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A transseptal coronary artery course, also known as a transconal course, is an anomalous course of the left main coronary artery (LMCA) or the left anterior descending artery (LAD) through the conal septal myocardium. The conal septal myocardium is the posterior wall of the right ventricular outflow tract (RVOT), acting as a dividing myocardial wall between the subaortic and subpulmonary outflow tracts. The initial segment of a transseptal coronary artery has an extraconal course between the aorta and the RVOT cranial to the true intramyocardial segment. The transseptal coronary artery then emerges out of the conal septal myocardium at the epicardial surface on the lateral aspect of the RVOT. Many consider the transseptal coronary artery to be a benign entity. However, there are few case reports of severe cardiac symptoms such as myocardial ischemia, arrhythmia, and even sudden cardiac deaths due to potential coronary artery compression in the systolic phase.​ In this article, we seek to describe the imaging findings of transseptal coronary artery course on coronary computed tomography angiography (CTA), discuss their clinical analysis, and briefly discuss the management of these lesions. [ABSTRACT FROM AUTHOR]

Coronary artery anomalies and their potential sequelae are not well studied in association with stillbirth. Herein, we report the autopsy findings in two term stillborn fetuses with coronary artery anomalies. Both fetuses showed identical findings consisting of an abnormal origin of the left coronary artery from the right sinus of Valsalva and an interarterial course of the left coronary artery. Histologic vascular and myocardial changes were also present. These coronary artery findings are associated with sudden death in adults and neonates, and therefore, their potential to be a cause and/or contributor to fetal death is suspected. [ABSTRACT FROM AUTHOR]

A 14-year-old male with no significant medical history presented with intermittent palpitations for 2–3 months that occurred at rest and were associated with light-headedness. Electrocardiogram in clinic showed sinus arrhythmia with early repolarization and no ischemic changes. The echocardiogram showed normal cardiac structure and function, however, there was a concern for possible anomalous origin of the left coronary artery. Contrast-enhanced CT coronary artery angiogram confirmed a single coronary origin from the right coronary sinus. The single main coronary artery gave rise to the right coronary artery (RCA) and the left coronary artery (LCA). The LCA demonstrated a trans-septal course before it gave rise to the left anterior descending and left circumflex artery. There were intraarterial and intramural portions of the LCA, and the sinoatrial node artery arose from the LCA. The RCA demonstrated a normal course to the right atrioventricular groove, and the posterior descending artery arose from the RCA. Treadmill exercise stress test showed excellent functional capacity without exercise-induced chest pain or ischemic ECG changes. Invasive coronary angiography ruled out luminal narrowing or dynamic compression. Given the absence of physiologic or anatomic evidence of coronary flow restriction, no intervention was pursued and the palpitations were deemed to be likely unrelated to the coronary anomaly and eventually subsided spontaneously on 6 month follow-up.

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Anomalous coronary artery originating from the opposite sinus of Vasalva with interarterial course (ACAOS-IAC) is associated with sudden cardiac death (SCD) in young athletes. If identified in adulthood prognosis is usually more benign, resulting in a dilemma regarding revascularization. This is a retrospective observational single-center study, including adults with ACAOS-IAC. Medical records between 2012 and 2019 were reviewed for management approach, mortality, cardiac death and coronary related adverse events. Coronary computed tomographic angiography (CCTA) were reviewed. We provide a literature review in regard to clinical outcome. We identified 40 patients with ACAOS-IAC (mean age 51). Presentation was acute in 7/40 (18%). Ischemia detection with single photon emission tomography (SPECT), cardiac magnetic resonance (CMR) or dobutamine stress echocardiography were performed in 25/40 (63%) patients. Ischemia in the vascular territory of the anomaly was present in 2/25 (8%). In 39/40 (98%) patients were treated expectative. During median follow-up of 2.7 years (IQR 1.5–5.3) no cardiovascular death was observed. Mortality occurred in 1/40 (3%) and coronary related adverse events in 2/40 (5%). We identified 20 studies describing 1194 patients. Revascularization was performed in 376/1154 (32.6%) patients. Mortality stratified for clinical management was 23/431 (5.3%) in the non-revascularization versus 16/253 (6.3%) in the revascularization group during 4.0 years follow-up (weighted median). Cause of death was cardiovascular in 10/596 (1.7%) in 4.2 years (weighted median) follow up. Both revascularization and non-invasive management have good prognosis in adults with ACAOS-IAC during early follow up. There is need for guidelines and long-term surveillance. • Adults with interarterial coronary artery show benign prognosis treated expectative • Benefit of revascularization for adults not demonstrated in literature • Prognostic value of high-risk anatomic features show conflicting results [ABSTRACT FROM AUTHOR]

Three cases of teenagers with anomalous aortic origin of the coronary arteries (AAOCA) are presented with typical exercise induced symptoms (chest pain, syncope or dizziness). Using multimodal imaging, diagnoses was confirmed showing interarterial and/or intramural course of the coronary artery explaining the ischemia induced symptoms. Successful surgical correction with unroofing of the AAOCA was performed in all three cases with a favorable outcome. Even though AAOCA are rare, some variants may be relevant and potentially life threatening, therefore treating physicians should be aware of correctly diagnosing and treating these individuals.

Anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva is increasingly recognized by cardiac imaging. Although most AAOCA subtypes are benign, autopsy studies report an associated risk of sudden death with interarterial anomalous left coronary artery (ALCA) and anomalous right coronary artery (ARCA). Despite efforts to identify high-risk ALCA and ARCA patients who may benefit from surgical repair, debate remains regarding their classification, prevalence, risk stratification, and management. We comprehensively reviewed 77 studies reporting the prevalence of AAOCA among >1 million patients, and 20 studies examining outcomes of interarterial ALCA/ARCA patients. Observational data suggests that interarterial ALCA is rare (weighted prevalence = 0.03%; 95% confidence interval [CI]: 0.01% to 0.04%) compared with interarterial ARCA (weighted prevalence = 0.23%; 95% CI: 0.17% to 0.31%). Recognizing the challenges in managing these patients, we review cardiac tests used to examine AAOCA and knowledge gaps in management. [ABSTRACT FROM AUTHOR]

The diagnosis of anomalous origin of the left main coronary artery from the right coronary sinus with an interarterial course in children and adolescents is considered life-threatening and clinical guidelines recommend surgical correction. The prognostic implications of this diagnosis in adults are not clear. This anomaly may present in adults as sudden cardiac death or may be diagnosed incidentally. Treatment of this anomaly in adults should be tailored individually taking into account the clinical presentation and patient characteristics. [ABSTRACT FROM AUTHOR]

Certain coronary anomalies are associated with high risk features. We sought to determine the diagnostic accuracy of coronary computed tomographic angiography (CTA) in determining high-risk features, particularly intramural segments. Anomalous coronary arteries can be associated with adverse clinical events. Anomalous coronaries that course between the great vessels (interarterial) have been associated with sudden death. High-risk features of interarterial vessels described in the literature include; a slit-like orifice, acute angle of origin, and intramural segments (within the wall of the aorta). Although computed tomography (CT) findings of acute angle and slit like orifice have been described previously no prior evaluations regarding CT identification of an intramural segment have been reported. An intramural segment has distinct surgical management implications. All interarterial anomalous coronary arteries do not have an intramural segment. Since October 2004, 15 patients were diagnosed by CTA as having an anomalous coronary artery with an interarterial course, which were then confirmed by intraoperative examination of their coronary origins and course during aortic root/coronary artery surgery. The CTA images were retrospectively analyzed for the presence of high-risk features by a radiologist blinded to the surgical findings. Comparison of these findings was made to the findings at surgery. The anomalous coronary was the right coronary artery in 10 patients and the left coronary artery in 5. Eleven patients had an intramural segment identified at surgery. Pre-operative coronary CTA showed that all patients with an intramural course of the anomalous artery, had slit-like orifice, an acute angle of origin (mean 18.4 ± 3.4°), and an elliptical shaped cross-section throughout the intramural segment of the anomalous vessel. The average vessel height/width ratio for anomalous coronary vessels without an intramural segment was 1.03; compared to a ratio of 2.19 for anomalous vessels with an intramural segment ( P = 0.003). Coronary CTA can identify an intramural segment of an anomalous interarterial coronary artery by its elliptical shape. Identifying an intramural segment has important clinical and surgical implications. [ABSTRACT FROM AUTHOR]