Unique Echocardiographic Markers of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) in the Adult

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect in adults. We report a 38‐year‐old male presenting with exertional syncope. He was referred for the evaluation of multiple muscular ventricular septal defects diagnosed on an outpatient echocardiogram. Echocardiography revealed mild left ventricular enlargement, abnormal flow‐pattern in the ventricular septum and dilatation of the right coronary artery. Pulsed‐wave Doppler with sample volume placed in the coronary ostium showed systolic coronary flow predominancy. This unique finding is characteristic for ALCAPA and can differentiate it from other coronary anomalies. Coronary angiography confirmed ALCAPA syndrome. Surgical correction was planned.     

A rare case of anomalous origin of the left anterior descending artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease, with an incidence of <1 in 300 000 live births. We describe a rare case of a 23‐month‐old infant admitted for cough and fever. Echocardiography showed a branch of the left coronary artery originating from the pulmonary artery. The coronary computed tomographic angiography revealed anomalous origin of the left anterior descending (LAD) artery from the pulmonary artery with the left circumflex artery and right coronary artery arising normally from the aorta. The infant successfully underwent surgical reimplantation of the LAD to the ascending aorta. Our case emphasizes that echocardiography and computed tomographic angiography are valuable imaging modalities for making an accurate diagnosis and determining the precise surgical plan.     

Adult‐type ALCAPA syndrome: A rare coronary artery anomaly

Anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA or Bland–White–Garland syndrome) is a rare but serious congenital coronary artery anomaly, with a poor prognosis without surgical repair. There are two types of ALCAPA syndrome: infant type and adult type. We present a rare case of a 63‐year‐old female patient, with isolated left anterior descending artery origin from the pulmonary artery. Coronary computed tomography angiography revealed giant and tortuous coronary arteries with many collaterals between the left and right coronary system. The patient refused any surgical treatment.     

An Unusual Combination of an Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) and a Right Coronary Artery System with Two Separate Ostia from the Aorta in an Adult

We describe a patient with an infrequent combination of variants in both the right and the left coronary arterial ostia, namely a combination of two separate right coronary artery (RCA) ostia from the aorta, and an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). To our knowledge, such a combination has not been previously reported. Based on published statistics for individual variants, such a combination is expected to occur approximately once for every 500,000 to one million live births. ALCAPA and dual RCA anatomy was characterized in our patient by echocardiography, conventional angiography, and multidetector computed tomography before and after Takeuchi repair. (Echocardiography 2010;27:E13-E17)     

Anomalous left coronary artery from the pulmonary artery: case report and review of the literature

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is an uncommon occurrence, usually diagnosed at a young age. We report a 71-year-old patient who died suddenly of acute bilateral bronchopneumonia, and was found to have ALCAPA at autopsy. The patient had reported no cardiac symptoms during his lifetime. Autopsy revealed collateral connections between the left coronary artery (LCA) and right coronary artery (RCA), which were diffusely dilated, calcified and atherosclerotic.     

Comprehensive intraoperative transesophageal echocardiography of anomalous left coronary artery from pulmonary artery: What to look for and where to look?

Anomalous origin of left coronary artery from pulmonary artery (ALCAPA) accounts for 0.4% of the congenital heart diseases. Comprehensive 2D and 3D transesophageal echocardiographic imaging of a well‐collateralized subset of ALCAPA is described. A nonstandard short‐axis view of both aorta and pulmonary arteries showed the origin of left coronary artery from the posterior sinus of the pulmonary artery and right coronary artery in its usual position. Pulse‐wave interrogation of the coronary arteries showed the direction of flow in opposite directions. Using the real time‐3D, the en‐face views of the origins of both coronaries were also demonstrated.     

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 ventriclular 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.     

Respiratory Syncytial Viral Infection in an Infant with Unrepaired Anomalous Left Coronary Artery from the Pulmonary Artery

Abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary anomaly in children that requires necessary and urgent repair. We report a child who was hospitalized with respiratory failure due respiratory syncytial viral (RSV) infection and was subsequently diagnosed with ALCAPA. Aggressive treatment for RSV included synagis and nebulized ribavirin prior to surgical repair. After waiting 4 weeks for the RSV infection to resolve, she underwent successful left coronary artery reimplantation on hospital day 27 and has regained normal left ventricular size and function.     

Myocardial Flow Reserve in Long-Term Survivors of Repair of Anomalous Left Coronary Artery From Pulmonary Artery

Objectives. This study sought to evaluate regional myocardial flow reserve in long-term survivors of repair of anomalous left coronary artery from pulmonary artery (ALCAPA) and to relate the flow abnormalities to the patients’ exercise performance.Background. Patients with ALCAPA usually present during infancy with severe ischemic cardiomyopathy. The left ventricular function recovers after surgical repair. However, the extent of recovery of myocardial blood flow (MBF) and its potential physiologic significance in long-term survivors are unknown.Methods. We evaluated MBF (ml/g per min) at baseline and during maximal coronary vasodilation by adenosine in 11 patients after ALCAPA repair (median age 17 years, range 7 to 22) using nitrogen-13 ammonia and dynamic positron emission tomographic imaging. Patients also underwent an incremental exercise test with metabolic monitoring. In each patient, MBF was quantified in the three major vascular territories: the left anterior descending and left circumflex coronary artery territories and the right coronary artery (control region) territory.Results. Basal MBF was mildly reduced in the left coronary territories versus the control region (0.79 ± 0.14 vs. 0.85 ± 0.19, p = 0.05). During hyperemia, flow in the left coronary territories was significantly lower than that in the control region (2.1 ± 0.5 vs. 2.6 ± 0.5, p < 0.001). As a result, myocardial flow reserve was lower in the left coronary territories than in the control region (2.6 ± 0.7 vs. 3.2 ± 0.7, p < 0.001). Exercise performance was impaired in patients when compared with age-matched control subjects. Maximal oxygen consumption correlated linearly with maximal hyperemic flows in the left coronary artery territories (r = 0.73, p = 0.03).Conclusions. Long-term survivors of ALCAPA repair demonstrate regional impairment of myocardial flow reserve. This may contribute to impaired exercise performance by limiting cardiac output reserve.     

Repair of an Anomalous Left Coronary Artery from the Pulmonary Artery in a Premature Neonate

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect. It is typically diagnosed at a few months of age. The infant presents with cardiac failure secondary to left ventricular dysfunction from the coronary steal that develops as pulmonary vascular resistance (PVR) falls. With the diagnosis made, surgical repair is typically performed expeditiously in order to try and restore left ventricular perfusion and stop the ongoing coronary steal. We present an unusual case of a preterm infant undergoing a routine echocardiogram, who was incidentally found to have an ALCAPA. Management strategies between neonatology and cardiology may differ in this setting. In this case, elevated PVR helps to preserve myocardial perfusion prior to surgical repair. Therefore, common neonatal management strategies typically utilized for respiratory distress, elevated PVR, and cardiac dysfunction may have potential for detrimental effects on myocardial perfusion in this specific lesion. This case also emphasizes the importance of identifying the coronary origins as a routine part of a complete pediatric echocardiogram. It also presents a rarely encountered problem: when to repair an ALCAPA in an as yet asymptomatic patient. We believe this to be the youngest and smallest patient reported with the diagnosis and successful surgical correction of an ALCAPA.     

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.     

A rare case of anomalous left coronary artery from the pulmonary artery (ALCAPA) presenting congestive heart failure in an adult

A 41-year-old woman with no modifiable coronary risk factors presented with a progressive exertional dyspnea. Chest radiography showed an enlarged cardiac silhouette with reinforced pulmonary vasculature in bilateral lower lung fields and both pleural effusion. Echocardiography revealed a dilated, globally hypokinetic left ventricle with an ejection fraction of 40%. Multislice cardiac computed tomography revealed abnormal origin of LCA from the main pulmonary artery receiving collaterals from a normally originating dilated tortuous right coronary artery. Coronary angiography revealed a single, large, and tortuous RCA arising from the right sinus of Valsalva and giving off extensive collateral vessels coursing over the right ventricular wall, the interventricular septum, and the apex to the left coronary artery that was drained into the proximal main pulmonary. The diagnosis of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) was confirmed. The patient underwent successful reimplantation of LCA to aorta after closure of the ostium (in the main pulmonary artery) of the anomalously originating LCA. After surgical and medical treatment, the patient's symptoms were relieved and both pleural effusions were improved on chest radiography. After then, the patient was discharged on medication.     

Incidental Discovery of Anomalous Left Coronary Artery Arising from the Pulmonary Artery in a Coronavirus Disease-2019 Patient: A Blessing in Disguise

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a serious congenital malformation. Reports about asymptomatic, incidentally discovered ALCAPA in adults are scarce. We describe a patient with no known pre-existing cardiac condition admitted to our hospital with coronavirus disease 2019 (COVID-19) and was incidentally found to have ALCAPA. To the best of our knowledge, this is the first reported case of incidentally discovered ALCAPA in a COVID-19 patient and highlights the importance of appropriate investigation of the coronary status by Multidetector Cardiac Computed Tomographic Angiography (MDCCTA) in individuals with asymptomatic left ventricular dysfunction. The presentation of this case, discussion and literature review serves to iterate the necessity of appropriately investigating patients with asymptomatic LV dysfunction.     

Pulmonary artery origin of the left coronary artery: diagnosis by transoesophageal echocardiography in infancy

In two infants the anomalous origin of the left coronary arteryfrom the pulmonary artery (ALCAPA) was detected prospectivelyby transoesophageal echocardiography (TEE). Colour flow mappingand pulsed Doppler ultrasound revealed a predominant right-to-leftshunt from the pulmonary artery to the left coronary artery.These findings were confirmed by angiography. Transihoracic echocardiography (TTE)failed to show the anomalousorigin of the left coronary artery or any abnormal pulmonaryflow pattern. TEE may be useful in the diagnosis of ALCAPA in selected cases,when TTE is inconclusive.     

The Management of the Older Adult Patient with Anomalous Left Coronary Artery from the Pulmonary Artery Syndrome: A Presentation of Two Cases and Review of the Literature

ALCAPA (anomalous left coronary artery from pulmonary artery) syndrome is a rare congenital abnormality that involves an anomalous insertion of the left coronary artery into the pulmonary artery. Ninety percent of patients present in the first year of life with signs and symptoms of heart failure or sudden cardiac death secondary to chronic myocardial ischemia. There have been an increasing number of reports of ALCAPA patients surviving to adulthood. There seems, however, to be a tendency to die suddenly in the third decade of life. Adult survivors are either asymptomatic or present with mitral regurgitation, cardiomyopathy, myocardial ischemia, or malignant arrhythmias. The management of the older patient presenting with symptoms resulting from ischemia and progressive left ventricular dysfunction remains a challenge. Treatment is largely based on guidelines for adult congenital heart disease management and an extrapolation of evidence from heart failure practice. Currently, surgical reimplantation of the anomalous coronary onto the aorta is the mainstay of treatment. The management of heart failure, sudden cardiac death, and ventricular arrhythmia present problems that are not addressed by reimplantation of the anomalous vessel alone. In this report, we present two cases with different modes of presentation and discuss treatment options.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in an Asymptomatic Adult

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly with a mortality of 90% in the first year of life, if not surgically corrected. Adult presentation of the anomalous origin of the left coronary artery from the pulmonary artery syndrome is extremely rare but may occur if a well‐developed collateral circulation from right coronary artery is present. We present the case of a 22‐year‐old asymptomatic female with anomalous origin of the left coronary artery from the pulmonary artery.     

Resynchronization and Strain Recovery Following Reimplantation of Anomalous Coronary Artery: A Tissue Doppler and Three-dimensional Echocardiographic Study

Resynchronization may play a significant role in recovery following surgical reimplantation of anomalous left coronary artery from the pulmonary artery (ALCAPA). Three-dimensional echocardiography and tissue Doppler may quantify this recovery. A 6-week-old infant presented with signs of congestive heart failure due to ALCAPA. Two-dimensional echocardiography showed a severely dilated left ventricular (LV) cardiomyopathy and the presence of ALCAPA was confirmed by catheterization. Three-dimensional echocardiography and tissue Doppler imaging showed severe abnormalities of systolic and diastolic synchrony and decreased contractility with a left ventricular ejection fraction (LVEF) of 13%. The infant underwent surgical coronary reimplantation and was discharged 5 weeks later with an LVEF of 54%. Serial quantitative assessment showed resynchronization and normalization of global LV function following reimplantation. However, segmental contractility improved significantly but remained depressed at discharge. The immediate recovery observed following reimplantation of anomalous coronary may be largely due to resynchronization.     

A 72 year old woman with ALCAPA

ALCAPA syndrome (anomalous origin of the left coronary artery from the pulmonary artery), which causes the left coronary artery to grow with an anomalous origin from the pulmonary artery, is a rare disease which may result in myocardial infarction, congestive heart failure, and sometimes death during the early infantile period. A 72 year old woman with ALCAPA syndrome is presented. The asymptomatic patient presented with a cardiac murmur which was discovered during a routine check up for a gynaecological intervention. Coronary cineangiography established the diagnosis. Although surgical correction is the usual treatment for such cases, medical treatment was preferred for this patient because she was asymptomatic without clinical signs of heart failure.


Keywords: coronary vessel anomalies; ALCAPA syndrome     

Anomalous Origin of Left Coronary Artery From Pulmonary Artery: Recovery of Left Ventricular Function After Dual Coronary Repair

Objectives. We reviewed our institutional experience with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) after dual coronary repair to assess preoperative variables predictive of outcome, the time course for postoperative recovery of cardiac function, the short- and long-term complications and our experience with left ventricular assist devices (LVAD) in these patients.Background. Outcome after surgical repair of ALCAPA remains incompletely defined.Methods. The surgical records and echocardiograms of 42 patients were reviewed. Left ventricular function was assessed by fractional shortening z-score (FSz) and stress-velocity index.Results. The overall survival rate was 86%. All six patients who died were <1 year old and died within 3 days of the operation. More severe preoperative mitral regurgitation (MR) was associated with increased mortality, but age, body surface area, preoperative FSz and end-diastolic dimension were not. We used an LVAD for 7 of 28 patients who underwent repair for ALCAPA since its introduction at our institution, with a survival of 5 of 7 patients. The degree of MR improved in 62% of patients and remained unchanged in 38%. Complications included supravalvar pulmonary stenosis (16 of 21 patients) and baffle leaks (11 of 21 patients) with the intrapulmonary baffling technique. Supravalvar pulmonary stenosis developed in 1 of 11 patients after direct coronary reimplantation. Left ventricular function became normalized in all 28 patients with follow-up past 1 year, regardless of preoperative FSz. Of 13 patients who underwent serial postoperative echocardiography, the average time to normalization of function was 2 to 7 months.Conclusions. The degree of preoperative MR was predictive of outcome, whereas the severity of preoperative cardiac dysfunction and ventricular dilation were not. Mild and moderate MR tended to improve without mitral valvuloplasty. Complete recovery from myocardial dysfunction is expected after dual coronary repair of ALCAPA.     

Percutaneous coil closure of recanalised anomalous origin of left coronary artery from pulmonary artery

An asymptomatic boy underwent surgical correction of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) with trans-pulmonary artery interruption and saphenous vein grafting to left anterior descending coronary artery. He developed a shunt through the re-canalised pulmonary artery end of the ALCAPA which was successfully embolised using a detachable PDA coil delivered into the left main coronary artery from the pulmonary artery.