Search Results (114)

Objectives: Sudden cardiac death (SCD) is a leading cause of mortality, accounting for approximately 50% of all cardiovascular deaths and 20% of all-natural deaths in Western countries. In individuals over 50 years of age, coronary artery disease (CAD) is responsible for more than 80% of cases, whereas in younger subjects SCD is more frequently associated with non-ischemic myocardial diseases, including hypertrophic cardiomyopathy (HCM), arrhythmogenic cardiomyopathy (ACM), dilated cardiomyopathy (DCM), and myocarditis. Additional causes in young adults include coronary artery anomalies and primary arrhythmic disorders related to channelopathies. This study evaluated the diagnostic performance of post-mortem cardiac magnetic resonance imaging (PM-CMR) in identifying morphological substrates underlying SCD in formalin-fixed explanted hearts, with particular attention to the concordance between PM-CMR findings and autopsy results in cases of sudden coronary death. Material and Methods: We retrospectively reviewed 110 PM-CMR examinations from the Regional Register of Sudden Cardiac Death of Friuli-Venezia Giulia, of which 101 were included in the final analysis. Results: PM-CMR detected pathological findings in 60 hearts (59%), including acute ischemic lesions in 39 cases and other conditions, such as hypertrophic cardiomyopathy, chronic fibrotic ischemic changes, and adipose metaplasia in 21 cases. A good agreement between PM-CMR and autopsy findings was observed (Cohen’s kappa = 0.8). Conclusions: Overall, PM-CMR proved effective in identifying relevant morphological and signal alterations, supporting conventional autopsy. Despite some limitations, particularly in hyperacute ischemic lesions, PM-CMR appears to play a promising role in the diagnostic work-up of SCD and in supporting family screening programs for primary prevention. 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

The coronary arteries are a pair of arteries that branch off from the aorta and encircle the heart, providing oxygenated blood to the myocardium. Although coronary artery atherosclerosis remains a main cause of morbidity and mortality worldwide, coronary artery anomalies (CAAs) are increasingly recognized as a clinically relevant cause of ischemic events and can be subdivided into origin, course, or termination anomalies. The aim of this narrative review is to summarize the cross-sectional diagnostic imaging and clinical background of CAAs. 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

Background and Clinical Significance: Bicuspid aortic valve (BAV) is the most common congenital heart defect and may coexist with other cardiovascular anomalies. Among these is a single coronary artery (SCA), a rare congenital condition in which the entire coronary circulation originates from a single coronary ostium. Cardiac computed tomography (CCT) enables simultaneous evaluation of coronary artery anatomy and aortic valve morphology with high spatial resolution, which may influence procedural strategy in patients undergoing valve interventions. Case Presentation: This report represents the first documented case of a 59-year-old male with mixed aortic valve disease in whom preoperative CCT revealed the coexistence of BAV, SCA (Lipton type L-I), and myocardial bridging (MB) involving the mid segment of the left anterior descending artery (LAD). Identification of these findings was crucial for preoperative assessment and contributed to the selection of an appropriate surgical strategy. Conclusions: CCT plays a key role in the preoperative evaluation of valvular heart disease, including in patients with coexisting BAV and SCA. It enables individualized procedural planning and minimizes the risk of perioperative complications. Full article

Myocardial bridging (MB) is a congenital coronary anomaly characterized by systolic compression of the intramyocardial arterial segment and delayed early diastolic artery relaxation, resulting in reduced vessel luminal diameter in diastole. Current evidence suggests that MB, particularly in the left anterior descending artery, may cause anginal symptoms and/or myocardial ischemia through several different pathophysiological and cellular mechanisms acting independently or synergistically: (1) delayed early diastolic relaxation of intramyocardial arterial segment; (2) impaired endothelial-dependent vasodilation with vessel smooth muscle cell hyperactivity in the coronary artery with MB, especially within the bridged segment; (3) focal (septal) ischemia due to “septal steal” phenomenon; and (4) development and progression of an atherosclerotic lesion in the coronary artery segment proximal to MB. Patients with isolated-MB may also experience anginal pain and/or myocardial ischemia due to concomitant structural and/or functional abnormalities of the coronary microcirculation. Both MB and coronary microvascular dysfunction refer to a subgroup of patients with angina and/or ischemia with non-obstructive coronary arteries (ANOCA/INOCA). Therefore, it may be challenging to determine whether MB is causing anginal pain and/or ischemia, particularly since both phenomena have also been reported without MB’s existence. Therefore, comprehensive coronary physiology testing should be encouraged in patients with this coronary anomaly to identify the underlying cause of anginal pain and/or myocardial ischemia, enabling optimal therapeutic strategies in these patients. This review is focused on different pathophysiological and cellular mechanisms of MB-related angina and/or ischemia and future perspectives in the functional assessment of MB severity, bearing in mind the complexity of coronary physiology in the presence of this anomaly. Full article

Background: Neonatal and infant aortic arch reconstruction remains a high-risk cardiovascular procedure requiring effective cerebral and myocardial protection. Variability in perfusion strategies may influence early hemodynamic stability and postoperative recovery. This study aimed to evaluate the early and short-term cardiovascular outcomes of a standardized beating-heart aortic arch reconstruction strategy incorporating simultaneous antegrade selective cerebral and continuous coronary perfusion. Methods: In this retrospective single-center cohort study, 31 consecutive neonates and infants undergoing aortic arch reconstruction between November 2022 and December 2025 were analyzed. A standardized surgical protocol was applied, consisting of extensive ductal tissue resection, interdigitating posterior end-to-end anastomosis, anterior autologous pericardial patch augmentation, and moderate hypothermic antegrade selective cerebral perfusion combined with continuous coronary perfusion via innominate artery cannulation. Early postoperative outcomes and short-term echocardiographic follow-up results were assessed. Results: The cohort included 31 patients, 22.6% of whom had complex associated cardiac anomalies requiring concomitant procedures. Median cardiopulmonary bypass and aortic cross-clamp times were 119 and 64 min, respectively. There was no in-hospital mortality. Major complications were infrequent, and median intensive care unit stay was 5 days. During a median follow-up of 6.8 months, one patient (3.2%) developed recoarctation requiring reintervention. No late mortality was observed. Conclusions: A fully standardized beating-heart aortic arch reconstruction strategy incorporating simultaneous cerebral and coronary perfusion demonstrated favorable early cardiovascular and short-term outcomes, even in anatomically complex cases. Preservation of continuous coronary perfusion may be associated with improved myocardial stability and early postoperative recovery; however, these findings should be interpreted as observational and hypothesis-generating given the absence of a control group. Larger multicenter studies with longer follow-up are warranted to confirm these findings. Full article

Background: Intramyocardial bridge (MB) is a coronary anomaly characterized by a segment of the artery tunneling within the myocardium. While often asymptomatic, it may lead to ischemic events. Despite traditional disqualification from competitive sports, 2023 guidelines now permit participation for athletes with MBs that do not meet specific high-risk morphological criteria. This study aims to evaluate a novel combined provocative test, integrating Cardiopulmonary Exercise Testing (CPET) and stress echocardiography for the assessment of myocardial deformation (twist), to assess the functional impact of MB in asymptomatic athletes. Methods: This cross-sectional case–control study included 18 participants (nine cases with “significant” MB diagnosed via Computed Tomography (CT) coronary angiography and nine healthy, trained controls), aged 18–78 years. All subjects underwent evaluation at our facility for competitive certification. Assessment protocols included resting echocardiography, Global Longitudinal Strain (GLS), and Cardiopulmonary Exercise Testing (CPET) to quantify exercise capacity and dynamic myocardial function. Results: No significant differences in echocardiographic parameters were observed between groups at rest. However, during exercise, athletes with MB demonstrated a significant reduction in GLS and ventricular twist compared to the control group. These findings indicate a notable loss of apical reserve in the MB cohort during physical stress. Conclusions: The integration of CPET and myocardial deformation analysis provides an effective diagnostic tool for identifying functional impairment in asymptomatic athletes with MB. This combined approach offers a superior follow-up strategy for managing athletes who may be at risk for ischemic events despite lack of clinical symptoms. 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

Background: Sudden cardiac death (SCD) and sudden cardiac arrest (SCA) remain leading causes of mortality in young athletes, highlighting the importance of cardiac screening prior to play. The guidelines on screening protocol are continually evolving but are often inconsistent across organizations. We explore the role of different screening modalities including electrocardiography (ECG) and various cardiac imaging testing; their effectiveness, cost considerations and clinical utility regarding sports cardiac screening, comparing European and American cardiac screening protocols. Additionally, we also discuss the divergence between the European and American guidelines and appraise the literature surrounding this topic. Methods: A comprehensive literature review was conducted using studies published between 2010–2025 on cardiac screening for young athletes. Parameters included an English filter with review of observational studies, systematic reviews, meta-analyses, and randomized controlled trials (RCTs). Manual searches of PubMed, Embase, and Google Scholar libraries were also performed to enhance the reach of our investigation. Articles were chosen based on relevance to the topic. Results: When compared to PPCS physical exam, ECG advantages include increased detection of cardiac conditions leading to SCD, improved sensitivity and specificity compared to history alone, Disadvantages are the need for trained specialists to complete testing properly, unreliability for detecting congenital coronary anomalies, cost-effectiveness concerns, and false positive risk. However, these disadvantages can be improved with improved ECG interpretation training for non-cardiologists and policies easing access to advanced cardiac care. Conclusions: ECG remains the cornerstone of cardiovascular screening due to its affordability and sensitivity in detecting electrical abnormalities, its limitations necessitate a multimodal approach. Integrating targeted ECG screening with echocardiography and advanced imaging in select cases may enhance diagnostic accuracy while balancing cost-effectiveness and accessibility. While the benefits of routine ECG are strongly supported by the literature, nationwide implementation of it remains challenging due to economic, geographical and logistical restraints. Therefore, more research needs to be conducted on the mortality benefits and cost-effectiveness of routine ECGs implementation in PPCS screening for young athletes. Full article

Background/Objectives: Ventricular repolarization abnormalities (VRA) represent a grey area in athlete screening: some patterns are physiological, while others are precursors to heart disease. Objective: to clarify the natural history of VRA and the associated factors of structural diagnosis. Methods: Retrospective observational single-center study of athletes with resting or stress VRA at the first evaluation, with normal echocardiography; minimum follow-up of 2 years. Clinical data, resting and stress ECG, echocardiography, and selective advanced imaging throughout follow-up were collected. Primary outcome: cardiovascular diagnosis at follow-up; time-to-event analysis and associations between ECG characteristics and diagnosis. Results: Fifty-three athletes (mean age 22.2 ± 9.2 years; 92.5% male) were included; 60.4% had resting VRA, and 100% had exercise-induced VRA at baseline. Over 7.3 ± 4.5 years, 28/53 (52.8%) received a diagnosis; median time-to-detection was 7.0 years (95% CI 6.0–not reached); RMST10 was 6.7 years (95% CI 5.7–7.7). Diagnoses included hypertrophic cardiomyopathy (24.5%), non-ischaemic left-ventricular scar (11.3%), myocardial bridging (7.5%), hypertensive remodelling (5.7%), coronary anomaly (1.9%), and ventricular pre-excitation (1.9%). Persistence of resting VRA from baseline to follow-up was more frequent in athletes with a final diagnosis (p = 0.01), whereas topography and exercise-induced abnormalities did not discriminate groups. Advanced imaging contributed substantially to case ascertainment. No major adverse cardiovascular events have been identified throughout follow-up. Conclusions: In athletes with screening-detected VRA and normal echocardiography, persistence of resting VRA was associated with higher detection of a cardiovascular diagnosis, while exercise-induced changes alone show limited diagnostic yield. The long median time-to-detection supports prolonged, pre-planned surveillance, with priority for advanced imaging in profiles with persistent abnormalities. These findings align with a risk-adapted, personalized management strategy in sports cardiology. Full article