Eur J Cardiothorac Surg 2008;33:239-243. doi:10.1016/j.ejcts.2007.09.041
Copyright © 2008, European Association for Cardio-thoracic Surgery. Published by Elsevier. All rights reserved.
Long-term outcomes and social independence level after arterial switch operation
Akira Yamazaki*,
Noboru Yamamoto,
Takahiko Sakamoto,
Kazuaki Ishihara,
Yusuke Iwata,
Goki Matsumura,
Hiromi Kurosawa
Department of Cardiovascular Surgery, Tokyo Women's Medical University, Japan
Received 8 July 2007;
received in revised form 11 September 2007;
accepted 27 September 2007.
* Corresponding author. Address: Department of Cardiovascular Surgery, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku, Tokyo 162-8666, Japan. Tel.: +81 3 33538111; fax: +81 3 33560441. (Email: syamaaki{at}hij.twmu.ac.jp).
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Abstract
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Objectives: Various issues regarding the long-term survivors of arterial switch operation (ASO) have been clarified according to the improvement of surgical mortality. We reviewed the long-term results and social independence level after ASO. Methods: Two hundred and four (204) patients who had undergone ASO more than 15 years ago were studied retrospectively. ASO was performed as a primary operation (group I, n
= 99) or as a secondary operation (group II, n
= 105). Lecompte procedure was performed in 197 patients, modified Aubert procedure in 5, and original Jatene procedure in 2. Results: There were 11 late deaths. Kaplan–Meier survival rate (not including operative deaths) was 94.9% at 10 years and 94.9% at 15 years in group I, and 96.9% at 10 years and 94.4% at 15 years in group II. Forty-eight reoperations were performed (aortic valve replacement in 6, aortic valvoplasty in 2, Konno procedure in 1, double valve replacement in 1, right ventricular outflow tract reconstruction in 35). The reoperation-free rate including late death was 82.2% at 10 years and 75.7% at 15 years in group I, and 88.2% at 10 years and 78.1% at 15 years in group II. One hundred and seventy-eight patients were classified as NYHA class I and 7 patients as class II. All the patients except those with mental disorder (1) or neurodevelopmental impairment (3) were attending school or working. There was no significant difference in left ventricular function between group I and II, both showing values within the normal range. Conclusions: The long-term (>15 years) outcome of ASO survivors was satisfactory. Most patients showed excellent cardiac function and were socially independent.
Key Words: Arterial switch operation • Cardiac function • Social independence level
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1. Introduction
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More than 30 years have passed since the first reported case of successful arterial switch operation (ASO) for complete transposition of the great arteries (TGA) [1]. Satisfactory results were obtained following remarkable progress in surgical procedures, extracorporeal circulation, pharmacological agents, and perioperative management. With improvement in diagnostic techniques and operative results, neonatal one-stage ASO came to be performed widely, but most of the patients operated on in the era of two-stage ASO are now surviving adults. There have been many long-term survivors, with many living a normal life.
In this study, we investigated the social independence of postoperative ASO patients, and compared those after one-stage ASO and two-stage ASO.
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2. Materials and methods
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Two hundred and forty-four (244) patients underwent ASO at our institution before August 1991. Operative death was encountered in 28 patients (11%). The study group consisted of 204 operative survivors. Cases of five re-switch, three double-switch, and four TGA with ventricular septal defect and pulmonary stenosis were excluded.
2.1 Patients’ characteristics
Of these 204 patients, 138 had TGA with an intact ventricular septum (TGA-IVS), 51 had TGA with a ventricular septal defect (TGA-VSD), and 15 had TGA-type double outlet right ventricle. Preoperative catheterization was performed in all patients and coronary patterns of all were identified with aortography or coronary angiography. The coronary pattern was divided by Shaher's classification. Coronary pattern of type I was observed in 143 patients, type II in 20, type III in 15, type IV in 14, type V in 6, and type IX in 6. Ninety-nine patients underwent one-stage ASO (group I) and 105 patients underwent two-stage ASO (group II). Changes in surgical strategy through the years are demonstrated in Fig. 1
. In group II, pulmonary artery banding (PAB) alone was performed in 21 patients, PAB and Blalock-Taussig shunt (BTS) in 80 patients, and aortic arch repair in 7 patients as the primary procedure. Patients’ age ranged from 4 days to 1.7 years and body weight was 2.1–8.6 kg in group I, versus 48 days to 6.7 years old and 3.0–20.1 kg in group II. The Lecompte procedure was performed in 197 patients, modified Aubert procedure in 5, and original Jatene procedure in 2.
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Fig. 1. Changes in surgical strategy through the years in our institution. Half-toned bars, number of one-stage ASO; solid bars, number of two-stage ASO.
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2.2 Perioperative course
Cardiopulmonary bypass (CPB) was instituted using ascending aortic perfusion and bicaval drainage. Myocardial protection was achieved by crystalloid cardioplegia. After weaning from CPB, blood pressure (left ventricular pressure) was maintained at about 80% of preoperative systemic pressure to reduce afterload by catecholamine and nitroglycerine administration. We do not usually use deep hypothermic circulatory arrest (DHCA) in ASO operation. There were 11 cases with aortic arch anomaly in this study and 8 patients who required surgical repair for aortic arch. Seven of those eight patients underwent two-stage ASO.
2.3 Follow-up
The average observation period was 35 days to 22.9 years (17.0 ± 4.3 years) in group I and 65 days to 23.8 years (18.4 ± 3.6 years) in group II. Postoperative cardiac function was evaluated by cardiothoracic ratio in the chest X-ray, and left ventricular internal diameter in diastole (LVIDd), left ventricular shortening fraction (LVSF), and right ventricular systolic pressure on echocardiography. Thirty-seven patients (37%) of group I and 35 patients (33%) of group II could be followed with echocardiography more than 15 years after ASO. Right ventricular systolic pressure (RVsP) was repeatedly estimated with tricuspid regurgitation detected by Doppler echocardiography [2] as a screening study after the operation. If elevation of RVsP was found with echocardiography, a catheter study would be scheduled.
2.4 Analysis
We retrospectively analyzed the medical records, operative notes, and echocardiographic and cardiac catheterization findings. The Kaplan–Meier estimator and log-rank test were used for survival rate, and Student's t-test and 
2-test for univariate analysis. The contribution of variables was evaluated by univariate and multivariate logistic regression analyses. For all analyses, a p-value less than 0.05 was considered significant.
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3. Results
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Only one patient underwent ASO and aortic arch repair (end-to-end anastomosis for coarctation of the aorta) simultaneously with 25 min DHCA, who had no developmental impairment. There were 11 late deaths (sudden death in 4, chronic heart failure (CHF) in 2, pulmonary hypertension in 2, infection in 2, cancer in 1). One patient of sudden death (coronary; Shaher's type I) had been identified as having asynergy of left ventricular wall motion in echocardiography and two of the patients who died of CHF (coronary; Shaher's type I and V) also showed such findings. However, those three patients had not undergone coronary angiography just before death. The survival rate was 94.9% at 10 years and 94.9% at 15 years in group I, and 96.9% at 10 years and 94.4% at 15 years in group II (Fig. 2
). In the late follow-up period, the cardiothoracic ratio in the chest X-ray and LVIDd and LVSF on echocardiography were within normal limits in both groups, and there was no significant difference in each item between groups (Table 1
). Reinterventions after ASO were revealed in Table 2
. Forty-eight reoperations were carried out, with patients with aortic regurgitation (AR) or right ventricular outflow tract obstruction (RVOTO) accounting for the greatest number. There were eight patients with more than moderate AR in group I (8%), three of them underwent reoperation for AR; one aortic valve replacement (AVR), one aortic valvoplasty (AVP), and one Konno procedure were performed. In group II, 16 patients (15%) had more than moderate AR, 7 of them underwent reoperation; 5 AVR, 1 AVP, and 1 double valve replacement were performed. For RVOTO after ASO, catheterization (balloon or stent) was performed in 38 patients (19%), who had 48–107 mmHg (60.3 ± 12.8 mmHg) pressure gradient over RVOTO, and surgical repair (right ventricular outflow tract reconstruction) was carried out in 27 patients (13%), whose pressure gradient was 50–120 mmHg (71.6 ± 20.8 mmHg). Reintervention for RVOTO (catheterization or surgical repair) would be considered when RVsP exceeded 60% of the left ventricular systolic pressure. The reoperation-free rate including late death was 82.2% at 10 years and 75.7% at 15 years in group I, and 88.2% at 10 years and 78.1% at 15 years in group II (Fig. 3
). The reoperation-free rate does not include catheter intervention. One-stage ASO was a risk factor for RVOTO (p
= 0.04).
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Fig. 2. Kaplan–Meier curve demonstrating survival rate. Numbers represent numbers of patients at risk at 5, 10, and 15 years after operation. Dotted line, group II; solid line, group I.
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Fig. 3. Kaplan–Meier curve demonstrating reoperation-free rate including the late death (not including catheter intervention). Numbers represent numbers of patients at risk at 5, 10, and 15 years after operation. Dotted line, group II; solid line, group I.
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All surviving patients were in NYHA class I or II, with 98% in class I in both group I and group II. In the late follow-up period, 86% of patients in group I and 81% of group II received medication, with no significant difference between them. The age as of August 2006 was 15–31 years old (Fig. 4
); 62% were students (university and vocational school students 28%, junior-high and high school students without physical limitation 39%, and junior-high and high school students prohibited long-distance running 5%), 20% were working (full-time 17%, part time 3%), and 8% including 2% of patients with developmental impairment were prohibited from participating in society.
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4. Discussion
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The hemodynamics of TGA-IVS is characterized by an increase in left ventricular end-diastolic volume in accordance with the progressive increase in pulmonary blood flow. At the same time, the decrease in left ventricular afterload with the physiological fall in pulmonary arterial pressure causes an increase in left ventricular ejection fraction above normal, but later produces thinning of the left ventricle wall 2 or 3 months after birth. The left ventricle, which is in charge of the systemic circulation after ASO, requires adaptation to the change from low pressure of the pulmonary circulation to high afterload of the systemic circulation. Patients with TGA-IVS after infancy, when the left ventricle pressure decreases with regression of physiological pulmonary hypertension, often present with acute left heart failure after ASO. Two-stage ASO with left ventricle training by PAB, as reported by Yacoub et al. [3,4] is one preventive option for this problem. Some cases reveal exacerbation of hypoxemia by PAB; so simultaneous BTS operation is required to maintain pulmonary blood flow in such patients. There were 80 patients in this study, who underwent the same procedure. The rapid afterload increase by PAB may be the cause of interstitial fibrosis and cardiomyopathy due to left ventricular myocardial hypertrophy, and the timing, duration, and degree of training remain controversial. In this study, no significant difference in left ventricular function in the late follow-up period was noticed between the two groups, but another study [5] showed significantly better left ventricular function in the late follow-up period with one-stage ASO; so at present, when neonatal open heart surgery is performed, one-stage ASO should be accomplished as much as possible.
The mechanism of AR is unknown, but the anatomic pulmonary valve becomes unable to stand up to the systemic pressure and/or the suture lines of coronary reimplantation and the ascending aorta causes distortion with somatic growth. AR progresses slowly and slightly, but the incidence increases with time; [6] therefore, long-term follow-up is necessary. Though the mechanism of the RVOTO progress is not yet clarified, the flattening of the main pulmonary artery with somatic growth is suspected to be one of the causes of RVOTO [7,8]. The transformation with growth occurs around the circumference of the suture line, and the development of fibrous tissue around that region may lead to a more serious condition.
The distribution of the NYHA classification in the late follow-up period was not different from that in another study [9]. Cardiac function does not necessarily accord with the clinical symptoms, and a difference in social independence level is often observed independently of clinical symptoms. In this study, there were patients in NYHA class II who could become independent. On the other hand, there were patients in NYHA class I who could not be independent. Even in the Japanese general population from 15 years old to 34 years old, 2% of youths were not in education, employment, or training. Therefore, it was supposed that poor social integration in some of the operated patients is not due to the operation but to simple lack of motivation. The social independence level is also influenced by factors other than symptoms: the effort of the patients, the environment, mainly welfare system, and so on. Not only medical and technological progress but also support to the patient and social maturity are indispensable so that postoperative patients with a complicated heart malformation are able to participate in society. The congenital cardiac surgery nowadays has more ambitious goals than just improving mortality. The long-term postoperative outcome of comparatively mild congenital cardiac disease like patent ductus arteriosus and atrial septal defect is stable, and a high degree of social independence was achieved in those patients after the early postoperative days. In the future, a similar outcome may be expected in patients after operations for complex heart malformations.
There was one patient in whom mental disorder developed (0.49%), and the prevalence was not different from that in the normal population. Three patients (1.5%) were not independent because of neurodevelopmental impairment, and the incidence was high as compared to the normal population. A long CPB time and perioperative circulatory failure are considered to be risk factors for neurodevelopmental impairment according to Hovels-Gurich et al. [10]. We think that cardiac surgeons have to cooperate more closely with pediatric cardiologists, intensivists, and clinical engineers to reduce the incidence of neurodevelopmental impairment associated with cardiac surgery.
There are several limitations to this study. First, the operative death was not considered. Therefore, the survival rate in this study appears better than the actual survival rate after ASO. Second, we did not classify the patients, who had undergone two-stage ASO, by the procedures of primary operation, and the interval length between the primary operation and the secondary operation. That may be one of the reasons why there was no significant difference between one-stage and two-stage ASO in postoperative cardiac function unlike other studies.
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5. Conclusions
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Cardiac function was well maintained in the late follow-up period, and did not show a significant difference between one-stage ASO and two-stage ASO. One-stage ASO was a risk factor for RVOTO (p
= 0.04). At more than 15 years after ASO, most patients were independent socially.
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Appendix A
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Conference discussion
Dr R. Pretre (Zurich, Switzerland): In the patients with social dependency, do you know if deep hypothermic circulatory arrest was used more often, or if the patients had associated VSDs more often compared to the others?
Dr Yamazaki: Sorry, once more, please.
Dr Pretre: Were the patients with social dependence operated on with deep hypothermic circulatory arrest more often than those with a better socio-neurological outcome?
Dr Yamazaki: We usually did not use circulatory arrest, but one patient had aortic arch coarctation and we used deep hypothermic circulatory arrest.
Dr M. Masuda (Yokohama, Japan): I would like to ask only one question about the cause of the sudden deaths. We also had some cases of sudden death, and when we saw the post-angiogram for the coronary arteries, we noticed that the left anterior descending artery was quite short compared with the normal population and we assumed that a coronary event might be the cause of sudden death after the arterial switch operation. Do you have any ideas or comments on this?
Dr Yamazaki: Coronary problems are very important, but we had no evidence of sudden death from coronary problems in this study. In the sudden death patients, two patients had echocardiographic asynergy. Perhaps they had some coronary troubles before death.
Dr J. Stark (London, UK): Dr Sidi, you have a very large experience with restudied patients for coronary problems. Could you comment on the relationship between coronary problems and sudden death?
Dr D. Sidi (Paris, France): 1000 patients had been switched and about 800 had coronary artery angiograms. Our experience is that we had some deaths in the 4 months after the switch that were related to coronary abnormalities. Otherwise we had no sudden deaths in our whole cohort, surprisingly. No patient died after 6 months after the switch. Despite that, there were about 2% of the patients who had coronary artery abnormalities that were detected. About 30 had coronary patch and there were no late deaths. There were no sudden deaths in this cohort after 6 months after the switch.
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Footnotes
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\#9734; Presented at the 21st Annual Meeting of the European Association for Cardio-thoracic Surgery, Geneva, Switzerland, September 16–19, 2007.
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References
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Abstract
1. Introduction
