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Ann Thorac Surg 2007;83:1463-1471
© 2007 The Society of Thoracic Surgeons
a Department of Cardiovascular Surgery, German Heart Center Munich, Technical University Munich, Munich, Germany
b Department of Paediatric Cardiology and Congenital Heart Defects, German Heart Center Munich, Technical University Munich, Munich, Germany
Accepted for publication November 1, 2006.
* Address correspondence to Dr Hörer, German Heart Center Munich, Dept. of Cardiovascular Surgery, Lazarettstr. 36, Munich, D-80636 Germany (Email: hoerer{at}dhm.mhn.de).
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Abstract |
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Methods: Records of 56 patients with ALCAPA, operated between 1977 and 2002, were reviewed retrospectively. Patients were divided into two groups: subclavian artery anastomosis group (n = 25), and coronary transfer group (n = 31).
Results: Concomitant mitral valve repair was performed in one patient. Thirty-day mortality was 14.3% (subclavian artery anastomosis group, n = 3; coronary transfer group, n = 5). Preoperative age (p = 0.007) and left ventricular ejection fraction (LVEF) less than 0.35 (p = 0.020) were predictive for 30-day mortality, whereas preoperative mitral regurgitation, surgical technique, and cardiopulmonary bypass time were not. For both groups, LVEF (p = 0.006) and mitral regurgitation (p = 0.013) were improved at the time of hospital discharge. Mean follow-up was 11.0 ± 7.2 years. Survival of hospital survivors at 20 years was 94.8 ± 3.6%. At final follow-up, 95.5% of the patients presented with a LVEF greater than 0.50, and 84.1% with a mitral regurgitation less than grade 2. Mitral valve replacement was performed in three patients.
Conclusions: In the majority of patients with ALCAPA, both ventricular function and mitral valve regurgitation normalize over time. Concomitant mitral reconstruction may not be required upon initial repair. Coronary transfer is the technique of choice today. However, subclavian artery anastomosis may remain an option in selected cases.
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Introduction |
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TopAbstractIntroductionPatients and MethodsResultsCommentReferences |
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Different surgical approaches have been suggested, including ligation of the anomalous left coronary artery [3], various forms of bypass grafts from the aorta [4, 5], subclavian-left coronary artery anastomosis [6], transpulmonary baffling or Takeuchi procedure [7], and direct reimplantation of the coronary artery into the aorta [8]. Currently, immediate surgical correction upon diagnosis with the aim of restoring a two-coronary circulation system is considered the standard treatment for patients with ALCAPA.
Improvement of left ventricular function after successful reestablishment of a two-coronary system was uniformly documented [9, 10]. However, mitral valve reconstruction upon the initial repair has been discussed controversially. While some authors recommended concomitant mitral annuloplasty with the initial repair [11, 12], others clearly demonstrated that mitral valve function generally improves after ventricular remodeling without any intervention [13, 14].
Only a few long-term studies on large collectives are available, and different techniques for repair have not yet been evaluated with respect to the fate of ventricular and mitral valve functions. Hence, we compared the long-term results after either coronary transfer or subclavian anastomosis based on our single-center experience.
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Patients and Methods |
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TopAbstractIntroductionPatients and MethodsResultsCommentReferences |
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Data Collection
The clinical records were reviewed to document the clinical features, operative procedures, and the perioperative course. The latest echocardiographic examination was reviewed by one of the authors. Ventricular function was classified as normal or mildly impaired (ejection fraction [EF] > 0.50), moderately impaired (EF 0.35 to 0.50), and severely impaired (EF < 0.35). Ventricular function was assessed in M-mode in the parasternal long and short axis view. Mitral regurgitation (MR) was evaluated with Doppler echocardiography (0 = no MR; 1 = mild MR; 2 = moderate MR; 3 = severe MR; 4 = severest MR).
The composite endpoint was defined as left ventricular EF (LVEF) less than 0.50, MR grade 2 or more, or death after hospital discharge after the initial operation.
Statistics
Descriptive data for continuous variables are presented as means ± standard deviation or as medians with ranges; categoric variables are presented as relative frequencies. The Fischer exact test or Mc Newar test was performed to detect significant differences between groups. For comparison of continuous variables between two groups, the t test was used (two-tailed tests were used for all analyses). The probability of freedom from events was estimated according to the Kaplan-Meier method. Freedom-from-events curves were compared by means of the log-rank test. Values of p less than 0.05 were considered as statistically significant. According to Concato and colleagues [15] multivariate analysis could not be performed because the ratio of events per variable was too small. Analyses were performed with SPSS 12.0.2 for Windows (SPSS Inc, Chicago, IL).
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Results |
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TopAbstractIntroductionPatients and MethodsResultsCommentReferences |
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Surgical Technique
The subclavian-left coronary artery anastomosis was performed in an end-to-end fashion after excision of the coronary button from the pulmonary artery. Coronary transfer was performed by mobilization of the button, and creation of a trap-door flap in the aorta. Techniques of coronary elongation [16, 17], such as described by Sese and Imoto [18], were also used, creating an inferior wall flap and a superior pulmonary artery flap. The pulmonary artery was closed directly, or reconstructed with glutaraldehyde-fixated autologous pericardium or Gore-Tex patch material (Gore-Tex, Inc, Flagstaff, AZ), respectively.
Simultaneous mitral annuloplasty was performed in one patient, in whom the morphology of the mitral valvar apparatus was pathologic (distinct prolapse of the anterior leaflet). The patient was four-months old at the time of operation, and was treated with a Whooler annuloplasty and additional shortening of anterior leaflet chordea.
Operations were performed on cardiopulmonary bypass in 52 patients (93%). The aorta was clamped in 28 patients (50%). Crystalloid cardioplegia was administered simultaneously in both great vessels after cross-clamping and after snaring of both pulmonary arteries. In the subclavian artery anastomosis group, cardiopulmonary bypass time (p = 0.034) and cardiac arrest time (p < 0.001) were shorter than in the coronary transfer group (Table 1). Sixteen percent of the patients in the subclavian artery anastomosis group were operated without cardiopulmonary bypass.
Postoperative Data
In both groups, LVEF (p = 0.006) and MR (p = 0.013) improved from preoperative to the time of hospital discharge (Figs 1; 2). There was no significant difference in the fate of left ventricular function and MR between the subclavian artery anastomosis group and the coronary transfer group (Table 1).
Early Mortality
Thirty-day mortality was 14.3% for both groups, with three deaths in the subclavian artery anastomosis group, and five deaths in the coronary transfer group, respectively. There was no mortality in the last 13 years (17 patients operated). Preoperative age (p = 0.007) and LVEF less than 0.35 (p = 0.020) were predictive for 30-day mortality (Table 2). Preoperative MR, surgical technique, cardiopulmonary bypass time, and cardiac arrest time did not influence 30-day mortality.
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Late mortality was 3.3% (two patients). One patient (coronary transfer group) with an extremely rare morphology, a single-trunk anomalous origin of both coronary arteries, died four months after coronary transfer during a reoperation. One patient (subclavian artery anastomosis group) died eight years after initial repair due to malignant arrhythmias. This patient died three weeks after her last outpatient visit. She was clinically in heart failure due to severe MR and therefore had ventricular extrasystoly with couplets and triplets (on 24-hour Holter monitoring). On last echo, there were no signs for myocardial failure (EF 0.65). Thus, it seems to be obvious that she had ventricular tachicardia as the most obvious reason for sudden cardiac death. Estimated survival of hospital survivors at 20 years was 94.8 ± 3.6%.
Three patients presented during follow-up with an EF less than 0.50, with no evidence of impaired coronary circulation upon angiography. At final follow-up one of these patients presented in New York Heart Association class I (NYHA I). Two patients are symptomatic, one is in NYHA II, with tachypnea on exertion, and one suffers from severely impaired ventricular function, remaining currently in NYHY II-III. Accordingly, freedom from more than mildly impaired ventricular function at 20 years was 81.5 ± 11.7%. At final follow-up, 95.5% of the patients presented with a LVEF greater than 0.50 (Fig 1).
Seven patients exhibited moderate or severe MR during the follow-up period. Three of these underwent mitral valve replacement: One patient (age, 14 months) underwent two attempts for reduction of mitral valve insufficiency one year postoperatively due to a hugely dilated annulus and significant prolapse of the anterior leaflet, and finally had valve replacement (St Jude, size 21 mm; St Jude Medical, Inc, Minneapolis, MN) the same year. One patient (age, 17 months) had mitral valve replacement (size 23 mm; St. Jude Medical, Inc) after 13 months. One patient (age, 4 years) had aortic valve replacement with a homograft and mitral valve replacement (size 25 mm; CarboMedics, Inc, Austin, TX) after one month, and subsequent aortic valve replacement (size 16 mm; ATS Medical, Inc, Minneapolis, MN) after five years. Freedom from MR greater than grade 1 at 20 years was 85.7 ± 5.4% (Fig 3). Three additional patients were reoperated. One patient (age, 12 years) had aortic valve replacement (size 23 mm; CarboMedics) five years after initial repair. Pulmonary artery augmentation was performed in two patients (age, 7 and 4 years) after three and five years, of which both had had previous subclavian anastomoses. Both patients had primary closure of the pulmonary artery. Freedom from reoperation at 20 years was 81.0 ± 6.2%, with no difference between the subclavian artery anastomosis group and the coronary transfer group.
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respectively. Currently, four patients are on angiotensin-converting enzyme inhibitors, two of whom are on a combination with digitoxin. Two patients are on ß-blocker therapy and two on amiodarone.
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Comment |
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In the present study, the subclavian artery anastomosis and coronary transfer technique were investigated. We thought to ascertain if both techniques were equally effective in the long-term recovery of left ventricular and mitral valve function.
Most patients with ALCAPA present with symptoms of severely impaired left ventricular function and mitral valve regurgitation. Left ventricular damage resulting from myocardial ischemia in patients with ALCAPA is associated with a range of pathologic findings. These include endocardial and subendocardial fibrosis, damage to the papillary muscles, patchy myocardial necrosis, and dilatation of the ventricle [22]. As a consequence, mitral incompetence frequently develops [23, 24]. In the present study, 62.5% of the patients exhibited a preoperative EF below 0.35, and 37.5% showed moderate to severe MR.
Various risk factors for operative survival have been identified in the treatment of ALCAPA, including the preoperative impairment of LV function [25–27] and young age at operation [27]. In the present study, both parameters were also found to be significant predictors for 30-day mortality. Because younger patients presented with significantly lower LVEF, we believe that small size and low age at the operation are markers for earlier clinical manifestation of myocardial malperfusion. This might be explained by the original anatomy: patients with good collateralization might be asymptomatic for a long time and become evident only by a cardiac murmur. Patients with little collateralization become symptomatic early in life, with cardiac failure due to ischemic heart lesions.
The severity of preoperative MR has also been identified as a risk factor for survival after surgery [28], although this is not supported unanimously [8, 29, 30]. Looking at our patients, it could not be identified as a risk factor for the 30-day mortality. Based on our experience, we have developed the policy not to address MR by repair or replacement at the initial operation. In ALCAPA, even severe mitral insufficiency has been reported to fully regress after reperfusion alone in the majority of cases [14, 31]. In the present study, mitral valve regurgitation had already improved significantly by the time of hospital discharge when compared with the preoperative finding. Furthermore, freedom from MR greater than grade 1 at 20 years was 85.7 ± 5.4%. Thus, the primary goal in surgery should be coronary reperfusion and myocardial salvage. Presumably, the added ischemic time to perform mitral valve precedures in the setting of severely compromised ventricular function, is potentially more deleterious than helpful [13, 14]. Furthermore, the results from surgical treatment of ischemic MR in adults are relatively disappointing [32], and "overcorrection" of mitral annulus diameter is not an option in children. Other techniques for correction of ischemic regurgitation, such as Whooler annuloplasty, artificial chordae, chordal shortening, and others may not be applicable in newborns and infants. Thus, the policy of not approaching the mitral valve upon the initial operation, but rather wait for ventricular remodeling to ensue, is also advocated by many others [8, 11, 16, 33]. Early postoperative mild-to-moderate mitral insufficiency is usually acceptable [8, 11, 16] and, after improvement of left ventricular function, persistently symptomatic or hemodynamically significant MR may be treated at a later time [8, 13, 34, 35]. Interestingly, our data showed that mitral function recovers completely in the early postoperative period in most patients, In contrast, ventricular function recovered incompletely during hospital stay, but improved further in the long term. An opposing finding has been reported by Azakie and colleagues [36]. The authors found that improvements in MR lagged behind normalization of EF and left ventricular dilatation. In our experience, the first phase of ventricular remodeling after ischemic dilatation of the left ventricle is a reduction in left ventricular diameters that can be measured on M-mode. When the left ventricle becomes smaller ventricular systolic and diastolic function improves. These geometrical and functional changes (smaller mitral valve orifice, less stroke volume, and better cardiac output) might contribute to an improvement in MR.
Some authors [12], however, recommend routine mitral valve repair at the time of coronary artery revascularization on the grounds that early postoperative cardiac output is improved and operative mortality reduced. It remains speculative whether their excellent results would have been achieved without associated mitral valve procedures. Others advocate mitral annuloplasty [11, 29, 37] or mitral valve replacement [37, 38] only in the presence of severe MR. In our patients, the mitral valve was addressed during the initial repair in only one patient, who presented with associated mitral valve pathology not related to ischemia. However, in the long term, three patients needed additional treatment for persistent severe mitral valve regurgitation, and the mitral valve eventually was replaced in all cases. Hence, postoperative mitral incompetence was identified as a risk factor for reintervention, but not for death.
Mechanical assist devices or extracorporeal membrane oxygenation were not used in any of our patients. The question of the need for mechanical circular support in patients undergoing ALCAPA repair is addressed in a series of 16 patients corrected by direct aortic reimplantation [33]. These authors state that ventricular support systems may only be necessary in very few selected cases if ventricular function does not improve early postoperatively, despite adequate coronary revascularization. Using repeated antegrade crystalloid cardioplegia with simultaneous administration to both great vessels, combined with appropriate postoperative inotropic support, may allow for favorable results in the majority of the cases, without the need of mechanical circulatory support. Since 1992, no patient (n = 17) has died at our institution applying this strategy. Because of excellent results worldwide, we also do not think that concomitant resection of left ventricular muscle during initial surgery, as has been proposed in the past, is still justified today [8, 17, 26].
At present, coronary reimplantation is the technique of choice for repair of ALCAPA and may be performed in almost every case [16]. The present study confirms excellent perioperative and long-term results with this technique, which has also been reported by others [14, 28]. Our approach toward coronary transfer, like that of other groups, has evolved over time, aided by increasing confidence in the arterial switch procedure. Several methods have been described for lengthening of the coronary pedicle, such as creating an inferior wall flap and a superior pulmonary artery flap facilitating coronary transfer in complicated cases [16–18]. Very rarely, the Takeuchi tunnel technique may also still be appropriate, if the coronary ostium lies extremely lateral and so close to the pulmonary valve commissure that excision may not generate sufficient arterial wall to support coronary transfer. In the present study group, the subclavian artery anastomosis was preferentially performed in patients with a lateral postion of the ostium of the left coronary artery (10 of 12 patients). The surgeons might have seen a problem in translocating the coronary artery to the aorta. However, with the growing experience in coronary transfer by the arterial switch operation, presently, coronary transfer is considered to be possible in almost all of the cases, at least in neonates and small children.
Half of the patients of the present study population were operated before 1988. Two-thirds of them received a subclavian artery anastomosis, whereas only two-fifths of the patients who were operated thereafter received a subclavian artery anastomosis. At the time when surgeons were not yet as acquainted with the coronary reimplantation technique from the arterial switch procedure, the subclavian artery anastomosis seemed to be a safe alternative, especially in small children. Cardiopulmonary bypass time was shorter and, according to our experience, 16% of the patients could even be operated on without cardiopulmonary bypass. The present investigation revealed no difference between the two revascularization techniques for any parameter studied. To our knowledge, only one other report exits about the long-term results (average 10-year follow-up) of the subclavian artery to coronary artery anastomosis in seven infants with ALCAPA [27]. Similar to the findings of the present investigation, the authors report on good exercise tolerance and normalization of ventricular function. Hence, although the subclavian artery to coronary artery anastomosis is no longer performed, this technique yielded the same results as the now generally accepted coronary transfer technique. Our current series with this technique is, to our knowledge, the largest reported to date with the longest follow-up, showing that it was, in the past, a valid option for repair of ALCAPA; especially as it could be performed without cardiopulmonary bypass or temporary aortic occlusion.
In conclusion, our findings show that patients with ALCAPA surviving the perioperative period have an excellent prognosis for both immediate improvement of mitral valve function and functional recovery of the left ventricle in the long term. Because preoperative impairment of ventricular function turned out as a risk factor for operative mortality in our analyses, we suggest that patients with ALCAPA should undergo surgery as soon as the diagnosis is confirmed, in order to salvage myocardial tissue, regardless of the finding that age at repair was revealed also as a risk factor. In most cases, the mitral valve can savely be approached at a later time. Left subclavian artery to coronary artery anastomosis and direct implantation of the left coronary artery were equally effective surgical techniques.
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TopAbstractIntroductionPatients and MethodsResultsCommentReferences |
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