The hidden dangers of the coronary anomalies: an ARCAPA case report.

An anomalous origin of the right coronary artery from the pulmonary artery case report. The diagnosis was made by angiotomography. Reimplantation of the right coronary artery into the ascending aorta and reconstruction of the pulmonary artery were conducted.

Surgical Management of Anomalous Right Coronary Artery in the Adult: Technique and Case Series.

BACKGROUND: The management of adult patients with anomalous aortic origin of the right coronary artery (ARCA) from the left aortic sinus poses important challenges. The presence of symptoms or documented ischaemia, the anatomical characteristics of the ostium, and the course of the coronary determine decision-making. METHODS: A retrospective review was performed of all cases of surgical management of ARCA at a single centre. The primary endpoints were mortality and myocardial infarction at 30 days. Secondary endpoints included recurrence of symptoms, freedom from re-intervention, and mortality during long-term follow-up. RESULTS: From October 2019 to August 2023, 15 adult patients underwent surgery for ARCA; 13 patients were included in this study (mean age 53.9±11.1 years; 10 female). A slit-like orifice, a long intramural segment, and an interarterial course were found in all patients. Twelve (12) patients (92.3%) were symptomatic: nine with angina, combined with dyspnoea on exertion in seven. One (1) patient had history of pre-syncope. One (1) patient presented with out-of-hospital cardiac arrest. All patients underwent formal unroofing of the orifice and intramural portion of the ARCA; five patients had a concomitant procedure. No 30-day mortality nor myocardial infarction was recorded. At a mean follow-up of 20.1±12.8 months, all patients were alive. One (1) patient (7.6%) developed recurrent dyspnoea; investigations showed no ischaemia. No repeated interventions were required. CONCLUSIONS: Surgical unroofing of anomalous coronary artery in the adult is safe and effective; correction of both the slit-like orifice and intramural portion of the anomaly provides a durable result in patients with ARCA.

Dobutamine Stress Cardiac MRI in Pediatric Patients with Suspected Coronary Artery Disease.

Following current practice, pediatric patients with treated congenital coronary malformations or acquired coronary disease undergo Cardio-Pulmonary Exercise Test (CPET), stress Echocardiography and Electrocardiography (sEcho, sEKG), and Coronary Angiography (CA). Stress cMRI can assess cardiac function, myocardial viability, and stress/rest perfusion deficit-without radiation exposure, general anesthesia, and hospitalization-in a single non-invasive exam. The aim of our pilot study is to assess the feasibility and diagnostic accuracy of Dobutamine stress cMRI compared to the current procedures (sEcho, CPET, CA). The prospective study is focused on pediatric patients, at risk for or with previously diagnosed coronary artery disease: d-looped TGA after arterial Switch, Kawasaki disease, and anomalous origin of left coronary artery from pulmonary artery (ALCAPA) after coronary artery reimplantation. We have compared the results of MRI coronary angiography, and Dobutamine stress cMRI with traditional tests. All these diagnostic exams were acquired in a timeframe of 3 month, in a blinded fashion. All the 13 patients (age: 12 ± 2 years, median 12,7 y) recruited, completed the study without major adverse events. The mean heart rate-pressure product was 25,120 ± 5110 bpm x mm Hg. The target heart rate of 85% of the maximal theoretical was reached by 10 (77%) patients. The comparison between cardiac MRI coronarography versus the gold standard Coronary Angiography to identify the patency of the origin and the proximal pathway of the coronary arteries shows a sensitivity of 100% (confidence interval: 2,5-100%), specificity 92% (confidence interval: 64-100%). The stress test was well tolerated for the 77% of the patients and completed by the totality of patients (Table 3). Three patients (23%) had mild symptoms: nausea, vomiting, or general discomfort. In pediatric patients with a potential or definite diagnosis of coronary artery disease, stress cMRI combines an effective assessment of proximal coronary arteries anatomy with cardiac function, myocardial perfusion, and viability in a single examination. Stress cMRI can be proposed as alternative, standalone test.

A rare disease detected in a school-age child aortopulmonary window with anomalous right coronary artery from the pulmonary artery.

Aortopulmonary window is a condition characterized by a communication between the pulmonary artery and the ascending aorta. The coexistence of aortopulmonary window and an anomalous right coronary artery originating from the pulmonary artery is rarely observed together, as mentioned in previous studies. In this report, we aim to describe our diagnostic and treatment experiences with a 6-year-old patient diagnosed with aortopulmonary window associated with an abnormal origin of the right coronary artery from the pulmonary artery.

Evolution of surgical repair of intraseptal anomalous left coronary artery with myocardial ischaemia.

BACKGROUND: Anomalous aortic origin of the left coronary artery with intraseptal course is a rare coronary anomaly associated with an increased risk of myocardial ischaemia. The role and techniques for surgical intervention are evolving, with numerous novel surgical techniques for this challenging anatomy reported in the last 5 years. We report our single-centre experience with surgical repair of intraseptal anomalous left coronary artery in the paediatric population, including clinical presentation, evaluation, and short- to mid-term outcomes. METHODS: All patients with coronary anomalies presenting to our institution undergo standardised clinical evaluation. Five patients aged 4 to 17 years underwent surgical intervention for intraseptal anomalous aortic origin of the left coronary artery between 2012 and 2022. Surgical techniques included coronary artery bypass grafting (n = 1), direct reimplantation with limited supra-arterial myotomy via right ventriculotomy (n = 1), and transconal supra-arterial myotomy with right ventricular outflow tract patch reconstruction (n = 3). RESULTS: All patients had evidence of haemodynamically significant coronary compression, and three had evidence of inducible myocardial ischaemia pre-operatively. There were no deaths or major complications. Median follow-up was 6.1 months (range 3.1-33.4 months). Patients who underwent supra-arterial myotomy (with or without reimplantation) had improved coronary flow and perfusion based on stress imaging and catheterisation data. CONCLUSIONS: Surgical approaches to intraseptal anomalous left coronary artery with evidence of myocardial ischaemia continue to evolve, with new techniques demonstrating promising improvement in coronary perfusion. Further studies are warranted to determine long-term outcomes and refine indications for repair.

Transection and reimplantation of anomalous right coronary artery from single left coronary artery in a collegiate athlete.

Coronary artery anomalies may induce myocardial ischaemia and sudden cardiac death when associated with areas of compression or stenosis. We present a case of transection and reimplantation of an anomalous interarterial right coronary artery arising from a single left main coronary artery. The 18-year-old collegiate athlete had exertional chest pain and haemodynamically significant compromise to coronary blood flow.

A very rare interarterial communication: total occlusion of right coronary artery with antegrade collateral supply from left circumflex artery through conus branch.

PURPOSE: The purpose of the present case report is to describe an extremely rare and unusual coronary interarterial communication. METHODS: A 65-year-old female patient admitted with acute coronary syndrome underwent a coronary angiography performed with Judkins technique to obtain standard angiographic views. RESULTS: We have demonstrated a very rare interarterial communication traversing an unusual retroaortic path between the body of left circumflex artery and the conus branch of the right coronary artery. CONCLUSION: Coronary interarterial communications are rarely encountered; however, may fulfill important tasks in the coronary circulation. Therefore, invasive cardiologists and cardiovascular surgeons should be aware of their presence.

Coronary arterial dominance in patients with congenital heart disease.

Coronary arterial dominance is concerned in the management of ischemic heart disease. In particular, right coronary arterial dominance is having a risk for three-vessel coronary artery disease. Thus, this study aimed to explore coronary arterial dominance in patients with congenital heart disease. The study involved 250 patients, of which 105 patients were with tetralogy of Fallot (TOF), 100 patients with ventricular septal defect (VSD), and 45 patients with Kawasaki disease (KD). We retrospectively reviewed their ascending aortography to determine their coronary arterial dominance, Z-scores of coronary artery diameter, and the ascending aortic curvature, which pertained to the angle between the aortic annulus plane and ascending aortic plane. We identified relevant factors that contribute to having right coronary arterial dominance. Age and weight of the 250 subjects were 2.9 (1.0-8.7) months and 7.7 (5.0-9.4) kg, respectively. The Z-scores of right coronary and anterior descending arteries significantly differed among patients with TOF, VSD, and KD (P < 0.001, P = 0.001). However, there were no significant differences in the Z-scores of left main trunk and circumflex arteries. Right coronary arterial dominance occurred in 89%, 49%, and 61% in patients with TOF, VSD, and KD, respectively (P < 0.001). The presence of TOF was the most powerful predictor for right coronary arterial dominance (odds ratio: 10.31, 95% confidence interval: 4.11-27.2, P < 0.001). We found the robust relationship between right coronary arterial dominance and TOF. Patients with TOF may have an increased risk for the development of coronary artery disease during adulthood.

Anomalous Aortic Origin of the Coronary Arteries - State of the Art Management and Surgical Techniques.

Anomalous aortic origin of a coronary artery (AAOCA) can be associated with myocardial ischemia and sudden cardiac arrest. We describe and compare the management and surgical techniques for patients with AAOCA. Patients presenting to the Coronary Artery Anomalies Program are evaluated and managed following a standardized approach. Our approach and data were compared to other single-center and multi-institutional data and results. Patients with AAOCA present as an incidental finding approximately 50% of the time. Advanced axial imaging is essential to define the anatomic characteristics of this lesion. Preoperative and postoperative assessment of myocardial perfusion with provocative testing is feasible and contributes to risk stratification. The surgical techniques for AAOCA repair include coronary unroofing, transection and reimplantation, and neo-ostium creation, among others. In general, surgical repair of AAOCA can mitigate the risk of ischemia with low mortality. The specific morbidities and complications of each different technique should be considered during the surgical planning. Surgical repair of AAOCA can mitigate the risk of ischemia with a low associated mortality but with clinically relevant morbidities. Long-term follow-up is necessary to accurately balance the risks of repaired and unrepaired AAOCA.

Anomalous Coronary Arteries: When to Follow-up, Risk Stratify, and Plan Intervention.

PURPOSE OF REVIEW: Coronary artery anomalies are a diverse group of entities, ranging from benign variations of normal anatomy to life-threatening conditions. There is, however, no universal consensus in their classification, risk stratification, and management. The aim of this review is to develop a straightforward clinical approach for the assessment and care of patients with anomalous coronary arteries. RECENT FINDINGS: Autopsy series and population screening studies have recently provided useful clinical data on the prevalence and outcomes of coronary anomalies. Also, findings on coronary computed tomography angiography, magnetic resonance imaging, and invasive angiography, enriched with fractional flow reserve and intravascular ultrasound, have allowed identification of several high-risk features associated with specific coronary anomalies. Management of patients with anomalous coronary arteries requires an individualized approach based on clinical, physiological, and anatomic features. High-quality studies are paramount for further development of this fascinating field.

Myocardial infarct late after Fontan-type surgery in pulmonary atresia and intact ventricular septum: a must-know complication! About a case report.

Coronary abnormalities are frequent in pulmonary atresia and intact ventricular septum, mainly in patients with a very diminutive right ventricle. They severely impact on early and late prognosis. We describe an 8-year-old girl who presented with myocardial ischaemia, late after uneventful Fontan completion. The importance of precise delineation of the coronary anatomy upon initial assessment and during follow-up is emphasised.

The first multicentre study on coronary anomalies in the Netherlands: MuSCAT.

BACKGROUND: Current guidelines on coronary anomalies are primarily based on expert consensus and a limited number of trials. A gold standard for diagnosis and a consensus on the treatment strategy in this patient group are lacking, especially for patients with an anomalous origin of a coronary artery from the opposite sinus of Valsalva (ACAOS) with an interarterial course. AIM: To provide evidence-substantiated recommendations for diagnostic work-up, treatment and follow-up of patients with anomalous coronary arteries. METHODS: A clinical care pathway for patients with ACAOS was established by six Dutch centres. Prospectively included patients undergo work-up according to protocol using computed tomography (CT) angiography, ischaemia detection, echocardiography and coronary angiography with intracoronary measurements to assess anatomical and physiological characteristics of the ACAOS. Surgical and functional follow-up results are evaluated by CT angiography, ischaemia detection and a quality-of-life questionnaire. Patient inclusion for the first multicentre study on coronary anomalies in the Netherlands started in 2020 and will continue for at least 3 years with a minimum of 2 years of follow-up. For patients with a right or left coronary artery originating from the pulmonary artery and coronary arteriovenous fistulas a registry is maintained. RESULTS: Primary outcomes are: (cardiac) death, myocardial ischaemia attributable to the ACAOS, re-intervention after surgery and intervention after initially conservative treatment. The influence of work-up examinations on treatment choice is also evaluated. CONCLUSIONS: Structural evidence for the appropriate management of patients with coronary anomalies, especially (interarterial) ACAOS, is lacking. By means of a structured care pathway in a multicentre setting, we aim to provide an evidence-based strategy for the diagnostic evaluation and treatment of this patient group.

Echocardiographic Diagnosis of Anomalous Single Coronary Artery from the Pulmonary Artery: Use of Bubble Contrast Echocardiography.

Anomalous origin of a coronary artery from the pulmonary artery (PA) is a rare congenital anomaly with the most common pattern being an anomalous left coronary artery (LCA) from the pulmonary artery (ALCAPA). A very rare pattern is the anomalous origin of a single coronary artery from the pulmonary artery (ASCAPA) wherein the single coronary ostium supplies both the right and left coronary systems with profound myocardial ischemia developing once PA pressures begin to fall after birth. Previous reports of this anomaly have all been based on post-mortem findings or pre-mortem angiographic diagnosis [1-3]. Notably, these children often present in extremis due to cardiogenic shock. Institution of veno-arterial extracorporeal life support can be catastrophic as it would lead to myocardial ischemia due to PA decompression. We present here the first echocardiographic-only diagnosis of ASCAPA in a 2-month-old child with the use of bubble contrast echocardiography to help confirm the diagnosis. The patient was resuscitated from a cardiac arrest on arrival with subsequent echocardiographic-only diagnosis. Surgical repair was undertaken with administration of cardioplegia into the pulmonary root with snaring of the branch PAs, and re-implantation of the single coronary to the aorta. At 3-month follow-up, he is thriving clinically with echocardiogram showing improving-though still somewhat depressed-left ventricular function.

Ectopic Origin of the Left Anterior Descending Artery from the Proximal Right Coronary Artery: An Imaging Report.

Anomalies of the coronary arteries represent rare congenital disorders, which are characterized by a wide spectrum of clinical manifestations. Usually, they are asymptomatic, but sometimes they cause myocardial ischemia or sudden cardiac death. Here, we describe the case of a patient who suffered from angina. Coronary angiography revealed an ectopic origin of the left anterior descending coronary artery from the proximal trait of the right coronary artery and the left circumflex artery, originating from the left sinus; the whole coronary tree was free of atherosclerosis. To better define the coronary anatomy, we performed computed tomography angiography with a three-dimensional reconstruction. The patient was discharged from the clinic after 48 h under optimal medical treatment.

Comprehensive literature review of anomalies of the coronary arteries.

Coronary artery anomalies (CAA) are vanishingly rare, affecting less than 1% of the general population. While the majority of anomalies do not cause significant symptoms; those that do, have devastating outcomes on the patient. Seventeen percent of deaths from exercise is attributed to CAA, and over half of these present as sudden death making CAA the second most common cause of sudden cardiac death in individuals. Computed tomography is generally regarded as the first-line investigation due to its superior ability to delineate the course of the coronary vessels and the surrounding structures, while intravascular coronary angiography can be helpful in assessing the vessels if there is evidence of stenosis. A multidisciplinary approach is adopted with patient expectations at the core of the management. Once the decision to operate has been made, there are multiple techniques available to the surgeon for the management of anomalous vessels. Surgical repair forms the key management step in such patients. Currently, surgery in elective cases is associated with extremely low morbidity and mortality and it is considered a safe option with a fantastic long-term prognosis. The ideal approach for assessment and risk stratification remains uncertain, and the inherent variability of coronary anomalies and patient factors demands a multidisciplinary team with an individualized approach.

Can we predict potentially dangerous coronary patterns in patients with transposition of the great arteries after an arterial switch operation?

INTRODUCTION: Coronary artery complications are the main reason for early mortality after an arterial switch operation. Late complications are relatively rare, and there is no consensus regarding the need or indications for routine follow-up coronary artery evaluations or the best first-line assessment modality. The aim of this study was to present the long-term post-operative frequency of coronary abnormalities in asymptomatic patients with transposition of the great arteries discovered by coronary CT angiography and potential "red flags" revealed by other examinations. PATIENTS AND METHODS: A group of 50 consecutive asymptomatic patients who underwent routine long-term coronary artery evaluation after an arterial switch operation according to our institutional protocol were qualified for this study. This routine in-hospital visit included a detailed medical interview, electrocardiography, echocardiography, Holter electrocardiography examinations, and laboratory and cardiopulmonary exercise tests. Patients who showed significant abnormalities were qualified for perfusion scintigraphy. RESULTS: Unfavourable coronary abnormalities were detected in 30 patients (60%) and included ostial stenosis, muscular bridge, coronary fistula, interarterial course, proximal kinking, high ellipticity index, proximal acute angulation (<30 degree) of the left coronary artery, and proximal acute angulation of the right coronary artery. These features could not be predicted based on the medical interviews, surgical reports, or non-invasive screening test results. CONCLUSION: Complex coronary configurations with potentially dangerous coronary features are common in patients with transposition after an arterial switch operation. Such high-risk patients cannot be identified indirectly, and coronary CT angiography provides accurate information that is useful for post-operative management.

Novel approaches to study coronary vasculature development in mice.

BACKGROUND: Coronary artery development is an intensely studied field. Mice are a popular genetic model for developmental studies, but there is no widely accepted protocol for high-throughput, high-resolution imaging of their developmental and adult coronary artery anatomy. RESULTS: Using tissue-clearing protocols and confocal microscopy, we have analyzed embryonic and juvenile mouse hearts in Cx40:GFP knock-in models with a special focus on septal artery development. We found that the septal artery, which supplies the interventricular septum, was initially formed as an arterial plexus that connected to both the left and right coronary arteries. During development, the plexus was remodeled into a single tube, which then remained connected only to the right coronary artery. Since optical imaging became limited at postnatal stages, it was supplemented with injection techniques using India ink and Microfil; the latter was subsequently analyzed with micro-CT to visualize the anatomy of coronary vessels in 3D. CONCLUSIONS: The techniques described here enable us to study the finer details of coronary artery development in mice and can, therefore, be implemented to study the pathogenesis of coronary malformations in various mouse models. Developmental Dynamics 247:1018-1027, 2018. © 2018 Wiley Periodicals, Inc.

Acquired right ventricular outflow tract aneurysm in pulmonary atresia and intact ventricular septum: a nidus for thromboembolism.

Pulmonary atresia with intact ventricular septum may be complicated by coronary aneurysms and myocardial ischaemia. We report a case of an acquired postoperative right ventricular outflow tract aneurysm with communication to the left ventricular outflow tract. Multimodality imaging helped in the characterisation of this structural abnormality, which led to treatment modification of the patient.

Paediatric-onset coronary artery anomalies in pregnancy: a single-centre experience and systematic literature review.

OBJECTIVES: Individuals with childhood-onset coronary artery anomalies are at increased risk of lifelong complications. Although pregnancy is thought to confer additional risk, a few data are available regarding outcomes in this group of women. We sought to define outcomes of pregnancy in this unique population. METHODS: We performed a retrospective survey of women with paediatric-onset coronary anomalies and pregnancy in our institution, combined with a systematic review of published cases. We defined paediatric-onset coronary artery anomalies as congenital coronary anomalies and inflammatory arteriopathies of childhood that cause coronary aneurysms. Major cardiovascular events were defined as pulmonary oedema, sustained arrhythmia requiring treatment, stroke, myocardial infarction, cardiac arrest, or death. RESULTS: A total of 25 surveys were mailed, and 20 were returned (80% response rate). We included 46 articles from the literature, which described cardiovascular outcomes in 82 women (138 pregnancies). These data were amalgamated for a total of 102 women and 194 pregnancies; 59% of women were known to have paediatric-onset coronary artery anomalies before pregnancy. In 23%, the anomaly was unmasked during or shortly after pregnancy. The remainder, 18%, was diagnosed later in life. Major cardiovascular events occurred in 14 women (14%) and included heart failure (n=5, 5%), myocardial infarction (n=7, 7%), maternal death (n=2, 2%), cardiac arrest secondary to ventricular fibrillation (n=1, 1%), and stroke (n=1, 1%). The majority of maternal events (13/14, 93%) occurred in women with no previous diagnosis of coronary disease. CONCLUSIONS: Women with paediatric-onset coronary artery anomalies have a 14% risk of adverse cardiovascular events in pregnancy, indicating the need for careful assessment and close follow-up. Prospective, multicentre studies are required to better define risk and predictors of complications during pregnancy.

Left main coronary artery atresia in an infant with Shone's complex.

A 6-month-old infant with Shone's complex was found to have left main coronary artery atresia during evaluation for recurrent subaortic stenosis with depressed left ventricular function. The ventricular function improved after surgical subaortic resection without coronary re-vascularisation. This case demonstrates first the rare finding of left main coronary artery atresia and second that coronary re-vascularisation is not necessarily required in all cases of left main coronary artery atresia.