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A 56-year-old man with a 5-year history of paroxysmal palpitations, which have worsened over the past year, was diagnosed with atrial fibrillation. During evaluation, transesophageal echocardiography revealed a left atrium (LA) tumoral mass attached to the atrial septal fossa ovale, with intra-tumoral blood flow and blood stream draining from the mass. Both coronary computed tomography angiography and coronary angiography demonstrated a coro-cameral fistula connection between the left circumflex artery (LCX) branch and the LA. In addition, they showed feeding arteries of the mass arising from the LCX. The patient underwent surgical resection of the LA mass and repair of the coronary artery fistula. Intraoperative exploration revealed a 1.7â cm × 1.0â cm jelly-like, brittle LA mass and confirmed a rupture of the supplying artery, leading to a coronary artery-left atrial fistula. Surgical ligation was executed to ensure complete sealing of the supplying coronary branch within the atrial septum. Histopathological examination confirmed the diagnosis of left atrial myxoma. The 6-month follow-up indicated no recurrence of the myxoma and restoration of sinus rhythm after radiofrequency ablation. In the literature, cases of a left circumflex artery branch-left atrial fistula due to rupture of the artery supplying a left atrial myxoma are rare.
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Atrial myxoma is a rare cardiac tumour that is associated with serious complications such as sudden cardiac death and stroke and warrants early surgical resection. We report a case of a 73-year-old male with an incidental diagnosis of left atrial myxoma undergoing general anaesthesia for laparoscopic subtotal gastrectomy, D2 lymphadenectomy, and Roux-en-Y gastroduodenectomy for a newly diagnosed pyloric tumour. Careful anaesthetic considerations and management need to be taken when undergoing non-cardiac surgery to mitigate the peri-operative complications of the left atrial myxoma. Collaborative management under a multidisciplinary team of anaesthetists, surgeons, cardiologists, and cardiothoracic surgeons is essential.
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Myxomas are the most common type of benign cardiac tumors, and the right atrium is the second most common location among them, with an incidence ranging from 8.6% to 20% of all cardiac myxomas. Herein, we described a rare case of right atrial myxoma originating from the inferior vena cava orifice. In this case, main pulmonary artery cannulation and moderate hypothermic circulatory arrest were helpful to obtain a clear and simple view of the operative field without blood, which led to complete resection of the tumor with safety and better outcomes with no future recurrence.
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Cardiac masses are rare and of the neoplastic group, myxomas are the most common. An elderly male with a background of multiple myeloma and previous autologous stem cell transplant presented with dyspnea and chest heaviness with a subsequent cardiac mass found. Multimodal imaging, including three-dimensional (3D) echocardiography and cardiac magnetic resonance (CMR) imaging, was utilized to guide the diagnostic process, particularly given the differential of a cardiac plasmacytoma in the setting of multiple myeloma. CMR was used to identify characteristic features of the mass and 3D echocardiography highlighted the anatomical relationships of the mass in relation to surrounding structures to complement surgical planning. The different imaging approaches and clinical decision-making were implemented to aid in definitive surgical management.
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PURPOSE: Carney complex (CNC) is a rare, autosomal dominant syndrome, most commonly caused by PRKAR1A gene mutations and characterized by pigmented skin and mucosal changes with multiple endocrine and non-endocrine tumours. This case report highlights the diagnostic challenges associated with CNC in a patient with multiple neoplasms and a complex medical history, including cortisol-producing adrenal adenoma, breast cancer, melanoma, and atrial myxoma. METHODS: We report the case of a 41-year-old woman with a medical history of left adrenalectomy for cortisol producing adenoma (2005) with no sign of adrenal insufficiency at follow-up, right mastectomy for BRCA1/2 negative carcinoma (2013) and left parotid BRAF-V600E wild-type melanoma (2019), treated with nivolumab adjuvant therapy. In August 2019, following the fifth nivolumab administration, the patient developed central hypocortisolism due to iatrogenic hypophysitis, confirmed by brain MRI and properly treated with oral hydrocortisone. Nivolumab was discontinued due to the patient's decision. In October 2020 and April 2021, the patient had ischaemic strokes, requiring systemic thrombolysis. Echocardiographic examination then revealed a left atrial mass, with histological finding of myxoma. RESULTS: Given the rarity of this neoplasm and the suspicion of a syndromic disorder, a genetic evaluation was conducted, which confirmed a PRKAR1A gene mutation and the diagnosis of Carney complex. CONCLUSION: This case illustrates the diagnostic challenges in CNC, especially in patients with multiple tumourous manifestations and a wide spectrum of life-threatening clinical presentations. It underscores the importance of a multidisciplinary approach to diagnose and manage rare diseases, improving patient outcomes through timely genetic testing and coordinated care.
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Objective: Cardiac myxomas (CMs) are the most common primary cardiac tumour in adults. They are a rare cause of peripheral embolisation and may present as acute lower limb ischaemia (ALI). A scoping review was undertaken and a case of ALI due to CM embolisation is presented in this paper. Methods: MEDLINE, Scopus, and Embase were systematically searched for studies reporting data on ALI as a presentation of CM embolisation. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews (PRISMA-ScR) was followed. Results: A healthy 26 year old female presented to the emergency department with bilateral ALI. Urgent bilateral aorto-iliac embolectomy and distal embolectomy of the left femoropopliteal axis were performed. The retrieved embolic material exhibited a yellowish appearance and jelly like consistency, and histological analysis provided a diagnosis of a myxomatous embolus. Transoesophageal echocardiography confirmed the left atrial origin of a myxomatous tumour, but the residual mass was considered too small for further excision. At a two year clinical follow up, the patient was alive and well without recurrence. Between 1989 and 2023, 59 patients with ALI due to CM embolisation were identified in the literature. An in hospital mortality rate of 12.1% (n = 7) was reported, while the in hospital complication and re-intervention rates were 34.5% (n = 20) and 27.6% (n = 16), respectively. No post-discharge deaths, complications, or re-interventions were reported; fasciotomies were the most reported (n = 10). Post-discharge follow up was reported in 22 (37.3%) patients. Mean follow up was 18.0 ± 18.8 months (range 1-120), and 86.4% of patients (n = 19) were alive and well at last follow up. Conclusion: This review and the associated case report underline that CM embolisation should be considered in healthy young patients presenting with cryptogenic ALI. Early transoesophageal echocardiography and histological analysis of the retrieved embolus are recommended to minimise misdiagnosis in these populations.
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BACKGROUND: The presence of cardiac myxoma in the right atrium (RA) is rare. There is limited knowledge regarding the clinical symptoms and diagnosis of RA myxoma. This systematic review aimed to provide a summary of the clinical and laboratory characteristics, electrocardiogram (ECG) findings, and outcome previous cases with RA myxoma. METHODS: A comprehensive search was conducted in PubMed, Web of Science, and Scopus to identify relevant studies. Inclusion criteria were case reports and case series written in English that provided sufficient data on the manifestation of RA myxoma. Descriptive statistics were used for quantitative analysis. RESULTS: The search identified 619 patients from 480 eligible studies. The patient's mean age was 45.7 ± 17.6 years, and 55.4% of cases were female. The most common clinical manifestations of RA myxoma were cardiac, systemic, and neurologic manifestations which reported in 77.0%, 34.8%, and 21.1% of cases, respectively. Besides, 11.7% of RA myxoma were asymptomatic. ECG findings revealed normal in 39.4% reported cases. The ECG abnormalities included tall or peaked P-wave, RA and LA enlargement (19.2%), abnormal T-wave (14.0%), sinus tachycardia (11.8%), and incomplete or complete RBBB (11.2%). Echocardiography remained the diagnostic method in a majority of the cases. The mortality rate of RA myxoma was low (9.2%) during the follow-up. CONCLUSIONS: This systematic review provides a comprehensive summary of the clinical and laboratory manifestations and outcomes of RA myxoma, contributing to the existing knowledge on this rare cardiac tumor.
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Atrial Myxoma is the most common primary benign tumour of the heart, commonly found in the left atrium. It typically presents in young females with characteristic features such as, constitutional symptoms, chest pain, and cardiac murmurs. However, atypical presentations can occur; causing a diagnostic challenge. This case report describes a 75-year-old male who visited the cardiology outpatient department of Dow Institute of Cardiology, Karachi on 18th April, 2023 with a left-sided atrial myxoma in late adulthood without typical features including constitutional symptoms, chest pain, syncope, dizziness, digital clubbing or neurologic findings. Further discussed are the diagnostic techniques used to find the tumour and the treatment strategy. This case report highlights the need for cardiologists to consider Atrial Myxoma as a potential diagnosis, even in the absence of typical symptoms, in elderly male population.
Assuntos
Átrios do Coração , Neoplasias Cardíacas , Mixoma , Humanos , Mixoma/cirurgia , Mixoma/diagnóstico , Masculino , Neoplasias Cardíacas/cirurgia , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/diagnóstico por imagem , Idoso , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/patologia , EcocardiografiaRESUMO
BACKGROUND: The occurrence of cerebral aneurysm in a case of cardiac myxoma is rare with less than 60 cases reported worldwide. The course of management is still debatable given its rarity. We present a case of multiple intracranial aneurysms secondary to atrial myxoma in a young lady with a brief review of the literature. Case presentation A young lady in her late 30s with a history of right middle cerebral artery territory stroke eight years ago presented with gradually progressive symptoms in the form of holocranial headache, inattention and forgetfulness for the last few years. On neuroimaging, she was found to have multi-territorial lacunar infarcts and multiple intracranial artery aneurysms which was confirmed with a digital subtraction angiogram. A cardiac evaluation revealed a left atrial myxoma. The aetiology of subcortical cognitive decline and intracranial aneurysms was attributed to the myxoma with secondary myxomatous embolism. Other secondary causes were ruled out. She is being followed up medically after resection of the myxoma. CONCLUSION: Intracranial aneurysms are rare complications of cardiac myxoma which may present before, concurrent or many years after diagnosis of the myxoma. Nonspecific neurological complaints occasionally are the ominous signs of intracranial aneurysms which mandate a low threshold for neuroimaging in a case of cardiac myxoma. Given the absence of definitive risk factors and unclear natural history, clinical and radiological follow-ups are critical. Learning Points Intracranial aneurysms are rare complications of cardiac myxoma that may present before, concurrent or many years after diagnosis of the myxoma. Special attention must be given to nonspecific neurological complaints with a low threshold for neuroimaging in those with a prior history of cardiac myxoma. Given the absence of definitive risk factors and unclear natural history, clinical and radiological follow-up including conventional angiography and/or magnetic resonance imaging is critical.
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Cardiac myxomas are the most common benign tumors of the heart, with clinical manifestations varying significantly based on tumor size. Symptoms can range from asymptomatic and mild non-specific presentations to severe obstructive cardiac and systemic findings. This case report describes a 68-year-old female patient who presented with acute decompensated heart failure. Diagnostic evaluation revealed a left atrial myxoma causing significant mitral valve obstruction. The patient underwent emergency cardiac surgery for tumor removal, complicated by severe mitral and tricuspid valve regurgitation. Following valve replacement and repair, the patient required extracorporeal life support. Despite these complexities, she achieved significant recovery and was discharged in good condition. At follow-up, she remained asymptomatic with no signs of cardiac decompensation. This case highlights the importance of considering cardiac myxoma as a differential diagnosis in such cases to prevent potential complications.
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To date, no significant association has been reported between atrial septal defects (ASD) and cardiac myxomas. This study reports a 56-year-old woman with cardiac myxoma following transcatheter closure of ASD. She presented with a 3-month history of recurrent dizziness, vertigo, palpitations, and generalized weakness after undergoing ASD occlusion a year earlier. Echocardiography and cardiac computed tomography scans identified a large, mobile mass (7.2 cm × 2.8 cm) in the left atrium, protruding through the mitral valve. The patient underwent median sternotomy and pericardiotomy, and the histopathological examination confirmed the diagnosis of atrial myxoma. The current case illustrates the challenges in determining whether an atrial mass is a benign myxoma or a dangerous thrombus. While there is no definitive link between the implantation of an ASD closure device and the formation of a myxoma, the emergence of this tumor is a potential occurrence.
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Atrial myxoma is a rare primary tumour of the heart that typically arises from the left atrium. Patients typically present with obstructive symptoms such as dyspnoea, but constitutional and embolic symptoms can be seen as well. Gastrointestinal symptoms in the absence of embolisation are rarely reported in the literature. Our case presents a 55-year-old female who was found to have a large left atrial myxoma after presenting with gastrointestinal symptoms, which resolved upon resection of the tumour. This case illustrates that atrial myxomas can have an atypical presentation with gastrointestinal symptoms, which could be related to inflammation of gastric mucosa from interleukin-6 produced by the tumour cells. Careful history-taking followed by early detection and prompt treatment is important as atrial myxomas can lead to potentially devastating complications. LEARNING POINTS: Atrial myxomas are primary tumours of the heart that can present with a wide spectrum of symptoms.Early consideration and recognition of atypical presentations of atrial myxomas can be crucial in preventing serious consequences such as cardiac arrest.
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A cardiac myxoma is an authentic tumor that develops within the heart. Despite the typically benign histological characteristics, a cardiac myxoma may, on occasion, exhibit behavior reminiscent of malignant tumors. Most of these myxomas localize in the left atrium, often originating from a stalk near the foramen ovale region. The conventional presentation of cardiac myxomas includes a combination of obstruction, clot formation, and systemic symptoms, mirroring various other prevalent systemic diseases. They may manifest either spontaneously or through hereditary transmission. While familial myxomas are commonly linked to discernible genetic mutations, the precise molecular mechanisms underlying spontaneous myxomas remain somewhat enigmatic. Many individuals with myxomas may remain asymptomatic. However, should symptoms manifest, they can prove nonspecific and pose challenges in interpretation, particularly in instances of spontaneous heart myxomas. This report describes a 58-year-old female patient who presented with increasing severity of exertional dyspnea over a six-month duration. Initial differential diagnoses included common pulmonary and cardiac conditions, with a primary focus on chronic obstructive pulmonary disease and congestive heart failure. An echocardiogram revealed a large mass in the left atrium suggestive of a cardiac myxoma. Surgical resection confirmed the diagnosis. This case underscores the significance of including cardiac myxoma in differential diagnoses for progressive exertional dyspnea. Early detection and surgical intervention are crucial in mitigating potential complications like stroke, heart failure, or sudden cardiac death.
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Sudden cardiac death is a phenomenon that affects more than 200,000 individuals annually in the United States. Among sudden cardiac deaths, only a fractionally small portion is thought to be attributable to intracardiac neoplasms. Though rarely implicated as the primary cause of death, cardiac myxomas are the most common benign neoplasms of the heart and have been known to cause death due to multifactorial embolic events, fatal arrhythmias, and frank obstructive events. In this case, a healthy, asymptomatic young male was found unresponsive in his residence. Examination of the heart at autopsy revealed dilation of the right atrium and a large, hemorrhagic, irregular, and obstructing mass arising from the right atrium, consistent with cardiac myxoma. When unusual cardiac findings are discovered at autopsy, a systematic investigation of the heart, including retaining the organ for cardiac pathology consultation, is mandatory to correctly identify the cause of death.
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Cardiac myxomas are the most common benign primary heart tumors, with the majority occurring in the left atrium. Clinical manifestations are a result of constitutional, obstructive, and/or embolic events. Complications include myocardial infarction and stroke, as well as renal and limb ischemia. Our unusual case is a middle-aged female who presented with a one-week history of progressively worsening abdominal pain and was found to have a large splenic infarction on a CT scan. There was no personal or family history of autoimmune diseases or hypercoagulable states. The evaluation revealed a large left atrial myxoma confirmed on biopsy after surgical resection. Our patient's clinical presentation was relatively benign compared to the size of her mass. Although her myxoma was very large, morphologically solid, and attached to the interatrial septum, she did not have any evidence of congestive heart failure. The tumor's irregular surface and mobility likely led to splenic embolization. Hence, the differential diagnosis of splenic infarction should include left atrial myxoma.
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Atrial myxomas are benign primary cardiac tumors. They can present with nonspecific symptoms, ranging from constitutional symptoms and embolic phenomena such as transient ischemic attacks (TIAs) or strokes to sudden cardiac death. Early diagnosis may be a challenge due to the nonspecific presentation of atrial myxoma. A high degree of suspicion is needed in patients with TIA having no known cardiovascular risk factors. Although benign, if left untreated, it can lead to serious complications ranging from embolic phenomena and obstructive symptoms to sudden cardiac death. An echocardiogram is of fundamental importance in diagnosing atrial myxoma, and surgical resection is the ultimate treatment of choice. Here, we discuss a case of TIA as the initial presentation of atrial myxoma.
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Atrial myxomas are the most common form of primary benign cardiac tumors. The left atrium is typically the most common location while right atrial myxomas are much rarer and only occur in about 15%-25% of all myxoma patients. Typically, left atrial myxomas have the ability to cause symptoms such as syncope. We report a case of a 67-year-old female who presented with complaints of palpitations, dizziness, and near-syncope that had been ongoing for about a year. Other causes of syncope were investigated and ruled out. A transthoracic echocardiogram (TTE) found a large 4.3 x 4.0 cm spherical mass in the right atrium which was confirmed by surgical resection and immunohistochemistry to be a myxoma. The patient's condition of syncope-like symptoms warrants elevating atrial myxomas to a higher position in the diagnostic differential.