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.

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.

[Analysis on missed diagnosis or misdiagnosis of anomalous origin of left coronary artery from pulmonary artery by echocardiography from one single medical center].

Objectives: To analyze the reasons of missed diagnosis or misdiagnosis on anomalous origin of left coronary artery from pulmonary artery (ALCAPA) by echocardiography. Methods: This is a retrospective study. Patients with ALCAPA who underwent surgical treatment in Union Hospital, Tongji Medical College, Huazhong University of Science and Technology from August 2008 to December 2021 were included. According to the results of preoperative echocardiography and surgical diagnosis, the patients were divided into confirmed group or missed diagnosis/misdiagnosis group. The results of preoperative echocardiography were collected, and the specific echocardiographic signs were analyzed. According to the experience of the doctors, the echocardiographic signs were divided into four types, namely clear displayed, vague/doubtful displayed, no display and no notice, and the display rate of each sign was calculated (display rate=number of clearly displayed cases/total number of cases×100%). By referring the surgical data, we analyzed and recorded the pathological anatomy and pathophysiological characteristics of the patients, and the rate of missed diagnosis/misdiagnosis of echocardiography in patients with different characteristics was compared. Results: A total of 21 patients were enrolled, including 11 males, aged 1.8 (0.8, 12.3) years (range 1 month to 47 years). Except for one patient with anomalous origin of left anterior descending artery, the others were all originated from the main left coronary artery (LCA). There were 13 cases of ALCAPA in infant and children, and 8 cases of adult ALCAPA. There were 15 cases in the confirmed group (diagnostic accuracy was 71.4% (15/21)), and 6 cases in the missed diagnosis/misdiagnosis group (three cases were misdiagnosed as primary endocardial fibroelastosis, two cases were misdiagnosed as coronary-pulmonary artery fistula; and one case was missed diagnosis). The working years of the physicians in the confirmed group were longer than those in the missed diagnosis/misdiagnosed group ((12.8±5.6) years vs. (8.3±4.7) years, P=0.045). In infants with ALCAPA, the detection rate of LCA-pulmonary shunt (8/10 vs. 0, P=0.035) and coronary collateral circulation (7/10 vs. 0, P=0.042) in confirmed group was higher than that in missed diagnosis/misdiagnosed group. In adult ALCAPA patients, the detection rate of LCA-pulmonary artery shunt was higher in confirmed group than that in missed diagnosis/misdiagnosed group (4/5 vs. 0, P=0.021). The missed diagnosis/misdiagnosis rate of adult type was higher than that of infant type (3/8 vs. 3/13, P=0.410). The rate of missed diagnosis/misdiagnosis was higher in patients with abnormal origin of branches than that of abnormal origin of main trunk (1/1 vs. 5/21, P=0.028). The rate of missed diagnosis/misdiagnosis in patients with LCA running between the main and pulmonary arteries was higher than that distant from the main pulmonary artery septum (4/7 vs. 2/14, P=0.064). The rate of missed diagnosis/misdiagnosis in patients with severe pulmonary hypertension was higher than that in patients without severe pulmonary hypertension (2/3 vs. 4/18, P=0.184). The reasons with an echocardiography missed diagnosis/misdiagnosis rate of≥50% included that (1) the proximal segment of LCA ran between the main and pulmonary arteries; (2) abnormal opening of LCA at the right posterior part of the pulmonary artery; (3) abnormal origin of LCA branches; (4) complicated with severe pulmonary hypertension. Conclusions: Echocardiography physicians' knowledge of ALCAPA and diagnostic vigilance are critical to the accuracy of diagnosis. Attention should be paid to the pediatric cases with no obvious precipitating factors of left ventricular enlargement, regardless of whether the left ventricular function is normal or not, the origin of coronary artery should be routinely explored.

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.

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Associated with Right Coronary Giant Aneurysm.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital malformation, usually diagnosed in the infant period due to myocardial ischemia and heart failure, with the need for emergency surgery. Less commonly, it can be asymptomatic until adulthood. Coronary artery aneurysms are also rare anatomical anomalies with symptoms of acute or chronic angina or even remain completely asymptomatic. We present an unusual case of ALCAPA, associated with a giant aneurysm of the right coronary artery. Meeting presentation: American Association for Thoracic Surgery 102nd annual meeting, Boston MA, USA, May 16, 2022.

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.

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.

Antemortem diagnosis of anomalous origin of the left coronary artery from the pulmonary artery in a dog.

BACKGROUND: In both humans and animals, anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary artery anomaly. In veterinary medicine, ALCAPA is reported to be discovered only during autopsy or necropsy, and diagnostic methods and prognosis remain poorly understood in dogs. CASE PRESENTATION: A 6-month-old Kaninchen Dachshund was diagnosed with functional mitral valve regurgitation and ALCAPA. Echocardiography identified anomalous vessels in the left ventricular wall and abnormal origin of the left coronary artery from the pulmonary artery. Further evaluation with coronary computed tomographic angiography demonstrated the left coronary artery arising from the posterior aspect of the main pulmonary artery together with the characteristic findings of ALCAPA. The right coronary artery was found to be dilated and tortuous. Furthermore, dilated coronary collateral arteries within the ventricular septum and along the epicardial surface were observed. The dog underwent surgery, but the origin of the anomalous artery could not be ligated, and it died from pulmonary edema 5 months after surgery. CONCLUSION: Anomalous origin of the left coronary artery from the pulmonary artery is overlooked in clinical practice due to its rarity. Coronary computed tomographic angiography was useful to definitively diagnose ALCAPA in a low-invasive manner. Antemortem diagnosis of ALCAPA was shown to be possible in dogs for the first time, and presence of unexplained mitral valve regurgitation should raise concern to this anomaly.

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.

Unique Association of Aortopulmonary Window With Anomalous Origin of Left Coronary Artery From Pulmonary Artery in an Infant: A Blessing in Disguise?

Aortopulmonary window (APW) seen in association with anomalous origin of left coronary artery from pulmonary artery (ALCAPA) is extremely uncommon. When faced with this combination, ALCAPA usually goes undetected since most of the clinical and echocardiographic features of ALCAPA, including ventricular dysfunction are absent due to co-existent pulmonary hypertension. Herein we report a 5-month-old child with a large APW in whom a preoperative computed tomography angiogram facilitated the diagnosis of ALCAPA. The case is described for its rarity and the challenges faced during management. Also, it is extremely crucial that this coronary anomaly is identified and corrected during surgery, since failure to do so results in unforeseen postoperative ventricular dysfunction.

Anomalous origin of left main coronary artery from pulmonary artery: Patient characteristics and imaging associations on multidetector computed tomography angiography.

AIM: To evaluate the various imaging features and associations on multidetector computed tomography (CT) angiography in patients with anomalous origin of left main coronary artery (LMCA) from pulmonary artery (ALCAPA). MATERIALS AND METHODS: We retrospectively reviewed multidetector CT angiography studies done for the evaluation of congenital heart diseases at our institution through 2014 to 2021. Cases with ALCAPA were identified and relevant history and imaging findings including the origin of coronary arteries, left ventricular (LV) morphology and functions, intercoronary collaterals, and associated abnormalities were evaluated. RESULTS: Twelve patients (eight males, three adults, and nine children; age range: 2 months to 54 years) with ALCAPA were included. Gradually progressive dyspnea and failure to thrive (6/9; 66.67% each) were the most common symptoms among children, whereas adults were commonly asymptomatic (2/3; 66.67%). The LMCA was originating from pulmonary sinus, main, and right pulmonary artery in 6 (50%), 5 (41.66%), and 1 (8.3%) patients, respectively. In adult-type ALCAPA, right coronary artery (RCA), left anterior descending artery (LAD), and left circumflex artery (LCx) were dilated and tortuous, with the presence of well-developed intercoronary collaterals and preserved LV ejection fractions; these features were not seen in patients of infantile ALCAPA. LV dysfunction with global hypokinesia was the most common wall motion abnormality (7/12; 58.33%). CONCLUSION: Degree of collateralization could be the key factor determining the time of presentation, clinical symptoms, and LV function, thus influencing clinical outcomes. Patients with infantile ALCAPA present with features of heart failure and have dilated and dysfunctional LV consequent to lack of collaterals, unlike adult-type ALCAPA.

Unexplained near-drowning can reveal ALCAPA in children.

An 11-year-old girl experienced an episode of near-drowning. She was immediately rescued and was defibrillated. Transthoracic echocardiography and coronary computed tomographic angiography confirmed the diagnosis of anomalous left coronary artery from the pulmonary artery (ALCAPA). We report a rare description of this congenital coronary anomaly in a child, revealed after exercise-induced sudden cardiac arrest while swimming.

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.

Síndrome de ALCAPA em uma Mulher Jovem / ALCAPA Syndrome in a Young Woman

Mulher de 18 anos com histórico de síncope, angina e palpitações há um ano. Uma indicação crucial era artéria coronária direita dilatada na ecocardiografia transtorácica. Os achados da tomografia computadorizada resultaram no diagnóstico da origem anômala da artéria coronariana esquerda proveniente da síndrome da artéria pulmonar.(AU)

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)

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) diagnosed in children and adolescents.

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially fatal congenital coronary anomaly associated with early infant mortality and sudden adult death. By the development or lack of coronary collateral, it can be classified as infantile or adult type. However, even with the compensatory mechanism in adult patients, there is an estimated 80 to 90% incidence of sudden death at the mean age of 35 years. METHODS: We enrolled 9 patients with ALCAPA within the age group 5 to 16 years. RESULTS: Only one patient developed symptoms (apsychia), whereas other patients were asymptomatic, and there was no evident left ventricular dysfunction found in any of the cases. CONCLUSION: With the development of imaging techniques, asymptomatic adult-type ALCAPA patients could be identified and diagnosed in childhood or adolescence. As a potential cause of sudden death, ALCAPA should be surgically repaired soon after the diagnosis.

Transthoracic echocardiography features of adult-type anomalous left coronary artery from the pulmonary artery before and after surgery: highlights from observational study in a single center of China.

To review the imaging characteristics of adult-type anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) and evaluate the post-operative echocardiographic features. The transthoracic echocardiography (TTE) records and invasive coronary angiography (ICA), coronary computed tomography angiography (CTA) or operative findings of thirty adult patients with final diagnosis of ALCAPA were reviewed at our center. The diagnostic reliability of TTE was determined by comparing its results with that of ICA/CTA, and the echocardiographic features of the patients during early visit and post-operative follow-up were summarized. The coincidence rate of TTE and ICA/CTA diagnosing ALCAPA was 96.3% (26/27), and one case was misdiagnosed as coronary artery fistula. All patients showed abnormal left coronary ostium arising from the pulmonary trunk with retrograde coronary artery flow, 20 patients had enlarged right coronary artery (RCA), and 20 showed abundant inter-coronary septal coronary collaterals. Left ventricle (LV) abnormalities such as wall thinning, abnormal wall motion, papillary muscle fibrosis, mitral valve (MV) prolapse and moderate-severe mitral regurgitation (MR) were respectively observed in 4, 7, 10, 8 and 10 patients. Three patients showed ventricular septal defect, patent foramen ovale and tetralogy of Fallot respectively. Post-operative data was available for 12 patients, and showed that the diameter of RCA, size of left atrium (LA) and LV were decreased after surgery compared to the respective pre-operative values. Furthermore, inter-coronary septal coronary collaterals lessened in 8 of these patients. Although 6 patients showed residual mild MR, moderate or severe MR was not observed, and the ejection fraction (EF) also showed no obvious changes before and after surgery. TTE is a non-invasive diagnostic tool for adult-type ALCAPA, and can indicate abnormal coronary origin, collateral arteries and other associated malformations. Some of the structural and hemodynamic parameters of adult-type ALCAPA were improved after surgery.