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.
References:
- Davis JA, Cecchin F, Jones TK, Portman MA. Major coronary artery
anomalies in a pediatric population: incidence and clinical
importance. J Am Coll Cardiol. 2001;37:593-7.
- Poynter JA, Williams WG, McIntyre S, Brothers JA, Jacobs ML,
ZiemerG,etal. Congenital Heart Surgeons Society AWG. Anomalous aortic
origin of a coronary artery: a report from the Congenital Heart
Surgeons Society registry.World J Pediatr Congenit Heart Surg.
2014;5:22-30.
- Cheezum MK, Liberthson RR, Shah NR, Villines TC, O’Gara PT, Landzberg
MJ, et al. Anomalous aortic origin of a coronary artery from the
inappropriate sinus of Valsalva. J Am Coll Cardiol. 2017; 69:1592-608.
- Angelini P. Sudden cardiac death: do we know what we are talking
about? Circulation. 2002;105: E182
- Mery CM, Lopez KN, Molossi S, Sexson-Tejtel SK, Krishanamurthy R,
McKenzie D. Analysis to define the optimal management of athletes with
anomalous aortic origin of a coronary artery. J Thorac Cardiovasc Surg
2016; 152:1366-75
- Brothers JA, Frommelt MA, Jaquiss RDB, Myerburg RJ, Fraser Jr CHD,
Tweddel JS. Expert consensus guidelines: Anomalous aortic origin of a
coronary artery J Thorac Cardiovasc Surg 2017; 153:1440-57
- Jegatheeswaran A, Devlin PJ, Williams WG, Brothers JA, Jacobs ML, De
Camply DW et al.
Outcomes after anomalous aortic origin of a coronary artery repair: A
Congenital Heart Surgeons’ Society Study. J Thorac Cardiovasc Surg 2020;
160:757-71
- Jegatheeswaran A, Alsoufi B. Anomalous aortic origin of a coronary
artery: 2020 year in review J Thorac Cardiovasc Surg 2021 Aug; 162
(2): 353-359
- MoscaRS, PhoonCK. Anomalous aortic origin of a coronary artery is not
always a surgical disease. Semin Thorac Cardiovasc Surg Pediatr Card
Surg Annu. 2016; 19:30-6.
- Vouhe PR. Anomalous aortic origin of a coronary artery is always a
surgical dis- ease. Semin Thorac Cardiovasc Surg Pediatr Card Surg
Annu. 2016; 19:25-9.
- Mery CM, De Leon LE, Molossi S, Sexson-Tejtel K, Agrawal H,
Krishnamurthy R et al. Outcomes of surgical intervention for anomalous
aortic origin of a coronary artery: A large contemporary prospective
cohort study J Thorac Cardiovasc Surg 2018;155:305-19
- Mostefa-Kara M, Fournier E, Cohen S, Hascoet S, Van Aerschot I,
Roussin R, El Zoghby J, Belli E. Anomalous aortic origin of a coronary
arteries: is the unroofing procedure always appropriate? Eur J
Cardiothorac Surg. 2021 Apr 13; 59(3): 705-710
- Gaillard M., Pontailler M, Danial P, Moreau de Bellaing A, Gaudin R,
du Puy-Montbrun L et al. Anomalous aortic origin of coronary arteries:
an alternativevto the unroofing strategy. Eur J Cardiothorac Surg
2020; 58:975–82.
- Jegatheeswaran A, Devlin PJ, McCrindle BW, Williams WG, Jacobs ML,
Blackstone EH, et al. Features associated with myocardial ischemia in
anomalous aortic origin of a coronary artery: a CHSS study. J Thorac
Cardiovasc Surg. 2019; 158:822-34
- Brothers JA, McBride MG, Seliem MA, Marino BS, Tomlinson RS, Pampaloni
MH, et al. Evaluation of myocardial ischemia after surgical repair of
anomalous aortic origin of a coronary artery in a series of pediatric
patients. J Am Coll Cardiol. 2007;50:2078-82.
- Bianco F, Colaneri M, Bucciarelli V, Surace FC, Iezzi FV, Primavera M
et al. Echocardiographic screening for the anomalous aortic origin of
coronary arteries. Open Heart 2021;8:e001495
- Gaudin R, Raisky O, Vouhe´ PR. Anomalous aortic origin of coronary
arteries: ‘anatomical’ surgical repair. Multimed Man Cardiothorac Surg
2014;2014: mmt022.
- Molossi S, Agrawal H, Mery CM, Krishnamurthy R, Masand P. Outcomes in
Anomalous Aortic Origin of a Coronary Artery Following a Prospective
Standardized Approach Circ Cardiovasc Interv. 2020;13: e008445
- Angelini P, Velasco JA, Ott D, Khoshnevis GR. Anomalous coronary
artery arising from the opposite sinus: descriptive features and
pathophysiologic mechanisms, as documented by intravascular
ultrasonography. J Invasive Cardiol. 2003; 15:507-14.
- Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM
et al. 2018 AHA/ACC guideline for the management of adults with
congenital heart disease: a report of the American College of
Cardiology/American Heart Association Task Force on Clinical Practice
Guidelines. Circulation. 2019;139: e698–e800
- Baumgartner H, De Backer J, Babu-Narayan SV , Budts W, Chessa M,
Diller GP et al 2020 ESC Guidelines for themanagement of adult
congenital heart disease European Heart Journal (2020) 00, 1_83
- Sundt TM. Guidelines or gospels? J Thorac Cardiovasc Surg. 2016;
151:1472-4.
- ML Jacobs Anomalous aortic origin of a coronary artery: The gaps and
the guidelines J Thorac Cardiovasc Surg 2017; 153:1462-5
- Nees SN, Flyer JN, Chelliah A, Dayton JD, Tourchette L, Kalfa D et al.
Patients with anomalous aortic origin of the coronary artery remain at
risk after surgical repair J Thorac Cardiovasc Surg 2018;155:2554-64
- Courand PY, Bozio A, Ninet J, Boussel L, Bakloul M, Galoin-Bertail C
et al. Diagnosis and treatment of anomalous aortic origin of coronary
artery: A twenty-year retrospective study of experience and
decision-making in children and young adults International Journal of
Cardiology 337 (2021) 54–61
- Gharibeh L, Rahmouni K, Hong SJ, Crean AM, Grau JB. Surgical
Techniques for the treatment of Anomalous Origin of Right Coronary
artery from the left sinus: A comparative reviewe J Am Heart Assoc.
2021 Nov 16;10(22):e022377
- de Leval MR, Migliavacca F, Guadagni G, Dubini G. Computational fluid
dynamics in the evaluation ofhemodynamic performance of cavopulmonary
connections after the Norwood procedure for hypoplastic left heart
syndrome J Thorac Cardiovasc Surg 2003; 126:1040-7
- Razavi A, Sachdeva S, Frommelt PC, La Disa Jr JF . Patient-Specific
Numerical Analysis of Coronary Flow in Children with Intramural
Anomalous Aortic Origin of Coronary Arteries. Semin Thorac Cardiovasc
Surg 2021;33(1):155-167.