Congenital Coronary Artery Anomalies Research Articles

Comparison between diaphragmatic-navigated and self-navigated coronary magnetic resonance angiography at 3T in pediatric patients with congenital coronary artery anomalies.

Coronary magnetic resonance angiography (CMRA) is being increasingly used in pediatric patients with congenital coronary artery anomalies (CAAs). However, the data on the free-breathing self-navigation technique, which has the potential to simplify the acquisition plan with a high success rate at 3T, remain scarce. This study investigated the clinical application value of self-navigated (sNAV) CMRA at 3T in pediatric patients with suspected CAAs and compared it to conventional diaphragmatic-navigated (dNAV) CMRA. From April 2019 to March 2022, we enrolled 65 pediatric patients (38 males and 27 females; mean age 8.5±4.4 years) with suspected CAAs in this prospective study. All patients underwent both dNAV and sNAV sequences in random order with gradient recalled echo (GRE) sequence during free breathing, with 39 (20 males and 19 females; mean age 10.2±3.6 years) of them additionally undergoing coronary computed tomography angiography (CCTA) or invasive coronary angiography (ICA). We measured and compared the success rate, scan time, visual score of the 9 main coronary artery segments, vessel sharpness, and vessel length between the two sequences. The diagnostic accuracy was compared using CCTA or ICA as a reference. The success rate of sNAV-CMRA (65/65, 100%) was higher than that of dNAV-CMRA (61/65, 93.8%) (P<0.001), and the scan time of sNAV-CMRA (7.3±2.5 min) was significantly shorter than that of dNAV-CMRA (9.1±3.6 min) (P=0.002). The acquisition efficiency of dNAV-CMRA was 40.5%±12.9%, while for sNAV-CMRA, 100% acquisition efficiency was achieved. There was no significant difference in vessel length of any of the coronary arteries, visual score, or vessel sharpness of the left circumflex coronary artery (LCX) between the two sequences (all P values >0.050). The visual score and vessel sharpness of the right coronary artery and left anterior descending coronary artery (LAD) were significantly improved in dNAV-CMRA compared with sNAV-CMRA (all P values <0.050). The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) for the detection of CAAs were not significantly different between the two sequences (all P values >0.050). Our findings demonstrated that both sNAV and dNAV in CMRA provide clinical application value in pediatric patients with CAAs and have similar diagnostic performance. Although the image quality of sNAV-CMRA is slightly inferior compared to that of dNAV-CMRA, sNAV-CMRA allows for a simpler scanning procedure.

Abstract B043: Challenges in managing cancer patients with congenital coronary artery anomalies: A case report

Abstract Introduction: Congenital coronary artery anomalies are rare cardiac abnormalities that can increase the risk of sudden cardiac death, particularly during exercise. Patients with cancer and concurrent coronary artery disease face higher mortality risks, as they often receive delayed percutaneous coronary intervention. Additionally, anthracycline chemotherapy, commonly used in cancer treatment, can cause cardiotoxicity and worsen pre-existing cardiac abnormalities. Despite the complexity of managing cancer patients with coronary artery anomalies, there is a lack of clear guidelines and consensus on optimal approaches. This report presents a unique case of a patient with a malignant inter-arterial course of the right coronary artery and newly diagnosed Human T-lymphotropic virus type I-associated adult T-cell leukemia/lymphoma, discussing the challenges and implications of chemotherapy in such cases. Case Presentation: A 38-year-old male presented with weakness and palpitations, exhibiting abnormal laboratory findings. CT angiography revealed a malignant inter-arterial course of the right coronary artery with severe narrowing. Electrocardiograms showed ST elevations and subsequent T-wave inversions. Despite the increased risk of cardiomyopathy due to the coronary anomaly, the patient required chemotherapy for life-threatening tumor lysis syndrome and refractory lactic acidosis. Anthracycline-based chemotherapy was initiated, and a follow-up echocardiogram after six months showed no acute changes. Conclusion: Managing cancer patients receiving anthracycline chemotherapy remains challenging due to limited guidelines. Anthracycline-induced cardiotoxicity is a concern, especially in patients with pre-existing cardiac anomalies like the malignant inter-arterial course of the right coronary artery. This case emphasizes the importance of recognizing and managing such anomalies in cancer patients to reduce the risk of sudden cardiac death. The higher mortality risk in cancer patients with coronary artery disease highlights the need for guidelines in managing anomalous coronary vessels in cancer patients. Further research is necessary to develop evidence-based strategies for managing cardiac complications in cancer patients, particularly those with pre-existing cardiac anomalies. Citation Format: Aliya M. Khan, Sophia Navajas Urisote, Danish A. Sheikh. Challenges in managing cancer patients with congenital coronary artery anomalies: A case report [abstract]. In: Proceedings of the 16th AACR Conference on the Science of Cancer Health Disparities in Racial/Ethnic Minorities and the Medically Underserved; 2023 Sep 29-Oct 2;Orlando, FL. Philadelphia (PA): AACR; Cancer Epidemiol Biomarkers Prev 2023;32(12 Suppl):Abstract nr B043.

Abstract B042: Chemotherapy in a patient with the malignant inter-arterial course of the right coronary artery A choice between cancer or the heart?

Abstract Introduction: Congenital coronary artery anomalies can increase the risk of sudden cardiac death, especially during exercise. Cancer patients with concurrent coronary artery disease face higher mortality rates due to delayed percutaneous coronary intervention. Furthermore, anthracycline chemotherapy, a common cancer treatment, can worsen pre-existing cardiac abnormalities. Despite the complexity of managing cancer patients with coronary artery anomalies, there is a lack of clear guidelines. This report presents a case of malignant interarterial course of the right coronary artery in a patient with Human T-lymphotropic virus type I-associated adult T-cell leukemia/lymphoma and discusses the challenges of chemotherapy in such cases. Case presentation: A 38-year-old male presented with weakness and palpitations. Laboratory findings revealed abnormalities, including elevated troponin and severe narrowing of the right coronary artery. Electrocardiograms showed ST elevations initially, followed by T-wave inversions. Although the patient had a pre-existing coronary anomaly, chemotherapy was initiated due to life-threatening tumor lysis syndrome and refractory lactic acidosis. Cardiac catheterization or surgical intervention was not possible due to comorbidities. A follow-up echocardiogram after six months of chemotherapy showed no acute changes. Discussion: Managing cancer patients with congenital coronary artery anomalies is challenging. Malignant interarterial course of the right coronary artery is particularly concerning due to the risk of sudden cardiac death. In this case, the patient's symptoms and abnormal electrocardiogram led to the identification of the coronary anomaly. However, immediate intervention was not feasible due to the patient's critical condition. The initiation of anthracycline-based chemotherapy in this patient posed a dilemma. Anthracyclines can cause cardiotoxicity, worsening pre-existing cardiac anomalies. Despite the lack of guidelines, chemotherapy was administered due to the urgent need for cancer treatment. Regular monitoring was essential to detect any cardiac changes. Conclusion: Managing cancer patients with coronary artery anomalies and administering anthracycline chemotherapy remains challenging due to limited guidelines. The case highlights the importance of recognizing and managing these anomalies to reduce the risk of sudden cardiac death. Guidelines for managing cardiac complications in cancer patients with pre-existing anomalies are needed. Further research is necessary to provide evidence-based guidance for such cases. Citation Format: Aliya M. Khan. Chemotherapy in a patient with the malignant inter-arterial course of the right coronary artery A choice between cancer or the heart? [abstract]. In: Proceedings of the 16th AACR Conference on the Science of Cancer Health Disparities in Racial/Ethnic Minorities and the Medically Underserved; 2023 Sep 29-Oct 2;Orlando, FL. Philadelphia (PA): AACR; Cancer Epidemiol Biomarkers Prev 2023;32(12 Suppl):Abstract nr B042.

Surgical strategies and outcomes for myocardial bridges coexisting with other cardiac conditions

BackgroundMyocardial bridges are congenital coronary artery anomalies. There are still many controversies surrounding surgical treatment strategies for myocardial bridges combined with other heart disorders. The purpose of this study was to evaluate the surgical treatment strategies and outcomes in patients with these conditions.MethodsBetween March 2004 and October 2021, our institution witnessed 77 patients diagnosed with myocardial bridging who underwent surgical intervention. According to the myocardial bridge and combined heart disorder, four groups were identified: 1. isolated LAD supra-arterial myotomy group, 2. LAD CABG and(or not) myotomy group, 3. LAD supra-arterial myotomy and grafting of other branches group, and 4. LAD supra-arterial myotomy and other cardiac surgery group. The perioperative outcomes, symptoms, life quality, mortality, and major adverse cardiac events (MACEs) were analyzed.ResultsThere were no deaths during hospitalization and no rethoractomy for postoperative bleeding or major adverse cardiac events (MACEs). The follow-up period ranged from 2 months to 199.2 months (55.61 ± 10.21) months, the 10-year cumulative survival rates for the four groups of patients were 95.0%, 100%, 100% and 74.1%, and the 10-year freedom rates from the MACEs were 83.9%, 92.0%, 87.5% and 76.2%, respectively.ConclusionsSupra-arterial myotomy is preferred in patients with isolated myocardial bridge, and acceptable results can be achieved by choosing supra-arterial myotomy in combination with CABG or other cardiac surgery simultaneously for patients with myocardial bridges and other heart disorders.

Narrative Review of Anomalous Origin of Coronary Arteries: Pathophysiology, Management, and Treatment.

Coronary artery anomalies (CAA) are a diverse group of congenital anomalies and are the second most common cause of sudden cardiac death in the young population after Hypertrophic Cardiomyopathy (HCM). Symptoms range from chest pain, syncope, or sudden cardiac arrest to completely asymptomatic. The prevalence of congenital coronary artery anomalies in the general population is estimated to be between 1% and 2%. CAA often gets underdiagnosed due to the lack of knowledge of the disease process. Approximately 5% of patients with acute myocardial infarction do not have atherosclerotic coronary artery disease or luminal narrowing due to other causes. Congenital coronary artery anomalies account for 50-60% of this 5% of patients. Most patients are asymptomatic for most of their lives, and chest pain is the most common symptom in symptomatic patients when referred for coronary angiography, typically when the diagnosis is typically made. The malignant coronary artery is a rare presentation of a coronary anomaly when associated with atherosclerotic coronary artery disease or valvular heart disease. Patients with symptoms of an abnormal coronary artery origin will receive medical treatment/observation, exercise restriction, coronary angioplasty with stent deployment, or surgical repair.

Co-existing cardiomyopathy in the setting of congenital coronary artery anomalies: Further insights into pathogenetic and clinical aspects.

Co-existing cardiomyopathy in the setting of congenital coronary artery anomalies: Further insights into pathogenetic and clinical aspects.

Sudden Cardiac Death in a Neonate Due to Bilateral Absence of Coronary Artery Ostium

Introduction: Congenital heart disease is a leading cause of neonatal mortality linked to birth defects. Despite the widespread availability of prenatal screenings, detection rates remain low. Accurate early detection of these lesions is pivotal to reducing neonatal morbidity and mortality. Methods: In this case, we present a neonate who experienced sudden cardiac death due to a rare, undiagnosed congenital cardiac anomaly - the bilateral absence of coronary artery ostium. Discussion: This case highlights the importance of prenatal detection of congenital cardiac anomalies. While fetal echocardiography is frequently utilized, it only identifies CHD in 36-50% of cases. This is attributed to inadequate imaging procedures, varied operator skills, and regional discrepancies. Early detection of severe CHD is essential for specialized treatment, thereby mitigating neonatal health risks and improving survival rates. Conclusion: Prenatal detection of CHD, especially coronary anomalies, continues to pose significant challenges. There is a pressing need to establish and enforce standardized protocols for fetal echocardiography aimed at these anomalies. To enhance care and improve outcomes, a joint effort between academic institutions and community centers is encouraged. Learning Objectives: • Congenital coronary artery anomalies are a significant cause of sudden cardiac death in children. • The absence of a coronary artery ostium is known to be associated with other congenital heart diseases, particularly pulmonary atresia with an intact ventricular septum. However, isolated coronary disease has also been reported in this case. • Prenatal echocardiography is a valuable tool for diagnosing congenital heart disease. However, certain limitations may be encountered when diagnosing coronary artery anomalies.

Congenital coronary artery anomalies in sports medicine. Why to know them.

The anomalous origin of a coronary artery (AOCA) is a challenging topic, due to its rarity, the complexity of the pathophysiological aspects, the clinical presentation (often silent), the difficulty of diagnosis, and the potential risk of causing acute cardiovascular events up to sudden cardiac death, particularly when triggered by heavy physical exercise or sport practice. Increasing interest in sport medical literature is being given to this topic. This paper reviews current knowledge of AOCAs in the specific context of the athletic setting addressing epidemiological and pathophysiological aspects, diagnostic work-up, sports participation, individual risk assessment, therapeutic options, and return to play decision after surgery.

The importance of color doppler echocardiography and advanced imaging in evaluation of pediatric cardiac symptoms

Congenital coronary artery anomalies (CCAs) are rare and can be very challenging to diagnose. We present 4 cases of abnormal coronary arteries origins, a child with a left main coronary artery (LMCA) from the right coronary artery (RCA) and 3 others with an RCA from the LMCA.

Sudden cardiac death in athletes – can we prevent it?

Introduction and purpose: Sudden cardiac death (SCD) is a rare condition, however it is the leading medical cause of death in population of athletes. This study reviews the epidemiology and common causes of SCD in this group. The literature on the role of pre-participation screening and of the other preventive measures was also reviewed. Review methods: A review of the available literature on sudden cardiac death in athletes was conducted using PubMed and Google Scholar databases. Description of the state of knowledge: SCD is the leading cause of death in athletes. However, the incidence is difficult to estimate and varies in different populations. The etiology is age-dependent, in young individuals the most common cause of sudden cardiac death are hypertrophic cardiomyopathy and congenital coronary artery anomalies. In the older athlete population (over 35 years old) atherosclerotic coronary artery disease is the main cause. Screening process varies between countries and besides taking the medical history and physical examination can involve electrocardiogram, echocardiography and other examinations. Conclusions: A review of the following studies has shown, that pre-participation screening of athletes may help to reduce the incidence of sudden death. Equal focus should be put on the availability of medical facilities during sports events, including experienced medical team and access to automated external defibrillation.

Evaluation of Congenital Coronary Arteries Anomalies on Cardiac Computed Tomography Angiography (CTA) for Congenital Heart Diseases

Evaluation of Congenital Coronary Arteries Anomalies on Cardiac Computed Tomography Angiography (CTA) for Congenital Heart Diseases

Prevalence and Pattern of Congenital Coronary Artery Anomalies in Patients Undergoing Coronary Angiography at a Tertiary Care Hospital

Objective: This research aimed to characterize the clinical presentation, frequency, and distribution of CAAs in people experiencing angiography. In this study, we reviewed the presence of CAAs in 4072 patients. Study design: Cross-section study Study Setting: Current research was conducted at Department of interventional cardiology, MTI- Hayat Abad Medical Complex, Peshawar from Ist July 2021 to 30th June 2022. Methods: The current research analyzed data from 4072 angiograms performed. The medical record of the patients was used to recruit the CAAs patient’s profile. Angelini's categorization was used to categorise the coronary anomalies and two interventional cardiologists reviewed each coronary angiography separately, and in the event of a discrepancy, a senior cardiologist was contacted to achieve a consensus. GE 520 and GE 2100 were used for the coronary angiogram. Operators have the option of using either a radial or femoral incision. Results: Finding of the CAAs was found in 103 cases of CAAs (a prevalence of 2.5%). According to the data, the average age of the patients was 59.64±13.73. Of them, 89 (2.19%) individuals had anomalous coronary origin and/or course, 9 (0.2%) had intrinsic coronary arterial system abnormalities, 3 (0.07%) had anomalous coronary termination, and 2 (0.04%) had a single coronary artery. Overall, 36 individuals (0.44%) were found to have an aberrant right coronary artery (RCA), with the RCA emerging from the left coronary sinus; this was followed by 54 patients (1.3%) who lacked a left main trunk and instead had a distinct origin for the LAD and LCX. Practical implication: Our understanding of CAAs has considerably improved from the development of novel diagnostic techniques, although the majority of the data available today comes from case reports and small case series. Further research is needed to fill the knowledge gap regarding the clinical effects of CAAs and their prognosis because epidemiological data are taken from studies conducted in other countries. Conclusion: Overall, 2.5 percent of research participants had congenital coronary abnormalities. The highest prevalence was found in anomalies origin and course. This study's prevalence of CAAs was comparable to that of other research, although the distribution of abnormalities was a little bit different. Keywords: Congenital Coronary Artery Anomalies, Coronary Angiography, Prevalence, Pattern

1027 CONGENITAL CORONARY ARTERY ABNORMALITIES: THE DIAGNOSTIC AND PROGNOSTIC VALUE OF CARDIAC COMPUTED TOMOGRAPHY

Abstract Introduction To the best of our knowledge, there are no previous univocal data on how CCT can accurately describe morphological variants and subtypes of congenital coronary artery abnormalities. Taking into consideration the extensive application of CCT in clinical practice in the last decades, it is not uncommon to have a congenital coronary artery anomaly detected at CCT. Thus, the study aim is to evaluate the diagnostic and prognostic impact of specific cardiac CT parameters in subjects with a diagnosis of congenital coronary artery anomalies. Material and Methods This is an intermediate evaluation of a prospective clinical registry on a population of subjects who underwent a cardiac computed tomography imaging evaluation in the period between January 2007 and October 2015 and were diagnosed to have a congenital coronary abnormality. For the present preliminary analysis only coronary anomalies of origin have been considered and 92 patients have been included. Follow-up have been collected either through a structured telephonic interview or through the evaluation of clinical records of subsequent hospitalization or ambulatory visits. Results The population enrolled in the present study has an average age of 63,0 ± 12,8 years (range 34 to 85 years), with a male prevalence of 69,6%. The left main artery is missing in 46 individuals (50%), with separate origin of LAD and LCX. Only two duplications (2.2%), one for the left coronary artery and one for the right coronary artery, have been discovered. Six participants (6.5%) had a non-coronary artery origin abnormalities and pulmonary artery was the site of origin in the majority of cases. 51 subjects (55,4%) have an anomalous origin of the coronary artery from a different coronary sinus and 50 subjects had also an anomalous course with the retro-aortic being the most common (30,4%). 11 participants (12,0%) displayed also an intramural segment and 16 (17,4%) had abnormal ostial morphology. The high take-off of the vessel was uncommon (3,3%). Age appears to be the only relevant characteristic; indeed, it displays a significant correlation both to MACE (OR 95% CI 1.03, 1.01-1.07; p=0.0349) and to all-cause of death (OR 95% CI 1.14, 1.02-1.27; p=0.0151). None of the traditional cardiovascular risk factors were found to be significantly linked with adverse outcomes in this study sample of coronary anomalies. Conclusions The result of this intermediate evaluation is that cardiac CT can be successfully used to define the anatomy and features of CAA. However, it demonstrated that in middle-aged patients, the tomographic finding of an abnormality of coronary origin might not have a meaningful, strong negative prognostic value in terms of major cardiovascular events and all-cause of death. This means that probably, in the majority of the cases, once the diagnosis is performed later on in life, no further systematic assessment is needed but a personalized approach should be suggested.

Coronary artery anomalies and variants among adult patients undergoing coronary angiography in Azadi heart center, Duhok/Iraq

Background: Congenital coronary artery anomalies are rare among general population and are usually asymptomatic. And diagnosed accidentally during coronary angiography. Methods: A total of 2177 patients underwent diagnostic coronary angiography at Azadi cardiac center in Duhok city/Iraq between January 2018 and January 2020 and their angiogram were searched for coronary anomaly. Results: Among 2177 patients who included in this study, 26 patients (16 males and 10 females) had coronary artery anomalies. The vast majority of anomalies were in the origin and distribution of coronary arteries which was seen in 22 patients (84.6 % of anomalies), while anomalies of coronary artery termination were found in only 4 patients (15.4 %). Separated origin left anterior descending and left circumflex artery was the most common anomaly that was found in 11 patients (42.3 % of anomalies). Conclusion: Congenital coronary arteries anomalies are uncommon finding in patients undergoing coronary angiography.

Two genetic variants in NEXN and ABCC6 genes found in a patient with right coronary artery to right ventricle fistula combined with giant coronary aneurysm and patent ductus arteriosus.

Coronary artery fistula, defined as communication between a coronary artery and a great vessel or a cardiac chamber, is a relatively rare anomaly with an estimated incidence of 0.002% in the general population. It could be combined with a giant coronary artery aneurysm, with an incidence of 5.9% of the total incidence rate of CAF in the general population. The pathogenesis of these two combined anomalies is not clear, and we aimed to detect whether genetic abnormalities underlie the pathogenesis of these rarely combined anomalies. A 6-year-old patient with a diagnosis of the right coronary artery to right ventricle fistula combined with a giant right coronary artery aneurysm and patent ductus arteriosus underwent a surgical repair at our center. The diagnosis was confirmed by echocardiography, CT, and surgery. DNA was extracted from the peripheral venous blood samples of the patient and his mother after informed consent was obtained. Hematoxylin and Eosin (HE) and Alizarin red staining were performed on the excised coronary artery aneurysm. Exome sequencing and in silico analyses were performed to detect detrimental genetic variants. No obvious abnormalities were found in the excised coronary artery aneurysm. A heterozygous truncated variant (NM_144573: c.G298T; p.G100X) in the NEXN gene and a missense variant (NM_001171: c.G1312A; p.V438M) in the ABCC6 gene were carried by the patient but not by his mother. The NEXN-truncated variant, NEXN-G100X, is associated with the development of coronary arteries and congenital coronary artery anomalies.

Surgical Management for Congenital Coronary Artery Anomalies during Childhood

Surgical Management for Congenital Coronary Artery Anomalies during Childhood

Abstract 10907: Woven Coronary Artery: A River With Many Currents

Introduction: Congenital coronary artery anomalies can increase the risk of cardiovascular events and sudden death. A woven coronary artery is a rare abnormality with multiple lumens in a single vessel, giving the appearance of complex coronary plaque or a dissection flap on coronary angiography. Clinical Case: A 57-year-old man with group IV pulmonary hypertension and chronic right-sided heart failure presented for consideration of pulmonary endarterectomy. He underwent preoperative coronary angiography which demonstrated nonobstructive disease within the left anterior descending and circumflex arteries and a complex lesion in the right coronary artery (RCA- Figure 1). The lesion was further evaluated using intravascular ultrasound (IVUS) which demonstrated numerous, complex, communicating lumens and fenestrations within a single external elastic lamina suggestive of a woven coronary artery anomaly (Figure 2). This was recognized as a benign abnormality for which the patient was managed medically, and he underwent a successful pulmonary endarterectomy several days later. Discussion: Woven coronary arteries are rare and complex lesions made up of numerous lumens often found in the proximal to mid-RCA. These lesions typically permit normal blood flow and are found incidentally in asymptomatic patients. Woven coronary arteries can be mistaken for complex lesions, dissection, or recanalized thrombus, leading to unnecessary and potentially dangerous percutaneous coronary intervention. Woven arteries should not be treated with angioplasty in the absence of thrombotic occlusion as this can lead to arterial vessel damage. Intracoronary imaging, including IVUS, is vital to differentiate woven coronary arteries from other coronary pathologies. Conclusion: Woven coronary arteries may mimic dissection or recanalized thrombus, but can be promptly recognized as a benign congenital anomaly with intracoronary imaging modalities such as IVUS.

UNUSUAL BEGINNINGS: A CASE OF ABERRANT CORONARY ARTERY ANATOMY

UNUSUAL BEGINNINGS: A CASE OF ABERRANT CORONARY ARTERY ANATOMY

Anomalous Aortic Origin of Coronary Artery: A Cases Series

Congenital coronary artery anomalies (CCAA) are not that common, they are usually found in 0.3 to 5.6% of people undergoing coronary angiography [1], these numbers are still to be widely confirmed since they can be biased by the fact that angiography or autopsy are usually preformed in patients with suspected cardiac disease. However, they are particularly important as they occupy the second place of most common cause of sudden death in young athlete according to a series reported by Basso et al., [2]. Definition, clinical presentation, physiopathology of symptoms, diagnostic workup and prognosis, can be challenging and varies according to different subgroups established by classifications, this last one can falls into two paths, anatomical based on the description of origin, course, termination and functional based on the degree of ischemia that can be caused by it. The management and treatment of these anomalies is still not firmly entrenched, it depends mostly on the patient, circumstances of diagnosis and proof of hemodynamic significance. In this case series of 3 patients, we will focus mainly of anomalous aortic origin of a coronary artery, encountered in our cathlab.

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 presentationA 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.ConclusionsALCAPA 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.