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There are fewer than 100 reported cases of congenital left main coronary artery atresia. In this report, we present an adolescent male presenting with exertional syncope in the setting of this rare coronary defect, and review important diagnostic and therapeutic considerations imperative to obtain a favorable outcome., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

A one-year-old male intact American bulldog was presented for evaluation of previously diagnosed pulmonary stenosis. Echocardiography identified ultra-severe stenosis with an instantaneous trans-pulmonary pressure gradient of 240 mmHg. Angiography confirmed the presence of an anomalous coronary artery with a prepulmonic course of the left coronary artery arising from a single right coronary ostium consistent with a type R2A coronary anomaly. A trans-pulmonary stent was successfully placed transvenously with diameter sizing based on coronary compression testing. No coronary compression was present on postimplantation angiography. A marked reduction in the pressure gradient was obtained on postoperative echocardiography (reduction to 68 mmHg), despite selecting a stent diameter less than the pulmonary annulus diameter. This is the first report of the use of coronary compression testing in transvenous trans-pulmonic stent implantation in a dog with a type R2A coronary artery anomaly. Selection of a stent diameter less than the pulmonary annulus diameter conveyed a clinically relevant reduction in the trans-pulmonic pressure gradient while avoiding coronary compression in this case., Competing Interests: Conflict of Interest Statement The authors declare no conflicts of interest., (Published by Elsevier B.V.)

Objective: This study aims to present the midterm outcomes of surgical correction of the anomalous left coronary artery from the pulmonary artery (ALCAPA)., Methods: This is a retrospective study of patients undergoing anomalous origin of the LCA from the pulmonary artery repair between 2010 and 2019., Results: Forty-nine patients (20 boys and 29 girls) underwent ALCAPA repair. Patients were divided into two groups based on their age at ALCAPA repair: infant (< 1 year of age: n = 24) and non-infant ( ≧ 1 year of age: n = 25). Median age at time of repair was 23 months(7-60months). LCA reimplantation was performed in 47 patients, and Takeuchi repair was performed in 2 patients. Hospital mortality in the infant group was 8.2% (4 of 49). Infant group had significantly lower LVEF in pre-operation (p < 0.05), but there was not significantly different between the two groups about LVEF at discharge. The median follow-up duration was 43(18-85)months. The freedom from reoperation was not significantly different between two groups (infants vs. non-infants: 68.8% vs. 87.5% at 10 years; p = 0.096)., Conclusions: Surgical treatment of ALCAPA had an excellent early and midterm outcomes. Left ventricular dysfunction in pre-operation was the main risk of mortality in-hospital. The freedom from reoperation did not differ significantly between infant group and non-infant group., (© 2024. The Author(s).)

Coronary artery anomalies (ANOCOR) are congenital anomalies with various anatomical forms. Percutaneous treatment can be offered in certain situations, most often to address associated atherosclerotic disease or, more rarely, to correct a congenital stenosis. Due to the frequent difficulties of catheterization, percutaneous coronary interventions for ANOCOR are recognized as complex procedures. A thorough anatomical understanding facilitates the identification of the connection site and the initial ectopic course of an ANOCOR during coronary angiography. Selecting an appropriate catheter is a crucial step in the procedure. There is a higher prevalence of atherosclerotic disease along retroaortic courses compared to other ectopic courses. When treating atherosclerotic stenosis downstream of an ectopic course, techniques typically used for complex coronary procedures can be helpful. While angioplasty for congenital stenosis is technically feasible, its role in management algorithms remains to be defined. Currently, this type of percutaneous treatment may be offered to right ANOCOR with interarterial course in adults over 35 years old and with ischemic symptoms or myocardial ischemia., Competing Interests: Conflits d'intérêts Les auteurs déclarent n'avoir aucun conflit d'intérêts en lien avec ce travail., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Spontaneous coronary artery dissection (SCAD) is a pathophysiologically heterogenous, infrequent but high-risk cause of acute coronary syndrome in a young, unsuspecting population without traditional cardiovascular risk factors. Diagnosis requires a high index of clinical suspicion, knowledge of the angiographic features of SCAD, and comfort with using the necessary ancillary diagnostic tools. Although most of the patients are medically managed, a small percentage do require revascularization. Knowing the high risk of complications and failure rate associated with SCAD percutaneous coronary intervention, interventional cardiologists should familiarize themselves with the recommended strategies and best practices to maximize success and improve patient outcomes., (Copyright © 2024 Elsevier Inc. All rights reserved.)

The transposition of the great arteries (TGA) associated with a complete atrioventricular septal defect is a rare and serious congenital cardiac anomaly. In this report, we describe the successful biventricular repair of a TGA with a complete atrioventricular septal defect in an infant. Due to the low body weight of the patient and a complex coronary pattern anomaly, an arterial switch operation was executed, with the Mee procedure and pulmonary arterial banding as initial palliative measures when the infant was 22 days old and weighed 2.5 kg. Subsequently, atrioventricular septal defect repair using the modified one-patch method was performed when the patient was 1.3 years old and weighed 8.8 kg. Remarkably, the postoperative course of the patient demonstrated no notable incidents. To our knowledge, this is the first time a two-stage strategy was applied to repair these complex defects, presenting a promising approach for managing similar cases in future medical practice., (© 2023. The Author(s).)

Background: Congenital left circumflex coronary artery fistula (LCX-CAF) is a relatively rare type of coronary artery fistula (CAF); little is known about the outcomes of transcatheter closure (TCC) of LCX-CAF., Methods and Results: All consecutive patients admitted to Fuwai Hospital and scheduled for TCC of LCX-CAF between January 2012 and December 2022 were reviewed retrospectively. Of the 25 consecutive patients (mean [±SD] age 34±20 years; 48% male) admitted and scheduled for TCC of congenital LCX-CAF, the procedure was feasible in 22 (77.3%). The mean (±SD) diameter of the fistulas was 6.99±2.04 mm; 21 (84%) patients had a large fistula (i.e., diameter >2-fold greater than non-feeding coronary artery). Occluders were deployed via a transarterial approach and arteriovenous loop in 6 (27.3%) and 16 (72.7%) patients, respectively. No procedural complications were recorded. Although the procedural success rates are similar for single LCX-CAF and left anterior descending CAF (81.25% vs. 92.86%; P=0.602), the mean time from initial angiography to first occluder deployment is significantly longer for LCX-CAF (83.06±36.07 vs. 36.00±9.49 min; P<0.001). The mean (±SD) follow-up time was 62.2±45.5 months. The incidence of myocardial infarction and recanalization of the fistula was 4.5% (1/22) and 9.1% (2/22), respectively., Conclusions: TCC of LCX-CAF is a feasible and effective alternative to surgical repair, with comparable outcomes in selected patients. Optimal medical therapy to prevent post-closure myocardial infarction requires further investigation.

Spontaneous coronary artery dissection (SCAD) is a rare cause of ST-segment elevation myocardial infarction (STEMI), predominantly affecting women. Because primary percutaneous coronary intervention (PPCI) is reserved for a select group of patients, vulnerable and minority patients may experience delays in appropriate management and adverse outcomes. We examined the racial differences in the outcomes for patients with SCAD who underwent PPCI for STEMI. Records of patients aged ≥18 years who underwent PPCI for SCAD-related STEMI between 2016 and 2020 were identified from the National Inpatient Sample database. Clinical, socioeconomic, and hospital characteristics were compared between non-White and White patients. Weighted multivariate analysis assessed the association of race with inpatient mortality, length of stay (LOS), and hospitalization costs. The total weighted estimate of patients with SCAD-STEMI who underwent PPCI was 4,945, constituting 25% non-White patients. Non-White patients were younger (56 vs 60.7 years, p <0.001); had a higher prevalence of diabetes, acute renal failure, and obesity; and were more likely to be uninsured and be in the lowest income group. Inpatient mortality (7.7% vs 8.4%, p = 0.74) and hospitalization costs ($34,213 vs $31,858, p = 0.27) were similar for non-White and White patients, and the adjusted analysis did not show any association between the patients' race and inpatient mortality (odds ratio 0.60, 95% confidence interval [CI] 0.32 to 1.13, p = 0.11) or hospitalization costs (β [β coefficient]: 215, 95% CI -4,193 to 4,623, p >0.90). Similarly, there was no association between the patients' race and LOS (incident rate ratio 1.20, 95% CI 1.00 to 1.45, p = 0.054). The weighted multivariate analysis showed that age; clinical co-morbidities such as diabetes, acute renal failure, valvular dysfunction, and obesity; low-income status; and hospitalization in the western region were associated with adverse outcomes. In conclusion, our study does not show any differences in inpatient mortality, LOS, and hospitalization costs between non-White and White patients who underwent PPCI for SCAD-related STEMI., Competing Interests: Declaration of competing interest Dr. Garcia-Garcia receives institutional grant support from MedAlliance, Biotronik, Neovasc, Boston Scientific, Abbott, Shockwave, Chiesi, Philips, Spectrawave, Angiowave, InfraReDx, Medis, Pulse Medical, and MedHub; is a consultant of Biotronik and Abbott; and receives speaker's fee form Abbott, Biotronik, Boston Scientific, and InfraReDx. Dr. Chitturi is a consultant for Glass Health. Dr. Case is a speaker for Asahi Intecc USA, Zoll Medical. Dr. Waksman is on the advisory board of Abbott Vascular, Boston Scientific, Medtronic, Philips IGT, and Pi-Cardia Ltd.; is a consultant for Abbott Vascular, Biotronik, Boston Scientific, Cordis, Medtronic, Philips IGT, Pi-Cardia Ltd., Swiss Interventional Systems/SIS Medical AG, Transmural Systems Inc., and Venous MedTech; receives grant support from AstraZeneca, Biotronik, Boston Scientific, Chiesi, Medtronic, and Philips IGT; is part of the speakers bureau of AstraZeneca; and is an investor of MedAlliance and Transmural Systems Inc. The remaining authors have no competing interest to declare., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Rationale: Single coronary artery (SCA) is a rare coronary artery malformation. SCA combined with atherosclerotic plaques can cause severe and widespread myocardial ischemia and infarction, leading to hemodynamic instability and even sudden death., Patient Concerns: A 48-year-old Chinese man was admitted for treatment of persistent chest tightness and panic for 5 hours. The patient was a lorry driver with high work intensity and mental stress, with body mass index of 33.78, history of smoking and alcohol consumption, but no history of hypertension and diabetes., Diagnoses: Admission examination showed Troponin was 183.083 µg/L and CK-MB value was >300 µg/L. The patient was diagnosed with a congenital single right coronary artery (RCA) with acute myocardial infarction (AMI) by coronary angiography (CAG). Due to atherosclerotic plaques rupture, a complete occlusion of the proximal RCA with thrombolysis in myocardial infarction grade 0 of distal blood flow were found., Interventions and Outcomes: The patient was treated with thrombus aspiration and thrombolytic therapy by percutaneous coronary intervention under the support of intra-aortic balloon pump. Postoperative the chest tightness and panic were relieved, and CAG revealed that the proximal thrombus of the RCA was reduced, and distal blood flow was restored to thrombolysis in myocardial infarction grade 3. After 2 weeks of intensive antithrombotic and lipid-regulating drug therapy, the patient was successfully discharged. Follow-up for 6 months, the patient was able to live and work normally without experiencing chest tightness and chest pain. Computed tomography angiography (CTA) confirmed a congenital single RCA with patent lumen and no severe stenosis., Lessons: The congenital single RCA is very rare, and it is fatal in conjunction with acute coronary syndrome. Early detection and appropriate treatment is critical for AMI patient with single RCA. CAG is the gold standard for diagnosis of single RCA, and CTA is a necessary to describe the anatomical course of abnormal coronary arteries., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Introduction: Reconstruction surgery of the proximal aorta in most cases involves the use of an aortic conduit, followed by reimplantation of the coronary ostia. Although uncommon, the origin of the coronary arteries in certain anatomical variants poses additional difficulties when performing surgery on the aortic root and requires a different treatment rationale. Case report: We hereby present the case of a 60-year-old patient with multiple cardiovascular risk factors (smoking, arterial hypertension and dyslipidemia), suffering from severe degenerative stenosis of a bicuspid aortic valve, associated with ascending aorta aneurysm and a significant extrinsic stenosis of the left coronary artery caused by the aneurysm. The patient presented with severe degenerative bicuspid aortic valve stenosis associated with ascending aorta aneurysm and a significant extrinsic stenosis of the left coronary artery caused by the aneurysm. Following the preoperative assessment, it was decided that the best course of action was to perform surgery on the aortic valve and ascending aorta. During the surgery, the origin of the right and left coronary ostia were found at the level of the left coronary cusp, both forming a common coronary button. Due to this particular anatomical variant, it was decided to reimplant them as a common button onto the main conduit by means of an interposed No.10 PTFE (Polytetrafluoroethylene) vascular prosthesis. Conclusion: A rare case of aortic root surgery associated with coronary ostia origin variant "shotgun barrel", which required a different method of reimplantation: modified Cabrol technique., (Celsius.)

Purpose: To develop and validate a deep learning (DL)-model for automatic reconstruction for coronary CT angiography (CCTA) in patients with origin anomaly, stent or bypass graft., Material and Methods: In this retrospective study, a DL model for automatic CCTA reconstruction was developed with training and validation sets from 6063 and 1962 patients. The algorithm was evaluated on an independent external test set of 812 patients (357 with origin anomaly or revascularization, 455 without). The image quality of DL reconstruction and manual reconstruction (using dedicated cardiac reconstruction software provided by CT vendors) was compared using a 5-point scale. The successful reconstruction rates and post-processing time for two methods were recorded., Results: In the external test set, 812 patients (mean age, 64.0 ± 11.6, 100 with origin anomalies, 152 with stents, 105 with bypass grafts) were evaluated. The successful rates for automatic reconstruction were 100% (455/455), 97% (97/100), 100% (152/152), and 76.2% (80/105) in patients with native vessel, origin anomaly, stent, and bypass graft, respectively. The image quality scores were significantly higher for DL reconstruction than those for manual approach in all subgroups (4 vs. 3 for native vessel, 4 vs. 4 for origin anomaly, 4 vs. 3 for stent and 4 vs. 3 for bypass graft, all p < 0.001). The overall post-processing time was remarkably reduced for DL reconstruction compared to manual method (11 s vs. 465 s, p < 0.001)., Conclusions: The developed DL model enabled accurate automatic CCTA reconstruction of bypass graft, stent and origin anomaly. It significantly reduced post-processing time and improved clinical workflow., (© 2024. Italian Society of Medical Radiology.)

Background: Anomalous aortic origin of a coronary artery from the inappropriate sinus of Valsalva (AAOCA) is a rare congenital heart lesion. It is uncommon for patients with AAOCA to present with severe symptoms at a very young age., Case Presentation: We describe a very rare but critical presentation in a young infant with AAOCA that requires surgical repair and pacemaker placement. A three-month-old infant was referred because of syncope. Cardiac arrest occurred shortly after admission. The electrocardiogram indicated a complete atrioventricular block and a transvenous temporary pacemaker was implanted. A further coronary computed tomographic angiography (CTA) showed the anomalous origin of the right coronary artery from the left sinus of Valsalva. Coronary artery unroofing was performed due to an interarterial course with the intramural component, and a permanent epicardial pacemaker was implanted. The postoperative recovery was uneventful, and this patient was thriving and asymptomatic at the nine-month follow-up. However, the electrocardiogram still indicated a complete pacing rhythm., Conclusions: By timely diagnosis and treatment, this patient is successfully rescued. Although rare, AAOCA may be fatal even in infants., (© 2024. The Author(s).)

Background: An anomalous origin and inter-arterial course of the right coronary artery is a rare anomaly that can lead to sudden ischemic cardiac death if left untreated. We present a case of a patient with an anomalous right coronary artery originating from the left coronary sinus and an inter-arterial course that was managed with coronary artery bypass surgery using a suitable internal mammary artery conduit. The proximal right coronary artery was ligated to prevent competitive flow., Case Presentation: A 69 year-old-male with a ten-year history of intermittent chest pain and dyspnea with a negative workup underwent a cardiac catheterization, which showed an anomalous right coronary artery (RCA) originating from the left coronary sinus, with an inter-arterial course between the ascending aorta and pulmonary artery, and approximately 70% narrowing of the proximal RCA. The patient underwent an on-pump coronary artery bypass using the right internal mammary artery (RIMA) as a conduit, with segment 2 of the RCA being the target. The proximal RCA was ligated. Intra-operatively, there were no signs of ischemia or arrhythmia. The patient was successfully taken off cardiopulmonary bypass and eventually discharged home., Conclusion: Symptomatic anomalous origin of the right coronary artery with an inter-arterial course can be treated successfully with coronary artery bypass surgery with the internal mammary artery as a conduit. Ligation of the proximal right coronary artery is essential to minimize competitive flow through the bypass graft., (© 2024. The Author(s).)

Anomalous left coronary artery from the pulmonary artery is a rare CHD. It is the most common type of anomalous coronary origin. It may cause myocardial ischaemia or infarction, mitral regurgitation, congestive heart failure, and early death in infancy if left untreated. Surgery is the only treatment for anomalous left coronary artery from the pulmonary artery. In recent years, with advancements in surgical techniques and the widespread utilisation of extracorporeal cardiac assist devices such as extracorporeal membrane oxygenation, the treatment outcomes for anomalous left coronary artery from the pulmonary artery have demonstrated significant improvements. However, the surgical indications and methods of anomalous left coronary artery from the pulmonary artery, especially the surgical methods of anomalous left coronary artery from the pulmonary artery with intramural coronary artery, and whether to treat mitral regurgitation at the same time are still controversial. The long-term complications and prognosis remain discouraging simultaneously, with significant variations in outcomes across different centres. The present review specifically addresses these aforementioned concerns. Based on the literature published at home and abroad, we found that no matter what type of anomalous left coronary artery from the pulmonary artery patients, even asymptomatic patients, regardless of the collateral circulation between the left and right coronary arteries, should immediately undergo surgical treatment to promote the recovery of left ventricular function. Based on different coronary artery anatomical morphology and preoperative cardiac function, the long-term follow-up results of individualised surgical treatment of anomalous left coronary artery from the pulmonary artery children show good prognosis, and most children have significant improvement in cardiac function. Patients with moderate to severe mitral regurgitation should undergo mitral valve operation at the same time as anomalous left coronary artery from the pulmonary artery repair. Mitral valvuloplasty can quickly improve mitral regurgitation and promote the early recovery of cardiac function after operation, and does not increase the risk of operation. Mechanical circulatory support is a safe and effective means of early postoperative transition for children with severe anomalous left coronary artery from the pulmonary artery. Anomalous left coronary artery from the pulmonary artery with intramural coronary artery is a rare anomaly. According to different anatomical types, different surgical methods can be used for anatomical correction, and satisfactory early and mid-term results can be obtained.

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., (© 2024. The Author(s).)

Background: Aberrant left coronary artery from pulmonary artery (ALCAPA) is a very rare congenital heart defect. Its coexistence with patent ductus arteriosus (PDA) is extremely rare. The high pressures created by the left-to-right shunt in the pulmonary arteries can delay symptoms and create a real challenge in diagnosing ALCAPA. Missing this diagnosis can have severe results, including extensive ischemia and sudden death., Case Presentation: We present a case of an infant born with a large PDA. Initially treated conservatively, however, due to congestive heart failure and lack of weight gain, she underwent surgical ligation of the PDA at the age of four and a half months. Following surgery, she developed pulmonary edema. Echocardiography revealed decreased ventricular function. ECG revealed ST elevations on lateral leads, and serum troponin was significantly increased. The patient underwent cardiac magnetic resonance imaging (MRI), which revealed signs of wall ischemia and decreased function of the left ventricle (LV) with unclear coronary anatomy. Diagnostic catheterization revealed an ALCAPA. She underwent surgical intervention, and the left coronary artery was re-implanted in the aortic sinus. Follow-up revealed slow improvement of cardiac function., Discussion and Literature Review: The coexistence of PDA and ALCAPA is a very rare occurrence. We found at least 10 reported cases in the literature. Delayed diagnosis might be detrimental. The prognosis of these patients is variable., Conclusion: An unusual post-surgical course following PDA repair requires a high index of suspicion and appropriate evaluation for ALCAPA, preferably with angiography., (© 2024. The Author(s).)

Background: Although anomalous aortic origin of a coronary artery (AAOCA) is associated with risk of sudden cardiac arrest (SCA), there is a spectrum of disease, with the appropriate management for many remaining unclear. Increasing data warrant review for an updated perspective on management., Methods: A panel of congenital cardiac surgeons, cardiologists, and imaging practitioners reviewed the current literature related to AAOCA and its management. Survey of relevant publications from 2010 to the present in PubMed was performed., Results: The prevalence of AAOCA is 0.4% to 0.8%. Anomalous left coronary artery is 3 to 8 times less common than anomalous right coronary, but carries a much higher risk of SCA. Nevertheless, anomalous right coronary is not completely benign; 10% demonstrate ischemia, and it remains an important cause of SCA. Decision-making regarding which patients should be recommended for surgical intervention includes determining anatomic features associated with ischemia, evidence of ischemia on provocative testing, and concerning cardiovascular symptoms. Ischemia testing continues to prove challenging with low sensitivity and specificity, but the utility of new modalities is an active area of research. Surgical interventions focus on creating an unobstructed path for blood flow and choosing the appropriate surgical technique given the anatomy to accomplish this. Nontrivial morbidity has been reported with surgery, including new-onset ischemia., Conclusions: A proportion of patients with AAOCA demonstrate features and ischemia that warrant surgical intervention. Continued work remains to improve the ability to detect inducible ischemia, to risk stratify these patients, and to provide guidance in terms of which patients warrant surgical intervention., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Injury to coronary arteries during mitral surgery is a rare but life-threatening procedural complication, an anomalous origin and course of the left circumflex artery (LCx) increase this risk. Recognizing the anomaly by the characteristic angiographic pattern and identifying its relationship with the surrounding anatomical structure using imaging techniques, mainly transesophageal echocardiography (TOE) or coronary computed tomography angiography (CCTA), is of crucial importance in setting up the best surgical strategy. We report a case of anomalous origin of a circumflex artery (LCx) from the proximal portion of the right coronary artery (RCA) with a pathway running retroaortically through the mitro-aortic space. An integrated diagnostic approach using a multidisciplinary team with a cardiologist and an imaging radiologist allowed us to decide the surgical strategy. We successfully performed a mitral valvular repair using a minimally invasive minithoracotomic approach and implanting a complete semirigid ring., (© 2024. The Author(s).)

Anomalous aortic origin of the left coronary artery (AAOLCA) confers high risk for sudden cardiac arrest (SCA). This series aims to describe consecutive admissions with interarterial AAOLCA presenting with SCA and distinct clinical trajectories. An eight-year-old boy collapsed at school and received 10-min of cardiopulmonary resuscitation (CPR) and defibrillation prior to return of spontaneous circulation. He had no end-organ dysfunction and underwent uneventful coronary unroofing. In contrast, a 14-year-old boy presented with collapse while jogging. He received 40-min of CPR prior to extracorporeal membranous oxygenation cannulation with multisystem dysfunction and persistent severely depressed left ventricular function. He is now rehabilitating following uneventful orthotropic heart transplantation. These cases illustrate the diverse outcomes of AAOLCA with SCA following exertional syncope., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Coronary artery aneurysms (CAAs), coronary arteriovenous malformations (CAVMs), and spontaneous coronary artery dissections (SCADs) are rare clinical entities, and much is unknown about their natural history, prognosis, and management., Methods: A systematic search of MEDLINE, Embase, and Cochrane Library databases was performed in March 2023 to identify published papers related to CAAs, CAVMs, and SCADs., Results: CAAs are found in 0.3% to 12% of patients undergoing angiography and are often associated with coronary atherosclerosis. They are usually asymptomatic but can be complicated by thrombosis in up to 4.8% of patients and rarely by rupture (0.2%). CAAs can be managed medically, percutaneously with stents or coil embolization, and surgically. The most common surgical procedure is ligation of the aneurysm, followed by coronary artery bypass grafting. The incidence of CAVMs is 0.1% to 0.2% in patients undergoing angiography, and they are most likely associated with congenital abnormal development of the coronary vessels. The diagnosis of CAVMs is usually incidental. Surgical or percutaneous intervention is indicated for patients with large CAVMs, which carry a potential risk of myocardial infarction. SCADs represent 1% to 4% of all acute coronary syndromes and typically affect young women. SCADs are strongly correlated with pregnancy, suggesting the role of sex hormones in their pathogenesis. Conservative management of SCAD is preferred for stable patients without signs of ischemia as spontaneous resolution is frequently reported. Unstable patients should undergo revascularization either percutaneously or with coronary artery bypass grafting., Conclusions: Further evidence regarding the management of these rare diseases is needed and can ideally be derived from multicenter collaborations., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We present a rare case of tetralogy of Fallot accompanied with dextrocardia, situs inversus and anomalous origin of the right coronary artery. Total repair was accomplished successfully using a minimally invasive left axillary thoracotomy., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose of Review: This review describes the presentation, diagnosis, and management of congenital coronary artery fistulas (CAFs) in adults., Recent Findings: CAFs are classified as coronary-cameral or coronary arteriovenous fistulas. Fistulous connections at the distal coronary bed are more likely to be aneurysmal with higher risk of thrombosis and myocardial infarction (MI). Medium-to-large or symptomatic CAFs can manifest as ischemia, heart failure, and arrhythmias. CAF closure is recommended when there are attributable symptoms or evidence of adverse coronary remodeling. Closure is usually achievable using transcatheter techniques, though large fistulas may require surgical ligation with bypass. Given their anatomic complexity, cardiac CT with multiplanar 3-D reconstruction can enhance procedural planning of CAF closure. Antiplatelet and anticoagulation are essential therapies in CAF management. CAFs are rare cardiac anomalies with variable presentations and complex anatomy. CAF management strategies include indefinite medical therapy, percutaneous or surgical CAF closure, and lifelong patient surveillance., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Congenital coronary artery anomalies are rare and most often clinically benign. We present a case of a 67-year-old male with osteomyelitis and persistent bacteremia with an anomalous left coronary artery mimicking an aortic root abscess. A transesophageal echocardiogram revealed a hypoechoic potential space around the aortic root, highly suspicious for a root abscess. Urgent cardiac surgery was performed, revealing no infection but an anomalous coronary artery arising from the right coronary sinus. This case highlights the importance of considering atypical anatomy in the diagnosis of infectious cardiac processes. While this resemblance should not delay intervention for suspected abscesses, it emphasizes the need to be aware of congenital differences in imaging for patients with known anomalies or asymptomatic patients with unknown anatomy.

Coronary artery fistula (CAF) is characterized as a congenital or acquired abnormal communication between a coronary artery and any of the four chambers of the heart (coronary-cameral fistula) or great vessels (coronary arteriovenous fistula) bypassing the capillaries within myocardium. CAF is a rare disease, challenging to diagnose and treat depending on the anatomical location and type of the fistula and accompanying diseases. This study aims to report a case with multiple coronary artery to coronary sinus (CS) fistulas with giant left circumflex artery and multivalvular infective endocarditis., (© 2024. The Author(s).)

Background: The management of adult patients with anomalous aortic origin of the right coronary artery (ARCA) from the left aortic sinus poses important challenges. The presence of symptoms or documented ischaemia, the anatomical characteristics of the ostium, and the course of the coronary determine decision-making., Methods: A retrospective review was performed of all cases of surgical management of ARCA at a single centre. The primary endpoints were mortality and myocardial infarction at 30 days. Secondary endpoints included recurrence of symptoms, freedom from re-intervention, and mortality during long-term follow-up., Results: From October 2019 to August 2023, 15 adult patients underwent surgery for ARCA; 13 patients were included in this study (mean age 53.9±11.1 years; 10 female). A slit-like orifice, a long intramural segment, and an interarterial course were found in all patients. Twelve (12) patients (92.3%) were symptomatic: nine with angina, combined with dyspnoea on exertion in seven. One (1) patient had history of pre-syncope. One (1) patient presented with out-of-hospital cardiac arrest. All patients underwent formal unroofing of the orifice and intramural portion of the ARCA; five patients had a concomitant procedure. No 30-day mortality nor myocardial infarction was recorded. At a mean follow-up of 20.1±12.8 months, all patients were alive. One (1) patient (7.6%) developed recurrent dyspnoea; investigations showed no ischaemia. No repeated interventions were required., Conclusions: Surgical unroofing of anomalous coronary artery in the adult is safe and effective; correction of both the slit-like orifice and intramural portion of the anomaly provides a durable result in patients with ARCA., Competing Interests: Conflicts of Interests There are no conflicts of interest to disclose., (Copyright © 2024 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Objective: To analyze the diagnosis and surgical treatment of high-risk anomalous aortic origin of coronary artery (AAOCA). Methods: This is a retrospective case series study. From January 2016 to July 2023, 24 cases of high-risk AAOCA underwent surgical treatment in Department of Cardiac Surgery, Guangdong Provincial People's Hospital. There were 18 males and 6 females, operatively aged ( M (IQR)) 13 (26) years (range: 0.3 to 57.0 years). They were confirmed by cardiac ultrasound and cardiac CT, all of which had anomalous coronary running between the aorta and the pulmonary artery. There were 15 cases of the right coronary artery from the left aortic sinus of Valsalva, 6 cases of left coronary artery from the right aortic sinus of Valsalva, 3 cases of the sigle coronary artery. Only 3 patients had no obvious related symptoms (2 cases were complicated with a positive exercise stress test and 1 case with other intracardiac malformations), 21 cases had a history of chest tightness, chest pain, or syncope after exercise. Three patients suffered syncope after exercise and underwent cardiopulmonary resuscitation (2 cases were treated with an extracorporeal membrane oxygenerator (ECMO)). The gap from the first symptom to the diagnosis was 4.0 (11.5) months (range: 0.2 to 84.0 months). The detection rate of coronary artery abnormalities suggested by the first cardiac ultrasound was only 37.5% (9/24). Seven patients were complicated with other cardiac diseases (4 cases with congenital heart defects, 2 cases with coronary atherosclerotic heart disease, 1 case with mitral valve disease). Results: All 24 patients underwent surgical treatment (23 cases underwent abnormal coronary artery unroofing, 1 case underwent coronary artery bypass grafting), and 5 patients underwent other intracardiac malformation correction at the same time. There were no death or surgery related complications in the hospital for 30 days after the operation. A patient with preoperative extracorporeal cardiopulmonary resuscitation was continuously assisted by ECMO after emergency AAOCA correction and had complications such as limb ischemia necrosis and renal dysfunction after the operation. During the follow-up of 2.2 (3.3) years (range: 1 month to 7.2 years), one patient who previously underwent percutaneous transluminal coronary angioplasty with a stent implant experienced significant postoperative symptomatic relief, and the other discharged patients had no related symptoms. Conclusions: The accurate rate of initial diagnosis for high-risk AAOCA is still low, but the risk of cardiovascular accidents is high. For sports-related chest pain and other symptoms, more attention should be paid to the detection of AAOCA, especially for adolescents. Exercise stress testing can be helpful in evaluating the cardiovascular risk of asymptomatic AAOCA. Instant surgical treatment can achieve satisfactory curative effects.

Background: Anomalous aortic origin of the coronary artery (AAOCA) is a rare congenital heart disease. Therefore, optimal indications for surgery in patients with severe aortic valve stenosis (AS) complicated by AAOCA remain uncertain., Case Presentation: We report the case of a 57-year-old male patient who underwent aortic valve replacement (AVR) and aortic root enlargement using a Y-incision procedure for severe AS with an anomalous aortic origin of the right coronary artery (AAORCA). Since preoperative single-photon emission computed tomography revealed no ischaemic lesions, an aortic root enlargement with a Y-incision was performed to prevent the potential compression of the prosthetic valve on the AAOCA and prosthesis-patient mismatch., Conclusions: Preoperative evaluation of the coronary anatomy and myocardial ischaemia using advanced imaging modalities and aortic root enlargement with the Y-incision procedure is an effective strategy for preventing ischaemic complications in cases of severe AS with AAORCA., (© 2024. The Author(s).)

Objective: To evaluate the intermediate and long-term outcomes and technical aspects of transcatheter closure (TCC) of coronary cameral fistulas (CCF) in pediatric patients. Methods: This was a case-control study. All pediatric patients with CCF who underwent TCC between January 2005 and December 2019 were retrospectively reviewed. Data was collected from medical records, including demographic characteristics, procedural details, intraoperative and postoperative serious adverse events, follow-up results and prognosis. Patients with serious adverse events and without serious adverse events were compared regarding their clinical features and CCF characteristics. Comparisons between groups were performed with independent sample t test, chi-square test or Fisher exact test. Results: A total of 66 CCF patients (34 boys, 32 girls, 3.9 (1.9, 6.2) years old, 15 (11, 20) kg) underwent attempted TCC. All of the CCF were all medium or large fistulas including 55 proximal fistulas (83%) and 11 distal fistulas (17%). The CCF originated more frequently from the right coronary artery (38 cases (58%)), followed by the left coronary artery (28 cases (42%)). The incidence of coronary artery aneurysms (CAA) was 61% (40/66).Procedural treatment was achieved in 64 patients and procedural success was achieved in 59 patients (92%). Six (9%) serious adverse events occurred in 5 patients during the perioperative period. Acute complications included procedure-related death in one patient and acute myocardial infarction in one patient. Periprocedural complications occurred in 3 patients at one day postoperatively including acute myocardial infarction (2 cases), occluder detachment (1 case), and tricuspid chordae tendinae rupture (1 case). Clinical follow-up data were available in 58 of the 62 patients who underwent initial successful TCC with a follow-up period of 9.3 (6.5, 13.4) years. Ten adverse events occurred in 9 patients including 5 complications consisted of aortic valve perforation (1 case), coronary thrombosis (1 case), progressive aneurysmal dilation after reintervention (1 case), and new-onset tricuspid valve prolapse with significant regurgitation (2 cases) and large residual shunts due to fistula recanalization (5 cases). Therefore, the incidence of intermediate and long-term adverse events was 17% (10/58). During the periprocedural and follow-up period, 16 adverse events occurred in 13 patients, whereas no adverse events occurred in 51 patients. Patients with seriovs adverse events presented with larger proportion of large CCF (11/13 vs. 39% (20/51), P =0.005), giant CAA (10/13 vs. 14% (7/51), P =0.030), and higher mean pulmonary artery pressure ((20±9) vs. (16±6) mmHg, 1 mmHg=0.133 kPa, t =2.02, P =0.048) compared to patients without serious adverse events. Conclusions: TCC in CCF children appears to be effective with favorable intermediate and long-term outcomes. Strict indication of TCC is mandatory.

Anomalous left coronary artery originating from the pulmonary artery (ALCAPA) is an infrequent congenital anomaly. Presentation of this syndrome is rare in adults. Nevertheless, adult patients are at risk of ischaemia, arrhythmias or sudden cardiac death and always require surgical intervention. At our institution, a specific technique of interposition of the right internal iliac artery as a free-graft for left coronary artery reimplantation was used in adult ALCAPA patients. The aim of this report is to determine long-term results and experiences with this surgical technique., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

We illustrate the case of a 62-year-old man with a symptomatic anomalous right coronary artery from pulmonary artery (ARCAPA). Our patient had presented with dyspnea on exertion with electrocardiogram showing pronounced inferior Q waves and marked inferolateral ST-T wave changes. The patient had a nuclear stress test which showed inferior wall ischemia. Subsequently, the patient underwent coronary angiography which showed an ARCAPA. The patient underwent surgical repair with reimplantation of the right coronary artery to the ascending aorta which was tolerated well. Our case illustrates ARCAPA presenting late in adulthood with ischemic symptoms that was treated with corrective surgery., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

We describe the case of a newborn male with a large fistula from the left main coronary artery to the right ventricle. This case illustrates a rare congenital coronary artery fistula and its successful surgical management in the neonatal period., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Congenital coronary anomalies are not an infrequent occurrence and their clinical presentation typically occurs during early years, though may be manifested only in adulthood. In the setting of anomalous aortic origin of a coronary artery, this is particularly concerning as it inflicts sudden loss of healthy young lives. Risk stratification remains a challenge and so does the best management decision-making in these patients, particularly if asymptomatic. Standardized approach to evaluation and management, with careful data collection and collaboration among centers, will likely impact future outcomes in this patient population, thus allowing for exercise participation and healthier lives., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2023 Elsevier Inc. All rights reserved.)

We aimed to characterize the ranges, temporal trends, influencing factors, and prognostic significance of postoperative troponin levels after congenital heart surgery. This single-center retrospective study included patients from 2006 to 2021 who had ≥ 1 postoperative troponin-T measurement collected within 96 h of congenital heart surgery (CHS). Patients were grouped as Anomalous Aortic Origin of the Coronary Artery-"AAOCA repair," or congenital heart surgery with "Other Coronary Interventions" other than AAOCA repair, or "No Coronary Intervention." In each group, information on concomitant surgery requiring one or more of the following-atriotomy, ventriculotomy, right ventricular muscle bundle resection, and/or septal myectomy-was collected. Clinical correlates of troponin values were analyzed in three postoperative windows: < 8, 8-24, and 24-48 h. The highest median [range] troponin levels (ng/mL) for the samples were 0.34 [0.06, 1.32] at < 8 h for "AAOCA repair," 1.35 [0.14, 12.0] at < 8 h for those undergoing CHS with "Other Coronary Interventions," and 0.87 [0.06, 25.1] at 8-24 h for those undergoing CHS with "No Coronary Interventions." Atriotomy was associated with higher median troponin levels in the AAOCA group at < 8 h (0.40 [0.31, 0.77] vs. 0.29 [0.17, 0.54], P = 0.043) and in the Other Coronary Intervention group at 8-24 h (1.67 [1.04, 2.63] vs. 0.40 [0.19, 1.32], P = 0.002). Patients experiencing major postoperative complications (vs. those who did not) had higher troponin levels in the AAOCA group as early as 8-24 h (0.36 [0.24, 0.57] vs. 0.21 [0.14, 0.33], P = 0.03). Similar findings were noted in the Coronary Intervention (2.20 [1.34, 3.90] vs. 1.11 [0.51, 2.90], P = 0.028) and No Coronary Intervention (2.2 [1.49, 15.1] vs. 0.74 [0.40, 2.34], P = 0.027) groups but earlier at < 8 h. In the AAOCA group, 2/18 (11%) troponin outliers experienced cardiac arrest in comparison to 0/80 (0%) non-outliers (P = 0.032). In the Other Coronary Intervention group, troponin outliers had longer median times to ICU discharge (10 vs. 4 days) and hospital discharge (21 vs. 10 days) (both P < 0.001). Postoperative troponin levels depend on a multitude of factors and may have prognostic value in patients undergoing congenital heart surgery with coronary interventions., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Anomalous coronary arteries are rare congenital variations with cases ranging from asymptomatic to life-threatening. Given the wide variability of coronary anomalies, it is challenging to predict their clinical consequences. Here, we present the 'malignant' variant - interarterial course of the left coronary artery between the aorta and pulmonary trunk - given the highest risk of sudden cardiac death among the various coronary anomalies., Case Presentation: Our case presents a 22-year-old male presenting to the emergency department after a syncopal episode that occurred while the patient was driving a motor vehicle. Initial Computed Tomography (CT) of the chest performed as part of the trauma work-up revealed a rare case of an anomalous origin of the left main coronary artery (LMCA) originating from a common ostium with the right coronary artery (RCA). The LMCA was found to have a malignant course, as it was positioned between the aorta and pulmonary artery. Given the high risk of sudden cardiac arrest with this congenital variant, the patient underwent coronary artery bypass grafting., Conclusion: Anomalous coronary arteries remain the second leading cause of sudden cardiac death in young adult patients. The risk of sudden cardiac death depends on the congenital variant of the anomalous coronary artery as well as the course these vessels take. This case highlights a rare congenital variant featuring both the LMCA and RCA originating from a common ostium, with the LMCA having a malignant course, a variant with the highest risk of sudden cardiac death., (© 2023. The Author(s).)

As a result of increasing adoption of imaging screening, the number of adult patients with a diagnosis of anomalous aortic origin of the coronary arteries (AAOCA) has grown in recent years. Existing guidelines provide a framework for management and treatment, but patients with AAOCA present with a wide range of anomalies and symptoms that make general recommendations of limited applicability. In particular, a large spectrum of interventions can be used for treatment, and there is no consensus on the optimal approach to be used. In this paper, a multidisciplinary group of clinical and interventional cardiologists and cardiac surgeons performed a systematic review and critical evaluation of the available evidence on the interventional treatment of AAOCA in adult patients. Using a structured Delphi process, the group agreed on expert recommendations that are intended to complement existing clinical practice guidelines., (Copyright © 2023 American College of Cardiology and The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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)., Competing Interests: Conflits d'intérêts Les auteurs déclarent n'avoir aucun conflit d'intérêts en lien avec ce travail., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)