Anomalous Origin of the Left Coronary Artery from the Right Pulmonary Artery Presenting Following Relief of Left Heart Obstruction: A Distinct and Predictable Clinico-Pathological Syndrome

Introduction. Pre-operative recognition of significant abnormalities of the coronary arteries is important in a variety of congenital cardiac conditions. Failure to diagnose anomalous origin of the coronary artery from the pulmonary artery during repair of other anomalies is important because reduction in pulmonary artery pressure will reduce myocardial perfusion pressure. Patients. We report two cases of the rare association of anomalous origin of the left coronary artery from the right pulmonary artery, aortic coarctation, and mitral stenosis. Conclusions. Definitive imaging of coronary artery anatomy by echocardiography or other modalities should form a routine part of diagnostic assessment in all congenital heart disease patients but particularly those with left heart obstruction.     

Coronary Artery Fistulas: A Review of the Literature and Presentation of Two Cases of Coronary Fistulas with Drainage into the Left Atrium

We report 2 cases of infants presenting with a murmur shortly after birth and diagnosed with coronary artery fistulas with drainage into the left atrium. The first infant had a fistulous communication between the left main coronary artery and the left atrial appendage and presented with signs and symptoms of heart failure. The infant was repaired surgically in the first week of life. The second infant was asymptomatic and had a fistulous communication between the right coronary artery and the left atrium. The infant will have the fistula closed in the cardiac catheterization laboratory when the child is older. The literature on coronary artery fistulas is reviewed, and the diagnosis and management of coronary artery fistulas is discussed.     

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.     

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.     

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.     

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 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.     

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.     

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 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 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.     

Solitary Coronary Artery Fistulas: A Congenital Anomaly in Children and Adults. A Contemporary Review

Congenital solitary coronary artery fistulas (CAFs) in adults are uncommon anomalies, which by themselves may resemble the whole spectrum of cardiac presentations from asymptomatic behavior to life‐threatening and catastrophic events with syncope or shock and even sudden death. It may take decades to collect a reasonable series of patients in adults and children. From the literature between 1993 and 2004, 236 patients with CAFs were considered for evaluation. The present review is intended to assist cardiologists who are unfamiliar with congenital CAFs in adults by suggesting clues for decision making regarding diagnosis and management. Dyspnea and chest pain represented a frequent 91/128 (71%) clinical symptom in CAFs in adults while in the pediatric age group the majority were silent 105/133 (79%) and dyspnea and chest pain accounted for only 8% of the symptoms. The diagnostic modalities were mainly cardiac catheterization and coronary angiography. On the other hand, in the pediatric patients, echocardiography and coronary angiography mainly guided the diagnosis. Regarding treatment strategy in the reviewed subjects, percutaneous transluminal embolization was performed in 18% of the pediatric and in only 5% of the adult subjects. Surgical ligation (46% vs. 38%) and conservative medical strategies (36% vs. 24%) were reported in both pediatric and adult groups. Presentations of CAFs vary considerably in both groups. These differences include the diagnostic modalities, spontaneous closure, spontaneous rupture, and management. From this review, it seemed that—but it may be biased—surgical ligation remains the major mainstay for closure of CAFs in adult and pediatric populations. Recommendations are necessary for antibiotic prophylaxis and antiplatelet and/or anticoagulant therapy for prevention of endocarditis and thrombotic events in patients with CAFs associated with coronary artery dilatation or aneurysmal formation of the fistulous tract.     

Neonatal Myocardial Infarction: Case Report and Review of the Literature

Myocardial infarction in a neonate is rare. We describe the case of a full‐term male who presented with respiratory distress. A chest radiograph demonstrated cardiomegaly. An electrocardiogram revealed ST segment changes suggestive of ischemia. Cardiac enzymes were elevated and an echocardiogram revealed a regional wall motion abnormality. Cardiac catheterization was performed demonstrating occlusion of the ramus intermedius branch of the left main coronary artery. The patient decompensated, requiring extracorporeal membrane oxygenation (ECMO). The infant was able to be decannulated from ECMO support in 5 days and was ultimately discharged on hospital day 25. We review this case as well as the literature on neonatal myocardial infarction.     

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.     

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.     

肥厚型心肌病患者埋藏式心脏自动复律除颤器(ICD)植入后的长期生存率和风险

肥厚型心肌病是最常见的一种心肌病,而心脏性猝死是肥厚型心肌病患者的首位死因。埋藏式心脏自动复律除颤器（ICD）作为一种最有效的治疗手段被推荐用于改善猝死高危患者的长期生存率。但是,尽管埋藏式心脏自动复律除颤器在肥厚型心肌病患者猝死预防方面起到了不可否定的作用,但术后不良事件及进展性心力衰竭的风险居高不下,导致了部分植入ICD的患者预后改善不理想,引人深思,笔者就次做一综述。     

Single Coronary Artery with Right Ventricular Fistula: Case Report and Literature Review

We report a rare case of a 6-year-old boy who was diagnosed with coronary artery fistulae communicating with the right ventricle and a left single coronary artery. Preoperative angiography showed a dilated and tortuous single coronary artery draining into the right ventricle. Two coronary artery fistulae draining into the right ventricle were detected at operation and both of these were ligated. Postoperative angiography showed that the single coronary artery diameter was almost normalized, although the vessel was still slightly tortuous. His clinical course was uneventful. In this report, we summarize cases of coronary artery fistula with single coronary artery that have been reported in the literature as well as our case.     

Coronary Artery Embolism: Two Case Reports and a Review of the Literature

Coronary embolism (CE) is an uncommon and unique cause of acute myocardial infarction. In this report, we review 216 cases of CE including 2 new cases from our institution. The mean patient age was 52.5 years and 62% of the patients were males. Chest pain was the most common presenting symptom followed by dyspnea, and the most commonly affected vessel was the left anterior descending artery. Leading etiologies of the embolus were atrial fibrillation, septic emboli, and iatrogenic causes. Treatment approaches varied with thrombus aspiration being used in 30% of cases. In-hospital mortality rate was 36% and 13% of the cases were complicated by cerebrovascular accident. CE is a unique pathology that leads to acute myocardial infarction. It portends a high mortality rate and requires a high level of suspicion as symptoms may be misleading. Further research is needed in order to improve recognition and management and to lower associated mortality.     

Coronary Artery Dilatation in LEOPARD Syndrome. A Child Case and Literature Review

LEOPARD syndrome (LS) is a rare inherited disease with multiple somatic abnormalities. LS and Noonan syndrome (NS) share many features, including cardiovascular disorders, and PTPN11 gene mutation is commonly reported in both syndromes. We report a 10‐year‐old male patient who was diagnosed as LS based on typical phenotypes including multiple lentigines, electrocardiographic abnormalities, ocular hypertelorism and deafness. Although the most prevalent cardiovascular abnormalities in LS are pulmonary stenosis and hypertrophic cardiomyopathy, diffuse bilateral dilatation of the coronary arteries was found on angiography in addition to apical hypertrophic cardiomyopathy in the present case. The vessels showed slight increases in diameter on angiography conducted at an interval of 6 years. A literature review identified several case reports describing coronary ectasia in patients with NS as well as LS. Considering both syndromes share the mutation of PTPN11 gene, coronary arterial involvement could be related to the gene aberration and should be screened even if the patient shows no symptoms of ischemic heart disease.