Interposition of right internal iliac artery free-graft for left coronary artery reimplantation in adults with anomalous left coronary artery originating from the pulmonary artery: case series and long-term results.

Anomalous left coronary artery originating from the pulmonary artery (ALCAPA) is an infrequent congenital anomaly. Presentation of this syndrome is rare in adults. Nevertheless, adult patients are at risk of ischaemia, arrhythmias or sudden cardiac death and always require surgical intervention. At our institution, a specific technique of interposition of the right internal iliac artery as a free-graft for left coronary artery reimplantation was used in adult ALCAPA patients. The aim of this report is to determine long-term results and experiences with this surgical technique.

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.

Recurrent strokes in the setting of anomalous left coronary artery from the pulmonary artery.

A young woman presented with a syncopal episode. As part of the work-up for her presentation, an MRI of the brain was performed which showed a small acute stroke. She subsequently had a further embolic-appearing stroke 9 months later. The initial investigations for the work-up of her strokes did not reveal any major abnormalities, including an unremarkable ECG and transthoracic echocardiogram (TTE). A transoesophageal echocardiogram (TOE) showed aneurysmal apical and mid-anteroseptal akinesis with mildly reduced left ventricular ejection fraction. Anomalous left coronary artery from the pulmonary artery (ALCAPA) was confirmed on coronary angiogram. Although ALCAPA presenting in adulthood is rare, our case highlights the value of TOE over TTE in the work-up of cryptogenic strokes, particularly in young patients with embolic strokes of undetermined source.

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.

Complex Repair of Anomalous Left Coronary Artery From the Pulmonary Artery in a 55-Year-Old Patient.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a congenital malformation that classically presents within the first year of life. Few patients survive into adulthood, and initial presentation after the fourth decade of life is rare. We describe a 55-year-old woman who presented after cardiac arrest. She initially refused surgery and underwent automated implantable cardioverter defibrillator placement, followed later by surgical repair involving reimplantation of the left coronary artery to the aorta and pulmonary artery reconstruction using interposition grafts. We report this late presentation of ALCAPA and successful surgical management.

Outcome of surgical repair of anomalous left coronary artery from the pulmonary artery in a single-center experience.

BACKGROUND: This study aims to review the midterm results of surgical repair of anomalous left coronary artery from the pulmonary artery (ALCAPA) in our center and assess the postoperative cardiac function recovery and misdiagnosis. METHOD: Patients who underwent repair of ALCAPA between January 2005 and January 2022 at our hospital were retrospectively reviewed. RESULTS: A total of 136 patients underwent repair of ALCAPA, among which 49.3% were misdiagnosed before referral to our hospital. On multivariable logistic regression analysis, patients with low left ventricular ejection fraction (LVEF) (odds ratio = 0.975, p = 0.018) were at increased risk for misdiagnosis. The median age at surgery was 0.83 years (range, 0.08 to 56 years), and the median LVEF was 52% (range, 5% to 86%). The mortality rate was 6.6% (n = 9), and four patients underwent reintervention. The median postoperative recovery time of left ventricular function (LVF) was 10 days (1 to 692 days). Competing risk analysis revealed that a low preoperative LVEF (hazard ratio = 1.067, p < 0.001) and age younger than 1 year (hazard ratio = 0.522, p = 0.007) were risk factors for a longer postoperative recovery time of LVF. During the follow-up period, 91.9% (113/123) of the patients had no aggravation of mitral regurgitation. CONCLUSION: The perioperative and intermediate outcomes after ALCAPA repair were favorable, but preoperative misdiagnosis deserved attention, especially in patients with low LVEF. LVF normalized in most patients, but patients younger than 1 year and with low LVEF required longer recovery times.

Surgical Reconstruction of the Anomalous Left Coronary Artery From the Pulmonary Artery.

The anomalous left coronary artery from the pulmonary artery is a rare congenital disease. Early surgical reconstruction is mandatory to prevent adverse cardiac events. Direct coronary button transfer, vein graft interposition, ligation, and coronary artery bypass construction are the most commonly used techniques. This case report presents a modified technique of Dacron graft interposition and reimplantation anomalous left coronary artery from the pulmonary artery on the ascending aorta.

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.

Mitral intervention for anomalous left coronary artery from the pulmonary artery: midterm outcomes.

OBJECTIVES: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is frequently associated with significant mitral regurgitation (MR). We aim to identify surgical outcomes in patients with or without concomitant mitral intervention. METHODS: All patients with ALCAPA who presented with >mild degree of MR at our institution between January 2008 and June 2020 were included in the retrospective study. MR recovery was defined as ≤mild MR at the last follow-up. RESULTS: The study cohort included 101 patients. The median age at repair was 7.6 months. The concomitant mitral intervention was performed in 66 patients (65%). MR grade significantly improved at the last follow-up. The cumulative incidence of MR recovery 3 years after ALCAPA repair was 34% [95% confidence interval (CI), 19-50%) in patients with mitral intervention, compared to 59% (95% CI, 41-73%) in patients without mitral intervention (P = 0.050). MR grade on postoperative day 1 was the predictor for MR recovery in patients with mitral intervention (hazard ratio, 0.080; 95% CI, 0.018-0.366; P = 0.001), whereas preoperative mitral annulus diameter z-score was the predictor in patients without mitral intervention (hazard ratio, 0.480; 95% CI, 0.232-0.993; P = 0.048). Freedom from mitral reoperation in patients with mitral intervention was 94% and 88% at 3 and 5 years after surgery, while freedom from mitral reoperation in patients without mitral intervention was 100% at both timepoints (P = 0.177). CONCLUSIONS: Despite significant MR improvement after ALCAPA repair, MR grade may not always return to normal regardless of the initial mitral management strategy, and reoperation for persistent MR is not rare.

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.

Ischemic cardiomyopathy due to late diagnosis of alcapa syndrome

INTRODUCTION: ALCAPA Syndrome (Anomalous Left Coronary from the Pulmonary Artery) is the anomalous origin of the left coronary artery from the pulmonary artery. Also known as BlandWhite-Garland Syndrome, it is a rare congenital heart defect, present in 0.25-0.5% of patients with congenital heart defects. It can occur in isolation or be associated with other birth defects. Usually the diagnosis is early, made in the prenatal period and the need for its correction is immediate. CASE REPORT: A 63-year-old female patient sought emergency care with chest pain, dyspnea on moderate exertion, and palpitations. Myocardial scintigraphy was positive for ischemia, corresponding to the territory of the anterior descending artery. Cardiac catheterization was requested, in which an anomalous left coronary artery was observed in the pulmonary artery trunk. The transthoracic echocardiogram showed the following findings: akinesia of the apical and anteroapical septum, with hypokinesia of the anterolateral wall and of the mid-basal segments of the anterior wall, moderate left ventricular systolic dysfunction (ejection fraction of 37 %), pulmonary artery systolic pressure of 62 mmHg. It has magnetic resonance imaging of the heart, which shows the areas of infarction mentioned in the echocardiogram, without myocardial viability. DISCUSSION: ALCAPA syndrome is difficult to diagnose, and should be suspected in children with dilated cardiomyopathy. About 85% of cases manifest up to 2 months of age, but symptoms can be misinterpreted and the disease may be underdiagnosed. The disease requires surgical treatment to restore the flow of oxygenated blood to the myocardium. The procedure of choice consists of reimplanting the coronary artery in the aorta and it is estimated that the surgical mortality is less than 5-10%, with a good prognosis after reimplantation. The peculiarity of the case reported is due to the fact that the diagnosis was extremely late and with important irreversible hemodynamic REPERCUSSIONS: the patient has an ischemic cardiomyopathy without myocardial viability, a condition usually related to chronic coronary artery disease or acute coronary syndromes, rarely related to ALCAPA, since due to the severity of the syndrome, its diagnosis is early. Currently, due to the lack of myocardial viability, surgical correction would not bring benefits or recovery of heart muscle, opting for clinical treatment for cardiac remodeling.

Surgical Management of Acute Myocardial Infarction Caused by Intramural Anomalous Left Coronary Artery in a Young Female Athlete.

In the complex spectrum of coronary anomalies, the origin of the left coronary artery from the right sinus of Valsalva with intramural course could represent a catastrophic life-threatening condition leading to extensive myocardial infarction and sudden cardiac death, especially in young athletes. We report the case of a young female athlete with anomalous left coronary artery from the opposite sinus who survived a major non-ST-elevation myocardial infarction during the eighth kilometer of a running race. It was successfully treated by creating a neo-ostium of the left coronary artery in the left sinus at the point at which the artery left the aortic wall.

Pulmonary artery banding as adjunct therapy for ventricular recovery after anomalous left coronary artery from the pulmonary artery repair: a case report.

Anomalous left coronary artery from the pulmonary artery is a rare congenital coronary anomaly commonly associated with severe but reversible left ventricular dysfunction. We present an anomalous left coronary artery from the pulmonary artery case of persisting left ventricular failure with inability to wean off the ventilator and inotropes after successful coronary reimplantation, in whom pulmonary artery banding enhanced myocardial recovery.

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.

Repair of a remote pericommissural anomalous left coronary artery from the pulmonary artery.

We demonstrate a classic trapdoor technique to correct a late-presenting remote pericommissural anomalous left coronary artery from the pulmonary artery. The merits of the technique compared to alternative techniques are explained. The impact of late diagnosis on left ventricular function as well as on papillary muscle ischemia-induced mitral regurgitation is evident. The child's recovery without the need for a temporary ventricular assist device supports the efficacy of  the technique, despite the residual mitral regurgitation.

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).

Heart Failure as the Initial Presentation of Anomalous Left Coronary Artery From the Pulmonary Artery.

Coronary arteries arising from the pulmonary artery have an incidence of 0.002% in the general population. We present a 29-year-old woman who presented to our hospital with acute decompensated heart failure and atrial fibrillation with a rapid ventricular rate. She underwent a cardiac catheterization to rule out ischemic disease, which revealed retrograde contrast flow through the left coronary artery from the right coronary artery. A coronary computed tomography (CT) angiogram was pursued which showed the presence of an anomalous left coronary artery arising from the pulmonary artery (ALCAPA). For the management of her atrial fibrillation, she was electrically cardioverted. She was discharged on guideline-directed medical therapy for her heart failure, with a cardiac surgery referral for the surgical fixation of her 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.