Search Results (46)

Objective: To characterize the anatomical spectrum of a single coronary artery (SCA) and an anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS) and to assess high-risk features using Coronary computed tomography angiography (CCTA)-based quantitative parameters. Methods: A retrospective review of 2513 consecutive CCTA examinations performed between June 2021 and December 2025 was conducted. Patients diagnosed with ACAOS or SCA were included. Coronary origin, course patterns, and morphological characteristics were analyzed. High-risk features—including interarterial course, intramural segment, acute take-off angle, slit-like ostium, intramural length, and interluminal space (ILS)—were quantitatively assessed using multiplanar and curved MPRs. Results: Twenty-two patients (0.88%) were identified (ACAOS: 0.64%, n = 16; SCA: 0.24%, n = 6). All SCA cases showed benign courses. Among ACAOS patients, 62.5% had an interarterial course. In this subgroup, the mean take-off angle was 14.4° ± 4.5°, with universal slit-like ostium and intramural course. Mean intramural length was 9.21 ± 2.00 mm and minimal ILS was 0.87 ± 0.12 mm. Conclusions: CCTA enables comprehensive anatomical characterization of morphological features associated with higher risk in patients with ACAOS and SCA. Quantitative assessment of parameters such as the take-off angle, intramural course, slit-like ostium, intramural length, and interluminal space may complement clinical evaluation, although further prospective studies incorporating functional ischemia assessment and clinical outcome data are needed to clarify their clinical significance. Full article

Background: Exercise habits are unknown in pediatric patients with anomalous aortic origin of coronary arteries (AAOCA). In this study, we aim to identify differences in exercise habits and their relationship to BMI and other demographic factors in pediatric patients with AAOCA. Methods: A single-center, cross-sectional study of 573 patients with AAOCA (mean age = 10.5 years, SD 6.3, 63% males) was conducted. Exercise habits were classified as recreational or organized/competitive. BMI percentiles were stratified as normal (5th to ≤85th), overweight (85th to ≤95th), and obese (≥95th). Exercise habits and BMI were compared by demographic factors, presence of ischemia, and surgery status. Means were compared using two-tailed t-tests and multivariate linear regression with a false discovery rate correction. χ² tests were used for categorical variables. Results: Nearly two-thirds (63.5%) of patients exercise recreationally, and 35% of the total cohort are overweight or obese (BMI > 85th%ile). Those who exercise competitively (29%) were more likely to have had surgery (24% vs. 15%, p = 0.019) and less likely to be exercise-restricted (3% vs. 8.5% p = 0.018). BMI in right-AAOCA is significantly elevated compared to left-AAOCA (63.1 vs. 53.1 percentile, p = 0.018). There was no difference in BMI percentile in those restricted from exercise or in those who exercised competitively. BMI and exercise habits are unrelated to ischemia, AAOCA subtype, exercise habits or restriction on multivariate analysis. Conclusions: One-third of AAOCA patients are overweight or obese, and BMI is not associated with exercise habits in this cohort. Surgical patients were more likely to exercise competitively, and this may have implications for personal choices to engage in exercise. Future studies are needed to determine optimal exercise recommendations to promote active lifestyles while minimizing the risk of sudden death. Full article

Background: Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is an extremely rare and potentially fatal congenital heart defect, accounting for approximately 0.003% of all coronary artery anomalies. Methods: A literature search was performed in the PubMed and eLibrary databases using the keywords “ARCAPA” and “anomalous origin of the right coronary artery from the pulmonary artery” for the period from 2005 to 2025. A total of 158 papers were screened, from which 57 articles reporting 59 clinical cases in adult patients were selected. Results: The median age was 55 years [18–80]. Males accounted for 54.2% (32/59). ARCAPA was an incidental finding during evaluation for another condition in 35.9% (21/59) of cases. Clinical presentation: retrosternal pain—47.5% (28/59), dyspnea—44.1% (26/59), and reduced exercise tolerance—35.9% (21/59). A completely asymptomatic course was observed in 22.0% (13/59) of patients. In rare cases, the initial presenting manifestations included atrial fibrillation, cardiac arrest during a marathon, and worsening dyspnea during pregnancy. The most commonly used diagnostic modalities were computed tomography angiography—in 67.8% (40/59) of cases—and coronary angiography—in 49.2% (29/59) of cases. Cardiac catheterization confirmed the diagnosis in only 10.2% (6/59) of patients. Surgical correction was performed in 54.2% (32/59) of patients; the most frequently used technique was reimplantation of the right coronary artery into the ascending aorta—in 42.4% (25/59) of cases. One long-term complication was recorded—right coronary artery thrombosis 17 years after surgery—and one case of sudden cardiac death occurred in an unoperated patient. Conservative management was chosen in 40.7% (24/59) of patients, mainly due to an asymptomatic course, high surgical risk, or patient refusal. Conclusions: ARCAPA is a rare anomaly for which surgical treatment appears to be the preferred approach in symptomatic patients or those with documented ischemia, while conservative management may be acceptable in selected asymptomatic patients. Conservative management is acceptable in truly asymptomatic patients, but regular follow-up is recommended. Full article

Coronary anomalies in the form of anomalous aortic origin of the coronary arteries (AAOCA) are rare, but they may cause sudden cardiac death during physical activity in individuals under 35 years of age. We present the case of a 51-year-old man diagnosed with a coronary artery anomaly—anomalous origin of the right coronary artery (RCA) from the left coronary sinus (AAORCA). The patient complained of palpitations during exercise, and ventricular arrhythmia was detected during an exercise electrocardiographic stress test. During coronary angiography, selective cannulation of the RCA was unsuccessful despite the use of multiple catheters. Therefore, coronary computed tomography angiography (CCTA) was performed, which revealed a high-risk anatomical variant of AAORCA. Full article

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

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly with exceptional survival into adulthood. We present a 66-year-old woman with chest and back pain in whom ALCAPA was diagnosed using coronary computed tomography angiography (CCTA) with curved planar reformation and cinematic volume rendering technique (cVRT). Photorealistic three-dimensional reconstruction provided complementary three-dimensional visualization that may facilitate anatomic understanding and communication of the anomalous origin. Conservative management was adopted given the patient’s age and well-developed collateral circulation. This case underscores the value of advanced CCTA visualization in diagnosing rare coronary anomalies in elderly patients. Full article

Intramyocardial coronary artery course is a rare anatomical variant that can be increasingly recognized with coronary computed tomography angiography (CCTA). We present the case of a 22-year-old male who underwent CCTA for evaluation of chest pain. Imaging demonstrated an unusual course of the left anterior descending artery (LAD), which traversed toward the right ventricular cavity over an approximately 21 mm segment. Multiplanar reconstructions and three-dimensional volume-rendered images clearly depicted the intramyocardial trajectory of the vessel. Although usually asymptomatic, recognition of this variant is important because intramyocardial coronary arteries may be vulnerable to injury during intracardiac procedures. This case highlights the role of CCTA in accurately characterizing a rare intracavitary LAD course with clear delineation of its intramyocardial-to-intracavitary trajectory toward the right ventricle using multiplanar and three-dimensional reconstructions. 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

This case report highlights the coexistence of two rare coronary artery anomalies assessed by computed coronary tomography angiography (CCTA). We present the case of a 51-year-old hypertensive patient with a type II double left anterior descending artery (LAD) and an anomalous infundibular branch originating from the pulmonary artery (partial ARCAPA). This association may have contributed to a limited ischemic burden, explaining the patient’s asymptomatic state. Knowledge of these rare coronary anatomies is essential for accurate diagnosis and management. Full article

Introduction: Congenital structural anomalies of the pulmonary artery in children, encompassing defects such as pulmonary atresia (PA), pulmonary stenosis (PS), pulmonary artery hypoplasia, and tetralogy of Fallot (ToF), pose significant challenges in pediatric cardiac surgery due to impaired blood flow in pulmonary circulation. Traditional options for conventional repair—including autologous materials such as the native pericardium and synthetic materials such as artificial patches—have limitations including a lack of growth potential and vulnerability to restenosis over time. ProxiCor^® patches, based on the extracellular matrix (ECM), have emerged as biologically compatible substitutes capable of fostering tissue regeneration. The primary outcomes of this study were the safety (absence of patch-related complications such as restenosis, dilation, aneurysm, infection, or thrombosis) and feasibility (intraoperative handling and surgical success) of ProxiCor^® for pulmonary artery and right ventricular outflow tract (RVOT) reconstruction in a single-center pediatric cohort. Secondary outcomes included mortality, postoperative complications (prolonged mechanical ventilation > 72 h, need for continuous renal replacement therapy (CRRT), and intensive care unit (ICU) and hospital stay), and qualitative echocardiographic assessment of vessel patency during follow-up. Patients and methods: A retrospective analysis was conducted in 25 consecutive pediatric patients who underwent pulmonary artery or RVOT reconstruction with ProxiCor^® at the Department of Pediatric Cardiac Surgery in Poznań (Poland) between the years 2023 and 2024. Surgical techniques, clinical outcomes, and follow-up data were assessed using transthoracic echocardiography (TTE). Results: The median age was 224 (Q1–Q3: 124–362) days, and median weight was 4.2 (Q1–Q3: 2.8–8.5) kg. Procedures targeted repairs of the main pulmonary artery (MPA), right pulmonary artery (RPA), left pulmonary artery (LPA), and RVOT. Diagnoses included tetralogy of Fallot (ToF), pulmonary artery stenosis (PS), pulmonary atresia (PA), pulmonary artery hypoplasia, and anomalous left coronary artery from the pulmonary artery (ALCAPA). The mortality rate stood at 8% (2/25), stemming from multiorgan failure and hemorrhagic stroke, unrelated to the patch. Over a median observation period of 483 (Q1–Q3: 363–584) days, no patch-related complications (e.g., restenosis or dilation) arose. The median hospitalization time was 22 (Q1–Q3: 8.5–38.5) days. Conclusions: ProxiCor^® ECM patches appear to be safe and feasible for use in pulmonary artery and RVOT reconstruction, with favorable early outcomes. However, the small cohort size, lack of a control group, and limited mid- to long-term echocardiographic data preclude definitive conclusions about long-term outcomes or comparative effectiveness. 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

Coronary anomalies, although rare, should be considered when young patients present with angina. Clinical suspicion and multi-modality imaging including coronary angiography and tomographic imaging should be pursued for symptomatic patients such as the one we are presenting with anomalous right coronary artery from the pulmonary artery. She was promptly referred for surgical intervention with re-implantation of the right coronary artery onto the aorta. Full article

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital coronary anomaly, with an uncertain prevalence and often diagnosed incidentally. This case report presents a 62-year-old male with ARCAPA diagnosed during an evaluation for chest surgery. The patient had a history of colon cancer and active tuberculosis, complicating the clinical management. He reported chest pain, shortness of breath, and palpitations, with atrial fibrillation observed on a 24 h Holter ECG. Coronary angiography revealed robust collateral circulation and a suspected anomalous origin of the right coronary artery, confirmed by CT imaging. The patient’s stress MRI showed mildly reduced left and right ventricular ejection fractions and perfusion deficits in the apical segments (2/17) of the septal and inferior walls. Given the patient’s comorbidities, including active tuberculosis, the Heart team decided on a non-operative management approach, focusing on careful monitoring and pharmacological management rather than immediate surgery. This case emphasizes the complexity of managing ARCAPA in the context of significant comorbidities, highlighting the importance of individualized, multidisciplinary treatment strategies. Early diagnosis using advanced imaging techniques is crucial, and a non-operative approach can be considered in patients with preserved left ventricular function and no significant ischemia, as demonstrated in this case. Full article

Background: Congenital coronary artery anomalies (CAAs) are a significant cause of sudden cardiac death and a key factor in determining athletes’ eligibility for competitive sports. Their prevalence varies with diagnostic modalities and may present as asymptomatic or with life-threatening ischemic or arrhythmic events. This case series highlights the diverse manifestations of CAAs and the clinical approaches used to determine sports eligibility. Cases description: Five competitive athletes with different CAAs are presented. These cases include anomalous coronary origins, intramyocardial bridges, and coronary fistulas. Diagnostic tools, including coronary CT angiography (CCTA), cardiac magnetic resonance imaging (CMR), and stress tests, were essential in evaluating these anomalies and determining treatment strategies. In some cases, such as intramyocardial bridges, surgical intervention was necessary, while others required conservative management or exclusion from competitive sports. Conclusions: CAAs require individualized care based on risk stratification through advanced imaging techniques and functional assessment. Surgical interventions are reserved for high-risk anomalies, while others may be managed conservatively. Early detection and tailored management are crucial for ensuring athletes’ safety, and ongoing research is needed to optimize long-term outcomes. Full article

Background and Clinical Significance: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. Such patients are unlikely to survive adulthood without a major surgical correction. Case Presentation: We report a 30-year-old female with a lifelong murmur who presented to the sports cardiology clinic with progressively reduced exercise tolerance. She was eventually diagnosed with ALCAPA and underwent successful Takeuchi repair. Conclusions: Surgical correction is strongly recommended upon diagnosis to mitigate the associated risks and improve the prognosis for affected individuals. Full article