Cardiac Fibroelastoma Research Articles

Key Points and Rare Presentations of Cardiac Fibroelastoma in Pediatrics: Review Article

Key Points and Rare Presentations of Cardiac Fibroelastoma in Pediatrics: Review Article

Cardiac Papillary Fibroelastoma

Cardiac fibroelastomas are exceedingly rare etiologies of stroke, increasingly recognized with better diagnostic modalities. Surgical excision is the only curative option; however, timing of heparinization for cardiac bypass remains a challenging decision. We present a case of a 56-year-old female, diagnosed with multiple posterior circulation strokes and a left atrial mass, who successfully underwent anticoagulation and resection in 1 week. MRI of the brain demonstrated numerous embolic-looking posterior circulation strokes. Transthoracic echo was negative for atrial pathology; a transesophageal echo revealed a left atrial mass, histologically consistent with papillary fibroelastoma. Resection was delayed for up to 1 week to allow safe heparinization. We present a unique case of embolic stroke of undetermined source (ESUS) arising from a histologically rare tumor. We make the case for extensive workup for evaluation of posterior circulation strokes, including TEE, as well as timely resection of cardiac tumors.

Fishing an anemone in the brain: embolized cardiac fibroelastoma revealed after stroke thrombectomy.

Fishing an anemone in the brain: embolized cardiac fibroelastoma revealed after stroke thrombectomy.

The importance of early completion of cardiac investigations after ischaemic stroke: a case and systematic review of reperfusion therapy in stroke due to cardiac fibroelastoma

We describe a rare case of a 69-year-old woman presenting with sudden onset right-sided weakness, sensory disturbance and dysarthria due to a left-middle cerebral artery territory stroke. The patient was successfully thrombolysed, but shortly developed a recurrent stroke in a different vascular

Early surgical intervention for unusually located cardiac fibroelastomas

Papillary fibroelastomas are the second most common primary cardiac tumor in adults. Over 80% of fibroelastomas occur on the cardiac valves, usually on the left side of the heart, while the remaining lesions are typically scattered throughout the atria and ventricles. Although the optimal timing for surgery is controversial and depends on tumor size and location, prompt surgical resection is warranted in patients at high risk of embolism. A tumor on the cardiac valve can be removed using the slicing excision technique without leaflet injury. Here we present two cases of papillary fibroelastomas occurring on the ventricular surface of the aortic valve and in the right ventricle.

A Case Report about Cardiac Fibroelastoma Presenting with Stroke in a Young Patient

Primary cardiac tumors are rare and most of them are benign masses originating from valvular endocardium. The most common cardiac tumors are mixomas. Cardiac fibroelastomas which constitute 10% of all cardiac tumors are the second most common neoplasms [1]. Papillary fibroelastomas (PFE) are usually seen in the left side of the heart and in valvular structures. The most often affected valvular structure is non-coronary leaflet of the aortic valve [2]. They can cause different clinical conditions such as acute strokes without an obvious risk factor, arrhythmias and heart failure[3]. We present a case report about a patient with a fibroelastoma in chordae tendinea of anterior leaflet of mitral valve. He was applied to emergency department with acute stroke. At the end of his acute treatment, he was consulted to cardiology for etiological research.

Concomitant tumourous lesions in the left atrium and aortic valve suspected to be myxoma.

The occurrence of multiple primary cardiac tumours in a single patient is extremely rare. Despite growing expertise in non-invasive diagnostic modalities, especially in transoesophageal echocardiography, only a few cases report the coincidence of papillary fibroelastoma of the aortic valve and myxoma of the left atrium. Moreover, echocardiographic as well as a macroscopic distinction between cardiac myxoma and cardiac fibroelastoma may be difficult. Only post-excisional histopathology provides diagnostic safety. Cryoablation of the remnant tissue prevents recurrence in both the aortic valve and the left atrium.

Fibroelastoma: a rare cause of cardioembolic stroke in the young

Objectives The investigation and management of stroke in a young person remains challenging. The majority do not have traditional risk factors and the mechanism of stroke can be unclear. We present a case of a 34-year-old East Timorese woman who had major sequential strokes caused by a cardiac fibroelastoma. We reviewed the current literature on this rare condition and its cardioembolic stroke propensity. Methods We searched PubMED/OVID databases with the term ‘fibroelastoma’. We evaluated the epidemiology, diagnosis, complications and treatment of fibroelastoma. Results Fibroelastoma is the second most common cardiac tumour arising from or attached to valvular structures.1 Autopsy series estimate an incidence of 0.33%.2 With advances and availability of cardiac imaging, these tumours are more readily detected even in the asymptomatic patient. Transoesophageal echocardiogram (TOE) and cardiac MRI have greater resolution in identifying the tumour, as a proportion are missed on transthoracic echocardiogram (TTE).3 Fibroelastoma has great thrombogenic tendency; systemic embolisation is high with stroke risk estimated at 30%–40% per annum3,4, making stroke the most frequently encountered presentation of fibroelastoma followed by arrhythmia, heart failure and sudden cardiac death. High risk tumour features are those that are (1) freely mobile, (2) greater than 1 cm and (3) located on the left side of heart.2 In symptomatic patients there is compelling evidence to support surgical excision of the tumour in a timely manner. In those asymptomatic, the data is unclear. However given the relatively safe surgical profile and detrimental consequences, it is advisable to operate. Anticoagulation is prudent while awaiting surgery. Conclusions Fibroelastoma is a rare but important cause of cardioembolic stroke. Often these tumours are missed on routine TTE. A high index of suspicion is needed and young patients with cardioembolic strokes should be considered for TOE and cardiac MRI. Treatment with surgical resection is warranted to reduce future risk of stroke.

大動脈弁三尖より発生した心臓乳頭状線維弾性腫に対する手術経験

大動脈弁三尖より発生した心臓乳頭状線維弾性腫に対する手術経験

Des conséquences insolites d’une sclérothérapie à la mousse

Neurological disorders are rare complications of foam sclerotherapy. Visual disturbances and headache are the most commonly reported events and are thought to be equivalent to migraine with aura. Exceptionally, strokes have been reported. Papillary fibroelastoma is a rare cardiac tumor, which may embolize in cerebral arteries. We report the case of a patient in whom neurological disorders occurred during a session of foam sclerotherapy, and led to the discovery of a cardiac fibroelastoma.

Thrombotic thrombocytopenic purpura and cardiac papillary fibroelastoma

Thrombotic thrombocytopenic purpura (TTP), a complex thrombotic microangiopathy, remains an evolving enigma. A 49-year-old African-American woman presented with acute left hemiplegia, an ischemic cerebrovascular accident involving the right middle cerebral artery. Sequential appearance of thrombocytopenia and evidence of microangiopathic haemolysis led to the diagnosis of acquired idiopathic autoimmune TTP. This was managed with plasma exchange (PEX) and steroids. Early haematologic relapse within a month was managed with the addition of rituximab attaining sustained remission. The patient presented 3 years later with acute confusion and expressive aphasia due to multiple infarcts involving the left parieto-occipital cortex. Transoesophageal echocardiography demonstrated a pedunculated 6 mm mitral valvular mass consistent with a papillary fibroelastoma. Anticoagulation was instituted and the patient was continued on therapeutic oral warfarin. A haematologic relapse of TTP eventually emerged and was managed with PEX, steroids and rituximab. This vignette demonstrates several dilemmas in the clinical presentation, diagnosis and management of TTP in current day practice. Rituximab has adjuvant benefits to PEX and is being investigated as potential first-line therapy. Monitoring ADAMTS13 activity and inhibitor titre, as in our case, prove to have prognostic significance. Cardiac fibroelastomas are rare benign cardiac tumours usually arising from valvular endocardium with thromboembolic potential. One of the proposed mechanisms of origin of these masses is organizing thrombi in the setting of endocardial injury and inflammation questioning a possible link to thrombotic microangiopathy. To the best of our knowledge, this is the first report of this unique coexistence.

Fibroelastoma or atheromatous plaque: a case report

Cardiologia CROATICA Cardiac fibroelastoma is a rare, benign cardiac tumor. Usually, they arise from valvular endocardium and non-valvular location is rare. Although transthoracic echocardiography is sufficient for diagnosis of most cardiac tumors, small tumors may be missed. We describe an adult female patient who was clinically asymptomatic, but was submitted for the echocardiocraphic evaluation because of the high blood pressure. Transthoracic echocardiography showed a small lesion that was attached to the aortic wall near the aortic valve. In such a case, differential diagnosis consists of tumor, thrombus or atheromatous plaque and usually relies on localization of the structure and clinical presentation. In conclusion, echocardiography represents an invaluable diagnostic tool for the detection of intracardiac masses while it simultaneously provides information about their size, location, mobility and attachment site as well as the presence and extent of any consequent hemodynamic effects. However, it is almoust impossible to define tissue character using echocardiography alone, as in our case. Magnetic resonance imaging excels at making a differential diagnosis of various soft tissues and organs becuse of its high revolving power. According to the proper diagnosis the adequate treatment including conservative or surgical approach could be provided.

Acute‐onset chorea, dystonia, and cardiac fibroelastoma in a child: A paraneoplastic association?

Acute‐onset chorea, dystonia, and cardiac fibroelastoma in a child: A paraneoplastic association?

A Rare Case of Diffuse Mitral Valve Fibroelastoma

Papillary fibroelastomas are uncommon in daily cardiac surgery practice. They typically arise from the valvular endocardium, affecting the atrioventricular and semilunar valves equally [1]. Upon diagnosis, prompt resection should be undertaken because of possible devastating complications such as stroke. A young patient came to the emergency unit with partial left-sided hemiplegia. The patient’s history showed partial loss of sight with spontaneous recovery. Transesophageal echocardiography identified a solitary structure attached to the mitral valve suggestive of fibroelastoma (Fig 1). Intraoperatively, we were confronted with diffusely spread fibroelastomas on the anterior leaflet of the mitral valve (Fig 2), leaving no other option than to replace the valve. Histologic examination confirmed the diagnosis of fibroelastoma (Fig 3; hematoxylin and eosin staining). The patient’s postoperative recovery was uneventful. She was discharged on the eighth postoperative day. Cardiac fibroelastomas are rare tumors, typically involving the heart valves. The preferred therapy is to perform repair of the valve whenever possible. If the tumor has a diffuse character, this is not an acceptable option, and replacement of the valve should be performed.

An unusual case of giant cardiac fibroelastoma mimicking left atrial myxoma in a patient presenting with syncope

Cardiac papillary fibroelastomas are the most common primary valvular tumors. Generally benign, they account only for about 10% of all primary cardiac neoplasms, can occur in normal or diseased hearts, and are associated strongly with open heart surgery and radiotherapy. They are, in most cases, incidental findings, but can be discovered after syncope. We report the case of an elderly female, who was referred for syncope and was found to have a large fibroelastoma at the mitral valve annulus, intermittently obstructing the left ventricular inflow tract, and mimicking the presentation of left atrial myxoma. This case illustrates another potential mechanism of syncope in patients with fibroelastomas.

Papillary fibroelastoma of the pulmonary valve

A papillary fibroelastoma is rare, but it is the most common primary tumor of the cardiac valves. Most papillary fibroelastomas affect the left-sided heart valves, such as the aortic and mitral valves; however, they also rarely affect the pulmonary valve. Generally, surgical removal is strongly recommended to prevent its potential thromboembolic risks, especially in cases of left-sided cardiac involvement. However, there are few reports on the treatment of asymptomatic, small, right-sided cardiac fibroelastomas. We present a rare case of an asymptomatic papillary fibroelastoma occurring on the pulmonary valve, which was detected by transthoracic echocardiography, transesophageal echocardiography, and a 64-slice cardiac computed tomography scan, and which was surgically removed.

Facile conversion from mechanical to bioprosthetic composite aortic root replacement

Discussion This case describes a patient with the unusual finding of multiple cardiac fibroelastomas involving the mitral valve and the LVOT in addition to an altered aortic valve after prior valve endocarditis. We hypothesize that the local accumulation of mobile structures might be, at least in part, because of the congenital altered bicuspid aortic valve and consecutive inflammatory alteration. Furthermore, accelerated by aortic regurgitations, jet lesions in the subvalvular region of the mitral valve and the adjacent left ventricle might have contributed to the development of the tumor. After surgical replacement of the formerly infected aortic valve, tumor growth appeared to discontinue.

Rapid Development of a Papillary Fibroelastoma with Associated Thrombus: The Role of Transthoracic and Transesophageal Echocardiography.

Transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) are frequently utilized in patient's with suspected cerebral vascular ischemia. We describe a patient with suspected cerebral vascular ischemic event whom was found to have a mobile valvular mass by TTE and TEE. The lesion was unusual due to its rapid development over a period 6 months, which was documented on serial echocardiography. The mass was excised surgically and pathology showed a papillary fibroelastoma with extensive thrombus. The differential diagnosis of a cardiac valvular mass and the treatment of cardiac fibroelastomas are reviewed. In this case, both TTE and TEE were valuable in diagnosis and facilitating surgical management of a cardiac fibroelastoma.

Cardiac Papillary Fibroelastoma: A Treatable Cause of Transient Ischemic Attack and Ischemic Stroke Detected by Transesophageal Echocardiography

Transesophageal echocardiography (TEE) is used frequently in patients with cerebrovascular ischemia. On TEE, a typical appearance of a cardiac fibroelastoma is that of a pedunculated, mobile mass attached to a leaflet of a valve. Surgical excision of the lesion may lead to resolution of the symptoms and prevent further cerebrovascular ischemic events; valve replacement is seldom necessary. Herein we describe three patients with cerebral or ocular ischemia in whom histologic study confirmed a cardiac papillary fibroelastoma after initial detection by TEE. Cardiac papillary fibroelastomas should be considered in the differential diagnosis of transient ischemic attack and stroke, even in cases of recurrent events in the same vascular distribution. Although the use of echocardiography in the evaluation of stroke and transient ischemic attack is controversial, TEE must be considered in patients in whom the cause of cerebrovascular ischemia is unclear after noninvasive neurovascular studies or transthoracic echocardiography, even if the patient's cardiac history and the findings on physical examination are normal.

Cardiac Papillary Fibroelastoma: A Treatable Cause of Transient Ischemic Attack and Ischemic Stroke Detected by Transesophageal Echocardiography

Transesophageal echocardiography (TEE) is used frequently in patients with cerebrovascular ischemia. On TEE, a typical appearance of a cardiac fibroelastoma is that of a pedunculated, mobile mass attached to a leaflet of a valve. Surgical excision of the lesion may lead to resolution of the symptoms and prevent further cerebrovascular ischemic events; valve replacement is seldom necessary. Herein we describe three patients with cerebral or ocular ischemia in whom histologic study confirmed a cardiac papillary fibroelastoma after initial detection by TEE. Cardiac papillary fibroelastomas should be considered in the differential diagnosis of transient ischemic attack and stroke, even in cases of recurrent events in the same vascular distribution. Although the use of echocardiography in the evaluation of stroke and transient ischemic attack is controversial, TEE must be considered in patients in whom the cause of cerebrovascular ischemia is unclear after noninvasive neurovascular studies or transthoracic echocardiography, even if the patient's cardiac history and the findings on physical examination are normal.