Cardiac Papillary Fibroelastoma Research Articles

Valvular fibroelastomas complicated by Coxiella burnetti endocarditis

Cardiac papillary fibroelastoma (CPF) is a valvular tumor that may be mistaken for infective endocarditis (IE). We describe a case of CPF complicated by Coxiella burnetti IE. According to Duke's criteria, a diagnosis of IE was repeatedly considered as excluded or established during the clinical course, highlighting the criteria limitations.

Calcified Amorphous Tumour of the Mitral Annulus Imaged With Transoesophageal Three-Dimensional Echocardiography: A Rare Cause of Systemic Embolism

A 66-year-old man, with a history of diabetes, hypertension, and smoking was diagnosed with left-eye retinal ischaemia leading to complete ocular blindness. After hospital admission no evidence of atrial fibrillation at telemetric monitoring or carotid artery disease was found. Transthoracic echocardiography was performed, which showed a 5 mm intracardiac hyperechogenic mobile mass attached to the atrial side of posterior mitral ring, suggestive of cardiac papillary fibro-elastoma. Cardiac magnetic resonance was performed, but the results were inconclusive for tissue characterisation owing to the small dimensions and high mobility of the mass.

Cardiac papillary fibroelastoma and kidney infarction

Cardiac papillary fibroelastoma and kidney infarction

DELVE FOR THE VALVE MASS: A CASE OF PULMONARY FIBROELASTOMA MIMICKING ENDOCARDITIS

Cardiac papillary fibroelastoma (PFE) are rare and benign tumors of endocardium accounting for 7% of all cardiac tumors. Aortic and mitral valve area are the most common sites of its occurrence and rarely they involve pulmonary valve (PV) area and are typically found incidentally during a open heart

Cardiac papillary fibroelastomas: A 10-year single-center surgical experience and long-term echocardiographic follow-up study.

Limited contemporary data are available on the clinical and echocardiographic outcomes after surgery for cardiac papillary fibroelastoma (CPF). The aim of this study was to review the clinical manifestations, pathological characteristics, surgical management, and prognoses of patients with histologically verified CPF, who underwent surgery at our cardiac surgery center from 2008 to 2018. Twelve patients of median age 62 years (28 to 77 years) were treated. Embolic stroke or transient ischemic attack (five patients, 42%) were the only CPF clinical manifestations. Eleven (92%) tumors were localized on the valves, with the aortic valve being the most common tumor site (seven patients; 58%). Multiple factor analysis revealed no independent predictor of CPF-related embolization. Simple shave tumor excision was sufficient in most patients (10 patients, 83%). No operative or tumor-related late mortality during the median follow-up period of 4.7 years (1.1 to 10.2 years) was recorded. Asymptomatic metachronous valve tumor recurrence (in a location different from that of the original tumor) was revealed in two patients (17%) by transesophageal echocardiography (TEE), not detected by transthoracic echocardiography (TTE). One of these two patients underwent repeated surgery for CPF but later suffered a recurrent embolic stroke, due to another tumor recurrence. CPF can be safely and effectively treated surgically. TEE is superior to the TTE option in CPF post-operative recurrence detection. There is a clear need for a prospective study to determine criteria for embolization risk stratification and optimum management in patients with CPF.

Primary cardiac neoplasms: Coexistence of papillary fibroelastomas of the tricuspid valve and myxoma of the mitral valve

Abstract Both cardiac myxomas and papillary fibroelastomas are primary benign cardiac tumors, but it is very rare for tumors with different histologies to occur in different valves of the same heart. A 5-month-old infant underwent resection of a mitral valve mass + mitral valvuloplasty at our hospital. The tricuspid valve mass was still small, and no tricuspid regurgitation was observed; therefore, the tricuspid valve mass was not treated. The postoperative pathology showed that the mitral valve mass was a myxoma. The patient presented to our hospital for a follow-up examination two years later, and color Doppler ultrasound showed enlargement of the mitral valve and tricuspid valve mass as well as aggravation of regurgitation of the two valves. We performed resection of the mitral valve mass with mechanical valve replacement + resection of the tricuspid valve mass together with tricuspid valvuloplasty for this child. Postoperative pathology showed myxoid degeneration of the mitral valve and papillary fibroelastoma/tumor-like hyperplasia of the tricuspid valve. One month after the patient underwent surgery, the follow-up examination was satisfactory.

Cardiac papillary fibroelastoma, a cause of acute embolic stroke: Report of two cases

Cardiac Papillary Fibroelastoma (PFE) is a rare benign cardiac tumor mainly clinically asymptomatic that unexpectedly can cause a wide variety of embolic manifestations in heart and brain. Our first case was a 27 years old Indian man, with no previous cardiac or cerebral diseases history, presented in emergency department (ED) with sudden onset of acute nausea, vomiting, vertigo and imbalance. The neurological exam (NE) showed cerebellar ataxia. Brain imaging showed acute right cerebellar infarct. The transesophageal echocardiogram (TEE) showed a cardiac mass of the mitral valve that was subsequently defined as PFE by the cardiac MRI. The second case was a 50 years old Arabic male with no previous cardiac or cerebral diseases history, presented at our ED with acute onset of aphasia and right sided weakness. NE confirmed the aphasia and right hemiparesis. Brain imaging showed patchy left middle cerebral artery infarction. TEE showed mobile echo density noted attached to the aortic valve which was consistent with the PFE. Evidence of large vessels occlusion and post infective vegetation were excluded in both cases. We have presented two cases of PFEs associated with cerebral embolic stroke. These clinical cases strengthen the notion that cardiac tumors carried a high risk of cerebral embolic presentation and emphasize the need to suspect their presence at every age in the event of an embolic stroke. Advanced imaging use is important for a correct diagnosis and management.

P670Clincal and echocardiographic characteristics in patients with pathology proven cardiac papillary fibroelastomas

Abstract Background Papillary fibroelastoma (PFE) is a rare benign cardiac tumor with embolic potential. It is most commonly found on cardiac valves but can also be present on non-valvular endocardial surfaces. We sought to better understand the average PFE growth and recurrence rates, and characteristics that might be associated with embolization. Purpose To examine growth and recurrence rates and factors associated with embolization in patients with pathology-proven PFE. Methods Pathology-proven PFEs from 279 patients were identified at a single center between January 1995 and December 2018,and those with at least two transesophageal echocardiograms (TEE) more than 30 days apart were analyzed (n=62). Medical records were retrospectively reviewed for clinical characteristics and outcomes. In addition, intra-operative TEEs were manually reviewed and compared to previous TEEs at our institution to estimate overall size, location, and average PFE growth rates. Results The TEEs from 62 patients with pathology-proven PFE (mean age 65±12 years, female 63%) were reviewed. PFE was discovered incidentally during cardiac surgery in 18% of patients. Most PFEs were located on the aortic valve (AV) (73%) followed by the mitral valve (MV) (16%). The majority of PFEs (71%) presented with a stalk versus a sessile configuration. Average maximal length for PFE (including stalk if present) on the AV was 8.5±3.3 mm, and on the MV 6.8±2.0 mm. The number of PFEs located in other locations was too small to be analyzed for growth. PFE growth varied depending on location and configuration. The PFE growth on the AV was an average increase of 0.47 mm/year, those on the MV was 0.115 mm/year. Valvular PFE was associated with significant functional valve abnormality in 15% of patients, and the abnormality was attributed to the PFE in 9% of patients. There were 25 patients (40%) who had a documented stroke/transient ischemic attack prior to PFE removal (46% located on AV, 44% on MV, 10% other locations). In addition, some patients had other documented embolic complications, such as myocardial infarction, amaurosis fugax, and peripheral embolization to the spleen and kidney. PFE size and location (up-/ downstream of the valve) were not predictive of cardiovascular thromboembolic events. At a median follow up of 2 years, PFEs recurred in the same location in 7.5% of the patients. Conclusion PFE is highly associated with thromboembolic events and seem to grow slowly, with an average growth rate of 0.47mm/year on AV and 0.115mm/year on MV. Growth rates for PFE have not been previously described. PFE can be safely excised with preservation of the native valve and recurs in 7.5% of patients, suggesting that close follow-up maybe warranted.

Cardiac papillary fibroelastoma of a bicuspid aortic valve in an adolescent: A case report.

Cardiac papillary fibroelastomas (CPFE) are exceptional primary benign cardiac tumours affecting the heart valves. We report here the case of a 15-year-old boy in whom echocardiography performed for non-specific chest pain during follow-up for bicuspid aortic valve showed as accidental finding the presence of a round mobile mass without stalk attached on the inferior side of the aortic valve. The mass did not cause any outflow tract obstruction or aortic insufficiency. Electrocardiogram-gated cardiac computed tomography and magnetic resonance imaging allowed to suspect CPFE. Although the patient was asymptomatic, open cardiac surgery with elective surgical resection of the tumour was performed to avoid systematic emboli. Histology confirmed the diagnosis of CPFE. This is an exceptional case of acquired CPFE in a young patient with bicuspid aortic valve. Due to the risk of systemic embolization, aortic or coronary ostium obstruction, elective excision of such lesions is recommended.

Brain abscess complicating ischemic embolic stroke in a patient with cardiac papillary fibroelastoma – Case report and literature review

A 72-year-old man without obvious risk factors initially presented with acute ischemic stroke and fever, without concomitant infection. Broad spectrum antibiotic therapy was initiated. Transthoracic and Transesophageal echocardiography, and cardiac MRI revealed a 20 mm round mass attached to the anterior mitral valve leaflet, suggesting the diagnosis of a benign cardiac tumor or a vegetation. At the site of infarction an abscess of 11 mm in diameter developed 30 days later.The patient underwent surgical valve repair for the prevention of further embolic complications. Histology revealed a cardiac papillary fibroelastoma (PFE). He made complete clinical recovery.Secondary abscess formation after ischemic stroke is rare: 11 other cases have been reported. Because they develop at the site of a previous ischemic infarct, these abscesses usually do not manifest by additional neurologic deficits, making difficult their diagnosis. In most cases a concomitant infection cannot be individualized.Even if these abscesses are a rare entity, patients with cerebral infarct presenting with fever must be closely follow-up with cerebral imaging.Even if PFE is a rare cardiac source of embolic stroke, it should be considered in the differential diagnosis of stroke in a patient where an alternative etiology has not been established.

Papillary Fibroelastoma: A Unique Case of Distant Recurrence

Primary cardiac tumors are rare, with an incidence of 0.001%to 0.28% in autopsy series.(1) The third most common primary tumor, although likely under diagnosed, is the cardiac papillary fibroelastoma (CPF).(2) These tumors are benign, do not exhibit local infiltration on histopathologic examination, and carry a low likelihood of recurrence.(3) After excision, recurrence is rare, with a total of4 reported cases of recurrence. Tamin and colleagues2re-ported 3 cases, occurring at 1, 5, and 6 years after initial surgery, that they described as at or near the previous surgical excision site. Kammerer and associates (3) described a case of mitral valve recurrence after 3 years but hypothesized that incomplete resection in attempt to preserve the valve was the likely underlying mechanism. We report a unique case of recurrent CPF after previous resection,occurring at multiple alternate sites throughout the left ventricle (LV).

Aortic Valve Papillary Fibroelastoma: A Sea Anemone in the Heart, A Case Report

Cardiac papillary fibroelastomas are benign primary cardiac tumors usually arising from the normal components of the endocardium. Grossly they have a characteristic short pedicle and multiple papillary fronds similar to sea anemone. Clinically they have varying presentations ranging from asymptomatic to severe ischemic or embolic complications. We hereby present a case of a middle-aged man who presented with exertional dyspnea and palpitations and was discovered to have papillary fibroelastoma involving the aortic valve. The described case illustrates some of the presenting features, the workup and some of the management modalities that need to be implicated in cases of papillary fibroelastomas.

Mechanical Thrombectomy for Acute Ischemic Stroke Caused by Cardiac Papillary Fibroelastoma: A Case Report

Mechanical Thrombectomy for Acute Ischemic Stroke Caused by Cardiac Papillary Fibroelastoma: A Case Report

Cardiac papillary fibroelastoma: Report of a surgical series

IntroductionPapillary fibroelastoma (PFE) is a rare primary benign tumor of cardiac origin that predominantly affects the cardiac valves. Although most patients are asymptomatic, serious complications may result given their propensity for embolization. Advances in imaging technology have enabled earlier detection and more accurate characterization of these tumors. We report a case series, describing clinical presentation, treatment and outcome. MethodsInstitutional records of a tertiary center between 1997 and 2015 were reviewed for all patients diagnosed with PFE treated surgically and confirmed histologically. Demographic and clinical characteristics, echocardiography findings and treatment modalities were analyzed and recurrence at follow-up was studied. ResultsA total of 26 patients (69% male), aged 54±18 years, had a PFE. Clinically, PFE presented with neurologic deficits in eight cases and was asymptomatic in 65.4%. The mitral valve surface was the predominant tumor location (53.8%), followed by the aortic valve (34.6%). Tumor size ranged between 3 mm and 22 mm, 26.9% had a pedicle and 42.4% were mobile. All patients were treated successfully by complete resection, isolated in 88.5% and with valve repair in three cases. No other cardiac procedure was performed concomitantly and there were no major postoperative complications. Median follow-up was 61±49 months and no tumor recurrence or embolic events were documented. ConclusionsFibroelastomas are generally small, single and detected by chance during routine imaging exams. Complete surgical resection of the tumor has an excellent prognosis and appears to be a good strategy.

Cardiac papillary fibroelastoma: So small and yet so dangerous

Cardiac papillary fibroelastoma: So small and yet so dangerous

Cardiac papillary fibroelastoma: Report of a surgical series

Cardiac papillary fibroelastoma: Report of a surgical series

Multiple Cardiac Papillary Fibroelastomas — Are They Really Rare or Underdiagnosed?

Multiple Cardiac Papillary Fibroelastomas — Are They Really Rare or Underdiagnosed?

A rare case of papillary fibroelastoma presenting as a left ventricular mass

Cardiac papillary fibroelastomas (CPFE) are rare findings in patients who present to assessment of chest pain. We present a case of a 59 year old female, symptomatic of atypical chest pain, whom was found to have a left ventricular mass, later confirmed to be a papillary fibroelastoma on histology following surgical excision.Cardiac papillary fibroelastomas are an infrequent finding within cardiac tumours, and more commonly involve cardiac valves. Symptoms are often related to their mass effect, and vary with regards to their position in the heart. CPFEs are benign in nature, however pose a cardio-embolic risk, and often require surgical excisionThis paper presents a summary of the 39 cases of CPFEs involving the left ventricle published previously in English literature; together with summary of symptoms, and surgical approach. The aim of this paper is to bring to light the various presentations of CPFEs, together with a summary of previously performed management options.

A mobile mass on the tricuspid valve in a young female: The critical distinction between the vegetation and cardiac tumor

Papillary fibroelastomas (PFEs) are benign cardiac tumors with high embolic tendency, and usually originate from the cardiac valve. We report the case of a 34-year-old woman who experienced progressive dyspnea and fatigue. Transthoracic echocardiography revealed a pediculated, homogenous echo-dense, and highly mobile mass attached to the tricuspid valve. The patient was diagnosed with cardiac PFE because of its size and mobility, and surgical resection was scheduled.

Papillary fibroelastoma diagnosed through multimodality cardiac imaging: a rare tumour in an uncommon location with review of literature

We describe the case of a woman presenting with transient ischaemic attack, who was found to have a papillary fibroelastoma arising from the aortic wall, an extremely rare location. We...