Interarterial Course Research Articles

Abstract B042: Chemotherapy in a patient with the malignant inter-arterial course of the right coronary artery A choice between cancer or the heart?

Abstract Introduction: Congenital coronary artery anomalies can increase the risk of sudden cardiac death, especially during exercise. Cancer patients with concurrent coronary artery disease face higher mortality rates due to delayed percutaneous coronary intervention. Furthermore, anthracycline chemotherapy, a common cancer treatment, can worsen pre-existing cardiac abnormalities. Despite the complexity of managing cancer patients with coronary artery anomalies, there is a lack of clear guidelines. This report presents a case of malignant interarterial course of the right coronary artery in a patient with Human T-lymphotropic virus type I-associated adult T-cell leukemia/lymphoma and discusses the challenges of chemotherapy in such cases. Case presentation: A 38-year-old male presented with weakness and palpitations. Laboratory findings revealed abnormalities, including elevated troponin and severe narrowing of the right coronary artery. Electrocardiograms showed ST elevations initially, followed by T-wave inversions. Although the patient had a pre-existing coronary anomaly, chemotherapy was initiated due to life-threatening tumor lysis syndrome and refractory lactic acidosis. Cardiac catheterization or surgical intervention was not possible due to comorbidities. A follow-up echocardiogram after six months of chemotherapy showed no acute changes. Discussion: Managing cancer patients with congenital coronary artery anomalies is challenging. Malignant interarterial course of the right coronary artery is particularly concerning due to the risk of sudden cardiac death. In this case, the patient's symptoms and abnormal electrocardiogram led to the identification of the coronary anomaly. However, immediate intervention was not feasible due to the patient's critical condition. The initiation of anthracycline-based chemotherapy in this patient posed a dilemma. Anthracyclines can cause cardiotoxicity, worsening pre-existing cardiac anomalies. Despite the lack of guidelines, chemotherapy was administered due to the urgent need for cancer treatment. Regular monitoring was essential to detect any cardiac changes. Conclusion: Managing cancer patients with coronary artery anomalies and administering anthracycline chemotherapy remains challenging due to limited guidelines. The case highlights the importance of recognizing and managing these anomalies to reduce the risk of sudden cardiac death. Guidelines for managing cardiac complications in cancer patients with pre-existing anomalies are needed. Further research is necessary to provide evidence-based guidance for such cases. Citation Format: Aliya M. Khan. Chemotherapy in a patient with the malignant inter-arterial course of the right coronary artery A choice between cancer or the heart? [abstract]. In: Proceedings of the 16th AACR Conference on the Science of Cancer Health Disparities in Racial/Ethnic Minorities and the Medically Underserved; 2023 Sep 29-Oct 2;Orlando, FL. Philadelphia (PA): AACR; Cancer Epidemiol Biomarkers Prev 2023;32(12 Suppl):Abstract nr B042.

Abstract B043: Challenges in managing cancer patients with congenital coronary artery anomalies: A case report

Abstract Introduction: Congenital coronary artery anomalies are rare cardiac abnormalities that can increase the risk of sudden cardiac death, particularly during exercise. Patients with cancer and concurrent coronary artery disease face higher mortality risks, as they often receive delayed percutaneous coronary intervention. Additionally, anthracycline chemotherapy, commonly used in cancer treatment, can cause cardiotoxicity and worsen pre-existing cardiac abnormalities. Despite the complexity of managing cancer patients with coronary artery anomalies, there is a lack of clear guidelines and consensus on optimal approaches. This report presents a unique case of a patient with a malignant inter-arterial course of the right coronary artery and newly diagnosed Human T-lymphotropic virus type I-associated adult T-cell leukemia/lymphoma, discussing the challenges and implications of chemotherapy in such cases. Case Presentation: A 38-year-old male presented with weakness and palpitations, exhibiting abnormal laboratory findings. CT angiography revealed a malignant inter-arterial course of the right coronary artery with severe narrowing. Electrocardiograms showed ST elevations and subsequent T-wave inversions. Despite the increased risk of cardiomyopathy due to the coronary anomaly, the patient required chemotherapy for life-threatening tumor lysis syndrome and refractory lactic acidosis. Anthracycline-based chemotherapy was initiated, and a follow-up echocardiogram after six months showed no acute changes. Conclusion: Managing cancer patients receiving anthracycline chemotherapy remains challenging due to limited guidelines. Anthracycline-induced cardiotoxicity is a concern, especially in patients with pre-existing cardiac anomalies like the malignant inter-arterial course of the right coronary artery. This case emphasizes the importance of recognizing and managing such anomalies in cancer patients to reduce the risk of sudden cardiac death. The higher mortality risk in cancer patients with coronary artery disease highlights the need for guidelines in managing anomalous coronary vessels in cancer patients. Further research is necessary to develop evidence-based strategies for managing cardiac complications in cancer patients, particularly those with pre-existing cardiac anomalies. Citation Format: Aliya M. Khan, Sophia Navajas Urisote, Danish A. Sheikh. Challenges in managing cancer patients with congenital coronary artery anomalies: A case report [abstract]. In: Proceedings of the 16th AACR Conference on the Science of Cancer Health Disparities in Racial/Ethnic Minorities and the Medically Underserved; 2023 Sep 29-Oct 2;Orlando, FL. Philadelphia (PA): AACR; Cancer Epidemiol Biomarkers Prev 2023;32(12 Suppl):Abstract nr B043.

Risk factors for coronary artery complications after prosthetic pulmonary valve implantation in congenital heart diseases

Abstract Background The incidence and risk factors for coronary artery complications in patients who underwent pulmonary valve replacement or valved conduit interposition for congenital heart diseases were unknown. Purpose This study was an extension of the previous study† to investigate the incidence and risk factors of coronary artery complications among patients who underwent prosthetic pulmonary valve implantation. Methods Retrospective cohort study was performed for the patients who underwent cardiac CT or invasive coronary angiography after the prosthetic pulmonary valve implantation at the tertiary referral hospital from June 1986 to May 2021. The rotation angle of the aorta and angles of both coronary artery orifices were measured on a Picture Archiving and Communication System (Figure 1). The Cox regression analysis was used to identify variables predicting the occurrence of coronary artery complications. Survival rates were assessed by Kaplan-Meier analysis. Results Coronary artery complications were identified in 25 patients among the 341 patients (7.3%), and two of them died. The median age at diagnosis of coronary artery complications was 20 years (interquartile range: 12.5-25 years). The coronary artery complication-free survival rate was predicted to be 97.3% at 5 years, 92.5% at 10 years, 85.5% at 20 years, and 61.1% at 30 years. The risk of cardiac death was 1.865 times higher [95% CI: 0.403, 8.628] in patients with coronary artery complications than in those without it; nevertheless, the difference was not statistically significant (p-value = 0.425). Among the patients with coronary artery complications, congenital coronary anomalies were identified in 11 (44 %), interarterial course of coronary artery in 18 (72 %), and intramural course in 4 patients (16%). Interarterial course and intramural course of the coronary artery were associated with a 4.4 and 10.6-fold increased risk of coronary artery complications, respectively. When subgroup analysis was performed in patients with tetralogy of Fallot and pulmonary atresia, the aortic root rotated more clockwise in patients with coronary artery compression than in patients without it (128.0°± 19.9° versus 113.5°± 23.7°, p-value = 0.024). The cut-off value of the rotation angle to predict the occurrence of coronary artery compression was 133˚ (Figure 2). Conclusion Preoperative and postoperative long-term coronary assessment should be performed in patients with conotruncal anomalies undergoing prosthetic pulmonary valve implantation, especially in those with abnormal courses of coronary arteries or severe rotation of aortic root. The rotation angle of aorta could be measured on cardiac CT angiography prior to surgery, and the location of the prosthetic valve or conduit should be carefully determined.Measurement of anglesKaplan-Meier curves

Incidence of coronary anomalies in patients with D-transposition of great arteries (D-TGA) after arterial switch operation (ASO)

Abstract Background and purpose D-transposition of great arteries (D-TGA) is a congenital cardiac defect defined by atrioventricular concordance and ventriculo-arterial discordance. Arterial switch operation (ASO) is currently the surgical repair and implies reimplantation of the coronary arteries from the aorta into the neo-aortic root. As such, long-term coronary anomalies and complications are possible and potentially dangerous. Recently, coronary computed tomography angiography (CCTA) has gained a prominent role for characterization of the coronary anatomy in these patients (pts) and its relationship with adjacent structures. However, routine coronary evaluation among asymptomatic pts after ASO remains controverse, as the reported incidence of coronary-related complications is low. The purpose of this study is to perform a descriptive analysis of CCTA findings in pts with D-TGA after ASO. Methods We performed a retrospective single center study that included patients with D-TGA submitted to ASO who underwent CCTA. Demographic, clinical and imaging data were collected. Results We identified 57 pts with D-TGA submitted to ASO. Of these, 39 (68%) performed a CCTA. Mean age of pts was 20.4 (± 3.8) years; 54% were male; 32% had complex D-TGA. In 35 patients (90%), CCTA was performed as part of a routine screening. In 4 (10%) pts CCTA was done due to symptoms or an abnormal non-invasive test suggestive of myocardial ischemia. Eighteen (46%) pts had coronary anomalies: 5 (13%) had an acute angulation at the coronary origin; 13 (33%) pts had anomalous origin of the coronary arteries, ranging from presence of a single coronary artery in 3 (8%) pts, common independent origin from a single sinus of Valsalva in 6 (15%) cases and anomalous origin of the circumflex artery from the right coronary artery (RCA) in 4 (10%) pts. An interarterial course was described in 2 (5%) pts and a retroaortic course was reported in 8 (21%) cases. One patient had anomalous course of the LAD into the right ventricle, a mild calcified ostial stenosis of the left main coronary artery and a chronic total occlusion of the RCA. Conclusions D-TGA patients submitted to ASO have a high incidence of coronary anomalies. Routine screening with CCTA may be justified to identify patients who may be at higher risk of coronary events.

Le registre ANOCOR

Anomalous aortic origin of the coronary arteries are congenital anomalies with many anatomical forms. Due to the varying risk of sudden death, these abnormalities must be classified accurately. There are still questions about the mechanism and individual risk of sudden death, the natural history of these abnormalities and the benefits of a surgical correction. Large-scale observational registries may provide more evidence-based data to practitioners caring for the patients concerned. The ANOCOR registry, the largest in size published to date, enrolled 472 patients (mean age 63 years) with 496 coronary abnormalities. The angiographic representation (with invasive coronary angiography or coronary CT angiography) according to the coronary artery and initial ectopic course could be specified with the identification of two main phenotypes: the circumflex artery (n = 235) with a retroaortic course in 97% of cases and the right coronary artery (n = 165) with an interarterial course in 89.7% of cases. Two left coronary anatomical forms have been confused by non-expert cardiologists: those with a retropulmonary or interarterial course. Sudden death related to coronary anomaly was a very rare mode of presentation (3 patients or 0.6% of the cohort) in this population with very few young patients < 35 years (11 cases or 2.3% of the cohort).

Late follow-up of neo-aortic dimensions and coronary arteries in adult patients after the arterial switch operation

BackgroundAfter the arterial switch operation (ASO) for transposition of the great arteries (TGA), neo-aortic dilatation and coronary arterial anomalies, especially an interarterial course and acute coronary artery take-off angle, are commonly found. Long-term follow-up data after ASO is scarce. Aim of this study was to determine the prevalence of neo-aortic dilatation and coronary abnormalities, with special emphasis on acute coronary take-off angle, in adult TGA-ASO patients. MethodsIn this retrospective cohort study, all adult TGA-ASO patients with ≥1 CT-angiography (CTA) at the age of ≥16 years were included. ResultsEighty-one patients, 69 % male and median age 21.0 [18.5–22.8] years, were included. At baseline, maximum neo-aortic diameter was 39.2 ± 5.3 mm; 35 (43 %) patients had neo-aortic dilatation (neo-aortic diameter of >40 mm), 22 (27 %) patients had an acute coronary take-off angle (<30°), and 5 (6 %) patients had an interarterial course of the RCA (2 %) or LCA (4 %). Neo-aortic or coronary artery re-intervention occurred in 10 (12 %) patients. All 10 patients had neo-aortic dilatation or coronary take-off angle of <30° on baseline CTA. ConclusionThis study reports a prevalence of 43 % of neo-aortic dilatation, 6 % of interarterial coronary course and 27 % for acute coronary take-off angle (<30°) at a median term of 21.0 years post ASO. All patients with a neo-aortic re-intervention or coronary artery re-intervention during follow-up had a maximum neo-aortic diameter of >40 mm or a coronary take-off angle of <30° at baseline CTA. This hypothesis generating study suggests that an active surveillance in patients with neo-aortic dilation and/or an acute angulation of < 30° post ASO might be considered and requires prospective evaluation.

Management of anomalous origin of right coronary artery from left coronary sinus

Aberrant origin of coronary artery is a rare congenital anomaly associated with increased risk of myocardial ischemia and sudden death, with the highest risk lesions being those arising from the opposite sinus of Valsalva. We report a case with an aberrant right coronary artery arising superior to the left coronary cusp, with a slit-like ostium, having an inter-arterial and intramural course through the aortic root, that underwent repositioning of the right coronary artery. We believe such cases warrant surgical correction and reimplantation is a safe, effective and reproducible technique.

Anomalous origin of left circumflex artery from the right sinus of Valsalva: Clinical outcomes in a consecutive series of master athletes.

Aim of the study was to collect and describe a case series of consecutive master athletes in whom an anomalous origin of left circumflex artery (LCx) from the right sinus of Valsalva (ALCx) was detected at a clinically indicated coronary computed tomography angiography CCTA) to establish a focused clinical management and counseling about sport activity in those subjects. We analyzed a prospective registry of subjects referred to a clinically indicated CCTA. Information about the clinical status was obtained by previous clinical records and clinical evaluation at time of image acquisition; follow-up allowed to record symptoms, outcomes and downstream testing. The study population consisted in 14 subjects, of which one competitive athlete and 13 recreational master athletes. Mean age was of 67.2 ± 10.6 years (71% of male); follow-up lasted 6.4 ± 2.6 years. The major high-risk anatomy features (inter-arterial course, intramural segment, high take-off and slit-like ostium) were absent. None had abnormal ostial morphology and all had full retroaortic course; three subjects (21%) presented an acute take-off angle. Coronary artery disease (CAD) was present in 10 patients (71%). Major outcomes (cardiac hospitalization, death for all causes) recorded were not related to the anomalous LCx. Symptoms were most related to atherosclerotic CAD in different vessels whereas two subjects without CAD exhibited cardiac symptoms, without hospitalization. Our study suggests that the diagnosis of ALCx, being usually associated to low-risk anatomical characteristics, could be considered a benign finding, with scarce or no implications for physically active individuals neither for recreational athletes.

A Case Report of Primary Angioplasty: The Triple Whammy!

Congenital anomalies involving coronary artery origin are rare, with an incidence of 0.24%–1.3%. We present a case of inferior wall ST-elevation myocardial infarction in which an emergency coronary angiogram showed occlusion of the right coronary artery. However, we were unable to cannulate the left main coronary artery during the event. Interpretation of the infarct-related artery was complicated by the right arm-left arm lead reversal in electrocardiogram, adding to the dilemma in localizing the culprit vessel, namely right versus left circumflex coronary artery-related myocardial infarction. A computed tomography coronary angiogram subsequently showed the left main coronary artery having abnormal origin from the right sinus of Valsalva with inter-arterial course and no lesions in the left circumflex.

Transcatheter aortic valve implantation in a patient with anomalous right coronary artery originating from the left aortic sinus with interarterial course.

Transcatheter aortic valve implantation in a patient with anomalous right coronary artery originating from the left aortic sinus with interarterial course.

A Survey on the Management of Anomalous Aortic Origins of the Coronary Arteries.

The management of patients with an anomalous aortic origin of a coronary artery (AAOCA) remains controversial despite the publication of the 2017 American Association for Thoracic Surgery (AATS) expert guidelines. We surveyed the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery and the Pediheart.net online community regarding their care of patients with anomalous origins of the right or left coronary from the opposite cusp with inter-arterial courses and compared them to the AATS guidelines. We received 111 complete responses. Four notable variations from the AATS recommendations were identified. Respondents were more likely to use ECG exercise testing than the stress imaging recommended in the AATS guidelines. For a 16-year-old with AAOCA, recommendations for surgery generally followed the AATS guidelines. However, for asymptomatic left AAOCA without signs of ischemia on stress imaging, only 69.4% felt surgery was appropriate or somewhat appropriate. In the setting of a 16-year-old with right AAOCA free from signs or symptoms of ischemia, respondents were more likely to recommend surgery if the patient was a competitive athlete, a topic not directly addressed in the AATS guidelines. After surgical treatment of AAOCA, only 24% of respondents recommended lifelong antiplatelet therapy despite recommendations for this in the AATS guidelines. Respondents recommendations were generally consistent with the 2017 AATS guidelines but with important variations in the use of stress imaging, indications for surgery in asymptomatic left AAOCA, the impact of identification as a competitive athlete and duration of postoperative antiplatelet therapy.

Comparison of CT-FFR to invasively measured FFR adenosine and FFR dobutamine in patients with an anomalous aortic origin of a right coronary artery

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): FNSNF - Schweizerischer Nationalfonds zur Förderung der wissenschaftlichen Forschung. Introduction Anomalous aortic origin of a right coronary artery (AAORCA) is a rare congenital anomaly. Malignant AAORCA with an interarterial course between the great arteries inherit an anticipated higher risk for myocardial ischemia. Anatomical high-risk features are beside the interarterial course the slit-like ostium (SLO), elliptic vessel shape (EVS), proximal narrowing (PN), acute take-off angle (ATOA) and an intramural course (IM) inside the aortic wall. The complex anatomy lead to both a fixed and further dynamic component of anomalous vessel compression. The suggested gold-standard for the assessment of combination (i.e. fixed and dynamic component) is invasively measured fractional flow reserve (FFR) during dobutamine and volume challenge, whereas FFR under adenosine may only cover the fixed components. A possible non-invasive alternative for FFR assessment is novel CT-FFR method, which can calculate FFR from coronary computed tomography angiogram (CCTA) images using computational fluid dynamics (CFD). Purpose CT-FFR may be used as a non-invasive alternative to invasive FFR measurements. Our aim was to compare CT-FFR to invasively measured FFRadenosine and FFRdobutamine in patients with AAORCA. Methods Consecutive patients enrolled in the NARCO trial (i.e. Noninvasive anatomical assessment for ruling out hemodynamically relevant coronary artery anomalies – A comparison of coronary-CT to invasive coronary angiography), with AAORCA undergoing CCTA and invasively measured ischemia assessment were included. All patients underwent CCTA for quantification of anatomical high-risk features and analysis of CT-FFR, invasive coronary angiography using FFR with adenosine (140μg/kg/min) and secondly with dobutamine and volume challenge (40 μg/kg/min + saline: 1.5–3 l+ atropine: 1 mg). Comparison between different methods was performed. Results In total, 29 consecutive patients with an AAORCA with an interarterial course (56 ± 13 years old), 22 (76%) male patients who underwent both CCTA and invasive coronary angiography were included. SLO was present in 18 (62%), PN in 5 (17%), EVS in 29 (100%) and an ATOA in 29 (100%) patients. An IM was present in 28 (97%) with a mean length of 11 ± 4 mm. While there was no difference between CT-FFR and FFRadenosine (p = 0.656), the mean invasive measured FFRdobutamine (0.85 ± 0.11), was significantly lower than FFRadenosine (0.88 ± 0.10, p = 0.002) and trend towards lower values than CT-FFR (0.89 ± 0.06, p = 0.063). Conclusion In patients with AAORCA, CT-FFR shows no difference with FFRadenosine, but they both overestimate FFR compared to FFRdobutamine gold-standard. Hence, there is a need for CCTA derived CFD FFR models, including dynamic components and mimicking FFR under dobutamine stress.

CT-derived anatomic high-risk features in patients with anomalous aortic origin of a right coronary artery correlate with an impaired FFR assessed by computational fluid dynamics

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): FNSNF - Schweizerischer Nationalfonds zur Förderung der wissenschaftlichen Forschung. Introduction Anomalous aortic origin of a right coronary artery (AAORCA) are a rare subtype of coronary artery anomalies, which occur with a prevalence of 0.23% in the general population. Especially, AAORCA with an interarterial course between the aorta and the pulmonary artery confer high risk of myocardial ischaemia. The interarterial course is however only a surrogate marker for other anatomic high-risk-features (HRF) such as intramural course (IMC), slit-like ostium (SLO), proximal narrowing (PN), acute take-off angle (ATOA) and proximal elliptic vessel shape (PEV). Computed tomography-derived fractional flow reserve (CT-FFR) is a novel tool, which allows noninvasive assessment of haemodynamics, so far assessed in patients with atherosclerotic stenosis. Our aim was to investigate the correlation of HRF in AAORCA with CT-FFR of the anomalous vessel. Purpose In patients with newly detected AAORCA, it is important to evaluate the underlying mechanisms of hemodynamic relevance and currently it is unknown how- and which anatomical high-risk features correlate with ischemia in noninvasively assessed CT-FFR. Methods All patients with AAORCA, who underwent CT imaging between 12/2008 and 9/2022, were retrospectively enrolled. HRF were evaluated from CT images as following: IMC (i.e. length ≥5mm), SLO (i.e. stenosis grade of the proximal lumen compared to the distal lumen ≥50%), PN (i.e. stenosis grade of the proximal lumen to the first round lumen ≥50%), ATOA (i.e. &amp;lt;45°) and PEV (i.e. length/width ratio &amp;gt;1.3). Patients were divided into group 1 (≥4HRF) and group 2 (&amp;lt;4HRF). FFR was calculated using computational fluid dynamics (CT-FFR) using Siemens CT Research cFFR and the cut-off &amp;lt; 0.8 was considered hemodynamically relevant and correlations were performed. Results In total 47 patients, 56.7±14.2years, thereof 34 (72%) male were included in the final analysis. Mean CT-FFR for the anomalous vessel in the whole cohort was 0.93±0.05. IML, ATOA and PEV were present in all patients, whereas SLO was in 22 (46%) and PN in 11 (23%) present. 25 (53%) patients showed ≥4 HRF. In group 1 (≥4HRF), mean CT-FFR within the anomalous vessel was significantly lower compared to group 2 (&amp;lt;4HRF) with 0.91 versus 0.95 (p = 0.002). Three patients (6%) showed a CT-FFR&amp;lt;0.8 and were all within group 1. CT-FFR correlated significantly with SLO (r=-0.348, p = 0.017), but not with other HRF. Conclusion Patients with AAORCA and ≥4HRF showed lower CT-FFR values of the anomalous vessel compared to those with less high-risk features (i.e. &amp;lt;4HRF). A minority of patients showed abnormal CT-FFR, all within the group with ≥4HRF. From all HRF, only SLO correlated with CT-FFR. Future studies need to assess how the combination of anatomical high-risk features and CT-FFR can be used for decision-making towards revascularization in AAORCA patients.

Abnormal origin of the coronary arteries by echocardiography. And then what?

Abstract Funding Acknowledgements Type of funding sources: None. Introduction The anomalous origin of the coronary arteries from the opposite sinus (AOCA) is increasingly recognized. The most frequent diagnosed by echocardiography is the circumflex artery arising from the right sinus of Valsalva with a retroaortic course. Classically is believed to be a low-risk malformation, nevertheless, whether this anomaly is associated with other high risk features (coexistence with other high-risk AOACA, acute take off angle, intramural course, etc) not easily identify by echocardiography is unknown. Objectives The objectives of this study are to know the prevalence of anomalies of the origin of the coronary arteries diagnosed by echocardiography, to determine the use of additional diagnostic tests and to evaluate the coexistence of the anomalous origin of the circumflex artery with other anomalies and / or risk characteristics. Materials and methods A retrospective search of AOCA diagnosis was performed among echocardiography reports of outpatients in a high complexity cardiology center between March 2018 and December 2021. The clinical history was investigated to identify if the AOCA diagnosis led to further testing (coronary Angio CT or Ischemia diagnosis test) and if these studies provided clinically relevant information. Results Of a total of 26260 echocardiogram reports investigated, 50 presented suspected AOCA (0. 2%), 49 circumflex arteries with anomalous arising from the right sinus with retroaortic course and 1 left coronary artery arising from the right sinus with a subpulmonary course. 18 coronary CT angiography (36%) and 13 stress tests (26%) were requested. In 17 of 18 patients (98%) coronary CT angiography confirmed the diagnosis of anomalous origin of circumflex artery arising from the right sinus with retroaortic course (true positive). In 4 of the 17 OAAC confirmed by CT angiography, the study provided additional important information (2 associated anomalies of the origin of the anterior descending, 1 interarterial course and 1 circumflex artery arising from the right sinus with a retroaortic course and an angle take-off from the aortic wall ‹45°). Conclusion The diagnosis of AOCA by echocardiography is an uncommon finding and most of them are low risk. The most frequent variant is the circumflex artery arising from the right sinus with a retroaortic course. CT angiography adds additional information by identifying associated abnormalities of other coronary arteries and evaluating the angulation of the origin of abnormal coronary arteries.

Anomalous aortic origin of right coronary artery from left coronary cusp: a management conundrum: a case report

BackgroundCoronary artery anomalies are characterized by an abnormality in the course or origin of three main coronary arteries. There needs to be more scientific evidence to promptly treat coronary artery anomalies with poorly understood prognostic implications, especially anomalous aortic origin of the right coronary artery from the left coronary cusp.Case presentationA 58-year-old Caucasian female presented multiple times over 6 months with atypical chest discomfort and palpitations. The treadmill exercise test demonstrated exercise-induced non-sustained ventricular tachycardia. A coronary angiogram revealed no obstructive coronary artery disease and an anomalous aortic origin of the right coronary artery from the left coronary cusp with an interarterial course. She was managed conservatively with medications, despite persistent recurrent symptoms.ConclusionIt is essential to identify subtle symptoms and insidious onset of anomalous aortic origin of the right coronary artery symptoms as seen in our patient, which can contribute to significant morbidity. There are discrepancies in existing guidelines between different cardiovascular societies in managing selected subgroups of patients with anomalous aortic origin of the right coronary artery who do not have high-risk features, but continue to remain symptomatic.

New Application of FFRCT in Clinical Practice: Evaluation of Interarterial Anomalous Coronary Course

New Application of FFRCT in Clinical Practice: Evaluation of Interarterial Anomalous Coronary Course

New Application of FFRCT in Clinical Practice: Evaluation of Interarterial Anomalous Coronary Course

New Application of FFRCT in Clinical Practice: Evaluation of Interarterial Anomalous Coronary Course

Clinical Presentation and Medium-Term Outcomes of Children With Anomalous Aortic Origin of the Left Coronary Artery: High-Risk Features Beyond Interarterial Course

Anomalous aortic origin of the left coronary artery (AAOLCA) confers a rare, but significant, risk of sudden cardiac death in children. Surgery is recommended for interarterial AAOLCA, and other subtypes considered benign. We aimed to determine the clinical characteristics and outcomes of 3 AAOLCA subtypes. All patients with AAOLCA <21 years old were prospectively enrolled (December 2012-November 2020), including group 1: AAOLCA from the right aortic sinus with interarterial course, group 2: AAOLCA from the right aortic sinus with intraseptal course, and group 3: AAOLCA with a juxtacommissural origin between the left and noncoronary aortic sinus. Anatomic details were assessed using computed tomography angiography. Provocative stress testing (exercise stress testing and stress perfusion imaging) was performed in patients >8 years old or younger if concerning symptoms. Surgery was recommended for group 1 and in select cases in group 2 and group 3. We enrolled 56 patients (64% males) with AAOLCA (group 1, 27; group 2, 20; group 3, 9) at median age of 12 years (interquartile range, 6-15). Intramural course was common in group 1 (93%) compared with group 3 (56%) and group 2 (10%). Seven (13%) presented with aborted sudden cardiac death (group 1, 6/27; group 3, 1/9); 1 (group 3) with cardiogenic shock. Fourteen/42 (33%) had inducible ischemia on provocative testing (group 1, 32%; group 2, 38%; group 3, 29%). Surgery was recommended in 31/56 (56%) patients (group 1, 93%; group 2, 10%; and group 3, 44%). Surgery was performed in 25 patients at a median age 12 (interquartile range, 7-15) years; all have been asymptomatic and free from exercise restrictions at median follow-up of 4 (interquartile range, 1.4-6.3) years. Inducible ischemia was noted in all 3 AAOLCA subtypes while most aborted sudden cardiac deaths occurred in interarterial AAOLCA (group 1). Aborted sudden cardiac death and cardiogenic shock may occur in AAOLCA with left/nonjuxtacommissural origin and intramural course, thus also deemed high-risk. A systematic approach is essential to adequately risk stratify this population.

Unroofing of an anomalous right coronary artery originating from the left coronary sinus

Coronary abnormalities, including the anomalous aortic origin of a coronary artery, coronary fistula and myocardial bridge, are among the most common congenital cardiovascular anomalies. A left coronary artery arising from the right cusp is less common than the right coronary artery arising from the left cusp but is more often found in autopsy series of sudden cardiac deaths. A slit-like/fish-mouth-shaped orifice, acute angle take-off, intramural course, interarterial course and hypoplasia of the proximal coronary artery have all been proposed as reasons for symptoms, ischaemia and sudden cardiac death. Surgical intervention is recommended for those patients with signs or symptoms of myocardial ischaemia. Intervention in asymptomatic patients with an interarterial/intramural left coronary artery from the right sinus of Valsalva is recommended due to the higher calculated risk of sudden cardiac death. In asymptomatic patients with an intramural right coronary artery from the left sinus of Valsalva, provocative testing is recommended. The slit-like orifice is more commonly seen in an anomalous right coronary artery arising from the left sinus, and we believe it is the major factor responsible for myocardial ischaemia. Our unroofing technique focuses on: (i) transecting the endothelial tissue flap to create a neo-ostium; (ii) limiting the flap resection to the intramural portion to avoid extra-aortic incision; and (iii) marsupialization of a neo-ostium with interrupted sutures to reapproximate the endothelium and prevent aortic dissection.

Right Coronary Artery Origin from Left Sinus of Valsalva with an Inter-arterial Course: Case Report and Review of Literature

Introduction: Overally the anomaly of the coronary arteries are rare. Its incidence according to angiographic, autopsy and echocardiographic findings, are about 1%, 0.29% and less than 0.1% respectively. Usually these are the incidental finding during angiography without clinical presentation. These anomalies may be accompanied by myocardial ischemia, arrhythmia, syncope and sudden death. Case Presentation: We present a 14-year-old Iranian boy who experienced chest pain and shortness of breath after engaging in physical activity. The right coronary artery (RCA) had an intramural and inter-arterial course and was proven to have originated from the left sinus of Valsalva by computed tomographic coronary angiography (CTCA). The RCA began adjunct to the ostium of the left main coronary artery and coursed intramural at first and then continued between the aorta and pulmonary artery. The patient underwent corrective surgery. The RCA was cut from origin and reanastomosd to the right coronary sinus after preparing the sinus. The patient recovered well and he was discharged with the ability to perform sports activity. One year Follow up of the patient was uneventful. Conclusions: Even though single ostium coronary artery abnormalities are uncommon, they have become more frequently identified in recent years using non-invasive techniques such transthoracic echocardiography, which is then followed by CT angiography or conventional angiography. These patients are susceptible to sudden death, so attention to symptoms of the patients and comprehensive approach to them can be lifesaving.