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      Misconception of ‘malignant’ and ‘scissor-like compression’ of interarterial course in anomalous aortic origin of a coronary artery: a case series

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          Abstract

          Background

          The notion that the ‘interarterial’ segment of anomalous aortic origin of a coronary artery (AAOCA) is ‘malignant’ and ‘scissor-like’ compressed between the aorta and pulmonary artery (PA) is debated, owing to the lower pressure in the pulmonary system compared with that in the coronary system. However, data supporting or refuting this belief under stress conditions are lacking.

          Case summary

          Three cases of right AAOCA with interarterial/intramural courses (52, 66, and 51 years old) were assessed. Invasively measured fractional flow reserve (FFR) under dobutamine was 0.85, 0.82, and 0.81, respectively. Intravascular ultrasound illustrated lateral vessel compression of the intramural course with a decrease of minimal lumen area (MLA) (i.e. 5.71–3.47 mm 2, 5.88–4.00 mm 2, and 5.85–4.06 mm 2) under stress conditions with heart rates of 130, 140, and 150 b.p.m., respectively. Pulmonary artery pressure (PAP) increased from rest {s/d (m) [systolic/diastolic (mean)] 22/11 (15), 15/2 (5), and 24/6 (14) mmHg} to stress [s/d (m) 47/24 (36), 30/3 (11), and 36/22 (24) mmHg] and remained below aortic peak pressure (blood pressure, BP) rest [s/d (m) 116/64 (91), 94/48 (71), 99/53 and (62) mmHg]; BP stress [s/d (m) 142/63 (80), 123/63 (88), and 86/46 (62) mmHg]; coronary pressure (CoP) rest [s/d (m) 100/59 (80), 80/45 (62), and 83/47 (63) mmHg]; and CoP stress [s/d (m) 95/60 (69),101/54 (72), and 70/32 (50) mmHg].

          Conclusion

          This case series challenges the assumption that the interarterial segment of AAOCA is scissor-like compressed by both the aorta and PA. The decrease in MLA and FFR under stress is due to the aorta’s unidirectional lateral compression on the intramural segment. Additionally, the term ‘malignant’ should not be universally applied to all AAOCA cases with an interarterial course, as not all result in haemodynamic significance.

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          Most cited references19

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          Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 1980-2006.

          Sudden deaths in young competitive athletes are highly visible events with substantial impact on the physician and lay communities. However, the magnitude of this public health issue has become a source of controversy. To estimate the absolute number of sudden deaths in US competitive athletes, we have assembled a large registry over a 27-year period using systematic identification and tracking strategies. A total of 1866 athletes who died suddenly (or survived cardiac arrest), 19+/-6 years of age, were identified throughout the United States from 1980 to 2006 in 38 diverse sports. Reports were less common during 1980 to 1993 (576 [31%]) than during 1994 to 2006 (1290 [69%], P<0.001) and increased at a rate of 6% per year. Sudden deaths were predominantly due to cardiovascular disease (1049 [56%]), but causes also included blunt trauma that caused structural damage (416 [22%]), commotio cordis (65 [3%]), and heat stroke (46 [2%]). Among the 1049 cardiovascular deaths, the highest number of events in a single year was 76 (2005 and 2006), with an average of 66 deaths per year (range 50 to 76) over the last 6 years; 29% occurred in blacks, 54% in high school students, and 82% with physical exertion during competition/training, whereas only 11% occurred in females (although this increased with time; P=0.023). The most common cardiovascular causes were hypertrophic cardiomyopathy (36%) and congenital coronary artery anomalies (17%). In this national registry, the absolute number of cardiovascular sudden deaths in young US athletes was somewhat higher than previous estimates but relatively low nevertheless, with a rate of <100 per year. These data are relevant to the current debate surrounding preparticipation screening programs with ECGs and also suggest the need for systematic and mandatory reporting of athlete sudden deaths to a national registry.
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            Sudden cardiac death associated with isolated congenital coronary artery anomalies.

            Congenital coronary anomalies are associated with sudden death and exercise-related death. Clarification of the risk and mechanisms of sudden death in patients with coronary anomalies may aid in decisions on intervention. The clinicopathologic records of 242 patients with isolated coronary artery anomalies were reviewed for information on mode of death and abnormalities of the initial segment (acute angle takeoff, valvelike ridges or aortic intramural segments) and course of the anomalous coronary artery. Cardiac death occurred in 142 patients (59%); 78 (32%) of these deaths occurred suddenly. Of sudden deaths, 45% occurred with exercise. Sudden death (28 of 49, 57%) and exercise-related death (18 of 28, 64%) were most common with origin of the left main coronary artery from the right coronary sinus. Anomalous origin of the right coronary artery from the left coronary sinus was also commonly associated with exercise-related sudden death (6 of 13 sudden deaths, 46%). High risk anatomy involved abnormalities of the initial coronary artery segment or coursing of the anomalous artery between the pulmonary artery and aorta. Younger patients (less than or equal to 30 years old) were significantly more likely than older patients (greater than or equal to 30 years old) to die suddenly (62% vs. 12%, p = 0.0001) or during exercise (40% vs. 2%, p = 0.00001) despite their low frequency of significant atherosclerotic coronary artery disease (1% vs. 40%, p = 0.00001). Younger patients (less than or equal to 30 years old) with an isolated coronary artery anomaly are at risk of dying suddenly and with exercise. Therefore, greater effort for early detection and surgical repair of these lesions is warranted.
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              Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes.

              The purpose of this study is to characterize the clinical profile and identify clinical markers that would enable the detection during life of anomalous coronary artery origin from the wrong aortic sinus (with course between the aorta and pulmonary trunk) in young competitive athletes. Congenital coronary artery anomalies are not uncommonly associated with sudden death in young athletes, the catastrophic event probably provoked by myocardial ischemia. Such coronary anomalies are rarely identified during life, often because of insufficient clinical suspicion. However, since anomalous coronary artery origin is amenable to surgical treatment, timely clinical identification is crucial. Because of the paucity of available data characterizing the clinical profile of wrong sinus coronary artery malformations, we reviewed two large registries comprised of young competitive athletes who died suddenly, assembled consecutively in the U.S. and Italy. We reported 27 sudden deaths in young athletes, identified solely at autopsy and due to either left main coronary artery from the right aortic sinus (n = 23) or right coronary artery from the left sinus (n = 4). Each athlete died either during (n = 25) or immediately after (n = 2) intense exertion on the athletic field. Fifteen athletes (55%) had no clinical cardiovascular manifestations or testing during life. However, in the remaining 12 athletes (45%) aged 16 +/- 7, certain clinical data were available. Premonitory symptoms had occurred in 10, including syncope in four (exertional in three and recurrent in two, 3 to 24 months before death) and chest pain in five (exertional in three, all single episodes, < or =24 months before death). All cardiovascular tests were within normal limits, including 12-lead electrocardiogram (ECG) pattern (in 9/9), stress ECG with maximal exercise (in 6/6) and left ventricular wall motion and cardiac dimensions by two-dimensional echocardiography (in 2/2). With regard to congenital coronary artery anomalies of wrong aortic sinus origin in young competitive athletes, 1) standard testing with ECG under resting or exercise conditions is unlikely to provide clinical evidence of myocardial ischemia and would not be reliable as screening tests in large athletic populations, 2) premonitory cardiac symptoms not uncommonly occurred shortly before sudden death (typically associated with anomalous left main coronary artery), suggesting that a history of exertional syncope or chest pain requires exclusion of this anomaly. These observations have important implications for the preparticipation screening of competitive athletes.
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                Author and article information

                Contributors
                Role: Handling Editor
                Role: Editor
                Role: Editor
                Role: Editor
                Journal
                Eur Heart J Case Rep
                Eur Heart J Case Rep
                ehjcr
                European Heart Journal. Case Reports
                Oxford University Press (UK )
                2514-2119
                August 2024
                30 July 2024
                30 July 2024
                : 8
                : 8
                : ytae380
                Affiliations
                Department of Cardiology, Inselspital, Bern University Hospital, University of Bern , Freiburgstrasse, CH - 3010 Bern, Switzerland
                Department of Cardiology, Inselspital, Bern University Hospital, University of Bern , Freiburgstrasse, CH - 3010 Bern, Switzerland
                Department of Cardiology, Inselspital, Bern University Hospital, University of Bern , Freiburgstrasse, CH - 3010 Bern, Switzerland
                Department of Cardiology, Inselspital, Bern University Hospital, University of Bern , Freiburgstrasse, CH - 3010 Bern, Switzerland
                Department of Cardiology, Inselspital, Bern University Hospital, University of Bern , Freiburgstrasse, CH - 3010 Bern, Switzerland
                Author notes
                Corresponding author. Tel: +41 31 632 50 00, Email: christoph.graeni@ 123456insel.ch

                Conflict of interest: Dr C.G. received funding from the Swiss National Science Foundation, Innosuisse; the Center for Artificial Intelligence, University of Bern; Novartis biomedical research foundation; Swiss Heart Foundation; and GAMBIT foundation, outside of the submitted work. Dr. L.R. received funding from the Swiss National Science Foundation outside of the submitted work. None of the author authors reported conflict of interest and there was no additional funding for this case report.

                Author information
                https://orcid.org/0000-0002-3405-5443
                https://orcid.org/0000-0003-0824-3026
                https://orcid.org/0000-0001-6029-0597
                Article
                ytae380
                10.1093/ehjcr/ytae380
                11310694
                39132298
                9aca445c-eaa8-450a-bae6-c080264160af
                © The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 05 April 2024
                : 20 May 2024
                : 22 July 2024
                : 09 August 2024
                Page count
                Pages: 6
                Funding
                Funded by: Swiss National Science Foundation, DOI 10.13039/501100001711;
                Categories
                Case Series
                AcademicSubjects/MED00200
                Eurheartj/31
                Eurheartj/32
                Eurheartj/39
                Eurheartj/41
                Eurheartj/15
                Eurheartj/17
                Eurheartj/18

                coronary anomaly,anomalous aortic origin of a coronary artery,scissor-like,interarterial course,sudden cardiac death,mini case series

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