Anomalous Left Coronary Artery Arising From The Pulmonary Artery Research Articles

Abstract 4145822: The Enigma of Chest Pain: Rare Presentation of ALCAPA Syndrome in a Young Adult

Introduction: Anomalous Left Coronary Artery Arising from the Pulmonary Artery (ALCAPA), also known as Bland-White-Garland syndrome, is a rare congenital anomaly(incidence:1 in 300,000 live births) typically presenting during infancy. Adult presentations are even rarer due to a high mortality rate of nearly 90% in the first year of life without surgical intervention. This report examines an adult patient with exercise-induced chest pain who received a delayed diagnosis of ALCAPA via multimodal imaging. Case Summary: A 21-year-old male presented to the emergency department(ED) with severe retrosternal chest pain radiating to both shoulders and arms, which began during exercise 30 minutes prior. He had a history of similar, milder episodes during exercise, previously treated as musculoskeletal pain and gastroesophageal reflux disease in the ED. His medical, social, and family histories were unremarkable. Physical examination revealed a soft, continuous murmur along the left sternal border. During this visit, An EKG done showed sinus tachycardia at 110 bpm and deep T-wave inversions in the anterolateral leads, a dynamic ST-T changes from his baseline.Serial Cardiac biomarkers and a lipid profile were within normal ranges. Initially managed for acute coronary syndrome-unstable angina with antithrombotic therapy, beta-blockers, and nitrates, he underwent urgent coronary angiography. This revealed a dilated right coronary artery (RCA) with retrograde flow to the left coronary artery (LCA) into the main pulmonary artery (PA) via collaterals, with no evidence of atherosclerosis (Figure 1).Echocardiography (Figure 2) and coronary CT angiography (Figure 3) confirmed the diagnosis of ALCAPA. The patient underwent successful surgical repair with aortic reimplantation of the LCA and was discharged in good health. Discussion: In adult ALCAPA patients, good collateral circulation may develop; however, myocardial ischemia can occur due to the "coronary steal phenomenon" and poor collateral flow regulation, leading to angina, arrhythmias, and sudden cardiac death. ALCAPA should be considered a differential diagnosis in adults presenting with exercise intolerance, as most sudden cardiac death cases occur by the fourth decade. A high index of clinical suspicion and a multimodality imaging approach is essential for the early diagnosis of this rare coronary anomaly, underscoring the critical role of early surgical intervention in preventing catastrophic, life-threatening events.

Adult type anomalous left coronary artery arising from the pulmonary artery (ALCAPA)

Adult type anomalous left coronary artery arising from the pulmonary artery (ALCAPA)

Repair of anomalous origin of the left coronary artery from the pulmonary artery in infants

Anatomical repair seems an ideal method for the surgical treatment of the anomalous left coronary artery arising from the pulmonary artery (ALCAPA) in infancy. The medium-term outcome has been investigated for infants with ALCAPA following the restoration of a dual-coronary arterial circulation. Between April 1995 and July 2012, 23 infants with a median age of 4 months underwent surgical repair of ALCAPA in our department. Direct implantation of the anomalous coronary artery into the ascending aorta was feasible in 16 patients. A trap door flap method was used in 5 cases and a tubular extension technique in 2. No infant underwent mitral valve repair at the time of ALCAPA surgery. Left ventricular function and the degree of mitral valve regurgitation were assessed during a 10-year follow-up. Four patients died in the early postoperative period, without independent predictors associated with this mortality. During follow-up, improvement in myocardial function occurred in all patients both early and late. There was only one improvement in severe mitral valve regurgitation. Subsequently, 2 children needed mitral valve replacement. There were no early or late reoperations of the reimplanted coronary arteries. Aortic reimplantation is an effective surgical treatment for ALCAPA in infants burdened with a low risk of reoperation due to coronary artery stenosis. There was good potential for myocardial recovery within the first year after surgery. Restoration of the anatomical coronary circulation did not improve mitral valve function in infants with severe preoperative mitral incompetence.

Anomalous Left Coronary Artery from the Pulmonary Artery with a Large Patent Ductus Arteriosus: Aversion of a Catastrophe

We present an infant who had an anomalous left coronary artery arising from the pulmonary artery (ALCAPA) and a large patent ductus arteriosus (PDA), who was diagnosed before a potentially catastrophic closure of PDA. In the presence of normal left ventricular function and the absence of coronary artery collaterals, it is difficult to diagnose ALCAPA. A disproportionate degree of left ventricular dilation and severity of mitral valve regurgitation relative to the degree of PDA shunt, and echogenic papillary muscles on an echocardiogram should raise a suspicion of coronary artery anomalies. The infant underwent surgical ligation of PDA with translocation of coronary arteries and had an uneventful recovery.

Anomalous left coronary artery arising from the pulmonary artery : case report - online article

A 24-year-old female presented to her general practitioner with shortness of breath. She was referred for an echocardiogram, which demonstrated features suggestive of a right coronary artery fistula, and referred to our institute. We performed a contrast-enhanced, prospectively triggered cardiac CT angiogram, which demonstrated the primary and secondary features of anomalous left coronary artery arising from the pulmonary artery (ALCAPA), also known as the Bland-White-Garland syndrome, a rare congenital abnormality of the origin of the left main coronary artery.

ALCAPA and Heart Rate

We read with interest the article titled “The Anesthetic Management and Physiologic Implications in Infants With Anomalous Left Coronary Artery Arising From the Pulmonary Artery” by Fahy et al.1 We congratulate the authors for the successful management of 2 difficult cases of an anomalous left coronary artery arising from the pulmonary artery (ALCAPA). We agree that the main anesthetic goals in patients with an ALCAPA are to ensure adequate right coronary artery perfusion and to prevent any further increases in coronary artery “steal” by preserving the pulmonary vascular resistance by avoiding hyperventilation and high concentrations of inspired oxygen.

An Unusual Presentation of an Anomalous Left Coronary Artery Arising from the Pulmonary Artery (ALCAPA) in an Adult: Anterior Papillary Muscle Rupture Causing Severe Mitral Regurgitation

We describe a 29-year-old male, previously in good health, with no history of angina pectoris and no risk factors for ischemic heart disease presenting with biventricular failure and severe mitral valve regurgitation. There were no signs or serological test results to suggest infective endocarditis. Transthoracic echocardiography (TTE) revealed severe anterior mitral valve prolapse secondary to papillary muscle rupture, severe mitral valve regurgitation, as well as an anterior myocardial wall hypokinesis. Parasternal short-axis view showed an anomalous left coronary artery arising from the pulmonary artery (ALCAPA), which was confirmed on coronary angiography. This is an unusual presentation of ALCAPA in an adult.

Continuing Medical Education Program in Echocardiography

Article Title: An Unusual Presentation of an Anomalous Left Coronary Artery Arising from the Pulmonary Artery (ALCAPA) in an Adult: Anterior Papillary Muscle Rupture Causing Severe Mitral Regurgitation

Bland-White-Garland syndrome of anomalous left coronary artery arising from the pulmonary artery (ALCAPA): a historical review

The landmark 1933 case report from Massachusetts General Hospital by Bland, White and Garland (Am Heart J 8:787-801) described a 3-month-old child with progressive feeding problems, cardiomegaly on chest radiography, and EKG evidence of left ventricular damage. Of interest was the fact that the vigilant father of the infant was Aubrey Hampton, a radiologist and future chairman of radiology at Massachusetts General Hospital. At autopsy, the left coronary artery originated from the pulmonary artery rather than from the aorta. Effective treatment for this condition was not available until 1960 when Sabiston, Neill and Taussig showed that the blood flowed from the left coronary artery toward the pulmonary artery. The anomalous left coronary artery was ligated at its junction with the pulmonary artery and the child survived. This historical review of Bland-White-Garland syndrome, now known as anomalous left coronary artery arising from the pulmonary artery (ALCAPA), stresses the continued diagnostic significance of cardiomegaly on chest radiography and EKG changes suggesting left ventricular damage in 2- to 3-month-old infants with feeding intolerance or irritability. With a high index of suspicion, an echocardiogram can be obtained to confirm the diagnosis. Modern surgical methods involve left coronary artery translocation and afford excellent outcomes.

Fatal coronary artery anomaly presenting as bronchiolitis

Sir: We read with great interest the article from Piastra et al. [3] describing three cases of Anomalous Left Coronary Artery Arising from the Pulmonary Artery (ALCAPA) simulating a bronchiolitis. The article is interesting since it focuses on a congenital anomaly that can easily be misdiagnosed. However, it proposes an approach to avoid error that – in our view – would not be effective. Concerning the implantation of “ ...a training programme to favour limited echocardiography learning by the emergency physicians...”, we consider that the likelihood that such a specialist would be able to diagnose ALCAPA with reliability is unrealistic. Indeed, and as the authors controversially state in their abstract, “...paediatric cardiological evaluation with twodimensional echocardiography may eventually reveal this rare condition, whereas cardiac catheterisation remains the standard means of diagnosis...”. This statement illustrates how difficult it still is to clearly diagnose aberrant origin and course of the coronary artery even by an experienced paediatric cardiologist, and particularly whenever associated with other cardiac lesions simulating a normal physiological scenario [1, 2, 4] Thus, it seems too optimistic to allude that emergency physicians will be skilled enough to do so. Chest X-ray should remain mandatory, and as a matter of fact, a toddler with a suspected non-respiratory wheezing condition and a cardiomegaly, mostly if presenting with signs of circulatory failure, should systematically also have an electrocardiogram with the objective of revealing ischaemic changes evocating the diagnosis as well as echocardiography performed by a paediatric cardiologist, since many other entities other than ALCAPA might induce a dilated hypokynetic cardiomyopathy with similar clinical features: late-diagnosed aortic coarctation, primary cardiomyopathy, myocarditis, sustained arrhythmias, to mention some. The cardiologist would then, based upon the iconographic quality of the echocardiography, decide the need for the cardiac catheterisation. Last, but not least, one should not forget that ALCAPA and a bronchiolitis-like syndrome may co-exist in a same patient, all the more that the first might be unmasked by the last. Eduardo da Cruz, MD Yacine Aggoun, MD Cecile Tissot-Daguette, MD Maurice Beghetti, MD

Emergency ligation of anomalous left coronary artery arising from the pulmonary artery

We report two cases of successful emergency ligation of anomalous left coronary artery arising from the pulmonary artery (ALCAPA) in patients with previous cardiac arrest. Both patients had regained marginal cardiac output after cardiopulmonary resuscitation and had maximal doses of inotropic support. The ALCAPA ligation was then performed as a life-saving procedure in the absence of any kind of mechanical circulatory support.

Anomalous left coronary artery arising from the pulmonary artery: A series of 27 infants undergoing operation in the first years of life

Between 1977 and 1987, 27 infants (aged 3 to 54 months) underwent surgical treatment for correction of an anomalous left coronary artery arising from the pulmonary artery (ALCAPA). All had a direct aortic reimplantation. The overall operative mortality was 18.5% (five deaths). The follow-up period was from 2 months to 8 years (mean, 38 months). No late deaths occurred. During the 10 years, modifications of the surgical procedure and myocardial preservation were introduced (is, complete resection of the pulmonary trunk made anastomosis easier; since 1982, cardioplegia in both coronary systems has been used, and left atrial-to-aortic assistance was introduced). The latter was carried out when surgical repair was associated with acute cardiac failure and a high left atrial filling pressure at the end of cardiopulmonary bypass. In view of the results during the latter part of this series, it is felt that surgery should not be restricted in younger patients (<12 months). If medical treatment is unsuccessful, surgery aims to avoid irreversible left ventricular dysfunction and development of severe endocardial fibroelastosis.