Intramural course of an anomalous left coronary artery is not always associated with a slit-like ostium.

Anomalous aortic origin of the left coronary artery from an incorrect aortic sinus has been reported as the second most common causes of sudden cardiac arrest in young athletes. Intramural course of the proximal left coronary artery is considered a high-risk morphology. It is associated with a slit-like ostium and elliptical shape of the proximal artery. In this case, all pre-operative cardiac images demonstrated a round ostium and round luminal shape of the left coronary artery, which suggested no evidence of intramural course. On intraoperative inspection, although the ostium and proximal left coronary artery appeared round, the patient had a long intramural course of the left coronary artery which our surgeons performed a successful unroofing procedure. The post-operative images showed a patent new ostium from the correct aortic sinus. These findings are very unusual and proved that we should not exclude intramural course even in the absence of a slit-like ostium and elliptical shape of the proximal left coronary 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.

A Rare Complication of Anomalous Left Coronary Artery From the Pulmonary Artery Identified by Point-of-Care Ultrasound.

ABSTRACT: Point-of-care ultrasound is a valuable tool in the evaluation of undifferentiated respiratory distress in children. This case report describes a 9-month-old male infant who presented with 4 months of progressively worsening cough and was found to be tachypneic in the emergency department. Chest radiography revealed complete opacification of the left hemithorax with cardiomegaly. Point-of-care ultrasound demonstrated atelectasis of the entire left lung and severe dilation of the left ventricle with poor function. Electrocardiogram and echocardiogram findings were consistent with a diagnosis of anomalous left coronary artery from the pulmonary artery. The patient underwent successful cardiac surgery to improve myocardial perfusion. Bronchoscopy visualized the etiology of his atelectasis, pulsatile compression of the left mainstem bronchus by the dilated heart. Focused cardiac and lung ultrasound techniques, as well as pertinent sonographic findings, are reviewed.

Clinical manifestations and computed tomography angiography features of anomalous left coronary artery from the pulmonary artery syndrome. / å·¦å† çŠ¶åŠ¨è„‰å¼‚å¸¸èµ·æºäºŽè‚ºåŠ¨è„‰ç»¼åˆå¾ï¼šä¸´åºŠè¡¨çŽ°åŠCTA影像学特征.

OBJECTIVES: Anomalous left coronary artery from the pulmonary artery (ALCAPA) syndrome is a rare congenital coronary artery malformation with a high rate of clinical missed diagnosis and misdiagnosis. At present, there is a lack of reports on the clinical manifestations of ALCAPA and the imaging features of coronary computed tomography angiography (CTA). This study aims to summarize the clinical characteristics and coronary CTA imaging features of ALCAPA to improve the clinical diagnosis and treatment. METHODS: The clinical data of 24 ALCAPA syndrome patients were retrospectively collected, analyzed and summarized from January 1, 2013 to February 1, 2023 in the Second Xiangya Hospital of Central South University. According to the left and right coronary collateral circulation, the patients were classified into infantile type (7 cases) and adult type (17 cases). The differences of clinical manifestations and CTA imaging features were compared between the 2 types of patients. RESULTS: Of the 24 patients, a male-to-female ratio was at 1꞉5, and the median age of onset was 3.22 months for the infant type and 22.0 years for the adult type. The infantile type showed symptoms of left heart insufficiency at an early stage, while the adult type had a variety of clinical manifestations in 7 patients being asymptomatic and presented with a cardiac murmur on physical examination, 6 with symptoms of chronic myocardial ischemia, and 2 with malignant arrhythmias. CTA showed that 11 patients' the left coronary artery originated from the left posterior sinus of the pulmonary artery. All 7 infantile type patients had an enlarged left heart, left ventricular hypertrophy, reduced left heart function, lack of collateral circulation between the left and right coronary arteries, and normal coronary artery diameter. All 17 adult type patients showed dilated and tortuous coronary arteries with rich collateral circulation, and 7 adult type patients had preserved left heart function. CONCLUSIONS: The clinical manifestations and CTA imaging features of patients with the 2 types of ALCAPA are different, while CTA performance is characteristic and can be used as a means of definitive diagnosis, staging, surgical evaluation, and postoperative follow-up of ALCAPA syndrome.

A 'Masked' Anomalous Left Coronary Artery From Pulmonary Artery: Utility of Endoluminal Views.

We present the cardiac CT images of 5-month infant with aorto-pulmonary window along with anomalous left coronary artery from pulmonary artery along with endoluminal views which help the surgeons in pre-planning of baffle orientation.

Congenital Aortic-Left Atrial Tunnel with Coarctation and Anomalous Left Coronary Artery from the Pulmonary Artery: A First-of-its-kind Case Report.

Aortic-left atrial (Ao-LA) tunnel is an extremely rare vascular anomaly that involves an abnormal channel originating from the sinuses of the Valsalva and terminating in the left atrium. We present an unusual case of prenatally diagnosed Ao-LA tunnel with postnatal diagnosis of coarctation of the aorta and anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA).

Unusual intramural course in an anomalous left coronary artery from the opposite coronary sinus.

We report a case of a patient with an anomalous left coronary artery originating from the opposite coronary sinus with evidence of intramural course at the level of the septal commissure and no slit-like deformation of the coronary ostium, acute take-off, or proximal vessel narrowing. According to previous publications, patients with anomalous coronary artery and intramural segments identified at surgery had coronary CT findings of acute take-off angle or proximal vessel narrowing; slit-like orifice; and elliptical cross-sectional shape. Although further investigation is required, we suggest that the intramural course may not be ruled out based on the absence of slit-like ostium, acute take-off, or proximal vessel narrowing.

Anomalous left coronary artery from pulmonary artery (ALCAPA) as a silent cause of mitral regurgitation in children.

Anomalous left coronary artery from pulmonary artery (ALCAPA), also known as Bland-White-Garland syndrome, is a rare cardiac disease. This condition may present with complications such as myocardial infarction, left ventricular dilatation, mitral regurgitation, and left heart failure in children. We report a case of a four-year-old boy who presented with shortness of breath, palpitations, and recurrent upper respiratory tract infections. He was diagnosed with mitral regurgitation. During the surgery, left coronary artery (LCA) was not present in its anatomical position and ALCAPA was identified. One should keep in mind the possibility of ALCAPA in presentation of mitral regurgitation in children despite not being reported in echocardiography.

Anomalous left coronary artery from pulmonary artery repair: Outcomes from the European Congenital Heart Surgeons Association Database.

INTRODUCTION: We sought to determine the surgical outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) enrolled in the European Congenital Heart Surgeons Association (ECHSA) database. MATERIALS AND METHODS: From 1999 to 2019, 907 patients with ALCAPA underwent surgical repair and were included in the current study. The primary outcome was in-hospital mortality. Secondary outcomes included frequency and results of concomitant mitral valve surgery and postoperative mechanical circulatory support (MCS). RESULTS: The overall in-hospital mortality was 6% (54/907) and was significantly higher in neonates (p = .01), patients with lower body surface area (BSA) (p = .01), and those requiring postoperative MCS (p = .001). Associated mitral valve surgery was performed in 144 patients (15.9%) and was associated with longer cardiopulmonary bypass (CPB) and aortic cross-clamp times (AOX) (p ≤ .0001) but was not significantly related to an increase in in-hospital mortality. Postoperative MCS was required in 66 patients (7.3%). These patients were younger (p ≤ .001), had a lower BSA (p ≤ .001), and required a longer CPB (p ≤ .001) and AOX time (p ≤ .001). CONCLUSIONS: ALCAPA repair can be achieved successfully, and with low surgical risk. Concomitant mitral valve procedures can be performed without increasing operative mortality. The use of MCS remains a valuable option, especially in younger patients.

Impact of Reverse Left Ventricular Remodeling on Outcomes of Patients with Anomalous Left Coronary Artery from the Pulmonary Artery after Surgical Correction.

The impact of reverse left ventricular remodeling (r-LVR) on clinical outcomes after surgical correction of anomalous left coronary artery from the pulmonary artery (ALCAPA) remains unclear. This study aims to examine the prognostic significance of r-LVR in patients with ALCAPA after surgery. We prospectively identified 61 patients undergoing surgical correction for ALCAPA; 54 patients had adequate echocardiographic image quality with quantitative biplane analysis performed both at baseline and at 30-day postoperative follow-up. Postoperative r-LVR was defined as a reduction of ≥ 10% in left ventricular end-diastolic volume index during follow-up. Cox proportional-hazards regression was used to investigate the independent association of r-LVR and all-cause mortality. Among 54 patients (age: 21.2 ± 7 months; 37% females), r-LVR occurred in 35 patients (64.8%) after surgery. Compared to patients with r-LVR, patients without r-LVR had significantly higher level of N-terminal pro B-type natriuretic peptide (NT-proBNP) [2176 (711, 4219) vs 998 (623, 2145) P < 0.001] and lower survival rate (47.3% vs 82.9%, HR = 5.72 [1.96 to 17.20], P < 0.001) at 1-year follow-up. NT-proBNP (OR = 2.27 [1.67 to 18.3], P = 0.02) was an independent predictor of r-LVR in multivariate analysis. Moreover, r-LVR was significantly associated with a lower rate of all-cause mortality (HR = 0.27 [0.08 to 0.98], P = 0.03) in multivariate analysis, even after adjustment for clinical and echocardiographic variables. R-LVR occurred in more than half of patients with ALCAPA undergoing surgical correction and it was associated with better clinical outcomes. NT-proBNP is an independent predictor of r-LVR.

Anomalous left coronary artery from the pulmonary artery repair outcomes: Preoperative mitral regurgitation persists in the follow-up.

OBJECTIVE: This study aims to present the midterm outcomes of surgical correction of the anomalous left coronary artery from the pulmonary artery (ALCAPA) with a focus on mitral regurgitation (MR). METHODS: Medical records of 36 ALCAPA patients who underwent surgery in a single center were retrospectively reviewed. RESULTS: There were one (2.7%) neonate, 19 (52.7%) infants, 15 (41.6%) children, and 1 (2.7%) adult patient operated. Coronary reimplantation, tube reconstruction, and coronary artery bypass surgery techniques were performed in 29 (80.5%), 6 (16.6%), and 1 (2.7%) patient, respectively. Sixteen (44.4%) patients had a low ejection fraction and 13 (36.1%) patients had at least moderate MR preoperatively. None of our patients underwent a concomitant mitral surgery during the initial repair. Three and two patients died in the early and late postoperative period, respectively. Two patients underwent reoperation due to MR and pulmonary stenosis, separately. Preoperative MR was the only factor associated with at least moderate MR at the final follow-up (p < .01) and the presence of preoperative moderate or over-moderate MR estimated at least moderate MR at the final follow-up with 100% sensitivity and 80% specificity. CONCLUSION: Although the mitral valve was not repaired in the first operation, the reoperation rate is low. However, a moderate or higher preoperative MR predicted MR at the last follow-up. Performing annuloplasty in such patients can be a strategy to be researched.

Vaping Associated Cardiac Arrest at School in a Teenager with Anomalous Left Coronary Artery.

The prevalence of electronic cigarette use is increasing at an astonishing pace, particularly in the teenage population. While at school, a healthy 13-year-old male experienced a sudden cardiac arrest after vaping multiple times throughout the day. Workup revealed an anomalous left coronary artery originating from the right sinus of Valsalva. Given this patient's underlying anomalous left coronary artery, we suspect that sympathoexcitatory and arrhythmogenic effects of high dose nicotine from vaping led to his cardiovascular collapse. This is the first published case report of a vaping associated cardiac arrest in a patient of this age.

Implantation of a Coronary Sinus Reducer to Treat Refractory Angina in a 38-Year-Old with an Anomalous Left Coronary Artery and No Revascularization Options.

A 38-year-old female with anomalous left coronary artery from the pulmonary artery presented with refractory angina (Canadian Cardiovascular Society [CCS] class 4). Having failed two previous internal mammary artery grafts to the left anterior descending artery and with no percutaneous revascularization options, she underwent coronary sinus reducer implantation, which improved her symptoms (CCS 0), quality of life, and corresponded to an improvement in ischemia on myocardial perfusion scanning. This case report describes an unusual case of refractory angina in the context of congenital heart disease, illustrates the benefit of this novel hourglass-shaped stent in improving ischemia, quality of life, depression and anxiety, and highlights the importance of managing these patients in multidisciplinary teams.

Effective myocardial perfusion and concomitant haemodynamic status determine the clinical diversity of anomalous left coronary artery from the pulmonary artery.

BACKGROUND: Anomalous left coronary artery from the pulmonary artery is a rare congenital heart disease (CHD) with diverse clinical presentations despite the same anatomy. Factors determining this heterogeneous presentation are not well understood. METHOD AND RESULTS: We retrospectively investigated 14 patients (12 females) who underwent surgical repair of anomalous left coronary artery from the pulmonary artery. These patients were divided into three groups based upon the severity of initial presentation: (1) severe, life-threatening condition (n = 5), (2) mild-to-moderate distress (n = 6), and (3) asymptomatic (n = 3). All patients presented with left ventricular dilation and retrograde flow in left coronary artery by echocardiogram. Eight patients in (1) and (2) presented with severe left ventricular dysfunction. All but one showed abnormal ECG consistent with myocardial ischemia or infarction. Asymptomatic patients had preserved left ventricular systolic function despite ischemic findings on ECG. In 13 patients after surgical repair, all but one normalised left ventricular geometry and systolic function, suggesting nearly full myocardial recovery upon improvement of myocardial perfusion; 8 patients had residual echogenic papillary muscle with variable degree of mitral regurgitation. CONCLUSIONS: Evidence of myocardial ischemic injury was present in all patients with anomalous left coronary artery from the pulmonary artery regardless of their initial presentation. Retrograde flow in left coronary artery, implying collateral vessel development from right coronary artery to left coronary artery, was noted in all patients, yet only few patients had preserved systolic function at the time of diagnosis. The balance between effective myocardial perfusion and a deleterious fistulous flow provided by these collaterals and the simultaneous haemodynamic status are what determine the clinical diversity of anomalous left coronary artery from the pulmonary artery.

Successful surgical correction of tetralogy of fallot associated with anomalous left coronary artery connection to pulmonary artery.

Tetralogy of fallot is rarely associated with anomalous left coronary artery connection to main pulmonary artery. High index of suspicion is needed preoperatively to diagnose this association and treat successfully. We present a case of 9-year-old boy with the rare association of tetralogy of fallot and anomalous left coronary artery connection to pulmonary artery with a giant steal intercoronary collateral crossing right ventricular outflow tract who was treated successfully by single-stage surgical correction.

Prevalence and morphologic features of dual left anterior descending artery subtypes in coronary CT angiography.

OBJECTIVES: The purpose of this study was to identify the prevalence of dual left anterior descending artery and define the morphological features using coronary computed tomography angiography in order to raise awareness of dual left anterior descending artery among radiologists. MATERIALS AND METHODS: The radiologic reports of 1912 patients who underwent coronary computed tomography angiography at our institution were searched retrospectively for the presence of dual left anterior descending artery. Computed tomography images of patients with dual left anterior descending artery were then reviewed, and the morphological features of dual left anterior descending artery were evaluated. RESULTS: Dual left anterior descending artery was identified in 1.3% of the patients in this study population. Type 1 dual left anterior descending artery was the most common subtype (76%). Four additional cases that were not classified before were also detected. CONCLUSION: Dual left anterior descending artery has a variety of subtypes reported mostly as odd cases, and gaining diagnostic awareness of dual left anterior descending artery is more critical, rather than listing and counting the subtypes. With the widespread use of coronary computed tomography angiography, it has become essential for radiologists to have knowledge about dual left anterior descending artery, as it is vital, especially for surgical planning.

Anomalous left anterior descending artery diagnosed on pulmonary artery computed tomography.

Typically, the left anterior descending artery (LAD) and left circumflex artery (LCX) arise from the left main coronary artery. However, uncommon coronary anomalies may be found in clinical practice. This case presents with a rare finding where the LAD originates from the right coronary artery (RCA) separately from the LCX and takes an interarterial pathway to reach its perfusion territory. A 49-year-old Hispanic female with hypertension and diabetes mellitus presented to the emergency department with a 7-day history of chest pain. She denied nausea, diaphoresis, syncope, or other symptoms. A grade 3 out of 6 systolic murmur was noted on physical examination. Computed tomography of the pulmonary arteries (CTPA) revealed that the patient had no left main coronary artery. The patient's LAD arose from the proximal RCA and took an inter-arterial course. Subsequent coronary catheterization showed no stenosis of the coronary arterial system. The patient's chest pain subsided during the course of her admission and she was deemed stable for discharge with close cardiology follow up. In general, coronary artery anomalies are an uncommon finding in clinical practice. However, it is important to realize the different pathways of coronary artery anomalies because those with the inter-arterial subtype, such as our patient, may result in sudden cardiac death. All cases of clinically suspected inter-arterial coronary artery anomalies are recommended to undergo imaging studies to help visualize anatomic features as a guide for further management. This case represents the first reported diagnosis of this type of anomalous coronary artery on CTPA.

Oblique coronary artery prolongation in anomalous left coronary artery arising from the pulmonary artery: A case series.

BACKGROUND: Anomalous left coronary artery (LMCA) arising from the pulmonary artery (ALCAPA) is a life-threatening congenital birth defect. Prompt surgical correction by reimplantation of the LMCA into the aorta is the treatment of choice, however, unfavorable LCA location or length can make direct reimplantation difficult or even impossible without causing significant stretching or tension on the LMCA. This tension can lead to stenosis of the artery and coronary ischemia and must be avoided if possible. METHODS: A chart review from January 2010 to December 2018 of patients who had ALCAPA repair. Patients were excluded if direct reimplantation was possible. Herein, we describe four cases in which remote LMCA position necessitated the creation of a tubular prolongation of the LMCA. RESULTS: A generous coronary button from the pulmonary artery along with the oblique flap of aorta served as the prolongation of the left main coronary artery. This allowed for a tension-free anastomosis using native tissue with the ability to grow with the patient. The pulmonary artery and the aorta were then patched using pericardial tissue. All four patients had improvement in left ventricular function and degree of mitral regurgitation within weeks of surgery. No patient required extracorporeal membrane oxygenation support. Subsequent long-term angiographic follow-up has revealed excellent durability and patency of these coronary conduits. CONCLUSIONS: The variable nature of ALCAPA requires modification of the surgical approach on a patient by patient basis, and the above described technique adds an alternate and reliable long-term treatment option.