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.

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.

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 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.

Anomalous right coronary artery from the main pulmonary artery (ARCAPA): Incidental finding in an asymptomatic septuagenarian.

Anomalous right coronary artery (RCA) from the main pulmonary artery (ARCAPA) is a rare finding. Clinical presentations range from asymptomatic to sudden death. We present the case of ARCAPA in a septuagenarian initially suspected on a screening chest computed tomography (CT) and later confirmed on cardiac CT. A summary of important points related to this entity is also discussed.

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.

A Rare Case of Single Coronary Artery: Getting to the Heart of the Matter.

Single coronary artery anomaly represents a particularly rare entity, which may present with variable clinical scenarios, but in most cases remains asymptomatic. It is considered to be one of the pathological states to cause sudden death, especially in young adults [1]. We hereby report a rare case of a R-III type of single coronary artery as classified by Lipton et al., which is only about 15% of all the cases of coronary anomalies. Coronary CT angiography as well as invasive coronarography provide accurate details regarding the origin, course and termination of coronary anomalies, as well as evaluation of associated coronary lesions, further guiding the optimal treatment strategy in each case. Teaching Point: The main teaching point of this case report is to clearly underline the importance of coronary CT angiography in obtaining a comprehensive evaluation of coronary artery anatomy and associated coronary lesions, representing important aspects, which further guide accurate treatment and management.

Anomalous origin of the left circumflex artery from the pulmonary artery associated with non-compaction of the left ventricle: usefulness of multimodality imaging-a case report.

Background: The anomalous origin of the left circumflex artery from the pulmonary artery (ACXAPA) is a very rare coronary anomaly. Only a few cases have been reported until today, from incidental findings to autopsy reports after sudden cardiac death. Case summary: We report here for the first time the case of a man, previously monitored for asymptomatic left ventricular non-compaction cardiomyopathy, who presented with non-ST myocardial infarction and was diagnosed with ACXAPA. Complementary tests confirmed ischaemia in the corresponding territory, and the patient was referred to surgery for reimplantation of the circumflex artery. Discussion: Left ventricular non-compaction cardiomyopathy is a rare congenital cardiomyopathy whose association with coronary anomalies, not with ACXAPA, had previously been described until now. A related embryological origin could potentially explain this association. The management of a coronary anomaly should indicate dedicated multimodality cardiac imaging in order to not disregard the association with underlying cardiomyopathy.

Origin of all Three Coronaries from Single Ostium: A Rare Case Report.

Origin of coronary arteries from the opposite sinus of Valsalva is rare and origin from a single ostium is extremely rare. Patients with these anomalies may have myocardial ischemia because of altered ostial configuration, exit angulation from the aorta, the course between great arteries, and atherosclerosis. Usually, these anomalies are diagnosed during coronary angiography either by computed tomography or catheter-based. Management depends upon the coronary anomaly and associated clinical condition.

Bridge of the left anterior descending artery revealed by syncope: a case report.

The myocardial bridge is a congenital coronary anomaly defined as the presence of a region of myocardium overlying an epicardial coronary artery. This is a 51-year-old patient, diabetic for 4 years on oral hypoglycemic, has had stress angina for 4 years, neglected by the patient. The current history goes back to 2 months by the installation of an episode of syncope occurring with the effort, then of a second episode the day of its admission. Electrocardiogram on admission showed complete atrioventricular block with an heart rate of 32 beats per minute, the patient spontaneously recovered sinus rhythm with a heart rate of 88 beats per minute and a PR interval of 200 ms, coronary angiography was performed showing coronary arteries without stenosis with an intramyocardial bridge of the left anterior descending artery. During exercise and in the presence of a myocardial bridge on the left anterior descending artery, systolic compression leads to a decrease in flow to the septal branches, which is responsible for an alteration of the vascularization of the sub-nodal tissue with paroxysmal conduction disorders leading to syncope. Conduction disorders of ischemic origin are not always associated with atherosclerotic or thromboembolic lesions, but may also be secondary to myocardial bridges.

Abnormal Origin of the Left Coronary Artery: When the Bloodstream Finds its Way.

Teaching Point: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) is a rare congenital anomaly characterized by a steal phenomenon; it must be recognized, as a prompt treatment may prevent life-threatening complications such as myocardial infarction, mitral dysfunction, and malignant dysrhythmias in adults.