Asymptomatic Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly of the coronary arteries which usually presents with heart failure in early infancy. We describe the unusual occurrence of this in an older child with no signs of cardiac ischemia. Electronic supplementary material  The online version of this article (doi:) contains supplementary material, which is available to authorized users.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in a Premature Infant with Preserved Left Ventricular Function

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare condition. The majority of cases present with impaired left ventricular function. We report on a premature infant who was diagnosed at a gestational age near term with ALCAPA during routine cardiac examination. The patient showed no signs of myocardial ischemia and is doing well after corrective surgery. This is the youngest patient reported with ALCAPA and preserved myocardial function.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in Children: 15 Years Experience

This study aimed to illustrate the experience of treating children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). The clinical data for 19 children with ALCAPA admitted to Beijing Anzhen Hospital from August 1993 to June 2009 were reviewed. According to the data, 47.4% (9/19) of the patients had a misdiagnosis of endocardial fibroelastosis, and 15.8% (3/19) had a misdisgnosis of dilated cardiomyopathy. Electrocardiography showed abnormal Q waves with T wave inversion in leads I, avL, and V4-V6 of 18 patients, especially in lead avL. The ratio of proximal right coronary artery diameter to aotic root diameter exceeded 0.20 for 15 of 16 patients. Apical ventricular aneurysm or aneurysmal dilation (52.6%,10/19), enhanced echogenicity of papillary muscles (84.2%, 16/19), and increased coronary collaterals (78.9%, 15/19) were detected frequently during echocardiography. A total of 18 patients underwent cardiac surgery including left coronary artery (LCA) ligation for 1 patient (5.6%), LCA ligation plus coronary artery bypass grafting for 1 patient (5.6%), Takeuchi operation for 7 patients (38.9%), and LCA reimplantation for 9 patients (50.0%). Five patients died in the hospital, and the remainder were asymptomatic during a follow-up period of 6 to 166 months. Their abnormal Q waves gradually regressed, and left ventricular systolic function and size returned to normal with alleviation of mitral insufficiency. The clinical features of ALCAPA are helpful for determining an accurate diagnosis. This anomaly can be treated successfully by several types of operations with good prognosis.     

Association of Common Origin of the Carotid Arteries with Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery

Common origin of the carotid arteries (COCA) is a common pattern of aortic arch vessels and is the single most common cause of tracheobronchial compression by a congenital cardiovascular anomaly. By no means all affected patients are symptomatic. Symptoms may range from recurrent pulmonary infections and “noisy respiration” to stridor and apneic spells. In our study of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) we found a highly significant association of COCA with ALCAPA (85%), although no patient with ALCAPA in this study had evidence of tracheal stenosis documented in the hospital chart. As COCA is easily correctable, we suggest consideration of COCA during evaluation and surgery of patients with ALCAPA so that, if the patient also has symptoms possibly related to COCA, the artery can be suspended from the posterior wall of the sternum.     

Anomalous Origin of Left Pulmonary Artery from the Aorta with Partial Anomalous Pulmonary Venous Return

Anomalous origin of left pulmonary artery (AOLPA) from the aorta is a rare congenital cardiac malformation. We report a case of AOLPA from the aorta with partial anomalous pulmonary venous return (PAPVR) in a 3.2 kg infant. Surgical correction was performed with direct anastomosis of AOLPA to the main pulmonary artery with subsequent baffling of the anomalous right pulmonary veins to the left atrium and patch augmentation of the left pulmonary artery performed at 1-month of life. Recurrent stenosis of the proximal left pulmonary artery was treated with stent placement at 17-months. Pulmonary hypertension has persisted at 30-month follow up.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) Presenting as a Complete Heart Block

A 51-year-old previously asymptomatic man presented with complete heart block (CHB). During pacemaker implantation, fluoroscopy showed a peculiar pattern of cardiac calcification. Coronary angiography, performed to determine the origin of calcification, demonstrated an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). A left ventriculogram showed normal ventricular contraction. Echocardiography demonstrated normal systolic function without any regional wall motion abnormality. The endocardium of the mid and basal portions of the anteroseptal, anterior and anterolateral walls as well as both of the papillary muscles were calcified. Specifically noted was a calcific bar extending across the base of the interventricular septum (IVS) on both the echocardiogram and the left ventricle angiogram. The development of CHB in the absence of transmural myocardial infarction is intriguing. It is likely that endocardial fibroelastosis during infancy led to endocardial fibrosis and scarring subsequent calcium deposition. Extension of this calcification into the conduction system may have led to CHB. This is the first report of an adult patient with ALCAPA presenting with CHB.     

Electrocardiographic and Echocardiographic Features That Distinguish Anomalous Origin of the Left Coronary Artery from Pulmonary Artery from Idiopathic Dilated Cardiomyopathy

Many authors have reported noninvasive means of diagnosing anomalous left coronary artery from pulmonary artery (ALCAPA) and differentiating ALCAPA from idiopathic dilated cardiomyopathy (DCM). Systematic evaluation using these noninvasive diagnostic modalities is not available. To distinguish between ALCAPA and DCM using a systematic approach, we examined 23 patients with ALCAPA (age 1 month to 23 years, median 7 months) and 23 patients with DCM (age 5 days to 16 years, median 6.6 months). Standard 12-lead electrocardiograms (ECG) and 2-dimensional (2-D) and color Doppler echocardiograms were performed. A logistic regression model was applied using ALCAPA diagnosis as the dependent variable and ECG and echocardiographic findings as independent variables. A scoring system was created to establish the ALCAPA diagnosis based on results from the logistic regression. On the logistic regression, the ECG feature of QT pattern in aVL (Q wave ≥ 3 mm deep with an inverted T wave) and echocardiographic features of right coronary artery diameter to aortic annulus ratio ≥ 0.14, increased papillary muscle echogenicity, and Doppler color flow of LCA from aorta or pulmonary artery were the most significant differentiating features between the ALCAPA and DCM groups. A scoring system was developed using the previous five variables and assigning a score of 1 to each variable (−1 to Doppler color flow of LCA from aorta). The scoring system had sensitivity of 100% and specificity of 91% for ALCAPA diagnosis. Compared with previous reported diagnostic features in differentiating ALCAPA and DCM, the scoring system had a much higher specificity and positive predictive value. In conclusion, we selected the most useful ECG and echocardiographic features to differentiate between ALCAPA and DCM and created a scoring system to aid clinical diagnosis. This scoring system may be useful in evaluating children with acute congestive heart failure.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Associated with Ventricular Septal Defect and Mitral Stenosis

We report a case of a child with ventricular septal defect, mitral stenosis, and patent ductus arteriosus, who was also found to have anomalous origin of the left coronary artery from the pulmonary artery. Preoperative diagnosis allowed successful surgical correction.     

Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery Confirmed by 320-Slice Computed Tomography

Anomalous origin the left coronary artery from the pulmonary artery (ALCAPA) is an extremely rare congenital coronary abnormality that may be difficult to diagnose by echocardiography. Most patients present with a potentially fatal illness leading to sudden cardiac death during infancy. This report describes a 15-year-old girl who had 15-year history of cardiac murmur but with no clinical symptoms. Echocardiographic examination was normal, but a 320-slice computed tomographic (CT) scan showed the anomalous origin of the left coronary artery form the pulmonary artery. This case demonstrates that the 320-slice CT scan is a sensitive and reliable technique for establishing the diagnosis of ALCAPA in both symptomatic and asymptomatic patients when it cannot be visualized by echocardiography.     

Anomalous Origin of Left Pulmonary Artery from Aorta with Atrial Septal Defect

Anomalous origin of left pulmonary artery (ALPA) from aorta with atrial septal defect (ASD) is a rare congenital cardiac malformation. We report a case of ALPA from the aorta with ASD in a 1.8-kg baby. Repair was needed at an early age due to progressive congestive heart failure. Surgical correction was performed with cardiopulmonary bypass for closure of ASD and direct anastomosis of the ALPA onto the main pulmonary artery.     

Origin of the Main Pulmonary Artery from the Left Coronary Artery in Complex Pulmonary Atresia

We describe a 22-year-old woman with a history of unrepaired pulmonary atresia with ventricular septal defect. This woman was interesting in that her main pulmonary artery and right pulmonary artery arose from the left main coronary artery. She developed significant pulmonary hypertension in addition to isolation of the left pulmonary artery following ductal closure, subsequent to which the majority of her pulmonary blood flow was coronary dependent.     

Electrocardiogram of Anomalous Left Coronary Artery From the Pulmonary Artery in Infants

The electrocardiogram of an infant with anomalous left coronary artery from the pulmonary artery (ALCAPA, Bland-White-Garland syndrome) usually shows typical signs of an anterolateral myocardial infarction, manifested by abnormal Q waves in leads I, avL, V5, and V6, as well as by transient ST changes in these leads. Because 20–45 % of such patients do not show abnormal Q waves, the diagnosis should be strongly suspected if there is an abnormal R wave progression in the chest leads.     

Anomalous Origin of the Right Coronary Artery from the Left Sinus of Valsalva

A 1-month-old boy without precedent clinical signs of heart disease died unexpectedly. At autopsy, the child was found to have origin of the right coronary artery from the left sinus of Valsalva immediately leftward to the thickening of the aortic wall at the cuspal commissure and adjacent to the ostium of the left main coronary artery. The association of this coronary anomaly with otherwise unexplained prenatal or early postnatal death has only recently been appreciated as a potential cause of sudden infant death and mimic of sepsis, dysrhythmias, congestive heart failure, or metabolic storage diseases.     

Hoarseness as the Initial Clinical Presentation of Anomalous Left Coronary Artery from the Pulmonary Artery

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly that, if left untreated, will most often result in severe myocardial ischemia and significant morbidity and mortality. We report an unusual presentation of this defect in a 2-month-old infant who had an initial complaint of a “hoarse cry.” We theorize that impingement of the recurrent laryngeal nerve due to dilatation of the pulmonary artery was the most likely etiology of the patient's symptoms. This case serves as an important reminder that serious congenital heart disease may present with any number of complaints and unusual findings.