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Ann Thorac Surg 2003;76:581-588
© 2003 The Society of Thoracic Surgeons
a Department of Medico-Chirurgico di Cardiochirurgia e, DMCCP, Ospedale Pediatrico Bambino Gesù, Roma, Italy
b Department of Cardiologia Pediatrica, DMCCP, Ospedale Pediatrico Bambino Gesù, Rome, Italy
Accepted for publication February 13, 2003.
* Address reprint requests to Dr Michielon, Dipartimento Medico-Chirurgico di Cardiologia Pediatrica, Ospedale Pediatrico Bambino Gesù, Piazza S. Onofrio 4, 00165 Rome, Italy
e-mail: guido.michielon{at}tin.it
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Abstract |
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METHODS: Between 1987 and 2002, 31 patients (median age, 7.1 months) underwent repair for anomalous origin of the left (n = 28), right (n = 2), or both (n = 1) coronary arteries from the pulmonary artery. Repair was accomplished by subclavian interposition in 5 patients, intrapulmonary tunnel in 12, and direct aortic reimplantation in 14. Primary mitral valve repair was never associated with coronary revascularization. Total follow-up was 186.4 patient-years (mean, 77.2 months).
RESULTS: Fifteen-year actuarial survival was 92.9% ± 4.9% for coronary transfer, 40.0% ± 21.9% for subclavian interposition, and 89.9% ± 7.5% for intrapulmonary tunnel (p = 0.019). Five patients required further intervention for supravalvular pulmonary stenosis (n = 3), baffle leak (n = 1), and mitral valve replacement (n = 1). Coronary transfer allowed best freedom from long-term reoperation (92.3% ± 7.4%). Left ventricular shortening fraction increased from 17.3% ± 6.3% before operation to 34.1% ± 4.6% at last follow-up (p < 0.01). Regression analysis demonstrated a linear relationship between age at repair and shortening fraction recovery (r2 = 0.573, p < 0.01). Patients younger than 6 months of age showed worse preoperative shortening fraction (15.9% ± 5.2%) and best longitudinal shortening fraction recovery (36.4% ± 5.1%; p < 0.001). Major improvement in mitral valve function was observed within 1 year from surgery in 90.4% of survivors.
CONCLUSIONS: Repair of anomalous coronary artery origin from the pulmonary artery in younger symptomatic infants offers the best potential for recovery of left ventricular function, despite a worse initial presentation. Coronary transfer is associated with superior long-term survival and freedom from reoperation. Most patients with patent two-coronary repair will recover normal mitral valve function; therefore, simultaneous mitral valve surgery seems unwarranted.
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Introduction |
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TopAbstractIntroductionMaterial and methodsResultsCommentAcknowledgmentsReferences |
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Material and methods |
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Preoperative evaluation
Clinical examination, chest roentgenograms, electrocardiogram, and echocardiography were performed preoperatively in all patients. Congestive heart failure, defined as need for maximal medical therapy (including vasodilator agents, intravenous diuretic agents, intravenous inotropic support, or mechanical ventilation), was the presenting symptom in 19 patients (61.2%). Preoperatively, 5 patients required substantial inotropic support (>6 µg · kg-1 · min-1 dopamine intravenous infusion), 2 of them receiving mechanical ventilation as well. Electrocardiographic evidence of myocardial infarction, defined as presence of Q waves in leads I and aVL, was present in 7 patients (22.6%). Preoperative shortening fraction (SF) was calculated in all patients by two-dimensional echocardiography using both parasternal long-axis and short-axis views. The mean preoperative SF was 17.3% ± 6.3%. Preoperative mitral valve incompetence, evaluated by two-dimensional echocardiography and color Doppler flow in all patients, was graded as mild in 7 patients, moderate in 10, and severe in 14. Moderate to severe preoperative mitral valve incompetence (grade 2 to 3+) was therefore detected in 24 patients (77.4%). Indication for surgery was exclusively based on detailed echocardiographic study, with demonstration of diastolic color Doppler flow from the anomalous coronary into the pulmonary artery in 5 patients (16.1%). Echocardiographic presumptive diagnosis was confirmed by cardiac catheterization in the remaining 26 patients (83.9%). The development of the collateral circulation was visualized by angiographic technique and judged as "good" when rapid filling through well-developed lateral–lateral, mainly septal-type collaterals, allowed prompt visualization of the anomalous coronary and its branches, whereas the collateral circulation was defined as "poor" when primarily based on terminal–lateral type collaterals with late visualization of the anomalous coronary. Using these criteria, the collateral circulation was judged as good in 16 patients and poor in 10. One patient underwent repair of aortic coarctation by end-to-end anastomosis before diagnosis of anomalous origin of both coronary arteries from the pulmonary artery. There were no other associated anomalies in this cohort.
Operative details
Surgery was performed by means of a median sternotomy while the patient was placed on hypothermic cardiopulmonary bypass (25°C rectal temperature). Antegrade perfusion in the anomalous coronary was promoted by snaring the pulmonary artery branches soon after institution of cardiopulmonary bypass. Cardiac arrest was induced by infusion of cold crystalloid or blood cardioplegic solution in the aortic root. Administration of cardioplegic solution in both the aortic and pulmonary roots was accomplished in 6 patients with poor collateral circulation from the contralateral coronary artery. Antegrade plus retrograde cardioplegia was recently selected in 3 patients. The anomalous coronary artery origin was identified through a transverse incision in the pulmonary trunk. The coronary ostium arose from the posterior right pulmonary sinus in 14 patients (45.2%), from the posterior left pulmonary sinus in 10 (32.2%), and from the anterior sinus in 3 (9.7%). Origin of the coronary ostium from the right (n = 3) or left (n = 1) pulmonary artery branches represented a less common entity (12.9%). A two-coronary repair was accomplished by either subclavian interposition (5 patients), intrapulmonary tunnel (10 patients), or coronary transfer (14 patients). The strategy of surgical repair evolved over the years. A subclavian interposition technique was preferred in the early experience. This technique included excision of the coronary ostium with a surrounding cuff of pulmonary artery wall. The left subclavian was divided proximal to the vertebral artery takeoff; the proximal stump was turned down anteriorly to the aortic arch and anastomosed in an end-to-end fashion to the coronary artery cuff. Since 1988 the dual coronary repair was accomplished by either direct aortic reimplantation (14 patients) or by creation of an intrapulmonary tunnel with a polytetrafluoroethylene (PTFE) gusset (10 patients), according to the anatomic location of the anomalous coronary artery ostium. Coronary transfer was preferred when the coronary ostium arose from the right facing or anterior sinus of Valsalva of the pulmonary artery, whereas an aortopulmonary window with intrapulmonary tunnel was more frequently accomplished when the anomalous coronary ostium arose from the nonfacing pulmonary sinus. Trapdoor (3 patients), coronary elongation with aortic and pulmonary artery wall flaps (1 patient), and pericardial hood augmentation (1 patient) implemented a tension-free anastomosis in coronary transfer. Relocation of the coronary button was accomplished under direct vision through a separate incision in the anterior aortic wall to avoid any damage to the aortic valve leaflets or commissures. Whenever possible, the coronary button was relocated in the middle of the posterior aortic sinus. The pulmonary artery was patch repaired in 27 patients (87.1%). Mean cross-clamp time was 62.1 ± 35.1 minutes, whereas mean cardiopulmonary bypass time was 149.1 ± 84.2 minutes.
Although available in our unit since 1995, extracorporeal membrane oxygenation or LV assist device support was not necessary for the postoperative recovery of any of the 12 patients with ACAPA syndrome operated on since then.
Follow-up
Total follow-up was 186.4 patient-years, ranging from 0.3 to 180 months (mean, 77.2 months). Follow-up was 100% complete. Recovery of LV function after surgical repair was evaluated by two-dimensional echocardiography at regular intervals on all hospital survivors at the outpatient clinic. End-systolic diameter, end-diastolic diameter and fractional shortening were calculated before surgical repair, at hospital discharge, and at 6 months, 12 months, and yearly thereafter. The degree of mitral valve incompetence was moreover evaluated by pulsed and colored Doppler mapping of the atrial jet and subjectively graded from 0 to 3+ on a crescent basis from none to severe.
Statistical methods
Kaplan-Meier survival, actuarial survival, and freedom from reoperation were calculated using the SAS Statview-1998 statistical software (SAS Institute Inc, Cary, NC). Selected end points were death, reoperation, or interventional cardiologic procedure (balloon dilatation). Difference in survival was estimated by the log-rank test. Dichotomous variables were analyzed by the 
2 and Wilcoxon rank-sum tests. Longitudinal evaluation of continuous variables was compared by one-way analysis of variance. Variables associated with increased risk of early (30-day) death and risk of reoperation were assessed by univariate and multivariate logistic regression. Longitudinal changes in SF recovery were stratified according to age at repair and analyzed by repeated-measures analysis of variance. Longitudinal changes in mitral valve function were evaluated in a similar fashion. Moreover, a regression analysis was conducted to verify the relationship between age at repair (natural logarithm of age) and the last calculated SF at a mean follow-up of 77.2 months from surgical repair.
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Another patient with anomalous origin of the left coronary artery ostium from the left posterior pulmonary sinus underwent ACAPA repair by creation of an intrapulmonary tunnel with a PTFE baffle. She died on the first postoperative night of persistent low cardiac output. Necroscopy showed an ischemic left ventricle with no evidence of fibroelastosis. Inspection of the intrapulmonary tunnel showed kinking of the PTFE baffle in its midportion.
The last patient had previously undergone aortic coarctation repair by end-to-end anastomosis. Persistent impairment of ventricular function prompted an echocardiographic diagnosis of anomalous origin of both coronary arteries from the right pulmonary artery branch. She underwent relocation of both coronary arteries with a single pulmonary artery wall button on the right side of the aortic root, leaving the left coronary course behind the aorta. She died on the first postoperative day of persistent low cardiac output. Necroscopy showed patent coronary ostia in the presence of myocardial sclerosis with endocardial fibroelastosis. Retrospectively, aortic relocation by separate coronary buttons could have probably resulted in a more favorable proximal course of the left coronary artery.
There was no late mortality. Kaplan-Meier survival is 82.8% ± 7.1% at 180 months. Since 1993 Kaplan-Meier survival improved to 93.8% ± 6.7% at 105 months follow-up, showing a trend toward borderline significance (p = 0.08; Fig 1).
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Four patients underwent reoperation to relieve supravalvular pulmonary stenosis 32.9 ± 16.4 months (range, 4 to 67 months) after ACAPA repair by intrapulmonary tunnel (n = 3) or subclavian interposition technique (n = 1). Concomitant repair of residual baffle leak and supravalvular pulmonary stenosis was accomplished in 1 patient 67 months after modified Takeuchi repair. Augmentation of the pulmonary artery trunk with a PTFE patch allowed successful decompression of the right ventricle in three cases, whereas the last patient required a transannular PTFE patch, 19 months after subclavian interposition for recurrent stenosis unresponsive to balloon angioplasty.
Coronary transfer allowed 92.3% ± 7.4% actuarial freedom from reoperation at 13.9 years, as opposed to 64.8% ± 11.5% for intrapulmonary tunnel at 11.2 years and 50.0% ± 19.4% for subclavian interposition at 15 years (p = 0.45). Logistic regression indicated intrapulmonary tunnel technique as a borderline risk factor for reoperation (coefficient, 1.73; p = 0.079).
Left ventricular and mitral function recovery
Longitudinal assessment of LV function by two-dimensional echocardiography showed a progressive recovery of LVSF. The mean preoperative LVSF was 17.3% ± 6.3%. At 1 month after repair the mean SF increased to 25.1% ± 4.8%; by 6 months it was 29.8% ±3.6%, reaching 32.8% ± 4.3% at 1 year and 34.1% ± 4.6% at the last follow-up. Paired mean comparisons showed progressive SF improvements at each step compared with the previous evaluation, although the most remarkable recovery was observed within the sixth postoperative month (Fig 2).
Patients younger than 6 months of age at repair showed worse preoperative SF and best longitudinal SF recovery, according to repeated-measures analysis of variance (Fig 3).
Preoperative need for substantial inotropic support (exceeding 6 µg · kg-1 · min-1 dopamine intravenous infusion) did not affect recovery of postoperative SF. Regression analysis demonstrated a linear relationship between age at repair, expressed as natural logarithm of age, and LVSF at last follow-up (Fig 4).
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Comment |
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Soon after the fall of pulmonary vascular resistance, perfusion of the ventricular wall supplied by the anomalous coronary artery is critically impaired by a stealing mechanism. The combined effect of left-to-right shunting through the anomalous coronary artery and the critical reduction in coronary perfusion frequently result in compromised SF and cardiac output. Development of collateral circulation from the contralateral coronary artery mitigates clinical presentation; nevertheless, ischemic cardiomyopathy is a dominant feature of this disease. Younger age and evidence of acute myocardial infarction have been identified in the past as powerful predictors of early mortality [10]. Delayed surgical repair with interim medical therapy [10] or heart transplantation [11] have been advocated in the critical infant requiring high-dose inotropic support. Nonetheless, late functional studies have demonstrated excellent recovery of LV function after surgical repair, despite the apparent extent of preoperative LV damage [9]. Therefore two-coronary repair with postoperative mechanical support of a failing left ventricle [12, 13] has been recommended even in the very high-risk patients who cannot be weaned from cardiopulmonary bypass, to obviate the detrimental effects of high-dose inotropic support on organ perfusion. On the other hand, late outcome with different surgical techniques of ACAPA repair remains incompletely defined. Currently, simple ligation of the anomalous coronary artery is not recommended because of the risk of sudden death associated with a single coronary artery system and reduced myocardial flow reserve, as demonstrated by impaired treadmill exercise tolerance. However, emergency ligation of the anomalous coronary artery in infancy has been recently reported as a lifesaving procedure for patients with compromised cardiac output presenting in centers where circulatory assist devices are not available [14]. The general consensus towards restitution of a dual coronary circulation is supported by the superb results of early and late survival, associated with apparently complete recovery of LV function at late follow-up [9].
We have adopted a policy of two-coronary repair since the late 1980s, although our surgical strategy has evolved during the years. The initial experience with a modified subclavian interposition technique [2] was indeed associated with high early mortality. We believe that the high incidence of early failures with this approach was related to the potential proximal kinking of the left subclavian turndown and to the cranial rotation of the coronary button and the left main segment of the anomalous coronary artery.
This technique was abandoned in favor of aortic reimplantation of the anomalous coronary artery button, especially when the coronary ostium arose from the facing or anterior pulmonary sinus. Construction of an intrapulmonary tunnel with a PTFE baffle, otherwise known as a modified Takeuchi procedure, was initially preferred when the ostium of the anomalous coronary artery was located in the nonfacing pulmonary sinus, as creation of a tension-free anastomosis is probably key for early and long-term coronary patency. Growing experience with the arterial switch operation and the introduction of technical modifications like the pericardial hood and the trapdoor technique [15] have made aortic relocation technically feasible even in the most unfavorable anatomic scenarios [16]. We have successfully relocated an anomalous left coronary artery originating from the nonfacing pulmonary sinus using a combination of aortic and pulmonary flaps, as suggested by Sese and Imoto [6], and transferred an anomalous left coronary artery originating from the right pulmonary artery branch, applying the pericardial hood augmentation technique, described for intramural coronary artery relocation. Moreover, late complications of the intrapulmonary tunnel, like baffle leak and recurrent right ventricular outflow tract obstruction, have made this surgical option less appealing in our practice. However, we have observed no difference in survival and recovery of LV function between these two options of dual coronary repair, and we still consider the modified Takeuchi procedure a valid alternative in case of leftward location of the coronary ostium, especially when extensive collaterals surround the pulmonary sinuses.
This study confirms that recovery of LV function is achieved in most patients with ACAPA and a patent two-coronary repair, irrespective of preoperative clinical presentation and LV systolic function. This finding supports the hypothesis of a hibernating myocardium in ACAPA [17], with global systolic dysfunction for downregulation of contractility and myocyte adaptation to chronic ischemia, but with preserved myocardial viability. Moreover, this study demonstrates that age at repair is associated with a logarithmic trend of SF recovery at long-term. We infer that younger symptomatic infants, who usually exhibit the poorest preoperative LV function, are the ones with the potential for best LV function recovery. These results suggest that an aggressive approach toward early repair of ACAPA is probably beneficial per se, to achieve the best functional recovery of the ischemic myocardium, preventing the structural cellular adaptation observed in chronic myocardial hypoperfusion. Moreover, myocyte hyperplasia could play an important additional role in recovery of LV function in the very young. Abnormalities in ventricular septal motion [8, 18, 19], possibly secondary to patchy fibrosis, are commonly documented on longitudinal noninvasive evaluation of LV function in patients undergoing revascularization at an older age. This is confirmed by the intriguing findings of Stern and associates [20], who most frequently detected perfusion defects on thallium 201 imaging whenever repair of ACAPA was accomplished at an older age, further supporting the strategy of early repair of this anomaly.
The issue of mitral valve management in ACAPA remains controversial [21–23]. Mitral incompetence, a common presenting finding in ACAPA, ensues a multifactorial pathophysiology for the combination of papillary ischemia, LV free wall dyskinesia, and LV dilatation. We have adopted a conservative approach, avoiding primary mitral valve repair, irrespective of the degree of preoperative incompetence. This strategy was based on the idea that reversal of LV dilatation and papillary dyskinesia with coronary revascularization could restore, or at least improve, valve function. The adult cardiac experience in mitral valve plasty has indeed demonstrated that major improvements in mitral competence are difficult to achieve when the mechanism of valve insufficiency is secondary to myocardial ischemia. Furthermore, adequate mitral exposure in a small infant might force a transseptal approach, adding ischemic time for a true open heart procedure on a compromised myocardium. We verified that adequate recovery of mitral valve competence is ensured in a patent two-coronary repair, with no discernible correlation with the degree of preoperative incompetence. The single patient requiring mitral valve replacement in our cohort had experienced an early occlusion of the reimplanted left coronary artery with posterolateral wall myocardial infarction. This is consistent with the speculation of Huddleston and colleagues [22] on the correlation between persistent or recurrent mitral incompetence after ACAPA repair and residual coronary stenosis or occlusion.
In conclusion, ACAPA repair in younger symptomatic infants offers the best potential of LV function recovery, despite worse initial presentation. Coronary transfer yields superior long-term survival and freedom from reoperation. Most patients with patent two-coronary repair will recover normal mitral valve function; therefore, simultaneous mitral valve surgery appears to be unwarranted.
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Acknowledgments |
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TopAbstractIntroductionMaterial and methodsResultsCommentAcknowledgmentsReferences |
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References |
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TopAbstractIntroductionMaterial and methodsResultsCommentAcknowledgmentsReferences |
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