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 Alagille Syndrome

Alagille syndrome is a dominantly inherited multisystem disorder involving multiple organs including the liver, heart, eyes, face, and skeleton. Congenital heart defects, the majority of which are right-sided, contribute significantly to the mortality of these patients. We report a patient with Alagille syndrome who presented with mitral valve regurgitation requiring valvuloplasty and subsequent mitral valve replacement. The patient was ultimately diagnosed with anomalous origin of the left coronary artery from the pulmonary artery and underwent successful reimplantation.     

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.     

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.     

Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery Diagnosed as an Incidental Finding

A 2‐year‐old boy was referred for evaluation of a systolic heart murmur. Two‐dimensional Doppler echocardiogram showed an abnormal flow through the interventricular septum, directed upward and toward the posterior wall of the main pulmonary artery. Left coronary angiogram showed a normal distribution of the anterior descending and circumflex arteries. The right coronary artery (RCA) was fully filled through collaterals from the left coronary system, and arising from the main pulmonary artery. Successful surgical reimplantation of the RCA was undertaken. Although uncommon, it is important to recognize the anomalous origin of the RCA arising from the pulmonary artery since it can be associated with serious adverse cardiac events.     

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.     

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.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: A Case Series and Brief Review

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiovascular defect that occurs in approximately 1/300 000 live births or 0.5% of children with congenital heart disease. The mortality of untreated ALCAPA has been estimated to range from 35% to greater than 85% in the first year of life. However, in some cases patients can survive past infancy and into adulthood and do not present with symptoms until later in life. These older patients often manifest their anomalies as congestive heart failure, malignant arrhythmias, or even sudden death. We report a series of 3 cases from our institution illustrating the various late presentations of this unusual diagnosis. We review the pathophysiology of this rare congenital anomaly and discuss some of the signs, symptoms, and diagnostic tests that can help diagnose this unusual condition in adults.     

Congenital Left Ventricular Splint in an Adult Patient with Unrepaired Anomalous Left Coronary Artery from the Pulmonary Artery

A 24‐year‐old woman presented with a recent increase in dyspnea on exertion and development of presyncope. The patient stated that she has reproducible episodes of dizziness and near fainting when she climbs a flight of stairs and activity is limited to a slow gait.     

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.     

右冠状动脉病变对左冠状动脉狭窄患者左心室功能的影响

目的　探讨右冠状动脉病变对左冠状动脉狭窄患者左心室功能的影响及其机制。方法　对比分析左冠状动脉狭窄患者在合并与不合并右冠状动脉病变时的左心室射血分数。结果　与相应部位单纯左冠状动脉狭窄患者相比 ,合并右冠状动脉病变患者左心室射血分数均呈不同程度地下降 ,其中在左前降支、左前降支 +左回旋支狭窄基础上合并右冠状动脉病变时左心室射血分数下降有统计学意义 (P <0 .0 5或 0 .0 1) ,左主干合并右冠状动脉狭窄患者下降幅度最大 ,但无统计学意义。结论　右冠状动脉病变可在单纯左冠状动脉狭窄的基础上使左心室收缩功能进一步恶化 ;当左冠状动脉狭窄部位为左前降支、左主干或左前降支 +左回旋支时 ,对左心室收缩功能影响更为严重     

Coronary Recanalization Due to Presumed Thrombosis Following Surgical Ligation of a Large Right Coronary Artery to Right Ventricle Fistula

We report angiographic findings in an infant with congestive heart failure due to a large right coronary artery to right ventricular fistula who underwent surgical ligation. Repeat catheterization 2 years later unexpectedly showed extensive thrombosis of the right coronary artery with multiple recanalized channels supplying the right coronary distribution. Review of the literature showed that this may not be an uncommon finding.     

婴儿期左冠状动脉起自肺动脉的外科治疗

本文报告两例婴儿左冠状动脉起自肺动脉的外科治疗。1例行肺动脉内隧道术，1例行冠状动脉再植术。前者存活，3年随访良好。后者死于肺动脉后壁缝合口出血。作者对本病的诊断、手术指征、手术方法的选择及继发病变的处理进行了讨论。     

Anomalous Origin of the Left Coronary Artery from the Proper Sinus with Acute Angulation and an Intramural Segment

This is the first report of a coronary artery with an anomalous origin from the proper sinus resulting in ischemic events in a child. Transthoracic echocardiogram, computed tomogram, and coronary angiogram revealed that, although the left main coronary trunk originated from the left sinus, its ostium was displaced horizontally and was located near the commissure between the left and noncoronary valve cusps. Moreover, it was associated with an acute take off angle and an intramural segment, which are known contributing features for ischemia in cases of anomalous origin of a coronary artery from the wrong sinus. Surgical intervention, involving the unroofing procedure, was employed successfully to eliminate the ischemic events. At the latest follow up, no chest pain was reported and the transthoracic echocardiogram showed no stenosis of the neo‐ostium. Even in a coronary artery that originates from the proper sinus, an abnormal ostial location could be associated with an acute takeoff angle and an intramural segment. This finding is extremely rare but entails the risk of ischemia and sudden death.     

Anomalous Left Coronary Artery from the Pulmonary Artery in a Preterm Infant: Presentation after Ligation of Ductus Arteriosus

Surgical or medical closure of a patent ductus arteriosus (PDA) is a very common practice in premature infants in the neonatal intensive care unit, but often the coronary arteries are not delineated prior to closure. In this report, a 32‐week gestational age premature infant who underwent surgical ligation of a PDA developed new‐onset left ventricular systolic dysfunction. A repeat echocardiogram noted an anomalous left coronary artery from the pulmonary artery. Successful left coronary artery reimplantation with rapid normalization of left ventricular systolic function occurred. As previously not reported, ligation of a PDA in a premature infant can unmask anomalous left coronary artery from the pulmonary artery. Surgical left coronary artery reimplantation is the mainstay of therapy and should be performed to establish normal coronary blood flow, even in a premature, low birth weight infant.     

Anomalous Left Anterior Descending Coronary Artery from the Pulmonary Artery,Unroofed Coronary Sinus,Patent Foramen Ovale,and a Persistent Left‐sided SVC in a Single Patient: A Harmonious Quartet of Defects

Unroofing of the coronary sinus without complex structural heart defects is a rare congenital defect often seen in conjunction with a persistent left‐sided superior vena cava. Anomalous origin of the left anterior descending artery from the pulmonary artery with normal origin of the left circumflex coronary artery is an even rarer congenital cardiac defect. We report a case of a 54‐year‐old woman presenting with mild dyspnea on exertion who was found on invasive and noninvasive evaluations to have a unique combination of defects—unroofed coronary sinus, persistent left‐sided superior vena cava, patent foramen ovale, and anomalous origin of the left anterior descending artery from the pulmonary artery without evidence of previous coronary ischemia.     

老年冠心病合并代谢综合征患者的冠状动脉病变特点

目的探讨老年冠心病合并代谢综合征患者的冠状动脉病变特点。方法检测54例老年冠心病合并代谢综合征患者与108例老年冠心病不合并代谢综合征患者的腰围、空腹血糖、血脂、血压及冠状动脉造影。结果合并代谢综合征组冠状动脉血管多支病变、弥漫性病变及闭塞性病变较不合并代谢综合征组多(P>0.05),且主要累及左主干和左回旋支(P>0.05);合并代谢综合征组左心室功能减退高于不合并代谢综合征组(P>0.05)。结论合并代谢综合征患者在多重心血管危险因素聚集下,冠状动脉呈多支病变且病变严重,积极干预心血管危险因素,对防止冠心病的发生、发展,改善预后有重要的意义。