Diagnosis and cardiac transplantation of a Carney syndrome-induced cardiac myxoma combined with dilated cardiomyopathy: a case report.

BACKGROUND: Carney syndrome is an uncommon autosomal disorder closely linked to mutations in the PRKAR1A gene. Skin lesions are the most pronounced feature of Carney syndrome, affecting over 80% of individuals with this condition. This syndrome is characterized by a triad of myxomas, skin pigmentation, and endocrine hyperfunction, featuring multiple endocrine neoplasms with skin and cardiac involvement. Dilated cardiomyopathy, a primary cardiomyopathy, is defined as the dilation and impaired systolic function of the left or both ventricles. Its clinical presentation varies from being asymptomatic to heart failure or sudden cardiac death, making it a leading global cause of heart failure. Currently, Dilated cardiomyopathy has an estimated prevalence of 1/2500-1/250 individuals, predominantly affecting those aged 30-40 years, with a male-to-female ratio of 3:1. This case report describes a heart failure patient with cardiac myxoma caused by Carney syndrome combined with dilated cardiomyopathy. The patient was successfully treated for heart failure by heart transplantation. CASE PRESENTATION: Herein, we report a case of heart failure due to Carney syndrome that resulted in cardiac myxoma combined with dilated cardiomyopathy. A 35-year-old male was admitted to the hospital three years ago because of sudden chest tightness and shortness of breath. Echocardiography indicated myxoma, and a combination of genetic screening and physical examination confirmed Carney syndrome with cardiac myxoma. Following symptomatic management, he was discharged. Surgical interventions were not considered at the time. However, the patient's chest tightness and shortness of breath symptoms worsened, and he returned to the hospital. A New York Heart Association grade IV heart function was confirmed, and echocardiography indicated the presence of dilated cardiomyopathy accompanied by cardiac myxoma. Ultimately, the patient's heart failure was successfully treated with heart transplantation. CONCLUSIONS: Cardiac myxoma caused by Carney syndrome combined with heart failure caused by dilated cardiomyopathy can be resolved by heart transplantation.

Renal Embolism Associated with the Atrial Myxoma: A Case Report and Literature Review.

Renal embolisms due to cardiac myxomas are extremely rare; the clinical course, treatment, and prognosis of this disease are not established. A 69-year-old Japanese woman who underwent a nephrectomy for renal cell carcinoma 3 years earlier was hospitalized with a right occipital lobe cerebral infarction. Her renal function suddenly worsened 3 days post-admission: her serum creatinine rose from 1.46 mg/dL to 6.57 mg/dL and then to 8.03 mg/dL the next day, and hemodialysis therapy was started. Abdominal computed tomography (CT) scans showed patchy non-contrasted low-density areas in the right kidney, and chest CT scans and transesophageal ultrasonography revealed a left atrial tumor. We diagnosed renal infarction due to a left atrial myxoma. Hemodialysis and anticoagulant therapy (heparin) were continued, followed by the cardiac myxoma's resection. The patient's renal function gradually improved post-surgery, and the hemodialysis was discontinued. Considering our patient and 19 other case reports of renal infarction associated with cardiac myxoma, the treatment for such a renal infarction and the outcomes differ depending on the embolus site. The poor outcome of abdominal aortic embolism requires a prompt embolectomy, whereas a branch renal artery embolism requires anticoagulation therapy to prevent thrombosis formation around the myxoma.

A surgical resection case of myxoma arising from the posterior wall of the left atrium complicated with complete atrioventricular block.

An 80-year-old female was referred to our institution due to transient right upper limb weakness. Transthoracic and transesophageal echocardiography revealed a tumor in the left atrium. The tumor was attached to the posterior wall of the left atrium near the atrioventricular node. Intraoperative pathological examination revealed that the tumor was a myxoma, and complete resection was successfully performed. However, she experienced persistent complete atrioventricular block postoperatively and required pacemaker implantation.

Acute ST-Elevation Myocardial Infarction in a Young Adult: Rare Presentation of Giant Atrial Myxoma. / Infarto com Supra-ST em Adulto Jovem: Rara Apresentação de Mixoma Atrial Gigante.

Cardiac tumors are rare entities, among which atrial myxoma (AM) stands as the most frequent, accounting for approximately half of all reported cases. The incidence of AM is estimated to range from 0.001% to 0.3% within the general population, yet only about 0.06% of these cases present with coronary embolic events. We report on a 33-year-old male smoker who experienced acute, severe precordial pain radiating to the left upper limb, lasting for one hour. The electrocardiographic evaluation demonstrated ST-segment elevation in leads D2, D3, and aVF, alongside significantly elevated serum troponin levels, confirming a diagnosis of ST-segment elevation myocardial infarction (STEMI). Subsequent coronary angiography revealed proximal occlusion of the right coronary artery due to thrombus. An initial attempt of thrombus aspiration was unsuccessful, followed by primary angioplasty with balloon inflation without stent placement. Further diagnostic exploration through transthoracic echocardiography identified a homogenous, smooth-surfaced mass measuring 5.2 cm x 2.3 cm attached to the interatrial septum. This mass, characterized by lobulations, prolapsed into the mitral valve and left ventricle during diastole, consistent with AM. Surgical resection of the mass was successfully performed, with the patient being discharged asymptomatic. In the reported case, the patient's profile, notably his age, and gender, diverges from the typical epidemiological characteristics associated with AM. This case adds to the limited number of reports where the inferior wall is affected by the right coronary artery being occluded. This report emphasizes the significance of differential diagnoses in younger patients presenting with STEMI.

Neoplasias cardíacas são raras, tendo como principal representante o mixoma atrial (MA), que corresponde a cerca de metade de todos os casos. O MA tem incidência estimada entre 0.001% e 0.3% na população em geral, no entanto apenas aproximadamente 0,06% desses cursam com eventos embólicos coronarianos. Homem de 33 anos, tabagista, admitido com quadro de precordialgia intensa e irradiação para membro superior esquerdo com duração de uma hora. O eletrocardiograma evidenciou elevação de segmento ST nas derivações D2, D3 e aVF troponina sérica elevada, confirmando infarto com supra desnivelamento do segmento ST (IAMCSST). Foi realizada cineangiocoronariografia, a qual revelou oclusão em terço proximal de artéria coronária direita por trombo. Realizada tentativa de aspiração do trombo, sem sucesso, seguido por angioplastia primária com balão sem colocação de stent. Durante a investigação do quadro, paciente realizou ecocardiograma transtorácico o qual demonstrou massa homogênea de superfície regular, de 5.2 cm x 2.3 cm, aderida ao septo interatrial, com lobulações de características emboligênicas prolapsando para valva mitral e ventrículo esquerdo na diástole, compatível com MA. Foi realizada ressecção cirúrgica com paciente evoluindo assintomático, recebendo alta para seguimento ambulatorial. O caso relatado difere em idade e sexo do perfil epidemiológico típico sendo um dos poucos descritos com acometimento da parede inferior apresentando a artéria coronária direita como culpada. Este relato ratifica a importância do diagnóstico diferencial frente às apresentações de IAMCSST em jovens.

All that Doesn't Enhance Isn't a Thrombus: Pitfalls Using Cardiac MRI TI 600 Sequence to Distinguish Between Cardiac Thrombus Versus Myxoma.

A 51-year-old male with a complicated medical history presented with shortness of breath. Preoperative workup confirmed the presence of a large atrial mass. However, delayed gadolinium enhancement CMR with long inversion time (TI 600) showed lack of enhancement, which was suggestive of a thrombus. During cardiac magnetic resonance imaging, delayed gadolinium enhancement sequences with long inversion time (TI 600) are commonly used to distinguish between an avascular thrombus versus a vascular tumor.

Dizzy spells in pregnancy: successful antenatal removal of a huge left atrial myxoma with mitral valve obstruction.

Diagnosing atrial myxoma in pregnancy is challenging because patients may present with non-specific symptoms that might be overlooked. The timing of non-obstetric operation usually depends on the nature of the disease, after careful consideration of feto-maternal safety, including the use of cardiopulmonary bypass and placental transfer of anaesthetic drug. A woman in her 30s at 18 weeks of pregnancy presented with recurring dizziness. She underwent successful myxoma excision at 20 weeks under general anaesthesia and cardiopulmonary bypass. The 6×5 cm myxoma was histologically confirmed as myxoma. Early detection of atrial myxoma in pregnancy is crucial, and a clinician has to consider the diagnosis of left atrial myxoma with mitral valve obstruction as a cause of severe dizziness. Optimal outcomes require multidisciplinary management. In this case, surgery during the second trimester of pregnancy enabled a full-term pregnancy with the patient's and foetal well-being and normal postprocedural echocardiography.

Left atrial myxoma with cardiogenic shock following a myocardial infarction: a case report.

BACKGROUND: Myxomas are the most common primary cardiac tumor and typically originate in the left atrium. Atrial myxomas may present following complications of obstruction and emboli. If an atrial myxoma goes untreated, complications such as congestive heart failure, embolic stroke, and sudden death can occur. CASE PRESENTATION: A 58-year-old Caucasian male presented following a cardiac arrest. He was taken emergently to the cardiac catheterization lab and received two drug eluting stents. Following the procedure, he was found to have a left atrial mass that was intermittently obstructing the mitral valve on echocardiography. After leaving the cardiac catheterization lab, he was hypotensive and placed on multiple intravenous medications for hemodynamic support as well as an Impella device. Following medical optimization, he underwent one vessel coronary artery bypass graft as well as surgical excision of the left atrial mass, which pathology had shown to be an atrial myxoma. CONCLUSION: This patient's case of cardiogenic shock following revascularization was complicated by the identification of an atrial myxoma, which, when large enough, can obstruct blood flow through the mitral valve leading to acute mitral dynamic stenosis. This condition results in circulatory collapse due to obstruction of the left ventricle in diastole as the myxoma occludes the mitral valve.

Simultaneous surgical repair of a cardiac myxoma causing left ventricular outflow tract obstruction and a ventricular septal defect in a small dog.

Background: Cardiac myxomas are benign tumours that can occur in any heart chamber or valve. They are extremely rare in dogs. We present a novel case involving a cardiac myxoma in the left ventricular outflow tract (LVOT) and a ventricular septal defect (VSD) in a small dog. Case Description: A female miniature dachshund (age, 7 months; weight, 2.88 kg) presented with growth insufficiency, lethargy, and a cardiac murmur. Echocardiography revealed a small polypoid mass in the LVOT and a membranous VSD. Simultaneous surgeries were performed to resect the mass (aortotomy) and close the VSD (right atriotomy) using low-flow cardiopulmonary bypass with surface-cooling hypothermia and retrograde cardioplegia. The tumour was histopathologically identified as a myxoma. The dog survived with no cardiac complications for 11 years after surgery. Conclusion: To our knowledge, this is the first report of ante-mortem diagnosis and simultaneous surgical repair of a cardiac myxoma obstructing the LVOT and a VSD in a small-breed dog. In addition to describing this complicated case, this report presents what we believe is the first reported use of retrograde cardioplegia during open-heart surgery in a small-breed dog.

Our 10-Year Experience with Atrial Myxomas: Is Concurrent Valve Intervention Really Warranted?

INTRODUCTION: Primary cardiac myxomas are rare tumors. Concurrent valvular lesion is a common finding on evaluation which is thought to be due to annular dilatation secondary to tumor movement across the valve, functional obstruction across the valve, and severe pulmonary hypertension secondary to chronic obstruction. A common belief among surgeons is that excision of myxoma leads to abatement of symptoms, and further valve intervention may not be warranted. METHODS: A 10-year retrospective descriptive study was designed to analyze patients who underwent excision of cardiac myxoma at our center. Data was analyzed regarding presenting features, echocardiographic findings of myxoma and valve morphology, intraoperative assessment, and postoperative outcome with/without valve repair/replacement in all patients. RESULTS: A total of 22 patients underwent surgery for myxoma. Six patients underwent successful mitral valve repair with ring annuloplasty, two had moderate mitral regurgitation, three had severe mitral regurgitation, and one patient had no mitral regurgitation on preoperative assessment, but moderate mitral regurgitation was found intraoperatively. Four of these patients had no residual mitral regurgitation in follow-up period while two had mild residual mitral regurgitation. One patient had severe mitral stenosis of concurrent rheumatic etiology and successfully underwent mitral valve replacement. CONCLUSION: Cardiac myxomas are rare benign tumors commonly associated with mitral valve insufficiency. Mitral valve should be assessed intraoperatively after excision of mass as preoperative assessment might often be insufficient. Concomitant mitral valve intervention might be needed with a case-specific tailored approach, and mitral valve repair with ring annuloplasty offers best surgical outcome in such cases.

Considerations and anesthetic management of a patient with giant right atrial myxoma: A case report and literature review.

BACKGROUND: Myxoma is a common type of primary cardiac tumor. However, there are few researches to illustrate challenge of safely inducing anesthesia in a patient with a giant right atrial myxoma at moderate altitude. PATIENT CONCERNS AND DIAGNOSES: A 54-year-old female patient lived in a city with an average altitude of 1932 m with scheduled surgical treatment for giant right atrial myxoma, prompting discussions on appropriate anesthesia modalities given her prolonged residence at moderate altitude. METHODS AND RESULTS: Considering the potential impact of moderate altitude on perioperative management, this study emphasizes the necessity of adequate volume preload therapy and the utility of transthoracic echocardiography or transesophageal echocardiography to prevent hemodynamic compromise. Furthermore, it highlights the unique consideration that, post-tumor removal, hypotension may not necessarily lead to decreased oxygen saturation in these patients. CONCLUSION: This case underscores the importance of avoiding hypotension, as pre-tumor resection blood pressure maintenance primarily determines blood oxygen concentration. Additionally, it sheds light on the intriguing observation that post-tumor removal hypotension may not result in decreased oxygen saturation. These findings have significant implications for the perioperative care of patients with giant right atrial myxoma at moderate altitudes.

Cerebral Embolism and MINOCA Secondary to Left Atrial Myxoma after Occlusion of Atrial Septal Defect by Amplatzer Occluder: A Case Report.

Primary heart tumors are rare, with atrial myxomas being the most common type. Atrial myxomas can lead to embolisms, heart obstruction, and systemic symptoms. Herein, we report a case of 72-year-old woman who presented with a left atrial myxoma at the atrial septal defect occluder, a new acute cerebral infarction, and MINOCA (myocardial infarction with no obstructive coronary atherosclerosis). Left atrial myxoma is a common primary cardiac tumor; however, left atrial myxomas arising after percutaneous atrial septal defect occlusion are rare. Additionally, the patient presented with a new case of multiple systemic emboli. The patient underwent surgical resection of a left atrial myxoma, occluder, and left atrium, and atrial septal repair, and was discharged with good recovery for outpatient follow-up. The possibility of a cardiac tumor, especially an atrial myxoma, which can lead to a series of complications, should be considered at the closure site after percutaneous atrial septal closure. Therefore, active surgical treatment and long-term follow-up are warranted in such cases.

Rapid expansion of a left atrial myxoma caused by acute multiple internal hemorrhages: a case report and literature review.

BACKGROUND: Left atrial myxoma is the most common benign tumor, with the growth rate remaining unknown because specific symptoms do not present until the tumor grows to a certain size. Early surgical management is performed in most cases once it is detected by physicians. Despite cardiac myxomas commonly being perceived as slow-growing tumors, rapid enlargement of myxomas has been reported. CASE PRESENTATION: A 64-year-old woman was referred to our hospital with a diagnosis of a left atrial tumor. The pointed tumor changed morphologically in a few hours, and her respiratory condition, which had been normal at admission, suddenly deteriorated. Emergent surgery was performed, and the diagnosis was myxoma with multiple intratumor massive hematomas. The patient recovered uneventfully and was discharged on postoperative day 12 without any complications. CONCLUSIONS: We report an extremely rare case of left atrial myxoma rapidly expanded due to acute multiple hemorrhages within itself. Massive internal hemorrhage alters the size, shape, and fragility of the tumor. We should recognize the potential risk of internal hemorrhage that may lead to acute deterioration of the so-called "slow-growing benign" tumors, such as myxomas.

Systematic Review and Case of Thrombectomy for Pediatric Stroke Due to Myxoma Embolism.

BACKGROUND: Stroke presentation secondary to a cardiac myxoma thromboembolism is rare in the pediatric population. Because of such rarity, the reported cases in the literature are primarily case reports. Additionally, general pediatric stroke management lacks evidence-based guidelines because of its low incidence and lack of clinical trials. In pediatric strokes identified from a cardiac myxoma, the incidence favors boys with the classical presentation of unilateral weakness and aphasia. We present a pediatric patient who presented with strokelike symptoms secondary to an intracranial embolus from a previously undiagnosed cardiac myxoma. METHODS: We performed a systematic review by searching PubMed, Google Scholar, Web of Science, and Embase databases for cases of pediatric myxoma causing stroke (n = 2431) and identified 19 reported uses of surgical management in treating pediatric patients who present with stroke symptoms secondary to a cardiac myxoma thromboembolism. RESULTS: The most common imaging modality was magnetic resonance imaging in 42% of cases, computed tomography in 36.8%, followed by computed tomography angiography in 31.6% of cases. Of these 19 children treated with procedures, 36.8% of pediatric patients aged between 4 and 14 years underwent neurosurgery (n = 7). CONCLUSIONS: We describe an urgent mechanical thrombectomy and share preoperative and postoperative images and pathology slides confirming a stroke from myxoma origin. We provide added insight in the safe use of mechanical thrombectomy as treatment for pediatric strokes secondary to a thromboembolism.

Cardiac myxomas: causes, presentations, diagnosis, and management.

Cardiac myxomas (CM) are one of the most common benign tumors which are typical in adults with a yearly incidence of 0.5-1 case per million individuals. This review article includes discussions based on existing literature on the role of interleukin interactions in the pathophysiology of cardiac myxoma which can lead to embolic complications, aneurysms, and CNS involvement. The objective of this narrative review was to study the variable clinical presentations of cardiac myxoma, its detection and diagnosis involving multiple modalities like genetic and hematological testing, echocardiography, CT, and MRI, of which transoesophageal echocardiogram shows excellent precision with a 90% to 96% accuracy in diagnosing CM. Individuals with the Carney complex are prone to such neoplasia. Cardiac myxomas are challenging to diagnose due to the ambiguity of their differential with thrombi. Myxomas can also be diagnosed by tumor markers like interleukin-6 and endothelial growth factors. The management of CM includes surgical excision like median sternotomy and robotic minimally invasive surgery. The use of robotic surgery in CM increased from 1.8% in 2012 to 15.1% in 2018. Tumor recurrences are uncommon but can occur due to inadequate surgical resection.

Rare Presentation of Postpericardiotomy Syndrome After Left Atrial Myxoma Removal.

Postpericardiotomy syndrome (PPS) is a known complication of cardiac valve surgery, but it has not been commonly reported as a postoperative complication of cardiac myxoma removal. A 78-year-old female with hypertension and atrial fibrillation presenting with angina was found to have a large left atrial myxoma (7.5 cm × 4.4 cm). The myxoma was resected; however, 1-week postoperation hemoglobin and blood pressure decreased with elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Limited transthoracic echocardiogram (TTE) showed moderate pericardial effusion, confirming the diagnosis of PPS. This case highlights the importance of monitoring patients postremoval of myxoma for symptoms of PPS.

Left atrial myxoma presenting with acute pulmonary edema and syncope in a middle-aged black African woman: a case report.

Cardiac myxoma is a very rare benign cardiac neoplasm. Its annual incidence globally is between 0.5 to 1 case per one million individuals. It has a 0.03% prevalence rate in the general population. It commonly occurs in the left atrium, but can also be located in the other heart chambers. Its clinical presentations are variable, non-specific, and can mimic various cardiovascular and systemic diseases, posing a diagnostic dilemma. Thus, a high index of suspicion with appropriate use of radiologic and laboratory diagnostic tools is essential for its accurate diagnosis and management. The diagnosis and management of a rare case of left atrial myxoma in a middle-aged African woman who presented with heart failure-like symptoms, features of acute pulmonary edema, and syncope is presented in this literature. The diagnosis was suspected following echocardiography. The tumor was surgically excised, and the diagnosis was confirmed histopathologically. The patient´s post-operative condition has been excellent.

Atrial myxoma embolization of the basilar artery presenting with a convulsive seizure: Case report.

BACKGROUND: Basilar artery occlusion (BAO) is a rare cause of convulsive seizure. Such patients who are treated for epilepsy will miss the optimal time for treatment. Atrial myxoma is a rare cause of stroke and should be surgically removed as soon as possible after diagnosis. CASE SUMMARY: We report a patient who presented with convulsions as the initial symptom and was diagnosed with BAO by computed tomographic angiography. After transthoracic echocardiogram, the cause of the disease was diagnosed as atrial myxoma. The patient recovered well after endovascular treatment and resection of the atrial myxoma. CONCLUSION: A small number of patients with BAO present with convulsive seizures. It is very important to make a timely diagnosis. Direct thrombaspiration may be the best choice for basilar artery cardioembolization, and thrombectomy for distal moderate vascular occlusion in posterior circulation is feasible. Atrial myxoma is a rare cause of cardioembolic stroke and should be resected as soon as possible to prevent further embolic complications.

Extensive systemic thrombo-embolism including intra-cardiac thrombosis mimicking an atrial myxoma in a patient with beta thalassaemia major - a case report.

BACKGROUND: Sepsis and thrombo-embolic disease are well known complications of thalassemia major. Intracardiac thrombi are however rare and can lead to diagnostic dilemmas. CASE PRESENTATION: We report the case of a 20-year-old female splenectomised thalassaemia major patient with severe iron overload, who presented with life threatening sepsis associated with a liver abscess. Discovery of a large oscillating intra cardiac lesion on 2D echocardiogram confirmed by Contrast Enhanced Computed Tomography (CECT) chest in the right atrium extending from the left hepatic vein through the inferior vena cava complicated the clinical course. After a prolonged Intensive Care Unit (ICU) stay supported with antibiotics and anticoagulation, she recovered with evidence of resolution of the intra cardiac thrombus. CONCLUSIONS: Early recognition and prompt aggressive treatment of sepsis in patients with thalassemia is essential to prevent complications. Intracardiac thrombosis is a potentially treatable cause for an intra cardiac mass in patients with thalassemia major, which should not be missed.