Search Results (11)

Coronary artery anomalies (CAAs) are a rare but clinically significant group of congenital abnormalities that are associated with myocardial ischaemia, malignant arrhythmias and sudden cardiac death, particularly in young people and athletes. Despite increasing recognition of these conditions and advances in cardiovascular imaging, there are still significant challenges regarding their classification, risk stratification and management, particularly with respect to surgical indications. This review provides a comprehensive overview of the current evidence on the classification, pathophysiology, diagnosis and management of CAAs, with a particular focus on surgical decision-making and contemporary guideline recommendations. A systematic literature search was conducted up to February 2025 using PubMed and Google Scholar. Priority was given to international guidelines, consensus statements, systematic reviews, meta-analyses and large observational studies. CAAs encompass a broad spectrum of anatomical variants and clinical presentations. Among these, anomalies of coronary origin are the most extensively studied. Surgical management is well established for coronary arteries arising from the pulmonary artery, particularly for anomalous left coronary artery from the pulmonary artery (ALCAPA). Substantial advances have also been made in the diagnosis, risk stratification, and treatment of anomalous aortic origin of a coronary artery (AAOCA), which has become a major focus of contemporary guideline recommendations. For patients with AAOCA, surgical correction, including unroofing, coronary reimplantation or coronary artery bypass grafting, is recommended for individuals with symptoms and/or high-risk anatomical features. In contrast, the diagnosis and management of myocardial bridging, coronary artery fistulas, and coronary artery ectasia remain controversial, with considerable variability in the indications for medical, percutaneous, and surgical treatment. The management of CAAs is an evolving field. While there is consensus for a limited number of anomalies, most cases still require individualised decision-making. It is essential to develop standardised diagnostic frameworks, improved risk stratification tools and outcome-based management criteria. A multidisciplinary, evidence-based approach involving cardiologists, cardiac imagers, interventional cardiologists and cardiac surgeons is crucial in order to optimise patient outcomes and reduce the risk of adverse cardiovascular events, including sudden cardiac death. Full article

Coronary artery fistulae (CAF) are uncommon congenital or acquired coronary anomalies. A CAF occurs when a coronary artery bypasses the myocardial capillary bed to directly communicate with a cardiac chamber, a great vessel, or another vascular structure. Many CAFs are found by chance. If haemodynamically significant, a CAF may cause a variety of phenomena e.g., myocardial ischaemia, arrhythmias, heart failure, pulmonary hypertension, infective endocarditis/endarteritis, aneurysm formation, and late thrombotic complication. Management is anatomy-driven and dependent on the precise definition of the CAF’s origin, course, termination, multiplicity, associated coronary remodeling, and complications, together with an assessment of physiological relevance. Invasive coronary angiography is indispensable for real-time haemodynamics and transcatheter therapy, yet the two-dimensional projection nature can incompletely characterize complex CAF anatomy. Gated computed tomography coronary angiography (CTCA) produces high-resolution volumetric imaging with robust three-dimensional (3D) reconstruction and is central to contemporary diagnosis, quantitative risk stratification, procedural planning, and follow-up. This review examines the role of CTCA for the diagnosis and management of CAF and aims to provide a comprehensive overview for physicians managing this esoteric group of patients. Full article

Coronary–bronchial artery fistulas (CBAFs) represent a rare subset of coronary artery fistulas characterised by an abnormal communication between an epicardial coronary artery and the bronchial arterial circulation. Although historically considered incidental findings, the widespread use of multimodality cardiovascular imaging—particularly coronary computed tomography angiography—has led to increasing recognition of these anomalies in contemporary clinical practice. The clinical significance of CBAFs varies widely and depends primarily on fistula size, shunt magnitude, and associated cardiopulmonary conditions. While many small fistulas remain asymptomatic, larger or haemodynamically significant lesions may result in myocardial ischaemia due to coronary steal, ventricular remodelling, pulmonary manifestations such as haemoptysis, and aneurysmal degeneration of the fistulous tract. A comprehensive evaluation typically requires an integrated multimodality approach combining anatomical imaging, functional ischaemia testing, and, in selected cases, invasive haemodynamic assessment. Management strategies range from conservative surveillance in small asymptomatic fistulas to percutaneous or surgical closure in symptomatic or haemodynamically significant lesions. This review provides an updated overview of the epidemiology, pathophysiology, diagnostic evaluation, and management of CBAFs. Particular emphasis is placed on size-based clinical stratification, multimodality imaging strategies, and contemporary therapeutic approaches, with the aim of offering a practical framework for the diagnosis and longitudinal management of patients with this uncommon but clinically relevant coronary anomaly. Full article

Anomalous coronary artery origin from pulmonary artery and coronary–pulmonary fistulas are the major causes of ischemic cardiopathy in children. Both anomalies are characterized by a connection between a higher and a lower pressure chamber causing coronary steal. However, several mechanisms and associated lesions may be responsible of the different presentations of the “coronary steal phenomenon”. The aim of this review is to highlight the different embryology, anatomical features, clinical presentation, and the diagnostic and therapeutic strategy of these coronary anomalies, despite their similar pathophysiology. Full article

Anomalous origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA), also known as Bland-White-Garland syndrome, is a rare congenital coronary anomaly with an estimated incidence of 1 in 300,000 live births. While commonly diagnosed in infancy, adult presentations are exceedingly rare and pose significant diagnostic challenges. Delayed diagnosis may result in progressive myocardial ischemia, heart failure, arrhythmias, or sudden cardiac death. Surgical correction is the definitive treatment, with the goal of restoring a dual coronary artery system and preventing irreversible myocardial damage. We present the case of a 30-year-old male with a prior history of non–ST-elevation myocardial infarction who was referred for evaluation of exertional angina and symptoms of heart failure. Transthoracic echocardiography revealed a dilated left ventricle with an ejection fraction (LVEF) of 35%. Coronary angiography and cardiac MDCT identified an anomalous origin of the left circumflex artery (LCx) from the right pulmonary artery (RPA) and a coronary–pulmonary artery fistula involving the LAD. The patient underwent successful surgical correction with reimplantation of the LCx into the ascending aorta. Postoperative recovery was uneventful. At 3-month follow-up the patient was symptom-free, though echocardiography revealed persistent LV dilation and reduced LVEF, necessitating continued pharmacologic therapy and monitoring. This case highlights the importance of maintaining a high index of suspicion for ALCAPA in adult patients with unexplained cardiomyopathy or ischemic symptoms. Early diagnosis and surgical intervention remain crucial for improving long-term outcomes and preventing life-threatening complications. Full article

Background: Takayasu’s arteritis is a rare type of vasculitis with severe complications like stroke, ischemic heart disease, pulmonary hypertension, secondary hypertension, and aneurysms. Diagnosis is achieved using clinical and angiographic criteria. Treatment is medical and surgical, but unfortunately, the outcome is limited. Case presentation: A 34-year-old Caucasian woman had an ischemic stroke (2009). She was diagnosed with Takayasu’s arteritis and received treatment with methotrexate, prednisolone, and antiplatelet agents, with a mild improvement in clinical state. After 6 years (2015), she experienced an ascending aorta aneurysm, pulmonary hypertension, and mild aortic regurgitation. Surgical treatment solved both the ascending aorta aneurysm and left carotid artery stenosis (ultrasound in 2009 and computed tomography angiogram in 2014). Morphopathology revealed a typical case of Takayasu’s arteritis. Tumor necrosis factor inhibitors (TNF inhibitors) were prescribed with methotrexate. At 48 years old (2023), she developed coronary heart disease (angina, electrocardiogram); echocardiography revealed severe pulmonary hypertension, and angiography revealed normal coronary arteries, abdominal aorta pseudoaneurysm, and arterial–venous fistula originating in the right coronary artery with drainage in the medium pulmonary artery. The patient refused surgical/interventional treatment. She again received TNF inhibitors, methotrexate, antiplatelet agents, and statins. Conclusions: This case report presented a severe form of Takayasu’s arteritis. Our patient had multiple arterial complications, as previously mentioned. She received immunosuppressive treatment, medication targeted to coronary heart disease, and surgical therapy. Full article

This case report presents a 34-year-old male with Ehlers–Danlos syndrome, type 2 diabetes mellitus, aortic valve regurgitation, and aortic bulb aneurysm. Following spine surgery for thoracic–lumbar stabilization, the patient underwent assessment for aortic bulb aneurysm and aortic valve replacement surgeries. Five months post spinal surgery, a coronary computed tomography angiography was performed. The coronary computed tomography angiography revealed unique findings, including the absence of the left main coronary artery, right coronary artery dominance, ectopic origin of the left circumflex artery from the right sinus of the valsalva, a coronary–pulmonary arterial fistula originating from the right sinus of the valsalva, and an additional right pulmonary vein. The patient was qualified for surgical treatment for an aortic bulb aneurysm, was informed about the high surgical risk, and is awaiting surgery. This case underscores the rarity of Ehlers–Danlos syndrome coexisting with multiple coronary artery anomalies. The presence of a coronary–pulmonary arterial fistula further emphasizes the need for specialized patient monitoring when Ehlers–Danlos syndrome and coronary anomalies converge. Full article

Coronary-artery-to-pulmonary-artery fistulae represent rare vascular anomalies defined as abnormal communications between the coronary arteries and the pulmonary arterial system. Takotsubo Syndrome represents a stress-induced cardiomyopathy defined by transient regional systolic dysfunction of the left ventricle, with minimal elevation of cardiac biomarkers, without angiographic evidence of obstructive coronary artery disease. We hereby richly illustrate an unusual and rare case of a female patient with Takotsubo Cardiomyopathy and left-anterior-descending-coronary-artery-to-pulmonary-trunk fistula through multi-modality imaging evaluations, obtaining a detailed anatomical representation of the coronary arteries and the fistulous connection, which further guided the optimal treatment strategy. The patient was treated conservatively. The main teaching points of this case are the following: (1) The coronary fistula may represent just an incidental finding in a Takotsubo Cardiomyopathy clinical scenario. (2) The particularly rare association between left-anterior-descending-coronary-artery-to-pulmonary-trunk fistula and Takotsubo Cardiomyopathy presentation is mainly due to the stress-induced overstimulation of myocardial beta-1 receptors, accentuating the coronary steal phenomenon in the setting of the coronary fistula, manifesting as anginal pain, and also the stress-induced adrenergic drive causing the Takotsubo-like presentation with apical ballooning of the left ventricle. Full article

The existence of a coronary-to-pulmonary artery fistula (CPF) in pulmonary atresia with ventricular septal defect (PAVSD) potentially affects treatment; however, its clinical features have not been comprehensively described due to the disease’s rarity. We reviewed 69 cases from 42 studies to reveal the clinical overview of patients with CPF and PAVSD. Among the included patients, the male-to-female ratio was exactly 1:1, and only two patients (3%) exhibited the 22q11.2 microdeletion syndrome. Regarding anatomical features, CPFs originated from the left coronary artery in 65% of patients, and 62% had other major aortopulmonary collateral arteries. Thirty-nine percent of patients had a definitive CPF diagnosis at 0 years of age, whereas 10% were diagnosed in adulthood. Seventy percent underwent catheter angiography to obtain a definitive CPF diagnosis. Ninety-five percent of patients underwent cardiac surgery, and among them, 43% underwent palliative surgery, whereas 52% underwent one-stage repair. Four patients including three adult patients developed cardiac dysfunction due to myocardial ischemia, and three of them exhibited improved cardiac function after the intervention for CPF. Of all the patients, 88% survived and 12% died. The surgical strategy and prognosis were similar to those in PAVSD patients without CPF. This review provides detailed clinical phenotypes that are potentially useful in enhancing the management of patients with this rare disease. Full article

Coronary computed tomography angiography (CCTA) is a non-invasive diagnostic method used (apart from the diagnosis of coronary artery disease) in the diagnosis of malformations of the coronary circulation and monitoring the effects of their treatment. In this paper, the authors present the case of recanalization of the coronary-pulmonary fistula, which was surgically closed in the past. This case highlights that follow-up CCTA after surgical treatment of coronary artery fistula should be performed in every patient. The recommendations regarding the frequency of such follow-up should be made. Full article

Coronary artery anomalies represent a disease spectrum from incidental to life-threatening. Anomalies of coronary artery origin and course are well-recognized in human medicine, but have received limited attention in veterinary medicine. Coronary artery anomalies are best described in the dog, hamster, and cow though reports also exist in the horse and pig. The most well-known anomaly in veterinary medicine is anomalous coronary artery origin with a prepulmonary course in dogs, which limits treatment of pulmonary valve stenosis. A categorization scheme for coronary artery anomalies in animals is suggested, dividing these anomalies into those of major or minor clinical significance. A review of coronary artery development, anatomy, and reported anomalies in domesticated species is provided and four novel canine examples of anomalous coronary artery origin are described: an English bulldog with single left coronary ostium and a retroaortic right coronary artery; an English bulldog with single right coronary ostium and transseptal left coronary artery; an English bulldog with single right coronary ostium and absent left coronary artery with a prepulmonary paraconal interventricular branch and an interarterial circumflex branch; and a mixed-breed dog with tetralogy of Fallot and anomalous origin of all coronary branches from the brachiocephalic trunk. Coronary arterial fistulae are also described including a coronary cameral fistula in a llama cria and an English bulldog with coronary artery aneurysm and anomalous shunting vessels from the right coronary artery to the pulmonary trunk. These examples are provided with the intent to raise awareness and improve understanding of such defects. Full article