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Ann Thorac Surg 2003;75:1535-1541
© 2003 The Society of Thoracic Surgeons
a Departments of Surgery and Pediatrics, Divisions of Division ofCardiovascular Surgery, Toronto, Ontario, Canada
b Division of Cardiology, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, Ontario, Canada
c Department of Surgery and Pediatrics, Division of Pediatric Cardiac Surgery, University of California, San Francisco, San Francisco, California, USA
Accepted for publication November 11, 2002.
* Address reprint requests to Dr Williams, Division of Cardiovascular Surgery, Hospital for Sick Children, 555 University Ave, Room 1525, Toronto, Ontario M5G-1X8, Canada.
e-mail: azakiet{at}surgery.ucsf.edu
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Abstract |
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METHODS: From January 1952 to July 2000, 67 patients presented with anomalous coronary artery from the pulmonary artery. Forty-seven patients who had repairs performed by aortic reimplantation are the subject of this study. The median age at repair was 7.7 months. Before repair, 10 infants (21%) presented in extremis requiring ventilatory and inotropic support, and 38 infants (80%) presented in heart failure. Autologous pericardial hood coronary arterioplasty was used in 4 patients, and concomitant mitral valve repair was used in 1 patient.
RESULTS: Hospital survival was 92%. Five children required postoperative extracorporeal membrane oxygenation for a median of 4 days (range, 2 to 8 days). Patients who had extracorporeal membrane oxygenation were significantly more likely to have presented in critical condition (40% vs 3% if no extracorporeal membrane oxygenation; p = 0.006) or with ventricular arrhythmias (67% vs 7%; p = 0.027), to have presented with significantly lower preoperative repair median ejection fraction (10%, n = 5 vs 40%, n = 38; p = 0.01) or to have presented with more severe left ventricular dilatation (p = 0.03). Within a 15-year or less follow-up (mean, 4.7 years) there were no late deaths. Kaplan-Meier survival was 91% at 5 years, and freedom from reoperation was 93% at 10 years. At late follow-up, echocardiography demonstrated significant improvements in mean ejection fraction (64% ± 9% vs 33% ± 21% preoperatively, p < 0.0001); moderate mitral regurgitation (9% vs 38% preoperatively, p < 0.02); and wall motion abnormalities (15% vs 81% preoperatively, p < 0.002). The ratio of measured left ventricular end-diastolic dimension to the 95th percentile of normal declined from 1.4 ± 0.3 to 1.0 ± 0.1 (p < 0.0006). Children who had extracorporeal membrane oxygenation had normal ejection fractions and ventricular dimensions at follow-up (n = 3). Repeated measures of mixed linear regression analysis demonstrated that normalization of ejection fraction and left ventricular function occurred within 1 year of repair. Improvements in mitral regurgitation lagged behind normalization of ejection fraction and left ventricular dilatation.
CONCLUSIONS: Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation yields excellent early survival and late functional outcomes even in critically ill infants.
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Introduction |
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Surgical repair to establish a two coronary system is indicated. Recent reports of good outcome favor either aortic reimplantation or intrapulmonary baffling techniques for repair [3–11]. The postoperative course is variable and may require a period of left ventricular support to allow for recovery of both stunned and hibernating myocardium [12]. Midterm and long-term ventricular function and clinical status improve over the ensuing months [3, 4].
Increased experience with the arterial switch operation has made mobilization of the left coronary artery and reimplantation into the aortic root the favored technique for establishing a two coronary system. Although the Takeuchi repair for ALCAPA allows in situ rerouting of the left coronary artery, intrapulmonary baffling may result in supravalvar pulmonary stenosis, baffle leaks, tunnel stenosis, and compromised long-term functional outcomes of patients with ALCAPA [3]. Assessments of late ventricular function and size, and exercise capacity of patients with ALCAPA managed by aortic reimplantation are limited [4]. The purpose of this study is to determine the early and late outcomes of all patients presenting with ALCAPA who had repair by the aortic reimplantation technique. Factors associated with mortality, need for postoperative ventricular support, and need for reoperation are defined. Serial and late assessment of ventricular function and size, status of the mitral valve, and functional capacity of patients undergoing physiologic surgical correction of ALCAPA by aortic reimplantation are evaluated.
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Patients and methods |
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Electrocardiographic analysis (n = 44) demonstrated myocardial ischemia in 32 patients (anterolateral in 25 and posterolateral in 7), combined with gross evaluation at the time of surgery, myocardial infarction was confirmed in 35 patients (anterolateral wall, n = 13; lateral wall, n = 10; apex, n = 6; posterolateral wall, n = 5).
Preoperative echocardiographic data
Median preoperative ejection fraction (n = 32) was 33% (range, 7% to 73%). Ten patients (23%) had a preoperative ejection fraction less than 20%. Mean preoperative left ventricular end-diastolic diameter (n = 36) was 4.0 cm ± 0.73 and when indexed to the 95th percentile of normal it was 1.4 cm ± 0.3 (range, 0.8 to 2.2). Preoperatively, the degree of mitral valve regurgitation was 0 in 1 patient (2%), mild in 18 (43%), mild to moderate in 7 (17%), moderate in 11 (26%), moderate to severe in 3 (7%), and severe in 2 (5%). Endocardial fibroelastosis as assessed by echocardiography (n = 39) was present in 36 patients (92%). Aneurysm formation or mural dyskinesis was evaluated in 27 patients and present in 13 (48%). Left ventricular wall motion abnormalities were evaluated in 32 patients and included 0 wall motion abnormalities in 6 patients (19%); isolated anterior wall hypokinesis in 2 (6%); isolated lateral wall hypokinesis in 1 (3%); apical hypokinesis in 4 (13%); global hypokinesis in 12 (37.5%); postero-septal hypokinesis in 2 (6%); posterolateral hypokinesis in 1 (3%); anterolateral and posterior wall hypokineis in 1 (3%); posterior, anterior and apical wall hypokinesis in 1 patient (3%); and anterolateral hypokinesis in 2 (6%). The mean preoperative diameter of the right coronary artery (n = 16) was 3.1 ± 1.2 mm (range, 1.9 to 6 mm). The mean preoperative diameter of the left coronary artery (n = 7) was 2.5 ± 1.4 mm. The mean ratio of the right coronary artery to left coronary artery (n = 7) was 1.32 ± 0.4.
Morphology and associated diagnoses
Of the 47 patients who had undergone aortic root reimplantation, the anomalous left coronary artery originated from the left posterolateral sinus (sinus 1) of the pulmonary artery in 43 patients, the right posterolateral sinus (sinus 2) in 1, and the right pulmonary artery in 2 [13]. One patient had an anomalous circumflex coronary artery from the pulmonary artery that was managed by aortic reimplantation. Associated diagnoses in the reimplantation group included ventricular septal (n = 2), patent ductus arteriosus (n = 1), atrial septal defect (n = 1), pulmonary stenosis (n = 1), anomalous right subclavian artery from the descending aorta (n = 1), and Smith-Lemli-Opitz syndrome (n = 1).
Exercise testing and radionuclide studies
Exercise testing or stress myocardial perfusion, or both, were performed in 19 patients. Nineteen exercise studies were performed using a modified Bruce protocol treadmill test (n = 16) or a bicycle ergometer test with a ramp protocol (n = 3). Continuous electrocardiographic monitoring, peak heart rate, and blood pressure, and duration of exercise were recorded. For the cycle ergometer test, maximal workload and maximal oxygen consumption were also measured. Twenty stress myocardial perfusion studies were performed in 11 patients using thallium-201 (n = 4) or technetium-99mSestamibi (n = 16) to identify myocardial perfusion defects. Inadequate duration of exercise for age, a hypotensive response to exercise, or segment elevation changes (> 2 mm of J point depression with a relatively flat or downsloping ST segment in three consecutive beats) were considered positive exercise results.
Operative technique and strategy
Standard cardiopulmonary bypass was achieved using ascending aortic and either bicaval or two stage venous cannulation with left ventricular venting through the right superior pulmonary vein and moderate hypothermia. Deep hypothermia with circulatory arrest was used in 4 patients. Upon institution of cardiopulmonary bypass, the right and left pulmonary arteries were snared to avoid runoff of coronary blood flow into the pulmonary artery (n = 28) or the aorta was immediately cross-clamped (n = 14), and cold blood cardioplegia was simultaneously delivered into the aortic root and anomalous left coronary artery (either through its orifice or through the root of the main pulmonary artery). Myocardial protection was instituted by either warm or cold blood cardioplegia through the aortic root in all patients and supplemented by delivery into the pulmonary root in 10 patients, into the orifice of the anomalous left coronary artery in 11 patients, or retrograde cardioplegia was instituted through the coronary sinus in 5 patients, or a combination thereof. Complete transection of the pulmonary artery was followed by excision of a large button of pulmonary artery, extensive mobilization of the anomalous left coronary artery to its branch point, and reimplantation into the aortic root with an aortic based flap. Reimplantation was facilitated by interposition grafting using saphenous vein (n = 2) or polytetrafluoroethylene (n = 1) early in the experience. A hood of autologous pericardium was used in 4 patients to avoid kinking and stretching of the left coronary artery. The pulmonary artery was reconstructed with autologous pericardium in 42 patients and was primarily repaired in 5. In addition to reimplantation of the anomalous left coronary artery into the aorta, other procedures were performed in 7 patients including ligation of a patent ductus arteriosus (n = 2), closure of the ventricular septal defect (n = 2) or the atrial septal defect (n = 1), a pulmonary valvotomy (n = 1), and a mitral valve annuloplasty for severe mitral regurgitation (n = 1). Sternal closure was delayed in 7 patients.
The median time of cardioplegic cardiac arrest was 45 minutes (range, 17 to 133 minutes). The median cardiopulmonary bypass time was 105 minutes (range, 42 to 300 minutes).
An extracorporeal membrane oxygenation (ECMO) circuit [14] (Biomedicus portable bypass system; Minimax plus oxygenator; Avecor cardiovascular oxygenators, Carmeda; minimax and maxima oxygenator, Carmeda-coated tubing, Medtronic, Canada) was used for mechanical circulatory support in 5 patients in which multiple attempts to wean from cardiopulmonary bypass failed after prolonged reperfusion was encountered. Right and left atrial venous and ascending aortic cannulation was used in all patients.
Statistical methods
Data are described as frequencies, medians with ranges, and means with standard deviations. With missing data, the number of non missing values is given. Analysis focused on patients who had reimplantation. Characteristics and outcomes of patients in the preoperative period versus those at last follow-up were compared using Fisher’s exact tests, McNemar’s test, 
2 tests, Mantel-Haenszel 2 tests, t tests, and Kruskal-Wallis analysis of variance as appropriate. Estimates of time-related survival and freedom from reoperation after reimplantation were calculated using the Kaplan-Meier method.
Factors associated with postimplantation mortality, need for left ventricular support, time to extubation from mechanical ventilation, discharge from the intensive care unit, or development of postoperative ventricular arrhythmias were sought in Cox’s proportionate hazard modeling or stepwise multiple logistic regression, with patients censored at the time of death as appropriate. The variables assessed as possible predictors of these outcomes included age, sex, weight, body surface area, preoperative symptom severity and duration, initial presentation with ventricular arrhythmia, preoperative infarction or ischemia by electrocardiography, preoperative ejection fraction, left ventricular end-diastolic diameter, severity of mitral regurgitation, presence of associated diagnoses, date of ALCAPA repair by aortic reimplantation, and aortic cross-clamp time. Need for ventricular assistance was also used as a variable assessed as a predictor of late outcomes.
To determine the time course for recovery of left ventricular functional variables and factors, repeated measures of mixed linear regression analysis was performed. All analyses were performed using SAS statistical software (Versions 7 and 8; SAS Institute, Inc, Cary, NC) using default settings. A p less than 0.05 was set as the level of statistical significance.
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Results |
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ECMO
After coronary reimplantation, 5 patients had circulatory support using ECMO caused by failure to wean from cardiopulmonary bypass (n = 4) or cardiogenic shock in the intensive care unit (n = 1).
Factors associated with postreimplantation use of ECMO were sought. Patients who had ECMO were significantly more likely to present in critical condition (40% vs 3% if no ECMO; p = 0.006) or with ventricular arrhythmias (67% vs 7%; p = 0.027). Median preimplantation ejection fraction was significantly lower (p = 0.01) for patients who had ECMO (10%; range, 7 to 20%; n = 5) versus those who did not (40%; range, 9 to 73%; n = 38). The mean preimplantation ratio measured to the 95th percentile of predicted left ventricular end-diastolic diameter was significantly higher (p = 0.027) for patients who had ECMO (1.74 ± 0.27; n = 3) versus those who did not have ECMO (1.34 ± 0.28; n = 33). No other preoperative or intraoperative characteristic was significantly associated with postreimplantation use of ECMO.
Four of 5 children requiring ECMO survived, and three were available for midterm follow-up (2.6 ± 0.5 years). All 3 patients had normal left ventricular ejection fractions and end-diastolic dimensions.
Resource use
Sternal closure was delayed in 7 patients (15%) for a median duration of 2 days (range, 1 to 3 days). Median duration of ventilatory support after reimplantation (n = 41) was 3.5 days (range, 0.5 to 30 days). Inotropic support was used in 31 patients (1 inotrope, n = 18; 2 inotropic agents, n = 4; 3 inotropic agents, n = 8; 4 inotropic agents, n = 1) for a median duration of 4 days (range, 1 to 14).
The development of postoperative ventricular arrhythmias necessitating lidocaine infusions was observed in 13 patients (n = 42). By stepwise multiple logistic regression analysis only critical condition (ie, requiring ventilatory and inotropic support) at the time of presentation and prolonged duration of cardiopulmonary bypass were significantly associated with need for postoperative ventricular antiarrhythmics. Initial clinical presentation with a ventricular arrhythmia was not associated with the need for ventricular antiarrhythmics in the postoperative period.
Median duration of stay in the intensive care unit (n = 41) was 5.5 days (range, 1 to 33 days). Factors associated with postreimplantation stay in the intensive care unit were sought. In Cox’s proportionate hazard modeling, age at operation and initial presentation in critical condition were significantly associated with a prolonged duration of stay in the intensive care unit. After controlling for these variables, no other variable was significantly associated with intensive care unit length of stay.
The median duration of hospital length of stay after repair (n = 44) was 16 days (range, 12 to 48 days).
Late survival
The mean duration of follow-up was 4.7 ± 4.4 years (including date of death [n = 4] and date of hospital discharge for patients lost to follow-up [n = 3]). There were 43 hospital survivors, 36 of whom were in New York Heart Association functional class I, 3 who were in class II, and 1 who was in class III. There were no late deaths. Kaplan-Meier survival was 91% (95% confidence interval, 84% and 98%) at 1 month, 1 year, and 5 years(Fig 2).
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Time course for recovery of left ventricular functional factors
Normalization of ejection fraction and left ventricular dilatation, improvement in mitral regurgitation, and wall motion abnormalities occurred within 1 year of repair. By mixed linear regression analysis, ejection fraction normalized by 3.5 months (Fig 4A).
Left ventricular dilatation normalized by 4 months (Fig 4B), and the degree of mitral regurgitation improved to mild or less by 7.5 months after repair (Fig 4C).
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Of the 20 perfusion studies (11 patients) there were positive tests in 6 patients all demonstrating reversible perfusion defects in the anterior or anterolateral left ventricular wall. Two patients with small defects who were asymptomatic continue to be followed without intervention. In 1 patient, cardiac catheterization and coronary angiography did not reveal any gross abnormality in the coronary perfusion. One patient had a positive perfusion scan develop 8 years after an initially negative study. This patient underwent reoperation for patch repair of the reimplantation site after coronary angiography. Repeat perfusion scanning after surgery showed no perfusion abnormality. Another patient who progressed from a negative to a positive scan accompanied by echocardiographic evidence of decreased left ventricular function underwent coronary bypass surgery. Postoperatively, perfusion scanning was negative. The final positive test was a nonreversible defect that was investigated by a cardiac catheterization demonstrating normal coronary angiography. Two subsequent scans in this patient have been negative.
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Comment |
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TopAbstractIntroductionPatients and methodsResultsCommentReferences |
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Use of ECMO
In those moribund infants with severe myocardial ischemia and ventricular dysfunction, the use of postoperative circulatory support has a positive impact on survival. We have used ECMO in 5 children who could not be weaned from cardiopulmonary bypass, in which we had 4 survivors. In 3 of 5 children that were available for follow-up, ventricular function and size were normal. These infants presented in critical condition, requiring ventilatory support and inotropic agents for cardiogenic shock. Use of circulatory support in the postoperative period ranged from 0% to 25%. Other authors have confirmed that the use of left ventricular assist devices after repair of ALCAPA in children who cannot be weaned from cardiopulmonary bypass can be a lifesaving maneuver with good long-term recovery [3, 4]. del Nido and colleagues [12] reported 31 children with ALCAPA, 7 of whom required left ventricular assist device support postoperatively, 5 of whom were survivors.
ECMO use rather than left ventricular assist device support has the advantage of providing right ventricular support. The right ventricle is certainly at risk for myocardial injury because of shunting of the right coronary arterial blood to pulmonary circulation. Right ventricular dysfunction may also be related to mitral regurgitation and pulmonary hypertension. In all patients who had ECMO, a left atrial cannula was inserted.
Backer and colleagues [11] have reported excellent results after ALCAPA repair without the use of assist devices. They point out that careful management of cardiopulmonary bypass and conscientious delivery of cardioplegia are necessary to avoid further shunting of coronary blood flow and myocardial ischemia. Their avoidance of the use of assist devices may in fact be a result of their surgical strategy, but may also relate to the preoperative condition of the child. In our experience, circulatory support after repair was more likely in children who were critically ill at presentation, or with poor ejection fractions, severe left ventricular dilatation, or ventricular arrhythmias.
Fate of the mitral valve
The management of the regurgitant mitral valve at the time of ALCAPA repair is controversial and variable [3–5, 7, 10]. As myocardial ischemia, ventricular dilation, and papillary muscle dysfunction improve after repair, associated mitral regurgitation may also improve [3]. Vouhe and colleagues [5] suggested that the mitral valve should not be addressed at the time of ALCAPA repair, and that clinically important regurgitation will persist in only a few patients requiring later operation. On the contrary, the group from Melbourne [4] reported that 44% of patients had minimal or no improvement in mitral regurgitation after surgical repair of ALCAPA, and one third of patients had significant mitral regurgitation despite recovery of left ventricular function; three of 21 children (15%) in that series required late mitral valve surgery. If the mechanism of severe mitral regurgitation in the older child or adolescent is caused by irreversible myocardial injury, or papillary muscle infarction and then repair of the mitral valve at the time of coronary artery reimplantation, as pointed out by Cochrane and colleagues [4], may be warranted. In a series of 37 patients reported by Lambert and colleagues [10], mitral valve repair was undertaken in 3 of 7 patients with severe preoperative mitral valve regurgitation. All 3 children were beyond infancy. In our experience with 47 patients with ALCAPA having repair by aortic reimplantation more than moderate mitral regurgitation was present in 38% of children. At follow-up, less than 10% (n = 3) had moderate or moderate plus mitral regurgitation, and only 1 patient required repair for severe mitral regurgitation.
Patterns of ventricular recovery
This study demonstrates that normalization of ejection fraction and left ventricular functional factors occurred within 1 year of repair, which concurs with other reports [3, 4]. Ejection fraction and ventricular dilatation improved by 3 to 4 months, probably reflecting the recruitment of hibernating or chronically ischemic myocardium. Improvements in mitral regurgitation lagged behind, suggesting that not only ischemic recovery, but remodeling of the ventricle may be required for recovery of mitral function and competence.
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