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Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) is a rare congenital coronary abnormality carrying a poor prognosis. We present a clinical case series of ALCAPA-related anterior mitral valve leaflet prolapse and discuss its mechanism as a key to suspicion of ALCAPA syndrome. [ABSTRACT FROM AUTHOR]

Background Coronary arteriovenous fistulas present an abnormal connection between the coronary arteries and an adjacent systemic or pulmonary vessel. They are rare, representing 0.002% of the general population. The majority is congenital but may additionally occur related to trauma or interventional cardiac procedures. Case summary We present the case of a 48-year-old male with a history of untreated bacterial endocarditis developing a right coronary/superior vena cava fistula. We detail the imaging findings of this rare phenomenon to arrive at this diagnosis. We describe his clinical course and the interventions considered, including surgical extraction. Unfortunately, this patient left against medical advice before completing recommended treatment. Discussion We present the first documentation of a right coronary/superior vena cava fistula secondary to chronic untreated bacterial endocarditis. Clinicians should be aware of this rare complication. [ABSTRACT FROM AUTHOR]

Background Anomalous aortic origin of a coronary artery from the opposite sinus is a rare congenital abnormality that may be encountered during primary percutaneous coronary intervention (PCI) for ST-elevation myocardial infarction (STEMI). Case summary A 65-year-old man presented with chest pain and signs of heart failure. Electrocardiogram demonstrated atrial fibrillation with ST elevation in the high lateral leads, and he was taken emergently to the cardiac catheterization laboratory for primary PCI. Coronary angiography identified the culprit to be an occluded anomalous left main coronary artery (LMCA) arising from the right coronary cusp, and primary PCI was successfully performed in the LMCA and the left anterior descending artery (LAD). Computed tomography angiography confirmed a benign retroaortic course of the anomalous LMCA with no additional high-risk features, as well as a new left atrial appendage thrombus. He subsequently developed deep venous thrombosis, acute pulmonary embolism, and acute kidney injury secondary to renal artery embolism with associated infarction. Workup for patent foramen ovale and thrombophilia were negative, and he was discharged in a stable condition. At 2-month follow-up, he was asymptomatic with no evidence of myocardial ischaemia on stress cardiac magnetic resonance imaging. Discussion We present the first reported case of an occluded anomalous LMCA arising from the right coronary sinus in a patient presenting with STEMI. Rapid recognition of this congenital anomaly and selection of an appropriate guide catheter were keys to achieving timely reperfusion and a good outcome in this case. [ABSTRACT FROM AUTHOR]

A hyperdominant left anterior descending coronary artery variation is a rare but important diagnosis because of the risk for large-territory ischemia. We describe a very rare presentation of spontaneous coronary artery dissection in the distal portion of a hyperdominant left anterior descending coronary artery. No similar cases have been described.

We present a case of a complex congenital coronary artery fistula between the right coronary artery, left anterior descending artery, and the main pulmonary artery complicated by massive aneurysms and a left-to-right shunt. We highlight the multimodality approach to assessment and the importance of individualized management of complex coronary fistulas.

Coronary artery fistulas (CAFs) are rare coronary anomalies involving the communication of an epicardial coronary artery and another cardiovascular structure. CAFs are usually easily distinguished from nearby coronary arteries. Here, we report a unique case of CAF that mimics the size, branching pattern, and appearance of a native epicardial left anterior descending artery.

We report an unusual case of a patient with hypertrophic obstructive cardiomyopathy, anomalous aortic origin of a coronary artery, obesity hypoventilation syndrome, and acquired long QT syndrome who was able to defy the odds of sudden cardiac death in the rarest of circumstances. (Level of Difficulty: Advanced.).

We report on a 2-week-old infant with huge left main coronary artery-to-right ventricular outflow tract fistula causing myocardial ischemia due to global coronary steal who was successfully submitted to percutaneous closure guided by a 3-dimensional–printed model using a duct-occluder vascular plug. (Level of Difficulty: Advanced.)

We present Stanford’s experience with patients post–arterial switch operation presenting with chest pain found to have hemodynamically significant myocardial bridging. The evaluation of symptomatic patients post–arterial switch should not only include assessment for coronary ostial patency but also for nonobstructive coronary conditions such as myocardial bridging. (Level of Difficulty: Advanced.)

Coronary artery anomalies are estimated to be present in 1.8% of the population. Though majority of them are benign, some may lead to angina pectoris, myocardial infarction, cardiac arrhythmias, congestive heart failure and sudden cardiac death, especially in the young. Hence the need arises to identify and treat possible malignant anomalies.

Surgical treatment of aneurysmal distal congenital coronary artery fistulas depends on size and anatomy. From 2008 to 2021, we applied a new surgical technique in 7 adult patients: proximal and distal fistula closure, opening of aneurysmal artery, and revascularization of branches rising from the fistula under cardiopulmonary bypass and cardiac arrest. (Level of Difficulty: Intermediate.)

A 41-year-old man with repaired tetralogy of Fallot and a single coronary artery (CA) arising anteriorly presented with dyspnea in the setting of moderate right ventricle-pulmonary artery conduit (RV-PAC) stenosis and moderate-to-severe extrinsic left main CA compression between the aorta and RV-PAC. His CA stenosis resolved after successful RV-PAC replacement., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Congenital coronary artery anomalies are rare. The most common anomalous variation is Anomalous Left Circumflex Artery (ALCx) which is a congenital anomaly. ALCx usually originates from the right sinus of Valsalva or as a proximal branching of the Right Coronary Artery (RCA). The clinical presentation has a spectrum which varies from asymptomatic presentation to angina or myocardial infarction with no atherosclerotic lesion due to kinking/compression of the vessel to ST-segment elevation myocardial infarction (STEMI) due to atherosclerotic occlusion. A 45-year-old female with a past medical history of hypertension, hyperlipidemia, type 2 diabetes, tobacco abuse, and a history of ischemic stroke, presented to the hospital due to chest pain. Electrocardiogram revealed inferior ST-elevation myocardial infarction (STEMI) and the patient was taken to the catheterization lab. Angiography revealed 90% stenotic Left Circumflex Artery (LCx) which was anomalous, arising from the right coronary cusp, whereas other coronary arteries were diffusedly atherosclerotic. A drug-eluting stent was placed in the ALCx reducing the stenosis from 90% to 0% and the patient was discharged in a stable condition on dual antiplatelet therapy and statin with plans for possible coronary artery bypass graft due to multivessel disease (severe diffuse disease of LAD, 90% mid-RCA stenosis, 80% proximal RCA stenosis). The patient was eventually lost to follow-up. Typically, anomalous LCx originating from RCA is benign, but there are many cases where there is myocardial infarction or sudden cardiac death due to acute angle take-off at the origin site. This anatomical variation is more important during cardiac surgeries because during valve replacement surgeries, there are cases of ALCx compression resulting in myocardial infarction. Our patient developed STEMI secondary to atherosclerotic stenosis in anomalous LCx. Based on her diffuse atherosclerotic disease and risk factors, it is likely that her anomalous anatomy did not cause her atherosclerotic disease. Overall, clinicians should remain vigilant for these anatomic abnormalities in their practice., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Vadher et al.)

Anomalous left coronary artery arising from the noncoronary cusp (LCANCC) is a rare congenital disorder. We herein describe a 17-year-old female patient with sudden cardiac arrest followed by refractory cardiogenic shock. LCANCC-induced acute myocardial infarction with left main coronary artery involvement was subsequently diagnosed, and the patient required a durable left ventricular assist device. < Learning objective: Anomalous left coronary artery arising from the noncoronary cusp is extremely rare, but poses a life-threatening condition. Coronary computed tomography angiography plays a crucial role for the assessment of high-risk anatomic features and decision-making for surgical repair.> [ABSTRACT FROM AUTHOR]

Cell therapy is a promising tool to prevent and treat heart failure in congenital heart disease. We report the first case of intramyocardial injection of allogeneic mesenchymal stromal cells as rescue therapy in a neonate with ischemic heart failure following arterial switch procedure for isolated transposition of the great arteries. (Level of Difficulty: Advanced.)

An asymptomatic patient presented at our hospital exhibiting a Brugada electrocardiography pattern with coronary artery fistulas. Coronary artery fistula is a congenital or acquired rare abnormal condition with increased symptoms and complications over time. In the absence of the therapeutic consensus, we discuss the association and management for this condition. (Level of Difficulty: Advanced.)

A 4-year-old boy with multisystem inflammatory syndrome in children before widespread recognition of this disease developed complications, including coronary artery aneurysm, without anti-inflammatory treatment. With delayed treatment, all sequelae resolved. This case demonstrates a natural history supporting the role of anti-inflammatory treatment even with delayed or equivocal diagnosis. (Level of Difficulty: Intermediate.)

A novel coronary anatomy in the form of anomalous left anterior descending coronary artery from pulmonary artery with a retroaortic left circumflex arising from the right coronary artery is presented. This unreported anatomy was discovered in a 7-month-old girl with failure to thrive. (Level of Difficulty: Intermediate.)

Transcatheter aortic valve replacement (TAVR) in the setting of an anomalous left circumflex coronary artery (LCX) has had a variety of outcomes. Most commonly an anomalous LCX originates as a separate ostium arising from the right coronary sinus or is found branching off of the proximal right coronary artery. The artery courses around the aortic annulus before taking the course seen in typical anatomy. Given this deviation from typical anatomy and increased aortic annulus pressure by the replacement valve, there is an increased risk of a complication such as acute coronary artery occlusion. Special consideration and preparation are needed to prevent adverse outcomes, including death. We report a case in which intraprocedural anomalous LCX rescue stenting proved to be effective for treatment of acute coronary occlusion. Follow-up angiography provided an opportunity to demonstrate long-term patency in rescue stenting during TAVR. [ABSTRACT FROM AUTHOR]

Congenital left main coronary artery atresia is an exceedingly rare condition with potentially fatal consequences if not diagnosed in a timely fashion. We present a case series in children and adolescents, including surgical repair and outcomes. We describe the presenting symptoms and subsequent management of each patient, including surgical repair and outcomes. (Level of Difficulty: Advanced.)

Anomalous aortic origin of coronary artery (AAOCA) can range from benign anatomic variants to those presenting with sudden cardiac arrest. This unique case of right AAOCA demonstrates detailed anatomic findings from cardiac computed tomography and the effects of transient acute coronary ischemia by cardiac magnetic resonance. (Level of Difficulty: Advanced.)

Abstract Background Management of coronary artery fistula (CAF) is based on obliterating the fistula communication between the cardiac arteries and other thoracic vessels. Case presentation We describe the presentation of an 85-year-old female with progressive exertional dyspnea on a background of a long standing left anterior descending diagonal to pulmonary artery fistula. We utilized neuro-interventional techniques to perform coil embolization via use of a Scepter XC dual lumen micro catheter. Conclusions Dual lumen balloon catheters allow for super-selective artery interrogation, stability of balloon positioning, with less trauma to vessel architecture and accurate embolization. There were no complications and the patient reported improvement of symptoms on review.

Coronary artery fistula are anomalous connections with coronary vessels or cardiac chambers, potentially resulting in coronary dilatation and pseudoaneurysm formation. We present the case of a 68-year-old woman referred to our institution for a voluminous coronary pseudoaneurysm secondary to coronary artery fistula presenting as a nearly completely obstructive left atrial mass. (Level of Difficulty: Intermediate.)

Coronary artery fistula (CAF) is an abnormal connection between a coronary artery and a heart chamber resulting in left-to-right shunt. A large CAF was an unexpected cause of heart failure in a 58-year-old woman who underwent transcatheter closure of the CAF with improvement in symptoms but complicated by myocardial infarction. (Level of Difficulty: Beginner.)

Coronary artery aneurysms are rare conditions with potentially devastating consequences. We describe the case of an athletic 40-year-old woman who received a diagnosis of giant right coronary artery aneurysm while she was undergoing a work-up for lower extremity varicose veins. She underwent successful surgical treatment without any complications. (Level of Difficulty: Intermediate.)

A 52-year-old woman presented with a continuous heart murmur at an annual health examination. Coronary computed tomography angiography showed confluent coronary artery fistulas meeting to form a large aneurysm fistulizing to the pulmonary artery. Percutaneous coil embolization was performed, resulting in the disappearance of the coronary artery fistulas and aneurysms. (Level of Difficulty: Intermediate.)

We report an unusual case of a patient with hypertrophic obstructive cardiomyopathy, anomalous aortic origin of a coronary artery, obesity hypoventilation syndrome, and acquired long QT syndrome who was able to defy the odds of sudden cardiac death in the rarest of circumstances. (Level of Difficulty: Advanced.)

Cardiac 3-dimensional printing for pre-procedural planning of structural heart procedures is a promising new tool. Despite current potential drawbacks, 3-dimensional models can help cardiologists better understand and treat complex cardiac defects. We describe a successful coronary cameral fistula closure planned with the aid of a 3-dimensional model. (Level of Difficulty: Advanced.)

A 77-year-old man with history of ischemic cardiomyopathy and left ventricular assist device (LVAD) presented with abdominal pain after a lawn mower accident. Examination and imaging revealed a displaced LVAD driveline and a pericardial hematoma secondary to traumatic coronary artery injury. The patient was treated with coronary artery coil embolization. (Level of Difficulty: Advanced.)

Ventriculocoronary arterial connections are typically found in patients with pulmonary atresia with an intact ventricular septum. This report describes a case of ventriculocoronary arterial connections in a patient with pulmonary atresia with a ventricular septal defect. Our case supports recent data suggesting a primary coronary artery developmental anomaly in pulmonary atresia. (Level of Difficulty: Advanced.)

We describe a case of an adult patient with mitral valve regurgitation and the anomalous origin and course of the left circumflex coronary artery. Use of a ringless procedure or a microinvasive approach, such as transapical neochordae implantation, would have possibly avoided a life-threatening post-operative complication. (Level of Difficulty: Advanced.)

Anomalous origin of the left main coronary artery (LMCA) from the right sinus of Valsalva (RSV) is a rare congenital anomaly that can cause myocardial ischemia, ventricular arrhythmia, and sudden cardiac death. We report the case of a 74-year-old male with a history of hyperlipidemia and hypertension who presented with non-ST elevation myocardial infarction (NSTEMI). On coronary angiogram, the patient was found to have LMCA originating from the RSV and a 90% stenosis of the left anterior descending (LAD) artery. The patient subsequently underwent computed tomography angiography (CTA) to assess the course of the LMCA, which was found to be intramyocardial with no compression. The echocardiogram reported a reduced ejection fraction (EF) of 40% and wall motion abnormalities in the anterior wall. The patient had a successful staged percutaneous coronary intervention (PCI). The patient on follow-up reported no symptoms and EF improved to 50%. Anomalous origin of the LMCA from the RSV is a rare but potentially life-threatening congenital anomaly. Once the course of the anomalous artery is established, immediate reperfusion using PCI is being increasingly used in place of surgical unroofing, noting a change in clinical practice. Further research is needed to determine the optimal treatment for this anomaly and to improve the long-term outcomes of affected individuals., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Prakash et al.)

A hyperdominant left anterior descending coronary artery variation is a rare but important diagnosis because of the risk for large-territory ischemia. We describe a very rare presentation of spontaneous coronary artery dissection in the distal portion of a hyperdominant left anterior descending coronary artery. No similar cases have been described., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

A 74-year-old man presented with symptoms and noninvasive diagnostic studies suggestive of myocardial infarction. Coronary angiography revealed total occlusion of the distal right coronary artery with a unique accessory coronary ring that provided retrograde collateral flow to the left ventricle, demonstrating the importance of considering non-native vessels when identifying target lesions., Competing Interests: Dr Drachman has received consulting fees for Boston Scientific, Broadview Ventures, Cardiovascular Systems Inc, and Cordis not relevant to the current submission. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

A 25-year-old male with neurofibromatosis type 1 presented with acute ST-segment elevation myocardial infarction. Coronary imaging revealed an aberrant right coronary anatomy, ectatic coronary arteries, and significant stenosis. Based on previous literature and clinical presentation, this case highlights the potential role of neurofibromatosis in the pathogenesis of coronary artery disease., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

We describe an extremely rare case of a 37-year-old female patient who presented with exertional angina and was diagnosed with a unique coronary anomaly with an anomalous right coronary artery with origin in the left anterior descending artery and a fistula between this anomalous coronary artery and the pulmonary artery. Most patients with coronary anomalies are asymptomatic but some may have angina caused by a coronary steal phenomenon, myocardial infarction, or even sudden death depending on the circuit and characteristics of the anomaly. The combination of multiple coronary anomalies is extremely rare. Despite being a rare diagnosis, coronary anomalies should always be considered as a cause of myocardial ischemia, especially in young patients with low probability for coronary obstructive disease. [ABSTRACT FROM AUTHOR]

Background Multisystem inflammatory syndrome in children (MIS-C) with features resembling Kawasaki disease has been reported in association with coronavirus disease 2019 (COVID-19). Case summary We report the rare case of a 22 months old boy with a history of operated simple transposition of the great arteries (TGA), who developed features of MIS-C likely to be associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) infection and involving the coronary arteries. Cardiovascular magnetic resonance imaging and cardiac catheterization showed long-distance ectasia of both coronary arteries after their origins and an origin stenosis of the right coronary artery with a perfusion defect. The patient was treated with oral anticoagulation together with antiplatelet therapy and remains under careful monitoring. Discussion This rare case demonstrates that also patients with TGA after the arterial switch operation (ASO) can develop coronary artery dilatation in association with MIS-C. The most interesting finding in this patient was that the origins of the reimplanted coronary arteries were not dilated. We speculate that scar tissue formation in the area of coronary artery transfer after ASO has prevented proximal coronary artery dilation. [ABSTRACT FROM AUTHOR]

An 83-year-old man with significant background comorbidities was admitted with an inferior ST-segment elevation myocardial infarction. During primary percutaneous coronary intervention, a giant aneurysm is seen in the right coronary artery. (Level of Difficulty: Beginner.)

Coronary artery fistula is a rare condition that can be congenital or acquired. Most patients with these anomalies remain asymptomatic, but symptoms and complications may develop with increasing age. We discuss the clinical presentation, diagnostic work-up, and management options for this condition. (Level of Difficulty: Intermediate.)

Myocardial bridging (MB) is a congenital coronary anomaly, which is defined as cardiac muscle overlying a portion of a coronary artery. Although traditionally considered benign in nature, increasing attention is being given to specific subsets of MB. Sports medicine recognizes MB as a cause of sudden death among young athletes. We present a case of a 30-year-old man who suddenly collapsed during a marathon running. Diagnostic workup with coronary computed tomography angiography revealed the presence of three simultaneous myocardial bridges in this patient, possibly explaining the exercise-induced syncope. The other diagnostic tests excluded seizures, cranioencephalic lesions, ionic or metabolic disturbances, acute coronary syndromes, cardiomyopathies, myocarditis, or conduction disturbances. Exertional syncope is a high-risk complaint in the marathon runner. In the context of intense physical activity, the increased sympathetic tone leading to tachycardia and increased myocardial contractility facilitates MB ischemia. In this illustrative case, the patient’s syncope might probably be associated with an ischemia-induced arrhythmia secondary to MB and potentiated by dehydration in the context of prolonged stress (marathon running). In conclusion, this case highlights that MB may be associated with dangerous complications (myocardial ischemia and life-threatening ventricular arrhythmias), particularly during intense physical activity and in the presence of a long myocardial bridge.

Background Anomalous right coronary artery (RCA) from the pulmonary artery (ARCAPA) is a rare congenital heart abnormality with varying clinical presentations, for which multiple imaging modalities are often required for diagnosis. Case summary We present a case of a 76-year-old female presenting with 2 weeks of palpitations and shortness of breath who was found to be in rapid atrial fibrillation (AF) with congestive heart failure. Despite initial medical management, the patient developed cardiogenic shock with anuric renal failure. Emergent right and left heart catheterization did not demonstrate any significant obstructive coronary artery disease but showed severe right ventricular (RV) failure and raised the possibility of an ARCAPA. This diagnosis was further corroborated by findings on a subsequent transoesophageal echocardiogram. In view of profound decline and limited anticipated improvement, the patient ultimately decided to pursue comfort measures in a hospice setting. Discussion We postulate that the underlying aetiology of our patient's shock state was multifactorial, notably progressive RCA-territory ischaemia and RV failure, sepsis, and new-onset uncontrolled AF. In adults, unrecognized congenital heart disease can uncommonly cause cardiogenic shock. In our case, echocardiography and invasive angiography were integrated for the diagnosis of ARCAPA given the clinical circumstances that limited the use of cardiac computed tomography angiography. [ABSTRACT FROM AUTHOR]

A 40-year-old African American woman presented with dyspnea, orthopnea, weight gain, and ankle edema. She was admitted for acute decompensated heart failure. Coronary angiography revealed coronary cameral fistula. We used multiple imaging modalities to confirm the fistula’s pathway. Her left ventricular systolic function improved after repair of coronary cameral fistula. (Level of Difficulty: Beginner.)

We present a case of a complex congenital coronary artery fistula between the right coronary artery, left anterior descending artery, and the main pulmonary artery complicated by massive aneurysms and a left-to-right shunt. We highlight the multimodality approach to assessment and the importance of individualized management of complex coronary fistulas., Competing Interests: The authors report that they have no relationships relevant to the contents of this paper to disclose., (Crown Copyright © 2024 Published by Elsevier on behalf of the American College of Cardiology Foundation.)

Abstract Background Dual left anterior descending (LAD) artery or duplication of LAD is a rarely reported coronary anomaly, consisting of two branches supplying the usual distribution of the LAD. Type IV dual LAD, in which a short LAD arises from the left main coronary artery and a long LAD arises from the right coronary artery is remarkably rare, and has not been reported in a Bangladeshi subject. Case presentation We describe the case of a 70-year old Bangladeshi male who presented with breathlessness in the background of a prior inferior myocardial infarction. Coronary angiography revealed an anomalous dual LAD. The short LAD which arose from the left main coronary artery gave off the first septal branch and terminated after giving off a large diagonal branch which continued further down towards the apex. The long LAD arose from the proximal right coronary artery and after traversing a distance, arrived at the interventricular septum, terminating at the apex after giving off diagonal branches. The right coronary artery was totally occluded from its early mid part and well-collateralized with retrograde flow from the left system. Conclusion We describe a case with unique variation of dual LAD type IV, which has previously not been described in a Bangladeshi subject thus far. Coronary angiography is vital to determine this coronary anomaly, which is usually detected incidentally on routine angiography for chest pain, sometimes with involvement of significant lesion of other coronary arteries, as in this case.