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.

Anomalous right coronary artery (RCA) from the left aortic sinus with significant arrhythmic burden in a pediatric patient: a case report

A rare twist: STEMI triggered by an anomalous right coronary artery

Invasive Right Anomalous Coronary Arteries Assessment

Acute myocardial infarction in the setting of anomalous single coronary artery

Three-dimensional computed tomography provides an accurate interpretation of anomalous coronary arteries and related anatomy, which helps determine the ideal surgical approach in rare cases.

Anomalous aortic origin of coronary arteries (AAOCA) is the second leading cause of sudden cardiac death in young athletes and poses significant diagnostic and management challenges due to its variable anatomy and unpredictable clinical course. We retrospectively analyzed 17 pediatric patients (10 male, 7 female; median age 12 years) diagnosed with AAOCA between 2016 and 2025. All patients underwent echocardiography and electrocardiogram-gated computed tomography angiography. Risk stratification considered interarterial course, intramural segment, and ostial morphology. Anomalous right coronary artery was most common (n=11, 64.7%), followed by anomalous left coronary artery (ALCA) (n=4, 23.5%) and anomalous circumflex arteries (n=3, 17.6%). Interarterial course was present in 12 patients (70.6%), and intramural course in 9 patients (52.9%). The main presenting symptoms were exercise-induced chest pain (n=10, 58.8%), syncope (n=8, 47.1%), dizziness (n=7, 41.2%), and palpitations (n=6, 35.3%). Syncope was predominantly observed in ALCA patients with interarterial course. Among 15 patients aged ≥8 years who underwent exercise stress testing, 6 (40%) showed inducible ischemia. Nine patients (52.9%) underwent surgical intervention, including unroofing (n=7), ostial reimplantation (n=1), and pulmonary artery translocation (n=1). The remaining eight patients (47.1%) were managed conservatively, six of whom received beta-blocker therapy. Transthoracic echocardiography reliably identified AAOCA, showing excellent concordance with computed tomography and surgical findings. Standardized echocardiographic protocols are crucial, especially for children presenting with unexplained syncope or exertional symptoms, and can guide optimal management strategies.

Background: Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA), also known as Bland-White-Garland syndrome, is a rare congenital anomaly in which the left coronary artery arises from the pulmonary artery instead of the aorta. This leads to myocardial ischemia via a coronary steal phenomenon. While typically fatal in infancy, a small proportion of patients survive into adulthood due to the development of extensive collateral circulation from the right coronary artery (RCA). Prompt surgical correction is essential to prevent fatal outcomes. Case Presentation: We report the case of a 36-year-old male who presented with a one-year history of exertional angina (CCS Class II) and dyspnea (NYHA Class III). Investigations revealed ALCAPA with a dilated, super-dominant RCA, and associated partial anomalous pulmonary venous return (PAPVR) and moderate mitral regurgitation. Surgical correction was performed using the Takeuchi procedure with simultaneous mitral valve repair and PAPVR correction. The patient had an uneventful recovery. Results: The patient demonstrated marked postoperative improvement and was discharged in stable condition on postoperative day four. At two-year follow-up, cardiac CT confirmed a patent intrapulmonary baffle with no ischemic changes or mitral regurgitation. The patient remained asymptomatic and resumed normal daily activities, indicating excellent long-term functional recovery. Conclusion: This case highlights the importance of early recognition and individualized surgical planning in adult ALCAPA, particularly when associated with structural defects such as mitral regurgitation and PAPVR. The Takeuchi procedure, when appropriately applied, offers sustainable long-term outcomes and should be considered a viable option in anatomically challenging adult presentations.

Anomalous Left Coronary Artery from the Pulmonary Artery: Hiding in Cath Lab

BackgroundPercutaneous coronary intervention (PCI) for anomalous right coronary artery (ARCA) remains technically challenging due to variable ostial anatomy. The Extra Back-Up (EBU) catheter, although designed for left coronary interventions, may offer advantages in ARCA PCI, but its performance across anatomical subtypes is not well defined.MethodsThis single-center retrospective case series included 17 patients (2019–2024) with ARCA from the left coronary sinus or with high take-off anatomy, in whom an EBU catheter was used. Fourteen patients underwent PCI, one had fractional flow reserve (FFR)-only assessment, and two underwent diagnostic angiography alone. Anatomical subtypes were: Type A (ostium above left coronary artery [LCA]), Type B (below LCA), Type C (midline), or high take-off (≥10 mm above sinotubular junction). Primary outcomes were procedural success (stable engagement, full device delivery, no catheter exchange) and safety.ResultsProcedural success with the EBU catheter was 100% in Type A (2/2) and 83.3% in Type B (5/6) when used as the initial guide. In Type C and high take-off anatomies, success was achieved in one of two cases each. EBU was also employed as a second-choice catheter in five patients(one Type A, three Type B, one high take-off) after failed engagement with standard guides catheters. No catheter-related dissections occurred. One minor in-hospital complication (5.9%) and one patient required unplanned re-intervention within 30 days.ConclusionsIn this descriptive series, the EBU catheter was successfully employed as a primary or rescue tool in a majority of select ARCA cases, with the highest success in Types A and B. These observations, while not comparative, suggest it is a viable option in the operator’s arsenal for these challenging anatomies and warrant further prospective evaluation.

Anomalous aortic origin of a coronary artery (AAOCA) is a rare but potentially life-threatening congenital heart defect, occurring in approximately 0.4–0.8% of the population. While many patients remain asymptomatic, certain variants—particularly anomalous origin of the left coronary artery (AAOLCA) with interarterial or intramural courses—carry an elevated risk of sudden cardiac arrest (SCA), especially during exertion. Primary care providers play a critical role in the early identification and coordination of care for patients with AAOCA. Although routine electrocardiograms are typically normal, exertional chest pain or syncope should prompt referral to pediatric cardiology. Echocardiography is often the first-line diagnostic tool, but further imaging with cardiac computed tomography (CCT) and/or magnetic resonance imaging (MRI) is essential for confirming the diagnosis and risk-stratification. Identifiable high-risk anatomical features include a slit-like ostium, intramural course, and acute angle of take-off. Exercise stress testing is used for risk stratification but has low sensitivity and is recommended to be used with adjunctive testing such as stress perfusion imaging and occasionally cardiac catheterization. Surgical repair is indicated in patients with AAOLCA, symptomatic individuals, or those with demonstrable ischemia. Asymptomatic patients with AAORCA and low-risk features may be followed conservatively. Regardless of surgical status, all patients require lifelong cardiology follow-up, periodic imaging, and individualized sports clearance. Additionally, first-degree relatives may warrant screening due to potential familial clustering. AAOCA impacts quality of life, with patients and families often experiencing emotional distress due to lifestyle restrictions. Primary care providers should monitor for psychosocial concerns and coordinate mental health support as needed. This review aims to equip primary care clinicians with practical knowledge to ensure timely referral, appropriate counseling, and long-term support for children with AAOCA.

Objective: To investigate the impact of imaging anatomical features on the prognosis of middle-aged and elderly patients with anomalous coronary artery originating from the opposite sinus (ACAOS). Methods: This retrospective cohort study enrolled patients aged ≥45 years who were diagnosed with ACAOS by coronary computed tomography angiography (CTA) at the Affiliated Hospital of Xuzhou Medical University between January 1, 2011, and December 31, 2018. Baseline clinical data and coronary CTA images were collected. Anatomic imaging features were extracted, including ACAOS subtype, course of the anomalous vessel, and ostial angle. The primary endpoint was major adverse cardiovascular events (MACE), defined as a composite of acute myocardial infarction, cardiac death, and coronary revascularization. The Maxstat method was used to determine the cut-off values of specific anatomical features for predicting MACE risk. Cox proportional hazards models were employed to analyze the impact of these imaging features on MACE in middle-aged and elderly ACAOS patients. Results: A total of 203 patients were enrolled, aged (60.0(51.0, 66.0)) years, including 119 (58.6%) males. Over a follow-up of (98.2±30.9) months, 39 patients experienced MACE. Maxstat identified an optimal cut-off value of 34.3° for the ostial angle of the anomalous vessel. Multivariable Cox proportional hazards regression analysis revealed that the following factors were independent risk factors for MACE: left-sided ACAOS subtype (HR=4.35, 95%CI 2.17-8.73, P<0.001), an interarterial course between the aorta and pulmonary artery (HR=3.21, 95%CI 1.23-8.37, P=0.017), an anomalous vessel ostial angle <34.3° (HR=2.62, 95%CI 1.18-5.77, P=0.017), and concomitant coronary artery disease (HR=2.92, 95%CI 1.49-5.73, P=0.002). Conclusion: In middle-aged and elderly patients with ACAOS, the imaging anatomical features of a left-sided ACAOS subtype, an interarterial course, and an ostial angle <34.3° are independent risk factors for MACE.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly typically diagnosed in infancy. Adult presentation is uncommon and often discovered incidentally. Management becomes more complex in the setting of prior cardiac surgeries, particularly mechanical valve replacement. We report a case of a 26-year-old male with a history of mitral valve repair in 2003 and mechanical mitral valve replacement in 2016, who presented with progressive fatigue and exertional dyspnea. Transthoracic echocardiography revealed severe patient-prosthesis mismatch. Preoperative computed tomography incidentally diagnosed ALCAPA, which had not been detected in previous surgeries. The patient underwent successful redo mitral valve replacement and ALCAPA revascularization using a 5-mm polytetrafluoroethylene vascular prosthesis tunneled through the pulmonary artery. Postoperative recovery was uneventful, and follow-up echocardiography demonstrated satisfactory mitral valve function, patent graft flow, and no significant pulmonary valve gradient. This case highlights the importance of preoperative multimodality imaging in redo cardiac surgery and demonstrates the feasibility of coronary artery revascularization using an intrapulmonary vascular prosthesis for an adult with ALCAPA in a surgically complex setting.

We report a four-year-old female with anomalous left coronary artery from the pulmonary artery who developed a large left ventricular (LV) aneurysm following coronary reimplantation in early infancy and subsequent mitral valve repair and replacement for progressive mitral valve regurgitation. While the patient was asymptomatic, echocardiogram demonstrated an enlarged LV aneurysm, which was confirmed by cardiac computed tomography angiography. Surgical exclusion of the aneurysm with a patch and replacement of the prosthetic mitral valve were performed. The etiology of late development of an LV aneurysm is inconclusive but could be associated with prior ischemic injury and additional local myocardial impairment following the mitral valve replacement. Routine postoperative surveillance is recommended.

ALCAPA (Anomalous Left Coronary Artery from the Pulmonary Artery) is a rare and dangerous congenital anomaly that is one of the leading causes of myocardial ischemia and infarction in pediatric patients. Without timely treatment and correction, the disease can lead to extremely severe consequences: up to 90 % of patients die within the first year of life. In adults, patients suffering from this syndrome may face various complications such as left ventricular dysfunction, mitral regurgitation, asymptomatic myocardial ischemia and infarction, and a high risk of sudden cardiac death. Given that this disease is rarely diagnosed, it is important to detect it early for preventive treatment and avoidance of serious disorders.This paper describes a unique case of first-diagnosed ALCAPA syndrome in a 27-year-old female patient. In addition to the malformation, the woman suffered from severe concomitant pathology — primary sclerosing cholangitis, which was complicated by liver cirrhosis and hypersplenism. The presence of these diseases significantly complicated the choice of therapeutic tactics, requiring a personalized approach. Due to the high risk of postoperative complications, preference was given to minimally invasive endovascular embolization of the left coronary artery. The operation showed good results and led to significant improvement of the patient’s condition, including the achievement of heart failure compensation up to functional class II (NYHA). This case highlights the importance of an individualized approach in the treatment of rare adult congenital heart disease, especially in the presence of serious concomitant diseases of other organs and systems.

The objective of our study was to establish the prevalence of ischemia during exercise stress echocardiography (ESE) in patients with anomalous aortic origin of coronary arteries (AAOCA). A cohort of 46 patients with AAOCA was retrospectively included in this study. Treadmill/bike exercise stress echo tests for ischemia assessment were performed and analyzed according to ESC guidelines. Computed tomography coronary angiography (CTCA), cardiac magnetic resonance, myocardial perfusionscintigraphy and invasive angiography with intravascular ultrasound if needed were used for coronary artery (CA) morphologyand myocardial perfusion assessment. Most patients (70%) were overall symptomatic at rest, 57% reported chest pain and 2% had cardiac arrest before the ESE. By contrast, only 2% of patients reported chest pain during ESE. CTCA revealed that 70% of patients had an inter-arterial course, 17% were found to have an intramural course and 24% had a slit-like ostium of their anomalous CA. Other high-risk features were less frequent findings. All myocardial perfusion studies were negative and only one patient with AAOCA developed ischemia during ESE. Following investigations, four patients were eligible for surgical interventions and were operated on while the remaining patients were followed up for a median of 3 years with no adverse cardiovascular events. Incidence of ischemia on exercise stress echo is extremely low among middle-aged patients with AAOCA despite symptoms at rest and malignant anatomical features. A good short-term outcome in unoperated AAOCA patients following negative exercise stress echocardiography has been shown.

Multidisciplinary approach to the management of anomalous left coronary artery

Primary angioplasty in an anomalous left main coronary artery arising from right coronary sinus presenting with extensive anterior wall STEMI

Background: Sudden Cardiac Death (SCD) is one of the common causes of death in young athletes, especially during exercise, and it continues to be a challenge. Although the incidence of SCD among the aged is low, this problem has strong emotional and societal implications. Hypertrophic cardiomyopathy, arrhythmogenic cardiomyopathy, and anomalous coronary arteries are all potentially fatal heart diseases that are frequently blamed for these types of tragic events when they are found at autopsy, and might be preventable with early recognition. Objectives: This review seeks to evaluate where we stand with SCD in the young athlete, determine the aetiology, and suggest guidelines for diminishing its occurrence. This paper underscores the significance of pre-participation screenings, the availability of Automated External Defibrillators (AEDs), and formalised Emergency Action Plans (EAPs) in the mitigation of SCD risks, centring in particular on inadequate resources in areas such as American Samoa. Methods: A literature review of the past decade was conducted, including the databases PubMed, Scopus, and Google Scholar. The search also included findings related to the prevalence of SCD, risk factors, screening and prevention in youth athletes and public health accounts highlighting systemic inequities in specific locales. Results: SCD in young athletes occurs with a prevalence of 1 per 300,000 per year. Activities with high athletic risk profiles, like soccer and basketball, are particularly linked with SCD. Undiagnosed heart disease is the main aetiological factor, while ECG screening and AED availability demonstrate a better survival. But there is still inequity, particularly in underserved areas like American Samoa. Conclusion: Strategies, including widespread screening programs, availability of AEDs, and trained personnel, are required to decrease the prevalence of SCD. A combined approach toward detection and response is required in order to avoid preventable deaths in young sportsmen and sportswomen.

Anomalous left coronary artery from the noncoronary sinus, diagnosed using transesophageal echocardiography.