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Address for reprints: Haibo Zhang, MD, PhD, Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, 1678 Dongfang Rd, Shanghai, China.
Although surgical outcomes for anomalous left coronary artery from the pulmonary artery (ALCAPA) are excellent in the modern era with the coronary reimplantantion technique, mortality remains high in Chinese population. This study was undertaken to review the surgical management for ALCAPA in our center and assess the midterm outcomes.
Methods
This was a retrospective review of 105 patients who underwent surgical repair for ALCAPA from January 2008 to January 2018.
Results
The diagnosis of ALCAPA was not made before referred to our hospital in 31 patients (30%). Median age at repair was 7.6 months (interquartile range, 4.3-25.4 months). Mean preoperative left ventricular ejection fraction was 47.3% ± 16.9%. Concomitant mitral intervention was performed in 52 patients (50%). Mechanical circulatory support was used in 15 patients (14%). Early mortality was 14%. Classification and regression tree analysis identified 3 risk groups for early mortality, among which patients with preoperative left ventricular ejection fraction ≤41.6% and age at repair >127 days were the most likely to suffer from mortality. Using nonlinear mixed-effect model to assess the time course for postoperative left ventricular ejection fraction, patients of this group also needed significantly longer time for recovery of left ventricular function after surgery (P < .0001).
Conclusions
Late referral of patients with ALCAPA is not uncommon in our center. Older age at repair in patients with preoperative left ventricular dysfunction is the main reason for higher early mortality, and is also associated with longer time to normalization of left ventricular function after surgery.
Late referral of patients with anomalous left coronary artery from the pulmonary artery is not uncommon in China. Patients who receive surgery at older age with preoperative left ventricular dysfunction are associated with suboptimal surgical outcomes. Timely detection and intervention will be of benefit to these patients.
If the coronary collateralization is not adequately developed after birth, severe left ventricular (LV) myocardial ischemia and dysfunction will occur. Mitral regurgitation (MR) can also be found in most patients secondary to LV dilation and papillary muscle ischemia. If left untreated, the mortality rate reaches 90% during infancy.
Anomalous origin of the left coronary artery from the pulmonary trunk: its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases.
including ligation of the anomalous left coronary artery (LCA), bypass grafting, and intrapulmonary baffle technique (Takeuchi procedure). Nowadays, coronary reimplantation technique to re-establish the normal dual coronary supply has become the method of choice with early mortality rate approaches zero in many cardiac centers worldwide in recent decades.
Repair of anomalous left coronary artery from the pulmonary artery in the modern era: preoperative predictors of immediate postoperative outcomes and long term cardiac follow-up.
Anomalous origin of the left coronary artery from the pulmonary artery in infants: clinical features and the perioperative treatment strategies [in Chinese].
Surgical strategies for anomalous origin of the left coronary artery from the right pulmonary artery with an intramural aortic course: a report of 10 cases.
Therefore we sought to review the surgical management of ALCAPA in our center, identify the risk factors for early mortality, and evaluate the midterm outcomes.
Patients and Methods
Patients
Between January 2008 and January 2018, 105 patients underwent coronary reimplantation technique for ALCAPA repair at Shanghai Children's Medical Center (Table 1). The hospital ethics committee approved this study and waived the need for individual consent.
Table 1Perioperative patient characteristics
Variable
Result
Sample size
105
Age at repair (mo)
7.6 (4.3-25.4)
Weight at repair (kg)
6.9 (5.6-10.7)
Female patient
62 (59)
Surgery since 2013
69 (66)
LVEF (%)
47.3 ± 16.9
LVEDD Z-score
+4.8 ± 2.0
MR grade
None/trivial
7 (7)
Mild
31 (29)
Moderate
48 (46)
Severe
19 (18)
Preoperative mechanical ventilation
5 (5)
Preoperative inotropic support
41 (39)
Concomitant mitral intervention
52 (50)
Cardiopulmonary bypass time (min)
118 (95-145)
Aortic cross-clamping time (min)
73 (62-84)
Mechanical ventilation (h)
100 (48-171)
Intensive care unit stay (d)
8 (5-14)
Mechanical circulatory support
15 (14)
Early mortality
15 (14)
Follow-up time (y)
3.0 (1.2-4.8)
Values are presented as median (interquartile range), n (%), or mean ± standard deviation. LVEF, Left ventricular ejection fraction; LVEDD, left ventricular end-diastolic dimension; MR, mitral regurgitation.
Early mortality was defined as death occurring within 30 days of operation or before hospital discharge. Normalization of the LV function after surgery was defined as postoperative value of LV ejection fraction (LVEF) >50%. Severity of the MR was graded as none/trivial, mild, moderate, and severe on the basis of vena contracta width as well as jet area.
Surgery was performed once the diagnosis was made (Video 1). All patients underwent surgical correction through a median sternotomy. The procedure was performed with distal aortic and bicaval venous cannulation. LV decompression was achieved by venting through the right superior pulmonary vein. Cardioplegia was injected into the aortic root and the main pulmonary artery with the left and right pulmonary arteries snared, or directly into the orifice of the LCA after transection of the main pulmonary artery.
Video 1Surgical repair for anomalous left coronary artery (LCA) from the pulmonary artery by coronary reimplantation technique. This was a 10-month-old girl with preoperative left ventricular ejection fraction of 21.5% and mild mitral regurgitation. After resection of the pulmonary artery, the anomalous LCA was identified originating from the conjunction of the right pulmonary artery and the main pulmonary artery. The LCA button was then harvested and directly reimplanted to the ascending aortic wall. The postoperative period was uneventful. Video available at: https://www.jtcvs.org/article/S0022-5223(19)32359-1/fulltext.
The main pulmonary artery was incised at the distal level and the orifice of the LCA was identified. The orifice of the LCA was harvested as button or flap and the LCA was mobilized. The orifice was close to the aortic side of the pulmonary artery in 37 patients (35%), therefore the LCA button was rotated and directly reimplanted to the aortic wall (Figure 1, A and B). In case of suspected tension on the anastomosis, an aortic flap was resected and the LCA flap was anastomosed to form a tunnel (Figure 1, C and D). This technique was used in 45 patients (43%). If the distance between the LCA orifice and the aorta was too long to be transferred, a tubular extension technique by suturing together the edges of the large LCA cuff was used (Figure 1, E and F). This was performed in 23 patients (22%). Additionally, an autologous pericardial patch was employed to avoid stretching when needed. The main pulmonary artery was either closed directly or reconstructed with autologous pericardial patch.
Figure 1Surgical repair of anomalous left coronary artery (LCA) from the pulmonary artery by 3 techniques of coronary reimplantation (for better illustration, both the ascending aorta and the main pulmonary artery have been transected). A and B, Direct reimplantation technique: The LCA button was harvested from the pulmonary artery and a corresponding portion of the aorta wall was excised (A). End-to-side anastomosis was then made between the LCA button and ascending aorta (B). C and D, Flap-to-flap technique: The LCA flap was harvested from the pulmonary artery, and an aortic flap was also harvested from the ascending aorta (C). Edge-to-edge anastomosis was then made between the 2 flaps to form a LCA tunnel connected to the ascending aorta (D). E and F, Tubular extension technique: A generous LCA cuff was harvested from the pulmonary artery, and a portion of the aortic wall was excised (E). The upper edge and the lower edge of the LCA cuff were then anastomosed to form a tube-like tunnel connected to the ascending aorta (F).
For patients with moderate or severe MR, the decision to perform concomitant mitral intervention was made according to surgeons’ preferences. Fifty-two patients (50%) underwent concomitant mitral intervention. The detailed mitral intervention techniques are summarized in Table E1. The most common techniques performed were posterior annuloplasty (25 patients [49%]) and commissure plication annuloplasty (24 patients [47%]). One patient underwent mitral valvuloplasty by the double orifice technique, and another by posterior leaflet extension with autologous pericardial patch. Cleft mitral valve was identified in 1 patient and was closed with interrupted sutures.
Follow-up
Data were collected retrospectively from hospital records and outpatient clinics, as well as telephone follow-up. A total of 755 postoperative echocardiograms were available for 99 patients (94% of the population). Follow-ups were scheduled 1 month, 3 months, and 6 months after discharge, then every 6 months for the next 18 months, and then every year. Median follow-up time was 3.0 years (interquartile range [IQR], 1.2-4.8 years). Thirteen percent of the living patients were followed up for more than 6 years. Number of patients with postoperative echocardiograms at monthly intervals within 6 years after surgery is shown in Figure E1.
Statistical Analysis
Presentation
Continuous variables were summarized using mean ± standard deviation, or median (IQR) for skewness variables. Categorical variables were summarized as frequency and percentage. Comparison between two groups was performed by Mann-Whitney U test for continuous variables, and by Wilcoxon signed-rank test for categorical variables. Freedom from reoperation was determined as time-related events using Kaplan-Meier curve.
Decision tree analysis
A classification and regression tree (CART) analysis
was performed to determine the risk factors for early mortality and mechanical circulatory support (MCS). Demographic and clinical variables included to the analysis for early mortality were age at repair, weight at repair, gender (female or male), preoperative LVEF, preoperative left ventricular end-diastolic dimension z-score, preoperative MR grade (none/trivial, mild, moderate, or severe), preoperative inotropic support (yes or no), preoperative mechanical ventilation support (yes or no), surgical era (before 2013 or since 2013), reimplantation techniques (direct reimplantation, flap-to-flap, tubular extension), concomitant mitral intervention (yes or no), cardiopulmonary bypass time, aortic crossclamping time, and need for MCS (yes or no). Variables included to the analysis for MCS since 2013 were the same as above with the exception of surgical era and need for MCS. The maximum tree depth was 3, and the minimum terminal node size was 10 cases. Minimum change in improvement was set at 0.001 and cross-validation was performed (10 sample folds). All the above analyses were performed using SPSS software version 22.0 (IBM-SPSS Inc, Armonk, NY).
Analysis of repeated echocardiographic data
The time course of the postoperative LVEF was analyzed longitudinally by repeated-measures nonlinear mixed modeling (SAS PROC NLMIXED; SAS Institute, Cary, NC).
The same approach was used to characterize the time course of the postoperative MR grade, and was presented as probability of postoperative moderate or severe MR. The 68% confidence intervals, comparable to ±1 standard error, were obtained using bootstrap percentile method.
Results
Patient Characteristics
The diagnosis of ALCAPA was not made before referred to our hospital in 31 patients (30%). Five patients (5%) required mechanical ventilation before surgery and 41 patients (39%) required preoperative inotropic support. Mean preoperative LVEF was 47.3% ± 16.9%. Moderate or severe MR was found in 67 patients (64%). Median age at surgery was 7.6 months (IQR, 4.3-25.4 months), with the youngest patient receiving surgery at the age of 43 days. Age distribution is summarized in Figure 2. Median cardiopulmonary bypass time and median aortic crossclamping time were 118 minutes (IQR, 95-145 minutes) and 73 minutes (IQR, 62-84 minutes), respectively.
Figure 2Age at surgery stratified by number of patients.
Early mortality was 14% (15 of 105 patients) in the entire cohort. Seven patients died within 3 days after surgery due to sudden cardiac arrest secondary to ventricular fibrillation before our wide use of MCS from 2013. One patient died of sudden cardiac arrest 2 weeks after surgery after returning to the ward from intensive care unit. Two patients died of progressive cardiac failure and multiple organ dysfunction. Five patients died despite receiving MCS due to refractory cardiac dysfunction.
Using CART analysis, 3 risk groups for early mortality were identified (Figure 3). Preoperative LVEF cutoff of 41.6% was the primary determinant of early mortality. Patients with LVEF >41.6% (Node 2, Group A) were more likely to survive after surgery. In patients with LVEF ≤41.6%, the second determinant was age at repair. Patients with age older than 127 days (Node 4, Group C) showed higher risk of mortality than patients who received surgery at earlier age (Node 3, Group B). The overall classification accuracy was 87%.
Figure 3Classification and regression tree analysis for early mortality after surgery. This analysis identified three risk groups for early mortality (Group A, Group B, and Group C). The primary determinant was preoperative left ventricular ejection fraction (LVEF), and the second determinant was age at repair. Patients in Group C (preoperative LVEF ≤41.6% and age at repair >127 days) were the most likely to experience mortality after surgery.
In total, 15 patients (14%) received MCS, by left ventricular assist device (LVAD) or extracorporeal membrane oxygenation (ECMO). LVAD support was performed with Rotaflow Centrifugal Pump (Maquet Cardiopulmonary AG, Hirrlingen, Germany). Since 2013, the rate of MCS was 20% (14 out of 69 patients). Nine patients (9%) required immediate MCS implantation due to raised left atrial pressure and reduced arterial pressure when attempting to be weaned from cardiopulmonary bypass. Seven patients were supported by LVAD and the other 2 patients by ECMO. During postoperative period, 6 other patients were unable to maintain arterial pressure despite high dose of inotropic support, and were supported by LVAD. The support strategy changed from LVAD to ECMO in 2 patients due to hypoxemia and elevated central venous pressure suggesting biventricular dysfunction 1 day after LVAD support. Using CART analysis, preoperative LVEF was the only determinant for MCS since 2013 (Figure 4). Patients with preoperative LVEF ≤30.6% were more likely to require MCS. The overall classification accuracy was 90%. MCS was successfully removed in 10 patients after a mean duration of 6.4 ± 3.3 days. Neurologic complication was found in 1 patient (7%). Five patients died, as described above.
Figure 4Classification and regression tree analysis for the use of mechanical circulatory support (MCS) since 2013. This analysis identified two risk groups, stratified by preoperative left ventricular ejection fraction (LVEF). Patients with preoperative LVEF ≤30.6% were more likely to require MCS.
Overall, the LVEF transiently decreased from the preoperative values after surgery but rapidly recovered during the early postoperative period and then remained constant, with LV function returning to normal at 14 days after surgery (Figure 5, A). The time courses of postoperative LVEF differed significantly when stratified by the 3 risk groups for early mortality identified above (P < .0001) (Figure 5, B). Both patients in Group A and Group C experienced transient decrease in LVEF from preoperative values after surgery, whereas the LVEF in Group B showed immediate improvement. LV function returned to normal at 4 days, 39 days, and 486 days after surgery in Group A, Group B, and Group C, respectively.
Figure 5A, Time course for left ventricular ejection fraction (LVEF) after surgery in the overall cohort. Mean preoperative (pre-op) LVEF was 47.3% ± 16.9%. B, Time courses for LVEF after surgery stratified by the 3 risk groups for early mortality. Mean pre-op LVEF for Group A, Group B, and Group C was 58.2% ± 10.1%, 29.4% ± 6.5%, and 28.5% ± 8.2% respectively. Solid lines are parametric estimates of mean LVEF after surgery and are enclosed within 68% bootstrap percentile confidence limits.
Sixty percent (31 out of 52 patients) of the concomitant mitral interventions were performed in infants. The severity of preoperative MR was significantly different between patients with and without concomitant mitral intervention (P < .001) (Figure E2). Median aortic crossclamping time in patients who had concomitant mitral intervention was longer than patients who did not (78 minutes [IQR, 68-94 minutes] vs 69 minutes [IQR, 54-77 minutes]; P < .001). Median time for duration of postoperative mechanical ventilation (94 hours [IQR, 48-158 hours] vs 119 hours [IQR, 26-203 hours]; P = .689) and intensive care unit stay (7 days [IQR, 5-13 days] vs 10 days [IQR, 6-15 days]; P = .282) were slightly shorter in patients undergoing concomitant mitral intervention, although no significant differences were found.
In patients without concomitant mitral intervention, the probability of moderate or severe MR gradually fell during the follow-up period. In contrast, in patients with concomitant mitral intervention, although MR grade showed significant improvement immediately after surgery (P < .001), the probability of moderate or severe MR remained relatively constant thereafter (Figure 6). Three patients with deteriorate MR grade who previously received concomitant mitral intervention underwent mitral reintervention by commissure plication annuloplasty at 11 months, 13 months, and 3.5 years after initial surgery, respectively. Freedom from mitral reintervention in patients with concomitant mitral intervention at the initial operation was 97%, 94%, and 86%, respectively, at 1 year, 3 years, and 5 years after initial operation.
Figure 6Time courses for mitral regurgitation (MR) after surgery, stratified by patients with or without concomitant mitral intervention. Solid lines are parametric estimates of probablities of patients with moderate or severe MR after surgery, and are enclosed within 68% bootstrap percentile confidence limits.
Six patients underwent reoperation during follow-up period. Three patients underwent pulmonary patch arterioplasty due to main pulmonary artery stenosis at 10 months, 3 years, and 8 years after initial surgery, respectively. Two patients underwent mitral annuloplasty, and another patient underwent both pulmonary patch arterioplasty and mitral annuloplasty, as described above. Freedom from any reoperation at 1 year, 3 years, and 5 years after initial operation was 97%, 96%, and 90%, respectively.
Discussion
This was a contemporary single-center experience of surgical repair of ALCAPA by reimplantation technique, and is among the largest cohorts to our knowledge. Contrary to the excellent outcomes published in recent decades,
Repair of anomalous left coronary artery from the pulmonary artery in the modern era: preoperative predictors of immediate postoperative outcomes and long term cardiac follow-up.
the result of early mortality of 14% in our cohort was no doubt suboptimal. However, detailed analysis of our patient data demonstrated that older age at surgery in patients with preoperative LV dysfunction was the main reason for higher early mortality, which was also associated with longer duration of LV function recovery after surgery.
For patients with ALCAPA, surgery is indicated as soon as the diagnosis is made. Median age at repair for ALCAPA in the majority of the published literatures is around 5 months,
Repair of anomalous left coronary artery from the pulmonary artery in the modern era: preoperative predictors of immediate postoperative outcomes and long term cardiac follow-up.
However, in our series the median age at repair was 7.6 months. Only about 10% of our patients underwent operation within the age of 90 days, compared with 29% in the Society of Thoracic Surgeons Congenital Heart Surgery Database, which is representative of the major congenital cardiac centers in the United States.
suggested in 2007 that preoperative LVEF <35% was an independent risk factor for early mortality, so was younger age at surgery. The mortality falls dramatically in recent decades in published literatures
Repair of anomalous left coronary artery from the pulmonary artery in the modern era: preoperative predictors of immediate postoperative outcomes and long term cardiac follow-up.
; therefore no risk factors could ever be found. On the contrary, apart from preoperative LVEF, our study showed that the higher early mortality rate was in part due to later age at repair. Our hospital is a tertiary pediatric cardiac center in China, so 30% of patients did not get an accurate diagnosis of ALCAPA before referral to our hospital. Delayed diagnosis and referral for ALCAPA is a critical problem not only in our practice, but also in other centers in China.
The reason may be related to the lack of knowledge and awareness of ALCAPA among health care professionals, including some sonographers who are not familiar with congenital coronary anomalies, given the rarity of this disease.
Education targeted at these practitioners may be of benefit to improve the rate of timely diagnosis of ALCAPA. Besides, as is the case with other critical congenital heart diseases in China, lack of health insurance coverage in some patients may lead to the late presentation.
The strategy of MCS differs from center to center. It was used in 14% of our patients, which is in line with the rates reported in several other studies.
Reoperation and mechanical circulatory support after repair of anomalous origin of the left coronary artery from the pulmonary artery: a twenty-year experience.
Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival, patterns of ventricular recovery and late outcome.
However, MCS is not a risk factor for early mortality in our study. MCS would provide the opportunity for LV function to recover in the critical early postoperative period. The algorithm in our center now is to initiate MCS if a patient has severe LV dysfunction that is difficult in weaning from cardiopulmonary bypass in the operating room. If a patient is able to wean from cardiopulmonary bypass but requires high dose of inotropic support to maintain marginal ventricular function in the intensive care unit, MCS is then applied. It should be noticed that in our series, 5 patients died despite receiving MCS. Although cardiac transplant is the final option for these patients,
Reoperation and mechanical circulatory support after repair of anomalous origin of the left coronary artery from the pulmonary artery: a twenty-year experience.
LVEF showed a transient decrease from preoperative values immediately after surgery, possibly due to the effect of cardiopulmonary bypass. After establishment of the dual coronary supply, LV function rapidly recovered. Some believe that LV function recovery after ALCAPA repair is due to the revascularization of hibernating myocardium, and is not dependent on age.
In contrast, patients with preoperative LV dysfunction but older age at repair in our series showed significantly slow and incomplete recovery of LV function, suggesting different mechanisms in these patients. Even in patients with fully recovered LV function, LV wall motion abnormalities, perfusion deficits, and myocardial scarring could still be detected by magnetic resonance imaging in many patients.
Myocardial perfusion, scarring, and function in anomalous left coronary artery from the pulmonary artery syndrome: a long-term analysis using magnetic resonance imaging.
The management of MR at the time of ALCAPA repair continues to be the subject of debate. Some argue that MR should be left untreated during ALCAPA repair. One reason is that MR was the result of LV dilation secondary to LV ischemia, and could markedly improve with the restoration of dual coronary supply, particularly in infants, even without concomitant mitral intervention.
Does the degree of preoperative mitral regurgitation predict survival or the need for mitral valve repair or replacement in patients with anomalous origin of the left coronary artery from the pulmonary artery?.
reported that half of patients with untreated severe MR required late reintervention. In our series, the severity of MR did show gradual improvement after surgery in patients receiving coronary reimplantation alone. Because nearly all patients with severe MR underwent concomitant mitral intervention, whether or not MR grade could also improve if severe MR were initially left untreated is not known.
Various techniques for mitral intervention in ALCAPA patients have been reported, mainly by annuoloplasty.
Some centers did not recommend concomitant mitral intervention because it may require prolonged aortic crossclamping time on an already compromised myocardium.
In our center, concomitant mitral intervention is usually considered in patients with moderate or severe MR, but the technique is not so extensive. Although longer crossclamping time for concomitant mitral intervention was also the case in our series, we did not find it associated with increased risk of early mortality. Therefore, concomitant mitral intervention could be performed safely. Besides, MR grade showed immediate improvement after completion of mitral intervention. It seems to be a logical way to increase cardiac output during early postoperative period.
The limitations of this study are mainly due to its retrospective nature and relatively short period of follow-up time. Although this is among the largest cohort of ALCAPA patients to our knowledge, the relatively small sample size reduces the power of this study. Inference based on CART analysis in a small dataset may have limited strength. Moreover, few patients with severe MR in our series received coronary reimplantation alone, which precluded us from predicting their postoperative MR time course and reaching conclusion about whether the mitral valve could be left untreated in these patients.
Conclusions
Late referral of patients with ALCAPA is not uncommon in our center. Older age at repair for ALCAPA in patients with preoperative LV dysfunction is the main reason for higher early mortality, and is also associated with longer time to normalization of LV function after surgery.
Conflict of Interest Statement
Authors have nothing to declare with regard to commercial support.
Surgical repair for anomalous left coronary artery (LCA) from the pulmonary artery by coronary reimplantation technique. This was a 10-month-old girl with preoperative left ventricular ejection fraction of 21.5% and mild mitral regurgitation. After resection of the pulmonary artery, the anomalous LCA was identified originating from the conjunction of the right pulmonary artery and the main pulmonary artery. The LCA button was then harvested and directly reimplanted to the ascending aortic wall. The postoperative period was uneventful. Video available at: https://www.jtcvs.org/article/S0022-5223(19)32359-1/fulltext.
Appendix
Figure E1Number of patients with postoperative echocardiograms at monthly intervals within 6 years after surgery. The 6-month increases in echocardiographic measurements during the first 2 years after surgery and the yearly increases thereafter are suggestive of regularly scheduled follow-up.
Figure E2Severity of preoperative mitral regurgitation in patients without and with concomitant mitral intervention. The difference between the 2 groups were analyzed using Wilcoxon signed-rank test.
Anomalous origin of the left coronary artery from the pulmonary trunk: its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases.
Repair of anomalous left coronary artery from the pulmonary artery in the modern era: preoperative predictors of immediate postoperative outcomes and long term cardiac follow-up.
Anomalous origin of the left coronary artery from the pulmonary artery in infants: clinical features and the perioperative treatment strategies [in Chinese].
Surgical strategies for anomalous origin of the left coronary artery from the right pulmonary artery with an intramural aortic course: a report of 10 cases.
Reoperation and mechanical circulatory support after repair of anomalous origin of the left coronary artery from the pulmonary artery: a twenty-year experience.
Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival, patterns of ventricular recovery and late outcome.
Myocardial perfusion, scarring, and function in anomalous left coronary artery from the pulmonary artery syndrome: a long-term analysis using magnetic resonance imaging.
Does the degree of preoperative mitral regurgitation predict survival or the need for mitral valve repair or replacement in patients with anomalous origin of the left coronary artery from the pulmonary artery?.
The cardiac surgeon learns early in her or his career that any type of coronary artery problem—congenital, acquired, or iatrogenic injury—can result in an unrecoverable situation in the operating room. Anomalous left coronary artery from the pulmonary artery (ALCAPA) is unique in that significant systolic dysfunction at the time of presentation is often present and is not responsive to conventional resuscitative maneuvers. Early surgery with establishment of a dual coronary system involving reimplantation of the left coronary artery into the aorta is the procedure of choice.
The surgeons from Shanghai Children's Medical Center have reported their outcomes in infants and children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA).1 Their series of 105 patients in the past 10 years had an overall mortality rate of 14%. The most recent Society of Thoracic Surgeons Congenital Heart Surgery database report has a total of 135 patients operated on for this diagnosis between 2015 and 2018.2 In that series, there was no mortality! That is obviously quite a large difference in outcomes.
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