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.

A Rare Case of an Adult Pregnant Patient with the Left Coronary Artery Originating from the Pulmonary Artery: Successful Management and Healthy Maternal-Fetal Outcome.

An anomalous left coronary artery originating from the pulmonary artery (ALCAPA) refers to the abnormal origin of the left coronary artery either from the main pulmonary artery, pulmonary artery sinus, or the left and right pulmonary arteries, with the main pulmonary artery or pulmonary artery sinus being the most common sites. If not diagnosed and treated promptly, this condition can result in death within the first year of life in 90% of patients. Asymptomatic children can survive into adulthood, but they are at a high risk of sudden death. In this article, we report a case of a 24-year-old pregnant woman who was diagnosed with ALCAPA during prenatal examination. The pregnancy was successfully maintained until 36 weeks, after which a cesarean section was performed. The patient was then admitted to the cardiac surgery department to improve cardiac function, and six weeks later, a successful left coronary artery transplantation was performed. The patient was discharged and followed up for three months, during which her condition remained stable.

Anomalous arising of left coronary artery from the pulmonary artery (ALCAPA).

Computed tomography 3D reconstruction of a patient with Bland-White-Garland syndrome showing a dilated right coronary artery and thin left coronary artery.

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.

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.

Redo-ALCAPA Repair With Left Subclavian Artery-to-Left Main Coronary Bypass for Left Coronary Atresia.

Different surgical techniques have been described for the primary repair of anomalous left coronary artery arising from the pulmonary artery (ALCAPA); however, very few techniques are described for management of coronary artery occlusion following ALCAPA repair. We present a case of a 7-year-old girl with left main coronary atresia status-post left coronary button transfer for ALCAPA in infancy. She underwent redo-sternotomy and left subclavian artery-to-left main coronary artery bypass plus mitral valve repair and had an uneventful postoperative course.

Acute myocardial infarction in the elderly with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA): A case report and literature review.

INTRODUCTION: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary artery malformation, with a fatality rate of 90% at 1 year of age; only 10% to 15% of patients are diagnosed in adulthood. However, elderly survivors are particularly rare. Here, we report a case of elderly ALCAPA presented with acute myocardial infarction. CASE PRESENTATION: A 64-years-old female, complained of acute precordial pain in our hospital for 2 days. She was diagnosed with an acute non-ST-segment elevation myocardial infarction. Aortic angiography revealed emptiness of the left coronary sinus, and coronary angiography showed that the tortuous right coronary artery supplied blood to the left coronary artery through collateral circulation, and the contrast medium spilled from the opening of the left coronary artery. It was suspected that the left coronary artery was opened in the pulmonary artery. This finding was subsequently confirmed by coronary artery CT. The patient refused surgery to restore double coronary circulation and was administered standardized drug treatment. There was no chest pain during the 6-month follow-up. CONCLUSION: ALCAPA should be considered in patients with Myocardial Infarction with Non-obstructive Coronary Arteries, and surgical intervention is the first choice for such patients; However, chronic myocardial damage persists regardless of surgical treatment, prophylactic implantation of an ICD may be an important means of preventing sudden cardiac death and such patients should be followed up for a lifetime.

Influence of age at surgery on left ventricular strain in patients with anomalous origin of left coronary artery from pulmonary artery.

OBJECTIVES: Myocardial strain abnormalities are described after surgical repair of anomalous left coronary artery from pulmonary artery (ALCAPA) even after recovery of ventricular function. The factors that predispose to the presence of these strain abnormalities in the presence of normal ventricular function are unknown. The aim of this study was to find out whether the age at repair influences the presence of global and regional strain abnormalities on follow-up. METHODS: Repaired ALCAPA patients from a single centre (n = 40) with good ventricular ejection fraction on follow-up were recruited. Baseline and follow-up data were collected from electronic records. Global and regional myocardial strain assessment was done by speckle tracking echocardiography prospectively. The association between age at repair and strain abnormalities on follow-up was analysed. RESULTS: The patients who presented earlier had significantly worse ventricular function pre-operatively compared to older patients (P < 0.0005). Global longitudinal strain was abnormal in 40% of patients with normal ventricular ejection fraction on follow-up. Presence of longitudinal strain abnormalities was more in patients who underwent repair at older age than in those who were repaired earlier (P < 0.0005). The probability of having normal longitudinal strain on follow-up was 81.6% if surgery was done before 7.8 months of age. If operated before 6 months, the odds of having normal myocardial strain was 11 times higher. Regional strain abnormalities of varying severity were present in all patients in the left and in some patients in the right coronary artery territories. CONCLUSIONS: Older age at ALCAPA repair is associated with increased incidence of myocardial strain abnormalities. Regional strain abnormalities were found in both left and right coronary artery territories.

Anomalous origin of the left coronary artery from the pulmonary artery as a rare cause of mitral valve prolapse: a case report.

BACKGROUND: Mitral valve prolapse (MVP) is an etiologically heterogeneous disorder. Early diagnosis and prompt treatment of the underlying disease are of great significance. Herein, we present a rare case of MVP caused by anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). CASE PRESENTATION: A 22-year-old female presented with a 16-year history of anterior mitral leaflet prolapse. However, she had never experienced any discomfort before. At a routine follow-up, a transthoracic echocardiogram showed anterior mitral leaflet prolapse (A2) with moderate mitral regurgitation, and a retrograde blood flow from an extremely dilated left coronary artery (LCA). Further coronary angiography and coronary computed tomography angiography confirmed the diagnosis of ALCAPA. She subsequently underwent successful LCA reimplantation and concomitant mitral valve replacement. Intraoperatively, her mitral annulus was mildly dilated, anterior mitral valve leaflet appeared markedly thickened with rolled edges, and a chordae tendineae connecting the anterior leaflet (A2) was ruptured and markedly shortened. CONCLUSIONS: ALCAPA is a rare and potentially life-threatening congenital coronary artery anomaly that may cause mitral valve prolapse. Echocardiogram is an important screening tool for this disorder.

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.

Sudden Cardiac Arrest in an Adult with Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA): Case Report.

INTRODUCTION: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary artery anomaly that carries 90% mortality in the first year of life when left untreated. The diagnosis of ALCAPA is rare in adulthood, and it includes a broad spectrum of clinical manifestations, including sudden cardiac death (SCD). CASE REPORT: We report a rare case of resuscitated sudden cardiac arrest in a 55-year-old female, who was diagnosed with ALCAPA and underwent successful surgical correction and implantable cardioverter defibrillator (ICD) implantation for secondary prevention. DISCUSSION: ALCAPA diagnosis is not confined to childhood, and it represents a rare cause of life-threatening arrhythmias and SCD in the adult population. Surgical correction is recommended, regardless of age, presence of symptoms or inducible myocardial ischemia. Multimodality imaging is crucial for diagnosis, management planning and follow up. Assessment of the risk of recurrent ventricular arrhythmias, despite full revascularization, should be performed in all adults with ALCAPA. Myocardial scar detected via late gadolinium enhancement represents a potential irreversible substrate for ventricular arrhythmias, and it provides additional information to evaluate indication of an ICD for secondary prevention.

Midterm Surgical Outcomes for ALCAPA Repair in Infants and Children.

BACKGROUND: Surgical correction of an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) has been associated with excellent survival during recent years. The purpose of this study was to evaluate the effectiveness of reimplantation of the coronary artery and to investigate the recovery of postoperative cardiac and mitral valve (MV) function. METHODS: From 2005 to 2015, 80 patients who had ALCAPA received surgical correction. Among them, 49 were infants. The median patient age was 7.8 months. Operative strategies included reimplantation of the coronary artery in 71 patients, the Takeuchi procedure in another 7 patients, and coronary artery ligation in the remaining 2 patients. RESULTS: There were 11 hospital deaths and 2 late deaths. Six patients required intraoperative or postoperative mechanical circulatory support. A significant improvement in the ejection fraction (EF) and shortening fraction (SF) was present in all surviving patients at discharge, at a 3-month follow-up and at a 1-year follow-up. MV function improved gradually after surgical repair with no late secondary intervention. CONCLUSIONS: The repair of ALCAPA can be accomplished by establishment of a dual-coronary system, which offers an acceptable mortality rate and will rarely require a second surgery. Left ventricular (LV) recovery is a progressive process, especially for infants with impaired LV function. Concomitant MV annuloplasty is safe and reliable and can be performed as necessary in patients with moderate or severe mitral valve regurgitation.

Coronary Artery Z Score is Associated with Postoperative Outcomes in Patients with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiovascular anomaly that occurs in approximately 1 in 300,000 live births. This study aimed at identifying preoperative predictors of immediate postoperative outcomes. We conducted a retrospective, cross-sectional, single-center study and reviewed echocardiographic and hemodynamic data from all patients before and after surgical repair of ALCAPA at our center from January 2004 to February 2018. In all cases, the left coronary artery arose from the main pulmonary artery or a major branch. A total of 10 patients (age 1 month to 10 years, median 3 months) underwent ALCAPA surgical repair during the study period. No patients required a left ventricular assist device (LVAD) before surgery, but 4 patients (40%) received an LVAD after the surgery. The left ventricular ejection fraction (LVEF) improved in all patients following surgery. The utility of preoperative factors associated with pre- and post-procedure LVEF was investigated. LV dimension, as well as right coronary artery (RCA) and left coronary circumflex artery (LCX) Z scores were associated with a higher LVEF in the preoperative state. Patients with larger RCA, left ascending artery (LAD), and LCX Z scores also had a shorter duration of mechanical ventilation and ICU stay following surgery. Patients with a RCA Z score < 4 required implantation of an LVAD postoperatively. ALCAPA patients with larger RCA and LCX demonstrated a higher preoperative LVEF, while those with larger RCA, LAD, and LCX had superior postoperative hemodynamics and clinical outcomes.

Characteristics and Surgical Outcomes of Patients With Late Presentation of Anomalous Left Coronary Artery From the Pulmonary Artery: A Multicenter Study.

We sought to describe the clinical course and outcomes of patients who are diagnosed with anomalous left coronary artery from the pulmonary artery (ALCAPA) after infancy. We conducted a retrospective evaluation of patients who underwent ALCAPA surgery between January 2009 to March 2018 at 21 US centers. Clinical presentation, inpatient management, and postoperative outcomes of patients repaired ≥1 year of age were described. To characterize this cohort, we compared these data to patients repaired before 1 year of age. Of 248 ALCAPA patients, 71 (29%) underwent repair ≥1 year of age. Among this subset, the median age at diagnosis was 8.3 years. Chronic arrhythmia occurred in 7%. Patients had good postoperative recovery of left ventricle (LV) dysfunction (90%) and LV dilation (75%), although a low incidence of recovery of mitral regurgitation (40%). Compared to infants, older patients were more likely to present with cardiac arrest (11% vs 1%) and less likely to have moderate or worse LV dysfunction or mitral regurgitation. Older patients had significantly less postoperative extracorporeal membrane oxygenation use, and shorter ICU and hospital stay. In the older cohort, operative mortality occurred in only 1 patient and no patient died after discharge (median follow-up 2.7 years). Survival of patients who presented with ALCAPA beyond infancy was excellent, although chronic mitral regurgitation and chronic arrhythmia were not uncommon. Patients who underwent ALCAPA repair ≥1 year of age were less likely to present with LV dysfunction but more likely to present with cardiac arrest than younger patients.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in adults: Collateral circulation does not preclude direct reimplantation.

BACKGROUND: The adult type of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is characterized by late presentation due to collateral coronary flow. Patients often present with long time recurrent angina or dyspnea. Surgical correction can be a challenge due to the vast collateral coronary circulation. CASE PRESENTATION: A previously healthy 41-year-old male presented in the emergency room referring typical angina and dyspnea. Coronary angiography revealed ALCAPA. Surgical correction was indicated. Under cardiopulmonary bypass, the left main coronary artery ostium was isolated and directly reimplanted in the aorta. The patient was discharged from the hospital without complications and asymptomatic. Control coronary angiography showed enlarged left main coronary artery and regression of the collateral circulation. CONCLUSION: Even with a well-developed collateral circulation, detachment of the left coronary artery and direct implantation in the aorta is feasible, achieving good long-term results.

Remote ALCAPA Repair-Total Arterial Reconstruction Using Free Aortic Graft Technique.

A late presenting anomalous left coronary artery from pulmonary artery (ALCAPA) with remote origin may be associated with several technical caveats due to distance for coronary transfer and inadequate autologous tissues for reconstruction. A technique using full circumferential aortic wall as a free graft that is sutured as a posterior hood on an anterior pulmonary arterial flap is used to achieve reconstruction of a neo-left coronary that is tension free, with laminar flow and without the use of any prosthetic material. The technique with potential modifications described could potentially be applied to any variant of ALCAPA to achieve total arterial reconstruction to yield an optimal long-term outcome.

Origen anómalo de la arteria coronaria izquierda a partir de la arteria pulmonar / Anomalous origin of left coronary artery from the pulmonary artery

Introducción: El origen anómalo de la arteria coronaria izquierda que nace en la arteria pulmonar, conocido como síndrome de Bland, White y Garland, es una enfermedad cardiaca congénita, de baja incidencia, espectro clínico variable y no es exclusiva de la edad pediátrica. Tiene una alta mortalidad en el primer año de vida si no se diagnostica y trata oportunamente. Objetivo: Describir las características del diagnóstico del síndrome de Bland, White y Garland. Métodos: Se realizó la caracterización de la entidad en Cuba en un decenio (2005 a 2015). Se incluyeron todos los pacientes con manifestaciones clínicas sugerentes de síndrome de Bland, White y Garland y confirmación ecocardiográfica. Se realizó el cálculo de la prevalencia de la entidad y se empleó el porcentaje en las variables cualitativas y la prueba exacta de Fisher. La validación estadística de los resultados de la investigación adoptó nivel de significación menor a 5 por ciento. Resultados: La prevalencia estimada en esa década en Cuba estaba alrededor de 2,9 por 300 000 nacidos vivos. Se demuestra la variabilidad en sus manifestaciones clínicas y amplio rango de edad de presentación. El tratamiento es quirúrgico con excelente supervivencia y pronóstico. Conclusiones: El diagnóstico del síndrome de Bland, White y Garland es difícil mediante el método clínico debido a la diversidad en su presentación. La prevalencia estimada en Cuba es mayor que lo registrado en el mundo actual. El diagnóstico temprano y tratamiento oportuno mejora de forma significativa la supervivencia y el pronóstico de los pacientes aquejados por esta dolencia(AU)

Introduction: The anomalous origin of left coronary artery that originates in the pulmonary artery, known as Bland, White and Garland is a congenital heart disease, with low incidence, variable clinical spectrum and is not exclusive of the pediatric age group. It has a high mortality in the first year of life if not diagnosed and treated early. Objective: To describe the characteristics of the diagnosis of Bland, White and Garland syndrome. Methods: It was made the characterization of the entity in Cuba in a decade (2005 to 2015). There were included all the patients with clinical manifestations suggestive to Bland, White and Garland syndrome and echocardiographic confirmation. It was made the calculation of this entity´s prevalence and it was used the percentage in the qualitative variables and Fisher's exact test. The statistical validation of research results had a significance level lower than 5 percent. Results: The estimated prevalence in that decade in Cuba was around 2.9 per 300 000 live births. It was demonstrated the variability in its clinical manifestations and wide range of onset´s age. The treatment is surgical with excellent survival and prognosis. Conclusions: The diagnosis of Bland, White and Garland syndrome is difficult through the clinical method due to the diversity in its presentation. The estimated prevalence in Cuba is greater than what was registered in the world today. Early diagnosis and prompt treatment significantly improves the survival and prognosis of patients suffering from this condition(AU)