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.

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.

Surgical Outcomes for Children with Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery.

Objective of this study is to summarize surgical outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in a single center. The clinical data of 89 children undergoing surgical treatment in Beijing Children's Hospital from January 2007 to January 2022 were retrospectively analyzed. seven patients underwent ECMO support for acute left heart failure after operation, and 2 patients were discharged after weaning successfully. Eight patients died in the early postoperative period, all of them were infants, of which 5 patients underwent ECMO support, 2 patients died of cerebral hemorrhage, 2 patients died of multiple organ dysfunction, and 4 patients died of left heart failure. Three patients died late, 3 patients were lost to follow-up, and 78 patients (96.3%) completed long-term follow-up. A logistic regression model multivariate analysis showed that postoperative moderate or severe mitral regurgitation (MR) (OR 26.948 P = 0.024) and prolonged aortic cross-clamp time (OR 1.038 P = 0.050) were independent risk factors of early mortality. Compared with the Non-MVP group (20/36), the MVP group (patients with moderate or severe MR who underwent MVP at the same time) (16/36) had more significant improvement in early postoperative LEVEF [(50.68 ± 13.85)% vs (40.50 ± 13.58)% P = 0.033] and had a lower proportion of moderate or severe MR after operation (2/16 vs 11/20 P = 0.014). Children with ALCAPA can obtain a good prognosis by reconstructing the blood supply of both coronary arteries. Mitral valvuloplasty (MVP) is more helpful in improving the prognosis of children with moderate or severe MR and mitral valve structural disease. Reasonable placement of ECMO can help reduce the mortality of critically ill children after operation, but be alert to complications in the central system.

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.

Influence of age at surgery on left ventricular strain in patients with anomalous origin of left coronary artery from pulmonary artery.

OBJECTIVES: Myocardial strain abnormalities are described after surgical repair of anomalous left coronary artery from pulmonary artery (ALCAPA) even after recovery of ventricular function. The factors that predispose to the presence of these strain abnormalities in the presence of normal ventricular function are unknown. The aim of this study was to find out whether the age at repair influences the presence of global and regional strain abnormalities on follow-up. METHODS: Repaired ALCAPA patients from a single centre (n = 40) with good ventricular ejection fraction on follow-up were recruited. Baseline and follow-up data were collected from electronic records. Global and regional myocardial strain assessment was done by speckle tracking echocardiography prospectively. The association between age at repair and strain abnormalities on follow-up was analysed. RESULTS: The patients who presented earlier had significantly worse ventricular function pre-operatively compared to older patients (P < 0.0005). Global longitudinal strain was abnormal in 40% of patients with normal ventricular ejection fraction on follow-up. Presence of longitudinal strain abnormalities was more in patients who underwent repair at older age than in those who were repaired earlier (P < 0.0005). The probability of having normal longitudinal strain on follow-up was 81.6% if surgery was done before 7.8 months of age. If operated before 6 months, the odds of having normal myocardial strain was 11 times higher. Regional strain abnormalities of varying severity were present in all patients in the left and in some patients in the right coronary artery territories. CONCLUSIONS: Older age at ALCAPA repair is associated with increased incidence of myocardial strain abnormalities. Regional strain abnormalities were found in both left and right coronary artery territories.

Measures to improve in-hospital outcomes of patients undergoing surgical repair for anomalous origin of left coronary artery from pulmonary artery.

Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly leading to progressive left ventricular dysfunction and mitral regurgitation. We conducted this study to investigate various measures to optimize the outcomes of surgical correction for ALCAPA. Materials And Methods: This was a single-centre, retrospective, observational study including consecutive patients operated for ALCAPA. The main outcomes evaluated were in-hospital mortality, duration of mechanical ventilation, and duration of intensive care unit (ICU) stay. Independent sample t- test and Fisher's exact test were used for the analysis of continuous and categorical variables respectively. Results: 31 patients underwent surgical correction for ALCAPA during the study duration. The median age was 7.3 months with a range of 21 days to 25 months. All patients underwent coronary re-implantation with the coronary button transfer technique. There was no in-hospital mortality, the mean duration of mechanical ventilation and ICU stay was 117.6 hours and 10.7 days respectively. Age at admission, development of acute kidney injury after surgery, lactate levels at 12- and 24-hours post-surgery, and heart rate at ICU admission and 12-hours post-surgery were significantly associated with mechanical ventilation duration longer than 48 hours. Use of a combination of levosimendan and milrinone and elective intermittent nasal continuous positive airway pressure ventilation after extubation in all patients with severe left ventricular dysfunction were helpful in preventing low cardiac output and need for reintubation post-surgery respectively. Conclusion: Surgical correction for ALCAPA by coronary re-implantation has an excellent short-term outcome. Optimal postoperative management is of utmost importance for achieving the best results.

Anomalous origin of the left coronary artery from the pulmonary artery as a rare cause of mitral valve prolapse: a case report.

BACKGROUND: Mitral valve prolapse (MVP) is an etiologically heterogeneous disorder. Early diagnosis and prompt treatment of the underlying disease are of great significance. Herein, we present a rare case of MVP caused by anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). CASE PRESENTATION: A 22-year-old female presented with a 16-year history of anterior mitral leaflet prolapse. However, she had never experienced any discomfort before. At a routine follow-up, a transthoracic echocardiogram showed anterior mitral leaflet prolapse (A2) with moderate mitral regurgitation, and a retrograde blood flow from an extremely dilated left coronary artery (LCA). Further coronary angiography and coronary computed tomography angiography confirmed the diagnosis of ALCAPA. She subsequently underwent successful LCA reimplantation and concomitant mitral valve replacement. Intraoperatively, her mitral annulus was mildly dilated, anterior mitral valve leaflet appeared markedly thickened with rolled edges, and a chordae tendineae connecting the anterior leaflet (A2) was ruptured and markedly shortened. CONCLUSIONS: ALCAPA is a rare and potentially life-threatening congenital coronary artery anomaly that may cause mitral valve prolapse. Echocardiogram is an important screening tool for this disorder.

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.

Midterm Surgical Outcomes for ALCAPA Repair in Infants and Children.

BACKGROUND: Surgical correction of an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) has been associated with excellent survival during recent years. The purpose of this study was to evaluate the effectiveness of reimplantation of the coronary artery and to investigate the recovery of postoperative cardiac and mitral valve (MV) function. METHODS: From 2005 to 2015, 80 patients who had ALCAPA received surgical correction. Among them, 49 were infants. The median patient age was 7.8 months. Operative strategies included reimplantation of the coronary artery in 71 patients, the Takeuchi procedure in another 7 patients, and coronary artery ligation in the remaining 2 patients. RESULTS: There were 11 hospital deaths and 2 late deaths. Six patients required intraoperative or postoperative mechanical circulatory support. A significant improvement in the ejection fraction (EF) and shortening fraction (SF) was present in all surviving patients at discharge, at a 3-month follow-up and at a 1-year follow-up. MV function improved gradually after surgical repair with no late secondary intervention. CONCLUSIONS: The repair of ALCAPA can be accomplished by establishment of a dual-coronary system, which offers an acceptable mortality rate and will rarely require a second surgery. Left ventricular (LV) recovery is a progressive process, especially for infants with impaired LV function. Concomitant MV annuloplasty is safe and reliable and can be performed as necessary in patients with moderate or severe mitral valve regurgitation.

Long-term Outcomes of Children Operated on for Anomalous Left Coronary Artery From the Pulmonary Artery.

BACKGROUND: We examined the outcomes of children (<18 years) operated on for anomalous left coronary artery from the pulmonary artery (ALCAPA). METHODS: We linked patients undergoing ALCAPA repair between 1982 and 2003 in the Pediatric Cardiac Care Consortium with the National Death Index and the Organ Procurement and Transplantation Network to examine their outcomes through 2019. RESULTS: We identified 228 children (median age, 6.0 months) operated on for ALCAPA. At the time of repair, 38.6% had severe mitral regurgitation (MR), and 71.4% had severe left ventricular (LV) dysfunction. Repair included primarily coronary reimplantation in 173 and the Takeuchi procedure in 34; concurrently, 18 underwent mitral valve (MV) operation. In-hospital death occurred in 31 (13.6%) and was not associated with MR severity (P = .846); however, among patients with moderate or severe MR, risk of death was 28% lower when undergoing MV operation (P = .033). After adjustment for other risk factors, only infant operation reached statistical significance for in-hospital death (adjusted odds ratio, 12.99; 95% CI, 1.61-104.59; P = .016). Among those discharged alive with long-term data available (n = 155), the 30-year transplant-free survival reached 95.5% (95% CI, 92.3%-98.8%) and was not associated with the degree of preoperative MR or LV dysfunction. Coronary reimplantation was associated with better long-term survival compared with other surgical techniques (adjusted odds ratio, 0.11; 95% CI, 0.02-0.74; P = .023). CONCLUSIONS: Favorable long-term outcomes can be expected after coronary artery reimplantation for ALCAPA, even in patients with severe LV dysfunction at presentation. MV operation predicted decreased risk for in-hospital mortality in patients with moderate/severe MR, but MR severity predicted neither in-hospital nor longer-term outcomes.

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.

Unexplained near-drowning can reveal ALCAPA in children.

An 11-year-old girl experienced an episode of near-drowning. She was immediately rescued and was defibrillated. Transthoracic echocardiography and coronary computed tomographic angiography confirmed the diagnosis of anomalous left coronary artery from the pulmonary artery (ALCAPA). We report a rare description of this congenital coronary anomaly in a child, revealed after exercise-induced sudden cardiac arrest while swimming.

Echocardiographic Diagnosis of Anomalous Single Coronary Artery from the Pulmonary Artery: Use of Bubble Contrast Echocardiography.

Anomalous origin of a coronary artery from the pulmonary artery (PA) is a rare congenital anomaly with the most common pattern being an anomalous left coronary artery (LCA) from the pulmonary artery (ALCAPA). A very rare pattern is the anomalous origin of a single coronary artery from the pulmonary artery (ASCAPA) wherein the single coronary ostium supplies both the right and left coronary systems with profound myocardial ischemia developing once PA pressures begin to fall after birth. Previous reports of this anomaly have all been based on post-mortem findings or pre-mortem angiographic diagnosis [1-3]. Notably, these children often present in extremis due to cardiogenic shock. Institution of veno-arterial extracorporeal life support can be catastrophic as it would lead to myocardial ischemia due to PA decompression. We present here the first echocardiographic-only diagnosis of ASCAPA in a 2-month-old child with the use of bubble contrast echocardiography to help confirm the diagnosis. The patient was resuscitated from a cardiac arrest on arrival with subsequent echocardiographic-only diagnosis. Surgical repair was undertaken with administration of cardioplegia into the pulmonary root with snaring of the branch PAs, and re-implantation of the single coronary to the aorta. At 3-month follow-up, he is thriving clinically with echocardiogram showing improving-though still somewhat depressed-left ventricular function.

Bland-White-Garland syndrome with aortic stenosis.

BACKGROUND: While anatomical variations in the cardiac circulation are quite commonly encountered, isolated congenital coronary artery anomalies are rare. These conditions are only reported in 1% of patients who underwent coronary angiogram and 0.3% of patients at autopsy. CASE PRESENTATION: This is the case of a 65-year-old female who presented with a 20-day history of postexertion chest tightness and shortness of breath. Coronary computed tomography exam revealed an anomalous left coronary artery from the pulmonary artery. With surgical intervention, the patient fully recovered. CONCLUSION: Due to the low incidence of abnormal origin of coronary artery among adults, this condition is often misdiagnosed as coronary artery spasm, resulting in delayed management. Lack of timely surgical intervention may lead to a poor prognosis.