The Anterior Mitral Valve Leaflet Prolapse as a Key to Diagnosis of ALCAPA Syndrome.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) is a rare congenital coronary abnormality carrying a poor prognosis. We present a clinical case series of ALCAPA-related anterior mitral valve leaflet prolapse and discuss its mechanism as a key to suspicion of ALCAPA syndrome.

Aberrant left coronary artery from the pulmonary artery with patent ductus arteriosus - a case report and review of the literature.

BACKGROUND: Aberrant left coronary artery from pulmonary artery (ALCAPA) is a very rare congenital heart defect. Its coexistence with patent ductus arteriosus (PDA) is extremely rare. The high pressures created by the left-to-right shunt in the pulmonary arteries can delay symptoms and create a real challenge in diagnosing ALCAPA. Missing this diagnosis can have severe results, including extensive ischemia and sudden death. CASE PRESENTATION: We present a case of an infant born with a large PDA. Initially treated conservatively, however, due to congestive heart failure and lack of weight gain, she underwent surgical ligation of the PDA at the age of four and a half months. Following surgery, she developed pulmonary edema. Echocardiography revealed decreased ventricular function. ECG revealed ST elevations on lateral leads, and serum troponin was significantly increased. The patient underwent cardiac magnetic resonance imaging (MRI), which revealed signs of wall ischemia and decreased function of the left ventricle (LV) with unclear coronary anatomy. Diagnostic catheterization revealed an ALCAPA. She underwent surgical intervention, and the left coronary artery was re-implanted in the aortic sinus. Follow-up revealed slow improvement of cardiac function. DISCUSSION AND LITERATURE REVIEW: The coexistence of PDA and ALCAPA is a very rare occurrence. We found at least 10 reported cases in the literature. Delayed diagnosis might be detrimental. The prognosis of these patients is variable. CONCLUSION: An unusual post-surgical course following PDA repair requires a high index of suspicion and appropriate evaluation for ALCAPA, preferably with angiography.

A Rare Case of an Adult Pregnant Patient with the Left Coronary Artery Originating from the Pulmonary Artery: Successful Management and Healthy Maternal-Fetal Outcome.

An anomalous left coronary artery originating from the pulmonary artery (ALCAPA) refers to the abnormal origin of the left coronary artery either from the main pulmonary artery, pulmonary artery sinus, or the left and right pulmonary arteries, with the main pulmonary artery or pulmonary artery sinus being the most common sites. If not diagnosed and treated promptly, this condition can result in death within the first year of life in 90% of patients. Asymptomatic children can survive into adulthood, but they are at a high risk of sudden death. In this article, we report a case of a 24-year-old pregnant woman who was diagnosed with ALCAPA during prenatal examination. The pregnancy was successfully maintained until 36 weeks, after which a cesarean section was performed. The patient was then admitted to the cardiac surgery department to improve cardiac function, and six weeks later, a successful left coronary artery transplantation was performed. The patient was discharged and followed up for three months, during which her condition remained stable.

Outcome of surgical repair of anomalous left coronary artery from the pulmonary artery in a single-center experience.

BACKGROUND: This study aims to review the midterm results of surgical repair of anomalous left coronary artery from the pulmonary artery (ALCAPA) in our center and assess the postoperative cardiac function recovery and misdiagnosis. METHOD: Patients who underwent repair of ALCAPA between January 2005 and January 2022 at our hospital were retrospectively reviewed. RESULTS: A total of 136 patients underwent repair of ALCAPA, among which 49.3% were misdiagnosed before referral to our hospital. On multivariable logistic regression analysis, patients with low left ventricular ejection fraction (LVEF) (odds ratio = 0.975, p = 0.018) were at increased risk for misdiagnosis. The median age at surgery was 0.83 years (range, 0.08 to 56 years), and the median LVEF was 52% (range, 5% to 86%). The mortality rate was 6.6% (n = 9), and four patients underwent reintervention. The median postoperative recovery time of left ventricular function (LVF) was 10 days (1 to 692 days). Competing risk analysis revealed that a low preoperative LVEF (hazard ratio = 1.067, p < 0.001) and age younger than 1 year (hazard ratio = 0.522, p = 0.007) were risk factors for a longer postoperative recovery time of LVF. During the follow-up period, 91.9% (113/123) of the patients had no aggravation of mitral regurgitation. CONCLUSION: The perioperative and intermediate outcomes after ALCAPA repair were favorable, but preoperative misdiagnosis deserved attention, especially in patients with low LVEF. LVF normalized in most patients, but patients younger than 1 year and with low LVEF required longer recovery times.

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Associated with Right Coronary Giant Aneurysm.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital malformation, usually diagnosed in the infant period due to myocardial ischemia and heart failure, with the need for emergency surgery. Less commonly, it can be asymptomatic until adulthood. Coronary artery aneurysms are also rare anatomical anomalies with symptoms of acute or chronic angina or even remain completely asymptomatic. We present an unusual case of ALCAPA, associated with a giant aneurysm of the right coronary artery. Meeting presentation: American Association for Thoracic Surgery 102nd annual meeting, Boston MA, USA, May 16, 2022.

Acute myocardial infarction in the elderly with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA): A case report and literature review.

INTRODUCTION: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary artery malformation, with a fatality rate of 90% at 1 year of age; only 10% to 15% of patients are diagnosed in adulthood. However, elderly survivors are particularly rare. Here, we report a case of elderly ALCAPA presented with acute myocardial infarction. CASE PRESENTATION: A 64-years-old female, complained of acute precordial pain in our hospital for 2 days. She was diagnosed with an acute non-ST-segment elevation myocardial infarction. Aortic angiography revealed emptiness of the left coronary sinus, and coronary angiography showed that the tortuous right coronary artery supplied blood to the left coronary artery through collateral circulation, and the contrast medium spilled from the opening of the left coronary artery. It was suspected that the left coronary artery was opened in the pulmonary artery. This finding was subsequently confirmed by coronary artery CT. The patient refused surgery to restore double coronary circulation and was administered standardized drug treatment. There was no chest pain during the 6-month follow-up. CONCLUSION: ALCAPA should be considered in patients with Myocardial Infarction with Non-obstructive Coronary Arteries, and surgical intervention is the first choice for such patients; However, chronic myocardial damage persists regardless of surgical treatment, prophylactic implantation of an ICD may be an important means of preventing sudden cardiac death and such patients should be followed up for a lifetime.

Anomalous left coronary artery from pulmonary artery (ALCAPA) as a silent cause of mitral regurgitation in children.

Anomalous left coronary artery from pulmonary artery (ALCAPA), also known as Bland-White-Garland syndrome, is a rare cardiac disease. This condition may present with complications such as myocardial infarction, left ventricular dilatation, mitral regurgitation, and left heart failure in children. We report a case of a four-year-old boy who presented with shortness of breath, palpitations, and recurrent upper respiratory tract infections. He was diagnosed with mitral regurgitation. During the surgery, left coronary artery (LCA) was not present in its anatomical position and ALCAPA was identified. One should keep in mind the possibility of ALCAPA in presentation of mitral regurgitation in children despite not being reported in echocardiography.

Heart Failure as the Initial Presentation of Anomalous Left Coronary Artery From the Pulmonary Artery.

Coronary arteries arising from the pulmonary artery have an incidence of 0.002% in the general population. We present a 29-year-old woman who presented to our hospital with acute decompensated heart failure and atrial fibrillation with a rapid ventricular rate. She underwent a cardiac catheterization to rule out ischemic disease, which revealed retrograde contrast flow through the left coronary artery from the right coronary artery. A coronary computed tomography (CT) angiogram was pursued which showed the presence of an anomalous left coronary artery arising from the pulmonary artery (ALCAPA). For the management of her atrial fibrillation, she was electrically cardioverted. She was discharged on guideline-directed medical therapy for her heart failure, with a cardiac surgery referral for the surgical fixation of her ALCAPA.

Sudden Cardiac Arrest in an Adult with Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA): Case Report.

INTRODUCTION: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary artery anomaly that carries 90% mortality in the first year of life when left untreated. The diagnosis of ALCAPA is rare in adulthood, and it includes a broad spectrum of clinical manifestations, including sudden cardiac death (SCD). CASE REPORT: We report a rare case of resuscitated sudden cardiac arrest in a 55-year-old female, who was diagnosed with ALCAPA and underwent successful surgical correction and implantable cardioverter defibrillator (ICD) implantation for secondary prevention. DISCUSSION: ALCAPA diagnosis is not confined to childhood, and it represents a rare cause of life-threatening arrhythmias and SCD in the adult population. Surgical correction is recommended, regardless of age, presence of symptoms or inducible myocardial ischemia. Multimodality imaging is crucial for diagnosis, management planning and follow up. Assessment of the risk of recurrent ventricular arrhythmias, despite full revascularization, should be performed in all adults with ALCAPA. Myocardial scar detected via late gadolinium enhancement represents a potential irreversible substrate for ventricular arrhythmias, and it provides additional information to evaluate indication of an ICD for secondary prevention.

Unique Association of Aortopulmonary Window With Anomalous Origin of Left Coronary Artery From Pulmonary Artery in an Infant: A Blessing in Disguise?

Aortopulmonary window (APW) seen in association with anomalous origin of left coronary artery from pulmonary artery (ALCAPA) is extremely uncommon. When faced with this combination, ALCAPA usually goes undetected since most of the clinical and echocardiographic features of ALCAPA, including ventricular dysfunction are absent due to co-existent pulmonary hypertension. Herein we report a 5-month-old child with a large APW in whom a preoperative computed tomography angiogram facilitated the diagnosis of ALCAPA. The case is described for its rarity and the challenges faced during management. Also, it is extremely crucial that this coronary anomaly is identified and corrected during surgery, since failure to do so results in unforeseen postoperative ventricular dysfunction.

Early Presentation of Patients with Abnormal Origin of Left Coronary Artery from the Pulmonary Artery is a Predictor of Poor Mid-term Outcomes.

Abnormal origin of left coronary artery from the pulmonary artery (ALCAPA) is one of the most common causes of myocardial ischemia and infarction in childhood. This study aimed to determine the correlation between age at clinical presentation, level of ventricular dysfunction, and post-repair outcomes. This is retrospective study from 1993 to 2018 including thirty-one patients. The study cohort was divided into two groups according to age (< 6 months, > 6 months). The significance level was set at p 0.05.The median follow-up time was 72 [24-168] months. Median age was 4.7 [2.3-16] months. Median weight was 6.2 [4.3-9] kg. There was severe left ventricular (LV) dysfunction (ejection fraction < 35%) in 64.5% of patients. Mitral regurgitation (MR) was moderate to severe in 13 patients (41.9%). Two patients (6.4%) required extracorporeal membrane oxygenation (ECMO) support before surgery and 6 (19.4%) after correction. Age < 6 months was significantly associated with severe clinical presentation, severe LV dysfunction, delayed sternal closure, prolonged respiratory mechanical support, and prolonged length of ICU stay (p = 0.024, p = 0.042, p = 0.002, p = 0.042, p = 0.022, respectively). After surgery, ejection fraction improved to a median of 57% [50.7-60.5]. MR regressed in 12 patients (92.3%). Mortality rate after surgery was 9.7%. All patients were free from reoperation at the last follow-up. Young age at diagnosis was significantly associated with a more severe clinical presentation and poorer outcomes. After re-establishment of a two-coronary circulation, both ventricular function and MR tend to normalize over time regardless of age at repair.

Remote ALCAPA Repair-Total Arterial Reconstruction Using Free Aortic Graft Technique.

A late presenting anomalous left coronary artery from pulmonary artery (ALCAPA) with remote origin may be associated with several technical caveats due to distance for coronary transfer and inadequate autologous tissues for reconstruction. A technique using full circumferential aortic wall as a free graft that is sutured as a posterior hood on an anterior pulmonary arterial flap is used to achieve reconstruction of a neo-left coronary that is tension free, with laminar flow and without the use of any prosthetic material. The technique with potential modifications described could potentially be applied to any variant of ALCAPA to achieve total arterial reconstruction to yield an optimal long-term outcome.

Diagnosis and Surgical Outcomes of Patients with Anomalous Left Coronary Artery from the Pulmonary Artery: A Single Taiwanese Medical Center Experience.

BACKGROUND: Anomalous left coronary artery from the pulmonary artery (ALCAPA), a very rare congenital cardiac anomaly, is associated with a high mortality rate among infants who are not diagnosed or treated in a timely manner. Surgical intervention with the reconstruction for a two-coronary-system circulation is the main treatment; however, there have been very few reported cases from Taiwan. In this study, we aim to describe the clinical manifestations, diagnostic methods, surgery types, and surgical outcomes in patients with ALCAPA from a single Taiwanese medical center. METHODS: We retrospectively reviewed patients diagnosed with ALCAPA who underwent surgery at our institution between January 2001 and October 2018. Clinical presentations, noninvasive and invasive study results, surgical methods, and postoperative follow-up results were assessed from medical records. Moreover, literature on this particular cardiovascular anomaly was reviewed. RESULTS: The study included 6 patients (5 children and 1 adult). The diagnosis was confirmed using cardiac catheterization and coronary angiography in four patients and only echocardiography in two patients. All patients underwent surgical correction and survived. Four patients showed improvements in left ventricular function and mitral regurgitation (MR). CONCLUSION: Early diagnosis and timely surgical intervention could avoid mortality regardless of the method of operation. ALCAPA can be definitively diagnosed using noninvasive echocardiography. Both left ventricular systolic function and mitral insufficiency could improve after the surgical intervention in pediatric patients. Repair or replacement of the mitral valve could be reserved for persistent MR complicated with congestive heart failure, particularly in patients who received the initial operation beyond infancy.

Anomalous origin of the left coronary artery from the pulmonary artery: A surgical certainty.

This study aims to assess the clinical presentation and the outcomes of a surgical correction of an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). A retrospective review was carried out on the charts of six patients admitted for ALCAPA surgery at Aga Khan University Hospital, Karachi from March 2017 to May 2018.Dyspnoea, palpitation, poor feeding, fatiguability, pallor and a murmur of mitral regurgitation were the main presenting features. The pre-operative median left ventricular ejection fraction (LVEF) was 64%. Coronary reimplantation was performed in all the patients with a mitral valve repair being done in only one patient. The mea n LV EF was 66 .3%,p ost- ope rativel y. Mitra l regurgitation (MR) improved in patients post-operatively with trace in 2 patients and mild MR in one. Surgical correction by coronary re-implantation yields favourable outcomes in ALCAPA and significantly reduces the morbidity and mortality rates associated with the disease.

Abnormal Origin of the Left Coronary Artery From the Pulmonary Artery Discovered at Age 67: What to Do?

Anomalous left coronary artery from the pulmonary artery (ALCAPA) was described by Abbott in 1908. In most cases, it is an isolated lesion, being the most common cause of myocardial ischemia in children. The associated mortality rate without intervention reaches 90% during childhood. We report the case of a 67-year-old woman, who underwent coronary angiography for investigation of atypical chest pain and was found to have ALCAPA. The patient refused surgery and has remained asymptomatic on a medical regimen.

Mid-Term Outcome for Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery.

BACKGROUND: This study is a single-centre experience with surgical intervention of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). METHODS: A retrospective review was performed of patients who underwent ALCAPA repair between November 1991 and October 2017. RESULTS: A total of 138 patients with a median age of 36 months underwent ALCAPA repair. The median follow-up period was 3.5 years. Overall, 114 (82.6%) patients received coronary reimplantation, 21 (15.2%) underwent Takeuchi repair, and three (2.1%) were treated by closure of the ALCAPA plus coronary artery bypass graft. Concomitant mitral repair was performed in 50 patients with moderate-to-severe mitral regurgitation (MR). Three (2.1%) patients died in hospital. Three (2.1%) patients needed extracorporeal membrane oxygenation (ECMO) support. Seven (5.0%) patients needed delayed sternal closure. In patients with decreased preoperative left ventricular ejection fraction (LVEF) (<50%), the LVEF significantly improved from 25% to 33% at discharge (p = 0.001). The percentage of more than moderately significant MR decreased from 36.2% to 5.2% (p = 0.02). There was no late mortality. Two patients needed reoperation because of baffle leaks after Takeuchi repair. Three patients had severe MR. All patients had normal LVEF at last follow-up. The median normalisation time of left ventricular function was 6 months. CONCLUSIONS: Short-term and mid-term outcomes of surgical treatment for ALCAPA patients were excellent. The concomitant mitral valve repair did not increase the perioperative risk but had satisfactory mid-term outcomes. Normalisation of left ventricular function is expected within the first 6 months.

Oiigen anomalo de la arteria coronaiia derecha de la arteiia pulmonar en un lactante con soplo cardiaco: A proposito de un caso / Anomalous origin of the right coronary artery from the pulmonary artery in an infant with a heart murmur: Case report

El origen anómalo de la arteria coronaria derecha de la arteria pulmonar es una entidad rara con una incidencia del 0,002 % y potencialmente grave, que puede causar muerte súbita. El diagnóstico frecuentemente es incidental, secundario a evaluaciones por soplos cardíacos a diferencia del origen anómalo de la arteria coronaria izquierda de la arteria pulmonar, reconocido por presentar isquemia miocárdica e insuficiencia cardíaca. Se reporta el caso de una niña de 6 meses evaluada por un soplo y derivada por sospecha de fístula coronaria. La evaluación mediante ecocardiograma doppler color, cateterismo cardíaco y angiotomografía mostró la presencia de una comunicación interauricular ostium secundum pequeña y origen anómalo de la arteria coronaria derecha de la arteria pulmonar. A los 11 meses, se realizó una cirugía de reimplante de la arteria coronaria derecha en la arteria aorta y el cierre de la comunicación interauricular.

The anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare entity that has an incidence of 0.002 % and can potentially cause sudden death. Unlike to the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), usually presented with myocardial ischemia and heart failure; the diagnosis of ARCAPA is often incidental during evaluation of a heart murmur. We report a case of a 6 months old female patient referred to us with a cardiac murmur and a suspicion of coronary fistula. For a proper diagnostic assessment an echocardiogram, a computed tomography angiography and a cardiac catheterization were requested. A small ostium secundum-type atrial septal defect (ASD) and an ARCAPA were revealed. At 11 months old, the patient was scheduled for corrective cardiovascular surgery. The aim of this report is to describe a low incidence and potentially fatal disease.

Anomalous origin of the right coronary artery from the pulmonary artery in an infant with a heart murmur: Case report. / Oiigen anomalo de la arteria coronaiia derecha de la arteiia pulmonar en un lactante con soplo cardiaco: A proposito de un caso.

The anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare entity that has an incidence of 0.002 % and can potentially cause sudden death. Unlike to the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), usually presented with myocardial ischemia and heart failure; the diagnosis of ARCAPA is often incidental during evaluation of a heart murmur. We report a case of a 6 months old female patient referred to us with a cardiac murmur and a suspicion of coronary fistula. For a proper diagnostic assessment an echocardiogram, a computed tomography angiography and a cardiac catheterization were requested. A small ostium secundum-type atrial septal defect (ASD) and an ARCAPA were revealed. At 11 months old, the patient was scheduled for corrective cardiovascular surgery. The aim of this report is to describe a low incidence and potentially fatal disease.

El origen anómalo de la arteria coronaria derecha de la arteria pulmonar es una entidad rara con una incidencia del 0,002 % y potencialmente grave, que puede causar muerte súbita. El diagnóstico frecuentemente es incidental, secundario a evaluaciones por soplos cardíacos a diferencia del origen anómalo de la arteria coronaria izquierda de la arteria pulmonar, reconocido por presentar isquemia miocárdica e insuficiencia cardíaca. Se reporta el caso de una niña de 6 meses evaluada por un soplo y derivada por sospecha de fístula coronaria. La evaluación mediante ecocardiograma doppler color, cateterismo cardíaco y angiotomografía mostró la presencia de una comunicación interauricular ostium secundum pequeña y origen anómalo de la arteria coronaria derecha de la arteria pulmonar. A los 11 meses, se realizó una cirugía de reimplante de la arteria coronaria derecha en la arteria aorta y el cierre de la comunicación interauricular.