of the ASO for TGA with IVS50 and was seen in one child in this series as well. The length of follow-up and age of this patient population prevent formal evaluation of ventricular function with stress exercise testing, and long-term follow-up and investigation is clearly necessary before final recommendations can be made. At present, we continue to recommend follow-up cardiac catheterization and angiography for this group of patients as well as serial evaluations of ventricular function. Comparison of the Arterial Switch Operation in Patients With Either Ventricular Septal Defect or Intact Ventricular Septum Children having TGA with VSD or double outlet ventricle form a heterogeneous group because of many factors, including location and size of the VSD and a variety of associated intracardiac and extracardiac defects. In contrast, children having TGA with IVS represent a much more homogeneous group of patients, and comparison of a mode of surgical repair between groups may not be warranted. Some centers have reported that the presence of a VSD is a risk factor for the ASO; however, we have found that hospital mortality is similar and late supravalvar pulmonary stenosis has been less common in patients with a VSD. This may reflect in part the "learning curve" achieved early in our experience with patients having TGA with IVS. Significant late systemic ventricular dysfunction has not been identified in either group. Other late complications such as rare sinus node dysfunction and incidences of aortic regurgitation and coronary obstruction were similar in patients with VSD and IVS.50 Pulmonary hypertension, complete heart block, and lower grade ventricular conduction abnormalities are more common in patients with a VSD than with an IVS, but are unlikely to be arterial or atrial level repair per se. Rather, the presence of a VSD may be better considered as a risk associated with TGA that is independent of the mode of surgical correction. In our experience, the increase in risk associated with a VSD is limited to the development of pulmonary vascular disease or atrioventricular conduction abnormalities and not to hospital mortality or late reoperation. Comparison of Atrial With Arterial Repair The low hospital mortality and encouraging midterm follow-up data support the notion that a primary ASO is the preferred method of repair for Di Donato et al Arterial Switch Operation for TGA With VSD Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 children having TGA with VSD or double outlet ventricle. Late complications related to the great vessel and coronary anastomoses (supravalvar or coronary obstruction and semilunar valve regurgitation) have been, for the most part, hemodynamically insignificant or very infrequent. In contrast to atrial level repairs, resting ventricular function and systemic atrioventricular valve competence have been preserved during the short-term, as documented by cardiac catheterization and (in a more limited number of patients) two-dimensional echocardiography. Complications related to VSD closure (conduction abnormalities, tricuspid or anterior semilunar valve regurgitation, and residual defects) are likely to occur with either atrial or arterial repair; however, the hemodynamic effects of tricuspid regurgitation or a right ventriculotomy are of more significance after intra-atrial repairs that rely on the tricuspid valve and right ventricle to function in the systemic circulation. Because the VSD must be closed, the ASO offers the potential for a better hemodynamic result. Importantly, sinus node dysfunction, which is so common after the Mustard or Senning operations, appears to be a rare finding after ASO. Although arrhythmias are not completely absent with the ASO, significant arrhythmias have been infrequent and limited to the immediate perioperative period. Most arrhythmias after the ASO have been transient and benign.50,65,66 Thus, low operative mortality and an infrequent incidence of significant complications at midterm follow-up lead us to recommend the ASO for children with TGA with VSD. We recognize that a longer duration of follow-up will be necessary before final comparisons of the two alternative surgical approaches may be made. Acknowledgments The authors greatly acknowledge the support and cooperation of all of the referring cardiologists, especially those in the New England Regional Infant Cardiac Program. We also thank Dr. Thomas J. Hougen for helping to establish the follow-up protocol. We are indebted to Ms. Emily Flynn McIntosh for her assistance with the artwork and figures. References 1. 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Qureshi SA, Ettedgui JA, Jones ODH: Pulmonary regurgitation in transposition of the great arteries. Br Heart J 1987;57:199-201 1705 65. Vetter VL, Tanner CS: Electrophysiologic consequences of the arterial switch repair of d-transposition of the great arteries. JAm Coll Cardiol 1988;12:229-237 66. Martin RP, Radley-Smith R, Yacoub MH: Arrhythmias before and after anatomic correction of transposition of the great arteries. JAm Coll Cardiol 1987;10:200-204 KEY WORDS * transposition of the great arteries * arterial switch operation * ventricular septal defect * pulmonary vascular obstructive disease Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 Results of the arterial switch operation for transposition of the great arteries with ventricular septal defect. Surgical considerations and midterm follow-up data. R M Di Donato, G Wernovsky, E P Walsh, S D Colan, P Lang, D L Wessel, R A Jonas, J E Mayer, Jr and A R Castañeda Downloaded from http://circ.ahajournals.org/ by guest on June 18, 2017 Circulation. 1989;80:1689-1705 doi: 10.1161/01.CIR.80.6.1689 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 1989 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539 The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circ.ahajournals.org/content/80/6/1689 Permissions: Requests for permissions to reproduce figures, tables, or portions of articles originally published in Circulation can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office. 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