Summary
Background
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) can sometimes be diagnosed very early in newborns and thus be corrected surgically before any myocardial ischaemia occurs.
Aim
To evaluate the influence of this very early surgery on intensive care management and long-term outcome.
Methods
All children operated on for ALCAPA before the age of 1 year between 1995 and 2010 were considered. Those operated on before the onset of ischaemia (group B) were compared with a matched group of children of similar weight who were operated on by the same surgeon on a close surgery date (group A).
Results
We obtained a homogenous population of 13 children (eight in group A; five in group B) with no significant weight difference between groups. The surgical technique and peroperative variables were similar in both groups. There was one death in each group. The group B death was due to postoperative occurrence of global myocardial infarction despite correct surgical reparation. Mechanical ventilation duration, inotropic support duration, intensive care duration and long-term complications were lower in group B.
Conclusion
Very early ALCAPA surgical correction before occurrence of ischaemia must be encouraged whenever possible because it simplifies intensive care management and prevents persistent mitral regurgitation, but it does not reduce peroperative risk.
Résumé
Contexte
Il est parfois possible de dépister une coronaire gauche naissant anormalement du tronc pulmonaire (Alcapa) à un très jeune âge et donc de corriger chirurgicalement cette anomalie avant la survenue de l’ischémie.
Objectif
Évaluer l’influence d’une telle chirurgie précoce sur la réanimation et le devenir à long terme.
Méthodes
Tous les enfants opérés d’une Alcapa avant l’âge d’un an entre 1995 et 2010 dans nos deux centres ont été inclus. Ceux opérés avant l’ischémie constituée (groupe B) ont été comparés à un groupe témoin (groupe A) appareillé sur le poids, la date opératoire et le chirurgien.
Résultats
Nous avons constitué une population homogène de 13 patients (huit dans le groupe A et cinq dans le groupe B). Le poids, la technique chirurgicale et les paramètres peropératoires étaient similaires dans les deux groupes. Il y a eu un mort dans chaque groupe. Le décès du groupe B a été imputé à la survenue d’un infarctus massif au décours immédiat de la chirurgie malgré une réparation correcte. Pour autant, la durée de ventilation, du support inotrope et du séjour en réanimation ainsi que les complications à long terme ont été moins importantes dans le groupe B.
Conclusion
La réparation très précoce des Alcapa avant constitution de l’infarctus doit être choisie dès que possible car la réanimation s’en trouve simplifiée et les régurgitations mitrales persistantes moins fréquentes. Cependant, ce choix ne diminue pas le risque opératoire.
Background
ALCAPA is a rare but well-known cause of paediatric myocardial ischaemia. The global incidence of ALCAPA is close to 1 in 300,000 living births , even if some regional studies report much higher figures . Newborns are usually asymptomatic in the first month of life, but congestive cardiac failure occurs 90% of the time between the second month and the first year, when a decrease in pulmonary artery pressure results in lower left coronary pressure . The anomaly must be corrected by surgery, in order to improve left coronary artery perfusion. Reimplantation into the aortic root is the most efficient technique . However, postsurgery intensive care management can be very hazardous in this setting of myocardial ischaemia and stunning phenomenon. Some children are diagnosed by chance at a very early stage, before the onset of ventricular dysfunction, and thus can benefit from surgery before the occurrence of myocardial infarction. The option to undertake very early surgery could be considered obvious and safe, but our experience shows that surgery can trigger a massive myocardial infarction, leading to very complex intensive care management. Therefore, we designed this retrospective study in our centres to compare the influence of presurgical myocardial ischaemia on the short- and long-term outcomes of surgical ALCAPA correction.
Methods
Patients
The cardiovascular surgery databases in our centres were reviewed for all patients with a diagnosis of ALCAPA corrected during their first year of life, from January 1995 to July 2010. Diagnoses associating ALCAPA with other complex heart defects were excluded. Fifty-eight patients were identified whose median age at surgery was 130 days. Within this population, we identified a group of five children without any electrical or biological sign of constituted ischaemia on the day of surgery (hereafter called group B = surgery before ischaemia). The selection criteria were: absence of significant Q waves, and normal troponin, creatine phosphokinase and MB isoform plasma concentrations, if available. We sought to match every child in group B to two other children operated on after the onset of ischaemia, using three criteria: same surgeon, similar weight at surgery (weight difference < 1 kg) and close surgery date (children operated on within 1 year). Two group B children could only be matched to one child. We therefore included eight children in this second group (hereafter called group A = surgery after the onset of ischaemia). Therefore, the study population consisted of 13 children who underwent direct reimplantation of the anomalous left coronary artery into the ascending aorta.
Data
The clinical records were reviewed to document presentation, preoperative state and postoperative course. The following data were retrieved: age at diagnosis; preoperative state (age at surgery; weight; clinical status assessed by the Ross classification [class I–IV] ; mitral regurgitation assessed by echocardiography (classified as none, mild, moderate or severe); left ventricular function evaluated by the shortening fraction in TM mode; ECG anomalies in the anterior and lateral zones (classified as none, ST depression or negative T wave, ST elevation or Q wave); and troponin plasma concentration if available); peroperative or postoperative course (surgeon identity; extracorporeal circulation duration; circulatory assistance duration; cardioplegia (blood or crystalloid); delayed sternal closure; mechanical ventilation duration; inotropic support duration; intensive care duration; long-term complications (none, mitral regurgitation, ejection fraction alteration or death); and follow-up duration).
Statistical analysis
Continuous variables were compared by the non-parametric Mann–Whitney U test adapted for small groups . Two categorical variables were analysed by Fisher’s exact test and multicategorical variables by a unilateral two-sample Kolmogorov-Smirnov test for small samples of different sizes. A value of p < 0.05 was considered to be of statistical significance.
Results
Diagnosis and preoperative state
Of the 13 patients, eight had come to attention because of signs of heart failure (group A), whereas five (group B) were diagnosed at the median age of 10 days before any symptoms of heart failure. In two of the five children in group B, initial echocardiography was performed to check aortic coarctation (reduced femoral pulses in one and antenatal ventricular imbalance in the other). In two other children in this group, echocardiography was performed to look for associated cardiac malformation after neonatal surgical correction of oesophageal atresia and omphalocele. The fifth child was diagnosed because of global anterolateral ST elevation (ECG justified by antenatal tachycardia).
The children’s characteristics and their preoperative states are presented in Table 1 . Our population sex ratio was four males to nine females. There was no weight difference between groups. Among the collected data, only age at surgery, clinical status (Ross class), lateral anomalies in ECG, troponin plasma concentrations and echographic criteria differed between groups A and B.
| Characteristic | Group A ( n = 8) | Group B ( n = 5) | p |
|---|---|---|---|
| Men a | 3 (37) | 1 (20) | 0.61 |
| Age at diagnosis (days) b | 42 [6; 60] | 10 [1; 55] | 0.08 |
| Age at surgery (days) b | 67 [50; 78] | 10 [2; 60] | 0.003 |
| Mean weight at surgery (kg) | 3.75 | 3.11 | 0.13 |
| Ross class ( n ) | 0.001 | ||
| I | 0 | 5 | |
| II | 3 | 0 | |
| II | 2 | 0 | |
| IV | 3 | 0 | |
| ECG anomalies ( n ) | |||
| Anterior anomalies | 0.78 | ||
| None | 2 | 2 | |
| Negative T wave | 1 | 0 | |
| ST + | 2 | 3 | |
| Q wave | 3 | 0 | |
| Lateral anomalies | 0.001 | ||
| None | 0 | 2 | |
| Negative T wave | 0 | 1 | |
| ST + | 0 | 2 | |
| Q wave | 8 | 0 | |
| Shortening fraction (%) b | 18 [10; 30] | 28 [23; 34] | 0.009 |
| Mitral regurgitation ( n ) | 0.18 | ||
| None | 1 | 3 | |
| Grade I | 2 | 2 | |
| Grade II | 5 | 0 | |
| Grade III | 0 | 0 | |
| Grade IV | 0 | 0 | |
| Mean troponin Ic concentration (μg/L) | 0.5 | < 0.03 | 0.018 |
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