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.     

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.     

Adult‐Type Anomalous Origin of the Left Coronary Artery from the Main Pulmonary Artery: One Case Report

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital defect that presents even less frequently in adults. Here we described a 40‐year‐old patient presenting with palpitations. Electrocardiography revealed frequent ventricular ectopy. Echocardiography revealed a dilated left ventricle and an abnormal flow pattern in the pulmonary artery and at the right side of the interventricular septum. Coronary angiography demonstrated an enlarged right coronary artery (RCA) with collateralization to the left coronary artery (LCA) and reflux of contrast into the pulmonary artery. Computed tomography confirmed ALCAPA syndrome. Surgical corrections were planned. (ECHOCARDIOGRAPHY, Volume **, ***********)     

Massive Pulmonary Artery Aneurysm Causing Left Main Coronary Artery Compression in the Absence of Pulmonary Hypertension

We report the case of a 62-year-old woman who presented with classic symptoms of stable angina. Cardiac images and catheterization results revealed absent pulmonary valve syndrome and compression of the left main coronary artery by a massively dilated pulmonary artery aneurysm. The patient''s anginal symptoms were relieved after pulmonary arterioplasty.Others have described proximal left main coronary artery compression in the presence of a dilated and hypertensive pulmonary artery. To our knowledge, this is the first case in which a pulmonary artery aneurysm caused left main coronary insufficiency in the absence of pulmonary hypertension—a clinically important complication of congenital pulmonary valve-related pulmonary arteriopathy.     

Anomalous Origin of the Left Main Coronary Artery from the Right Coronary Artery with a Preaortic Course

We report the case of a 51-year-old woman who presented with stable angina pectoris and Canadian Cardiovascular Society class II functional capacity. An electrocardiogram during a treadmill exercise test showed substantial ST-segment depression in the inferolateral leads. Coronary angiograms revealed an anomalous origin of the left main coronary artery from the opposite sinus of Valsalva and an interarterial course between the ascending aorta and pulmonary artery. Although this phenomenon is dangerous, the patient refused further examination. We discuss the diagnosis and treatment of patients who have an anomalous origin of a coronary artery from the opposite sinus of Valsalva.     

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.     

Cardiac magnetic resonance imaging,myocardial scar and coronary flow pattern in anomalous origin of left coronary artery from the pulmonary artery

BackgroundAnomalous origin of left coronary artery from pulmonary artery (ALCAPA) is a very rare congenital heart defect characterized by myocardial ischemia and ultimately scaring. The scar burden will determine eventual recovery of left ventricular function after corrective surgery.Material methodAll patients with proven diagnosis of ALCAPA and who underwent treatment at present centre were included. Detail echocardiography and cardiac magnetic resonance imaging (CMR) (delayed Gadolinium enhancement) was performed before and after surgery.ResultsThere were 4 patients (3 females, age group 3 months to 3 yr, follow up 6 months to 20 months.) There was no peri operative mortality. All patients had significant improvement in symptom class and LVEF (increase of more than 10%) when evaluated at last follow up. Three patients had pre operative CMR and 3 post operative CMR. All patients had improvement in post operative LVEF, but >50% was observed only in one patient who had less than half thickness delayed gadolinium enhancement. The right coronary flow pattern were unique to disease. The left coronary flow pattern were had significant variation and could predict extent of scared myocardium.ConclusionIschemia in ALCAPA can lead to myocardial scarring even in early infancy. The recovery in left ventricular function is a closely related to scar burden. Coronary flow patterns are unique and give useful insight into disease process and natural history.     

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.     

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.     

婴儿左冠状动脉异常起源于肺动脉的诊疗分析

目的 探讨左冠状动脉异常起源于肺动脉（anomalous origin of the left coronary artery from the pulmonary artery，ALCAPA）的临床特点、诊断方法、外科治疗及疗效分析。方法 2006年6月~2015年1月阜外医院诊断为ALCAPA并行手术治疗患儿29例，对这些患儿的临床资料、诊断与治疗情况及随访结果进行回顾性分析。随访结果根据包括复查结果及电话随访。结果 本组患者男性16例，女性13例，年龄2月~1（0.75±0.27）岁，体质量4.8~11.5（8.0±2.2）kg，左心室射血分数18~66(42±18)%。患儿均出现临床心功能不全症状，并通过心电图、心脏彩超及64排螺旋CT检查以明确诊断。并发二尖瓣轻度反流10例，中度反流5例，重度反流4例。手术中将左冠状动脉从肺动脉游离后，直接将左冠状动脉移植到升主动脉15例，用部分肺动脉干和主动脉壁或心包片作左冠状动脉延伸与升主动脉吻合13例，应用肺动脉内通道术（Takeuchi方法）1例。9例并发二尖瓣中度及以上反流者同期作二尖瓣成形术。术毕3例行体外膜肺氧合(extracorporeal membrane oxygenation，ECMO)辅助左心系统。无手术早期死亡。术后随访资料24例（83%，24/29），随访1~80（22±20）个月，患儿术后症状均明显改善。结论 ALCAPA的患儿应早期诊断，及早手术治疗。冠状动脉直接移植术是目前重建双冠状动脉系统的首选方案， 外科治疗效果良好。     

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.     

Percutaneous Closure of a Coronary Artery-to-Vein Graft Anastomotic Pseudoaneurysm Presenting as Acute Coronary Syndrome after Recent Coronary Artery Bypass Grafting

Pseudoaneurysm formation has been reported in degenerated coronary artery saphenous vein bypass grafts, as well as in native coronary arteries after interventional procedures or blunt trauma. In contrast, pseudoaneurysm formation arising from the anastomotic site of native coronary vessels soon after coronary artery bypass grafting is rare, and neither the clinical presentation of this phenomenon nor its treatment is well described.We present the case of a 63-year-old man, a recent coronary artery bypass grafting patient, who presented with acute coronary syndrome due to a large and expanding pseudoaneurysm of the saphenous vein-to-ramus intermedius artery graft anastomosis. After several attempts, we successfully treated the pseudoaneurysm by means of percutaneous coil embolization. To our knowledge, this is the first report of acute coronary syndrome secondary to a pseudoaneurysm at the coronary artery–saphenous vein graft anastomosis. In addition, this appears to be the first report of the percutaneous treatment of such a pseudoaneurysm by means of coil embolization.     

Echocardiographic diagnosis of anomalous origin of the left coronary artery from the pulmonary artery

The objective of this study was to analyze echocardiographic characteristics of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) and to explore the diagnostic value of transthoracic echocardiography. The echocardiographic characteristics of 8 patients hospitalized with ALCAPA from 2000 to 2005 were analyzed retrospectively, including the results of real time three-dimensional echocardiography in one case, and compared with angiographic results. Eight cases included 6 older type patients and 2 infant type patients. Echocardiography showed abnormal vessel inserting into pulmonary artery (PA), continuous shunt into PA and intercoronary collateral signals within the ventricular septum in all cases and bifurcate structure of the abnormal vessel with retrograde filling in 4 cases. The morphological and functional changes and valvular regurgitation induced by insufficient myocardial perfusion were also evaluated. In former 4 patients, 2 cases were misdiagnosed as right coronary artery-PA fistula and the other 2 cases were given an uncertain diagnosis of anomalous origin of the coronary artery because of the visualization of the echo-free linear structure which apparently arose from the aorta resembling a normal left coronary artery. The latter 4 patients were correctly diagnosed by excluding the aforementioned interference. The diagnosis of ALCAPA was confirmed by angiocardiography in all patients and by intraoperative findings in 4 patients. Based on the apprehension of ultrasonic features and the enhancement of diagnostic alertness, the echocardiography can evaluate ALCAPA accurately and give more information than angiography. It may be the first diagnostic choice.     

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.     

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.     

Management of symptomatic infants with anomalous origin of the left coronary artery from the pulmonary artery

There is disagreement concerning the optimal management of infants with congestive heart failure due to anomalous origin of the left coronary artery from the pulmonary artery. This report describes treatment in 23 such patients who had congestive heart failure before age 6 months. Eight patients had an operation in the 1st year of life and only two (25 percent) of these patients survived. Fifteen patients received initial medical treatment, and operation was delayed until an average age of 7.5 years; 13 (87 percent) of these patients survived. The two patients who survived early operation are still alive at an average age of 2.5 years. The 13 survivors in the medical group are still alive at an average age of 14.5 years. The age at onset and the severity of congestive heart failure were similar in the two groups. The frequency of the electrocardiographic pattern of myocardial infarction was similar in both groups. The radiographic cardiothoracic ratio was 0.74 in the surgical and 0.66 in the medical group. In 10 patients the left ventricular ejection fraction was less than 20 percent in the 1st year of life. In 7 of these 10 patients operation was performed before age 1 year and 2 (28 percent) survived; of the 3 patients treated medically, 1 (33 percent) survived.The outcome of surgical or medical treatment of the infant with anomalous origin of the left coronary artery from the pulmonary artery may be biased by patient selection. In the infants with depressed left ventricular function, the results of surgical and medical treatment are equally disappointing. Because coronary arterial surgery in the 1st year of life in infants with severely depressed myocardial function has an extremely high mortality rate (75 percent) and because many symptomatic infants with anomalous origin of the left coronary artery from the pulmonary artery can be treated medically and survive, we recommend delay of operative intervention until at least age 18 months.     

Late presentation of an anomalous origin of the left coronary artery from the pulmonary artery: case report and review

Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) is a rare cause of ischemia, heart failure and/or sudden death. A premortem diagnosis beyond early childhood is exceedingly rare because over 90% of untreated infants die in the first 12 months of life. We present a case of an asymptomatic fourteen-year old male with ALCAPA diagnosed by multidetector computed tomography (MDCT) angiography, who was successfully treated by surgical coronary transfer of the ALCAPA with reimplantation of the LCA to the aortic root.     

Single Coronary Ostium in Right Coronary Sinus: Previously Unreported “One for All” Configuration

We report our identification of a single coronary ostium arising from the right coronary sinus of Valsalva, in a 63-year-old woman who presented with chest pain atypical of angina. Coronary angiograms showed that the left anterior descending coronary artery arose from a right ventricular branch and that the left circumflex coronary artery arose from a right posterolateral branch. Both arteries reconstituted themselves in a backward fashion from the apex to the base of the heart—a configuration that to our knowledge has not been reported. The patient was treated conservatively and reported no chest pain 24 months later.     

Sex-Specific Biatrial Volumetric Measurements Obtained with Use of Multidetector Computed Tomography in Subjects with and without Coronary Artery Disease

Atrial volumetric measurement has proven clinical implications. Advances in cardiac imaging, notably the precision enabled by multidetector computed tomography (MDCT), herald the need for new criteria of what constitutes normal volumetric measurements. With use of 64-slice MDCT, we compared the atrial volumes in healthy individuals with those in individuals with coronary artery disease.By means of manual segmentation, we measured biatrial volume in 686 participants who underwent retrospective electrocardiographic-gated MDCT angiographic evaluation. The study population included a control group of 203 persons with no cardiac abnormalities, and a study group of 483 patients with obstructive coronary artery disease. All variables were compared between men and women and between the groups.We found a significant difference in left atrial end-systolic and end-diastolic volumes between men and women in the control group (P <0.05); however, right atrial volumes were similar. In comparison with the entire control group, the coronary artery disease group had significantly higher left atrial volume, significantly lower right atrial stroke volume, and significantly lower biatrial ejection fraction, except for left atrial ejection fraction in men. Right atrial volume and left atrial stroke volume were not significantly different. The results imply that a sex-specific reference value is necessary for left atrial volumetric evaluation, and that left atrial volume and biatrial ejection fraction (excluding left atrial ejection fraction in men) might be useful during diagnosis and prognosis in patients who have coronary artery disease.