DISCUSSION
AAOCA is the second common congenital heart malformation associated with sudden cardiac death [1,4]. Several anatomic variants have been described but AAORCA with inter-arterial and intra-mural course is the most frequently observed pattern. AAOLCA is less frequent but more associated with fatal events, especially in young athletes [1-4]. Other described coronary anomalies are the high origin of the coronary more than 1 cm above the sinu-tubular junction, the slit-like shape of coronary orifice or the common orifice for both left and right coronary arteries. These anomalies can be observed isolated or in association with the previously described [1].
The overall prevalence in the population of AAOCA is currently estimated between 0.1 and 1%. A recent improvement of cardiac screening in competitive athletes is presumably destined to increase their incidence in the next year [5]. Many groups, including our, have developed specific programs for the screening and management of AAOCA [11,16]. Risk stratification and indication to surgery, besides the anatomic evaluation, have encompassed different functional studies aiming to asses cardiac perfusion. Molossi and coll. showed interesting results with dobutamine stress cardiac magnetic resonance for assessment of myocardial perfusion and Angelini and coll. proposed IVUS (intra-vascular ultra sound) for anatomic and functional evaluation by dedicated guided catheters [18,19].
In the main time, guidelines and expert consensus have been developed and the debate on this topic was very prolific [8, 20,21]. Citing T.M. Sundt and M. Jacobs “it is not the intended purpose of guidelines to take the place of clinical judgment and personalized care, but rather to provide a foundation that is a starting point for clinical decision making” [22,23]. Based on this statement and looking to the current literature, it is our opinion, that at the moment indication to surgery are guided primarily by clinical and anatomical criteria [8,11,20,21,24]. In our experience a lot of symptomatic patients were observed among those with AAORCA (previously described as less symptomatic). Typical oppressive chest pain at rest and during efforts (sometimes not strenuous) was the most frequent symptom leading to further cardiologic investigation. Fifty-six percent of our patients referred typical oppressive chest pain while another 12% described palpitation. All of them showed ventricular arrhythmia at ECG. This results account for an overall frequency of cardiac symptoms of 68% in our series. 75% of these symptomatic patients had AAORCA (86% of these with inter-arterial and intra-mural course). These results, reinforced previous observation that severe cardiac symptoms may be present similar in AAORCA as in AAOLCA, despite functional test do not reveal cardiac ischemia [25]. In our population functional tests were negative in 50% of the patients who underwent to the studies. Probably, functional test may have the limit to not reproduce the dynamic condition of the heart’s perfusion during sport’s efforts or normal life (i.e. emotional stress or other factors that alter neuro-vegetative system), that continues to be the focus of the current research in AAOCA.
For these reasons, despite many efforts have been made to standardize the management of AAOCA, several groups continue to publish their own policy and results [12,12,13,24]. The lack of multi-centric prospective, randomized studies, ethnic and socio-economic differences, and the wide range of clinical presentation, probably hampers at the moment the possibility to define a standard management that can fits for all type of AAOCA.
Surgery have demonstrated to be safe in terms of mortality [14,15]. Freedom from reoperation as well was very low in all surgical published series. However, recurrence of symptoms is described up to 40% after surgical management [14,15,24]. Several techniques are used at the moment to approach AAOCA. Coronary unroofing seem to be the most simple and reproducible technique, and currently is the most adopted technique in the United States [7]. However, this result may be due because intramural course is the more frequent anatomic pattern of AAOCA.
All patients with intra-mural course underwent to coronary unroofing at our institution regardless of type and length of AAOCA. In our experience with coronary unroofing we have focused our surgical technique in extending the opening of the roof until the “real” orifice of the coronary artery was detected to match perpendicularly the external take-off of the coronary from the aortic wall. Comparing pre-operative and post-operative imaging (echocardiography and CT scan) we have observed an improvement of the take-off angle (that frequently was associated with an enlargement of the internal diameter of the first segment of the vessel). Our results with coronary unroofing are comparable to those published by Mostefa-Kara and coll. that defined this technique as the “gold standard” for management of AAOCA [12]. However, despite results with coronary unroofing were excellent, there were different anatomic subtypes of AAOCA that in our opinion, are not suitable for this approach (i.e. absence of intramural course). Moreover, as described by Gharibeh and coll. the presence of intra-mural course is not an absolute recommendation for unroofing procedure. It is the overall neo-coronary shape that determines the improvement in coronary flow for these authors [26]. Our surgical policy aimed just to this point. Gaillard and coll. and Courand et coll. have showed optimal results in this way using neo-ostioplasty and coronary reimplantation as alternative technique for both left and right anomalous aortic origin of coronary artery, in children and young adults [13,25]. In our experience patients managed by neo-ostioplasty or coronary reimplantation showed overall same results to coronary unroofing. No patients developed cardiac related complications as ostial calcification sometimes described for neo-ostioplasty [13]. On the contrary, it was impressive, in our experience, the benefit of surgery on symptoms disappearing and this was the principal finding of our study. None of the symptomatic patients described symptoms recurrence postoperatively and at a median follow up of 5.3 years (min 9 months max 13 years). All young athletes returned to practice competitive sport.
The improvement of take-off angle probably reduces flow turbulence at coronary ostia. This mechanism improve perfusion and reduce ischemia. 4D flow MRI may be a useful method to validate this statement that at the moment is only a speculation, and may be the base for future investigation in this subgroup of patients. Previous study of fluid-dynamic has already demonstrated flow differences related to different surgical procedures [27]. Razavi and coll. have recently demonstrated how the improvement in the angle orifice is associated with a low coronary share stress and improved perfusion. This study is unfortunately limited only to coronary unroofing and do not account for different surgical techniques in order to compare them [28].
In conclusion, we believe that the disappearing of the symptoms and the return to sport activities may be considered as important outcome, besides mortality and freedom from reoperation, when we discuss surgical results and we want to offer surgery for AAOCA especially to young athletes.
Limits of our study are the retrospective nature of the research, the unnormal distribution of age of our patients and the lack of a complete long-term follow-up. Moreover, this represent a selected group of patients with the common features of an anatomic pattern described as at risk for SDC.
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