Congenital Heart Surgery

Anomalous aortic origin of coronary arteries: is the unroofing procedure always appropriate?

Anomalous aortic origin of coronary arteries: is the unroofing procedure always appropriate? Mostefa Kara M, Fournier E, Cohen S, Hascoet S, Van Aerschot I, Roussin R, El Zoghbi J, Belli E.Eur J Cardiothorac Surg. 2020 Nov 9:ezaa379. doi: 10.1093/ejcts/ezaa379. Online ahead of print.PMID: 33167026   Teaching Points Unroofing is a safe and effective technique for the majority of AAOCA. Surgical correction of AAOCA should be offered to symptomatic patients and high-risk asymptomatic patients. Intramural course is underestimated on CT and should always be rule out intraoperatively.   Commentary from Dr. Charles Laurin (Quebec City, QC, Canada), guest editor of Congenital Heart Surgery Journal Watch: Kara et al, from Marie Lannelongue Hospital, published in the European Journal of Cardio-Thoracic Surgery in November 2020 their last fifteen years experience with anomalous aortic origin of coronary arteries (AAOCA). The retrospective analysis of 39 consecutive cases (median age at surgery of 14 years) aim to define the optimal surgical technique for the majority of AAOCA and to discuss the justification of surgery for all AAOCA, with or without symptoms.   The cohort included 11 anomalous left coronary artery (ALCA) and 28 anomalous right coronary arteries (ARCA), from which respectively 10 (90%) and 21 (75%) were symptomatic. The two patients operated after sudden death episode had ARCA. Preoperative cardiothoracic scans (CT) showed 19 (49%) intramural course (5 ALCA; 15ARCA) and 27 (69%) interarterial course (8 ALCA;19 ARCA). Intraoperative intramural course was noted in 28 patients (8 ALCA;20 ARCA). Unroofing technique was successful in 30 (77%) patients with 11 needing adjunct pulmonary translocation and 3 resuspensions of the inter-coronary commissure. Survival rate was 100% at median time of 4 years (3-6). Three patients needed reoperations for recurrence of symptoms, which all had thrombosed bypass grafts.   With 64% of the cohort operated in the last five years, management guidelines of AAOCA have become more aggressive, especially regarding asymptomatic AAOCA. Surgical treatment has been extended to high-risk anatomy and asymptomatic patient (small lumen area, high degree of proximal stenosis, long interarterial course, small proximal segment width). ARCA was more prevalent and seemed at higher risk of sudden death event. The majority of symptomatic patients had intramural course. Nevertheless, 11 patients had no intramural course, and all were symptomatic. As reported by the authors, AAOCA intramural course was underestimated by preoperative imaging (49% vs 72% intraoperatively), which should warrant intraoperative systematic evaluation for undiagnosed intramural course. Coronary unroofing is a safe technique to address AAOCA with favourable midterm outcomes. Depending on anatomy, alternative techniques can be performed on top of unroofing. Bypass grafts should be avoided because of highly competitive flow with the native coronary artery, which lead to bypass occlusion.    

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The significance of symptoms before and after surgery for anomalous aortic origin of coronary arteries in adolescents and adults

The significance of symptoms before and after surgery for anomalous aortic origin of coronary arteries in adolescents and adults. Meijer FMM, Egorova AD, Jongbloed MRM, Koppel C, Habib G, Hazekamp MG, Vliegen HW, Kies P.Interact Cardiovasc Thorac Surg. 2020 Nov 22:ivaa234. doi: 10.1093/icvts/ivaa234. Online ahead of print.PMID: 33221843   Take Home Points: Most symptomatic patients with anomalous origin of coronary artery have atypical symptoms. Surgical management of anomalous origin of coronary artery disease resolves symptoms in about 60% of patients. Major adverse cardiovascular events are at least 7.5% at a median follow-up of 3 years.   Commentary from Dr. Frederic Jacques (Quebec City, QC, Canada), chief section editor of Congenital Heart Surgery Journal Watch: Meijer et al. published a study in the Interactive CardioVascular and Thoracic Surgery trying to answer: What is the significance of symptoms among adult and teenage patients with anomalous origin of coronary arteries in the preoperative and in the postoperative period? These patients were mainly adults with a mean age 44±15 years. Their follow up, described as mid-term, has a median time of 3 years after surgical correction. They included all their 53 patients referred for surgery of a coronary artery originating from the opposite sinus of Valsalva from 2001 to 2018. Follow up data was available for about two thirds of them. Ninety-six percent of patients had some sort of symptoms before the operation. Almost 80% had suspicion of myocardial ischemia but only 35% of patients had typical angina pectoris prior to surgical correction. The remaining symptomatic patients had aborted sudden cardiac death (6%). For About 15% of patients, the coronary problem was either found during a familial screening or incidentally. Three quarter of the patients had a functional diagnostic test. From a surgical standpoint, almost 75% of patients had an unroofing procedure. Reimplantation, osteoplasty and CABG were also performed. Eight percent of patients had a combination of coronary procedures. Concomitant procedures were performed in 28%. To provide mid-term outcomes, the authors disregarded events occurring immediately after surgery. Only one death was reported. The proportion of symptom-free patient was 59%, which is significantly higher than before surgery. Three patients had typical de novo angina pectoris at follow up which required another intervention.   This is an interesting paper focusing on symptoms related to anomalous coronary origin. As the authors states, anomalous origin of the coronary arteries is a rare disease that may lead to major complications or sudden death in some. However, as the actual denominator of patients living with this condition is unknown and the available corrective strategies are not devoted of complications, no clear consensus was ever established to clinically follow those patients or to surgically treat them. The paper does not answer any question about this denominator. However, it depicts the clinical trajectory of patients who have reached surgical management. It was already known that complications of this surgical management may range from recurrence of symptoms to sudden death. When no symptoms are identified preoperatively, the surgery would be considered prophylactic. As such, to be fully in line with ethics principles, surgeons and cardiologists should prove that intervening on these patients is safer than doing nothing. Here, the question is how successful the surgical management in correcting symptoms of coronary ischemia (96% of patients had some evidence of symptoms (not necessarily typical of myocardial ischemia)) among mostly symptomatic patients. Surgeons and cardiologists should be reassured that intervening on symptomatic patients is likely to help in most of them (59%). This is in line with previous publications on this matter. Having said that, if the success rate of coronary artery bypass grafting were only 60% in the adult acquired coronary disease, it would be considered a failure. It is worth noting that the comparison of pre- and post-operative symptoms was available for only 64% of patients. In other words, we cannot evaluate the resolution or the increase of symptoms in about one third of these patients. This is a major limitation of this study. Regardless, we learn that among those we know about the follow up, at least 1 patient died, and 3 other patients required another intervention. This is concerning because even though the study does not report any stroke or neurological complications and does not report immediate postoperative complication, it already shows a significant complication rate. In truth, from the available data, we know that the classical 3-point major adverse cardiovascular events is at least 7.5%. It is de facto underestimated but again beware that this complication rate that would be considered unacceptable in most contemporary acquired coronary artery disease revascularization trials. This is even more clinically significant as the mean age of the patients of the present cohort would be considered an exceptionally low risk subgroup for acquired coronary artery disease management. Overall, a significant proportion of patients with anomalous origin of coronary artery was helped, but our congenital community should work together to make surgeries and overall management of these patients more sound and safer.   

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Anomalous aortic origin of coronary arteries: an alternative to the unroofing strategy

Anomalous aortic origin of coronary arteries: an alternative to the unroofing strategy.   Gaillard M, Pontailler M, Danial P, Moreau de Bellaing A, Gaudin R, du Puy-Montbrun L, Murtuza B, Haydar A, Malekzadeh-Milani S, Bonnet D, Vouhé P, Raisky O.Eur J Cardiothorac Surg. 2020 Nov 1;58(5):975-982. doi: 10.1093/ejcts/ezaa129.PMID: 32572445   Take Home Points: In anomalous aortic origin of the coronary artery (AAOCA) with an intramural and interarterial course, unobstructed blood flow to the coronary from the appropriate sinus is the desired goal. Although unroofing is the most frequent procedure, alternative surgical techniques may achieve the goal with particular advantages, especially when unroofing is not possible or desirable. Despite success in the surgical treatment of AAOCA with little or no mortality, coronary events and reintervention rates remain significant.     Commentary from Dr. Luis Quinonez (Boston, MA, USA), section editor of Congenital Heart Surgery Journal Watch: This paper describes the experience of Hopital Necker, Paris with the surgical treatment of anomalous origin of coronary artery (AAOCA) from an inappropriate sinus and an interarterial course. Sixty-one patients are included with a median age of 14.7 years (3.7-66.1). There were 40 anomalous right and 21 left coronaries; 5 anomalous left coronaries had an intraseptal course. 70% of the patients were symptomatic, most commonly chest pain. 5 patients had sudden death. Interestingly, of the 34 patient that were tested for inducible ischemia, 41.2% were positive. The surgical techniques describe include osteoplasty (37 patients, 60.7%), which is opening the coronary artery at its exit point in the appropriate sinus and carrying the incision proximally into the aorta and then adding a patch (mostly autologous pericardium; the other technique is coronary translocation (18 patients, 31.1%), where the coronary is transected at its exit point and relocated in the appropriate sinus augmenting the anastomosis with an autologous pericardial patch. The last technique described is one for the intraseptal course (5 patients, 8.2%) where the pulmonary root is harvested like a Ross, the muscle bridge is unroofed, and the autograft is re-implanted. There were no operative deaths. Complications are described. Follow-up was 38 months (1-15 years).   This series has a respectable number of patients in which surgical techniques are presented. The descriptions, illustrations and video are sufficient to understand and reproduce the techniques. Notably, the operations are not unroofings, which is the probably the “standard” approach to AAOCA from an inappropriate sinus. In the operations described, the intramural segment of the anomalous coronary is left untouched. This is important, given the concern that manipulation of the intercoronary commissure risks early or late aortic valve insufficiency. This complication has been brought to the forefront by the recently published Congenital Heart Surgeons Society multicenter report and will likely garner interest in techniques that will avoid the commissure. For the osteoplasty technique, there are now two sources of blood flow to the coronary, the intramural course and the osteoplasty site. Does this put at risk the osteoplasty site (competitive flow) or is the intramural course at risk of thrombosis? Although there were no operative deaths, there were 3 acute post-operative coronary events, all requiring re-intervention and 1 needing ECMO. This is 3 of 61 patients, or 5%, which is not a small number. In the follow-up period there were no reported deaths, yet another 3 patients had re-interventions. Although they report all patients, but one, were asymptomatic, their follow-up testing was incomplete, with only about half the patients having inducible ischemia testing and about 60% having CT imaging, making the ability to draw conclusions limited. A very good part of the paper is the discussion, in which the authors describe the technical lessons learned from their experience. Overall, this paper is worth reading for anyone tackling AAOCA. Although the operations described may be more technically demanding, they are options if unroofing is not possible or desirable.   

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Flow Dynamics in Anomalous Aortic Origin of a Coronary Artery in Children: Importance of the Intramural Segment

Flow Dynamics in Anomalous Aortic Origin of a Coronary Artery in Children: Importance of the Intramural Segment. Hatoum H, Krishnamurthy R, Parthasarathy J, Flemister DC, Krull CM, Walter BA, Mery CM, Molossi S, Dasi LP.Semin Thorac Cardiovasc Surg. 2020 Nov 23:S1043-0679(20)30422-6. doi: 10.1053/j.semtcvs.2020.11.027. Online ahead of print.PMID: 33242612   Take Home Points: 3D printing model helps to assess the coronary flow and fractional flow reserve in ex-vivo. Coronary flow and fractional flow reserve decrease in the intramural portion during the aortic pressure increases. This ex-vivo functional assessment enables us to stratify the risk of myocardial ischemia and sudden death, thereby may help to make a decision for surgical intervention in patients with anomalous aortic origin of right coronary artery without any symptom.     Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), section editor of Congenital Heart Surgery Journal Watch: Hatoum and his colleague published the paper regarding the risk assessment of anomalous aortic origin of a coronary artery (AAOCA) in children. They used 3D printing flow models placed in the aortic position of a pulse duplicator to measure the hemodynamics in the specific anatomy in AAOCA. Main findings from this study was that pressure, fractional flow reserve (FFR), and coronary flow dropped at the site of intramural course of right coronary artery arising from left sinus when the aortic pressure increased in patient with ischemia. The model for patient who had an unroofing repair of AAOCA demonstrated the improved the coronary flow and FFR.   Significance: It is extremely difficult to stratify the risk in patients with AAOCA, especially anomalous right without any symptom, hence surgical indication is controversial. This ex-vivo model represents the unique anatomy of AAOCA and enables to functional assessment without any invasive test.   Comment: Through the clinical experience of the repair of AAOCA, we became to know its unique anatomy and function, yet still not uncertain for risk stratification in each patient because of a wide variety of anatomic characteristics, such as the location of the ostium, the length of intramural course, and the relation to the pulmonary artery. Surgery for left coronary anomaly is recommended even in asymptomatic patients, however, we are still not known which patients should undergo the surgery in anomalous right coronary without any symptoms. Stress test can help to guide us to go for the repair but does not always show positive. The unroofing procedure is relatively simple procedure that experienced congenital surgeon can perform with very low risk, but the creation of new aortic regurgitation after surgery mainly due to manipulation of the commissure is not negligible. The 3D printing patient specific model can make functional assessment possible in ex-vivo and help us to determine the surgical indication if the model demonstrates significant drop in coronary flow and FFR. As AAOCA is very rare condition, nationwide database is necessary to build the guideline in the future. As accumulation of the experience of the treatment of AAOCA, database can also help to provide the tailormade recommendation for the choice of surgical technique in each patient.   

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