Anomalous Right Coronary Artery From Pulmonary Artery Presenting as Inferior Wall Ischemia: A Case Report.

We illustrate the case of a 62-year-old man with a symptomatic anomalous right coronary artery from pulmonary artery (ARCAPA). Our patient had presented with dyspnea on exertion with electrocardiogram showing pronounced inferior Q waves and marked inferolateral ST-T wave changes. The patient had a nuclear stress test which showed inferior wall ischemia. Subsequently, the patient underwent coronary angiography which showed an ARCAPA. The patient underwent surgical repair with reimplantation of the right coronary artery to the ascending aorta which was tolerated well. Our case illustrates ARCAPA presenting late in adulthood with ischemic symptoms that was treated with corrective surgery.

Misconception of 'malignant' and 'scissor-like compression' of interarterial course in anomalous aortic origin of a coronary artery: a case series.

Background: The notion that the 'interarterial' segment of anomalous aortic origin of a coronary artery (AAOCA) is 'malignant' and 'scissor-like' compressed between the aorta and pulmonary artery (PA) is debated, owing to the lower pressure in the pulmonary system compared with that in the coronary system. However, data supporting or refuting this belief under stress conditions are lacking. Case summary: Three cases of right AAOCA with interarterial/intramural courses (52, 66, and 51 years old) were assessed. Invasively measured fractional flow reserve (FFR) under dobutamine was 0.85, 0.82, and 0.81, respectively. Intravascular ultrasound illustrated lateral vessel compression of the intramural course with a decrease of minimal lumen area (MLA) (i.e. 5.71-3.47 mm2, 5.88-4.00 mm2, and 5.85-4.06 mm2) under stress conditions with heart rates of 130, 140, and 150 b.p.m., respectively. Pulmonary artery pressure (PAP) increased from rest {s/d (m) [systolic/diastolic (mean)] 22/11 (15), 15/2 (5), and 24/6 (14) mmHg} to stress [s/d (m) 47/24 (36), 30/3 (11), and 36/22 (24) mmHg] and remained below aortic peak pressure (blood pressure, BP) rest [s/d (m) 116/64 (91), 94/48 (71), 99/53 and (62) mmHg]; BP stress [s/d (m) 142/63 (80), 123/63 (88), and 86/46 (62) mmHg]; coronary pressure (CoP) rest [s/d (m) 100/59 (80), 80/45 (62), and 83/47 (63) mmHg]; and CoP stress [s/d (m) 95/60 (69),101/54 (72), and 70/32 (50) mmHg]. Conclusion: This case series challenges the assumption that the interarterial segment of AAOCA is scissor-like compressed by both the aorta and PA. The decrease in MLA and FFR under stress is due to the aorta's unidirectional lateral compression on the intramural segment. Additionally, the term 'malignant' should not be universally applied to all AAOCA cases with an interarterial course, as not all result in haemodynamic significance.

Coronary artery anomalies: what are they? when to suspect? how to treat?-a narrative review.

Background and Objective: In the literature have been widely discussed different classification criteria for coronary artery anomalies (CAAs), some authors have tried to categorize them only as "major" or "hemodynamically significant" anomalies versus "minor" or "not hemodynamically significant" ones. However, the most recent literature has concluded that all possible coronary anatomy should be taken into consideration in a comprehensive classification of CAAs. The aim of the article is to review the most recent literature regarding CAAs to provide a comprehensive overview of this challenging topic. Methods: We propose a narrative overview of the most impactful and recent literature, synthetizing and re-elaborating the most important articles concerning CAAs. Key Content and Findings: The important gap of knowledge on the specific characteristics of CAAs has led to a progressively increased interest of the current research in this field. Albeit their nature is still unclear, an increased awareness of their fatality is spreading among clinicians and the general population, mostly associated with their clinical relevance among young patients and athletes. On the other side, we do believe that clinical and hemodynamic repercussions are of crucial importance and should always be integrated to understand the true nature of this important pathology. Conclusions: In the field of pediatric cardiology, CAAs are one of the most fascinating and studied subject. We propose a state-of-the art review to provide a comprehensive and systematic description and subsequently an approach to the epidemiological, pathophysiological, and clinical aspects of the most important CAAs in the pediatric population.

Deep learning reconstruction for coronary CT angiography in patients with origin anomaly, stent or bypass graft.

PURPOSE: To develop and validate a deep learning (DL)-model for automatic reconstruction for coronary CT angiography (CCTA) in patients with origin anomaly, stent or bypass graft. MATERIAL AND METHODS: In this retrospective study, a DL model for automatic CCTA reconstruction was developed with training and validation sets from 6063 and 1962 patients. The algorithm was evaluated on an independent external test set of 812 patients (357 with origin anomaly or revascularization, 455 without). The image quality of DL reconstruction and manual reconstruction (using dedicated cardiac reconstruction software provided by CT vendors) was compared using a 5-point scale. The successful reconstruction rates and post-processing time for two methods were recorded. RESULTS: In the external test set, 812 patients (mean age, 64.0 ± 11.6, 100 with origin anomalies, 152 with stents, 105 with bypass grafts) were evaluated. The successful rates for automatic reconstruction were 100% (455/455), 97% (97/100), 100% (152/152), and 76.2% (80/105) in patients with native vessel, origin anomaly, stent, and bypass graft, respectively. The image quality scores were significantly higher for DL reconstruction than those for manual approach in all subgroups (4 vs. 3 for native vessel, 4 vs. 4 for origin anomaly, 4 vs. 3 for stent and 4 vs. 3 for bypass graft, all p < 0.001). The overall post-processing time was remarkably reduced for DL reconstruction compared to manual method (11 s vs. 465 s, p < 0.001). CONCLUSIONS: The developed DL model enabled accurate automatic CCTA reconstruction of bypass graft, stent and origin anomaly. It significantly reduced post-processing time and improved clinical workflow.

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA): Echocardiographic diagnosis in a critically ill newborn.

ARCAPA echocardiographic diagnosis. A network of small multiple coronary vessels, a systo-diastolic jet of flow into the pulmonary artery and a retrograde inverted «blue¼ flow into the RCA are the key signs. CT-scan confirms the diagnosis .

Right Coronary Artery-to-Coronary Sinus Fistula with Giant Tortuous Dilatation of the Right Coronary Artery.

Teaching points: A coronary artery fistula (CAF) is an uncommon anomaly characterized by a diverse clinical spectrum, ranging from asymptomatic cases to severe complications, including heart failure and myocardial infarction.

ALCAPA in Children with Complex Congenital Heart Disease: A Multicenter Study.

We sought to report characteristics and outcomes of children with complex anomalous left coronary artery from the pulmonary artery (ALCAPA) and to compare the outcomes to children with isolated ALCAPA. We performed a retrospective data analysis of children with ALCAPA who underwent cardiac surgery between 1/2009 and 3/2018 at 21 centers. Characteristics and outcomes of patients with complex ALCAPA are provided using descriptive statistics. Outcomes were compared between complex ALCAPA and isolated ALCAPA using Fisher's exact test. We reviewed 258 patients who underwent surgical repair of ALCAPA at 21 centers. We identified 10 patients (3.9%) with complex ALCAPA. Median age at initial cardiac surgery was 49 days (range: 4 days, 12.8 years). Cardiac lesions associated with ALCAPA were HLHS (n = 3); scimitar syndrome (n = 2); VSD with aortic coarctation (n = 2); VSD with right pulmonary artery discontinuity (1); DORV with mitral atresia (n = 1); and ToF (n = 1). ALCAPA was diagnosed prior to surgical intervention in 1 patient; during the initial cardiac surgery in 4 patients; in the early postoperative period via cardiac catheterization in 3 patients; and later in childhood after initial surgical repair in 2 patients. Following ALCAPA repair, patients with complex ALCAPA, as compared to patients with isolated ALCAPA, were more likely to be placed on ECMO (50% vs 12%, p = 0.002), receive CPR (30% vs 6%, p = 0.017), or suffer operative mortality (50% vs 3%, p < 0.001). Complex ALCAPA is uncommon. All but one with complex ALCAPA was not diagnosed preoperatively and postoperative morbidity and mortality were significantly greater in these complex patients compared to patients with isolated ALCAPA.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) in adult patients - a multimodality imaging approach.

Purpose: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital ano-maly of the origin of the coronary arteries. The prevalence of this anomaly in the adult patient population is low, and therefore there is virtually no original research on this topic. Reports are limited to case reports. Material and methods: We evaluated 16,264 computed tomography (CT) exams (cardiac and chest) performed in our heart imaging department between 2015 and 2022 on a dual-source 128-slice CT scanner (SOMATOM Definition Flash, Siemens Healthineers, Forchheim, Germany) and established a retrospective registry of adult patients (> 18 years old) with ALCAPA. The study included 7 cases. Next, we collected clinical and echocardiographic data, which could be assessed retrospectively. Results: We found 7 cases of ALCAPA in adult patients (0.043%). Three of them were female, and 4 were male. The age varied between 20 and 60 years. Echocardiographic findings, as well as the clinical course, varied widely. Conclusions: ALCAPA is an extremely rare anomaly, which nonetheless must be taken into clinical consideration. This lesion may be fatal during infancy. Data regarding adult patients is scarce. Multicentre registries are needed to establish a more detailed clinical profile of adults with this anomaly.

64-Slice ECG-gated computed tomographic angiography for assessment of coronary arteries in brachycephalic dogs with pulmonary stenosis.

BACKGROUND: Brachycephalic dogs with pulmonary stenosis are known to have a higher incidence of concurrent coronary artery abnormalities than non-brachycephalic breeds, which increases risk when performing balloon valvuloplasty. The use of ECG-gated CT angiography has been reported for the evaluation of coronary arteries in normal dogs and dogs with pulmonary stenosis. The purpose of this study was to report findings of coronary artery origination and morphology of main branches using ECG-gated CT angiography in brachycephalic dogs with pulmonary stenosis. METHODS: An ECG-gated CT angiographic protocol was used to image coronary artery anatomy in nine brachycephalic dogs with pulmonary stenosis. Images were assessed for quality as well as coronary artery morphology by one veterinary radiologist, one veterinary cardiologist and one veterinary radiology resident. RESULTS: All nine dogs had good to excellent image quality. Coronary artery anomalies were identified in three of nine dogs: one R2A anomaly, one L2A anomaly and one L2C anomaly. Two dogs were assessed to be poor balloon valvuloplasty candidates based on CT angiographic images. LIMITATION: Coronary artery morphology was not confirmed via postmortem examination in all patients. CONCLUSION: ECG-gated CT angiography is a minimally invasive imaging modality capable of diagnosing various coronary artery anomalies in brachycephalic dogs with pulmonary stenosis and aiding in the determination of patient candidacy for balloon valvuloplasty.

Congenital dual anterior interventricular artery.

Dual anterior interventricular artery is a rare type of CHD. We reported a fifteen-year-old girl who underwent CT angiography that demonstrated one anterior interventricular artery from aorta and another from pulmonary artery.

Anaesthetic management of a parturient with an unrepaired coronary arteriovenous fistula for caesarean section.

Coronary arteriovenous fistulas are an abnormal conduit between a coronary artery and another cardiovascular lumen, without an intervening capillary bed. The reported prevalence is 0.002-0.3%. Physiologic consequences such as congestive heart failure, coronary steal phenomenon and fistula aneurysm formation and rupture are possible. There are limited reports of symptomatic coronary arteriovenous fistulas in association with pregnancy. We describe a 19-year-old woman with symptomatic left circumflex artery to coronary sinus fistula, terminating into a large exophytic varix in the right atrium, presenting for an elective caesarean section at 37 weeks gestational age. Our anaesthetic management strategy aimed to optimise myocardial perfusion, maintain euvolemia, avoid right ventricular obstruction from exophytic varix and avoid sympathetic stimulation or sudden increases in pulmonary vascular resistance. A slowly titrated epidural was used as the primary anaesthetic. Our patient tolerated the procedure well and was discharged home on postoperative day two. Understanding of the potential physiologic consequence of coronary arteriovenous fistulas, and interaction with the physiologic changes of pregnancy and delivery, are essential for the management of these cases.

Transcatheter pulmonary valve replacement in surgically-created double-barrel right ventricular outflow tracts: A single center case-series.

This manuscript describes the feasibility and approach to the assessment and performance of transcatheter pulmonary valve replacement (tPVR) in patients with surgically-created "double-barrel" right ventricular outflow tracts (RVOT). Patients with tetralogy of Fallot may have coronary anomalies which prohibit the performance of traditional tetralogy of Fallot repair. In certain cases, this may necessitate the placement of a right ventricle to pulmonary artery conduit in addition to the native RVOT, which is left in situ, creating so-called "double-barrel" RVOTs. When these patients develop RVOT dysfunction later in life, they would typically be referred for reoperation due to concerns for risk of coronary compression associated with a transcatheter approach. However, whether a transcatheter approach with valve replacement in the native RVOT is feasible or safe is unknown. This was a retrospective review of patients with a surgically created "double-barrel" RVOTs who underwent cardiac catheterization for assessment of tPVR at Boston Children's Hospital. From July 2012 to July 2022, there were four patients with "double-barrel" RVOTs who underwent assessment for tPVR. The age at catheterization ranged between 22 and 39 years. In three out of four patients, coronary compression testing was negative. These three patients had successful tPVR in the native RVOT. At follow up, all three patients were free of greater than mild regurgitation by echocardiogram and had a maximum instantaneous gradient across the RVOT ranging between 20 and 33 mmHg. Performance of tPVR in patients with surgically created "double-barrel" RVOTs is feasible. The safety of this procedure depends crucially on coronary artery assessment at all stages.

Transcatheter Aortic Valve Implantation in a Patient With Anomalous Left Main Coronary Artery from Right Coronary Artery.

Transcatheter aortic valve implantation (TAVI) is now a well-established modality of treating severe symptomatic aortic valve stenosis in patients deemed at great surgical risk. In patients with coexisting anomalous coronary arteries, however, TAVI presents technical challenges, and coronary obstruction is a feared complication. In this study, we present the case of successful TAVI in a 78-year-old patient with anomalous left main coronary artery originating from the right coronary artery.

Anomalous Origin of Left Circumflex Artery from the Right Sinus of Valsalva in Cardiac Computed Tomography in a Group of 16,680 Patients-Radiologic and Clinical Characteristics.

BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) is the most prevalent form of coronary anomaly. One variant of AAOCA is the anomalous origin of the left circumflex artery from the right sinus of Valsalva, which can be detected using cardiac computed tomography (CT). However, limited data are available regarding the natural history of this anomaly, its impact on myocardial function, and associated symptoms. METHODS: We conducted a retrospective analysis of 16,680 CT exams (cardiac and chest) performed between 2015 and 2022 at our Heart Imaging Department, utilising a dual-source 128-slice CT scanner (SOMATOM Definition Flash, Siemens Healthineers, Forchheim, Germany). A registry of patients with anomalous origin of the circumflex artery from the right sinus of Valsalva (RCx) was established. The study included 56 cases of RCx (0.33%). Clinical information was obtained from medical records. RCx was defined as a circumflex artery originating from the right sinus of Valsalva (type I or II) or the right coronary artery (type III). Two researchers independently reevaluated each CT exam in our study group to ensure accurate radiologic descriptions and provide additional precise radiologic information regarding the anomaly, including high-risk features. RESULTS: Our study comprised 56 patients, with approximately equal distribution between males (n = 30, 54%) and females (n = 26, 46%), and with a median age of 59 years. Coronary heart disease (CAD) was known in 23% of patients (n = 13), while 11% (n = 6) were obese (defined as a BMI > 30 kg/m2), and 13% (n = 7) were diagnosed with type 2 diabetes. Only 9% of patients (n = 5) were smokers. Dyslipidemia was the most prevalent atherosclerotic risk factor, affecting approximately one third of patients (n = 17, 30%). In 14% (n = 8) of patients, heart failure was observed, while 13% (n = 7) were diagnosed with atrial fibrillation. Type I RCx was the most common subtype, identified in 48% of patients (n = 27) with this anomaly. Type II and Type III were found in 25% (n = 14) and 27% (n = 15) of patients, respectively. CONCLUSIONS: Our findings suggest that RCx is frequently encountered as an incidental finding, and we did not identify a consistent clinical characteristic in all patients with this type of anomaly. Furthermore, no gender predominance was associated with RCx. The natural history of this anomaly and its clinical implications seem benign. Further research is warranted to better understand this anomaly's natural course and clinical implications.

Life long surveillance is warranted as coronary artery remodels variably after treatment of adult patients with anomalous left coronary artery origin from pulmonary artery.

Anomalous left coronary artery origin from pulmonary artery causes heart failure in infancy from ischemia and secondary mitral regurgitation. Rich intramyocardial collateralization may permit survival to adult age, where coronaries become tortuous and aneurysmally dilated. Surgery in adults involves left coronary ligation and providing a bypass graft to the left system, unlike coronary translocation adopted in infants. Unfavorable coronary remodeling in operated adults may lead to late coronary thrombotic occlusions. Two adults with markedly dilated tortuous coronary arteries showed variable remodeling after corrective intervention that impacted outcomes on follow-up. We stress the need for lifelong angiographic surveillance in older patients.

Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review.

As a result of increasing adoption of imaging screening, the number of adult patients with a diagnosis of anomalous aortic origin of the coronary arteries (AAOCA) has grown in recent years. Existing guidelines provide a framework for management and treatment, but patients with AAOCA present with a wide range of anomalies and symptoms that make general recommendations of limited applicability. In particular, a large spectrum of interventions can be used for treatment, and there is no consensus on the optimal approach to be used. In this paper, a multidisciplinary group of clinical and interventional cardiologists and cardiac surgeons performed a systematic review and critical evaluation of the available evidence on the interventional treatment of AAOCA in adult patients. Using a structured Delphi process, the group agreed on expert recommendations that are intended to complement existing clinical practice guidelines.

Acute coronary syndrome in an anomalous mid-LAD right coronary artery: Don't forget to look twice before turning left.

A 68-year-old female with past medical history of hypertension, hyperlipidemia, multiple sclerosis, diverticulitis, and tobacco use presented with 1 day of atypical chest pain after a recent diverticulitis flare. Initial workup was notable for a normal electrocardiogram but elevated high sensitivity troponin T (616 ng/L). Due to persistent symptoms, the patient was given antiplatelet therapy and taken urgently to the catheterization lab where she was found to have complete occlusion of an anomalous right coronary artery branching off the mid-left anterior descending artery. Angioplasty was performed with a drug-eluting stent and her symptoms resolved. The patient recovered well and was discharged on appropriate medical therapy. This case demonstrates a case of acute coronary syndrome in an extremely rare coronary congenital abnormality. Further research is needed on when to be suspicious for coronary anomalies on patients presenting with myocardial infarction.